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gallstone disease is very common in the united states and western europe with a prevalence of around 10% . however while mirizzi syndrome means compression of the common bile duct by a jammed gallstone in the cystic duct leading to biliary obstruction , bouveret 's syndrome causes gastric outlet obstruction by a gallstone that enters the small bowel through a bilioenteric fistula and gets impacted in the duodenum or stomach [ 1 , 2 ] . a 77-year - old woman was admitted to the accident and emergency department complaining of postprandial abdominal pain for the last 10 days . she had noted a single spike of fever ( 38.5c ) during the previous week and had been avoiding food because of postprandial abdominal pain . vital signs were normal , the abdomen was soft and non - tender with normal bowel sounds . liver enzymes [ asat : 114 u / l ( normal < 40 u / l ) ; alat : 131 u / l ( normal < 55 u / l ) ] and c - reactive protein [ 63 mg / l ( normal < 8 mg / l ) ] were elevated . transabdominal ultrasonography showed marked pneumobilia , a non - detectable gallbladder and dilation of the intrahepatic biliary tracts ( fig . 1 ) . suspecting bouveret 's syndrome , we performed upper endoscopy and found an impacted gallstone in the duodenal bulb ( fig . videos 1 and 2 ; for all online suppl . material , see www.karger.com/doi/10.1159/000364818 ) . despite disproportion between the diameter of the stone and the pylorus , we tried to extract the gallstone endoscopically through the pylorus , but did not succeed ( fig . bouveret 's syndrome was first described in two case reports by lon bouveret in 1896 . the current literature only reports single cases and few very small case series with up to 6 patients with bouveret 's syndrome . local perforation of the gallbladder wall can occur because of chronic inflammation and increased pressure to the gallbladder wall . this can cause local necrosis and lead to fistula formation and migration of a gallstone to the duodenum . bouveret 's syndrome occurs primarily in elderly women , which is likely due to the higher incidence of gallstones in women . the bilioenteric fistula stays asymptomatic in 4050% of cases until the gallstones pass from the gallbladder to the duodenum . interestingly , the symptoms of gastric outlet obstruction are often unspecific , with epigastric pain , nausea , vomiting and subileus [ 5 , 6 , 7 ] . sometimes fever , gastrointestinal bleeding and rarely icterus or signs of cholecystitis occur [ 8 , 9 ] . one should consider bouveret 's syndrome in patients with pneumobilia , without a history of biliary surgery or recent endoscopic retrograde cholangiopancreatography and with unspecific obstructive symptoms . pneumobilia is present in nearly half of the cases , especially when the gallbladder is not well detectable . plain abdominal radiography may show the typical triad of pneumobilia , small bowel ileus and an x - ray - positive ectopic stone that is pathognomonic for bouveret 's syndrome . this so - called rigler 's triad was first described by leo g. rigler [ 5 , 10 ] . even though this triad is highly specific for bouveret 's syndrome endoscopy is the first - line treatment approach , in particular because of old age and comorbidities in these patients and the greater perioperative risk of surgical treatment . however , endoscopic treatment has a success rate of only 10% . if unsuccessful as in our case , patients have to undergo surgery for stone extraction . bouveret 's syndrome should be suspected in any patient who presents with pneumobilia without recent endoscopic retrograde cholangiopancreatography or abdominal biliary surgery .

the case of a 77-year - old woman with symptoms of gastric outlet obstruction is presented . transabdominal ultrasonography findings were suspicious of bouveret 's syndrome . upper endoscopy confirmed this diagnosis . bouveret 's syndrome is a rare complication of gallstone disease caused by a bilioenteric fistula leading to gastric outlet obstruction by a gallstone and should be suspected in any patient who presents with pneumobilia without recent endoscopic retrograde cholangiopancreatography or biliary surgery .

cardiac myxoma is reported in the literature as the most common primary cardiac tumor , and the left atrium ( la ) is the most common tumor location . clinical pathologic studies have identified two specific types of cardiac myxoma , type 1 and type 2 . a soft and villous like outer surface characterizes type 1 and is most likely to give rise to embolization to various organs . long - term prognosis after total resection is usually exceptional with sporadic cases of tumor recurrence . this case report describes the late occurrence of a cerebral vascular ischemic event in a 46-year - old female after surgical resection of a left atrial myxoma . a 46-year - old female with a history of dyslipidemia and nephrolithiasis presented to a urology clinic with complaint of recurrent pelvic pain ( preoperative day 7 ) . the patient was evaluated and referred for an abdominal and pelvic computer tomography ( ct ) for further evaluation . ct showed a 3.49 2.74-cm mass in the region of the left adnexa , suggestive of an ovarian cyst ( preoperative day 6 ) . the ct of the urinary tract was normal with no evidence of calculi , masses , or obstructive uropathy . ct imaging at the level of the lower thorax incidentally showed a low - density mass in the la , which measured 2.88 2.94 cm , suggestive of a possible myxoma , lymphoma , or thrombus . transthoracic echocardiogram was done ( preoperative day 5 ) and showed a large - sized ( 3.6 2.9 cm ) mobile , heterogeneous mass in the la attached at the level of the fossa ovalis in the atrial septal wall ( fig . 1 ) . la was mildly dilated and other chambers were within normal size and function with a calculated left ventricular ejection fraction of 63% . cardiothoracic surgery was consulted and the patient was subsequently scheduled for the atrial mass resection 5 days later ( postoperative day 0 ) . a minimally invasive removal of the left atrial mass through mini right thoracotomy at the fourth intercostal space was performed . three days after discharge ( postoperative day 3 ) , the patient developed progressive back pain and bilateral pleuritic chest pain and was admitted to another hospital . her chest x - ray showed significant bilateral pleural effusions ; costophrenic angles completely obscured , suggestive of pneumonia complicated with parapneumonic effusion ( fig . a total of 50 ml of serosanginous fluid was removed and the initial pleural gram stain was continuously negative . additional pleural fluid studies showed 2,300 wbc , 20,000 rbc , ldh 880 , glucose 116 and a tp fluid / serum of 0.6 , and the patient was treated with piperacillin / tazobactam , vancomycin , azithromycin and discharged on levofloxaxin after 3 days ( postoperative day 6 ) for completion of antibiotic therapy . four days later ( postoperative day 11 ) , while at home the patient developed slurred speech and right - sided weakness . there was hyperdensity of the left middle cerebral artery ( mca ) at the distal m1/proximal m2 level . a small area of faintly diminished attenuation involving the left lentiform nucleus that is compatible with evolving infarct was also observed . ct angiogram and ct perfusion imaging showed left mca and m1 occlusion with hypoperfusion of the of the left mca territory ( fig . 3 ) with a national institute of health stoke scale ( nihss ) score of 11 . due to the recent surgery , she underwent mechanical thrombectomy with a merci retriever within 8 h from the time of initial presentation . on the next day following the thrombectomy ( postoperative day 13 ) , the patient 's right - sided motor function was fully recovered . the reported cases of cardiac emboli leading to cerebral ischemia have primarily occurred from atrial fibrillation , patent foramen ovale , followed by myxomas and lipomas . cardiac myxomas are benign primary tumors of mesenchymal origin , with a 2:1 female - to - male ratio and usually occur during the third to sixth decades of life . signs and symptoms leading to cardiac myxomas include normal to abnormal auscultation findings on physical examination ( tumor plops ) , unexplained syncopal episode and in most cases sudden cerebral ischemia . two specific types of myxoma are known based on pathologic findings , type 1 and type 2 . type 1 is generally soft in consistency and has an irregular villous outer surface that makes it morphologically prone to embolization . the annual incidence of myxoma in the general population is less than 1 in 1 million . the la is the most commonly reported location , and transthoracic echocardiogram is the most prevalent method of diagnosis . the uniqueness of this case is the occurrence of cerebral ischemia some days after surgical resection of myxoma . according to the literature , in very rare instances stroke this 46-year - old female had no documented history of atrial fibrillation or prior history of cerebral vascular accident . in addition , the patient had neither evidence nor history of left ventricle mural thrombus resulting from previous myocardial infarction as those conditions can increase susceptibility for embolic stroke event . calcified and infected valves disease are other culprits reported in the literature as possible causes of embolic neurovascular events which were not reported in this patient 's medical history . the patient underwent mechanical embolectomy , an accepted alternative in patients with absolute thrombolytic contraindication . prior to the procedure , a cerebral angiogram showed a complete occlusion of the left mca , mid and distal m1 segment with a thrombolysis in cerebral infarction ( tici ) score equal to 0 . a postembolectomy angiogram performed through the left internal carotid artery demonstrated a cerebral flow improvement from a tici score of 0 to a tici score of 2a . a potential complication with the merci retriever procedure includes vessels perforation , intramural arterial dissection , or embolization of a previously uninvolved territory . short- and long - term outcome of myxoma - related stroke , prior to surgical resection of the tumor is usually favorable . most patients recover fully with minimal residual deficits . in our case , although rare in incidence , the stroke occurred late following the mechanical resection of the tumor and also resulted in a good outcome with no residual neurological sequelae . within 3 months of rehabilitation therapy , possible etiology includes : residual cardiac tumor debris , circulating thrombotic fragments from tumor washed out during the cardiac procedure , thrombus formed during the surgical procedure or other combinations of thrombogenic risk factors . more data from observational or investigational studies are needed to establish etiology of a late ischemic cerebral event after surgical resection of myxomas that may provide better guidance on perioperative management of the disease .

abstractatrial myxoma is one of the most common primary cardiac tumors reported in the literature . in very rare instances , stroke has been the sequelae after a myxomatous tumor resection . we report this unique case of late ischemic cerebral event in a 46-year - old female some days after resection of a left atrial myxoma .

to follow the changes in the electroretinograms ( ergs ) in a patient with primary intraocular lymphoma ( piol ) who had a complete remission after chemotherapy . ergs were recorded in a 41-year - old woman with piol during and after complete remission with chemotherapy . the patient was diagnosed with piol from both the ocular signs and the medical history of cranial lymphoma . the amplitudes of the bright white flash b - waves were smaller than the a - waves , resulting in a negative type six weeks after the beginning of chemotherapy , the ocular changes had resolved , and the ergs , although not of the negative type , still showed signs that the amplitude had not returned to normal levels . the negative type ergs indicated that the inner retina had been damaged to a greater extent than the outer retina . in the convalescent stage , although only one case was studied , we suggest that ergs can be used to evaluate and follow patients with a piol . a primary intraocular lymphoma ( piol ) is a subtype of primary non - hodgkin s lymphomas of the central nervous system in which the retina , vitreous , and/or optic nerve head may be involved.13 patients with a piol typically present with a form of uveitis and vitritis that is unresponsive to corticosteroid therapy , thus the so - called uveitis masquerade syndrome . this can occur together with , or independently of , a primary cerebral nervous system lymphoma . the disease has a poor prognosis , and patients normally die as a result the growth of the cerebral lymphoma with a mean survival of 20 months from the diagnosis of the ocular disorder.4 as best we know , electroretinography ( erg ) has not been used to follow the changes in retinal function during the course of piol . we present the changes in the ergs during and after a complete remission of piol with chemotherapy . the patient , a 41-year - old woman , had been diagnosed with a primary intracranial malignant lymphoma ( non - hodgkins , diffuse medium - sized b cell lymphoma ) for three years . she presented with a 3-week history of blurred vision and photophobia , primarily in her right eye . slit - lamp examination showed mild vitritis in the right eye , and ophthalmoscopy showed no abnormalities . after 3 weeks of observation the right fundus had developed whitish exudates , massive subretinal infiltration from a macular lesion , and slight swelling of the optic disc . cells were detected in the anterior chamber , but the fundus of the left eye was normal ( figure 1 ) . the goldmann visual field of the right eye had a dense central relative scotoma , but was almost normal in the left eye ( figure 2 ) . full - field ergs were recorded according to international society for clinical electrophysiology of vision ( iscev ) standards.5 the scotopic , bright white , cone , and 30 hz flicker ergs 3 weeks after the initial visit are shown in figure 3 . the amplitudes of the different erg components were reduced in both eyes , but to a greater extent in the right eye . the amplitude of the bright white flash b - waves was distinctly reduced , and was smaller than the a - waves , resulting in the negative type this indicated that the functional depression was greater in the inner retina than the outer retina.6 focal macular ergs were recorded to assess macular function as described in miyake et al.7 the erg from the right eye was almost nonrecordable , and that of left eye was about one - half the normal amplitude ( figure 3 ; focal macular ergs ) . because of the rapid decrease in the visual acuity of the right eye to 20/600 , and the increasing retinal infiltration and exudations since the previous examination , diagnostic vitrectomy was performed ( figure 4 ) . cytological examination of the vitreal specimen did not show any abnormal lymphocytes or monocytes , in addition to which flow cytometry did not show monoclonality . although the results of the cytological examinations were negative , a primary intraocular lymphoma was strongly suspected . eight weeks after the first visit , chemotherapy with cyclophosphamide , cytosine arabinoside , etoposide , and dexamethasone was started . four days after the treatment , the visual symptoms and ophthalmoscopic appearance of the fundus improved . six weeks after beginning chemotherapy , her best - corrected visual acuity improved to 20/20 in both eyes . the ocular lesions were completely resolved , leaving atrophic pigmentary changes ( figure 5 ) . a relative central scotoma still remained in the right eye , but the visual field sensitivity had improved . the cells in the anterior chamber of the left eye had also disappeared . although the rod and cone components of the full - field ergs had improved after chemotherapy , the amplitudes had still not reached normal limits ( figure 6 ) . the recovery of the b - wave was greater than that of the a - wave in the bright flash erg , and so the waveform was not of the negative type at this point . three years after the therapy , the patient had no evidence of systemic disease or recurrence of the piol . the ergs were greatly improved but the amplitudes had still not reached the normal range . we have followed a patient with a piol by electroretinography , who went into complete remission after chemotherapy . we found that the bright flash ergs were the negative - type ergs before chemotherapy . these erg findings suggest that the inner retina was damaged more than the outer retina . as a differential diagnosis however , from the history of intracranial malignant lymphoma , and the clinical signs , we diagnosed the patient as having a piol , even though the vitreous biopsy did not detect any malignant cells or a change in the il-10/il-6 ratio . it is known that the interpretation of vitreous specimens is sometimes difficult , and more than 40% of vitrectomy specimens may be nondiagnostic . 8,3 after chemotherapy , the b - wave recovered more quickly than the a - wave . these results indicate that the inner retinal damage was transient , and may have resulted from secondary inflammation due to the presence of tumor cells . in the convalescent stage , when the ocular manifestations were resolved , both full - field and focal macular ergs were still not fully recovered . earlier histopathological studies of piol s have mentioned an infiltration of malignant lymphoma cells into the subretinal space , which would be detected as whitish exudates , and could also lead to damage to the retina.9,10 in our case , a marked prolongation of the focal macular erg in the right eye may have been as a result of photoreceptor damage caused by just such subretinal infiltration . in conclusion , we recorded ergs from a patient whose ocular findings and history indicated that she had piol . the full - field ergs were reduced in the right eye , showing a the negative - type erg . after chemotherapy although our conclusions are based on only one case , we recommend that ergs be used to diagnose and follow patients with a piol .

purposeto follow the changes in the electroretinograms ( ergs ) in a patient with primary intraocular lymphoma ( piol ) who had a complete remission after chemotherapy.methodsergs were recorded in a 41-year - old woman with piol during and after complete remission with chemotherapy . the patient was diagnosed with piol from both the ocular signs and the medical history of cranial lymphoma.resultsthe ergs were depressed in the subject . the amplitudes of the bright white flash b - waves were smaller than the a - waves , resulting in a negative type erg . six weeks after the beginning of chemotherapy , the ocular changes had resolved , and the ergs , although not of the negative type , still showed signs that the amplitude had not returned to normal levels.conclusionthe negative type ergs indicated that the inner retina had been damaged to a greater extent than the outer retina . in the convalescent stage , when the ocular manifestations were resolved , the ergs were still not fully recovered . although only one case was studied , we suggest that ergs can be used to evaluate and follow patients with a piol .

primary biliary cirrhosis ( pbc ) is a chronic cholestatic liver disease recognized at histology as chronic nonsuppurative destructive cholangitis with an autoimmune pathogenesis supported by th1 or th17 cells producing ifn- or il-17 [ 1 , 2 ] . several inflammatory cell populations , including t and b cells , are found around the affected intrahepatic bile ducts , and chemokines are believed to play a pivotal role for the infiltration of inflammatory cells . a better understanding of the role of specific chemokines in liver injury is ancillary to understanding the molecular mechanisms regulating the autoimmunity process and the observed patterns of chemokine expression in normal and pbc liver are illustrated in table 1 . in our recent experiments , we cultured epcam - positive cells ( i.e. , biliary epithelial cells and bec ) isolated by immunobeads from explanted liver tissue and examined the production of chemokines by protein array following the stimulation by inflammatory cytokines or toll - like receptor ( tlr ) ligands . our data illustrated that bec produce proinflammatory chemokines such as cxcl1 , cxcl5 , cxcl6 , and cxcl8 without any specific stimulation as shown in figure 1 . on the other hand , bec challenged with a tlr3 ligand ( poly i : c ) manifest a th1 shift and the production of ccl3 , ccl4 , ccl5 , and cxcl10 . such production of th1 chemokines was further prompted by the interaction between cd40 on bec and cd154 on liver infiltrating lymphocytes . taken altogether , the evidence support the observation that bec induces a proinflammatory environment in the absence of innate immunity stimulation and induces th1-sifted environment when such stimulation is present . fractalkine is characterized as a type-1 transmembrane molecule with the chemokine domain tethered by a 241-amino acid glycosylated stalk , a 19-amino acid transmembrane region , and 37-amino acid intracellular tail . the surface - expressed transmembrane fractalkine induces the firm adhesion of leukocytes expressing its receptor cx3cr1 . after shedding by the disintegrins and metalloproteinases ( adam ) 10 and 17 fractalkine is upregulated by inflammation cytokines such as tnf- or ifn- , it has been proposed to contribute to inflammatory diseases by promoting the transmigration of cx3cr1-expressing cells to inflamed tissues in crohn disease , rheumatoid arthritis , atherosclerosis , systemic lupus erythematosus , and most recently pbc . cx3cr1 is expressed on natural killer cells , monocytes , macrophages , mucosal dendritic cells , cd8 t cells , and a subset of effector - memory cd4 t cells [ 11 , 12 ] . human th1 cells express high levels of cx3cr1 mrna , different from polarized th2 cells [ 13 , 14 ] . fractalkine is expressed in limited amounts in the normal human liver , particularly near branches of the hepatic artery and in small bile ducts located at the interface between the portal tract and the hepatic lobule . in the case of acute or chronic viral hepatitis , fractalkine is detected in the areas of necrosis and inflammatory infiltration and also at the interface between the expanded portal tract and the regenerating nodule . regenerating epithelial cells of the ductular reaction are also positive for fractalkine . in kidney allograft transplantation , fractalkine is expressed in renal tubular epithelial cells , and the expression is upregulated by tnf- , the recognized key proinflammatory cytokine in acute rejection . the cd4 and cd8 t cells expressing cx3cr1 predominantly produce ifn- and tnf- , and these t cells infiltrate the synovium in patients with rheumatoid arthritis . in inflammatory bowel disease ( ibd ) , intestinal microvascular endothelial cells produce high amounts of fractalkine , and ibd mucosa as well as periphery contained significantly more cx3cr1 + cells than control . fractalkine is a major contributor to t- and monocytic - cell adhesion to endothelial cells . in hcv infection , it is unclear whether cx3cr1 positive cells are protective or trigger disease [ 2025 ] . fractalkine is peripherally expressed dominantly in patients with pbc , and is upregulated in bec of the pbc liver . cx3cr1 is expressed on infiltrating lymphocytes in the portal tracts and on intraepithelial t cells of injured bile ducts . bec manifesting senescent features in damaged small bile ducts also overexpress fractalkine . as previously introduced , in our recent work , we separated bec as epcam positive and endothelial cells as cd31 positive by immunobeads and evaluated the production of fractalkine as chemokine by elisa . figure 2(a ) illustrates the elevated production of fractalkine by endothelial cells challenged with tlr3 ligand ( poly i : c ) or tlr4 ligand ( lps ) . conversely , bec did not produce fractalkine with any other tlr ligand stimulation ( figure 2(b ) ) , and this was not reversed with the addition of established inflammatory cytokines such as tnf- or ifn-. further , we investigated the production of fractalkine following the interaction between bec or endothelial cells and liver infiltrating lymphocytes . as shown in figure 3 , mononuclear cells adhered with higher affinity to bec compared to endothelial cells in the tlr4 ligand ( lps ) stimulation , and this adherence was increased more in pbc than in other control diseases . fractalkine works to modulate inflammation in the bec of pbc , thus suggesting that novel therapies to block fractalkine induced environment may prove beneficial . based on our data , we propose a working model on the role of fractalkine as chemokine or cell adhesion molecule by vascular endothelial cells and bec , summarized in figure 4 . first , fractalkine as chemokine from vascular endothelial cells stimulated via tlr3 or tlr4 induce cx3cr1 positive monocytes or nk cells . second , fractalkine as cell adhesion molecule from tlr4-stimulated bec recruit cx3cr1 positive cells around target cells . this mechanism may trigger the onset of chronic nonsuppurative destructive cholangitis and autoimmune mechanism perpetuating the cholangitis . we further submit that th1 chemokines produced by bec stimulated from tlr3 are important contributors to the autoimmune mechanism .

primary biliary cirrhosis ( pbc ) is characterized by the autoimmune injury of small intrahepatic bile duct . on this basis , it has been suggested that the targeted biliary epithelial cells ( bec ) play an active role in the perpetuation of autoimmunity by attracting immune cells via chemokine secretion . to address this issue , we challenged bec using multiple toll - like receptor ( tlr ) ligands as well as autologous liver infiltrating mononuclear cells ( lmnc ) with subsequent measurement of bec phenotype and chemokine production and lmnc chemotaxis by quantifying specific chemokines , specially cx3cl1 ( fractalkine ) . we submit the hypothesis that bec are in fact the innocent victims of the autoimmune injury and that the adaptive immune response is critical in pbc .

in april 2006 , a 76-year - old man was admitted to the emergency department of pontchaillou university hospital , rennes , france , with acute onset of fever , lumbar pain , and dyspnea . ( 90.6% neutrophils ) , c - reactive protein 216 mg / l ( normal value < 5 mg / l ) , and arterial oxygen partial pressure 67 mm hg ( room air ) . the patient was treated with amoxicillin - clavulanate and ofloxacin . on day 3 , acute respiratory distress syndrome developed , and the patient required mechanical ventilation . computed tomographic scan of the thorax showed bilateral alveolo - interstitial infiltrates ( figure ) . a bronchoalveolar lavage ( bal ) was performed ; no pyogenic bacteria , l. pneumophila , mycobacterium tuberculosis , pneumocystis jiroveci , or aspergillus sp . negative results were obtained in m. tuberculosis pcr and immunofluorescence assays ( ifas ) for p. jiroveci and l. pneumophila . the result of a pcr for human herpesviruses , which used herpes consensus identification , was negative . results of testing for respiratory syncytial virus , influenza virus , parainfluenza virus , and adenovirus by ifa and elisa , which used specific monoclonal antibody , were all negative . in addition , serologic test results for l. pneumophila , mycoplasma pneumoniae , chlamydophila pneumoniae , c. psittaci , coxiella burnetti , and hiv were negative . three sets of blood cultures taken at the time of hospital admission remained sterile . test results for antinuclear factors , antiglomerular basement - membrane antibodies , and antineutrophil cytoplasmic antibodies were negative . bal was positive in 2 enteroviral pcr assays that used ev1 primer from rotbart et al . ( 3 ) and real - time pcr with primers and probe adapted from verstrepen et al . in addition , on day 3 , mrc-5 cell culture showed a specific cytopathic effect , which was confirmed as enterovirus by indirect ifa that used enterovirus mouse monoclonal antibody ( novocastra , newcastle , uk ) and fluorescein isothiocyanate conjugated affinipure goat anti - mouse ( jackson immuno - research , west grove , pa , usa ) . results of real - time pcr and cell culture ( mrc-5 and llc - mk2 ) were also positive for enterovirus on bal performed on days 8 and 14 . kinetics analysis in real - time pcr showed a 100-fold decrease in viral load by comparison of cycle thresholds between day 8 and day 14 bal . serologic testing for enterovirus showed an 8-fold increase in enterovirus antibody titration by complement fixation between days 3 and 11 . immunoglobulin m to echovirus / coxsackievirus was detected by elisa in serum on day 11 ( genzyme , virotech , chilly mazarin , france ) . no drug is currently approved for the treatment of enterovirus infection . pleconaril may be of value in severe enteroviral infections ( 5 ) but is no longer available because licensure was not pursued . histologic examination of postmortem pulmonary biopsy specimen showed diffuse alveolar damage and fibrosis , real - time pcr detected enterovirus , and viral cultures were negative . the enterovirus isolated on the day-3 bal was identified as cva-16 by partial sequencing of the vp1 region that encompasses the bc loop . this region was amplified with primers 292 as sense primer and 222 as antisense primer ( 6 ) . bankit 845096-dq993173 until definitive number assigned ) were aligned with the corresponding region in genbank . comparison of a 338-nt sequence with that of the cva-16 reference strain ( prototype brcr ) and the ev 71 reference strain ( prototype g-10 ) showed nucleotide identity rates of 78.6% and 64.6% , respectively . on subsequent questioning , the patient s wife reported that he had had close contact with his granddaughter , who had active hfmd , while in the united kingdom during the week before he was admitted . although a large hfmd outbreak occurred in the united kingdom in 1994 ( 7 ) , no enterovirus outbreaks had been reported at the time of the patient s admission . during the 1994 uk outbreak , cva was isolated in 28 of 40 patients ( type cva-16 in 21 patients and cva-10 in 7 patients ) , but secondary cases in family members were rare and no case of pneumonitis or death was reported . fatal cva-16 infection has been described infrequently in children who had hfmd associated with myocarditis ( 8) . we report a fatal cva-16 infection associated with pneumonitis in an adult ; to our knowledge , this is the first such report . our patient had neither myocarditis nor left ventricular dysfunction , as demonstrated by pulmonary artery catheterization results , echocardiograph results , and serum troponin levels . in 2003 , 7 fatal cases of hfmd in children were reported in singapore ( 9 ) . the cva-16 strain isolated from our patient had a low percentage of nucleotide identity with the reference strain ( 78.6% ) ; a threshold of 90% is usually required to define strain homology . a strain similar to that from our patient was circulating in china from 1999 through 2004 ( 98% nucleotide identity ; genbank accession no . ay821798 ) and was isolated from fecal samples of children with hfmd or suspected enterovirus infection ( 10 ) . this strain was associated with local yearly outbreaks in which only a few cases of neurologic disease and no deaths were reported . according to phylogenetic analysis based on vp4 207-bp nucleotide sequence , the authors concluded that 3 genetic lineages were circulating in asia at that time and suggested that the same tendency may apply in other continents ( 10 ) . we report what we believe to be the first case of cva-16 pneumonitis in an adult , with fatal outcome . preliminary sequence analysis revealed a low rate of homology between the cva-16 strain we isolated and those previously published , which suggests that a new , more virulent , strain of cva-16 could be emerging . to compare the sequences to those published in genbank , we are sequencing the complete part of the genome encoding the vp1 capsid protein .

coxsackievirus a-16 ( cva-16 ) is the agent of hand , foot , and mouth disease in children . we report a case of fatal pneumonitis in an adult due to a cva-16 strain with a low ( 78.6% ) rate of sequence homology with the reference strain . a modified , more virulent , strain of cva-16 could be emerging .

in the present issue of critical care , linder and colleagues present a new study in which they assess the clinical importance of serial measurements of heparin - binding protein ( hbp ) plasma levels in critically ill septic and nonseptic patients . they found that hbp plasma levels are significantly higher in patients with severe sepsis and septic shock in comparison with patients with a nonseptic critical condition . the authors also demonstrated that hbp plasma levels obtained at admission to the icu and during the last individual sampling are higher in nonsurvivors as compared with survivors in both the septic group and the whole study group . moreover , the high baseline hbp plasma levels in septic patients were associated with an increased 28-day mortality rate . altogether , these results indicate that serial hbp measurements might be very helpful in stratification of icu patients . however , there are some issues that should be raised before the study results can be translated into the daily routine . the study was designed to compare hbp plasma levels in patients with severe sepsis and septic shock with levels in patients with noninfectious critical illness . however , these two groups of patients were not equal in size . also , a significant proportion of patients from the nonseptic group developed infection and was treated with antibiotics . this raises the question of whether the comparison between septic and nonseptic icu patients is hindered by this treatment . nevertheless , the study design is logical because finding a biomarker that would predict development of any shock state is highly desirable . previous studies have demonstrated significant predictive values of elevated levels of lactate , cortisol and il-6 in the blood of patients with different etiologies of shock [ 2 - 4 ] . however , these biomarkers have some limitations : lactate levels are less influenced by arterial sampling , endogenous cortisol levels are downregulated by corticosteroids used in the treatment of septic shock or by relative adrenal insufficiency , and il-6 analysis is not generally available in regular hospital laboratories . other routinely measured biomarkers - such as procalcitonin , c - reactive protein , neutrophil and lymphocyte counts - have only a limited value in prognostic scoring of the critically ill patients and are mostly used in the early diagnostics of bacterial etiology of critical illness [ 6 - 8 ] . notably , hbp has antibacterial activity , which includes a direct microbicidal effect , and also helps neutrophils to migrate into the focus of infection . similarly to hbp , c - reactive protein and il-6 play an active role during the immune responses against infections : c - reactive protein is an inflammation opsonin , and the major function of il-6 is amplification as well as downregulation of inflammatory reactions , depending on the concentrations . regarding procalcitonin , there are only limited data from animal studies - which demonstrate that immunoneutralization of procalcitonin improved survival in experimental porcine sepsis . additionally , elevated cortisol levels in peripheral blood during sepsis are considered an integral part of compensatory anti - inflammatory response syndrome , leading to downregulation of exaggerated systemic immune responses . from the functional point of view , in comparison with the abovementioned biomarkers , hbp therefore plays the most complex role in severe sepsis and septic shock - highlighting its potential for clinical use . in conclusion , the serial measurements of hbp plasma levels can be a useful tool for close monitoring of critically ill septic patients . however , availability of a routine diagnostic method for hbp analysis is essential to confirm these interesting data . the authors thank dr e david mcintosh ( imperial college , london , uk ) for help with the manuscript .

heparin - binding protein ( hbp ) , also known as azurocidin , has multiple functions in the inflammatory process , especially during severe infections . beside its antimicrobial properties , hbp may induce vascular leakage leading to extravascular efflux , which is an important pathophysiologic event in the development of septic shock . not surprisingly , high hbp plasma levels are found in severe sepsis patients and in septic shock patients as well as in serious infections associated with endothelial damage . in the present issue of critical care , linder and colleagues demonstrate new aspects of hbp daily monitoring in icu patients . the authors observed that high hbp plasma levels are associated with an increased mortality rate in both septic and nonseptic critically ill patients , indicating that hbp may be a reliable prognostic biomarker . however , there are some limitations hindering rapid translation of these interesting findings into the daily routine . first , the group of nonseptic critically ill patients ( n = 28 ) enrolled in the study was rather small as compared with the septic group ( n = 151 ) . moreover , 50% of nonseptic patients developed infection while hospitalized in the icu , and to classify them as truly nonseptic patients is problematic . second , there is a lack of a routine diagnostic method for hbp analysis . nevertheless , if the results of the present study are validated in large clinical trials in different icu populations and cost - effectiveness data become available , the serial hbp measurements will have a promising future .

. it can be either localized or generalized , localized forms tending toward chronicity and generalized forms varying between acute , sub - acute or life - threatening course . generalized pustular psoriasis is an uncommon variant where the eruption has sterile pustules as its central feature . its relationship with psoriasis is clear as the patient may have plaque - type psoriasis prior to or after the onset of pustular psoriasis . there are several documented triggers of pustular psoriasis , the earliest of which was von zumbusch 's original patient who was provoked by irritating topical therapy ( coal tar , anthralin ) . in addition , physiological states like pregnancy , factors such as infection , hypocalcemia and hypoparathyroidism have also been implicated in its causation . we present a case of acute generalized pustular psoriasis exacerbated by telmisartan , which is an antihypertensive of the angiotensin ii receptor blocker category . to the best of our knowledge , a 50-year - old male patient , a known case of pustular psoriasis , well controlled on oral methotrexate presented with sudden exacerbation with fresh crops of pustules . the patient was a known hypertensive on 10 mg amlodipine per day for 4 years . on having a recorded persistently high blood pressure of 160/100 mm hg even after enforcing strict diet restrictions and exercise , the patient was prescribed telmisartan by his physician . the patient reviewed with the general medicine department 2 weeks later with no complaints and normotensive status . however , 1 week later , the patient visited the dermatology out - patient department ( opd ) with complaints of severe burning sensation and several new groups of lesions occurring over his arms and legs . on examination , multiple , well - defined , discrete , pin - point pustules [ figure 1 ] were seen over the bilateral arms , forearms , thighs and lower legs , with involvement of the bilateral axillae as well . the pustules were present over an erythematous base , seen in clusters , more over the flexor surfaces , and the surrounding skin showed multiple , well - defined areas of exfoliation [ figure 2 ] . a noticeable gradual regression of new lesions and healing of existing lesions was observed within few days of treatment . the resolution was completed by 1 month , following which the asymptomatic patient was discharged with addition of oral prazosin 2.5 mg after which there was neither exacerbation of older lesions nor provocation of new pustules of psoriasis . the patient had a symptom - free period of 6 months after which he was inadvertently prescribed telmisartan by another physician for control of hypertension . three weeks later , he visited the dermatology opd with complaints of similar pustules all over the body , occurring in crops , associated with photosensitivity and severe burning pain over the lesions . on admission and examination , multiple , discrete , pin - point pustules over an erythematous base with crusting over the surface of few pustules were seen over the right thigh , right side of the neck and bilateral axillae . multiple , discrete , hyperpigmented papules , erythematous erosions with patches of exfoliation were seen over the chest , abdomen , back , arms , forearms and lower legs bilaterally , suggestive of a previous crop of pustules in the recent past . the present episode also responded well to cessation of telmisartan with complete resolution of all symptoms within 1 week . normotensive status was obtained and maintained with increased dose of prazosin from 2.5 to 5 mg . psoriasis is influenced by several environmental and other risk factors such as alcohol , smoking , emotional stress , sunlight , infections and drugs . generalized pustular psoriasis is the most severe type of psoriasis and is characterized by diffuse erythema , scattering of pustules , high - grade fever and malaise , which were sudden in onset . . however , the drugs provoking pustulation among psoriatics are slightly different and listed below [ table 1 ] . drugs provoking pustular psoriasis there are innumerable new drugs being discovered and used in various fields of medicine , the effects of which on pustular psoriasis are unknown . the list of drugs triggering the onset of pustular psoriasis is therefore evergrowing , and the most common of these can be easily grouped as pils to stop , namely progesterone , iodide , lithium , salicylates , terbinafine , oxyphenbutazone , phenylbutazone and bupropion , used to aid cessation of smoking . the most important trigger however is corticosteroid therapy , which can act via the topical as well as systemic routes and also due to sudden withdrawal of corticosteroids . losartan , an angiotensin ii receptor blocker , has been implicated by lamba et al . , in triggering psoriasis . another drug , candesartan , belonging to the same class , was reported by kawamura and ochiai to induce pustular psoriasis by up - regulation of bradykinin . the effect of the present drug , temisartan , intriggering pustular psoriasis is only yet to be documented . this is of growing concern as telmisartan and other angiotensin ii receptor blockers are being used extensively in hypertension and hypertensive heart disease owing to its potent , long - acting nature , selective mechanism of action and considerable safety of use . pustular psoriasis provoked by telmisartan can be considered as drug - induced disease as the discontinuation of drug stopped the further progression of disease . this case highlights the fact that treating physicians should be aware of drugs that exacerbate the disease and that caution should be exercised while starting a new drug in patients with pustular psoriasis .

pustular psoriasis is characterized by abrupt onset of macroscopic pustules associated with erythema and symptoms of burning pain and constitutional symptoms . there are several precipitating factors , both physiological such as pregnancy and routinely prescribed drugs like antihypertensives , antifungals , corticosteroids and progesterone . we present a case of a 50-year - old male patient with pustular psoriasis , well controlled on oral methotrexate , who presented with sudden exacerbation of pustular psoriasis following the use of telmisartan . this case is presented due to the absence of prior reports of telmisartan aggravating pustular psoriasis .

the controversy surrounding choice of resuscitation fluid owes much of its intensity and longevity to economic concerns , yet reported pharmacoeconomic data comparing costs of various fluid regimens are remarkably limited . the expenditures for one fluid have typically been compared with those for another when cost data have been reported . these data have rarely rigorously addressed the comparative total costs of care taking into account potential differences in outcomes such as morbidity , mortality and length of intensive care unit or hospital stay . consequently , the available pharmacoeconomic evidence to date unfortunately fails to provide a solid foundation upon which to rest clinical decision - making . it will ultimately be necessary to define cost - benefit ratios with specific reference to particular clinical indications and fluid management regimens . in one randomized control trial ( rct ) , for example , acute albumin administration in hospitalized cirrhotic patients with ascites significantly shortened hospital stay and reduced total costs of care by nearly 60% . chronic albumin administration on an out - patient basis , however , did not afford a similarly favourable cost - benefit ratio . this natural colloid has been reported to account for a substantial fraction of the pharmacy budget at some hospitals . some clinical investigations have failed to provide evidence of significant patient benefit associated with albumin administration . such lack of benefit coupled with the comparatively high unit dose cost of this colloid has often prompted calls for the abandonment of albumin in fluid management . the rcts of albumin administration reported to date have nevertheless typically involved quite small numbers of patients and were accordingly statistically underpowered to detect differences in clinically relevant endpoints such as morbidity , mortality and length of stay . indeed , not a few studies have revealed significant differences favouring albumin in these clinically relevant endpoints . a recent rct of patients with cirrhosis and spontaneous bacterial peritonitis has attracted considerable notice in this regard . the incidence of renal impairment or severe hyponatraemia in an earlier rct of cirrhotic patients with tense ascites was significantly lower in patients treated by therapeutic paracentesis with versus without albumin infusion . development of either or both of these complications was significantly predictive of higher actuarial mortality rate . addition of albumin to the priming solution in a rct of patients undergoing cardiopulmonary bypass surgery was associated with a significant 34% reduction in mediastinal blood loss during the first 12 h postoperatively . furthermore , substitution of hydroxyethyl starch ( he s ) for albumin as the cardiopulmonary bypass priming fluid in a retrospective case - control study involving 288 patients , intended as a cost - saving measure , was associated with a dose - dependent increase in incidence of haemorrhage . the us$3458 median unadjusted hospital cost associated with treatment of haemorrhage in these patients was far greater than the difference in cost between he s and albumin . hypovolaemic and septic shock , gastrointestinal surgery and hypoalbuminaemia are additional indications in which rcts have provided evidence of significant benefit due to albumin administration . the incidence of pulmonary oedema , based upon radiographic evidence , was significantly four - fold greater for the saline than either the albumin or he s groups in a rct of 26 patients with hypovolaemic and septic shock . the severity of intestinal oedema during gastrointestinal surgery in a rct of 18 patients was significantly less among recipients of albumin than of either ringer 's lactate or he s . the total number of complications among 61 hypoalbuminaemic patients enrolled in a rct of albumin supplementation was significantly greater by 136% in control than albumin patients , and the number of control patients experiencing complications was significantly greater by 44% . furthermore , the numbers of control patients developing septicaemia and pneumonia were significantly greater than those of the albumin group . the unanswered question , of course , is the extent to which such differences in morbidity would affect the cost - benefit ratio of albumin . at a minimum , however , these differences suggest that , in certain indications and with certain administration regimens , the benefits of albumin may well justify its costs . another factor that needs to be considered is the time horizon for assessing total costs of care . delayed morbidity and mortality occurring after discharge might contribute substantially to the total costs of care , although such added costs have seldom been addressed in clinical investigations thus far . in the rct of sort et al , for instance , the total hospital mortality rate for both the albumin and control groups together was 19% . an additional 13% of patients had , however , died by 3 months . delayed severe persistent pruritus , most commonly manifested as pruritic crisis , has been reported in 32% of patients receiving extended he s treatment . such pruritus was encountered , on average , 25 days after commencement of treatment , and pruritus developed in the majority of patients after he s administration was discontinued . the repercussions of fluid therapy may extend to even longer time periods . in a renal transplantation study of 438 patients , for instance , intraoperative albumin administration was associated with a dose - dependent reduction in frequencies of delayed or absent perioperative graft function and a significantly higher graft survival rate at 1 year postoperatively . late morbidity and mortality after discharge from the intensive care unit or hospital can clearly impose additional costs that should be taken into account . there is little doubt that further studies are needed to establish the cost - benefit ratio of particular fluid administration regimens in specified clinical indications .

cost is a key concern in fluid management . relatively few data are available that address the comparative total costs of care between different fluid management regimens in particular clinical indications . relevant costs of fluid - associated morbidity and mortality , including those incurred after intensive care unit or hospital discharge , also need to be considered in evaluating the cost - benefit ratios of administered fluids . rigorously designed pharmacoeconomic studies are needed to delineate the costs and benefits of various approaches to fluid management .

langerhans cell histiocytosis ( lch ) is a rare , clinically polymorphous group of disorders , presenting with heterogeneous clinical manifestations and an unpredictable outcome . lch is characterized by proliferation of abnormal and clonal langerhans cells , in one or more organs like skin , bone , lymph nodes , lungs , liver , spleen , and bone marrow . occurrence of lch in adults is rare and is commonly seen in infants and early childhood . the disorder is difficult to diagnose in adults and once diagnosed , the multisystem lch has a poor prognosis . a 26-year - old man , presented with a one - week history of polymorphous eruptions over face , trunk , and extremities , associated with painful sores in the mouth as well as fever . the lesions started around the mouth with greasy scaling , which gradually progressed to involve scalp , neck , trunk , and extremities , including genitals . on general physical examination , jaundice , and inguinal lymphadenopathy were present . on cutaneous examination , multiple skin colored to yellowish papules and pustules were present over the scalp , retro - auricular area , face , neck , trunk , extremities , and genitalia , which were associated with facial edema [ figures 1 and 2 ] . oral mucosa showed multiple vesicles , pustules , and hemorrhagic crusting of lips [ figure 3 ] . scaling with crusting present over face , mimicking seborrheic dermatitis yellow - brown papules with pustules present over chest ulcers present over lips laboratory data showed : hb-16.1 gm / dl ; total leukocyte count ( tlc)-33,600/cu.mm ; neutrophil 54% , lymphocyte 25% , eosinophil 21% ; total bilirubin-5.9 mg / dl ; direct bilirubin-3.5 mg / dl ; serum glutamic oxaloacetic transaminase ( sgot)-179 u / l ; serum glutamic pyruvic transaminase ( sgpt)-269 u / l ; alkaline phosphatase ( alp)-167 u / l ; renal function test- within normal limits . biopsy from the skin , liver , and bone marrow showed dense infiltrate of histiocytes with longitudinal nuclear groove and numerous eosinophils and lymphocytes [ figures 4 and 5 ] . diffuse histiocytic infiltrate , h and e staining , 10 histiocytic infiltrate , h and e staining , 100 with the history , clinical findings , and investigation , the diagnosis of multisystem lch was made . he was given vinblastine and etoposide weekly for 6 weeks with daily prednisolone , followed by maintenance therapy of 12 cycles with same drugs at intervals of 3 weeks and daily 6-mercaptopurine . lichtenstein , in 1953 , coined the term histiocytosis - x to describe a group of disorders ( hand - schuller - christian disease , letterer - siwe disease , and eosinophilic granuloma ) characterized by infiltration of involved tissue with large number of abnormal histiocytes . subsequently , these histiocytes were found to be similar to langerhans cell normally present in the skin , and therefore termed as lch . lch is a clonal neoplastic disorder,[13 ] and its pathogenesis is unknown . whether the infiltrating cells are truly neoplastic or reactive in nature is still a matter of debate . the disease affects young children aged 1 - 4 years , but can occur at any age . the incidence is approximately 2 - 5 per million per year in infants and children and is even rarer in adults . the writing group of histiocytic society ( 1987 ) has recently defined the criteria for diagnosis of langerhans cell histiocytosis . the group proposed three levels of certainty in the diagnosis of lch based on clinical features , histopathology , and immunohistochemistry . the scalp is erythematous with greasy scales , appearing much like seborrheic dermatitis . on the trunk , the lesions are discrete , yellow - brown scaly papules , often showing areas of purpura . ulceration in the flexures , groins , and the perianal area is the common presentation in adults . diabetes insipidus is the most common endocrinal abnormality associated with lch caused by the infiltration of pituitary gland by langerhans cells . histologically , there is a dense dermal infiltrate of langerhans cell , recognized by abundant , amphophilic cytoplasm and round or kidney bean - shaped nucleus . by contrast , 20% of the patients with multisystem involvement have a progressive disease course despite treatment . the prognosis is directly related to the age of onset , numbers of organ involved , and the extent of organ dysfunction . the prognosis of the disease depends on systemic rather than cutaneous involvement . etoposide as a single drug is better than other drugs tested , eg , vinka alkaloid , methotrexate , 6-mp . our patient responded well to the combination therapy of vinblastine , etoposide , and oral corticosteroids .

langerhans cell histiocytosis ( lch ) , is a rare disorder , clinically presents with heterogeneous manifestations , and has an unpredictable outcome . commonly seen in infancy or early childhood , the disorder is characterized by proliferation of abnormal and clonal langerhans cell in skin , bone , lymph nodes , lungs , liver , spleen , and bone marrow . occurrence of lch in adults is rare . here , we report the case of an adult with acute onset of polymorphic eruptions all over the body , which on biopsy showed features of multisystem lch , and was confirmed by immunohistochemistry . although multisystem lch has a poor prognosis , our patient responded well to chemotherapy .

ocular cicatricial pemphigoid ( ocp ) is an uncommon , chronic autoimmune disease that affects mucous membranes , particularly the conjunctiva.1 the disease typically results in chronic conjunctivitis and causes conjunctival and corneal scarring , which can result in limbal stem cell deficiency and blindness.2 adequate control of ocular inflammation usually requires systemic as well as topical immunosuppressants . commonly used systemic medications include prednisone , methotrexate , mycophenolate mofetil ( mmf ) , and cyclophosphamide.3 the porphyrias are metabolic disorders caused by defective enzymes within the heme synthetic pathway.4 these defective enzymes cause an accumulation of intermediates from the heme synthetic pathway that results in various clinical manifestations.4,5 porphyria cutanea tarda is the most common of the porphyrias and results from a deficiency in uroporphyrin decarboxylase , which is the fifth enzyme in the heme synthetic pathway.6 this deficiency results in the accumulation of porphyrins in the liver and plasma . on exposure to light with a wavelength near 400 nm , the porphyrins enter an excited state that can lead to the damage of proteins , lipids , and basement membranes.6 this process results in blisters , fibrosis , and scarring of the skin in areas of the body exposed to sunlight.6 park et al7 have reported a case of a 31-year - old with cicatricial conjunctivitis who was biopsy negative for ocp and was later diagnosed with porphyria cutanea tarda ; in this case the patient s clinical symptoms significantly improved after initiating phlebotomy treatments . the present authors report a similar case , in which pathology and direct immunofluorescence confirmed a diagnosis of ocp and where the patient s clinical condition also improved significantly upon the diagnosis and treatment of porphyria cutanea tarda . a 64-year - old caucasian male complaining of redness and tearing for 3 years in both eyes was referred for evaluation of cicatricial conjunctivitis . he had been treated with tobramycin and dexamethasone ophthalmic ointment in both eyes as needed and doxycycline 100 mg by mouth daily with no improvement in his symptoms . on slit lamp examination the patient had subconjunctival fibrosis , symblepharon , forniceal foreshortening , and trichiasis in both eyes ( figure 1 ) . examination of the corneas revealed multiple punctate epithelial erosions . a schirmer s test was performed without anesthesia , showing 22 mm of wetting in the right eye and 13 mm in the left after 5 minutes . direct immunofluorescence studies of the conjunctival biopsy specimen revealed immunoglobulin g4 deposits in the basement membrane zone of the junctional area ( figure 2 ) , consistent with ocp . given the findings of subconjunctival fibrosis and symblepharon formation in both eyes , the patient was diagnosed with bilateral stage iii pemphigoid . the patient was started on methotrexate 15 mg by mouth weekly and prednisone 20 mg by mouth daily . the patient demonstrated gradual improvement in the conjunctival inflammation after starting the methotrexate and prednisone . the patient was subsequently tapered off the prednisone , while the methotrexate 15 mg by mouth weekly was continued . after 4 months of treatment with the methotrexate , the patient s conjunctival inflammation began to worsen and his regimen was subsequently changed from methotrexate to mmf 1000 mg by mouth twice daily . approximately 3 months after initiating mmf treatment , the patient was diagnosed with porphyria cutanea tarda . the patient s conjunctival inflammation appeared stable following initiation of the phlebotomy treatments and the mmf was subsequently discontinued . approximately 6 weeks following discontinuation of the mmf , the patient returned with mildly increased conjunctival injection and trichiasis in both eyes . epilation was performed and the patient was started on 1% prednisolone acetate ( one drop in both eyes twice daily ) . the patient s conjunctival inflammation stabilized and he was tapered down to one drop of 1% prednisolone acetate in both eyes once daily . since initiation of the phlebotomy treatments , the patient s conjunctival inflammation and subconjunctival fibrosis has remained quiescent for 4 months without requiring mmf ( figure 3 ) . ocp is believed to be an autoimmune disease of genetic predisposition , and it is likely that a second - hit environmental trigger is required to initiate onset of the disease.1 it has been thought this could include chemical exposure or microbial environmental triggers . the present case suggests that the patient s porphyria could be a causal factor associated with the ocp and could even have been the environmental trigger that stimulated the disease to occur . the porphyrins are present in plasma and therefore they would be present in the ocular surface vasculature.5 the ocular surface is constantly exposed to light . exposure to ultraviolet light would lead the porphyrins to enter into an excited state , resulting in inflammation and damage to the ocular surface . this process could be the trigger to either initiate or exacerbate ocp . in the present case , the patient s conjunctival inflammation was observed to significantly improve following initiation of treatment for his porphyria . a similar response was observed in the aforementioned case reported by park et al,7 although that particular patient was biopsy negative for ocp and was positive for hepatitis c virus infection ; in addition , the patient remained on methotrexate . the patient in the present case was unique in that he was biopsy positive for ocp and his clinical improvement was significant enough after starting phlebotomy treatments that his mmf was discontinued . the authors consider that this case , as well as the case reported by park et al,7 sheds new light on the search for the etiology of ocp and the subsequent treatment options for patients with this disease .

a 64-year - old caucasian male complaining of redness and tearing for 3 years in both eyes was referred for evaluation of cicatricial conjunctivitis . ocular cicatricial pemphigoid was suspected and this diagnosis was confirmed through biopsy . the patient s condition showed moderate improvement following treatment with methotrexate and mycophenolate mofetil . the patient was later diagnosed with porphyria cutanea tarda and phlebotomy treatments were subsequently initiated . the patient s ocular symptoms improved further after he began receiving these phlebotomy treatments , and conventional treatment was discontinued . the authors hypothesize that circulating porphyrins activated by ultraviolet light could be the cause of the ocular cicatricial pemphigoid in this patient .

an artifact in a computed tomography ( ct ) image can be described as any discrepancy in the hounsfield unit ( hu ) seen in the image obtained as opposed to what is expected . artifacts in ct can be either patient related , equipment related , or due to image processing . related artifacts can be minimized , corrected , or avoided by being alert , properly instructing the patient , and applying software corrections . however , equipment - related artifacts require active intervention , and early identification of the same is important to enable prompt resolution by the equipment support team . tube arcing ct artifact is an equipment - related artifact and rarely cited in literature . the short circuit is normally said to be caused due to presence of impurities obstructing the path of electrons emitted by cathode before they reach the anode , leading to momentary loss of x - ray output . in our case , we discovered that the tube arcing artifact did not occur due to the reasons described in literature . our department has a positron emission tomography / computed tomography ( pet / ct ) scanner ( discovery iq pet ; ge medical systems , milwaukee , usa ) . this equipment is used to perform pet / ct as well as routine ct procedures . on a busy afternoon , we encountered an artifact on two to three consecutive slices of the ct transaxial images of a patient , which showed near - parallel streaks across the transaxial image and a hypodense band with parallel bars on the corresponding sagittal and coronal images [ figure 1 ] . on careful examination and , in particular , viewing the ct images in the lung window , it became apparent that the streaks in the transaxial slices were not parallel , but appeared to converge at a point outside the circumference of the gantry . when we measured the hu of the tissues , we found that it was lower than that of neighboring slices . we felt that the probable reason for the artifact could be disruption of x - ray production in those slices possibly because of failure of voltage generation . literature search suggested this could be the rarely encountered tube arcing artifact . for confirmation of the artifact , we reviewed ct series of all other pet / ct studies performed on that day . we immediately brought this occurrence to the vendor 's support team , who also suspected voltage failure to be the possible cause . the next day , we were more vigilant and found more frequent occurrences of this artifact . preliminary investigation by the vendor 's support personnel revealed very short - lived , erratic drops in voltage corresponding in time with the seen artifacts . this drop in voltage as seen on a graph did not touch zero at any of the instances ; the voltage graph showed high standard deviations in the voltages [ figure 2 ] , and hence , the system did not generate any error or abruptly stop scanning . however , this observation still did not explain why these artifacts were not occurring in any of the other ct images acquired . we performed repetitive phantom scan in dedicated ct protocol and did not observe such an artifact . so , we performed the phantom ct scan in pet / ct whole body protocol and streak artifact was observed [ figure 3a and b ] . thus , we decided to review the ct acquisition parameters for diagnostic ct and ct in pet / ct protocols . our review revealed a difference in kv used in both protocols , i.e. , 140 kv used in pet / ct protocol and 120 kv used in diagnostic ct protocol . service engineer changed the hemit tank ( which is a transformer supplying voltage to the rotor of the rotating anode ) , considering the faulty current supply to the ct tube . after replacement , we performed repetitive ct scans of phantom in pet / ct protocol at 140 kv and did not observe any tube arcing artifact [ figure 3c and d ] . clinical image : ct tube arcing artifact seen in the head region on the transaxial image ( a ) and corresponding coronal image ( b ) ; in the thigh region on the transaxial image ( c ) and corresponding coronal image ( d ) plot of generator kv versus time in images showing drop of voltage corresponding to appearance of tube arcing artifact ( a and b ) phantom image : ct image of uniformity phantom in pet / ct whole body protocol showing streak artifact , transaxial image ( a ) ; arrow shows alternating lucent bands seen on the coronal image ( b ) . ct images , transaxial image ( c ) and corresponding coronal image ( d ) of uniformity phantom in pet / ct whole body protocol after replacing the hemit tank ( no tube arcing artifact seen ) x - ray tubes used in medical devices work at high voltages and are susceptible to electrical breakdown or short circuits . various sources of arcing are mentioned in the literature , namely , insulator surface flashover , insulator breakdown , and vacuum flashover . insulator surface flashover is tube design dependent ; vacuum flashover is a very common phenomenon and is generally caused due to the presence of particulate impurities resulting from tungsten evaporation or imperfect surface of the target . overheating of the focal spot and tungsten evaporation can result due to faulty anode rotor . in this particular case , short circuit was caused due to the defective transformer supplying voltage to the rotor of the rotating anode ( hemit tank ) . however , there was no abrupt termination of the ct scan as there was no error generated by the system . this was because at no point did the drop in voltage reach the threshold ( 0 kv ) to generate an error . the resulting artifact was seen as alternating hypodense streaks throughout the transaxial image , which corresponded with a horizontal hypodense band in sagittal and coronal images also . the correction algorithm could either use synthesized projections generated by using the neighboring unaffected slices or an adaptive filtering scheme or a partial data interpolation method . however , these methods are capable of correcting only moderate streak artifacts and not to the extent mentioned here . frequent tube arcing or tube spit of this magnitude can only be resolved by replacing the tube , which would have been very costly , considering that it had only been 6 months since installation of the pet / ct . however , in our case , the reason for disruption of x - ray was different . the most probable reason for disruption in x - ray production leading to the artifact was the disrupted current supply to the rotor of the rotating anode at 140 kv . although tube arcing artifact and its causes have been described in the literature , we have described a unique cause and remedy for it . the described tube arcing artifact seen in ct images was caused by a fault with the transformer supplying voltage to the rotor connected to the anode in the x - ray tube , which involved sudden transient drops in voltage generated whenever acquisition was done at 140 kv . using the ct at a relatively lower voltage of 120 kv could prevent such an occurrence , and in case such an artifact does occur , it would save time and cost if this reason is ruled out before proceeding to tube replacement .

tube arcing artifact is known to be caused by a temporary short circuit in the x - ray tube causing momentary loss of x - ray output . it is seen as near - parallel and an equidistant streak pattern on transaxial computed tomography ( ct ) images and as a horizontal hypodense band on the coronal and sagittal ct images . this artifact can be a random occurrence and was caused in this particular case due to voltage fluctuations in the high - voltage supply transformer supplying the rotor of the anode in the x - ray tube . this problem was initially corrected by reducing the tube voltage to 120 kv from the original 140 kv and , subsequently , replacing the faulty transformer . this kind of artifact , which is a very rare situation , can affect the image quality , and could also be an early sign of equipment failure . to the authors knowledge , such an artifact has not been reported till date in a clinical scenario . hence , we would like to report a rare situation of tube arcing artifact along with a unique remedy .

rapid advances in the field of laparoscopic surgery have called for newer instruments that will safely replicate and , where possible , improve the techniques used in open surgery . safe resection of tissues while maintaining hemostasis in laparoscopic colorectal surgery depends on good control of mesenteric vessels . currently , the us food and drug administration has approved the enseal tissue - sealing device ( ethicon endo - surgery , blue ash , ohio ) for vessels up to 7 mm in diameter . this is greater than or equal to the vessel diameter limit in other vessel - sealing systems that are widely used , such as ligasure ( covidien , boulder , colorado ) and the harmonic scalpel ( ethicon endo - surgery ) . anecdotal evidence suggests that the enseal device can safely seal vessels > 7 mm . at our institution , we have gravitated toward the enseal device for all colorectal procedures ; we find it to be effective and reliable . its versatility is evidenced by its use as a grasper , dissector , scissors , and vessel and soft - tissue sealer . if found to be effective , use of the device would further reduce tissue handling , operative time , and blood loss and thus hasten postoperative recovery . on the basis of previously published data , we determined an adequate sample size to be 50 cases . ninety specimens were harvested from pigs euthanized for other purposes , such as for training residents and fellows . the pig aortas were harvested from the infrarenal position to the bifurcation of the iliac vessels via laparotomy . the opposite end was attached to the pressure measurement system , harvard apparatus ( syringe pump ) ( figure 1 ) . the device measures pressure continuously as it infuses the segment with normal saline at a constant rate ( 50 ml / h ) . bursting pressure was the maximum pressure at which the sealed vessel leaked or burst open . a failure to seal harvard apparatus ( syringe pump ) . mean and standard deviation were used for continuous data . the pearson correlation coefficient was used to determine the relationship between continuous variables such as diameter and bursting pressure . vessels were divided into 3 groups on the basis of the diameter : 710 mm , 1115 mm , and > 16 mm . one - way analysis of variance was used to determine differences among these 3 groups . spss version 16 was used for the statistical analysis ( spss 16.0 , ibm inc . , the median vessel diameter was 14 mm ( range , 718 ) , and bursting pressure was 85 mm hg ( range , 24650 ) . thirty - one percent ( n = 29 ) of vessels failed to seal ( table 1 ) . the pearson test showed a negative correlation between vessel size and bursting pressure ( p = .25 ) . general characteristics vessels were divided into 3 groups based on diameter : group 1 ( 710 mm ) had 18 vessels , group 2 ( 1115 mm ) had 46 vessels , and group 3 ( > 16 mm ) had 26 vessels . overall , there was no significant difference in the mean bursting pressures among the groups ( p = .517 ) . intergroup analysis with the scheffe post hoc test also did not show any statistically significant difference in bursting pressure ; however , there was a trend toward a lower bursting pressure with increasing diameter ( table 2 ) . mean bursting pressures among groups based on diameter of vessel the rate of failure to seal in groups 1 , 2 , and 3 were 15% , 45% , and 40% , respectively ( p = .10 ) . specimen failure was scattered throughout all groups ( p = .10 ) ( table 3 ) . the us food and drug administration has approved the enseal bipolar sealing device for sealing vessels up to 7 mm in diameter . to our knowledge , there are no studies in the literature that have evaluated the efficacy of this device in vessels > 7 mm . our evaluation of the sealing ability of the enseal bipolar energy device showed significantly inconsistent bursting pressures , with a wide range between 24 and 650 mm hg . the larger vessels were found to have lower bursting pressures and higher failure - to - seal rates . the histologic nature of the vessels used in our study may have also contributed to the overall low bursting pressures and high failure rates . the aorta is histologically an elastic vessel with higher elastin content in the tunica media compared with nonelastic vessels and low collagen content mostly located in the tunica adventitia . the resulting low collagen to elastin ratio may be the cause of the low bursting pressures in our study . there are myriad new devices developed for the purpose of vessel sealing that have been found to be as effective as conventional methods such as clips and ties . studies have shown that it has much higher bursting pressures than other sealing systems ( 678 mm hg for enseal vs 489 mm hg for ligasure ) . other notable features of the enseal device include less adventitial damage detected on histopathologic evaluation . however , all of these studies have been done on vessels < 7 mm in diameter . to exclude the histologic factor , further studies are needed to evaluate the enseal device in vessels > 7 mm with a higher collagen to elastin ratio . another variable to consider is the duration of energy expenditure each time the instrument is applied . these would improve our understanding on the determinants of the quality of seal and lateral thermal damage with the enseal device . bursting pressures are low and inconsistent after tissue sealing with the enseal device in porcine vessels > 7 mm . the histologic results of the aorta segments ( ie , low collagen to elastin ratio ) may be responsible for the low bursting pressures . clinical use of vessels > 7 mm is therefore not recommended in light of the results of this study .

background : the enseal ( ethicon endo - surgery , blue ash , ohio ) tissue - sealing device has proven efficacy for ligation of vessels < 7 mm in diameter , even with significant supraphysiologic bursting pressures . we aimed to evaluate the safety of enseal in porcine vessels > 7 mm.materials and methods : the lumbar aortas of pigs that were euthanized for unrelated procedures were harvested . a 5- to 6-cm segment of aorta was sealed using the enseal device . the opposite end was attached to a pressure - testing device to measure pressures at leak or bursting . the bivariate pearson correlation was used to determine the relationship between diameter and bursting pressure . one - way analysis of variance was used to determine differences between the groups of vessels on the basis of their diameter.results:ninety samples of 5-cm aorta segments were used to assess bursting pressure . the median diameter was 14 mm ( range , 718 ) and bursting pressure was 85 mm hg ( range , 24650 ) . the pearson test showed a negative correlation between vessel diameter and bursting pressure ( p = .25 ) . one - way analysis of variance did not show any significant difference between vessel diameters grouped by size ( p = .517 ) , and neither did the scheffe post hoc test when comparing diameter with bursting pressure ; 31% of specimens failed to seal.conclusions:bursting pressures are low and inconsistent after tissue sealing with the enseal device in porcine vessels > 7 mm . these vessels also demonstrated a higher rate of failure to seal . the histologic results of the aorta segments ( ie , a low collagen - elastin ratio ) may be the cause of the low bursting pressures .

the optimization of ventilatory support is a crucial issue in the management of patients with acute respiratory failure , aiming at improving gas exchange and patient - ventilator interaction while preventing ventilator - induced dysfunction of the respiratory system . a very important study by levine et al . shows in humans , as previously observed in animal models , that the combination of diaphragm inactivity and mechanical ventilation is associated with marked diaphragm atrophy . diaphragm atrophy , which may occur after just 18 hours of controlled mechanical ventilation as a result of increased myofibril proteolysis , may contribute to prolongation and/or failure of the weaning process . this study , performed in brain - dead organ donors , underlines the importance of using modes of partial ventilatory support whenever possible and not contraindicated , in order to reduce the time spent on mechanical ventilation and its side effects . relevant clinical research has investigated the complex interaction between patient and ventilator during partial support . compared three different strategies conventionally aimed to optimize patient - ventilator interaction in pressure support ventilation ( psv ) : reduction of support level , reduction of insufflation time and application of positive end - expiratory pressure ( peep ) . the reduction of pressure support succeeded in eliminating ineffective triggering in two - thirds of the patients without increasing the work of the respiratory muscles or altering gas exchange , while the effects of reducing insufflation time and increasing peep were less important and not significant . the improvement in patient - ventilator synchrony becomes relevant when tidal volume ( vt ) is reduced to 6 ml / kg , which is therefore suggested by the authors as a target for adjustment of inspiratory pressure support level . neurally adjusted ventilatory assist ( nava ) is a new form of partial support wherein the ventilator applies positive pressure throughout inspiration in proportion to the electrical activity of the diaphragm , as assessed by transoesophageal electromyography . allowing ventilator functioning and cycling to be under direct control of the patient 's respiratory drive and rhythm , nava aims to enhance patient - ventilator interaction and minimize the risk of over - assistance . a recent study by colombo et al . compares the physiological response to different levels of assistance delivered with nava and psv . as opposed to nava , psv was prone to the risk of over - assistance , as identified by higher vt , significant mismatch between neural and flow - based timing and frequent occurrence of ineffective efforts . the study indicates that , compared to psv , nava has the potential to limit the risk of over - assistance , prevent patient - ventilator asynchrony , and , overall , improve patient - ventilator interaction . two recent trials have evaluated the impact of different ventilator settings in the prevention of ventilator - induced lung injury ( vili ) in patients with acute lung injury and acute respiratory distress syndrome ( ards ) . meade et al . compared the effects of a ventilatory strategy combining low vt , recruitment manoeuvres and high levels of peep with the established ventilatory strategy . in the experimental group , vt was set at 6 ml / kg , with plateau airway pressures ( paw - plateau ) 40 cmh2o . each trial started with a 40-second breath - hold at 40 cmh2o paw recruitment manoeuvre on a fraction of inspired oxygen ( fio2 ) of 1.0 , which could be repeated up to four times daily . the control group underwent the ards network ventilation protocol ( volume - assist control mode , vt 6 ml / kg and paw - plateau 30 cmh2o , with peep determined according to fio2 , no recruitment manoeuvre ) . results showed a significant difference in peep levels associated with lower incidence of hypoxemia and fio2 requirement , less recourse to rescue oxygenation therapies , and fewer deaths due to refractory hypoxemia in the intervention group , as opposed to the control group , while no significant difference in mortality rate and duration of mechanical ventilation was found . evaluated a strategy using high peep levels to increase alveolar recruitment while avoiding excessive hyperinflation by limiting paw - plateau and using low vt . in the control group , paw - plateau was kept as low as possible without falling below oxygenation targets , setting peep between 5 and 9 cmh2o . in the experimental group , peep was adjusted based on paw and kept as high as possible without increasing paw - plateau above 28 - 30 cmh2o , regardless of its effects on oxygenation . the experimental strategy significantly improved oxygenation and respiratory system compliance and reduced the time spent on mechanical ventilation and with organ failures , but failed to reduce mortality . in an intriguing study , consider vili a result of excessive lung stress and strain , as assessed by transpulmonary pressure and inflated volume to functional residual capacity ratio , respectively . as stress and strain can not be measured in clinical practice , the study aims to determine the accuracy at which the two parameters can be inferred by analyzing their commonly utilized surrogates , that is , paw - plateau and vt / kg . currently , prevention of vili is based on recognizing the harmful threshold for these surrogates , identified as 28 - 30 cmh2o ( paw - plateau ) and 6 ml / kg ( vt / kg ) . in a varied population ranging from postoperative patients with intact lung function to patients with ards , paw - plateau and vt interestingly , the stress to strain relationship was the same in all the subgroups , being linear in the ranges of pressures and volumes explored . the specific lung elastance was also similar in the various subgroups , remaining constant at approximately 13.5 cmh2o , regardless of ventilator settings . because stress and strain were demonstrated to be linked by a constant proportionality factor , that is , the specific lung elastance , stress can be calculated as 13.5 cmh2o strain . as a strain greater than 2 , corresponding to an end - inspiratory lung volume in the range of total lung capacity , has been identified as critical for the risk of development of lung injury , the harmful threshold of stress will consequently be 2 13.5 cmh2o ; therefore the recommended paw - plateau threshold of 28 - 30 cmh2o seems reasonable for most acute lung injury / ards patients . the identification of diaphragmatic atrophy after just 18 hours of controlled mechanical ventilation suggests the importance of adopting modes of partial ventilatory support as soon as possible . the optimization of patient - ventilator synchrony should be based either on the reduction of the assistance provided with the commonly used modes of partial support or on the application of new forms of mechanical assistance designed to improve the interaction between the patient and the ventilator , such as nava . in patients with ards , ventilatory strategies for optimizing peep with the aim of increasing alveolar recruitment while limiting hyperinflation succeeded in improving lung function and reducing the duration of mechanical ventilation , but failed to reduce mortality . the introduction of the measurement of lung stress and strain into daily clinical practice could allow a better identification of the safe limits of mechanical ventilation in the presence of lung injury . our institution received an icu ventilator with nava software , a portable pc , and dedicated data acquisition software from maquet critical care , used in the study conducted by colombo et al . .

recently , several studies have been performed to better outline the pathophysiology of acute respiratory failure and evaluate the therapeutic profile of different modes of ventilation and ventilator settings . here we briefly report those we consider most relevant for daily intensive care unit clinical practice .

anterior cervical spine fusion and stabilization is a well established procedure for cervical myelopathy , radiculopathy , neoplasms , and cervical trauma2 ) . although injuries to the pharynx and esophagus are known complications of anterior cervical spine surgery , delayed pharyngeal or esophageal perforation is rare7,9,10 ) . here , we describe a rare but potentially life - threatening complication after anterior cervical spine fusion and plating . the authors highlight this issue by presenting this case , which had no associated morbidity , and include a review of the relevant literature . a 43-year - old man was admitted to our institute with a 3-month history of dysphagia and neck pain with swelling . he was paraplegic due to a c6 - 7 fracture and dislocation and has been operated on 8 years previously . initial surgical treatment included anterior corpectomy of c7 and anterior iliac crest graft placement using a plate and screws . hematological studies including erythrocyte sedimentation rate ( esr ) and c - reactive protein ( crp ) were normal . a simple lateral radiograph and a computed tomography scan showed partial anterior migration of the lower screw . a hydro - soluble contrast swallow image confirmed esophageal perforation ( fig . the loose screw was removed and esophageal perforation was found during surgery and repaired directly by a cardiovascular team(fig . the patient was fed using a nasogastric tube for 3 weeks and subsequently oral feeding was gradually resumed . further progress was favorable , and a contrast study performed at 3 weeks postoperatively showed no evidence of fistula . anterior cervical fusion and plate fixation is an effective procedure for the treatment of cervical myelopathy or radiculopathy and cervical spine trauma . plating has been reported to achieve a fusion rate of up 98% , and to result in early mobilization , reduced graft - related complications ( especially for multilevel fusion ) , and to avoid late deterioration of the cervical spine alignment obtained at surgery1,4 ) . the complication rate after anterior cervical plating is generally low and decreases with surgeon 's experience . according to zeidmann14 ) , the overall complication rate associated with anterior cervical spinal fusion is approximately 5% , and pharyngo - esophageal perforation is uncommon , but nevertheless of the utmost importance because of the possibility of graft infection leading to osteomyelitis , mediastinitis , sepsis , and death6 ) . acute injury can be caused iatrogenically during surgical approach due to inappropriate placement or dislodgement of sharp - toothed retractor blades in the esophagus . retraction is particularly dangerous when a nasogastric tube is positioned because the wall of the hypopharynx or esophagus may be " trapped " by a high - pressure claw between the retractor and the tube , causing ischemic injury and secondary perforation8 ) . delayed esophageal injuries are due to chronic compression or contact and subsequent necrosis , abscess formation , and perforation due to graft dislodgement or screw migration with or without plate failure4,7 ) . screw dislodgement often follows a benign course and is completely asymptomatic , due to the small diameters of the screws used and slow migration from the external to the internal mucosa , which permits spontaneous tissue repair of the defect caused repetitive friction between the retropharyngo - esophageal wall and the plating system(normally positioned with adhesion ) , traction - type pseudodiverticulum , and perforation are other causes of delayed injury11 ) . the complications of esophageal perforation range from asymptomatic with local infection to mediastinitis and death . the clinical course depends on the etiology , location , and timing of the perforation . asymptomatic perforation has as well been reported as incidental oral extrusion of screw even years after anterior cervical spine stabilization5 ) . patients generally present with swallowing difficulty , regional swelling , neck pain , dysphagia , weight loss , dysphonia , subcutaneous emphysema , and fever ; our patient presented with dysphagia and neck pain with regional swelling12,13 ) . conservative treatment may be preferred for small , contained defects of less than 1 cm , and consists of the elimination of oral feeding , tube feeding to restore fluid and nutritional balance , and intravenous antibiotics . some cases need surgical repair , such as , perforation closure with a primary suture or sternocleidomastoid or pectoralis major flap repair13 ) . we operated on our patient to remove the offending screw due to evident fistula confirmed by esophagography and esophagoscopy . direct repair was effective in achieving a successful perforation repair of the esophageal perforation with an early return to oral feeding . we report a rare case of delayed esophageal perforation caused by screw displacement after anterior cervical spine plating . careful periodic follow - up is necessary , and when encountered , early surgical closure following removal of the offending screw is mandatory .

although anterior approaches to the cervical spine are popular and safe , they cause some of complications . esophageal perforation after anterior spinal fusion is a rare but potentially life - threatening complication . we present a rare case of delayed esophageal perforation caused by a cervical screw placed via the anterior approach . a 43-year - old man , who had undergone surgery for complete cord injury at another orthopedic department 8 years previously , was admitted to our institute due to painful neck swelling and dysphagia . radiological studies revealed a protruding screw and esophageal perforation . the perforation was found during surgery and was successfully repaired . this case emphasizes the need for careful long - term follow - up to check for delayed esophageal perforation in patients that have undergone anterior cervical spine plating .

despite advancements in modern medical science and the health management industry , the incidence of cerebral palsy ( cp ) continues to rise1 . the most common features of cp are decreased muscle strength and abnormal muscle tone2 . cp sufferers lack theability to generate enough force to maintain antigravity postural control , which result in abnormal postures3 . the development of movement and posture may be altered by non - progressive damage to the brain and subsequent neurological impairments ( spasticity , muscle weakness , co - contractions and visual impairment)5 . studies indicate that children and adults with both mild and severe forms of cp have postural impairments6,7,8 . the emergence of sitting postural control in early infancy changes the way infants interact with the world . from the sitting position , looking , reaching , and interacting become functional and allow exploration that supports learning and further development of motor skills . therefore , independent sitting , defined as not needing support from a caregiver or pillow while sitting , is one of the first developmental goals for every child . individual differences are present between children , and characteristic signs of developmental disorders during infancy are relatively unspecific . therefore , why a specific child is not able to achieve sitting postural control is not always clear . one method of examining postural control in adults and children is to measure the center of pressure ( cop ) at the base of support using a force platform during the task of remaining upright . cop has frequently been used to investigate postural control during standing by young children who are healthy or have cp9 , 10 . the purpose of this study was to investigate the differences of the pressure distributions of the sitting postures of typical developmental ( td ) children and children with cp . twelve cp childrens were recruited from an outpatient rehabilitation clinic . they were hemiparetic , had an mmse - k score above 2411 , could maintain an independent sitting posture without support . two of the cp children were subsequently excluded because they refused to participate in this experiment . all parents of the enrolled participants provided their written informed consent to their children s participation prior to this experiment , in accordance with the ethical principles established in the declaration of helsinki . as a result of the exclusion , this study used two school chairs mounted on a force platform to assess the quiet - sitting pressure distribution of the subjects . fsa seating assessment ( canada ) the acquisition frequency was set at 5 hz . the stated working range of the device is 0200 mmhg , with a resolution of 1 mmhg . the system was also calibrated to assign absolute pressure values to the digital output from an a / d converter connected to the sensing pad . this was done by applying a pressure distribution as similar to actual conditions as possible . then thus subjects sat on one of two school chairs according to their height . the chairs were those generally used in school . for research purposes , this study used two basic school chair because students spend a long time of day - to - day sitting on them . one chair had a 40 cm floor to seat height , a 35 cm seat depth , and a 32 cm seat width and is designed for 122.4133.5 cm height of subjects . the another had a 35 cm floor to seat height , a 38 cm seat depth , and a 35 cm seat width , and is designed for 133.6152.7 cm height of subjects . it has been used for the posture symmetry in other study13 statistical analyses were performed using pasw 18.0 . descriptive statistics were calculated ( frequency , mean , standard deviation , range ) . the mann - whitney u - tests and wilcoxon s signed rank tests were used to analyze differences between the groups and differences in lesion side , respectively . the si of the age matched td group was employed as the normal criteria . table 1table 1.general characteristics of the subjectscerebral palsygroup ( n=10)typicaldevelopmentalgroup ( n=10)age ( years)8.040.827.840.94gender ( male / female)5/54/6lesion side ( right / left)4/6symmetry index ( meansd)5.041.342.302.76 shows the general characteristics of the participants . the si of the td group was significantly closer to zero than that of the cp group of children . cp and right hemiparetic cp were respectively 4.96 ( 2.24 ) and 5.12 ( 0.83 ) with no significant difference between the hemiparetic sides . the purpose of the present study was to investigate the differences in sitting posture of children with cp while they sat on school chairs . it is known that dynamic postural control during sitting can be reliably assessed using cop data of infants who are developing typically or infants with or at risk of cp14 . the principal finding of this study is that cp children s sitting posture is asymmetrical , leaning to the less - paretic side . we thought that infants who are developing typically develop the ability to sit by exhibiting an optimal range of movement variability , whereas cp may present either too much or too little variability leading to a very rigid and narrow or unpredictable set of movement solutions to achieve independent sitting . the finding of this study is agreement with previous studies that have the dissimilarities of the cop patterns of infants with cp and td have been obviously demonstrated14 . the results can not be generalized to all cp children because the sample was limited to ten children and they were at the one stage of the developmental process . future studies should assess the relationship between symmetry of sitting posture and functional activities .

[ purpose ] the purpose of this study was to investigate the differences in symmetry of sitting posture between typical developmental ( td ) children and hemi - cerebral palsy ( cp ) children . [ subjects and methods ] a school chair mounted on a force platform was used to assess the quiet - sitting pressure distribution of 10 td and 10 cp children . [ results ] the symmetry index of the td children was significantly closer to zero than that of the cp children irrespective of the latter group s hemiparetic side . [ conclusions ] sitting posture on school chairs of cp children was more asymmetrical than that of td children .

anencephaly is a devastating yet common neural tube defect which occurs due to the failure of closure of rostral pore of the neural tube that leads to exposed undifferentiated neural tissue . meroanencephaly is the classic form which presents as a lack of cranial vault bones and exposed dorsal undifferentiated dorsal neural tissue . usually , cerebral hemispheres fail to develop and only brainstem and some parts of midbrain may be present . craniorachischisis is the most severe variety whereby the defect extends into continuous spina bifida down to the thoracic vertebrae . here , we present a case of meronencephaly cared for and discharged from our neonatal intensive care unit . a male baby was born at term by vaginal delivery to a primigravida mother who did not have any antenatal visits or folic acid supplementation during pregnancy . he showed gross malformation of the cranium in the form of meroanencephaly and bilateral proptosis [ figure 1a and b ] . he was on intravenous fluid for 3 days after which he was shifted to oral feeds which he tolerated well . the vital need for folic acid supplementation prior to conception was advised and emphasized on the family . ( a - b ) the affected newborn . the flat cranial vault with defective fusion and protrusion of brain matter are notable . the cause may be multifactorial involving genes , gender , teratogen exposure , environment , diet as well as physiological abnormalities such as hyperglycemia or hyperthermia . several studies have shown a protective role of periconceptional intake of folic acid in reducing both the occurrence and recurrence of neural tube defects . without folic acid supplementation the empirical recurrence risk after one affected child is 34% and after two affected children it is 10% . exposure to valproic acid and other antimetabolites of folic acid and other toxins like lead etc . , during critical period of neural differentiation interfere with normal folate metabolism and increases the likelihood of anencephaly . neural tube closure is the result of upward bending and eventual fusing of neural tube to form the hollow tube that becomes the brain and the spinal cord . this takes place at around day 2126 days of gestation beginning at the hindbrain / cervical boundary ( closure 1 ) and spreading bi - directionally into the hindbrain and spinal region . separate closure initiation sites occur at the midbrain - forebrain boundary ( closure 2 ) and at the rostral extremity of the forebrain ( closure 3 ) . however , closure 2 found in other mammals like mice may be absent from human neurulation . failure of closure 1 leads to craniorachischisis involving midbrain , hindbrain , and spinal region . anencephaly develops if closure 1 is completed , but the closure of the cranial neural tube is incomplete , the resulting lesion may be either confined to the midbrain ( meroanencephaly ) or extend into the hindbrain ( holoanencephaly ) . anencephaly can be diagnosed antenatally by ultrasound examination and by elevated maternal alpha - fetoprotein level . anencephaly has to be differentiated from encephalocele which is a cystic extension of the brain from overlying scalp and skull defect . in addition , iniencephalocele is another differential characterized by a triad of the occipital bone defect , rachischisis , and fixed retroflexion of the head with severe cervicothoracic lordosis . the role of 0.4 mg of periconceptional folic acid in reducing the incidence of neural tube defects has been revealed by the medical research council . although the prevalence of neural tube defects has decreased after preconceptional folic acid supplementation , they continue to remain common birth defects for the obstetrician to screen , diagnose , and manage in the pregnant patient . the authors certify that they have obtained all appropriate patient consent forms . in the form the patient(s ) has / have given his / her / their consent for his / her / their images and other clinical information to be reported in the journal . the patients understand that their names and initials will not be published and due efforts will be made to conceal their identity , but anonymity can not be guaranteed . the authors certify that they have obtained all appropriate patient consent forms . in the form the patient(s ) has / have given his / her / their consent for his / her / their images and other clinical information to be reported in the journal . the patients understand that their names and initials will not be published and due efforts will be made to conceal their identity , but anonymity can not be guaranteed .

neural tube defects are a group of congenital anomalies of brain development that carry a significant risk of morbidity and mortality . anencephaly is a serious form of this defect with a very poor prognosis . it can present in three forms meroanencephaly , holoanencephaly , and craniorachischisis . meroanencephaly is considered to be the classic form of anencephaly . it manifests as a lack of cranial vault bones and exposed dorsal neural tissue resulting from defective neural tube closure in the very early period of gestation . antenatally , the diagnosis may be suggested by ultrasound examination and by elevated maternal alpha - fetoprotein level . here , we describe a case of meroanencephaly who was discharged from the hospital in fair condition despite the life - threatening anomaly .

an 82-year - old caucasian male presented to a community ophthalmologist with 6 months of decreased vision in the left eye . initial workup revealed abnormal fundus findings and the patient was referred to a local retina specialist who diagnosed nonischemic central retinal vein occlusion ( crvo ) of the left eye , demonstrated by fluorescein angiography . at that time , visual acuity in the affected eye was 20/30 . several weeks later , the patient was seen in follow - up and , on fundus exam of the affected eye , was noted to have new , apparently unrelated changes . he was subsequently referred to the havener eye institute in july 2011 for further evaluation , with concern for a viral retinitis . the patient s past medical history was significant , as he had had a kidney transplant in 2007 following advanced diabetic nephropathy . his immunosuppressive regimen at the time of presentation consisted of tacrolimus 5 mg twice daily , mycophenolate mofetil 750 mg twice daily , and prednisone 5 mg daily . his cytomegalovirus ( cmv ) status at the time of transplant was donor and recipient positive , and he had no history of systemic cmv infection . prior to the clinic s evaluation , his most recent laboratory tests revealed a normal white blood cell count and a normal tacrolimus level . examination revealed 20/40 vision in the left eye with no afferent papillary defect and normal intraocular pressure . fundus examination showed a normal optic disc with scattered intraretinal hemorrhage in the macula , tortuousity , and sheathing of the superior arcades and , in the superior periphery , a 5-disc diameter area of granular whitening with intralesional hemorrhage consistent with cmv retinitis ( figure 1 ) . the patient was started on valganciclovir 900 mg twice daily for 2 weeks , which was then decreased to 450 mg daily . at one month , the left eye vision improved to 20/25 , with regression of the retinal lesion . the hematogenous dissemination of cmv is carried out by polymorphonuclear leukocytes and monocytes.1 endocytosis of infected leukocytes by retinal vascular endothelial cells , with subsequent intracellular replication , endothelial cell damage , and ultimate compromise of the blood - retina barrier is thought to be the primary pathogenic mechanism by which cmv invades the neuroretina.2 however , some autopsy studies of acquired immunodeficiency syndrome ( aids)- infected patients with cmv retinitis have failed to demonstrate cmv infection within the retinal vascular endothelial cells , suggesting the possibility of an alternative pathogenic pathway in some patients.3,4 it has been suggested that the vascular breaches and leakage from microaneurysms frequently seen in patients with aids may facilitate the passage of infected leukocytes through the blood retinal barrier , without infection of endothelial cells.5,6 mansour et al reported a case of hemiretinal vein occlusion in a patient with aids who subsequently developed cmv retinitis in the same neuroretinal distribution.7 the authors speculated that the facilitated passage of infected leukocytes across the blood - retinal barrier in aids patients might be further enhanced by the venous stasis and ischemic endothelial damage caused by venoocclusive disease.7 a review of the literature yielded two other cases of venoocclusive events followed by cmv retinitis both in locally immunocompromised patients . park and byeon reported a 77-year - old woman without any known immunocompromising disease state and who was not taking systemic immunosuppressive medications , who was diagnosed with cmv retinitis four months after intravitreal triamcinalone injection for crvo - related macular edema.8 similarly , vertes et al reported an immunocompetent 78-year - old woman who underwent intravitreal triamcinalone injection for a branch retinal vein occlusion , then 3 months later , presented with cmv retinitis in the same hemispheric distribution.9 these reports emphasized the potential of local intravitreal immunosuppression to predispose to cmv retinitis an occurrence which has been reported elsewhere.813 neither mentioned crvo - related vasculopathy as a possible contributing factor . the association of cmv retinitis with systemic immunosuppression following solid organ transplantation is well documented.1416 this study s patient was on systemic immunosuppresants for 4 years without complication , then , within 2 months of developing crvo , developed cmv retinitis in the same eye . while these events could have occurred independently and their sequence may have been coincidental , the authors suggest that the events may support the assertion made by mansour et al,7 that secondary venous stasis and endothelial damage after venooculsive events may facilitate the development of cmv retinitis in patients already at risk .

cytomegalovirus ( cmv ) retinitis remains the most common opportunistic ocular infection in immunocompromised patients . patients with immunocompromising diseases , such as acquired immunodeficiency syndrome , inherited immunodeficiency states , malignancies , and those on systemic immunosuppressive therapy , are known to be at risk . recently , it has been suggested that patients undergoing intravitreal injection of immunosuppressive agents may also be predisposed . one previous case report speculated that there may be an additional risk for cmv retinitis in acquired immunodeficiency syndrome patients with venoocclusive disease . this case study presents a case of cmv retinitis following central retinal vein occlusion in a patient on systemic immunosuppressants .

spontaneous intracranial epidural haematoma ( edh ) is a rare complication of sickle cell anaemia ( sca ) . an association with vaso - occlusive crises[13 ] and skull infarction.[48 ] were observed in the few reported cases . with no history of head trauma the pathogenesis of spontaneous edh in patients with sickle cell anaemia is still not clearly defined . non traumatic spontaneous edh has also been reported in association with other clinical conditions like coagulopathies , infectious diseases of the skull like sinusitis , vascular malformations of the dura mater and metastasis to the dural , or skull , and chronic kidney disease . this is a case of a young man with sca ( haemoglobin ss , hbss ) with non traumatic spontaneous intracranial edh following a vaso - occlusive crisis that was successfully managed with craniotomy and evacuation of haematoma without any undesirable sequel . we report a case of an 18-year - old boy with sickle cell anaemia ( hbss ) . he presented to our unit with seven days history of frontal headache which was preceded by an episode of vaso - occlusive crisis necessitating admission and blood transfusion in a peripheral hospital . a computerized tomography ( ct ) scan of the brain done due to non resolution of headache revealed a right frontal hyper dense biconvex epidural lesion compressing the underlying brain suggestive of edh . there was no skull fracture , osteomyelitis or ct scan evidence of brain infarction [ figure 1 ] . the patient underwent a right frontal craniotomy and evacuation of edh under general endotracheal anaesthesia . intraoperatively , the skull appeared grossly normal and the patient had an uneventful postoperative recovery with complete resolution of headache . brain computed tomography scan showing right frontal acute epidural haematoma post operative computed tomography scan showing complete evacuation of haematoma haematoma occurs as a result of bleeding from ruptured middle meningeal vessels or diploic veins . spontaneous edh in a patient with sickle cell disease ( scd ) represents a rare and frequently unmentioned complication of this haemoglobinopathy . in this category of patients , typically there is no evidence of head trauma , cephalhaematoma or skull fracture . the clinical presentation of edh in scd patients is different from the classic description of post traumatic edh which usually is characterized by a lucid interval . a high index of suspicion is needed for prompt diagnosis and treatment of this rare complication of scd . spontaneous edh in patients with hbss often occurred along with skull infarction.[148 ] however , the pathogenesis has not been fully elucidated . the frequent concurrence of skull infarctions along with spontaneous intracranial epidural haematoma has been noted to suggest a mechanism whereby infarction of the skull leads to periosteal elevation , disruption of the cortical bone margin , and bleeding into the epidural space . another theory proposes that insufficient venous drainage is the inciting event that leads to oedema and haemorrhage . in spontaneous edh not associated with skull infarction , the patients skull is said to have abnormal anatomy because of a pathological process of chronic medullary haematopoiesis . the proliferation of this hematopoietic skull tissue with resultant expansion in response to acute anaemia results in the expanding hematopoietic tissue disrupting the inner and outer skull margins and precipitating extravasations of blood and hematopoietic tissue into the subgaleal and epidural spaces . our patient presented with persistent headache which started a day after blood transfusion during a vaso - occlusive crisis , there was no evidence of skull infarction on ct scan and the skull looked normal during craniotomy . however as noted by nader , the violation of the cortical bone margin resulting in extravasations of blood into epidural space in our patient might have occurred at microscopic level . unlike spontaneous edh associated with sinus infection , an infective process is a remote possibility in the pathogenesis of spontaneous intracranial edh in patients with scd . despite the background haemoglobinopathy , this patient successfully underwent standard craniotomy and evacuation of edh under general endotracheal anaesthesia with complete resolution of symptom and no undesirable complication . two patients with small edh were managed conservatively in earlier reports with radiological evidence of haematoma resolution . however , surgical evacuation remains the standard of treatment for this condition and the presence of sickle cell haemoglobinopathy should not serve as a contraindication . a search of english literature on spontaneous intracranial edh in sickle cell disease yielded a total of 12 patients ; 10 patients with hbss,[168 ] 1 patient with hbsc , and 1 patient with hbs with -thalasemia . our patient is the first to be reported in nigeria despite the high prevalence of scd in the nigerian population . this may be because ct scan machines are just becoming widely available and affordable to many patients in the country . a high index of suspicion is required among clinicians to make prompt diagnosis of this rare condition . any scd patient with symptoms and signs of raised intracranial pressure ( icp ) should be referred for cranial ct scan which will diagnose edh in all cases where it is present . spontaneous edh is a rare occurrence ; its occurrence in scd patients is rarer still . a high index of suspicion is required in the diagnosis and treatment of edh in patient with sickle cell anaemia .

non traumatic spontaneous epidural haematoma is a rare and often unmentioned complication of sickle cell disease . it is often associated with skull bone infarction . we report an eighteen year old boy with sickle cell anaemia who developed persistence headache during a vaso - occlusive crisis . brain computed tomography ( ct ) revealed a right frontal epidural haematoma ( edh ) compressing on the brain . no other etiologic factor was identified . a right frontal craniotomy and evacuation of the haematoma was performed and he made good recovery . the possible pathogeneses of this rare condition are discussed .

the celiac trunk ( ct ) is the first ventral branch of abdominal aorta and arises at the level of t12/l1 . the main branches of ct include left gastric , common hepatic , and splenic arteries . ct supplies the primary organs of the supracolic abdominal compartment . after arising from ct , the common hepatic artery runs downward and to the right to reach the first part of the duodenum . at this level the hepatic artery proper ascends in the right free margin of lesser omentum and divides into right and left hepatic arteries . presence of collateral branches , complete absence of the trunk , and bifurcation of the trunk are the reported variations of ct . very few reports pertaining to the unusual course of rha have been reported in the literature . knowledge of arterial variations of ct and its branches is useful in planning and executing the radiological interventions and surgeries in supracolic abdominal compartment . the present case is of tremendous importance because the loop of the rha might get injured during laparoscopic cholecystectomy . during the routine dissection classes for first - year undergraduate medical students , we observed concurrent arterial variations in the supracolic abdominal compartment . these variations were found in a male cadaver of indian origin , aged approximately 60 years . the left gastric artery arose from the abdominal aorta , very close to the origin of ct , at the level of twelfth thoracic vertebra . the common hepatic artery was tortuous and it terminated in front of the portal vein by dividing into right and left hepatic arteries [ figures 1 and 2 ] . the rha gave origin to gda in front of the bile duct and then made a characteristic loop around the bile duct with convexity to the right side , and then entered the liver by passing through the porta hepatis behind the bile duct [ figure 2 ] . the splenic artery passed along the upper border of the pancreas to reach the spleen . dissection of the supracolic compartment of the abdominal cavity showing the origin of celiac trunk ( ct ) in the form of hepatosplenic trunk and origin of left gastric artery ( lga ) directly from abdominal aorta , close to the origin of ct . origin of right hepatic artery ( rha ) and left hepatic artery ( lha ) is also seen . note the origin of gastroduodenal artery ( gda ) from the right hepatic artery ( rha ) in front of bile duct ( bd ) . [ splenic artery ( sa ) , common hepatic artery ( cha ) , portal vein ( pv ) , stomach ( s ) , pancreas ( p ) ] dissection of the supracolic compartment of the abdominal cavity showing the characteristic loop of right hepatic artery ( rha ) around the bile duct with convexity to the right side , before entering into porta hepatis and detached portion of gastroduodenal artery ( gda ) from the right hepatic artery ( rha ) . [ celiac trunk ( ct ) , splenic artery ( sa ) , common hepatic artery ( cha ) , right hepatic artery ( rha ) , left hepatic artery ( lha ) , portal vein ( pv ) , bile duct ( bd ) , pancreas ( p ) , stomach ( s ) ] the variations in the ct are due to unusual embryological development of the ventral splanchnic branches of the aorta . usually , ct branches into the splenic , common hepatic , and left gastric arteries . all the three of its branches might come directly from the abdominal aorta as independent branches . additional branches of the ct other than its usual branches are commonly referred to as collaterals . mburu et al . have reported the prevalence of collateral branches from ct , in 20.3% cases . a study conducted on the branching pattern of ct in kenyan population observed that the ct trifurcated in 61.7% and bifurcated in 17.9% of cases . different patterns of bifurcation of the ct such as gastrophrenic , hepatosplenic , hepatogastric , and lienogastric trunks have been reported . chitra have reported the incidence of hepatosplenic trunk in 13.1% and 2% cases , respectively . in the present case , we observed the hepatosplenic trunk with left gastric artery arising from the abdominal aorta , close to the origin of ct . computerized tomographic angiography is usually used to reveal the vascular variations of the upper abdomen . a sound knowledge of possible existence of vascular variations , as observed in the present case , is essential for radiologists and surgeons to prevent iatrogenic injuries in this region . earlier , a cadaveric study documented the origin of gda from the ct , in 3.61% of cases . gda may also arise from the superior mesenteric artery and right or left hepatic artery . nishida et al . have reported a case of gda steal syndrome during liver transplantation . this syndrome is characterized by low arterial flow towards the graft caused by a shift of flow into gda . therefore , awareness of possible existence of aberrant origin of gda is important for recognition and successful ligation of gda to prevent such consequences . in the procedure of hepatic arterial infusion pumps in hepatic arterial chemotherapy and also for liver and/or colon resection , hence , the knowledge of varied origin of gda from the rha , as in our case , is important for radiologists and surgeons for successful cannulation . the variations in the course of rha , in relation to the bile duct , are very rare . earlier , few reports have showed the presence of the tortuous rha forming the caterpillar hump which is also called as moynihan 's hump . in their study , the loop was formed around the common hepatic duct . in the present case , we report a rare case of tortuous common hepatic artery and the tortuous rha forming caterpillar hump , winding around the bile duct . the characteristic sinuousness of the rha is more susceptible to injury following the surgical procedures such as resection of tumor of the pancreatic head and other invasive interventions such celiacography and chemoembolisation of pancreatic and liver tumors and during cholecystectomy . further , gda arose from this artery close to its loop . while performing hepatic arterial chemotherapy , the aberrant origin of gda from the looped course of rha invites injury unless it is carefully recognized and dissected . though hepatosplenic trunk is not a rare finding , tortuous course of both the cha and rha is not common . further , the origin of gda from the rha close to the looped course has immense importance in the procedure of hepatic arterial chemotherapy .

celiac trunk usually trifurcates and supplies the organs in the supracolic compartment . the vascular variations are common in this region . there are reports on the variant course of right hepatic artery ( rha ) . the tortuous rha forming a caterpillar hump is a rare finding and also its providing origin to gastroduodenal artery ( gda ) is an important observation . during routine dissection of abdomen of approximately 60-year - old male cadaver , concurrent arterial variations were observed . the celiac trunk bifurcated into splenic and common hepatic arteries . the left gastric artery arose from the abdominal aorta . the common hepatic artery was tortuous and divided into right and left hepatic arteries in front of portal vein . the rha gave origin to gda and then made a characteristic loop around the bile duct with the convexity to the right side . knowledge of arterial variations of celiac trunk and its branches is useful in planning and executing the radiological interventions and surgeries in the supracolic abdominal compartment .

the dengue virus is a single - stranded rna virus of the flaviviridae family causing dengue fever and dengue hemorrhagic fever . dengue encephalopathy is usually secondary to multisystem derangement like shock , hepatitis , coagulopathy , and concurrent bacterial infection . dengue encephalitis is a different entity , which occurs due to direct neuronal infiltration by the dengue virus . we report a case of dengue shock syndrome with encephalitis , with peculiar mri findings . a 17-year - old girl presented with a history of high - grade fever and rigors for three days . she had two episodes of vomiting , generalized headache , and altered sensorium , lasting for 15 to 20 minutes , few hours prior to admission . there was no significant past medical history . on admission she was febrile , with a temperature of 101f , pulse of 132 / minute , blood pressure of 90 / 40 mmhg ( map-60 mmhg ) and cold extremities . on pulse she was pale , and skin rash , edema , icterus , and clubbing were absent . first dose of third generation cephalosporin was given empirically in view of the possibility of bacterial infection . investigations revealed hemoglobin ( hb ) of 14.5 g / dl , packed cell volume ( pcv ) of 45 , white blood cell count ( wbc ) of 7100 / cumm , with neutrophils 64% , lymphocytes 34% , and monocytes 2% . the platelet count was 1.35 lacs / cumm and esr was 48 mm at the end of one hour . u / l , sgpt-182u / l. serum albumin was 3.65 gm / dl , and inr was 2.0 . blood urea nitrogen was 39 gm / dl with creatinine of 1.4 mg / dl . there was evidence of metabolic acidosis with blood lactate 2.00 mmol / l on initial blood gas . no hypoxia or hypercapnia was seen ; x- ray chest and 2 d echocardiography were normal . ultrasonography ( usg ) showed evidence of bilateral minimal pleural effusion with mild ascites . the patient 's blood pressure and urine output improved after initial resuscitation with crystalloids . a few hours later , this young girl had an episode of generalized tonic - clonic seizure with unresponsiveness . she was intubated and ventilated in view of the poor level of consciousness after the seizure . it showed [ figure 1 ] extensive parenchymal hyperintense lesions in the bilateral cerebellar cortex , vermis of the cerebellum , entire pons and midbrain , bilateral medial temporal lobes , and both thalami , on t2 and fluid attenuated inversion recovery ( flair ) images . these lesions look hypointense on t1w images , hyperintense on flair images , and dark on the adc map . small foci of hemorrhage in the thalamic lesions looking hypointense , were seen on the t2 gre images . t 2 flair and t2-weighted images showing involvement of thalamus , pons , and cerebellum , with presence of hemorrhage on t2 and restricted diffusion on dwi cerebrospinal fluid ( csf ) analysis was done . igg / igm antibodies for dengue were positive in the serum as also on the dna polymerase chain reaction ( pcr ) for dengue . titers for hepatitis viruses were negative ( hav , hev , hbsag , hcv ) . serum procalcitonin was 0.3 ng / ml ( negative less than 0.5 ng / ml ) . her liver functions improved subsequently , and serum ammonia and electrolytes were within normal limits . she remained comatose even after correction of shock , hepatitis , metabolic acidosis , and coagulopathy . the mri brain findings were in favor of the possibility of direct extensive neuronal injury . although the mri brain findings were in favor of je , csf analysis was negative for je . therefore , the possibility of viral encephalitis due to dengue was strongly suspected . during the hospital course , she developed multiorgan dysfunction , shock , and acute respiratory distress syndrome ( ards ) . empirical antibiotics and antimalarials were continued till reports of the causative agent were obtained , and then withdrawn . table 2 shows her investigations over the next 10 days . in spite of all the treatment she continued to remain comatose , with hemodynamic compromise , and expired on the eleventh day after admission dengue virus infections are among the most common cause for hospital admissions in western maharashtra . it is estimated that 50 to 100 million infections and 25,000 fatalities occur worldwide every year . the world health organization ( who ) surveillance shows that the global incidence is rising . numerous neurological manifestations like transverse myelitis , myositis , and gullian - barre syndrome have been reported . dengue encephalopathy is a well - recognized and common entity , the incidence ranging from 0.5 to 6.2 % . the possible mechanisms are liver failure ( hepatic encephalopathy ) , cerebral hypoperfusion ( shock ) , cerebral edema ( vascular leak ) , deranged electrolytes , and intracranial bleeding due to thrombocytopenia or coagulopathy , which is secondary to hepatic failure . _ there are subsets of patients in whom the cause for neurological injury remains unclear even after excluding the above - mentioned indirect mechanisms . dengue is thought to be a non - neurotropic virus . however , there are reports of the demonstration of dengue virus and igm antigen in the cerebrospinal fluid ( csf ) of patients with encephalopathy . there are case reports by misra et al . , from india , and solomon et al . , from vietnam , 11 patients were seen with confirmed dengue infection , but no csf study was reported . solmon et al . , diagnosed dengue encephalitis in nine patients , but virus or antibody was found in the csf of only two cases . kularatne et al . , had a similar study in which they showed the association of dengue with encephalitis . on admission our patient had a history suggestive of encephalopathy with shock , coagulopathy , metabolic acidosis , and deranged liver functions . the mri findings noted in our case are most characteristic of je and not commonly seen with dengue fever . [ bilateral thalamic involvement with foci of hemorrhage , with involvement of temporal lobe and brain stem ] is very uncommon with dengue . this case is presented to highlight the possible extensive involvement of the brain by dengue virus . dengue is not classically a neurotropic virus , although there is recent evidence of direct neuronal injury . dengue encephalitis must be thought of in differentials of encephalopathy , in patients with dengue . in such cases , neuroimaging and csf analysis the virus or antibody can be isolated from the serum , but the csf samples may be negative . the dengue encephalitis is thought to be benign , but can be fatal at times . the role of an antiviral in such cases needs to be further defined because of the extensive parenchymal involvement and possible unfavorable outcome .

we report a case of dengue fever with features of encephalitis . the diagnosis of dengue was confirmed by the serum antibodies to dengue and the presence of a dengue antigen in the cerebrospinal fluid . this patient had characteristic magnetic resonance imaging brain findings , mainly involving the bilateral thalami , with hemorrhage . dengue is not primarily a neurotropic virus and encephalopathy is a common finding in dengue . hence various other etiological possibilities were considered before concluding this as a case of dengue encephalitis . this case explains the importance of considering the diagnosis of dengue encephalitis in appropriate situations .

several epidemiological studies have examined computer use as a cause of low back pain ( lbp ) and neck and shoulder pain1 , 2 . in the sagittal plane , computer users readily adopt both of these positions due to the low muscle effort required , which places a greater load on passive paraspinal structures2 , 3 . yoo et al.5 reported that the trunk flexion angle was decreased significantly immediately after shifting from the initial upright sitting posture to the cross - legged sitting posture , and significantly decreases were observed at 10 , 20 , and 30 minutes . consequently , the static posture in visual display terminal workers results in an increased forward flexion sitting posture and increased static muscle tension in the shoulder and back regions6 . when spinal structures , such as passive ligaments or active muscles , are stretched or flexed during sitting , reflexive muscle activity may be reduced , passive structures may be lengthened , and their tension may be reduced due to unsuitable stretching stimulation from the central nervous system7 . this study compared the activities of the t10 and l4 paraspinal muscles over time ( 0 , 20 , 40 , and 80 minutes ) during continuous computer work . ten male computer workers , none of whom had a history of disease or any problems with walking , volunteered to participate in the study . the average age , height , and weight of the subjects were 31.3 4.3 years , 176.2 3.0 cm , and 70.5 6.3 kg , respectively . the study purpose and methods were explained to the subjects before their participation , and they provided informed consent according to the principles of the declaration of helsinki . the t10 and l4 paraspinal muscle activities were measured using a surface emg system after 0 , 20 , 40 , and 80 minutes of continuous computer work . for the t10 and l4 paraspinal muscles , the electrodes were placed 2 cm lateral to the midline of the respective muscles . the ground electrode was positioned at the c7 process . before attaching the electrodes , all of the procedures for electrode and kinematic marker placement were performed by the same investigator to reduce variability . using two electrodes , the muscular signals were measured by applying a 1001,000 hz band - pass filter . the speed of a muscle signal could be measured at 1,000 hz . the analogue signal was converted to a digital signal with an analogue / digital ( a / d ) converter ( ni usb-6009 ; national instruments , austin , tx , usa ) . the computer workstation featured a 23-inch monitor , a keyboard and mouse on a table , and a swivel chair with five wheels . to allow analysis of back muscle activities , the armrest and backrest both the table and chair were adjustable in terms of height and were initially set to ensure that the elbows , hips , and knees were flexed at 90. the keyboard and mouse were positioned frontally 30 cm from the trunk ; the monitor was reclined by 20 ; and the top of the display was set at eye level ( standard sitting posture ) . the t10 and l4 paraspinal muscle activities were measured using a surface emg system after 0 , 20 , 40 , and 80 minutes of continuous computer work using the program hansoft ( hansoft , uppsala , sweden ) . spss ver . 12.0 ( spss , chicago , il , usa ) was used to assess differences in the t10 and l4 paraspinal muscles over time ( 0 , 20 , 40 , and 80 minutes ) . repeated measures one - way analysis of variance ( anova ) was used to examine the significance of differences in measures . in all analyses , the t10 and l4 paraspinal muscle activities significantly increased after 20 ( 24.8 10.2% and 23.4 9.1% , respectively ) and 40 ( 26.3 15.2% and 28.6 11.8% ) minutes compared with the initial values ( 19.5 11.2% and 17.6 12.0% , respectively ) ( p < 0.05 ) . however , the t10 and l4 paraspinal muscle activities were decreased significantly after 80 minutes ( 16.7 8.2% and 13.9 9.3% ) compared with those at 0 , 20 , and 40 minutes ( p < 0.05 ) . this study investigated the changes in t10 and l4 paraspinal muscle activities over time during 80 minutes of computer work . when lumbar and thoracic muscle activation levels decrease , passive tissues ( ligaments , lumbodorsal fascia , etc . ) a prolonged load added to the ligaments of the lumbar spine may affect the lumbar spine , stimulate pain receptors , and eventually become a source of lbp in computer workers8 , 9 . in addition , prolonged flexion during sitting was found to result in redistribution of the nucleus within the annulus2 . mork and westgaard also suggested that constitutive exposure to a relaxed sitting posture exacerbates lbp in computer workers10 . our results showed that the t10 and l4 paraspinal muscle activities were increased significantly after 20 and 40 minutes of computer work compared with the initial values , while they were decreased significantly after 80 minutes compared with those at 0 , 20 , and 40 minutes . decreases in trunk muscle activity result in an increased load on the lumbar discs and ligaments2 , 11 . when the sitting posture changes from erect to slumped , postural muscle activity decreases , as the trunk posture supported by the passive paraspinal structures responds to gravity11 . this slumped sitting posture may cause strain , instability , or injury in the lumbopelvic region2 . yoo reported that regular , passive exercises are effective at preventing lbp in computer users9 . impaired proprioception in sitting and standing positions can be related to lbp2 , 3 . this results suggest that workers using computers for short periods would benefit from back muscle stretching exercises due to increased tension in these muscles , while strengthening exercises would be more effective than stretching for long - term computer work , due to decreased back muscle activation .

[ purpose ] this study compared the t10 and l4 paraspinal muscle activities over time during continuous computer work . [ subjects ] ten male workers participated in this study . [ methods ] the t10 and l4 paraspinal muscle activities were measured using a surface emg system after 0 , 20 , 40 , and 80 minutes of continuous computer work . [ results ] the t10 and l4 paraspinal muscle activities after 20 and 40 minutes were increased significantly compared with the initial values , while they were decreased significantly after 80 minutes compared with the initial readings . [ conclusion ] this study suggests that workers using computers for short periods would benefit from back muscle stretching exercises due to increased tension in these muscles . for longer computer work , strengthening exercises would be more effective than stretching due to decreased back muscle activation .

actinic granuloma ( ag ) was first described by obrien in 1975 , as a rare granulomatous disease . lesions present on sun - exposed sites , most commonly on the face , neck and scalp with a slight predominance in middle - aged women . they are generally asymptomatic , single or multiple , annular or polycyclic lesions that can measure up to 6 cm , showing slow centrifugal expansion , with an erythematous elevated edge and a hypopigmented , atrophic center . despite initial debate regarding whether ag would represent a variant of granuloma annulare , it is now considered a distinct entity , believed to be related to photo damage . a 60-year - old female rural worker presented with an annular lesion with elevated , erythematous borders and a hypopigmented , atrophic center , located on the right calf [ figure 1 ] . it measured approximately 14 7 cm and was slowly extending centrifugally for more than 2 years , despite treatment with systemic and topical antifungals . annular lesion with erythematous scalloped borders and an atrophic center occupying the right calf the patient had a previous history of dyslipidemia , treated with atorvastatine , 40 mg daily . she had no history of diabetes mellitus , temporal arteritis , polymyalgia rhematica , relapsing polychondritis and pseudoxanthoma elasticum . she had worked in the fields for several decades , usually with exposed legs due to the use of skirts . complete blood count , erythrocyte sedimentation rate , chemistry panel with renal and hepatic function and nuclear antibodies were all negative or within normal range . microscopic examination of skin scrapings was negative for fungal elements . a biopsy taken across the border of the lesion showed a superficial dermal granulomatous infiltrate with multinucleated giant cells that exhibited elastophagocytosis [ figure 2 ] . with verhoeff van gieson stain , solar elastosis in the upper and mid dermis became evident , as well as phagocytosis of elastotic fibers [ figure 3 ] . multinucleated giant cells composing a granulomatous infiltrate occupying the upper and mid dermis , with foci of elastophagocytosis ( arrow ) , adjacent to areas of solar elastosis ( star ) . ( h and e , original magnification 200 ) a detailed image of elastophagocytosis using verhoeff van gieson stain . ( original magnification 400 ) a diagnosis of o brien ag was made , and the patient started clobetasol 0.05% ointment , that she maintained daily for 2 months with slow but progressive extension of the lesion . she then began acitretin 25 mg daily for 6 months , with stabilization of the lesion and further discontinuation of therapy . according to obrien , ag consisted of a granulomatous reaction to solar damage , with histiocytes and foreign - body type multinucleated cells engulfing elastotic fibers within a background of solar elastosis . the absence of elastic fibers would characterize the center of the granuloma , corresponding clinically to the atrophic , hypopigmented center of the lesion . he concluded that this was also the likely diagnosis for other previously described entities such as miescher 's granuloma of the face and necrobiosis lipoidica presenting on the face and scalp and in 1985 he further characterized ag in four subvariants , the original giant cell annular form , the necrobiotic form , the histiocytic and the sarcoidal variant . predilection for sun - exposed skin and a three - zone typical histopathology were the clinical and pathological hallmarks of ag and seemed to support the actinic hypothesis proposed by obrien . hypothetically , elastotic fibers resulting from actinic radiation could represent an antigenic trigger for an autoimmune granulomatous reaction that would then ensue . a report associating ag with giant cell temporal arteritis ( gca ) further strengthened this belief . in gca , actinic damage of the internal elastic lamina of the artery would occur with surrounding presence of matrix metalloproteinase 9 and macrophages . while mcgrae was demonstrating a t - helper cell infiltrate with perivascular and interstitial distribution amidst the granulomatous reaction of ag , also elastase - derived elastin peptides were reported to be high in the sera of patients with gca , being targeted by t - helper lymphocytes , in an analogous manner to what was happening in ag . the actinic pathogenesis , however , was opposed by ragaz and ackerman that viewed elastolytic granuloma as a variant of granuloma annulare in solar - damaged skin . others preferred the descriptive term of annular elastolytic giant cell granuloma ( aegcg ) , stating its independent classification from granuloma annulare ( highlighted by the absence of mucin deposition ) but not finding definite evidence for the actinic hypothesis . they believed that the association between solar elastosis and granulomatous inflammation should not necessarily imply a cause case reports showing the occurrence of the granuloma on sun - protected skin further supported this idea . currently , the denomination of aegcg is still in use , with disregard for obrien 's original view on the actinic pathogenesis recently , reports associating lesions of ag in skin affected by late - onset x - linked protoporphyria and vitiligo again corroborate the causative role of elastotic fibers and solar damage . however , in our patient , this location was subjected to chronic sun exposure , in accordance to most described reports . other associated entities include diabetes and leukemia , polymialgia rheumatica , relapsing polychondritis and pseudoxanthoma elasticum , although the relevance of these associations remains unproven . obrien ag is reported to have a slow but self - limiting course and can take up to 10 years to resolve . nonetheless , because lesions are often extensive and frequently affect sites of cosmetic importance such as the face , therapy is necessary in order to prevent new lesions and enlargement of existing ones . although oral and topical corticosteroid therapy is generally ineffective , there are reports of successful treatment with systemic retinoids , such as acitretin but also with isotretinoin . in our patient , the progression of the lesion was halted after 6 months of the former drug and did nt return on its suspension . nonetheless , we can not prove whether this was due to acitretin or to the self - limiting nature of the disease . ag is currently a widely accepted entity , even though its pathogenesis is not fully clarified . its specific histopathologic findings suffice to establish it outside the granuloma annulare spectrum , however , the full grasp of the role of actinic damage and its consequences not only in the skin , but also systemically , still remains elusive . the role of actinic damage has been favored by the concomitant occurrence of obrien 's actinic granuloma , protoporphyria and vitiligo . its diagnosis should be considered in patients presenting with annular plaques with photodistribution even if they are located in an unusual site . despite its slow self - limiting course , therapeutic options include systemic retinoids , namely acitretin .

obrien first described the actinic granuloma in 1975 , as an infrequent granulomatous disorder occurring in sun - exposed skin , with a slow but often self - limited course . ever since its initial description , the actinic physiopathogenic hypothesis has been debated by many authors . we report a 60-year - old female rural worker that presented with a 14 7 cm annular lesion with erythematous elevated borders and an atrophic center on the right calf . the lesion was evolving for 2 years , and histopathology confirmed actinic granuloma . she started acitretin with halting of disease progression after 6 months of therapy . our case can also be associated to actinic damage , despite its unusual location , therefore highlighting the role of solar elastosis in the development of obrien actinic granuloma .

a 37-year - old man initially presented with subacute paresthesias characterized by numbness , tingling , and abnormal cold sensation . he was treated conservatively for presumed peroneal nerve palsy from compression phenomenon associated with crossing his legs . he had hyperreflexia at both his ankles and knees , worse on the right . at this time , the presumptive diagnosis was broad - based disk bulge causing lumbar radiculopathy . two weeks later , he returned noting abnormal sensation involving the right lower leg and the anterior and medial lower halves of the thigh , with associated right ankle weakness , hypoesthesia in the left first interdigital space , and decreased control of his right leg leading to a clumsy gait with several falls . family and past medical history was negative for osseous , neurologic , or dermatologic diseases . physical examination at this time revealed right - sided foot drop , asymmetric hyper - reflexia on both posterior tibialis and achilles tendons , more pronounced on the right , and bilateral ankle clonus . gait was abnormal ; he favored plantar flexion and inversion of right foot but not the left . romberg sign was positive with decreased balance on right leg standing more than the left . vitamin b12 level was normal and rapid plasma reagent ( rpr ) was negative for syphilis . lumbosacral spine magnetic resonance imaging ( mri ) did not show any nerve root compression . nerve conduction and electromyogram ( emg ) demonstrated an abnormality in nerve conduction in the spinal region . lumbo - sacral plexus and peripheral nerve conduction were normal . given the predominantly l4 to l5 deficits , and the knowledge that lumbar spinal cord segments are in the thoracic vertebral segments , a thoracic mri was performed ( fig . 1 ) . this revealed a large extramedullary mass at t7t8 with severe compression of the spine and deviation of the spinal cord to the right . follow - up mri showed return of the cord to its position in the spinal canal . annual mri scans of his spine for the subsequent 3 years have not shown tumor recurrence . t1-weighted axial mri thoracic spine , showing a dumbbell - shaped tumor arising from the dorsal nerve root , with mass effect on normal spinal cord . schwannomas , also known as neurilemoma , are rare , benign tumors of the nerve sheath of peripheral nerves . schwannomas have an incidence of 3% of all spinal tumors . in a paraspinal location , they are the commonest cause of intradural extramedullary tumors but may also be extradural or extramedullary ( 1 ) . paraspinal schwannomas involve the dorsal nerve roots , affecting people in the fourth and fifth decades of life ( 3 ) . paraspinal schwannomas are frequently asymptomatic and diagnosed incidentally on imaging of the spine ( 3 ) motor and sensory deficits , as seen in our patient , rarely occur ( 3 ) . schwannomas can develop anywhere along the course of the sciatic nerve causing radiculopathy ( 4 , 5 ) . these tumors may be sporadic and solitary or multiple , as in neurofibromatosis type 1 ( nf1 ) . schwannomas are associated with 60% of nf1 ; the tumors occur at an early age , are multiple , and are prone to recurrence and malignant transformation . patients with schwannoma may be screened for nf based on clinical features , including six or more light brown spots on the skin ( caf - au - lait spots ) , measuring 5 mm or more in children or more than 15 mm in adolescents and adults ; two or more neurofibromas , or one plexiform neurofibroma , freckling in the area of the armpit or the groin ; lisch nodules or iris hamartomas ; an optic nerve glioma , sphenoid dysplasia , pseudoarthosis or kyphoscoliosis , or the tibia ( one of the long bones of the shin ) . the presence of tumors in any of the afore - mentioned features is characteristic of nf1 . a parent , sibling , or child with nf is an additional clue , although 3050% of nf cases may follow spontaneous mutation . paraspinal schwannomas are frequently asymptomatic and are diagnosed incidentally on imaging of the spine ( 3 ) . motor and sensory deficits , as seen in our patient , rarely occur ( 3 ) . schwannomas can occur anywhere along the course of the sciatic nerve causing radiculopathy ( 4 , 5 ) . interestingly , in our patient , the lumbar radiculopathy was caused by a schwannoma at the t7 dorsal nerve root tumor as it exits through its foramen , compressing the adjacent lumbar spinal cord and lumbar nerve roots as they course down to exit through the lumbar foramen . therefore , the lumbar spinal cord may be located in the area of the thoracic region of the vertebra ( 6 ) . this explains why the lumbosacral spine mri did not reveal the cause of lumbar radiculopathy symptoms , since the lesion was not at the neuroforamen . the progression from lumbar radiculopathy to myelopathy was due to severe spinal compression from the nerve root tumor . this is shown on the mri ( figs . 1 and 2 ) . t1-weighted sagittal mri thoracic spine showing sharply defined margins of the schwannoma , which arises separately from the neural tissue . ct scan will show ill - defined mass in the affected area . on mri , they are hypointense to isointense on t1-weighted images and hyperintense on t2-weighted images ( 1 , 3 ) . contrast enhancement varies depending on cystic or solid components of the tumor . the mass may cause compression and deviation of the spinal cord , seen as in the mri of this patient ( 3 ) . hemorrhagic , cystic , or fatty degeneration may also occur , thus , distinguishing it from other paraspinal tumors ( 1 ) . , schwannomas do not arise from the nerve fibers and so the tumor is easily separated from nerve fibers without neurologic compromise . in the rare case that this is impossible , the remnant tumor may be followed up radiographically if it is histologically benign . postoperative transient neurologic deficit from surgical dissection and trauma may occur . in conclusion , patients with lumbar radicular pain may rarely have tumors along the course of the spinal nerve . in patients with equivocal clinical examination , emg may help localize the lesion along the very long course of the lumbar nerve fibers from the lumbar segment within the thoracic vertebrae to the nerve exit through the lumbar foramen to the supplied organs .

compression or irritation of the sciatic nerve and its branches , the common fibular and tibial nerves , causes sciatica which is a common syndrome characterized most often by radiating pain from the lower back down the legs and also manifesting as sensory and motor deficits . sciatica is a common presentation of lumbosacral disc prolapse and degenerative disease of the lumbar spine in ambulatory settings . schwannomas rarely cause sciatica ; hence , it is seldom considered in evaluation of a patient with radiculopathy . our patient presented with lumbar radiculopathy , mild degenerative changes on lumbar magnetic resonance imaging ( mri ) scan , and failed conservative treatment . myelopathy was confirmed with electromyogram ( emg ) . thoracolumbar spine mri revealed the schwannoma in the thoracic region . he recovered neurologic function after tumor excision . this case highlights the diagnostic challenge that may arise in evaluating a patient with lumbar radiculopathy , negative lumbosacral spine imaging , and failure of conservative therapy .

pulmonary artery sarcoma ( pas ) is a rare malignancy arising from the mesenchymal cells of the intima of the pulmonary artery . it is frequently misdiagnosed as pulmonary thromboembolism ( pte ) , although chest computed tomography ( ct ) can help differentiate pas from pte by showing a low - attenuation filling defect occupying the entire lumen of the proximal or main pulmonary artery , expansion of the involved arteries , or extraluminal tumor extension . therefore , a new noninvasive technique for the diagnosis of pas is needed to avoid a delay in diagnosis and in treatment and to prevent unnecessary surgery . fluorodeoxyglucose positron emission tomography ( fdg pet ) have helped to differentiate between benign and malignant tumors . in our case , fdg pet showed increased uptake in the pulmonary artery lesion which confirmed on biopsy as pas . ct chest showed focal hypodense filling defect in right pulmonary artery measuring 3.8 2.9 2 cm which extends extraluminally abutting left atrium . eccentric filling defect in left pulmonary artery was also noted . magnetic resonance imaging ( mri ) showed enhancement of the filling defect within right pulmonary artery . with the suspicion of pte or pas , pet / ct showed an intense uptake in the hypodense filling defect [ figure 1a - d ] with a standardized uptake value ( suvmax ) of 11 . also scattered subcentimetric pulmonary nodules was noted , largest measuring 8 mm in right upper lobe [ figure 1e ] . patient underwent endoscopic bronchial ultrasound - transbronchial needle aspiration ( ( ebus - tbna ) which confirmed spindle cell sarcoma of pulmonary artery [ figure 2 ] . whole body fluorodeoxyglucose positron emission tomography / computed tomography ( fdg pet / ct ) maximum intensity projection image ( a ) , axial ct ( b ) showing hypodense filling defect in pulmonary artery , axial fused pet / ct ( c ) , and coronal fused pet / ct ( d ) showing uptake in pulmonary artery lesion and axial fused pet / ct ( e ) showing lung nodules in both the lungs suggestive of metastasis ( a and b ) histopathological examination revealed small tiny cluster of atypical spindle cells ( hematoxylin and eosin stain ; original magnification 100 ) pas , first described by mandelstamm in 1923 , is a rare and potentially lethal tumor , usually diagnosed during surgery or autopsy . it is thought to arise from multipotential mesenchymal cells of the intima and generally arises from the right , left , and main pulmonary artery . the reported age ranges from 13 to 86 years with the majority of cases occurring in the middle age . clinical symptoms are generally nonspecific and often occur at the end stage of the disease . frequent symptoms are dyspnea , chest pain , hemoptysis , weight loss , malaise , cough , syncope , and fever . although pas is a rare tumor , the true prevalence is underestimated as many cases of pas are misdiagnosed as pte leading to inappropriate therapy . both diseases appear as intraluminal filling defects in the pulmonary artery system on contrast - enhanced ct scans and differentiating radiologically between pas and pte is relatively difficult . however , over the last decades , improvement of imaging techniques has allowed to distinguish the two entities before surgery . yi et al . , reported that ct findings favoring the diagnosis of pas included a low attenuation filling defect occupying the entire luminal diameter of the proximal or main pulmonary artery , expansion of the involved arteries and extraluminal tumor extension as shown in our case . in addition , pas has more likely a heterogeneous appearance with areas of necrosis , hemorrhage , and ossification . it occurs frequently unilaterally , in contrast with the often bilateral involvement of pulmonary arteries in the thromboembolic disease . pet - ct may help to make a differential diagnosis between pas and pte . according to the several case reports of fdg - pet , blood thrombi showed negative fdg uptake ; whereas , a malignant tumor such as sarcoma showed positive fdg uptake . while , some tracer activity within a thromboembolic disease may occur , intense tracer localization within a central pulmonary arterial filling defect strongly suggests primary pulmonary arterial malignancy . we think that the positive fdg uptake on pet - ct in the current case supports this suggestion . the cornerstone of the treatment consists of surgical resection through endarterectomy , tumorectomy , or pneumectomy . surgical resection remains the primary treatment to provide significant palliation and offers the only chance of cure . our case demonstrates 18f - fdg pet / ct uptake in pas and also lung metastasis .

pulmonary artery sarcoma ( pas ) , although rare , must be considered in the differential diagnosis of pulmonary thromboembolism ( pte ) . this tumor is highly malignant and the prognosis is very poor . as much as the standardized uptake values ( suvs ) at fluorine-18 fluorodeoxyglucose positron emission tomography ( 18f - fdg pet ) have helped in differentiating between benign and malignant tumors , visualization of a low - attenuation filling defect within a pulmonary artery on contrast - enhanced chest computed tomography ( ct ) can be suggestive of a malignancy , such as pas , if the lesion shows high fdg uptake at pet . we present a case of pas that showed high fdg uptake on integrated fdg pet / ct and with lung metastasis . patient underwent endoscopic bronchial ultrasound - transbronchial needle aspiration ( ebus - tbna ) , which confirmed spindle cell sarcoma .

it is treated with surgery , chemotherapy , radiation therapy , hormonal therapy , and newer biologicals . assessing the response to newer therapies is of paramount importance , not only to identify candidates who are not responding to therapy , but also to avoid unnecessary delay in institution of definite treatment and to reduce potential adverse effects . 18-fluoride fluorodeoxy glucose ( f - fdg ) positron emission tomography ( pet)-computed tomography ( ct ) is being increasingly utilized in evaluating early response to therapy . potential pitfalls exist even in this modality and the phenomenon of metabolic flare phenomenon is elucidated in this case report . a 53-year - old female , a case of bilateral breast cancer , presented to the oncologist with back pain of recent onset . she was a known case of bilateral breast cancer and was treated with bilateral mastectomy 6 months before . she was referred for a whole body f - fdg pet - ct to our center following the end of chemotherapy . she underwent whole body fdg - pet - ct scan as per standard institution protocol . the initial whole body f - fdg pet scan ( figure 1 ; a - maximum intensity projection [ mip ] , e - ct axial , i - axial fused pet - ct ) showed a metabolically active lesion in the d9 vertebra with evidence of lytic changes in the corresponding vertebra on ct . another focus of fdg uptake was also noted in the posterior elements of d7 vertebra . the maximum standardized uptake value normalized to the lean body mass ( standardized uptake values [ suvmax ] ) was 3.6 . she underwent three cycles of chemotherapy based on ( paclitaxel , adriamycin and bevacizumab ) . the whole body f - fdg pet scan performed 12 days after three cycles of chemotherapy ( b - mip , f - ct axial , j - axial fused pet - ct ) showed increase in the fdg uptake and the suvmax ( from 3.6 to 4.5 ) . the injected activity and the imaging time were not significantly different between the two scans . serial maximum intensity projection images and the axial computed tomography ( ct ) and axial fused positron emission tomography ( pet)-ct of the serial pet scans during the course of second line treatment of bilateral cancer breast are shown . initial images ( a , e , i - prechemotherapy ; showing a focus of elevated fluorodeoxy glucose ( fdg ) uptake in a lytic lesion in the posterior body and right pedicle of the d9 vertebra . follow - up scan ( b , f , j - three cycles after chemotherapy ) showed an increase in the fdg uptake ( flare phenomenon ) with sclerotic changes on ct ; pet scan after six cycles of chemotherapy ( c , g , k ) and nine cycles of chemotherapy ( d , h , l ) show complete suppression of fdg uptake with dense sclerotic change in the involved vertebra the patient underwent a f - fdg pet scan ( c - mip ) , at completion of six cycles of chemotherapy . dense sclerotic changes were noted on ct indicating healing response ( g - ct axial , k - axial fused pet - ct ) . patient underwent end of treatment pet scan 6 weeks after completion of nine cycles of chemotherapy ( d - mip , h - ct axial , l - axial fused pet - ct ) which did nt reveal any other new abnormality . flare phenomenon is a paradoxical increase in the tracer avidity in a previously diseased site during the course of treatment . flare phenomenon is a well - known process noted in bone scintigraphy during the course of treatment of osseous metastases . flare phenomenon has also been proposed as an indicator of treatment efficacy . however , flare phenomenon is rare in f - fdg pet scans in patients with breast cancer on conventional therapy . this is because , f - fdg uptake reduces when there is a favorable response to chemotherapy which is evidenced by fall in suv . f - fdg pet scan serves as an excellent tool in evaluating the response of tumor to chemotherapy . in patients with breast cancer , metabolic flare phenomenon flare phenomenon has been previously described in lung cancer patients receiving bevacizumab in addition to the standard treatment . the flare phenomenon noted in the index case might be due to addition of bevacizumab to the second line chemotherapeutic drugs . paradoxical increase in the suvmax on follow - up pet scan may also be due to variety of technical factors , the most important of which is significant variation in waiting period between the two scans ( delay in imaging increases the suv ) . follow - up scans should ideally be acquired with the same delay as the initial pet scans . flare phenomenon is a potential indicator of responsiveness of the breast cancer to hormonal therapy .

increase in radiopharmaceutical uptake is an indicator of progression of disease . paradoxical increase in the radiopharmaceutical uptake also occurs during favorable response to therapy , which is designated as flare phenomenon . flare phenomenon is well documented on bone scinitgraphy when initially noted lesions show increased radiotracer uptake after therapy is instituted . this happens despite favorable response to the treatment . the osteoblastic activity associated with healing response of bone tumors is the cause of flare phenomenon . recently , metabolic flare phenomenon has been described in patients with breast cancer who undergo hormonal therapy . changes in the hormonal level during initial part of the treatment is the cause of metabolic flare . we describe a patient with bilateral breast cancer who underwent second line chemotherapy along with bevacizumab . serial positron emission tomography scans done showed interesting phenomenon of metabolic flare .

an 83-year - old woman presented with a complaint of epiphora from her left eye . the patient returned three months after surgery , complaining of a mass at her previous skin incision site . according to the patient 's history , the mass had noticeably increased in size over the previous two months . upon examination , a dome - like brown - to - black , polipoid , greasy surfaced elevation of 11.5 cm in diameter was observed ( fig . a punch biopsy was performed for diagnosis , which demonstrated irritated sk with hyperkeratosis , basaloid cell proliferation , acatholysis with squamous eddies , and inflammatory infiltrates in the epidermis ( fig . the etiology of sk is unknown , although sun exposure , hpv , and egf have been suggested as possible etiologies . they hypothesized that the growth factor being induced by the healing graft site stimulated the appearance of the sk locally . in our patient , the sk also occurred postoperatively , and we think the egf may have had a role in the development of sk on the incision site . histologic changes in sk after its irritation have been well documented by several other authors . there are a few case reports describing the sudden , macroscopic transformation of irritated sk . mochizuki et al . reported a case of giant irritated sk that mimicked a verrucous carcinoma . our case shares many characteristics with those of previous cases , as the sk noticeably increased in size over several months . however , unlike the cases described before , our patient had no visible skin lesion at the incision site . we might hypothesize that in this case , the irritation being induced by the surgical incision stimulated very small or microscopic skin lesions at the incision site . therefore surgeons need to check the patient 's skin carefully for skin lesions on the incision site , and they should consider how to treat these lesions prior to surgery . to our knowledge , this is the first reported case of a irritated sk found on a previous skin incision site .

an 83-year - old woman had undergone an external dacryocystorhinostomy with silicone intubation . before the surgery , no skin lesions were observed on the incision site . three months after surgery , the patient complained of a brown - to - black pigmented elevation at her previous skin incision site . a punch biopsy of the pigmented mass was performed . the histopathologic findings confirmed the clinical diagnosis of irritated seborrheic keratosis ( sk ) . sk can occur several months postoperatively and can suddenly increase in size , so surgeons need to carefully check patients ' skin prior to surgery . to our knowledge , this is the first reported case of irritated sk discovered on a previous skin incision site .

transthoracic echocardiography is a commonly performed non - invasive investigation of cardiac anatomy and physiology and is indicated for the diagnosis of common cardiac pathologies . these include valvular heart disease , ischaemic heart disease , cardiomyopathies and infective endocarditis , to name a few . echocardiography is also indicated in the diagnosis and assessment of intra - cardiac masses , but additional investigations may be required to evaluate them further . it is recognised that non - cardiac pathology can present with cardiac symptoms and artefact on cardiovascular investigations [ 1 , 2 , 3 , 4 , 5 ] . we present the case of a patient diagnosed with hiatus hernia presenting with cardiovascular symptoms and appearance of intra - cardiac mass on transthoracic echocardiography , and also review the relevant literature . an 81-year - old female with a background of stable ischaemic heart disease and hypertension was referred to the cardiology department complaining of dyspnoea on exertion over a period of 12 months . she denied fever , weight loss , change in appetite , dyspepsia or gastrointestinal bleeding . her medication list included aspirin , a statin , a proton - pump inhibitor and an angiotensin receptor blocker . physical examination was normal ; in particular there were no finger clubbing , no cardiac murmurs on auscultation and no signs of fluid retention such as elevated jugular venous pressure and peripheral oedema . her haemoglobin , leukocyte count , renal and liver function blood tests were all normal . b - type natriuretic peptide was within normal limits . given her past medical history of cardiovascular disease , she was referred for echocardiography to assess for structural or functional cardiac disease possibly contributing to her unexplained dyspnoea . alarmingly , the study demonstrated a large echolucent mass with the appearance of a left atrial space - occupying lesion ( fig . 1 ) . left ventricular size and contractile function were preserved and there was no significant valvular disease . right ventricular size and function were normal and there was no pericardial effusion . to further delineate the intra - cardiac mass , the patient underwent cardiac magnetic resonance imaging ( mri ) the study revealed no intra - cardiac masses , with normal heart structure and function . it demonstrated a large hiatus hernia containing food and compressing and distorting the posterior wall of the left atrium ( fig . although often asymptomatic , hiatus hernia can present with symptoms mimicking those of cardiovascular pathology such as dyspnoea , chest pain and syncope . furthermore , by cardiac compression , hiatus hernia can produce dynamic electrocardiography changes and simulate the appearances of cardiac masses on echocardiography [ 1 , 2 , 3 , 4 ] . our patient presented with symptoms of breathlessness and was suspected of having congestive cardiac failure , given a past history of ischaemic heart disease and hypertension . it is believed that extrinsic compression of the left atrium results in these symptoms due to impaired left atrial filling leading to pulmonary venous hypertension . a case series of patients with significant left atrial compression by gastric structures presenting with haemodynamic instability and respiratory failure has been reported . the differential diagnosis of an intra - cardiac mass includes tumours ( benign or malignant ) , thrombi , vegetation , normal anatomical variants ( such as eustachian valve , chiari network , etc . ) and artefact . hiatus hernia mimicking intra - cardiac mass due to sonographic artefact was diagnosed in this case using multi - modality imaging . although transthoracic echocardiography is an excellent initial diagnostic technique to evaluate and diagnose cardiac masses , invariably definitive diagnosis and comprehensive view of these structures is confirmed by other imaging modalities such as cardiac mri , through the excellent spatial resolution and range of sequences of this modality to characterize tissue . cardiac mri has been found to have greater efficacy in the detection and morphological evaluation of cardiac masses compared to echocardiography , which may be limited by poor sonographic windows . the growing number of indications for performing echocardiography will undoubtedly result in misdiagnosis of cardiac masses in these patients , irrespective of symptoms . this in turn will have implications for the patient as well as for clinical resources . in conclusion , as highlighted by this case , one should consider extra - cardiac pathology such as hiatus hernia in the assessment of cardiac masses demonstrated on echocardiography , and appreciate that these can present with symptoms resembling those from primary cardiac disorders . the patient presented in this report gave informed consent to her case being submitted for publication and is aware that there are no patient - identifying details in the text or images submitted .

hiatus hernia is a common medical condition . an elderly woman being investigated for dyspnoea of unknown aetiology had a transthoracic echocardiogram as part of her investigations . this alarmingly demonstrated a large left atrial mass . further assessment with cardiac magnetic resonance imaging revealed a large hiatus hernia compressing the left atrium and no intra - cardiac mass . the case succinctly highlights gastrointestinal pathology simulating cardiac symptoms and masses .

factors adversely affecting short term survival include impaired consciousness , leg weakness and increasing age . on the other hand , long term survival the pattern of survival is changing with time in many populations with gradual improvement of survival and decrease in mortality.3 review of the literature suggests that immediate mortality after stroke approaches 30% in the first month.4 five year survival rates range between 35 - 45% . furlan reported a 5 year survival rate in stroke patients of 77% compared to 85% in matched normal controls.4 al rajah , in a study from saudi arabia showed that ischemic strokes accounted for 76.2% of all strokes . strokes caused by intro - cerebral and sub - arachnoid hemorrhage constituted 21.4% and 2.4% respectively.5 radiological changes in brain computed tomography ( ct ) correlates with survival . hankey reported that lesions greater than 50 mm in diameter have a higher mortality than lesions less than 50 mm8 . poor prognosis is also noted in ct findings of intracerebral hemorrhage , mass effect and deep intracerebral lesions.7 in this study , the prognostic effects of age , sex , hypertension , diabetes mellitus and presentation in coma on the survival pattern of stroke patients presenting to a referral hospital , are reviewed . all patients hospitalized with definite stroke at king fahd specialist hospital ( kfsh ) , buraidah , saudi arabia for the period between june 1986 and june 1991 were reviewed to determine the effect of age , sex , hypertension , diabetes mellitus , heart disease , atrial fibrillation , transient ischemic attacks , and the pathological type of the stroke on survival . the medical records of patients were reviewed retrospectively according to pre - established diagnostic and inclusion criteria . stroke is defined as acute focal or global loss of cerebral functions with symptoms lasting more than 24 hours , without an apparent cause other than the vascular origin . patients were included if they were saudi adults of 18 years of age or more , with a definite first stroke . the clinical diagnosis was made by the admitting physician and reviewed independently by two members of the study group ( em and mn ) . the ct brain scans were reviewed by two observers , a radiologist ( ka ) and a physician ( mn ) to determine the pathological type of the stroke . the reliability of the method was confirmed by assessing a random 10% sample of the radiographs without knowledge of prior reports . survival duration was estimated from the date of onset of stroke to the date of death or last contact . kaplan - meier analysis was used with log - rank procedure to compare unadjusted survival . cox proportional hazard analysis was used to investigate the effect of each prognostic factor on survival . in all data analysis , the bmdp statistical software package was used ( university of california press , berkeley , 1991 ) . there were 106 males and 68 females with a male to female ratio of 1.56:1 . hypertension , diabetes mellitus and heart disease were found in 54 ( 31% ) , 49 ( 33% ) and 57 ( 28% ) of patients respectively . at the time of analysis , 126 patients , 72.4% of all patients , were alive . over the follow up period , 19 patients ( 10.9% ) died within 2 weeks , 32 patients ( 18.4% ) died within 4 weeks and 38 patients ( 21.8% ) died within 24 weeks . while the median survival has not been reached , the lower 95% confidence limit for the median survival was estimated at 103.14 weeks . kaplan - meier survival curve for all patients in the study is shown in figure 1 . figures 2a , 2b and 2c show the survival curves of the patients who presented with or without coma , heart disease and age below and above 60 years consecutively . unadjusted analysis of survival as a function of various variables is shown in table 1 . a logistic regression analysis to examine the adjusted effect of these variables to estimate their independent influence on survival overall survival of patients with stroke survival of patients with heart disease compared to patients with no heart disease ( bold line ) . survival of patients presenting in coma compared to patients who were conscious on presentation ( bold line ) survival of patients below age 60 years ( bold line ) compared to patients of age above 60 years survival estimates ( unadjusted analysis ) logistic regression analysis of factors influencing survival adversely ( cox proportional hazards ) mortality risk was statistically significant for patients above age 60 ( p < 0.04 ) , patients presenting in coma ( p<0.003 ) and patients with heart disease ( p<0.0009 ) . there was no significant statistical difference in mortality risk due to gender , hypertension or diabetes mellitus . cerebral infarction was the most frequently observed pathological type of stroke seen in 142 patients ( 82% ) . there were 17 patients with intracerebral hemorrhage with a mean survival of 105 weeks , and 6 patients with sub - arachnoid hemorrhage with a mean survival of 29 weeks . in common with other reports , the single most important determinant of overall survival in the saudi stroke patients , was the state of consciousness on presentation , with the presence or absence of coma1237 . age over 60 years and the presence of heart disease also adversely affected survival . ischemic , valvular and hypertensive disease were the most frequent cardiac culprits . . the poor survival may be the result of the added mortality risk from heart disease or the associated high risk of cerebral vascular disease in patients with coronary artery disease . it was not possible to study other factors such as body mass index , previous stroke , hemianopia , and blood glucose due to the limitations of the retrospective study . several multi - variate models have been designed to predict the effects of different variables on the outcome of stroke . the prediction value for the belfast model is 75%,8 guy 's hospital 65%9 and for uppsala 50%.10 the state of consciousness and urinary incontinence predicted good and poor outcome with accuracy comparable to that of multi - variate models . these multi - variate methods may have possible values in defining prognostic factors for designing clinical studies . yet in prospective studies , these models did not show advantage over simple clinical variables . in this study , data from a regional referral hospital was analyzed to define the prognostic factors useful in predicting the outcome and mortality of stroke in the saudi population . these factors can be used to evaluate the effectiveness of interventions in the treatment of stroke . factors affecting survival after stroke in saudi patients , has been found to be more or less similar to those commonly observed in other populations.11

background : prognosis of stroke has been studied in various population . factors adversely affecting short term survival include impaired consciousness , leg weakness and increasing age.aim of the study : in this study , the prognostic effects of age , sex , hypertension , diabetes mellitus and presentation in coma on the survival pattern of stroke patients presenting to a referral hospital , are reviewed.methodology:the medical records of all patients hospitalized with definite stroke at king fahd specialist hospital , buraidah , for the period between june 1986 and june 1991 , were reviewed . the cranial ct scans were also reviewed.results:one hundred and seventy four patients , 106 males and 68 females , with a mean age of 64 years who had definite stroke were studied to estimate overall survival and the various risk factors influencing it . the case records and cranial ct scans were reviewed.conclusion : the factors adversely affecting survival in this study , were age above 60 ( p<0.04 ) , presentation in coma ( p<0.003 ) and pre - existing heart disease ( p<0.0009 ) . there was no significant effect on survival due to sex , hypertension and/or diabetes mellitus .

humans may be either definitive hosts ( adult tapeworms residing in the human intestines ) or intermediate hosts ( the larval stage residing in the tissues ) for t. solium . humans can become an intermediate host when they either ingest cysticerci ( encysted larvae ) or consume eggs . cysticerci are ingested by eating undercooked pork while eggs are usually ingested by eating contaminated foods , including uncooked or inadequately washed raw vegetables or salads . when eggs reach the stomach , gastric enzymes will break down the egg wall and release larvae . the larvae penetrate the intestinal mucosa into the blood circulation and form cysticerci which can be found in any part of the body , especially central nervous system ( neurocysticercosis ) . one is isolated cyst ( cysticercus cellulosae ) , and the other is racemose cyst ( cysticercus racemosus ) . we report a case of cerebral cysticercosis in bangkok , thailand of which imaging studies showed an atypical convoluted lesion , similar to sparganosis , another kind of cestode infection in the brain . a 30-year old thai female presented to the outpatient clinic with generalized tonic - clonic seizure 1 week ago . the brain mri revealed a conglomeration of several small ring enhancing lesions of 1.91.41.6 cm in size , at the cortical posterior left middle frontal gyrus . routine blood examinations showed an absence of eosinophilia , and stool examinations showed no parasites . a left frontal craniotomy was done . a greyish white cyst of about 121 cm in size filled with clear fluid and small white nodule was removed . multiple sections studied revealed necrotic material with neutrophils , mononuclear cells , some eosinophils , surround by granulation tissues ( fig . nearby the necrotic material , a cyst with cavitory larva of t. solium was found . the patient was treated with albendazole for 3 weeks and was doing well at 1 month follow up . parasitic infections of the central nervous system can share similar clinical presentations , depending on the location , size , and number of lesions , and the inflammatory responses evoked by the parasites . regarding cerebral infections in developing countries , the possible diagnosis of the patient with confluent ring enhancing lesion by mri can be due to cysticercosis , sparganosis , and even tuberculoma . regarding the imaging studies , kim et al . described the features of sparganosis by mri as widespread white matter degeneration and cortical atrophy , and mixed - signal lesions with irregular dense enhancement of central foci . viable cysticerci can be diagnosed on mri as cystic lesions which appear hypointense on t1 and hyperintense on t2 with an eccentric nodule . when the cyst degenerates , fluid leaks out and creates inflammation which is seen as peripheral enhancement on mri and ct . . claimed that soft tissue cysticercosis can be seen as elongated calcification along the muscle fibers on plain radiographs and ct scan , once the larvae become a final calcified stage , but not the early stage of live cysticerci . so , a combination of mri and ultrasonography can be confidently used to diagnose non - invasive cysticercosis in soft tissues . spargana differ from cysticerci by the absence of bladder walls and armed scolex and the presence of solid non - cavitated body . teitelbaum et al . claimed that the scolex in cysticercosis is found in nearly 50% of cases . the reason why we can not find the scolex is related to stages of the disease . according to the stage of the cyst , our case was compatible with colloidal vesicular stage , in which larvae begin to degenerate and scolex disintegrates , with surrounded striking inflammatory responses as shown in table 1 . unfortunately , we lack molecular facilities in our center . we hope that we will have a chance to investigate more in the future .

cysticercosis and sparganosis are not uncommon parasitic infections in the developing world . central nervous system infection by both cestodes can present with neurological signs and symptoms , such as seizure and mass effect , including brain hernia . early detection and accurate diagnosis can prevent a fatal outcome . histological examinations of brain tissues can confirm the diagnosis of cerebral cysticercosis , which differs from sparganosis by the presence of a cavitated body . we report here a case of cerebral cysticercosis which has the similar clinical and imaging findings as sparganosis .

currently for super - obese individuals , bariatric surgery is the only evidence - based approach that results in clinically significant and sustainable weight loss . with the increasing prevalence of obesity , there is an increased demand for bariatric surgery . subsequently , a large number of patients shall be presenting with the complications of bariatric surgery . a 42-year - old caucasian female underwent laparoscopic roux - en - y gastric bypass in february 2007 . she subsequently developed gallstones , a known complication of bariatric surgery due to rapid weight loss . she presented to our unit in february 2012 with a week long history of right upper quadrant pain associated with vomiting . blood tests revealed deranged liver function tests : alkaline phosphatase of 169 u / l and alanine aminotransaminase of 188 u / l . a computed tomographic scan was organized confirming the appearances of a dilated common bile duct and the potential of a calcified stone in the common bile duct . she was managed conservatively with analgesia and ursodeoxycholic acid to attempt to dissolve the stones . her liver function tests and pain improved , and she was discharged with an outpatient magnetic resonance cholangiopancreatography ( mrcp ) . in march 2012 , her mrcp showed a dilated common bile duct of 10 mm and two small filling defects suggestive of gallstones . again , this confirmed the dilated common bile duct , but also showed free drainage of bile into the duodenum and no gallstones ; hence , therapeutic interventions were not undertaken . she was referred to the gastroenterologists for further advice , but as of june 2012 she was still symptomatic . ultimately , an endoscopic retrograde cholangiopancreatography ( ercp ) and sphincterotomy was deemed to be necessary to alleviate her symptoms . owing to the altered anatomy post roux - en - y gastric bypass , this was to be a technical challenge . the bypass of the distal stomach and proximal small bowel is achieved by anastomosis of the gastric pouch and the jejunum known as the roux alimentary limb . the biliary pancreatic limb is then anastomosed to a small bowel . as a result it is technically challenging to perform an ercp in the conventional manner , two subsequent port sites were used : a 5-mm port site in the right upper quadrant and a 10-mm port in the left hypochondrium ( fig . 1 ) . stay sutures were placed in a diamond - shaped configuration around the gastrotomy site these can allow both traction and counter - traction . a sterile ercp scope inserted manually through a left 10-mm port site all under laparoscopic visualization . there were no obvious gallstones in our patient ( fig . 2 ) . the gastrotomy site was closed with a two layer closure : the first layer as a running stitch and then as a horizontal mattress with 2.0 vicryl . she was admitted for an overnight stay and discharged the following day . upon review of the patient in a routine 6-week follow - up clinic , she was asymptomatic and was discharged back to the care of her general practitioner . with the increased demand for bariatric surgery worldwide , there will be an increased role of endoscopy to diagnose and manage complications . with altered anatomy , the transgastric approach can be used as an access point into the gastrointestinal tract . we have demonstrated that the technique when performed correctly can be a safe and viable option in most district general hospitals , which would certainly have a laparoscopic surgeon and a gastroenterologist on site .

the prevalence of bariatric surgery is increasing worldwide and as a direct consequence , there shall be an increasing number of patients presenting with the complications of bariatric surgery , often to non - specialist units . the authors report a case of a 42-year - old caucasian female who had previous laparoscopic roux - en - y gastric bypass , open cholecystectomy and abdominoplasty presenting with right upper quadrant pain in keeping with retained common bile duct stones . after the failure of conservative management , a laparoscopic - assisted transgastric endoscopic retrograde changiopancreatography and sphincterotomy was performed . we shall be describing our technique .

pyomyositis is charactarized by abscesses in the skeletal muscles of mainly the pelvis and lower extremities and is rarely seen in temperate regions . due to an increase in immunocompromised patients the incidence of pyomyositis is rising in the western world . we describe a case of a young man with tenderness of his muscles , caused by multiple abscesses and swine flu . a 31 year old , otherwise healthy young man presented to our emergency department with muscle ache of his legs , a sore throat and fever . physical examination showed subfebrile temperature , pain of both upper legs and buttocks , but no local inflammation or swelling was noticed . laboratory examination showed slightly elevated levels of coreactive protein ( crp ) , white blood count ( wbc ) and creatine kinase ( ck ) . swine flu the patient was admitted to the department of internal medicine and therefore oseltamivir treatment was started . however this treatment rendered no success , since the pain increased and spread to both legs and arms . the subsequent ct - scan showed multiple abscesses ( fig . 1 , 2 ) in the psoas muscle , in both legs and arms . all wound and blood cultures showed staphylococcus aureus , mrsa and pv negative . our patient was also treated with intravenous flucloxacilline and rifampicine . pyomyositis was first described in 1885 by scriba as an infectious disease of mainly the skeletal muscles ( 4 ) . it can be seen in all stages of life , but most frequent in adolescents , with a male : female ratio of 3:1 . the incidence of pyomyositis in temperate regions is increasing , due to an increase of predisposing factors , such as immunocompromised patients , e.g. due to hiv , diabetes , malignancies and chronical diseases . preceding trauma of the affected muscles , by for example fierce exercise , has also been proposed as an important predisposing factor of pyomyositis . the etiology of pyomyositis however is still unknown ( 2,5,6,7 ) staphylococcus aureus is cultured in 70 - 95 % of the cases , which often is an mrsa strand . other microorganisms cultured are streptococcus sp , escherichia coli and mycobacterium tuberculosis . cultures and gram - stains are therefore very important to guide proper antibiotic treatment . ( 1,2,3 ) there are three stages of pyomyositis ; stage i is characterized by vague symptoms ; sore muscles , cramping , low - grade fever and discomfort . most patients only seek medical help in stage ii , because of formation of abscesses in the muscles , approximately 10 - 21 days after the onset of the disease . if not treated properly this will proceed into stage iii , with serious illness , metastatic abscesses , sepsis , necrosis of the muscles , multiorgan failure and higher mortality . ( 7,8 ) diagnosing pyomyositis may be challenging , due to the vague presentation in stage i. when the symptoms become more outspoken , apart from history and physical examination , imaging studies are usefull . mri is the diagnostic tool of choice , because it can detect affected muscles in an early stage . also more abscesses can be found at unexpected sites , especially when the patient is not responding to treatment . but , as with our patient , it is of great importance to scan the entire body to detect other abscesses in unexpected regions , if a patient does not respond adequately to the initial treatment . stage i can be treated with adequate antibiotic therapy alone . in case of abscess formation proper surgical or radiologic drainage of all abscesses the duration of administration of antibiotics is usually 7 - 10 days , but needs to be adjusted regarding extensiveness of the disease ; number of abscesses , osteomyelitis and immune status of the patient . ( 1 ) if treated properly , most patients heal without additional complications and low mortality rates have been described , up to 1.5% . ( 1 ) the presentation of our patient is very extraordinary , because of the extent of the disease and the abundance of abscesses . an explanation for the severity of the ilness might be found in the preceding or simultaneous swine flu infection . myositis due to influenza and even rhabdomyolysis due to h1n1infection have been described ( 9,10 ) . we consider muscletrauma due to the viral infection to be a predisposing factor for the extensiveness of the disease in this case . we conclude that pyomyositis can be widespread in patients , which is sometimes hard to diagnose , but should be thought off , especially in times of an influenza pandemic .

pyomyositis is a bacterial infectious disease of the large skeletal muscles , mostly seen in tropical regions . a case with such a multitude of abscesses has never been described in the western world , nor following an h1n1 infection . we report of a 31 year old man who presented himself with complaints of muscle pain and fever . his complaints were attributed to a proven h1n1 infection . however , despite proper therapy his condition worsened . he had developed multiple abscesses in both arms and legs . after surgical and radiologic drainage and antibiotic treatment our patient healed without any symptoms . pyomositis is related with immuno - compromisation . our patient might have been immunocompromised due to the h1n1 infection . rhabdomyolysis following influenza has been described before , however a relation with h1n1 never was . imaging studies help detect and confirm the diagnosis . when missed , serious complications may arise .

baclofen [ 4-amino-3-(-4-chlorophenyl)-butanoic acid ] is a centrally acting gamma - aminobutyric acid ( gaba ) agonist . it is currently used for alleviation of signs and symptoms of skeletal muscle spasticity and spasm , particularly in patients with multiple sclerosis or in patients with spinal or cerebral disorders . although the precise mechanism of action of baclofen is not fully known , it is capable of inhibiting polysynaptic and monosynaptic reflexes at the spinal level by hyperpolarization of afferent terminals.[13 ] ingested baclofen is rapidly and extensively absorbed ; the rate of absorption may be reduced with increasing doses . in addition , the drug is moderately lipophilic , approximately 30% of baclofen bound to protein and it can cross the blood - brain barrier . in a healthy person , most of the ingested baclofen ( 6985% ) is eliminated by the kidneys without changes in urine and 15% is metabolized by the liver to an inactive form . in the patients with the impaired renal function and especially in the patients with end stage renal disease ( esrd ) , half - life of the drug is prolonged ; it is between 4.5 and 6.8 h in healthy people , but increases in patients with esrd . exposure to as little as 5 mg baclofen may cause severe toxicity soon after ingestion . two esrd patients , a 48-year - old diabetic man ( case 1 ) and a 79-year - old non diabetic man ( case 2 ) presented with a profound central nervous system depression due to baclofen toxicity . the causes of esrd were diabetic nephropathy and unknown ; also they were on maintenance hemodialysis ( 4 h , three times a week ) for 1 and 2 years . they both had used this drug for their lower back pain . after receiving low dose of baclofen , the patients became disoriented , in a state of confusion and were referred to our hospital for evaluation ( cumulative dose of 30 mg in case 1 and 20 mg in case 2 ) . on presentation , they were in a confused state ( glasgow coma scale e4v3m6 in case 1 and e3v3m5 in case 2 ) and therefore , they were admitted in the intensive care unit ( icu ) . the vital sign revealed mild hypertension ( 150/80 and 140/90 mm hg in case 1 , 2 respectively ) and their respiratory rate and pulse rate were in the normal range , and they had no fever . in physical exam , there were no focal neurological deficits and the remainder of the examination was also not diagnostic . laboratory data including serum sodium , potassium , glucose , calcium , phosphorus , and magnesium , liver transaminases and thyroid function test were unremarkable except for azotemia and mild anemia as would normally be expected ( in case 1 hemoglobin 9.8 g / dl , urea 87 mg / dl , creatinine 7.6 mg / dl and in case 2 hemoglobin 10.8 g / dl , urea 76 mg / dl , creatinine 6.8 mg / dl ) . computed tomography ( ct ) of the brain was normal in case 1 and showed cortical atrophy in case 2 . according to the differential diagnosis of baclofen - induced encephalopathy and after exclusion of other identifiable causes , baclofen was stopped and they received intensive hemodialysis . with adequate supportive care including mechanical ventilation and intensive hemodialysis , their neurologic symptoms gradually disappeared without any neurologic sequelae over 3 and 4 days respectively . although , nervous system side effects including transient drowsiness , sedation , dizziness , weakness , fatigue , coma and respiratory depression are known side effect of baclofen in patients with normal renal function , however , they usually do not occur after use of low dose of the drug , and , in addition , most of the severe nervous system side effects were reported following intrathecal injection . on the other hand , in the patients with the impaired renal function and especially in the patients with esrd , the half - life of baclofen is significantly increased and the recommended dose or even low doses of baclofen as little as 5 mg daily or a cumulative dose of 15 mg could cause a rapid baclofen accumulation and severe baclofen intoxication . in addition , as we reported in the present case reports profound central nervous system side effects , including coma and respiratory depression could also develop soon after the initiation of therapy . there are few report of baclofen intoxication in the patients with chronic kidney disease who also demonstrated the importance of considering level of renal function when prescribing baclofen for each cause . chu - lin et al . reported two patients with chronic renal failure not requiring dialysis and baclofen - induced encephalopathy ; a 68-year - old male with urea of 45 mg / dl and creatinine of 3.4 mg / dl and a 73-year - old woman with urea of 55 mg / dl and creatinine of 4.8 mg / dl , who were admitted with altered consciousness that rapidly developed after use a low dose of baclofen for intractable hiccups . with supportive treatment , cessation of baclofen and one course hd in second patient , neurologic symptoms recovered completely without any neurologic sequelae . wu et al . also reported a 70-year - old woman with esrd who became disoriented and confused after receiving a cumulative dose of oral baclofen 45 mg in 3 days for leg muscle soreness . she received a single 4-h session of emergency hd , and the patient was discharged from the hospital 3 days later with complete recovery of consciousness . although , mechanism of baclofen elimination during hemodialysis is not well understood up to 79% of the serum baclofen eliminate during one hemodialysis session . therefore , although , more data are needed for a routine recommendation , however , according to previous reports and our cases , it seems that hd may be an option for baclofen overdose in the patients with chronic renal failure and especially in the patients with esrd . the half - life of baclofen in patients with crf and esrd is significantly increased baclofen could cause rapid and severe intoxication . therefore , physicians should be aware of this possibility and it is necessary to avoid use or reduce baclofen dosage in patients with the impaired renal function and in finally hd may be an option for baclofen toxicity in these patients and especially in the patients with esrd .

we report two end stage renal disease ( esrd ) patients , who rapidly developed neurotoxicity after taking baclofen , a centrally acting gamma - aminobutyric acid agonist . they presented to our hospital in a state of confusion . on physical examination , there were no focal neurological deficits and the remainder of the examination was also not diagnostic . laboratory data were unremarkable . the consciousness improved with supportive treatment and intensive hemodialysis over 3 - 4 days . in conclusion , physicians should be aware of the possibility of baclofen - induced neurotoxicity in patients with renal disease , especially in esrd patients and it is necessary to avoid or reduce baclofen dosage in these patients .

actinomycosis is a rare , chronic , suppurative , endogenous infection presenting with multiple draining sinuses . facial swelling without discharging sinuses , resembling desmoid tumor , cysts involving salivary glands or bone are rare entities . we report a rare case of cervicofacial actinomycosis in a female child with multiple discharging sinuses of upper lip mucosa . a 6-year - old female child presented with an upper lip swelling [ figure 1a ] discharging pus from the oral mucosa underlying central part of lip with mild pain exacerbating while eating . the swelling progressed from few millimeters to about 2 cm 2 cm over 1 year , started discharging thick , pus with white granules . no antecedent external trauma or dental ailments and procedures or self - manipulation were reported . ( b ) labial mucosa showing granular pus discharging sinus ( arrow ) the child was a term baby , delivered vaginally without instrumentation during labor . the milestones were average , but bacillus calmette guerin ( bcg ) vaccine was not administered . the past medical and family histories were non - contributory and other siblings were in good health . examination revealed a soft , fluctuant , erythematous , cystic midline upper labial swelling , 2 cm 2 cm with three openings on the mucosal surface discharging , granular white pus , and a single central sinus opening on the overlying skin discharging serous grey pus [ figure 1b ] . physical and systemic examinations were normal , dental hygiene good , and no congenital anomalies apparent . the possibilities of actinomycosis , infected congenital upper lip commissure and tubercular pathology were considered . the wet mount preparation of pus sample demonstrated granules and gram stained smears showed gram positive cocco - bacilli and polymorphonuclear cells . aerobic culture of pus on blood agar and mac conkey agar were sterile after 24 h of incubation at 37c . culture on brain heart infusion blood agar in candle jar grew pale cream , gritty , colonies after 1 week incubation at 37c [ figure 2a ] . smear from culture showed characteristic branching filamentous gram positive beaded rods , which were non - acid fast on ziehl neelsen and modified zn staining . ( a ) brain heart infusion blood agar slant showing straw colored colonies of actinomyces israelii . ( b ) photomicrograph of dermoid cyst showing keratinized stratified squamous epithelium with sebaceous glands , hair follicles , and skeletal muscle in sub - epithelial tissue ( h and e , 40 ) therapy was started with amoxicillin / clavulanic acid and surgical excision done with continuation of antibiotics post - operatively for 4 weeks . tissue histopathology revealed a cyst measuring 1.2 cm 0.6 cm 0.5 cm grossly , with keratinizing stratified squamous epithelial lining microscopically . sub - epithelia showed fibro - collagenous tissue , dilated congested blood vessels , hair follicles , skeletal muscle , and sebaceous glands entrapped in the muscle layer with chronic inflammatory cell infiltrate [ figure 2b ] . actinomycosis is seen once annually in major medical centers with a male to female ratio of 3:1 . the ambit of clinical disease encompasses cervicofacial actinomycosis , abdominopelvic , thoracic and cerebral involvement . the disease is uncommon in children and cervicofacial actinomycosis is rarer still . the causative bacteria , actinomyces spp . constitute normal endogenous flora in oropharynx , gastrointestinal and female genital tracts and are non - virulent . the antecedent factors for actinomycosis include poor dental hygiene and caries , extractions or other traumatizing procedures , perforating injuries , human bites and compound fractures . this marks the onset of a chronic , suppurative , granulomatous , diffuse swelling with multiple discharging sinuses . this child with good oral and dental hygiene and absence of previous trauma had a midline upper lip congenital defect diagnosed as dermoid cyst on histopathology , which came to light due to an actinomycotic infection . neck swellings due to tonsillar mass , isolated mid - cervical tumor like granuloma without discharging sinuses and ruptured cyst or desmoid tumor - like facial actinomycosis are reported in adults . cervical mass , painless , non - tender , non - inflammatory thyroid or expansile mandibular masses , salivary gland , and mandibular bone involvement are documented during childhood . an unusual pediatric case of facial actinomycosis with intracranial lesion symptomatic with headache , cranial nerve dysfunction , and ataxia is also reported . actinomycosis may mimic osteomyelitis , granulomatous disease or neoplasia and those with nodules and fistula resemble tuberculosis , sporotrichosis and nocardiosis . we rejected tuberculosis and nocardiosis due to a non - indurated mantoux test and absence of acid - fast bacilli in pus and mycobacterial culture . visualization of granules macroscopically and microscopically substantiated by culture of a. israelii clinched the diagnosis . though difficult , yet culture is by far the most accurate method of diagnosis and requires repeated attempts . the pathologist opined a congenital dermoid cyst , which is characterized by sac like birth defects containing hair , fluid , teeth , and skin glands although the specific identification of actinomyces spp . remained crypt on histopathology . clinically , dermoid cysts present as non - progressive midfacial swellings without inflammation . on the contrary , inflammatory swellings progress rapidly . in our case , progression over a year , positive microscopy and culture , and the presence of inflammatory cells were evidence of an infective process in a congenital cyst . this case is a rare case report , which highlights the occurrence of actinomycosis complicating an undiagnosed congenital defect . it also emphasizes the importance of collaborative efforts of clinicians in suspecting and laboratory physicians in confirming atypical cases .

pediatric cervicofacial actinomycosis is a rare occurrence consequent to dental infections and manipulations or maxillofacial trauma . the clinical presentation ranges from multiple draining sinuses to swellings resembling tumors and cysts . the present unusual case had congenital dermoid cyst of mid upper lip with actinomyces israelii infection identified on microscopy , culture , and histopathology . a successful outcome in the present case was obtained using combination of medical and surgical treatment .

bony tumours of the chest wall are rare ; the differential diagnostic considerations comprise benign tumours such as osteochondroma , chondroma , fibrous dysplasia ( fd ) and malignant tumours such as soft tissue sarcomas , osteosarcoma and askin tumours . surgical resection is the primary treatment for most benign and malignant chest wall tumours , which can be performed with acceptable morbidity . various methods for closure of chest wall defects have been described , including prosthetic mesh , musculocutaneous flaps , titanium plates and bone cement [ 24 ] . rigid fixation techniques provide good chest wall stability , but too rigid fixation might induce pain and increase the risk for infection . in this study , we present a case of a 59-year - old woman with a large tumour caused by fd , which was surgically resected with direct reconstruction using a sandwich technique of a mesh reinforced with methyl methacrylate in a double - layer marlex mesh . a 59-year - old woman presented at the outpatient clinic of the department of pulmonology with complaints of chronic cough . apart from gastroesophageal reflux disease , she had no history of disease . she presented with chronic cough without other pulmonary complaints ; specifically , she had no haemoptoe , shortness of breath or weight loss . pulmonary function tests revealed normal lung function with vital capacity of 4.5 l ( 130% of predicted ) , forced expiratory volume in 1s of 3.8 l ( 130% of predicted ) and a diffusing capacity of 105% . on plain x - ray , a mass in the left thoracic wall additional imaging was obtained by a chest ct scan , which showed a calcified tumour on the left thoracic wall growing from the second rib with slight impression of the lung with a maximal diameter of 4.7 cm ( fig . figure 1:(a ) a calcified tumour on the left chest wall growing from the second rib with slight impression of the lung with a maximal diameter of 4.7 cm . ( b ) bony tumour originating from the left second and third rib , with some pleural adhesions . ( a ) a calcified tumour on the left chest wall growing from the second rib with slight impression of the lung with a maximal diameter of 4.7 cm . ( b ) bony tumour originating from the left second and third rib , with some pleural adhesions . with no other lesions explaining the chronic cough , resection through video - assisted thoracoscopy ( vats ) a bony tumour was found originating from the left second and third rib , with some pleural adhesions ( fig . the procedure was converted to thoracotomy to perform wide resection of the thoracic wall ( fig . 2 ) . the size of the thoracic wall defect following resection measured 8 by 5.5 cm a patch was prepared using two polypropylene meshes and interpolate of methyl methacrylate ( fig . the patch was placed and tightened to the periosteum using prolene sutures ( fig . finally , subcutaneous tissue and skin were closed in layers using a vicryl and intracutaneous monocryl suture . figure 3:preparation of the patch with two polypropylene meshes and interpolate of methyl methacrylate . figure 4:positioning of the mesh and fixation to the periosteum of adjacent ribs . definitive pathology of the specimen revealed fd that was radically removed . at follow - up differential diagnostic considerations for chest wall tumours comprise benign tumours such as osteochondroma , chondroma , fd and malignant tumours such as soft tissue sarcomas , osteosarcoma , askin tumours or metastasis of the ribs . surgery of chest wall tumours indicated for confirmatory biopsy , correction of deformities , failure of non - surgical therapy , prevention of pathological changes and eradication of symptomatic lesions . in general , chest wall reconstruction is indicated for any chest wall defect greater than 5 cm , although on the posterior side , function might not be impaired with defects up to 10 cm . the methods for chest wall reconstruction can grossly be divided in two groups : rigid fixation ( using a sandwhich technique as described here or titanium ) and non - rigid fixation ( non - rigid mesh or myocutaneous flaps ) . the technique of using a polypropylene mesh with methyl methacrylate as described in our case was developed 25 years ago . pulmonary complications are the most important cause of morbidity after chest wall resection and reconstruction . such complications are better prevented by rigid fixation techniques , as they better prevent paradoxical chest movements [ 2 , 7 ] . furthermore , the technique is easy to apply by surgeons who only sporadically encounter large chest wall defects . in contrast to myocutaneous flaps and titanium plates , this technique does not require specific skill or equipment [ 4 , 8 , 9 ] . a further problem with titanium plating is that such osteosynthesis material might eventually break , as no bone healing is expected after large resections of the chest wall . , we achieved excellent functional and cosmetic results with this polypropylene meshes and a methyl methacrylate sandwich technique . in conclusion , fd of the chest wall is a rare condition that can present with chronic cough , pain or respiratory failure . the use of a sandwich technique a mesh reinforced with methyl methacrylate cement can ensure chest wall integrity while preventing a frail chest .

bony tumours of the chest wall are rare and radical surgical resection forms the cornerstone of treatment . closure of the defect following radical resection can be challenging . we report the case of a 59-year - old woman with a large tumour of the thoracic wall , which was surgically resected with direct reconstruction using a sandwich technique with a palacos patch placed in a double - layer marlex mesh . pathological examination showed a fibrous dysplasia of the rib . the patient had a good functional and cosmetic result .

cleidocranial dysplasia ( ccd ) , also known as marie and sainton 's disease or scheuthauer - mariesainton syndrome or mutational dysostosis . mutations in the core binding factor alpha-1 gene located on chromosome 6p21 have been suggested to be the cause of ccd.[24 ] rearrangement of long arm of chromosome numbers 6 and 8 has also been suggested in favor of ccd . it is also considered to be an autosomal dominant skeletal dysplasia caused by mutations in the bone / cartilage specific osteoblast transcription factor runx2 gene . the most striking features of this syndrome are partial or complete absence of clavicles causing unusual mobility of the shoulders ( seen in about 10% of cases ) and late closure of fontanelles resulting in frontal bossing . prolonged retention of deciduous teeth with delay in eruption of succedaneous teeth is the characteristic oral finding . involvement of facial bones , altered eruption patterns and presence of multiple supernumerary teeth makes it significant for a dentist . a 35-year - old male patient reported with complaint of absence of teeth in upper and lower arches . he was able to adduct his shoulders when asked to bring his shoulders forward to the midline . this confirmed an anomaly in clavicle formation [ figure 1 ] ; oral examination showed prolonged retention of deciduous teeth . adduction of shoulders the patient was subjected to radiographic examinations of chest , skull , hand wrist , cephalometric , and panoramic radiographs . panoramic radiographs revealed abnormally retained primary teeth , nine impacted permanent and supernumerary teeth ( excluding second and third molars ) [ figure 2 ] . lateral cephalogram showed shortening of cranial base anteriorly ; in the posterior region the physiological mandibular angle was replaced by a rounded outer contour of the mandible . the clinical findings of scheuthauer - marie - sainton syndrome , although present at birth , are often either missed or diagnosed at a much later date . the radiographic evaluation of patients is the most important and reliable means to confirm the diagnosis . the affected individuals are of short stature , with a broad head and frontoparietal bossing . one of the characteristic oral finding is prolonged retention of the deciduous teeth and a delay in eruption of the permanent teeth . various views regarding the etiology of noneruption , such as lack of cellular cementum , defectiveness in postcementum formation , presence of thick connective tissue between oral epithelium and dental follicle , delayed tooth formation and maturation are suggested . the reason for the formation of multiple supernumerary teeth is still unknown . a short lower facial height , anterior inclination of mandible and mandibular hypoplasia of the masseter muscles is another feature , which eventually leads to hyperfunction of the temporal muscles . this will make the anterior border of the mandibular ramus parallel to the posterior border , and the coronoid process will be directed upwards and backwards in such cases . radiographic examination reveals the widely patent anterior fontanelle and sutures with wormian bones in cranium . the clavicles typically are reduced to single or double fragments on each side with middle part being deficient . the widened symphysis pubis ( distance between pubic bones ) results from a delay in ossification during adulthood . other changes include hypoplasia and anterior rotation of the iliac wings and wide sacroiliac joints . the femoral epiphyses are large , the femoral necks broad . hands and feet demonstrate various anomalies including shortening and broadening of carpal , metacarpal , tarsal , metatarsal bones . there is hypoplasia of distal phalanges with massive epiphyses ( marked in thumb ) and shortening and premature epiphyseal closure of middle phalanges . congenital pseudoarthrosis is characterized by the absence of one of the clavicles ( usually the right clavicle ) . in majority of the cases , involvement is unilateral with a marked predominance of the right side . the cases are sporadic , there is no other bone involvement , and most cases presumably heal malformed bones . pycnodysostosis or the maroteaux - lamy syndrome is characteized by osteosclerosis , delayed suture closures , dysplasia of distal phalanges , anodontias , and delayed eruption of permanent teeth osteosclerosis along with the absence of supernumerary teeth are sufficient to distinguish scheuthauer - marie - sainton syndrome from pyknodysostosis . genetic counselling is appropriate for prospective parents with a family history of scheuthauer - mariesainton syndrome , where one or both parents are affected . complications may arise during delivery in case of narrow pelvis since scheuthauer - marie - sainton syndrome patients generally have multiple impacted permanent teeth , prolonged retention of primary teeth , and multiple supernumerary teeth , they can develop masticatory problems with aging . many approaches have been suggested for treatment of such problems , including the following : removal of the impacted permanent , supernumerary and deciduous teeth , combined with installation of over - dentures or orthodontic traction of the impacted permanent teeth ; removal of the supernumerary teeth immediately after completion of mineralization of their crowns combined with removal of the overlying bone of the permanent teeth to facilitate their eruption . thus , two critical factors in the success of these treatments are the correct timing of the treatment and the number of supernumerary teeth . in our case ,

scheuthauer - marie - sainton syndrome also known as cleidocranial dysplasia ( ccd ) is an autosomal dominant disorder characteristically presented with multiple supernumerary teeth ; partial or complete absence of the clavicles ; and open sagittal sutures and fontanelles . this condition was first reported by meckel in 1760 . there is also evidence that it existed in the prehistoric man . more than 1,000 cases have been reported in the medical literature regarding this syndrome . a case of a 35-year - male of ccd with multiple supernumerary teeth is being reported . the diagnostic and management aspects of this syndrome are discussed .

tick investigations were conducted in january 2006 in the fatick region of sine - saloum in west - central senegal ( figure 1 ) . this region is a major area of pig production and a center for trade with dakar and casamance in sengal and bissau - guinea ( 10 ) . despite no national reporting , o. sonrai has also been found in the fatick region of senegal in previous investigations on human relapsing fever ( 11 ) . sampling sites in the fatick region of sine - saloum , senegal . three criteria were selected to assess the role of o. sonrai in asf ( 12 ) : presence of this tick in domestic pig buildings and warthog habitats , its probability of contact with domestic pigs and warthogs , and its natural infection with asfv . we searched for o. sonrai in pigpens in 5 villages or groups of villages , 2030 km apart per sampling site , along a north - south transect , as well as in warthog burrows in wild areas from 3 different forests ( figure 1 ) . for tick collection , we used a portable gasoline - powered vacuum cleaner adapted for burrow - dwelling ticks ( 13 ) ( figure 2 , panel b ) . rodent or insectivore burrows , which are known to be favorable natural habitats for o. sonrai , were also examined at each sampling site to determine the presence or absence of the tick . collected ticks were tested for asfv infection by nested pcr amplification of the vp72 gene , a method considered most sensitive for detection of viral dna in ticks ( 14 ) . b ) unfavorable warthog burrows negative for o. sonrai dug under a termite mound . the portable gasoline - powered vacuum cleaner used for tick collection o. sonrai was found in 11 of the 25 examined pigpens in villages in the 4 most northern sampling sites ( table ) . specimens were always found in rodent and insectivore burrows or in deep hollows , in openings inside pig buildings , or near sleeping or foraging areas around pig buildings , as described for the closely related iberian soft tick o. erraticus during investigations of asf ( 5 ) ( figure 2 , panel a ) . o. sonrai was not found in litter or buildings , except at 1 farm in fatick , where nearby burrows were highly infested . the village of karang showed negative results , even in suitable microhabitats , a finding that confirmed the southern distribution limit of o. sonrai proposed by morel ( 8) . in wild areas , o. sonrai was not found in 10 warthog burrows examined ( figure 2 , panel b ) , although its presence was confirmed in contiguous rodent or insectivore burrows . of 36 ticks tested for asfv infection , 4 from the 4 most northern sampling sites were positive for asfv ( table ) . the farms where asfv was detected in ticks had reported recent outbreaks in the summers of 2004 and 2005 , except for the farm in fatick . this farm , which belonged to a fattener / collector , had a high turnvover rate of pigs that may have caused difficulties in monitoring their health . sequencing and blast analysis ( www.ncbi.nlm.nih.gov ) of pcr products confirmed a 100% relationship with asfv . three samples showed doubtful results when retested by pcr , which indicated low virus titers . * asf , african swine fever ; + , positive ; , negative ; s , summer ; sp , spring . two other ticks from wild areas in fatalah and sipo forests were tested for afsv and found negative . to our knowledge , this study demonstrated for the first time that o. sonrai is naturally infected with asfv . although these preliminary results suggest a role for o. sonrai in persistence of asfv within a sylvatic cycle , only experimental infections will enable formal testing of o. sonrai as a reservoir and competent vector for afsv . additional tick sampling and virus detection analyses are also being conducted to estimate its natural prevalence of infection . if one considers that the ability of pathogens to infect a wide range of hosts is a risk factor for disease reemergence ( 15 ) , our findings are useful for the design of control measures for asf in senegal , which currently focus only on pig slaughtering and environment disinfection . although contact between soft ticks and domestic pigs has been confirmed in villages in this study , this contact is considered limited . o. sonrai colonizes mainly rodent or insectivore burrows with high humidity and a cool temperature favorable for its development and survival . it is rare that such favorable microhabitats are near pigpens and enable ticks to feed on pigs instead of small mammals inhabiting burrows . in addition , heterogeneous and relatively low infestation rates for such microhabitats have been shown in a previous study ( 11 ) . conversely , in wild areas , contact between ticks and warthogs was unlikely , which is contrary to the situation in eastern and southern africa . in senegal , warthogs inhabit mainly dry forests and dig superficial burrows under termite mounds , which are not optimal conditions for o. sonrai . to more clearly quantify contacts between ticks and domestic pigs or warthogs and assess their effect on asf transmission , analyses of mammalian cytochrome b in tick blood meals and detection of antibodies to tick saliva in serum samples of pigs and warthogs are being conducted .

african swine fever is a highly contagious disease of pigs in africa . although its persistence in senegal may be caused by asymptomatic carriers involved in the domestic transmission cycle , we demonstrated that the soft tick ornithodoros sonrai can be naturally infected with the causative agent .

laparoscopic colectomy has been accepted for benign and malignant diseases of the colon for its quicker recovery time as well as from a cosmetic viewpoint [ 1 , 2 ] . in addition , long - term oncological studies have indicated that laparoscopic colectomy was equivalent to open surgery in survival rates for colon cancer [ 1 , 3 ] . we herein report a case of an internal hernia projecting through a mesenteric defect following laparoscopy - assisted right hemicolectomy . a 74-year - old woman was hospitalized for the surgical treatment of double colon cancer . her medical history showed that she had been suffering from permanent damage from a cerebral hemorrhage for several years . a colonoscopic examination demonstrated the presence of an ascending colon tumor and a transverse colon tumor . biopsies performed before surgery revealed that both lesions were well - differentiated adenocarcinomas . a laparoscopic - assisted right hemicolectomy was performed . we divided the sub - middle colic artery and the ileocecal artery that fed the tumors with surgical trunk dissection ( d3 lymph node dissection ) . the marginal vessels were ligated , and functional end - to - end anastomosis was performed extracorporeally using linear staples . the double cancers were diagnosed pathologically as pt4a , pn1b , m0 , pstage iiib in the ascending colon and pt4a , pn2a , m0 , pstage iiic in the transverse colon ( tnm classification seventh edition ) . a plain abdominal x - ray revealed air - fluid levels in upper abdomen . a computed tomography ( ct ) scan revealed a closed loop of small bowel in the upper side of the transverse colon . we performed a laparotomy finding the small bowel , which had passed into the bursa omentalis through the mesenteric defect ( 78 cm in diameter ) . we removed the necrotic small bowel 65 cm in length and closed the mesenteric defect by suturing ( fig . the patient s postoperative course was uneventful . at the 2-year follow - up examination , figure 1:preoperative ct scan revealed an internal hernia ( arrow).figure 2:a picture of the resected strangulated small intestine . with the publication of several reports demonstrating that the outcomes of laparoscopic surgery for colon cancer were equivalent to those in open surgery , this approach has become widespread in recent years [ 1 , 3 ] . closure of the mesenteric defect caused by anastomosis after colectomy is not performed routinely in laparoscopic surgery . laparoscopic closure of the mesenteric defect requires a longer operation time , and incomplete closure may produce internal hernia of the small bowel . small bowel obstructions related to mesenteric defect are rare in laparoscopic colectomy . in the case reported here , the patient developed a strangulated ileus . a narrow defect caused by mesenteric adhesion may produce the strangulation of the small bowel that passed through the mesenteric defect . the mesenterium of the colon was conglutinated to the pancreas and surgical trunk that were dissected in the first operation , resulting in the formation of a small defect of the mesenterium . papers reported various types of internal hernias after laparoscopic colectomy [ 59 ] . in this case , the right hemicolectomy and the lymph nodes dissection in a wide area were necessary because of the double and advanced cancers ( pt4a , pn1b , m0 , pstage iiib in ascending colon and pt4a , pn2a , m0 , pstage iiic in transverse colon ) . this might have allowed the tissues of the mesenterium to conglutinate more easily . internal hernia after laparoscopic colectomy is rare . an internal hernia projecting through a mesenteric defect following laparoscopy - assisted right hemicolectomy developed a severe strangulated bowel obstruction .

abstractmesenteric defects are often not closed in laparoscopic colectomy . we herein report a case of an internal hernia projecting through a mesenteric defect following laparoscopy - assisted right hemicolectomy . a 74-year - old woman was hospitalized for the surgical treatment of double colon cancer . preoperative colonoscopy demonstrated the presence of ascending colon and transverse colon cancers . a laparoscopic - assisted right hemicolectomy was performed . the mesenteric defect resulting from the colectomy was not closed . three months after the surgery , the patient developed a bowel obstruction . under a diagnosis of strangulated bowel obstruction , we performed a laparotomy , and found a necrotic small bowel , which had passed into the bursa omentalis through the mesenteric defect . we removed the necrotic small bowel and closed the mesenteric defect by suturing . the patient s postoperative course was uneventful . an internal hernia projecting through a mesenteric defect following laparoscopy - assisted right hemicolectomy developed a severe strangulated bowel obstruction .

the hirwa group consisted of 12 animals : 1 adult male , 6 adult females , 3 juveniles , and 2 infants . moderate to severe respiratory disease ( > 2 characteristic signs ) developed in 11 of 12 animals . five ( 3 juvenile males and 2 adult females ) received antimicrobial drug therapy ( ceftriaxone , 50 mg / kg for adults , 100 mg / kg for infants ) , 4 by remote delivery and 1 while chemically immobilized . two untreated animals ( 1 adult female and 1 male infant born to a symptomatic mother ) died . on june 30 , the adult female was first observed coughing and lethargic but still feeding . on july 3 , she left her night nest in the morning but did not join her group ; she exhibited severe clinical signs and was found dead on july 4 at 1:00 pm . clinical signs of respiratory illness had not been observed , although its mother showed severe clinical signs for 23 days before and after delivery ; before delivery , she had received antimicrobial drugs by remote delivery ( neither she nor her infant were handled by humans ) . gross postmortem examinations revealed bronchopneumonia in the adult and unilateral pulmonary congestion and an empty stomach in the infant . formalin - fixed ( 10% ) postmortem tissue samples from the adult and infant were prepared in 6 m sections for histologic studies , stained with hematoxylin and eosin according to standard methods , and examined by light microscopy . histologically , the respiratory tract of the adult was characterized by moderate mononuclear tracheitis , laryngitis , and air sacculitis ; severe pulmonary alveolar histiocytosis ; multifocal severe suppurative pneumonia ; and multifocal pulmonary thrombosis and hemorrhage . one section of lung from the infant showed pulmonary atelectasis , congestion , mild alveolar hemorrhage , and histiocytosis . the infant also had moderate neutrophil and macrophage infiltration of the umbilicus at the body wall ; neutrophilic inflammation in the media and adventitia of 1 umbilical artery at the level of the bladder ; and mild , unilateral , focal , segmental , neutrophilic glomerulitis and tubulointerstitial nephritis . multiplex pcr analysis for respiratory pathogens indicated sequences of human metapneumovirus ( hmpv ) in serum ; lung tissue ; and throat , nose , anus , and vagina swabs from the adult gorilla , and in lung tissue from the infant ( table ) . streptococcus pneumoniae was detected in lung tissue and in throat and nose swabs of the adult gorilla but not in the infant . the sample with the highest viral load , a throat swab from the adult female ( 6.2 10 genome copies/l ) , was pyrosequenced , yielding 607,484 reads comprising 3.8 kb of sequence ( 27.5% of the genome ) . simple pairwise analysis indicated that the strain belonged to lineage b2 of hmpv ( 4 ) . bayesian analysis revealed close relationship of the gorilla virus to human isolates from south africa ( figure ) . bayesian analysis of the g gene of human metapneumovirus ( hmpv ) isolated from an adult female mountain gorilla that died during an outbreak of respiratory disease , rwanda . bayesian phylogenetic analyses of sequence differences of the hpmv glycoprotein gene were conducted by using beast , beauti , and tracer analysis software packages ( http://beast.bio.ed.ac.uk/main_page ) . the black dot indicates the strain newly isolated from the gorilla ; trees are rooted at the midpoint ; scale is in years . experimental infections of cynomologus macaques with hmpv have suggested that pure infection with this virus causes minimal to mild lesions in conducting airways and increased macrophages in alveoli ( 5 ) . however , paramyxoviruses , including hmpv , can predispose animals to bacterial pneumonia ( 68 ) , as appeared to be the case in the adult female mountain gorilla reported here . that hmpv can be fatal for gorillas is supported by a report of a respiratory outbreak in wild , human - habituated chimpanzees in which several chimpanzees died ( 2,9 ) . we report conclusive evidence for association of a human virus with death in mountain gorillas ( 2,3 ) . viral rna in multiple tissue samples from the adult female indicates that she was infected by an hmpv strain at the time of her death . the pulmonary lesions indicated a bacterial bronchopneumonia as the proximate cause of death , compatible with an etiologic agent such as s. pneumoniae and k. pneumoniae , the 2 organisms detected by pcr . although the cause of death of the infant was likely inanition and acute dissemination of an umbilical infection to a kidney , detection of hmpv as the sole pathogen in the infant tissues supports the presence of this agent in the gorilla group during the respiratory disease outbreak . the source of the virus is unknown ; the strain was most recently described in south africa . the 2 hmpv - positive animals were not handled by veterinarians or park personnel during the course of their illness . although hmpv transmission as a result of human intervention to treat sick animals in the group is possible , it does not explain hmpv in the adult female , which died early in the outbreak before any clinical interventions were conducted . although human proximity to mountain gorillas is essential for their conservation , also crucial is minimizing the risk for human - to great ape transmission of respiratory pathogens . pathogens included in pcr of samples from mountain gorillas during outbreak of respiratory disease , hirwa , rwanda , june 28-august 6 , 2009 .

the genetic relatedness of mountain gorillas and humans has led to concerns about interspecies transmission of infectious agents . human - to - gorilla transmission may explain human metapneumovirus in 2 wild mountain gorillas that died during a respiratory disease outbreak in rwanda in 2009 . surveillance is needed to ensure survival of these critically endangered animals .

introduction : symptomatic cervical disc disease ( scdd ) causing radiculopathy is a common diagnosis traditionally treated surgically with anterior cervical discectomy and fusion ( acdf ) . objective : the purpose of this trial is to compare the safety and efficacy of total disc arthroplasty ( tda ) using the prodisc - c ( synthes spine company , lp , west chester , pa , usa ) implant to acdf in patients with single - level scdd between c3 and c7 . methods : this study is a report of the single - site results from a trial of 13 sites in a multicenter trial . patients for this study were enrolled and treated in accordance with the approved us food and drug administration protocol , using a non - inferiority design . patients were randomized to either tda using the prodisc - c device or acdf in a one - to - one method . all enrollees were evaluated preoperatively and postoperatively at 6 weeks , 3 , 6 , 12 , 18 , 24 , 36 , 48 , 60 , 72 , and 84 months . data was collected using the visual analog scale ( vas ) for neck and arm pain / intensity , and satisfaction . neck disability index ( ndi ) and short - form 36 ( sf-36 ) questionnaires were also completed . results : twenty - two patients were randomized to each arm of the study at this site . all operations occurred between c4 and c7 with most being at c56 and c67 and only two at c45 . operative time was similar ( prodisc - c 98 16 min ; acdf 95 23 min ; p = .59 ) . the ndi improved in the prodisc - c group more than in the acdf group ( prodisc - c preoperative 54.2 12.8 to 7 years 14.1 18.1 vs acdf preoperative 53.6 14.1 to 7 years 26.9 23.8 ) ( p = .11 ) . total range of motion was maintained in the prodisc - c while it diminished as expected in the acdf group . the vas scores and sf-36 scores all showed at minimum non - inferiority of the prodisc - c group and trended toward superiority of the tda group in some metrics including neck pain . seven additional operations were carried out among the entire study group , all in the acdf group with three reoperations at the same level and four operations at an adjacent level . four of 44 patients died within 7 years of the index operation , all from unrelated causes.1 conclusions : the prodisc - c implant appears to be safe and effective for the treatment of scdd . patients with the implant generally retained more motion at the involved segment than those with an acdf and had a lower reoperation rate . stock ; amedica corp / us spine , pioneer surgical technology . direct research / institutional support ; aospine north america . bod / owner / officer / committee h / c org ; csrs , lsrs . editorial / gov board med / ortho pubs ; jaaos , jsdt , journal of spinal cord medicine , spine .

selected abstracts delivered at the 10th annual aospine north america fellows forumconsistent with ebsj 's commitment to fostering quality research , we are pleased to feature some of the most highly rated abstracts from the 10th annual aospine north america fellows forum in banff , canada . enhancing the quality of evidence in spine care means acknowledging and supporting the efforts of young researchers within our aospine north america network . we look forward to seeing more from these promising researchers in the future .

cases of linear peritoneal calcification have been reported in literature , though they occur rarely . in encapsulating peritoneal sclerosis ( eps ) a peritoneal membrane damage develops an inflammatory cascade that results in sclerosis and eventually calcification . eps has been can be either primary or secondary , being long exposure to peritoneal dialysis solutions , the most common cause of the secondary form . the incidence of eps has only been studied in patients on peritoneal dialysis and is estimated to be 0.544.4% , although this number can rise considerably with the time on pd . the symptoms manifest disturbances in intestinal function such as abdominal pain , nausea , vomiting and ultimately anorexia and weight loss . among the imaging techniques available , ct is the modality of choice in the diagnosis of eps , demonstrating peritoneal thickening , calcification , bowel wall thickening , bowel tethering , dilation and fluid loculation . in august 2014 a 39-year - old female presented to the emergency department of our hospital . she referred a past history of one cesarean followed by a laparoscopic tubal ligation . in october 2013 peritoneal lavage by the peritoneal catheter was performed , but abdominal wall swelling was noticed and a leak within the laparoscopic port was suspected . a ct peritoneography ( fig . iodinated contrast was injected through the peritoneal catheter and the patient was encouraged to walk , allowing a good diffusion of contrast through the peritoneal cavity . shortly after contrast injection the patient developed intense abdominal pain and hypotension that were attributed to contrast adverse reaction the ct was obtanied 30 min after contrast injection , revealing a good diffusion of contrast , with no images of leak or abdominal collections . ten days later the patient was seen for abdominal pain and elevated inflammatory markers ( c - reactive protein = 40 ) and the tenckhoff catheter was removed for a suspected infection . the patient chose then to start hemodialysis . on the day she visited our emergency department , she reported complaints of lumbar pain and dysuria for the previous few days . laboratory data were normal , except for : hemoglobin = 8.9 ( normal 1216 g / dl ) , leukocytosis = 14,700 ( 400010,000 ) , leukocyturia > 200/field ( < 5/field ) ; c - reactive protein = 23.5 ( < 1.0 ) , urea = 22.4 ( 2.46.4 mmol / l ) , creatinine = 316 ( 4692 mol / l ) , pth = 324 ( 1687 pg / ml ) , calcium = 2.40 ( 2.102.55 mmol / l ) and phosphorus 1.47 ( 0.411.45 3 ) revealed extensive visceral ( arrows in a ) and parietal peritoneal calcification ( arrowheads in a ) with areas of focal thickening in the pelvic peritoneum ( arrowheads in c ) . she improved clinically , inflammatory markers decreased and she was then discharged from the hospital with no symptoms . cases of linear peritoneal calcification have been reported in patients on long - standing peritoneal dialysis and have previously been classified as calcifying peritonitis , . this term is not currently used but is now included in the spectrum of eps . long exposure to peritoneal dialysis solutions is the most cited caused , although it can also develop after renal - transplant , with hyperparathyroidism , , with the use of certain beta - blockers or after recurrent episodes of peritonitis , , among others . the two - hit model has been used to explain the pathophysiology of eps , hypothesizing that a predisposing factor ( injury ) and an initiating factor ( such as an inflammatory stimuli ) are required for eps to develop . from this model we can assume that , in a susceptible patient , it can develop after any peritoneal inflammatory stimuli . eisenberg and colleagues reported the development of peritoneal inflammation secondary to iodinated contrast agents in guinea - pigs . to the best of our knowledge no cases of eps secondary to intraperitoneal contrast have been reported . clinically , patients with eps can be asymptomatic or may present with symptoms caused by modifications of gastrointestinal transit . nakamoto divided eps into four clinical and pathological progressive stages , ranging from lack of symptoms in stage 1 to complete bowel obstruction and anorexia characterizing the stage 4 . imaging findings will translate the pathological progression of the disease , with peritoneal calcification predominating in the early stages and the most advanced cases presenting with findings of bowel obstruction such as air - fluid levels , dilated loops or even bowel clustering . on the different imaging techniques , abdominal radiography shows a low accuracy in this diagnosis , being either normal or revealing peritoneal calcification , air - fluid levels or loop dilations . contrast studies can reveal proximal small bowel dilatation , delayed transit time and , in advanced cases , cauliflower sign abdominal ultrasound can show peritoneal thickening , loculated peritoneal fluid , peritoneal calcification , adherences , dilated or diminished peristaltic intestinal loops . tarzi created a 22-point score , considering peritoneal thickening , peritoneal calcification , bowel wall thickening , bowel tethering ( 04 points each ) , loculation and bowel dilation ( 03 points ) . the mean score of eps patients was 9 ( 216 ) , comparing to an average score of 1 ( 03 ) in controls patients on peritoneal dialysis . positron emission tomography can show an increased peritoneal uptake but is not able to distinguish from acute peritonitis . in our case , the diagnosis was made by ct , although it could already be suspected by the radiography . our patient ct score is 5 , inferior to the average obtained by tarzi , but matching an early stage of disease that we expected due to the lack of symptoms ( stage 1 ) . the focal peritoneal thickening observed in the most dependent portions of the abdomen might be explained by the longer time exposure of these areas to the peritoneal contrast . laparotomy is indicated in later stages of the disease and shows peritoneal thickening , adhesions , tethering , fibrosis or bowel retraction . no specific treatment has been developed for eps , being proposed the use of total parenteral nutrition , , corticoids , , tamoxifen , and surgery with enterolysis of intestinal adhesions for advanced cases , . when presenting with symptoms eps has a high mortality , usually as a result of bowel obstruction , malnutrition and sepsis . we demonstrated an unusual case of peritoneal calcification , in which there was no history of long exposure to peritoneal dialysis solutions . we believe that intraperitoneal iodinated contrast exposure combined with alport syndrome have caused the abnormalities seen . long standing hyperparathyroidism might also have played a role explaining the increased susceptibility of our patient for the development of eps . the authors wish to confirm that there are no known conflicts of interest associated with this publication and there has been no financial support for this work that could have influenced its outcome .

the peritoneum is the largest serous membrane of the body and can be exposed to several injuries that may cause abnormal findings on imaging exams . linear peritoneal calcification is remarkably rare , usually secondary to long duration peritoneal dialysis.we report an uncommon case of extensive peritoneal calcification in a 39-year - old female without long exposure to peritoneal dialysis solutions , in which peritoneal calcification could be linked to alport syndrome and previous adverse reaction to intraperitoneal iodinated contrast.radiologist should be aware of this and related imaging findings , know when to search for them as well as understand their clinical value .

thiophenol ( 1 mmol ) and zni2 ( 20 mol % ) were added to a 15 ml tube equipped with a stirring bar . then , the tube was flashed with argon and vacuum three times , and 1 ml of toluene and 2 equiv of tbhp ( 70 % in water ) were injected through a syringe . after that , the tube was closed and heated to 80 c for 28 h. when the reaction was complete , the reaction mixture was cooled to room temperature . the reaction was quenched with distilled water and the solution was extracted with ethyl acetate . the crude product was purified by using column chromatography ( ethyl acetate / pentane=1:15 ) . 1,2diphenyldisulfane ( 1 mmol ) and ki ( 0.4 equiv ) were added to a 25 ml schlenk tube equipped with a stirring bar . then , the tube was flashed with argon and vacuum three times , and 1 ml of toluene and 5 equiv of tbhp ( 70 % in water ) were injected through a syringe . after that , the tube was closed and heated to 80 c for 24 h. when the reaction was complete , the reaction mixture was cooled to room temperature . the reaction was quenched with distilled water and the solution was extracted with ethyl acetate . the crude product was purified by using column chromatography ( ethyl acetate / pentane=1:15 ) . a 15 ml tube equipped with a stirring bar , benzenethiol ( 1 mmol ) , etoac or toluene ( 1 ml ) , and tbhp ( 1 equiv ; 70 % in water ) were added through the syringe . after that , the tube was closed and heated to 60 c for 16 h. when the reaction was complete , the reaction mixture was cooled to room temperature . the reaction was quenched with distilled water and the solution was extracted with ethyl acetate . thiophenol ( 1 mmol ) and zni2 ( 20 mol % ) were added to a 15 ml tube equipped with a stirring bar . then , the tube was flashed with argon and vacuum three times , and 1 ml of toluene and 2 equiv of tbhp ( 70 % in water ) were injected through a syringe . after that , the tube was closed and heated to 80 c for 28 h. when the reaction was complete , the reaction mixture was cooled to room temperature . the reaction was quenched with distilled water and the solution was extracted with ethyl acetate . the crude product was purified by using column chromatography ( ethyl acetate / pentane=1:15 ) . 1,2diphenyldisulfane ( 1 mmol ) and ki ( 0.4 equiv ) were added to a 25 ml schlenk tube equipped with a stirring bar . then , the tube was flashed with argon and vacuum three times , and 1 ml of toluene and 5 equiv of tbhp ( 70 % in water ) were injected through a syringe . after that , the tube was closed and heated to 80 c for 24 h. when the reaction was complete , the reaction mixture was cooled to room temperature . the reaction was quenched with distilled water and the solution was extracted with ethyl acetate . the crude product was purified by using column chromatography ( ethyl acetate / pentane=1:15 ) . a 15 ml tube equipped with a stirring bar , benzenethiol ( 1 mmol ) , etoac or toluene ( 1 ml ) , and tbhp ( 1 equiv ; 70 % in water ) were added through the syringe . after that , the tube was closed and heated to 60 c for 16 h. when the reaction was complete , the reaction mixture was cooled to room temperature . the reaction was quenched with distilled water and the solution was extracted with ethyl acetate . as a service to our authors and readers , this journal provides supporting information supplied by the authors . such materials are peer reviewed and may be reorganized for online delivery , but are not copyedited or typeset . technical support issues arising from supporting information ( other than missing files ) should be addressed to the authors .

abstractin this communication , we developed a new procedure for the synthesis of hydroxysulfides from thiophenols or diaryl disulfides with tbhp as the oxidant . in the presence of zinc iodide or potassium iodide , with tbhp as the oxidant and prereactant , thiophenols and diaryl disulfides reacted with the methyl group of tbuoh smoothly and selectivity to give the corresponding 2methyl1(arylthio)propan2ols as the terminal products in moderate to good yields .

glioblastoma is the most frequent tumor among all primary intracranial tumors with a frequency of approximately 15 - 50% . it can be seen in all age groups ; however , patients are generally older than 50 years . when compared with childhood tumors , localization in the posterior fossa in adults is rare . cerebellar glioblastoma multiforme ( gbm ) is a rare tumor that accounts for only 1% of all cases of gbm and its giant cell variant is even much rarely encountered in adults . a 51-year - old woman complained of headache with vomiting and gait ataxia of 1 month duration . magnetic resonance imaging scan showed a left cerebellar intra - axial mass lesion near cerebellopontine angle with t1-weighted hypointensity and t2-weighted hyperintensity with mild perifocal edema and mass effect [ figure 1 ] . on contrast study , the lesion was enhancing homogeneously with area of unenhanced cystic component in it . magnetic resonance ( mr ) spectroscopy showed choline peak with reduced n - acetyl aspartate ( naa ) and choline : naa ratio of 5.74 . metastatic tumor , malignant lymphoma , and gbm were considered for radiological differential diagnosis . workups for common metastatic lesions from breast , lung , colon , and secondary central nervous system lymphoma magnetic resonance imaging scan showing a heterogeneous intra axial mass in left cerebellar hemisphere with t1 hypointensity , ( a ) t2 hyperintensity , ( b ) with contrast enhancement , ( c ) and coronal section showing same tumoral aspect ( d ) mr spectroscopy showing choline peak with decrease in creatine and n - acetyl aspartate peak with at places lactate peak compatible with gbm pattern through a left retromastoid suboccipital craniectomy a greyish - pink , soft , noncapsulated , vascular tumor in the left cerebellar hemisphere was excised in piecemeal near totally [ figure 3 ] . diagnosis of giant cell variant of gbm was made on histopathological section [ figure 4a c ] . follow up after 2 months , the patient has been doing well without any cranial nerve deficit . post - op ct scan plain , ( a ) and contrast , ( b ) showing near total resection of the tumor photomicrographs of giant - cell n - acetyl aspartate showing numerous bizarre multinucleated giant cells , ( a ) h and e 200 , ( b ) h and e 400 and , ( c ) immunohistochemistry showing glial fibrillary acidic protein positivity ) glioblastoma is the most frequent tumor among all primary intracranial tumors with a frequency of about 15%-50% . it can be seen in all age groups ; however , patients are generally older than 50 years . when compared with childhood tumors , localization in the posterior fossa in adults is rare . the giant cell gbm is a histological variant of glioblastoma presenting a prevalence of bizarre , multinucleated ( more than 20 nuclei ) giant ( up to 400 m diameter ) cells . monstrocellular brain tumor by schmincke in early twentieth century due to its stromal reticulin network but it was first called giant cell glioma by meyer in 1913 . the astrocytic nature of the tumor was firmly established through the consistent glial fibrillary acidic protein ( gfap ) expression , so it belongs to world health organization grade iv tumor of astrocytic origin . prominent nucleoli , typical and atypical mitotic figures , irregularly shaped chromatin fragments , and necrosis , namely , pseudopalisading or large ischemic forms are characteristic . an in vitro study showed that some giant multinucleated cells might have originated from conversion of number of small tumor cells of astrocytic origin . numerous mitoses , atypical mitosis , and necrosis with pseudopalisading differentiate it from pleomorphic xanthoastrocytoma . cerebellar gbm is a rare tumor that accounts for only 1% of all cases of gbm and its giant cell variant is even much rarely encountered in adults . giant cell gbm predominates in cerebral hemispheres mainly temporal and parietal lobes but cerebellum , lateral ventricles , and spinal cord are rare sites . the mean age at clinical presentation is fourth decades of life and affects males more frequently than females ( m : f ratio is 1:6 ) . glioblastomas appear as heterogenous masses on mr imaging due to the formation of necrosis and/or cysts . on t1-weighted images , the cystic part looks hypointense and solid part looks isointense ; however , on t2-weighted images they are heterogeneously hyperintense . mr spectroscopy is a useful method in differentiating these tumors from other diseases . in gbm decrease in naa is related to neuron loss due to tumor , decrease in creatine due to metabolic changes , and increase in choline is due to increase in membrane synthesis and cells . diffusion - weighted imaging ( dwi ) is also used in the differential diagnosis of rim - enhancing cerebellar mass lesions . cystic or solid components of brain tumors display high apparent diffusion coefficient ( adc ) values and low signals in dwi with a high b value . cystic or necrotic components of these tumors exhibit low adc values on adc maps , and low signal on high b - value dwis . to the contrary , while cavity content of abscesses is seen as high signal intensities in diffusion sequence images , adc values are low . in a recent study , it was found that the intensely enhancing solid components of hemangioblastomas demonstrated low signals in dwi together with high adc values . considering the different treatment modalities available , it is very important to make a proper differential diagnosis between infratemporal gbm and metastasis , hemangioblastoma , and also cerebellar infarct with contrast enhancement . considering malignant glial tumors , it would be always reasonable to attempt a gross - total tumor resection ; however , the infiltrative nature of the gbm makes it difficult to achieve it many times . the use of radiation therapy after radical surgical resection is a well - established modality of treatment . chemotherapy is not well established but is the only reasonable adjuvant treatment for very young children . the biological behavior of cerebellar and supratentorial gbm is similar . on the other hand , it is possible that cerebellar gbm found in young patients has a better prognosis as in anaplastic astrocytomas , which have a longer survival time than supratentorial gbm . although gbm is the most frequent primary brain tumor in adults , its localization in the cerebellum is extremely rare . however , gbm must be considered in the differential diagnosis of aggressive mass lesions of the cerebellar hemisphere . therefore , mr spectroscopy , dwi , and brain perfusion imaging technique may be used in prospective evaluation .

cerebellar glioblastoma multiforme ( gbm ) is a rare tumor that accounts for only 1% of all cases of gbm and its giant cell variant is even much rarely encountered in adults . a case of cerebellar giant cell gbm managed at our institution reporting its clinical presentation , radiological and histological findings , and treatment instituted is described . in conjunction , a literature review , including particular issues , clinical data , advances in imaging studies , pathological characteristics , treatment options , and the behavior of such malignant tumor is presented . it is very important for the neurosurgeon to make the differential diagnosis between the cerebellar gbm , and other diseases such as metastasis , anaplastic astrocytomas , and cerebellar infarct because their treatment modalities , prognosis , and outcome are different .

halo phenomenon of melanocytic nevus is observed as a circular reaction of hypopigmentation , although it is an unusual circumstance around tumors . it has been reported as annular leukoderma in individual cases of melanoma , basal cell carcinoma ( bcc ) , neurofibroma , and angioma . the cause of this reaction remains unknown , although proposed to be either a biological event or a result of shunting of blood away from the central lesion . an otherwise healthy , 29-year - old caucasian woman presented with a pigmented lesion on the cheek since three years . she noted whitening of the skin around the lesion almost after a year following its appearance and spontaneous enlargement of the lesion during pregnancy , recently . dermatologic examination revealed a 12 10 mm reddish brown nodular lesion surrounded by a 1 cm wide halo of depigmentation on the left infraorbital region [ figure 1 ] . peripherally pigmented pinkish nodule on the face dermatoscopic analysis of the partially pigmented central nodule was suggestive of a nonmelanocytic lesion with the absence of pigment network , accompanied by a subtle peripheral halo of depigmentation . peripherally localized multiple blue - gray dots and globules , a blue - gray ovoid nest , spoke - wheel areas , and arborizing telangiectasia were prominently observed [ figure 2 ] . dermatoscopic view of the lesion in three quadrants according to figure 1 histopathologic characteristics of the totally excised lesion confirmed the diagnosis of infiltrative - type bcc . melanin was observed in the squamous epithelium , and lymphocytic infiltration was present in the dermis of the neighboring tumoral region . the area of peripheral hypopigmentation showed a decrease in the number of melanophages [ figure 3 ] . annular leukoderma is described around several benign and malignant lesions and could be mistaken as halo nevus . bcc is believed to develop hypopigmentation infrequently , and only a few cases have been reported . the previously reported three patients were older than 50 years , and bcc was located on the face in only one patient . the features of interest in our patient were young age and a usual medical history . therefore , the initial clinical impression was halo nevus and possibly melanoma , although findings of dermatoscopy made us consider bcc in differential diagnosis . recently , sclerodermiform bcc appearing on a vitiliginous patch was reported in a young female . differing from our case , the depigmented patch preceded sclerodermoid bcc in this young individual . dermatoscopy is a fast , simple , and advantageous procedure for differentiating between halo nevus and bcc to avoid unnecessary surgery for the patient . although very rare , young patients with bcc as was the case , might be mistakenly followed up as halo nevus for years , if initial examination including dermatoscopy was not done . in conclusion , the persistence of perilesional halo around an enlarging pigmented lesion should be carefully examined with accompanying dermatoscopic findings even in young patients , for early diagnosis of nonmelanocytic tumoral lesions . the features of interest in our patient were young age and lack of a history of multiple bccs , as was reported in a few cases previously . therefore , initial examination including dermatoscopy is mandatory , even in young patients with perilesional halo around an enlarging pigmented lesion .

halo phenomenon of nevus may be observed as a circular reaction , although it is unusual around tumors . a 29-year - old woman presented with a pigmented lesion on the cheek since three years . she noted whitening of the skin around the lesion almost after a year following its appearance . dermatologic examination revealed a pigmented nodular lesion with a hypopigmented halo on the left infraorbital region . the clinical impression was halo nevus , whereas basal cell carcinoma ( bcc ) was considered in dermatoscopic differential diagnosis . the diagnosis was infiltrative - type bcc histopathologically . the persistence of a perilesional halo around an enlarging pigmented lesion should be carefully examined with accompanying dermatoscopic findings even in young patients for early diagnosis of tumoral lesions .

variations in time , temperature , concentration , ph , and relative humidity may affect the sporicidal activity of various agents . accordingly , and especially for real - world situations , attention must be paid simultaneously to more than one controllable or uncontrollable factor . in tables 1 and 2 and in the discussion , we address some of the key ancillary factors . spores in liquid suspension exposed to flowing steam at 100c . rh , relative humidity ; conversions : 1 ppm = 1 mg / l ; mol / l = gram molecular weight / l ; 1 rad = 100 ergs / g ; and 1 watt = 10 ergs / s . boiling water for > 10 minutes , for example , can reduce b. anthracis spore counts by at least 10 ( table 1 ) . variations in time and temperature conditions required to reduce spore counts listed in table 1 can be attributed to differences in experimental conditions , strains of b. anthracis tested , or inoculum size . the u.s . environmental protection agency indicates that use of sodium hypochlorite as a sporicide is applicable under an emergency exemption ( section 18 : crisis exemption ; federal insecticide , fungicide , and rodenticide act ) ; as such , sodium hypochlorite may be used under the conditions specified ( 32 ) . given these conditions , the sporicidal effectiveness of hypochlorite solutions depends on the concentration of free available chlorine and ph . common household bleach ( sodium hypochlorite ) has a ph of 12 to prolong its shelf life . to achieve effective sporicidal activity , bleach must be diluted with water to increase the free available chlorine and acetic acid to change the ph of the solution to 7 ( 11 ) . ethylene oxide penetrates into porous material ( absorbed strongly by rubber and many plastics ) ; thus vapors are not readily eliminated by brief aeration . residual spores were not completely killed after a 30-minute exposure to chlorine dioxide at a relative humidity of 20% to 40% , whereas all spores were killed after a 15-minute exposure to chlorine dioxide with the addition of prehumidification at a relative humidity of 70% to 75% ( 21 ) . the amount of contamination , level of cleanliness of surfaces , and relative humidity will contribute to peracetic acid vapor s effectiveness as a sporicide ( 24 ) . the sporicidal property of ozone is affected by relative humidity : as relative humidity decreases , the time required for killing organisms increases ( 27 ) . decontamination of buildings from intentional release of b. anthracis is a new problem , and no accumulated scientific knowledge exists on the subject . two areas of prior scientific research may be relevant : food processing and laboratory decontamination . with modification based on further study , direct information on killing b. anthracis spores in foods by cooking is scarce , and the complexity of food matrices precludes easy extrapolation of the laboratory data into nonfood matrices . however , information on inactivating spores of bacterial species more resistant to environmental conditions than b. anthracis can provide guidance . the spores of clostridium botulinum are more resistant to heat inactivation than are b. anthracis spores ( 4 ) . the commercial retort process of canning achieves a 12-log reduction of c. botulinum spores , and by extension , should achieve a similar killing rate for b. anthracis spores . further research in this area is needed . historically , formaldehyde solution or gas has been used both as a disinfectant and chemical sterilant . formaldehyde was used to disinfect as early as the late 1880s and is still used to reprocess hemodialyzers for reuse on the same patient and to decontaminate biologic safety cabinets and laboratories ( 3537 ) . formaldehyde gas has been used for fumigation in the poultry industry and for disinfection of biologic safety cabinets and laboratories ( 38,39 ) . data from controlled experiments with b. globigii nctc 10073 spores have demonstrated the effect of humidity on formaldehyde concentration ( mg / m ) to obtain a > 8-log reduction in viable spores ( 15 ) . / l ) has also been used to treat a textile mill contaminated with b . gamma radiation was used in the 1960s and 1970s to disinfect b. anthracis contaminated imported bailed goat hair . a study by horne et al . suggested that a dose of 1.5 megarads from a 200,000-rad / hour cobalt source was sufficient to kill most resistant spores when mixed with goat hair ; after the intentional release of b. anthracis through the postal system in 2001 , pursuing a decontamination method for the undelivered mail was essential . gamma radiation was used to decontaminate all mail from contaminated facilities on the basis of these data . multiple technologies may be needed to decontaminate buildings and their contents . as in a laboratory , where some items are wiped , some items are autoclaved , and some spaces are treated with gas , more than one method may be required for decontamination . also , for certain decontamination tasks , e.g. , cleaning small heat - proof and water - proof objects , more than one option will be available . further , even within the context of one type of application ( e.g. , walls ; ducts for heating , ventilating , air conditioning , and refrigeration ; carpet ; and small objects ) , potentially conflicting priorities exist between bioefficacy , logistics , and safety . although transferring the methods used to decontaminate or sterilize laboratory or food industry settings to decontaminating buildings may be useful , this transfer of methods has not been scientifically tested . also , much of the data available is based on other bacillus species ; more testing with or correlation to b. anthracis contamination is suggested . second , choosing between technologies is a complex issue , and a formal decision process would be useful . various parties in the public and private sector have suggested numerous , sometimes disparate , methods for the inactivation of b. anthracis spores in contaminated environments . further research is needed regarding improved methods for remediation of environments contaminated with b. anthracis spores , and the literature summarized here provides a basis for that effort .

after the intentional release of bacillus anthracis through the u.s . postal service in the fall of 2001 , many environments were contaminated with b. anthracis spores , and frequent inquiries were made regarding the science of destroying these spores . we conducted a survey of the literature that had potential application to the inactivation of b. anthracis spores . this article provides a tabular summary of the results .

in adults , routine injections of botulinum toxin are the treatment of choice for the management of essential blepharospasm [ 1 , 2 ] . the clinical picture is characterized by recurrent involuntary spasms in the region of the orbicularis oculi muscle . typically , at the time of the first manifestation , the patients are in their 5th to 7th decade of life . until today , > 2,500 patients presenting with this clinical picture have been treated with botulinum toxin in the department of ophthalmology of the university of bonn , bonn , germany . among these patients , blepharospasm mostly occurs as reflex phenomenon in conditions of the anterior eye segment such as , for instance , corneal affections or trichiasis , in the sense of the defense triad consisting of blepharospasm , photophobia and epiphora . so far , congenital blepharospasm devoid of conditions of the anterior eye segment has not been described in the literature . the treatment of children affected by cerebral palsy with botulinum toxin is considered safe and effective , but is only approved for children 2 years of age . other authors described off - label - use in younger children , the youngest being 11 months old . a 4-week - old girl was referred to our department because of absence of eye opening . since her birth , more in the evening , slit - like eye opening occurred for a maximum of 1 h ( fig . the child reacted with eyes closed to direct flashlight in the dark , e.g. , by turning away or grimacing . immediate massive blepharospasm was present even when lid opening was attempted in her sleep or following administration of local anesthetic eyedrops . pregnancy and delivery had been normal . a neuropediatric examination including ultrasound study of the brain was unremarkable . at the age of 2 months , an examination under general anesthesia was performed which revealed regular organ findings devoid of ocular irritation . during anesthesia , marked regression of spasms was observed . hence , periorbital injection of botulinum toxin ( 6.25 iu xeomin in each eye at 6 injection sites each equivalent to half of the starting dose of adults with essential blepharospasm ) was performed to prevent deprivation amblyopia . for the first time , the child started eye contact and briefly followed with her eyes . four weeks later , the mother reported that both eyes continued to be opened and closed smoothly . the deep - seated eyes were opened slit - like with free pupils , pronounced inward squinting and reduced abduction capacity ( abduction only to midline ) . at the age of 7 months , alternating esotropia and only slightly impaired abduction with persistent lid opening was observed . meanwhile , the child had established a good eye contact ability and was able to follow objects . further ophthalmologic follow - up examinations conducted to the current age of 3 years revealed regular eye opening and complete lid closure without recurrence of blepharospasm ( fig . no side effects of botulinum toxin therapy have occurred . a signed patient consent - to - disclose the child presented in this case report is the youngest patient ever treated with botulinum toxin . typical indications for botulinum toxin in young children are spasticity of the upper or lower limbs due to infantile cerebral palsy . botulinum toxin reduces spasticity , avoids contractures and delays surgery , but is not able to improve the achievement of motor milestones . the safety profile of botulinum toxin was similar to older children except for two cases of acute respiratory events in children with severe cerebral palsy , mask anesthesia and high - dose botulinum neurotoxin therapy . in our case , a single periorbital injection of botulinum toxin induced lid opening already persisting for nearly 3 years . in typical essential blepharospasm in adults ( focal dystonia ) , botulinum toxin injections are only effective for about 3 months blepharospasm may occur in children with schwartz - jampel syndrome , a rare neuromuscular disorder with autosomal recessive inheritance characterized by myotonia , distinctive facial features including blepharospasm and a puckered chin , short stature as well as skeletal dysplasia . a case of congenital blepharospasm without other neurologic signs has never been described in the literature before . it may be due to some delayed midbrain maturation . in older children , blepharospasm devoid of ocular irritation or lid abnormalities ( reflex blepharospasm ) a direct influence of the periorbital botulinum toxin injection on the abduction deficit seems virtually impossible . diffusion from the region of the orbicularis oculi muscle into both extraocular muscles seems pathophysiologically impossible . hence , one may assume that the abduction deficit had already preexisted and could hardly be examined before the botulinum toxin injection due to blepharospasm . the botulinum toxin injection resulted in a normal visual development of the child ; otherwise , sensory deprivation would have occurred . botulinum toxin can therefore be helpful to treat special conditions even in very young children without relevant side effects of the neurotoxin . dr . wabbels received research grants from allergan , ipsen and merz for medical writing and statistics .

botulinum toxin injections are the treatment of choice for the management of essential blepharospasm in adults . no cases of congenital blepharospasm have been described in the literature so far , and no cases of botulinum toxin injection in an infant have been reported . a 4-week - old girl was referred to our department with absent eye opening and spasmodically closed eyes . pregnancy and delivery had been normal . a neuropediatric examination did not reveal useful findings . a periorbital injection of botulinum toxin was performed at the age of 2 months to prevent deprivation amblyopia . four days later , clearly visible bilateral eye opening and commencement of eye contact were observed . at the age of 3 years , her eyelids remain open and no side effects of botulinum toxin therapy have occurred .

the united states department of defense and department of veteran affairs are dedicated to providing superior rehabilitation care for veterans and service members who have suffered combat - associated traumatic limb loss . current literature reports that 70% of amputees with multiple limb loss are bothered by sweating and skin irritation inside of the prosthesis socket . we present a review of the literature examining the effectiveness of botulinum toxin a ( btx - a ) and btx - b for the treatment of hyperhidrosis related to prosthesis use . while both btx - a and btx - b are approved by the food and drug administration ( fda ) , indications for use are limited . hyperhidrosis continues to be a significant barrier to prosthesis comfort , and treatment with btx has the potential to address this concern . a review of the literature was conducted using the pubmed database , focusing on hyperhidrosis treatment after a traumatic limb amputation . we used the key search words : btx ; hyperhidrosis ; amputation ; trauma - related amputation ; prosthesis comfort . articles discussing hyperhidrosis treatment for axillary , palmar , plantar and amputations secondary to chronic medical conditions were excluded . botulinum toxin a is currently approved by the fda as botox ( allergan , inc . , irvine , california , usa ) supplied in 50 and 100 unit vials , and dysport ( ipsen , berkshire , united kingdom ) supplied in 300 and 500 unit vials , to treat cervical dystonia , severe primary axillary hyperhidrosis , strabismus , blepharospasm , and for temporary improvement of moderate to severe glabellar lines . the benefits of using btx - a for these conditions is well - established and it has few adverse effects at their respective therapeutic doses , which range from 20 to 360 units . the most common adverse effects reported are muscle weakness , fatigue , flu - like symptoms , dry mouth , dizziness , discomfort at the injection site and skin rash . large volume injections of btx - a > 600 units have been associated with systemic weakness related to injection dose and frequency , which should be a consideration in the treatment of the residual limb hyperhidrosis . current literature emphasizes the positive effects of btx - a on pain perception , itch and inflammation while simultaneously producing an anhidrotic effect . a study conducted by charrow et al . found that injections of btx - a successfully reduced residual limb hyperhidrosis and improved prosthesis fit and function when evaluated three weeks after treatment . in this study , 300 - 500 units of btx - a at a dilution of 100 units in 1 ml of 0.9% isotonic saline were injected intradermally with 2 - 3 units at 1 cm intervals in a circumferential pattern on the skin covered by the prosthesis socket . this approach however , did not have any effect on the residual limb pain ( rlp ) or phantom limb pain ( plp ) . however , jin et al . demonstrated in three cases that injection of btx - a at 200 , 300 , or 500 units using electromyographic guidance and targeting areas of the residual limb where strong fasciculations were present achieved marked improvement in rlp and plp lasting up to three months . three separate case studies utilized a starch - iodine test to identify hyperhidrotic areas on a residual limb , then injected btx - a at 100 u in 5 - 10 unit aliquots , or 300 units in 25 units aliquots . in all three reports , btx - a was diluted in preservative - free saline and produced anhidrotic effects lasting three months . because our facility is a government - funded hospital with extensive medical coverage for military personnel , poor prosthesis satisfaction due to hyperhidrosis is the only indication needed for treatment . while large volume injections can be costly , the literature has shown that improving prosthesis comfort is directly correlated with an improved quality of life . to inject btx - a at 1 - 2 cm intervals in a grid - like pattern over a surface area of 600 cm would require over 140 intradermal injections , typically achieved with a 30-gauge needle . the procedure lasts up to 30 min and is criticized as painful . a recent study by torrisi et al . investigated the use of pocketed microneedles ( pmn ) as an intradermal delivery system for btx - a . the width of the pmn used in this study is 340 m , which could serve as a less invasive administration method of btx - a in hyperhidrotic areas . botulinum toxin type b , supplied in 2500 , 5000 , and 10,000 units vials is approved by the fda as myobloc ( solstice neurosciences , inc . the use of btx - b for the treatment of palmar hyperhidrosis was deemed effective by baumann et al . and further studied by kern et al . in the treatment of the residual limb hyperhidrosis for lower limb amputees . btx - b administered in a low dose is believed to have a higher affinity for sympathetic nerve endings and better diffusion than btx - a . furthermore , btx - b and was found to be effective in reducing residual limb sweating in nine lower limb amputees . treated nine lower limb amputees with 1750 units of btx - b injected intracutaneously using a 27-gauge needle ( 20 injection sites 2 - 4 cm apart ) . in addition to subjective identification of hyperhidrotic areas , a starch - iodine test was used on six participants , to help guide injection locations . study reported a significant reduction in residual limb sweating and a significant improvement in use of the prosthetic device , duration of use and quality of life evaluated 4 weeks and 3 months after treatment . btx - b also decreased rlp and plp as well as improved quality of life and prosthesis use . reports that btx - b injections in muscular trigger points decreased rlp and involuntary movements of the stump for 4 - 12 weeks , which was dependent on the btx - b dose and location of amputation . two of the four patients had a trauma - related amputation , and all found the injections very painful . current literature investigating the use of btx to treat hyperhidrosis in trauma - related amputations is limited . however , current case studies all report positive outcomes related to treating hyperhidrosis with either btx - a or btx - b . hyperhidrosis , discomfort , and skin irritation related to prosthesis use in trauma - related limb loss remains a topic deserving of more attention as a larger , double - blind , placebo - controlled study evaluating the efficacy of btx - a and btx - b in treatment .

hyperhidrosis - related to prosthesis use in patients who have suffered a traumatic limb amputation presents itself as a barrier to comfort , prosthesis use and overall quality of life . this review intends to encourage dermatologists to consider the use of botulinum toxin a or b for the treatment of hyperhidrosis in the residual limb and may serve as a stimulus for a modern , in - depth , and more comprehensive study . a review of the literature was conducted using the pubmed database , focusing on hyperhidrosis treatment after traumatic limb amputation . articles discussing hyperhidrosis treatment for amputations secondary to chronic medical conditions were excluded . seven case studies published over the last 12 years have demonstrated positive outcomes of this treatment strategy . overall , there is little data examining this topic and current publications focus primarily on small case series . a larger , double - blind , placebo - controlled study would likely benefit veterans , service members , and civilians .

the complications related to drug abuse such as myocardial insufficiency , myocardial infarction , endocarditis , myocarditis , aortic dissection , neurologic damage , ischemic colitis and renal failure are known , but have been reported very rarely . amongst the illicit drugs , cocaine , amphetamines and cannabis have been studied and documented well to cause myocardial infarction by different mechanisms . there is very sparse data available on myocardial involvement after heroin abuse.[13 ] here , we report a young male who suffered from acute myocardial injury after heroin inhalation . a 28-year - old young male shopkeeper was brought to the emergency with complaints of chest discomfort , cold sweating and severe generalized weakness . there was no history of fever , headache , any flu - like symptoms , systemic illness or drug allergy . his parents were alive and there was no history of diabetes mellitus , coronary artery disease , hypertension or cerebrovascular disease in first degree relatives . he was not a cigarette smoker but his parents and patient himself gave history of heroin inhalation ( smack ) for last five years . as per history last heroin inhalation was done approximately 12 h before admission along with alcohol binge drinking . on general physical examination he was thin built , looked drowsy but arousable . his vital signs were pulse 64/min , blood pressure 70/50 mmhg , respiratory rate 24/min and temperature was 98.6f along with cold extremities . laboratory data showed mild leukocytosis with total leukocyte count of 14,200/ cmm , hemoglobin 14.6 gm% , platelet count 2.6 lacs / cmm . biochemical investigations including urea , creatinine , total protein , albumin , liver transaminases , glucose and fasting lipid profile were within normal limits . value<20u / l ) total cpk was 456 u / l(normal being < 200 ) and toponin - i was 3.02 ng / ml ( normal value < 0.01 ng / ml ) . in 2-d echocardiography hypo kinesis of left ventricular anterior wall was noticed while left ventricular systolic function and ejection fraction was within normal limits ( 64%).on the basis of history , clinical examination and investigations , diagnosis of acute coronary ischemic event involving left ventricular wall was made . since the patient had hypotension at presentation , he was treated with aspirin 325 mg and clopidogrel 300 mg and vasopressors , but nitroglycerine could not be given to him . the patient did not receive thrombolytic therapy , because of the pattern of electrocardiographic changes and time interval between the onset of chest tightness and hospitalization was more than 12 h. therefore , he was administered only subcutaneous low molecular weight heparin enoxaparin . he responded to treatment , got relief in the chest discomfort and was discharged after seven days . at the time of discharge , minimal st elevation persisted in electrocardiogram which became normal after a week during follow up along with the echocardiography . coronary angiography which was done one month later did not reveal any significant luminal stenosis . the patient was diagnosed to have acute myocardial involvement on the basis of symptoms of chest tightness , cold sweating and hypotension . his investigations that included electrocardiogram , echocardiography , cpk - mb and troponin - i and also favored the diagnosis of acute myocardial injury . he had bilateral constricted pupils with excessive salivation , basal crepitations in the chest and relative bradycardia despite hypotension . there was a definite history of heroin inhalation and binge drinking in the night prior to admission . no family history of premature heart disease or any conventional risk factors of cardiovascular disease were present in the patient . our patient presented with symptom of chest tightness , which was not localized , had no postural variation or relation with respiration . he possibly had acute myocardial ischemia which is being supported by elevated cardiac biomarkers , electrocardiographic changes and echocardiography . myocarditis can also masquerade as myocardial ischemia sometimes because of st - t changes , cardiac enzyme elevation and wall motion abnormalities which can be observed with myocarditis . myocarditis was a possibility in our patient that could not be excluded . during the hospitalization we could not subject the patient for either coronary angiography or myocardial scintigraphy to confirm the infarct or any occlusive lesion in the coronaries . heroin ( diacetylmorphine ) is rapidly hydrolyzed to 6-monoacetylmorphine which in turn is hydrolyzed to morphine . though heroin - induced myocardial infarction has been seldom reported , the underlying mechanisms have been still unclear . reported a case of heroin intoxication followed by rhabdomyolysis associated with myocardial injury , with symptoms , laboratory findings , ecg and echocardiography features of non - q wave infarction . however , a 201 thalium myocardial scintigraphy , performed after patient was discharged , did not show any abnormality . this shows that heroin probably has a direct myotoxic effect on both myocardium and skeletal muscle . there is a possibility that hypoxia , acidosis , vasoconstrictive substances released by muscle necrosis , or hypersensitivity reactions associated with heroin or some of its adulterants are involved in myocardial injury . reported a young woman with acute myocardial infarction after heroin abuse ; they had claimed that heroin might have a direct toxic effect on the coronary arteries and can cause coronary occlusion by provoking vasospasm or inflammation . there has been suggestion that heroin acts directly on vasomotor centre to increase parasympathetic activity and reduces sympathetic tone , which leads to vasodilatation and stimulates histamine release from mast cells . . there was history of binge drinking present in our patient which has been associated with development of acute myocardial infarction . men who consume five drinks ( 50 g ) or more and women who consume four drinks ( 40 g ) or more in single sitting are known as binge drinkers . it is well known that ethanol induces concentration dependent vasospasm in coronary arteries and coronary vasoconstriction may itself damage the endothelium thereby increasing the likelihood of platelet adhesion and thrombus formation . in conclusion , a 28-year - old young male developed acute myocardial injury after heroin inhalation and binge drinking while he had no other conventional cardiovascular risk factors . the cause might be heroin - induced cardio toxic effect or vasospasm compounded by the presence of binge drinking .

amongst the illicit drugs cocaine , amphetamines and cannabis have been studied and documented well to cause myocardial infarction by different mechanisms but there is very sparse data available on myocardial involvement after heroin abuse . we report a young man who developed acute myocardial injury after heroin inhalation and alcohol binge drinking . heroin induced cardio toxic effect and vasospasm compounded by alcohol were suspected to be the cause of this .

headache can be an accompanying symptom of cerebrovascular diseases ( up to 38% of cases ) , mostly depending on stroke etiology and localization . it is very common in posterior inferior cerebellar artery infarction with a frequency up to 76% in patients with wallenberg s syndrome . few papers reported that headache resembling trigeminal autonomic cephalalgias ( tacs ) is induced by dorsolateral medullary ischemic infarction [ 24 ] . here , we describe the case of a patient who developed headache and autonomic dysfunction after left dorsolateral medullary infarction . a 67-year - old caucasian male was admitted to our hospital for left orbital , retro - orbital and temporal continuous pressure - like pain of moderate intensity , and gait disorder that suddenly started 15 days earlier . his medical history included hypertension , diabetes , myocardial infarction , cistectomy for bladder tumor , and an asymptomatic cerebral meningioma in left parietal area with the dimension of 20 16 mm , never surgically treated . one month earlier , he arbitrarily stopped aspirin ( 100 mg / day ) and ticlopidine ( 250 mg / day ) intake . neurological examination revealed hypacusia on the left side , weaker corneal reflex , horner s syndrome , inferior facial hyposthenia and hypoesthesia , cold - like paresthesia in the first trigeminal branch territory , and no other signs of cranial nerves involvement or papillary edema . muscular tone and strength were conserved ; deep tendon reflexes were normal and symmetrical ; signs of pyramidal tract involvement were absent . however , the patient showed limb ataxia with left lateropulsion , but co - ordination and other cerebellar functions were intact . few days later , the continuous pressure - like pain had resolved , but the patient experienced dramatic pain flares triggered exclusively when moving from clinostatic to orthostatic position , which he could hardly maintain . pain exacerbations lasted as long as the patient stood and were associated with ipsilateral conjunctival injection , lacrimation , and nostril blockage ( fig . 1 ) . arterial blood pressure evaluation demonstrated orthostatic hypotension , changing from 150/90 mmhg in supine position to 115/80 mmhg in orthostatic position , persisting after 1 , 3 , and 5 min . 1the figure shows patient s lacrimation and conjunctival injection in the left eye associated with pain flairs the figure shows patient s lacrimation and conjunctival injection in the left eye associated with pain flairs common nsaids and pregabalin ( 150 mg / day ) were ineffective in controlling pain . indomethacin ( 100 mg / day ) , administered at the onset of pain for 3 days , slightly improved pain severity but not associated symptoms . verapamil ( 240 mg / day ) used on the basis of a previous report , successfully treated headache and vegetative phenomena but not horner s syndrome that partially recovered after 10 days . at the 3-month follow - up visit , headache characteristics had changed since the subacute phase of stroke . pain and associated symptoms clinically resembled tacs : attacks occurred several times a day ( 36 ) , twice a week , for about 1530 min , with milder pain than in subacute phase , and persistence of tearing and conjunctival injection . neurological examination showed left mild inferior facial palsy , dysesthesia in the first trigeminal branch territory , and ptosis ; left miosis as well as orthostatic hypotension was no longer present . central nervous system lesions can rarely present with cluster - like or sunct - like symptomatology [ 24 ] . the hypothalamus , via the hypothalamospinal tract , is a regulatory centre for integration of sympathetic and parasympathetic systems . experimental studies with functional mri and pet showed hypothalamic activation during tacs attack . moreover , stereotactic hypothalamic stimulation has been successfully used in drug - resistant patients , indirectly confirming the hypothalamic involvement in tacs pathophysiology . hypothalamospinal tract lies in dorsolateral medulla ; it is constituted by first order neurons responsible for orthosympathetic innervation of ipsilateral half face and body and projects to peri - acqueductal gray matter , thus activating the trigeminovascular system that is a well - known pain generator of headaches . in our patient , the ischemic lesion was located in the left posterior side of the upper medulla oblongata ( fig . descending fibers of the hypothalamospinal tract carry sympathetic innervation to the pericarotid plexus ( fig . 2b ) . in the subacute phase of stroke , horner s syndrome , as well as orthostatic hypotension , was symptomatic of a sympathetic impairment , while tearing and ocular injection reflected a parasympathetic activation . after 3 months , headache was still associated with vegetative symptoms but was no longer triggered by standing up , and lasted up to 30 min . in our opinion , the persistence of pain attacks with vegetative involvement in a chronic phase of stroke was due to an aberrant activation of trigeminovascular system by hypothalamospinal tract via the peri - acqueductal gray matter.fig . 2panel a : diffusion weighted ( left ) and fluid attenuated inversion recovery ( right ) magnetic resonance showing the dorsolateral medullary ischemic infarction . panel b : graphical representation of the hypothesized mechanism subtending our patient s symptoms panel a : diffusion weighted ( left ) and fluid attenuated inversion recovery ( right ) magnetic resonance showing the dorsolateral medullary ischemic infarction . panel b : graphical representation of the hypothesized mechanism subtending our patient s symptoms these clinical features resembled cluster headache . the weak response to indomethacin and the dramatic improvement after verapamil therapy supported this hypothesis . in fact , while indomethacin may be effective in treating paroxysmal hemicrania by inhibiting no - induced dural vasodilation , verapamil acts mainly as neuromodulator in the hypothalamus . this is the first case reporting the association of headache and orthostatic hypotension as part of an autonomic vascular impairment . in normal conditions , the hypothalamospinal tract is activated by standing up from a supine position via the baroreflex pathway so that the vasomotor reflexes lead to vasoconstriction and cardio - acceleration . we can speculate that in the subacute phase of stroke , the hypothalamospinal tract damage induces a dysfunction of sympathetic descending control of the peripheral vascular district ( i.e. , orthostatic hypotension ) and an aberrant trigeminovascular hyperactivation via the peri - acqueductal gray matter ( i.e. , headache with vegetative symptoms ) . this phenomenon may be interpreted as maladaptive plasticity or as an effect of ephaptic connections . after 3 months , the evolvement of this maladaptive plastic phenomenon led to a more typical cluster - like headache . in summary , our patient s case supports the hypothesis of a dysfunction of the hypothalamospinal tract in the pathophysiology of both pain and autonomic features of tacs .

stroke can present , among other signs , with headache . here , we describe the case of a man suffering from severe orbitary pain and autonomic dysfunction secondary to dorsolateral medullary ischemia . the anatomical relationship between lesion and symptomatology could be an indirect sign of hypothalamospinal tract involvement in the genesis of autonomic dysfunction and headache resembling a trigeminal autonomic cephalalgia .

isolated cranial nerve ( cn ) vi palsy is relatively common in the elderly population , in contrast to the pediatric population . most cn vi palsies in the elderly are attributable to microvascular ischemic disease , mainly diabetes mellitus or arterial hypertension . several other causes , including high intracranial pressure , giant cell arteritis , cavernous sinus mass and multiple sclerosis have also been reported . herpes zoster ophthalmicus ( hzo ) is the involvement of the ophthalmic division ( v1 ) of the trigeminal nerve and represents approximately 1020% of all cases of herpes zoster [ 3 , 4 ] . the acute course of hzo is usually benign ; however , serious ocular complications have been documented [ 4 , 5 ] . herein , we report a case of cn vi palsy which presented within a week of onset of a zosteriform rash . a 73-year - old white male presented to our emergency department with complaints of severe left - sided headache , diplopia , and limitation of abduction in the left eye . one week earlier he had developed multiple painful vesiculobullous eruptions on the left side of his forehead and tip of the nose . on presentation , dark pigmented and crusted patches were scattered in the patient 's left trigeminal nerve v1 distribution . polymerase chain reaction testing of the skin lesions and viral culture were deemed unnecessary . diagnostic workup , for an underlying primary or acquired immunodeficiency , turned out to be negative . at our initial examination , the patient 's visual acuity was 20/20 in the right eye and 20/30 in the left eye . there was no ptosis or anisocoria in the left eye and hertel 's exophthalmometry was normal . extraocular muscle movements were painless with restricted abduction of the left eye upon left gaze , compatible with a left cn vi palsy ( fig . slit - lamp examination and fundoscopy were normal in both eyes , except for mild conjunctival injection . pre- and post - contrast enhancement t1 images of the orbit and sellae provided no additional clues as to the cause of the ophthalmoplegia . a diagnosis of cn vi palsy due to hzo was made , and the patient was started on oral valacyclovir 3,000 mg / day for a week . the skin lesions resolved within 2 weeks and a gradual improvement in ocular motility was noted over the following months . in the 4th month of follow - up , the patient presented with a complete recovery of cn vi function with a minimal residual abduction deficit ( fig . 2 ) . at that time , his vision was 20/20 in both eyes . during follow - up , the patient had no post - therapeutic neuralgia and suffered no major side effects due to the treatment . the long intracranial course of cn vi and its fragile nature makes it highly susceptible to inflammation and injury . involvement of cn vi is the most common form of all cn palsies ( 43.8% ) , followed by cn iii ( 28% ) and cn iv palsies ( 15% ) . the leading causes of cn vi palsy are neoplasms ; however , in adulthood , vasculopathic disorders are the most common causes . inflammatory and infectious diseases can be listed as rare incidents among the etiological factors . in our case , an elderly patient presented one week after the onset of vesiculobullous eruptions with acquired diplopia and mild esotropia . due to a zosteriform rash and the cn vi palsy , the patient was diagnosed with hzo . four months after the initiation of antiviral treatment , his cn vi palsy improved dramatically and diplopia resolved . several hypotheses including vasculitis , muscle ischemia , contiguous intracavernous radiculomeningitis or cranial motor neuropathy have been postulated . presumably , the varicella zoster virus may be adopting more than one of these mechanisms . there is an ongoing debate regarding the importance of systemic antiviral treatment and corticosteroids [ 4 , 9 , 10 ] . systemic antivirals have been reported to reduce the risk of viral dissemination . besides , their beneficial effects in reducing the clinical signs of hzo have been demonstrated . therefore , we believe that antiviral therapy should be given in all hzo cases in order to minimize the frequency of potentially sight - threatening complications . on the other hand , some authors have recommended systemic corticosteroids to treat the possible vasculitic component and/or to prevent postherpetic neuralgia . it is well known that steroids should not be given alone ( without antiviral therapy ) due to concerns about promotion of viral replication . one should keep in mind that , especially in the elderly , it may present with cn palsy and subsequent morbidity . in addition to the more widely known ocular complications , recognition and management of cn palsies is of importance . in such a case , prompt treatment and close follow - up may provide an opportunity to suppress viral proliferation and persistence in the early stages of the disease . a detailed history and thorough examination of the skin

acquired palsy of the lateral rectus presents with horizontal diplopia and has a broad differential . herpes zoster ophthalmicus- ( hzo ) related cranial nerve palsy is a transient and self - limiting condition . systemic antiviral treatment is administered in order to prevent sight - threatening complications . in suspected cases , zosteriform rash should be questioned . one should keep in mind that acquired esotropia in the elderly may sometimes present following hzo .

malignant peripheral nerve sheath tumor ( mpnst ) of neck region is an extremely rare entity , especially , when presenting as a parotid mass . however , it is of diagnostic importance because it is one of the most aggressive malignant soft - tissue sarcomas and it is also called as neurofibrosarcoma as it arises from the neuroectodermal lining of the peripheral nerves . mpnsts are often associated with von - recklinghausen 's disease of skin . the incidence of mpnsts with von - recklinghausen 's disease of skin is 30 - 50% and with previous irradiation is 4 - 11% while rest of the cases arise de novo . a 78-year - old male presented with a gradually increasing painful swelling over left side of the neck since 8 years . physical examination revealed a mass in the lateral region of the neck behind the sternocleidomastoid muscle . fine needle aspiration cytology ( fnac ) was performed , which was suggestive of a cystic lesion of the salivary gland . computed tomography ( ct ) scan showed centrally necrotic rounded mass lesion arising from parotid gland [ figure 1 ] . the lesion was almost completely replacing the outer lobe of left parotid gland and the inner lobe was normal . ct findings suggested neoplastic lesion of the left parotid gland with involvement of bilateral submandibular nodes and left level ii lymph nodes . intra - operatively a 7 6 cm mass was seen involving the left parotid gland and all the branches of the facial nerve except the cervical branch . the entire tumor along with the parotid gland and the facial nerve was excised en block . computed tomography scan - neoplastic lesion of left parotid gland ( arrows ) histopathological examination revealed a tumor composed of spindle shaped cells with elongated hyperchromatic nuclei , arranged in long and short fascicles [ figure 2 ] . at places cells were arranged around hyaline bands in the form of cords and at places they were arranged in whorls . some of the tumor cells had epitheloid appearance , while some cells had marked nuclear pleomorphism with tumor giant cells . in some areas , mitotic figures were more than five per high - power field and areas of compressed parotid gland were present at the periphery of tumor . ( a ) tumor with normal parotid gland ( h and e , 100 ) , ( b ) tumor showing spindle shaped cells arranged in long and short fascicles ( h and e , 100 ) , ( c ) tumor showing chondroid differentiation in some areas ( h and e , 100 ) . tumor giant cells and abnormal mitosis ( h and e , 400 ) immunohistochemically , the tumor cells showed diffuse and strong positivity for s-100 , ( dako ) , however , were negative for pan - cytokeratin ( dako , ae1/ae3 ) , desmin ( dako , d33 ) , and human melanoma black ( hmb ) 45 ( dako ) [ figure 3 ] and vimentin ( dako ) . with the above features , a diagnosis of mpnst was made . immunohistochemical staining showing ( a ) positivity of tumor cells with s-100 and negativity for ( b ) pan cytokeratin , ( c ) hmb 45 , and ( d ) vimentin ( 400 ) post - operatively , the patient had facial palsy [ figure 4 ] and distant metastases were not found on whole body scan after 6 months of follow - up . post - operative photograph showing ( a ) location of excised tumor , ( b ) patient with post - operative facial palsy mpnsts also called as neurofibrosarcomas are malignant tumors developing from cells present in peripheral nerve tissue . the mpnst accounts for approximately 5 - 10% of all soft - tissue sarcomas and about one - fourth to one - half occur in the setting of neurofibromatosis ( nf-1 ) . the mpnst is typically a disease of adult life , as most tumors occur in patients of 20 - 50 years of age . the most common anatomical sites for mpnst include extremities , head and neck region and trunk . parotid gland involvement is very rare in this disease and it is very difficult to differentiate other parotid tumors from mpnsts on clinical or gross examination . , in their study on a series of 303 benign nerve sheath tumors found that 44.9% of the tumors occurred in head and neck region . in the neck , they may arise medially or laterally . medially , they arise from the last four cranial nerves ( glossopharyngeal , vagus , accessory , and hypoglossal ) or the sympathetic chain . laterally , they arise from cutaneous or muscular branches of the cervical plexus or the brachial plexus . schwannomas are encapsulated tumors of nerve sheath and their growth usually does not involve the nerve per se . however , neurofibromas are cellular ; have low or no mitotic activity and show minimal nuclear atypia . low - grade mpnsts arising from neurofibroma is diagnosed when there is generalized nuclear atypia , increased cellularity and unusually low - levels of mitotic activity . wide surgical excision of tumor and radical neck dissection is the treatment of choice for mpnsts arising in head and neck region . the prognosis of mpnsts of head and neck region is poor and have 5 year survival rate of 20 - 50% . large tumor size ( > 5 cm ) , presence of neurofibromatosis , and total resection are most important prognostic indicators for mpnst . histologic findings such as cellularity , pleomorphism , mitotic activity , and size also contributes in assessing the prognosis of the tumor . for surgeons the invasion of facial nerve and parotid gland is suggestive of malignant tumor of parotid gland . absence of neurofibromatosis and complete removal of tumor with radical neck dissection is probably the reason for better prognosis in our case . we present this case to show that an unusual tumor can be encountered when dealing with a parotid mass . the pathologists and surgeons should keep this in mind when dealing with a patient of parotid mass . wide surgical resection and adjuvant

malignant peripheral nerve sheath tumor ( mpnst ) is very uncommon tumor of parotid gland and it is an uncommon spindle cell sarcoma accounting for approximately 5% of all soft - tissue sarcoma . there is strong association between mpnsts and neurofibromatosis ( nf-1 ) and previous irradiation . structural abnormality of chromosome 17 is associated with nf-1 and so mpnst . we present a case of a 78-year - old male presenting with slowly growing parotid mass who underwent tumor resection .

pelvic varicocele is the cause of the so - called pelvic congestion syndrome , a quite frequent clinical condition in which the ectatic veins cause a congestion of all the pelvic organs ; this pathology is very often underestimated and misdiagnosed because it is characterized by some aspecific and vague symptoms , but sometimes invalidant , that make the diagnosis very difficult . on the contrary , the development of a right ovarian vein syndrome is a very rare clinical entity ; this pathology is thought to sometimes occur when the pelvic varicocele is associated to a dilation of the right ovarian vein ; the lumbar ureter at the crossing with the vascular iliac trunk is trapped into a kind of vascular forceps generated by the common iliac artery and the ectatic ovarian vein . the same ovarian veins dilation occurs , for example , after the assumption of a high dose of estrogens or for other reasons . the compression of the ureteral lumen may cause some indefinite symptoms up to serious clinical manifestations ; the decubitus of the ureteral walls generates a chronic inflammation of the urothelial mucosa with a plicar hypertrophia that produces recidivant lumbar and pelvic pain , as a renal colic . in this condition , we can find an aseptic leucocyturia ; the pyelonephritis developing and rare episodes of urinary bleeding could finally occur . we have not found in the literature any analogous cases in which the hematuria has been the first clinical manifestation of this rare syndrome in absence of typical pelvic pain and , above all , any experience about the radiologic endovascular management of the symptoms . we received a request of consultation for a 32-year - old woman with a 5-year history of frequent and recurring episodes of gross hematuria . the patient was admitted four years before in another university hospital of the north of italy where she underwent several diagnostic procedures ; among these , a cystoscopic evaluation showed a right ureteral hematuria and the ureteroscopy , with the associated mucosal brushing of the lumbar tract , indicated an aspecific phlogistic reaction with the presence of only inflammation cells . the research of the koch 's bacillus on the urinary culture was also negative . without a clear diagnosis and , above all , with a persistent hematuria , the patient was admitted in 2007 to our hospital where she practiced a sequential renal scintigraphy and a multidetector contrast - enhanced computed tomography , both negative . in fact , the delayed contrast phase performed during the renal excretion with the help of 3d reformatted images , indicated an irregular right ureteral morphology with a sharpening of its lumen due to an external compression where it crossed the right iliac artery and the correspondent ovarian vein . a slight pielic enlargement with a dilation of the proximal right ureter was also associated . finally , we appreciated some peri uterine and periovarian varices , like a pelvic varicocele ( figure 2 ) and a pelvic ultrasonographic examination with a color - doppler evaluation which confirmed our diagnosis ( figure 3 ) . our diagnosis was confirmed by a subjective symptom during our anamnesis with pain referred only on the right iliac fossa and independent from the decubitus . we did not find any clinical symptom of pelvic congestion ( no dull pelvic pain ) , neither lower extremity venous insufficiency . after informed consent , by a percutaneous common femoral vein access , we performed a catheterization and a subsequent selective phlebography of both the ovarian veins . like in the male varicocele , we used the scleroembolisation technique , injecting a sclerosing agent ( sodium tetradecilsolphate 3% ) . a complete sclerosis of the ovarian vein was first performed on the left side while , on the right one , only a sclerosis of the distal third , saving the lumbar tract where endoscopic evaluation had just showed a phlogistic reaction ( figure 4 ) . the treatment was successfully performed and the color - doppler evaluation was unable to depict the periuterine varices anymore ( figure 5 ) . ct examination confirmed the decreasing of the pielic and ureteral dilation with a good return to a normal caliber and morphology ( figure 6 ) . during the following 3 months , the patient was completely free from right - side pain and , at the followup at 1st , 6th , 12th , 18th , and 28th month , she never suffered hematuria anymore . the pelvic varicocele is very often unknown and underestimated but it may cause a lot of psychological and social problems to the young affected patients . the symptoms are quite aspecific and vague : chronic pelvic heaviness , especially referred after a long standing position , for example , after a long working day , dyspareunia , dysmenorrhea , menorrhagia , urinary urgency , and constipation . in a great number of cases , a lot of diagnostic examinations are performed , from endoscopic ( cystoscopy , ureteroscopy ) to radiologic ones ( scintigraphy , ct scan ) and the discovering of a pelvic varicocele is a casual event . instead , a simple pelvic u.s . frequently the radiologic signs of this pathology , the periuterine varices , are not considered a pathological finding and are not depicted by the radiologist either [ 3 , 4 ] . in our case , the hematuria was the only objective symptom referred by the patient and it is thought to be very unusual . on the contrary , the only subjective symptom was the right iliac fossa pain without correlation to the standing position . the diagnosis of a right ovarian vein syndrome has been made ex adiuvantibus because the endovascular treatment had reduced the periuterine venous congestion and , together with them , the pielic and ureteral dilation . on the right side , in particular , the ovarian vein had an evident dilation in absence of blood reflux , tightly compressing the lumbar ureter that was deformed and irregular . the first description of the right ovarian vein syndrome has been made by clark in the far 1965 ; by histological findings , he explained the true physiopathological mechanism subordinated to this pathology . a very close anatomic relationship between the right ovarian vein and the homolateral iliac artery generates a kind of vascular forceps that hold the lumbar ureter ; its mucosal walls decubitus causes a fibrotic entrapment , and the chronic compression is testified by a plicar hypertrophia that is responsible of the aseptic leucocyturia . the hematuria , the first clinical sign , is thought to be a very rare demonstration of this syndrome and the vague right iliac pain , localized approximately to l4 level , is the result of the phlogistic involvement of the right lumbar ureter . the endovascular treatment [ 79 ] removed the congestion of the pelvic varicocele and , consequently , the detention of the right ovarian vein solved the ureteral compression [ 10 , 11 ] .

we report the case of a young woman admitted because of several and recurring episodes of macroscopic hematuria beginned after her first pregnancy . contrast - enhanced multidetector computed tomography images showed dilated ovarian veins due to a typical pelvic varicocele . we supposed to be a right ovarian vein syndrome , a rare clinical situation characterized by an anomalous compression of the lumbar ureter by the ectasic ovarian vein ; this condition may cause a chronic inflammatory stimulus above the urothelial mucosa with a following hematuria . all symptoms were solved by an endovascular treatment through the sclero - embolisation of the pelvic varicocele . after eighteen months the patient did n't present hematuria anymore and she no longer complained about her right side lumbar pain .

granulocytic sarcoma or extramedullary myeloid tumor ( emmt ) is an uncommon neoplasm of immature hematopoetic cells . these tumors are reported in 3.1 - 9.1% of patients with myeloblastic leukemia and occur concomitantly with , after , or rarely before the onset of leukemia . we report a case with multiple tumors involving the lymph nodes , breast and orbit . the diagnosis was established on fine needle aspiration cytology ( fnac ) of the masses which subsequently led to the diagnosis of the underlying leukemia . a 15 year - old girl presented with bilateral , cervical lymphadenopathy , bilateral breast masses , and gradually developing proptosis of both eyes , in that order . nodes were firm to hard with restricted mobility and the breast masses were 4.5 cm ( left ) and 5.0 cm ( right ) along their maximum dimension . fine needle aspiration cytology ( fnac ) was done from the lymph nodes and breast masses . both wet - fixed and air - dried smears were prepared for papanicolaou and may - grnwald giemsa ( mgg ) staining respectively . smears from lymph nodes and the breast showed similar features with a polymorphous population of large atypical cells scattered in a background of mostly mature and a few reactive lymphoid cells [ figures 1 and 2 ] . cytoplasm was pale blue and a fair number of cells showed auer rods and a few fine granules ; the background showed lymphoglandular bodies . cell morphology from both sites was suggestive of myeloid blasts , so a diagnosis of granulocytic sarcoma / extramedullary myeloid tumor was made . smears from lymph node show infiltration by numerous myelomonoblasts ( mgg , 400 ) aspirate from breast with leukemic cells ( mgg , 400 ) the peripheral smear showed normocytic , normochromic anemia with leukocytosis . total leucocyte count ( tlc ) was 48000 cells / mm with the differential count showing 70% blasts , 2% promyelocytes , 8% myelocytes , 8% metamyelocytes , 2% band forms , and 10% lymphocytes . platelet count was adequate and the blasts were as described above with mostly indented nuclei and auer rods [ figure 3 ] . out of these , a considerable proportion showed monocytoid features ; the blasts were diffusely positive for sudan black b ( sbb ) and negative for periodic acid schiff ( pas ) . non specific enolase ( nse ) was noncontributory , based on which , a diagnosis of acute myeloid leukemia ( aml)-m4 was given . peripheral smear shows myeloblasts with auer rods and cells in mitosis ( giemsa , 1000 ) the patient was started on standard chemotherapy to which she responded well . granulocytic sarcoma or extramedullary myeloid tumor may be seen in association with chronic myeloid leukemia ( cml ) , myelodysplastic syndrome , ( mds ) , myeloproliferative syndrome ( mps ) , polycythemia vera , and essential thrombocytosis . emmt may be seen in known patients of aml or may occur ( rarely ) as the first manifestation of the disease . in other cases , it may be the first sign of systemic relapse of a case of treated aml . emmts may occur at any site in the body , common ones being the skin ( leukemia cutis ) , lymph nodes , and mediastinum . other sites such as epidural , uterus , ovaries , testis , and orbits have also been reported . a few case reports have described it in the breast[13 ] where it is often misdiagnosed as lymphoma . granulocytic sarcomas of the orbit have mostly been described as isolated masses which may precede systemic manifestations . the soft tissue counterparts may present months before the involvement of peripheral blood and bone marrow . they produce symptoms according to their anatomic location but they may be asymptomatic as well . problems in diagnosis arise when they are suspected in patients without a background of myeloprolifrative disease . they may often be misdiagnosed as non - hodgkin 's lymphomas , rhabdomyosarcomas , amelanotic melanomas , or undifferentiated carcinomas . light microscopy reveals collections of cells that can be usually recognised as being myeloid . undifferentiated or minimally differentiated blasts generally pose diagnostic problems and these are cases that are misdiagnosed as lymphoma . the cells in our case were positive for sbb and myeloperoxidase ; pas was negative . immunostaining with monoclonal antibodies against myeloperoxidase ; anti - cd43 , and anti - lysozyme are the most sensitive antibodies ; cd68 and cd20 are useful in differentiating it from lymphoma . it is regarded as a poor prognostic indicator by some , whereas others have not considered it as an independent prognostic factor . if they are diagnosed in patients previously treated for aml , they signify relapse whereas in a patient of chronic myeloproliferative disease , they may be indicative of an impending blast crisis . most tumors , whether detected prior to or during the therapy of leukemia , respond well to standard chemotherapeutic agents .

granulocytic sarcomas or extramedullary myeloid tumors represent the soft tissue counterpart of acute myeloid leukemia . the term is used for any solid collection of leukemic cells . there have been reports of these tumors occurring before the involvement of blood or bone marrow . our patient had simultaneous involvement of three sites , which was diagnosed on cytology . further confirmation was done on peripheral blood and bone marrow evaluation .

peripheral exudative hemorrhagic chorioretinopathy ( pehcr ) is a rare disorder of the peripheral retina characterized by either a hemorrhagic or an exudative retinal degenerative process.1 since the first report of this condition by reese and jones2 in 1962 , a few cases have been reported in the literature.3,4 more recently , shields et al5 reported 173 cases of pehcr . they showed that pehcr is predominantly a disease of elderly women , and manifests as bilateral lesions located in the temporal area between the equator and the ora serrata . most pehcr cases are accompanied by age - related macular degeneration , and sometimes cause sudden extensive subretinal and vitreous hemorrhage . the etiology is unknown , but involvement of peripheral choroidal neovascularization has been suggested.6,7 intravitreal injection of antivascular endothelial growth factor antibodies , such as bevacizumab and ranibizumab , is so far the most effective treatment for macular diseases accompanied by choroidal neovascularization.810 cryoretinopexy and photocoagulation11 have been used for the treatment of pehcr , and the efficacy of intravitreal injection of bevacizumab ( 2.5 mg in 0.1 ml ; genentech / roche , san francisco , ca ) has been reported recently.1215 we report a case in which intravitreal injection of ranibizumab was effective for the treatment of pehcr with choroidal neovascularization presenting with extensive subretinal hemorrhage . a 70-year - old woman visited our department because of a subjective sensation of a defective superior visual field in her left eye for 3 days before presentation . cataract surgery had been performed in both eyes 8 years earlier and intraocular lenses had been implanted . she had no past history of diabetic mellitus , hypertension , or hyperlipidemia . on examination , her best - corrected visual acuity was 20/20 in the right eye and 1/20 in the left eye , and intraocular pressures were 14 mmhg and 15 mmhg , respectively . fundus examination revealed extensive subretinal hemorrhage in the inferior temporal region near the arcade vessels of the left eye ( figure 1a and b ) and exudative age - related macular degeneration with a fibrotic scar in the left eye . fibrotic macular degeneration was also observed in the left eye ( figure 1a ) , which might have resulted from vitrectomy for removal of subretinal hematoma associated with age - related macular degeneration 4 years earlier . fluorescein angiography and indocyanine angiography revealed choroidal neovascularization corresponding to the region of subretinal hemorrhage ( figure 1a f ) . pehcr was diagnosed , and intravitreal injection of ranibizumab ( lucentis ; novartis , basel , switzerland ) was given in accordance with the protocol for treatment of age - related macular degeneration.16 after the first intravitreal injection of ranibizumab , the subretinal hemorrhage was obviously reduced compared with before treatment ( figure 2 ) . ophthalmoscopic examination showed complete resolution of subretinal hemorrhage ( figure 3a and b ) , and fluorescein angiography and indocyanine angiography showed reduction of choroidal neovascularization corresponding to the region of subretinal hemorrhage before treatment ( figure 3c f ) , although best - corrected visual acuity in the left eye remained unchanged at 1/20 because of fibrotic macular degeneration . no recurrence of choroidal neovascularization - induced retinal lesions , including subretinal hemorrhage , and no adverse effects of intravitreal ranibizumab injection were observed during one year after the final injection . in this case , pehcr developed in an elderly woman and was accompanied with age - related macular degeneration . moreover , these features correspond to the characteristics reported by shields et al.5 past reports6,7,1115 have suggested that polypoidal choroidal neovascularization was the origin of age - related macular degeneration . in this case , fluorescein angiography and indocyanine angiography revealed choroidal neovascularization corresponding to the region of subretinal hemorrhage . the origin of this case was considered to be the same as those in past reports . generally , pehcr has a favorable prognosis , but recurring hemorrhage sometimes enlarges the lesion , resulting in macular degeneration and visual loss.17 therefore , laser photocoagulation , cryoretinopexy , and intravitreal bevacizumab injection have been used for the treatment of pehcr . although laser photocoagulation and cryoretinopexy are beneficial if the causative peripheral vascular nets can be identified,18 their efficacy is uncertain in other conditions . on the contrary , laser photocoagulation may exacerbate subretinal hemorrhage,12 and induce cystoid macular edema presumably secondary to confluent ablative therapy.6 cryoretinopexy may also worsen subretinal hemorrhage and cause subretinal fibrosis.12 these treatments are useful to achieve rapid resolution in some cases of pehcr , but do not appear to be useful in improving the visual outcome.1 photodynamic therapy with verteporfin is useful in patients with subfoveal choroidal neovascularization , but often enlarges chorioretinal atrophy after treatment.18 recently , the efficacy of intravitreal bevacizumab injection for pehcr has been reported.1215 after three injections at monthly intervals , vision improved and lesions remained stable , with some regression of subretinal hemorrhage . no further subretinal hemorrhage was noted during postoperative convalescence and the retina remained flat and attached.13 however , bevacizumab is currently approved for clinical use in the treatment of cancers , such as colon cancer and metastatic non - small - cell lung cancer , and is not approved for ocular vascular diseases in japan . only ranibizumab is approved for the treatment of age - related macular degeneration by intravitreal injection route . therefore , although intravitreal ranibizumab injection for pehcr has not been reported , we used ranibizumab instead of bevacizumab as an antivascular endothelial growth factor antibody in the present case . the results of this case indicate the possibility that the curative efficacy of ranibizumab for pehcr would be equivalent to that of bevacizumab .

peripheral exudative hemorrhagic chorioretinopathy ( pehcr ) is a rare disorder that sometimes causes sudden subretinal and/or vitreous hemorrhage . choroidal neovascularization is involved in the pathogenesis , but the etiology is unknown . treatments with photocoagulation , cryopexy , and intravitreal bevacizumab injection have been reported . however , the therapeutic effect of intravitreal injection with ranibizumab for pehcr is unclear . a 70-year - old woman visited our department because of sudden loss of superior visual field in her left eye . she had a history of surgical removal of hematoma due to subretinal hemorrhage associated with age - related macular degeneration 5 years ago . peripheral subretinal hemorrhage was observed in the left eye , and fluorescein and indocyanine green angiography revealed choroidal neovascularization in the subretinal hemorrhagic region . pehcr was diagnosed . considering her past history , intravitreal ranibizumab injection was used for treatment . after three injections in the left eye , subretinal hemorrhage and choroidal neovascularization resolved completely . no recurrence was observed during 1 year of follow - up . this case demonstrates that intravitreal injection of ranibizumab is an effective treatment for pehcr with subretinal hemorrhage .

barotrauma - induced pneumocephalus resulting from a rapid change in the surrounding pressure is a major concern for airline passengers and scuba divers5,6,9 - 11,14 ) . recent advances in aircraft technology have led to a tremendous increase in air travel for the general population and for neurosurgical patients . neurosurgical procedures , including cranial and spinal surgery , could be a cause of residual intracranial air and could create a dormant fistulous tract that opens when the cabin pressure changes . during air travel , a neurosurgical patient could experience fatal tension pneumocephalus resulting from expansion of residual air in the head and pneumoencephalomeningitis secondary to cerebrospinal fluid ( csf ) leakage and the backflow of contaminated air through the bony defect . to generate interest and considerations about safe air travel of neurosurgical patient among practicing consultant neurosurgeons , the authors report a rare case of barotrauma - induced pneumocephalus experienced by a high risk patient after commercial air travel . a 49-year - old female presented to the emergency department with a severe headache , nausea , and vomiting after one hour flight on a domestic commercial airliner . a cranial nerve examination revealed anosmia through both nostrils and a visual field defect of the left inferior homonymous quadranopsia . additional cranial nerve function , motor and sensory function , the deep tendon reflex , and cerebellar function were normal . ten years ago , this patient was diagnosed with meningioma in the occipital lobe and underwent a suboccipital craniotomy to remove the tumor at another hospital . further questioning revealed that the patient underwent four additional otolaryngologic operations ( two operations involving the mastoid sinus and two involving the frontal skull base through the nose ) to manage csf rhinorrhea four years after initial the craniotomy . finally , a lumboperitoneal shunt was placed , and the patient did not experience further symptoms of a csf leak . the revision surgery for a recurrent tumor was performed last year ; at the time of the incident discussed here , the patient was returning home after gamma knife radiosurgery for the residual tumor . 1 ) demonstrated extensive air within the subarachnoid space of the basal cistern , bilateral lateral and third ventricle , and extending to the intraparenchymal portion of the left frontal lobe . the routine laboratory blood tests ( leukocyte count , hemoglobin , hematocrit , glucose , blood urea nitrogen , creatinine , sodium , potassium , chloride , and c - reactive protein ) were normal . normal saline and analgesics were administered via an intravenous line , and a gradual resolution of the headache was achieved within several days . seven days after presentation , the patient reported no complaints , and no evidence of meningoencephalitis was observed . the patient was warned not to fly before a definite diagnostic work up and sealing procedure for the csf leak site was performed , and the patient was then discharged . the second world war led to tremendous advances in the technologies related to airplanes and enabled many people to travel by air . although modern commercial aircrafts provide safe and comfortable travel for most healthy people , the in - flight environment consisting of a lowered barometric pressure , ambient oxygen partial pressure , and low humidity1 ) could expose a high risk patient ( such as the patient discussed here ) to danger . international jet airliners fly at an altitude of 9750 to 11580 meters ( 32000 to 38000 feet ) , and domestic airlines fly at 8530 to 8840 meters ( 28000 to 29000 feet ) . modern jet aircrafts are not pressurized to a sea level equivalent pressure ( 760 mm hg ) . during flight , the cabin is pressurized to maintain the pressure equivalent to the altitude of 1520 to 2440 meters ( 5000 to 8000 feet)1 ) . in other words , the cabin pressure is maintained within 536 to 611 mm hg . gas in the cabin has been shown to expand its volume by 25 - 30% during flights3 ) . neurosurgical , plastic , and otolaryngologic procedures , especially a craniotomy or skull base surgery , could leave air in the skull and create weak areas where a csf leak could occur . the traveling patient could potentially encounter two neurosurgical problems : tension pneumocephalus resulting from expansion of the residual air after a craniotomy or a csf leak through a bony defect made by surgical procedures , resulting in pneumocephalus or pneumomeningoencephalitis . until now , there have not been any reported cases of tension pneumocephalus caused by residual air during flight . donovan et al.7 ) reported no aggravated cases of neurological deficits and intracranial pressure among twenty - one military casualties with pneumocephalus caused by trauma or a craniotomy during air transport from iraq to germany under usual cabin pressure . two british studies focused on the medical advice given to post - craniotomy patients regarding air travel and addressed the unstandardized approach of airline companies and insufficient consensus among practicing consultant neurosurgeons2,13 ) . a cracked tooth5 ) , mastoid air cells10 ) , osteoma of the skull base11 ) , pansinusitis6,9 ) , and a fistulous tract made by cranial4 ) and spinal8 ) surgery were suggested as weak areas or causes of weak areas that were susceptible to pressure changes . in our case , pneumocephalus appeared to occur through the dormant fistulous tract because of the lowered cabin pressure and the synergistic effect of the lumboperitoneal shunt . the medical guideline recommends waiting at least 7 days to fly after a transcranial procedure or a trauma that introduced gas into the skull without reliable evidence from a plain skull radiograph or ct scan that no air is present . moreover , a person with a csf leak from any cause should not fly due to the risk of a backflow of air and microbial contamination1 ) . although it is difficult to make one guideline to apply to all cases , the concern for possible problems during air travel by patients could lead to consensus among practicing consultant neurosurgeons . creating guidelines for safe air travel for patients with neurosurgical problems is an important issue to consider because of the frequency with which some patients travel . a consultation with a health care provider addressing the cabin environment and neurosurgical conditions could be helpful for these patients .

a 49-year - old female with a history of several neurosurgical and otolaryngologic procedures for occipital meningioma and cerebrospinal fluid leaks was diagnosed with pneumocephalus after a one hour flight on a domestic jet airliner . despite multiple operations , the air appeared to enter the cranium through a weak portion of the skull base due to the low atmospheric pressure in the cabin . the intracranial air was absorbed with conservative management . the patient was recommended not to fly before a definite diagnostic work up and a sealing procedure for the cerebrospinal fluid leak site had been performed . recent advances in aviation technology have enabled many people to travel by air , including individuals with medical conditions . low cabin pressure is not dangerous to healthy individuals ; however , practicing consultant neurosurgeons should understand the cabin environment and prepare high risk patients for safe air travel .

rms can occur at any age ; the peak incidence is between 2 and 6 years of age , and the second peak occurs at ages 14 - 18 years old but extremely rare it may occur in the neonatal period [ 2 , 3 ] . there are only a few reports for rms in neonates [ 3 , 4 ] . rms may be present at birth : approximately 5 - 10% of all cases occur in patients aged<1 year , and 1 - 2% of all cases are congenital . it may arise anywhere in the body , but it has a predilection for the head and neck area , genitourinary tract , and the extremities . chest wall is a rare site for rms [ 3 , 6 ] . cooperative multidisciplinary treatment protocols have substantially improved the outcome of children with rms over the past 25 years . currently , patients with localized disease can be cured in > 70% of cases [ 1 , 7 ] , nonetheless , the small percentage of patients aged<1 year are particularly difficult to manage . the well - known physiologic immaturity of various organs is responsible for the vulnerability of infants to acute and late effects of therapy , and functional immaturity of liver leads to a different metabolism of drugs in infants as compared with older patients . infants with rms require tailored treatments , the use of radiotherapy is restricted due to very high risk of side effects , and the morbidity of full - dose chemotherapy is unacceptable . various reports indicate that the outcomes for infants are the same as older children and are not affected by dose reduction [ 7 , 8 ] but more recent data suggest a less favourable outcome for patients aged<1year . patient was the 3rd born child to a healthy mother at 38th weeks of gestation by normal vaginal delivery followed by dystochia . on examination , there was a hard , round , ecchymotic , nontender , slightly movable , warm and shiny 10x15 cm mass on her right axillary pits that was extended to the right side of the neck and chest wall ( figure 1 ) . the mass separated the shoulder from the chest wall and there was a paralysis of right hand . ultrasound showed a huge , heterogenic hypoechogenic mass 20 cm in size and enhanced vascular pattern and suspected to be a hemangioma . mri with contrast and ct - angiography demonstrated a soft tissue mass suspected to be a hemangioma and suggested the possibility of rms or neurofibroma ( figures2 , 3 ) . the mass rapidly increased in size along with rupture and bleeding despite aggressive steroid therapy . on the 45th post natal day neonate was taken to operating room to control the bleeding and if possible total excision of the mass . the mass separated easily from the surrounding tissue and was excised along with right upper extremity amputation . pathologic evaluation showed rms in this patient which was confirmed by immunohistochemistry ( ihc ) results ( myo - d : positive , cd99 : negative ) . molecular biology ( paxiii and paxvii ) is needed for definite diagnosis ( figure 4 ) . at the end of surgery only 2% of childhood tumors occur in the first month of life , of which 20% are mesenchymal in origin . the incidence of soft tissue sarcoma is believed to be approximately 8 in 1,000,000 and more than half of these are rhabdomyosarcoma . lobe te , et al . reported only 4% of neonatal rms in a large series of involved rms patients . embryonal rms is the most common type ( 60 - 70% ) , and also is the most predominant in neonates , infants and young children . rms usually occurs in the head and neck region , and approximately 20% in extremities [ 3 , 12 ] . it has been noted that in congenital rms , the disease may be metastatic at birth ( organs and placenta too ) . mri is the primary imaging modality in rms with its superior ability to depict soft - tissue changes . ct - scan of chest is mandatory in order to assess pulmonary metastases . in our case ct - scan of chest and upper abdomen was done , which suggested the possibility of rms or neurofibroma . the clinical management of malignancies in patients aged < 1 year is particularly difficult and requires special therapy modifications and careful monitoring to prevent severe complications . treatment of neonatal rms requires a multidisciplinary approach , where surgery and chemotherapy both have their own specific roles . complete resection of tumor is recommended ; embryonal rms generally responds to chemotherapy very well , and a long - term follow - up is necessary to evaluate the outcome of treatment . a good response to chemotherapy allows surgery less aggressively if needed , and the use of radiotherapy is restricted due to very high risk of side effects , and it should be avoided in the newborns . therefore , the overall survival rates for patients under 1 year of age and patients over 1 year of age did not differ significantly . the treatment for neonatal rms requires a multidisciplinary approach where surgery and chemotherapy both have their own specific roles . surgical option is radical or partial resection , and chemotherapy is recommended in all cases , but radiotherapy is necessary when the tumor can not be completely removed . when the tumor size is huge we should firstly consider biopsy and chemotherapy followed by a surgery . the treatment for neonatal rms requires a multidisciplinary approach where surgery and chemotherapy both have their own specific roles . surgical option is radical or partial resection , and chemotherapy is recommended in all cases , but radiotherapy is necessary when the tumor can not be completely removed . when the tumor size is huge we should firstly consider biopsy and chemotherapy followed by a surgery .

a 16-day - old female was referred with congenital swelling on her right shoulder . on examination , there was a hard , round , ecchymotic , nontender , slightly movable , warm and shiny 10x15 cm mass on the right axillary pits which was extended to the right side of neck and chest wall . the mass separated the shoulder from the chest wall causing paralysis of right hand . chest x - ray , ultrasound and mri with contrast demonstrated a soft tissue mass suspected to be a hemangioma . the mass rapidly increased in size despite aggressive steroid therapy with rupture and bleeding . on the 45th post natal day the baby was taken to operating room to control the bleeding and if possible total excision of the mass . the mass was separated easily from the surrounding tissue and was excised along with right upper extremity . at the end of surgery the baby had cardiac arrest , and apparently died of disseminated intravascular coagulation ( dic ) . the final pathology report was rhabdomyosarcoma ( rms ) .

hairy polyp ( hp ) is a developmental malformation that was described by brown kelly in 1918.1 they are most commonly a type of dermoid.2 they are frequently located in the orbit , nasal dorsum , floor of the mouth , infratemporal fossa , nasopharynx , oropharynx , and anterior and lateral side of the neck.3 4 5 6 7 8 they can also originate from the eustachian tube.1 9 only 1 to 7% of all dermoid cysts affect the head and neck region ; 23% of those are seen in the floor of the mouth.10 11 when dermoids and epidermoids are located in the head and neck region , they are the most congenital midline nasal masses.12 dermoid cysts , like teratomas , consist of embryonic germ layers . teratomas contain all three germ layers including ectoderm , mesoderm , and endoderm , and dermoid cysts contain only mesoderm and ectoderm.13 mesodermal elements , which include hair follicles and sweat glands within the wall of the dermoid cysts , differentiate it from simple epidermoid cysts.14 15 although both hps and dermoid cysts contain ectodermal and mesodermal germ layers histologically , dermoid cysts contain ectodermal inclusion cysts in their mesodermal layer , which is lacking in hps.12 the purpose of this report is to present and discuss a rare case of oropharyngeal hp resulting in an upper airway obstruction during crying . various congenital pathologies may cause upper airway tract obstruction during early childhood ( 1 to 3 years old ) such as teratoma , epidermoid , hemangioma , nasal glioma , meningocele , thyroglossal , or meningocele.4 16 17 because the management of these pathologies may be different , hp should be differentially diagnosed with these mentioned diseases . the differential diagnosis of naso - oropharyngeal teratomas should include an encephalocele or meningoencephalocele , as the treatment modalities are totally different . a computed tomography ( ct ) scan of the head is a reliable and sufficient way to diagnose the presence of an intracranial involvement of encephalocele or meningoencephalocele , and histopathologic examination is required to diagnose hamartomas , teratomas , and hps.16 17 a 3-year - old boy was referred to our department with a lifelong history of occasional pause of breathing while crying . otorhinolaryngologic examination revealed a pedunculated rubbery mass , originating from the left supratonsillar fossa and extending to nasopharynx ( fig . the mass moved from the nasopharynx to the orohypopharynx during swallowing and hence caused apnea . contrast - enhanced ct demonstrated a cystic mass in the nasopharynx , originating from the left lateral oropharyngeal wall , which had a lipoid density . following the opening the mouth with a mcivor retractor , the mass was located in the nasopharyngeal region . the mass was grabbed and the pedicle was dissected , which was then followed by a total removal using electrodiathermy ( fig . the upper airway was evaluated for edema , distortion , or hemorrhage particularly in the early postoperative period . the postoperative period was uneventful , and no recurrence was noted in the follow - up period of 3 months . a pedunculated mass originating from the supratonsillar fossa and posterior tonsillar fold . pilosebaceous units , hair follicles , fatty tissue , striated and smooth muscle fibers , and connective tissue bundles were observed in the stroma of the polypoid mass . hp symptoms depend on the size and location of the lesion.18 the most common symptoms of hp in the oronasopharynx may include rhinorrhea , recurrent cough , failure to gain weight , snoring , and sleep apnea during infancy2 3 4 ; however , the latter two symptoms , although they might be life - threatening , have rarely been reported in the clinical presentations.2 3 4 14 hps can also cause recurrent purulent otorrhea when the polyp originates from the eustachian tube and affects ventilation of middle ear.9 the major complaint of the case presented here was the occasional pausing of breath during crying and thus causing sleep apnea . this may be so serious and urgent that 25% of the patients might die before being able to surgically intervene.4 therefore , the naso - oropharyngeal hp should be removed surgically as soon as possible . as in this case , the respiratory problems completely recovered immediately following surgery , which was followed by the recovery of all other symptoms within the following couple of months . congenital nasopharyngeal teratomas contain a combination of mature and immature elements of all three germ layers.4 19 there is still a debate regarding the etiology of dermoids and hp . according to the totipotential rest theory , dcs ( dermoid cysts ) arise from totipotent cells derived from ectodermal and mesodermal germinal layers . the congenital inclusion theory proposes inclusion of germinal layers into deeper tissues of fusion lines that have failed to undergo complete closure during embryonic life , leading to trapping of epithelial debris . acquired implantation theory indicates traumatic events for the implantation of germinal derivatives into deeper tissues.8 20 hps may be best classified as choristomas . choristomas contain normal tissue in an anatomically foreign region.18 there is no family history of our case . although there have been some reports about inherited transmission in etiology , definite inheritance has not been clarified.8 20 21 hps are also not associated with congenital syndomes.18 malignant transformation has not been reported for oronasopharyngeal teratoma.22 in conclusion , in a pediatric age group with signs and symptoms of airway obstruction , oropharyngeal hps must also be included in differential diagnosis .

introduction dermoids frequently called hairy polyps and their nature have not been completely clarified . objectives to discuss the unusual presentation , symptoms , incidence , histology , and perioperative management of hairy polyps in the light of a case and current literature . resumed report a 3-year - old boy presented with intermittent respiratory distress since birth . oropharyngeal examination revealed a nasopharyngeal mass originating from the supratonsillar fossa . the mass was so mobile that it moved between the oropharynx and the nasopharynx during swallowing . the radiologic and pathologic examinations confirmed the mass as a hairy polyp . conclusion in a pediatric age group with airway obstruction , hairy polyps of the oropharyngeal region must also be included in the differential diagnosis .

henoch schnlein purpura ( hsp ) belongs to the group of nongranulomatous small vessel vasculitis . intussusception , gangrene of the bowel , bowel perforation , and massive hemorrhage are the most common gastrointestinal complications of hsp . a comprehensive search of pubmed , embase , and web of science was performed for all relevant papers published before july 1 , 2015 . because the full text of six articles was unavailable , only 13 cases described in 12 full - text articles were included in this study . then , we investigated the clinical features , treatments , and prognoses of hsp - related pancreatitis cases . the main clinical characteristics of the 13 patients ( six males , seven females ) are summarized in table 1 . the patients ranged in age from 3 to 70 years , half of cases were 520 years old . pancreatitis presented as the initial manifestation of hsp in eight cases . in addition , pancreatitis and typical purpura occurred at the same time in the two cases . from the onset of pancreatitis to the diagnosis of hsp , the time elapsed ranged from 1 day to 75 days , and 62.5% ( 5/8 ) of the patients began to have typical purpura within 7 days of the onset of pancreatitis . in addition to abdominal pain in all cases , seven patients presented with vomiting , one patient had hematemesis , one patient had hematochezia , and one patient had poor appetite . iu / l ) in all cases . abdominal computed tomography ( ct ) revealed pancreatic edema , ascites , and a wide range of intestinal wall edema in seven cases . a pancreatic cyst was found in one patient , it appeared over the course of 35 days and disappeared in 55 days ; however , five patients did not demonstrate any morphological changes in the pancreas . all patients experienced relief through treatment with fasting , gastrointestinal decompression , nutritional support , antiacid drug , glucocorticoid , and somatostatin . the abdominal pain relief time ( 144 days ) was noted in six cases . patient prognoses were described in three cases , and these patients were cured without recurrence through follow - up . main clinical characteristics of 12 previously reported cases of henoch - schnlein purpura - related pancreatitis hsp occurs about twice as often in boys as in girls , half of affected patients are younger than 10 years of age . however , our study suggested that hsp - related pancreatitis usually occurs in adolescent girls . the reason remains unclear ; it may be associated with the immune state of adolescent girls . compared with other types of pancreatitis , hsp - related pancreatitis has the following characteristics . in our study , before the onset of hsp - related pancreatitis , there was no prominent cause , such as biliary tract disease , overeating , drinking , hyperlipidemia , viral infection , or drugs . we speculate that hsp - related pancreatitis may be associated with small blood vessel thrombosis , vasculitis , and intimal thickening . although the clinical manifestation was relatively mild , acute pancreatitis may be the initial manifestation of hsp . hsp - related pancreatitis is diagnosed clinically but requires ct evaluation or ultrasound imaging to differentiate mild acute pancreatitis from severe necrotic pancreatitis . in our study , approximately 41.7% ( 5/12 ) of the patients with pancreatic morphology were normal , and only 41.7% of the patients presented with pancreas swelling . the imaging changes in hsp - related pancreatitis were atypical , and pancreatic necrosis or pseudocysts and other local complications were relatively rare . in our study , all patients had elevated serum amylase and urine amylase levels , with an increase of at least three times the upper limits in blood and urine , but the level was not positively correlated with the disease severity . some researchers believe that elevated amylase creatinine clearance and serum lipase levels are appropriate for the early diagnosis of pancreatitis . it has been reported that the measurement of plasma factor xiii could be useful for the early diagnosis of hsp , particularly when the typical purpura is preceded by abdominal pain . in addition to the conventional treatment for pancreatitis , more attention should be paid to hsp . in our study , the symptom of pancreatitis was improved after steroid treatment in all patients . somatostatin can inhibit gastric and pancreatic secretions , thereby reducing enzymatic activity ; it can also reduce capillary permeability , open the sphincter of oddi , and promote the excretion of pancreatic enzyme . after a diagnosis of hsp - related pancreatitis in addition to the symptomatic and supportive treatment , the use of corticosteroids to control hsp helps to alleviate hsp - related pancreatitis .

to summarize the experience of diagnosing and treating patients with henoch schnlein purpura ( hsp)-related pancreatitis , a systematic review of previously published cases was conducted . among 13 reported cases , there were six males and seven females whose age from 3 to 70 years . the clinical features of these patients indicated that acute pancreatitis could be the initial manifestation of hsp , the radiological change was atypical , and most cases were alleviated with steroidal treatment . good outcomes can be achieved in patients who are diagnosed early with hsp - related pancreatitis , and it is vital to begin timely treatment of hsp - related pancreatitis with corticosteroid .

vascular access is of vital importance for haemodialysis , and the radiocephalic arteriovenous fistula ( avf ) is the preferred access modality according to the k / doqi guidelines . this is primarily due to vascular stenosis , prompting the need for endovascular or surgical intervention for salvaging the fistula . we present a child with severe access stenosis and a remarkable salvage of his avf . the boy had esrd due to primary focal segmental glomerulosclerosis . at 4 years of age haemodialysis after 1 year , a radiocephalic fistula was created in his left wrist with a side - to - side anastomosis . haemodialysis was performed three times a week for 4 h using a single - needle technique with a blood flow of 250 ml / min , resulting in a single - pool kt / v between 1.7 and 2.1 . after 2 years , a gradual increase in the negative arterial pressure was noted during dialysis with increasing difficulty in maintaining adequate blood flow ( from 120 to 150 mmhg over 5 months ) . no abnormalities regarding pulse , bruit or thrill were noted during physical examination of the fistula . however , a fistulogram was performed and revealed 50% stenosis of the radial artery proximal to the fistula ( figure 1 ) . endovascular versus surgical treatment was considered but deemed difficult . in the mean time , a gradual decrease in the arterial pressure was noted during haemodialysis , returning to the initial values , and aforementioned problems subsided . fistulogram : catheter inserted into the cephalic vein and retrograde filling of contrast of the radiocephalic fistula . routine follow - up duplex sonography 1 year later , showed cessation of flow in the radial artery just proximal to the anastomosis and retrograde flow in the distal artery ( figure 2 ) . shunt flow was measured using an ultrasound dilution method ( transonic flow qc haemodialysis monitor ; ithaca , ny , usa ) and was 580 ml / min ( = 1250 ml / min/1.73 m ; increased risk for thrombosis : < 650 ml / min/1.73 m ) . physical examination revealed a normal palpable pulse over the anastomosis and radial artery and a normal thrill over the downstream veins . the left hand had an adequate colour , temperature and capillary refill , similar to the other hand . occlusion of the radial artery by applying pressure proximal to the anostomosis had no effect on pulse and thrill over the fistula . after occlusion of the ulnar artery , signs of blood flow through the fistula disappeared and his fingers demonstrated an inadequate capillary refill . occlusion of the radial artery distal to anastomosis had the same effect on the fistula , but a normal capillary refill of the hand was observed . ( b ) retrograde blood flow from the distal radial artery into the arteriovenous connection . we concluded that in our patient stenosis in the proximal radial artery initially compromised flow through the arteriovenous fistula . the decrease in pressure in the post - stenotic section gave way to a steal phenomenon , increasing retrograde distal radial artery flow via the palmar arch and ulnar artery . over time stenosis in the proximal artery progressed to total occlusion but collateral retrograde arterial supply via the arch . there were no complaints of limb ischaemia and his kt / v remained unremarkable . the palmar arch consists of two arterial connections between the ulnar and radial artery , the so - called deep and superficial arch . its main branches constitute the digital arteries delivering arterial blood to the fingers ; furthermore , the palmar arch is capable of maintaining blood supply when flow through one of both feeding arteries is compromised . collateral arterial flow via the palmar arch is common in patients with a radiocephalic fistula . . demonstrated retrograde flow in the distal radial artery in 23 out of 30 subjects 1 day after fistula construction . retrograde blood flow accounted for 26% of total flow through the fistula ( range 550% ) . a similar paper reported retrograde flow in the distal radial artery in all of 21 patients with a radiocephalic fistula . however , until now there was no report in the literature that solely collateral flow via the palmar arch could sustain haemodialysis . mc gill et al . reported collateral flow via the palmar arch in three adults following severe radial artery stenosis ; all had compromised urea kinetics , two underwent surgical revision , one declined and was put on nocturnal 8-h haemodialysis . proximal radial artery occlusion and the resulting increased retrograde blood flow via the palmar arch could cause ischaemic symptoms in the distal limb : a phenomenon called high - flow steal . steal can be defined as the enhanced arterial flow towards the low - resistance ateriovenous connection , compromising blood supply to the downstream limb . the associated ischaemia can cause symptoms such as pain , pallor , lower skin temperature , trophic lesions , diminished sensibility and loss of strength . it is mainly observed in elderly patients with vascular disease and diabetes mellitus and is in > 50% of cases related to arterial stenosis . steal is not often reported in children and this could be an indication of a low incidence in this age group . this in turn can be explained by their relatively healthy blood vessels that are probably better able to adept to growth and demand and are less challenged by degenerative processes such as atherosclerosis or calcifications . our paediatric patient was able to develop adequate flow via his palmar arch and sustain both dialysis and hand perfusion . to our knowledge this is the first description of a patient experiencing adequate haemodialysis treatment with complete arterial stenosis proximal to a radiocephalic fistula salvaged by collateral arterial supply via the palmar arch . we conclude that in children the palmar arch can be capable of sustaining sufficient blood flow for haemodialysis and the presence of retrograde flow in the distal radial artery should be taken into consideration in evaluating vascular access stenosis .

we present a child with mild problems during haemodialysis due to stenosis of the proximal radial artery of his radiocephalic fistula . however , his problems spontaneously subsided . follow - up sonography revealed arterial occlusion proximal to the fistula with retrograde flow in the distal segment . kt / v and shunt flow were normal . physical examination confirmed proximal radial artery occlusion with collateral blood supply via the palmar arch . there were no signs of steal . to our knowledge this is the first description of a patient experiencing adequate haemodialysis with arterial occlusion proximal to a radiocephalic fistula salvaged by collateral arterial supply via the palmar arch .

gender bias in different types of congenital defects is well know . a significant bias in sex ratio has been documented for congenital heart disease with several lesions occurring more frequently in males or in females.12 this difference may be related to differences in hormonal constitution . it has been proposed that foetal sex is partially determined by hormone levels of both parents around the time of conception has put forward35 but it is unclear whether such hormonal variations may also be responsible for sex - biasing of congenital anomalies . a literature search shows that transposition of the great arteries has been shown to have a gender bias ( table 1 ) but no explanation has been put forward in order to account for this observation . sex ratio of transposition of the great arteries in published reports in this study , we compare gender of patients with transposition with controls . in the general population , females tend to be smaller than male and in this study , we also attempt to relate gender ratios of patients with transposition with birth weight , maternal age and parental occupation . we evaluated 95 isolated livebirths with transposition of the great arteries cases referred to sicilian registry of congenital malformations from 1991 to 1998 , and compared these with a control group of 1000 consecutive newborns from the same geographic origin . all types of transposition of the great arteries were evaluated together and we excluded cases with other associated birth defects . we found a sex ratio ( male / female ) of 2.8 in transposition of the great arteries while the control group had a sex ratio of 1.04 . we did not find any statistical association between birth weights and gender bias in transposition of the great arteries and controls . stratification of our cases by gender and maternal age was not sigificant , with a mean maternal age of 29 years for both male and female cases of transposition of the great arteries ( p=1 ) . a statistically significant association both for maternal ( p=0.03 ) and paternal ( p=0.04 ) occupation related to agriculture was found for males with transposition , while none was found for affected females . moreover , in transposition of the great arteries , gender and birth weight are independent factors . hytten and leitch16 proposed that a high male sex ratio correlated with high maternal age , but james and rostron17 in a large sample of data found a decline in sex ratio of births at high maternal age . recently loffredo et al18 reported an association between transposition of the great arteries and maternal exposure to herbicides and rodenticides . . the aetiological agent / s may be chemicals used in agriculture that produce a hormonal disruptor effect . the results of the present study , despite the limitations attributable to the small number of sample and controls , suggest further studies on environmental agents and their possible teratogenic effect .

a significant bias in sex ratio has been documented for several congenital cardiac malformations . transposition of the great arteries has been associated with a such a bias but no explanation has been proposed for this bias . we evaluated 95 isolated livebirths with transposition of the great arteries cases referred to the sicilian registry of congenital malformations from 1991 to 1998 . we found a statistically significant male bias of 2.8 and this was significantly associated with both maternal and paternal occupational exposure to agricultural chemicals for male infants with transposition , but not for female infants . this study raises new questions about the possible role played by environmental chemicals in relationship to birth defects and to sex ratio imbalance .

the association of pain in the occipital region and ipsilateral cranial nerve xii ( cn xii ) paresis was consistent with the occipital condyle syndrome , first identified by greenberg et al . the occipital condyle syndrome has been associated with metastases to the base of the skull and primary head and neck tumors ( greenberg et al . , 1981 ; moris et al . , 1998 the largest case series ever reported about cn xii palsy indicated that almost half of these cases are due to malignancies ( keane , 1996 ) . our patient had many of the typical features of the occipital condyle syndrome ( capobianco et al . , 2002 ) including radiation of pain to the ipsilateral temporal area , improvement of symptoms by turning the head toward the painful side with worsening of pain by contralateral neck rotation . our patient had some atypical features including absence of neck stiffness or worsening pain by neck flexion . probably the most important syndromatic difference in this case is related to the etiology . to our knowledge , the occipital condyle syndrome has not been previously associated to non - neoplastic etiologies such as wd . wegener s disease is a clinicopathological syndrome characterized by extravascular granulomatous inflammation , granulomatous vasculitis of small size vessels , necrosis of upper and lower respiratory tracts , and pauci - immune glomerulonephritis ( deremee et al . , 1976 ; leavitt et al . , 1990 ) . recurrent middle ear involvement , as in our patient , has been well documented ( mccaffrey et al . neurological manifestations in wd include cerebral and subarachnoid hemorrhage , cerebral arterial and venous thrombosis , cranial and peripheral neuropathies ( primarily mononeuritis multiplex ) , diabetes insipidus , retinal vascular and optic nerve lesions , ocular myositis , orbital pseudotumor , and myopathy ( drachman , 1963 ; biller and grau , 2004 ; holle and gross , 2011 ) . a localized granulomatous involvement of the meninges , described as chronic granulomatous pachymeningitis , presents a very broad clinical spectrum including seizures , encephalopathy and , as in the case of our patient , headaches that only resolve with corticosteroids , meningismus , and cranial neuropathies ( anderson et al . , 1975 ; katrib et al . , setting , the most common cranial nerves affected are the optic nerve ( cn ii ) followed by the olfactory ( cn i ; nishino et al . , 1993 ) . a presentation with multiple cranial neuropathies , although uncommon , has been reported ( parker and sobel , 1988 ; fujikawa et al . , 2008 ) . csf analyses in these patients show lymphocytic pleocytosis with high protein concentration and normal glucose level ( reinhold - keller et al . , 2001 ) c - anca and anti pr3 are of great diagnostic value but do not reflect disease activity and should not be used to assess therapeutic response ( nowack et al . , 2009 ) . untreated patients with wd have a mean survival of 5 months and a 1-year mortality of 82% ( walton , 1958 ) . in 1993 , cyclophosphamide was the first immunosuppressant shown to reverse the meningeal disease ( weinberger et al . , 1993 ) . since then , corticosteroids and cyclophosphamide have been documented to achieve excellent control of disease activity ( de groot et al . , 2001 ) . modern immunosuppressants including infliximab and rituximab have been reported to successfully manage refractory cases of this condition ( mukhtyar and luqmani , 2005 ; sharma et al . , 2010 ) . the use of trimethoprim sulfamethoxazole has been described to reduce the incidence of relapses on patients with wd . these effect is maximum on patient in remission who presented with limited disease ( reinhold - keller et al . the authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest .

tumors or chronic inflammatory lesions of the occipital condyle may cause occipital pain associated with an ipsilateral hypoglossal nerve injury ( occipital condyle syndrome ) . we describe a young woman with recurrent otitis media and occipital condyle syndrome associated with a limited form of wegener s disease .

torsion of uterine adnexa is an important cause of acute abdominal pain reported in the literature.1 however , isolated torsion of fimbrial cysts has rarely been described as a cause for acute abdomen.23 we report a rare case of isolated torsion of fimbrial cysts leading to acute abdomen . a 22-yr - old female presented to the hospital with acute abdomen , amenorrhea for a half month and a half , and spotting on and off . the patient provided written consent to reproduce information or photographs . on physical examination , the patient s vital signs were found to be normal . laboratory investigations including hemoglobin , total leucocyte count , differential leucocyte count , and routine and microscopic examination of the urine were found to be normal . therefore , a serum beta human chorionic gonadotropin ( hcg ) test was done to confirm pregnancy . however , serum beta hcg levels were within normal range . the ultrasound report showed a tubo - ovarian mass in the right adnexa along with fluid in the pouch of douglas , suggestive of fimbrial cysts or ectopic pregnancy ( fig . on laprotomy , multiple cystic structures attached to the fimbriae were seen , which were twisted at their pedicle . many of them had ruptured leading to collection of about 200 ml of straw colored fluid . however , the fallopian tubes and ovaries were normal on both the sides , so a cystectomy was done . multiple cystic structures varying in size from 3 3 cm to 2 2 cm were seen . the cystic structures were filled with yellow colored fluid and were twisted at the pedicle ( fig . 2 ) . histopathology showed ciliated , columnar cells with underlying stroma and few chronic inflammatory cells , and a diagnosis of fimbrial cysts was made ( fig . paraovarian cysts represent approximately 10% of adnexal masses.4 they are more common in childbearing women.5 paratubal cysts arise from mllerian or wolffian structures and are common in adult females . these are hormone sensitive and are generally asymptomatic.6 malignant neoplasms arising from paratubal cysts are very rare.7 rarely , they can be associated with torsion of fallopian tubes.7 other complications include hemorrhage , rupture , and infection.3 paratubal cysts are difficult to diagnose preoperatively , and even transvaginal ultrasound in older women has detected only about 44% of paratubal cysts preoperatively.8 in only 1 of 15 patients is the diagnosis of fimbrial cyst suspected before surgery.4 thus , acute abodomen is a rare presentation of fimbrial cysts , which needs to be considered in a female of reproductive age group . timely diagnosis and excision of these cysts may prevent the rare complications of torsion and rupture that are associated with them .

we present a case of a 22-year - old female who presented with acute abdomen and amenorrhea . emergency laprotomy was done with a clinical diagnosis of ectopic pregnancy . on laprotomy , twisted fimbrial cysts were found . thus , although fimbrial cysts are rarely twisted , they should be considered as a cause of acute abdomen in a female of reproductive age group .

renal artery stenosis is one among a wide variety of uncommon , but potentially treatable causes of secondary hypertension . atherosclerotic renal artery disease is the most common cause of renal artery stenosis , accounting for 80% of renal arterial lesions . other causes such as fibromuscular dysplasia , aortic dissection , cholesterol emboli , renal arterial trauma , arteriovenous malformation , polyarteritis nodosa , and takayasu arteritis form the remaining 20% of renal arterial lesions . endovascular renal angioplasty and stenting is the current gold standard treatment for atherosclerotic and fibrous arterial lesions . re - stenosis is a less recognized long - term complication accounting for about 16% of cases ( range : 0 - 39% ) . those patients who undergo renal artery stenting , especially with a single functioning kidney , need periodic evaluation of flow across the stented artery . a 37 year old male presented with a history of hypertension for the last 3 years . the blood pressure was 220/120 mm of hg despite being on nifedipine 20 mg thrice daily , prazosin 5 mg twice daily and clonidine 0.1 mg 3 times daily . he was found to have severe renal insufficiency ( serum creatinine : 8.9 mg / dl ) , hypokalemia ( serum potassium 2.9 meq / l ) , bland urine sediment , contracted left kidney ( 6.3 cm 3.4 cm ) and normal sized right kidney ( 9.4 cm 4.0 cm ) with increased echotexture . the cardiac evaluation showed concentric left ventricular hypertrophy with good lv function ( ef : 71% ) and mild diastolic dysfunction . color doppler showed high resistive indices ( ri > 1.0 ) and peak systolic velocity ( > 180 cm / sec ) in both main renal arteries suggesting ostial stenosis . renal angioplasty and stenting was carried out on the right side with balloon expandable chromium cobalt stent . he received 5000 units of unfractionated heparin in the post procedure period and was started on anti - platelet agents . he continued to use anti - platelet agents ( aspirin 150 mg and clopidogrel 75 mg daily ) . when his follow - up visits with the local physician were reviewed it revealed a slowly rising blood pressure levels for which an angiotensin converting enzyme inhibitor ( ramipril 5 mg ) was added in the last 2 weeks . re - evaluation showed severe renal insufficiency ( serum creatinine 10.2 mg / dl ) . renal arteriogram showed near total stent occlusion with no collateral flow into the kidney [ figure 1 ] . stent - in - stent with well perfused kidney ramipril was discontinued and he was given two sessions of hemodialysis . a percutaneous balloon dilatation and stent - in - stent ( medtronic racer : 5.0 mm 18 mm , cobalt chromium ) was placed [ figures 2 and 3 ] . on the 2 day of stent placement his urine out put improved and serum creatinine started to decline and reached a nadir of 1.5 mg / dl on the 7 day . right renal artery stent with stent thrombosis right renal artery with balloon across the thrombotic segment in the existing stent early revascularization of a solitary functioning kidney not only improves blood pressure , but also the kidney function . acute and long - term complications of the endovascular interventions vary between institutions depending on the available expertise . although complications are not common , they can be catastrophic , including a theroembolic disease and aortic dissection . the potential benefits of early intervention can be seen either in the form of blood pressure control or salvage of renal function in ischemic nephropathy . large observational studies indicate either cure ( 12% ) or improvement ( 73% ) in hypertension rates . these studies also showed that either there is an improvement ( 30% ) or stabilization ( 42% ) of renal function after early intervention . our patient responded well to initial stent placement before the onset of ischemic nephropathy with prompt reduction of blood pressure and improvement of renal function , which were probably as a result of critical stenosis . re stenosis is an important and considerable draw back in the long - term . a meta - analysis by leertouwer et al . , showed the incidence of re stenosis as 16% over a follow - up period of 20 months . , indicates that in - stent stenosis should be treated with stent - in - stent placement favoring long - term patency rather than angioplasty alone . the likelihood of re stenosis depends on individual factors such as vessel diameter , compliance with anti - platelet agents , bilateral renal artery stenosis etc . elevated blood pressures in a hitherto controlled patient and worsening of renal function following the introduction of acei in our patient who has a solitary functioning kidney previously stented points toward the diagnosis of in - stent stenosis . following the establishment of diagnosis by renal arteriogram , our patient showed good clinical response in the form of improvement of blood pressure and renal function after balloon angioplasty and stent - in - stent placement . this report shows the importance of follow - up of renal stents with duplex ultrasound for evidence of restensosis .

renal artery stenosis is an important cause of secondary hypertension in young , which can be treated effectively with endovascular interventions including stenting . in - stent thrombosis is an infrequent complication and necessitates the need to follow - up these patients with renal artery stents . we present here a young individual who presented with in - stent thrombosis in a solitary functioning kidney and was successfully treated with stent - in - stent placement .

a 28-year - old man who was known to have fhi in the right eye was referred for secondary iol implantation . six years before referral , his right eye had undergone cataract surgery which was complicated by the capsular rupture and vitreous prolapse , for which the patient received complete anterior vitrectomy with removal of all capsular remnants . the patient was left aphakic and was prescribed with aphakic contact lens ; however , he developed contact lens intolerance over time . on presentation , his uncorrected visual acuity was 20/20 in the left eye and counting finger in the right eye which could be corrected to 20/20 with aphakic correction . slit - lamp examination of the right eye revealed diffuse fine keratic precipitates over the entire corneal endothelium and mild iris stromal atrophy with notable heterochromia . advantages and unknown risks of the surgery were thoroughly explained for the patient and he consented to have secondary iol implantation . under general anesthesia , an iris - claw iol ( artisan , ophtec , groningen , the netherlands ) was implanted in right eye through a limbal incision followed by a superior peripheral iridectomy . enclavation of the iol haptics was easily performed ; no intraoperative complication including hyphema was noted . the latter was prescribed as 0.1% betamethasone every 2 hours while awake for 1 week and then four times a day which was tapered within 6 weeks . postoperative follow - up examinations were performed at 1 , 2 , 3 , 5 , and 7 days , then weekly for 1 month , monthly for 3 months , and every 23 months thereafter until 1 year . postoperative course was uneventful with no significant anterior chamber inflammation ( more than 1 + cellular reaction ) or fibrin formation . on the first postoperative day , the examination showed 1 + cellular reaction and pigments in the anterior chamber which disappeared within 2 weeks . no subsequent exacerbation of the intraocular inflammation was observed during 12 months of postoperative follow - up ; therefore , no additional course of steroid was required . occasional cells in the anterior chamber were seen at some visits which were left untreated . one month after surgery , the patient achieved a best - corrected visual acuity of 20/20 in the right eye which was maintained for 12 months of follow - up . the iol remained stable with no subsequent iris atrophy at the enclavation sites , subluxation , or pupil ovalization . furthermore , the patient did not develop any anterior or posterior segment complication including glaucoma , vitreous inflammation , or clinical cystoid macular edema . secondary implantation of iris - claw artisan intraocular lens ( iol ) in an eye with fuchs heterochromic iridocyclitis . during 12 months of postoperative follow - up , no remarkable anterior chamber inflammation was observed in the right eye ( a ) and there were only few deposits on the iol surface ( b ) . the heterochromia in the involved eye is most obvious compared with the normal left eye ( c ) although secondary iol implantation in the ciliary sulcus has been reported to be safe in fhi , angle- and iris - supported iols have been feared because of the possible risk of postoperative uveitis , glaucoma , and hyphema . to the best of our knowledge , there has been no previous report of implantation of iris - claw artisan iols in eyes with fhi . even though our patient only received topical steroids , he did not show any significant postoperative inflammation or fibrinous reaction neither at the early postoperative period nor during 12 months of follow - up . therefore , it may suggest that in eyes with fhi the uveal irritation by iris - claw artisan iols is less than expected and the iol is more tolerable , even though recurrent or chronic anterior chamber inflammations has previously been reported in some eyes with these iols without preexisting uveitis . however , this lack of exacerbated postoperative inflammation in fhi may not be extrapolated to eyes with other more severe forms of uveitis . on the other hand , although fhi - associated iris atrophy in severe cases may theoretically make enclavation more difficult or compromise the long - term stability of an iris - claw iol , neither did develop in our case . therefore , it seems iris - claw iols , which have been shown to be safe in aphakic eyes without uveitis , may be an option in aphakic patients with fhi who do not have capsular support . however , studies on large number of patients with long - term follow up are required to determine the safety of these iols in eyes with uveitis including fhi .

implantation of iris - claw artisan intraocular lens ( iol ) is a surgical option for correction of aphakia ; however , these iols have not been used in eyes with uveitis including fuchs heterochromic iridocyclitis ( fhi ) due to possible risk of severe postoperative intraocular inflammation . in the case reported here , we secondarily implanted an artisan iol in a 28-year - old man with fhi who had aphakia with no capsular support due to a previous complicated cataract surgery . enclavation was easily performed and no intraoperative complication was noted . postoperative course was uneventful with no significant anterior chamber inflammation during 12 months of follow - up . although there were few deposits on the iol surface , the patient achieved a best - corrected visual acuity of 20/20 without developing glaucoma or other complications . therefore , artisan iol may be considered for correction of aphakia in patients with fhi . however , studies on large number of patients are required to evaluate safety of the procedure .

a 64-year - old man was referred to the ophthalmology department of pusan national university hospital for evaluation of tearing , nasal obstruction , and trifling epistaxis of the left side that had arisen 12 months earlier . ophthalmologic examination confirmed mild swelling in the left medial canthal area and obstruction in the area of the left lacrimal duct ( fig . 1a ) . visual acuity , intraocular pressure , ocular motility , and the anterior segment were within normal limits . the patient was referred to the otolaryngology department , and nasal endoscopies revealed the presence of a diffuse , smooth , and flush swelling mass in the inferior meatus . computed tomography ( ct ) revealed that the tumor extended from inferior meatus to the entire length of the left nasolacrimal duct and sac , with involvement of the medial wall of the maxillary sinus ( fig . we performed external dacryocystectomy to remove the lacrimal sac and proximal nasolacrimal duct and endoscopic inferior medial maxillectomy , including medial wall of maxillary sinus , inferior turbinate , and lateral wall of nasal cavity . microscopically , the tumor demonstrated a destructive , infiltrative growth pattern and was composed of numerous irregular small nests of pleomorphic oncocytic epithelial cells with abundant eosinophilic cytoplasm . adjuvant radiotherapy was recommended to the patient after surgery , but the patient refused further treatment . nine months after surgery , the patient complained of purulent discharge with tearing , swelling , and tenderness in the medial canthal area of the left eye . the orbital mass was removed through an inferior transconjunctival approach , and histological examination confirmed oncocytic carcinoma recurrence ( fig . he received computer controlled radiation therapy as 60 gy in 33 fractions over 8 weeks . although a neoplastic acquired nasolacrimal duct obstruction is an unusual condition , most of these tumors ( 75% to 90% ) are malignant . clinically , the presence of some specific signs , such as bloody reflux , epistaxis , failure of dacryocystorhinostomy , and a palpable mass above the medial canthal tendon , are helpful for diagnosing tumors of the lacrimal sac and nasolacrimal duct . a history of malignancy , especially of sinus or nasopharyngeal origin , also warrants further investigation into this unusual situation . however , a neoplasm should be considered in any patient presenting with nasolacrimal duct obstruction , and the most essential element in the early diagnosis and initiation of treatment is awareness of these rare clinical features . an oncocytic neoplasm is characterized by abundant , granular and eosinophilic cytoplasm , due to increased numbers of mitochondria , which are the most characteristic features of an oncocyte . diagnosis of malignancy in oncocytomas depends on the criteria defined by gray et al . destructive , infiltrating growth ; cellular pleomorphism with scattered mitoses ; lymphovascular or perineural invasion ; regional or distant metastasis . most of these carcinomas develop de novo , although malignant transformations of preexisting oncocytomas after long intervals have been reported . the majority of oncocytic tumors in the head and neck arise in the parotid gland , though they can arise in the minor salivary and seromucous glands . we assumed that the tumor in the current patient originated from a seromucous gland in the sinonasal tract , and enlarged and invaded into the nasolacrimal duct and sac . a malignant oncocytoma is a rare tumor of the head and neck ; approximately 60 cases of oncocytic carcinomas have been reported to date and the majority of these involved the major salivary gland . a nasal cavity oncocytic carcinoma is an extremely rare condition . to our knowledge , 14 cases of oncocytic carcinoma arising in the nasal cavity have been reported , and details of previous reports and the current case are given in table 1 . oncocytic carcinomas in the head and neck are a high grade neoplasm and appear aggressive from the onset : almost 85% of oncocytic carcinomas develop regional or distant metastases and 32% develop local recurrence . wide , complete local excision is the treatment of choice , and adjuvant radiation therapy should be strongly considered to prevent recurrence . due to the rarity of these tumors affecting nasal cavity and nasolacrimal duct , the pattern of clinical behavior and long - term follow - up is difficult to predict . however , postoperative radiation therapy has been demonstrated to reduce local recurrence rates in parotid gland malignancies and previous case reports . although tumors of the lacrimal sac and nasolacrimal duct are rare , physicians must consider the possibility of lacrimal sac tumors , nasolacrimal duct tumors , or other nasal cavity pathology , such as an oncocytic carcinoma , whenever symptoms are not typical .

here , we report an extremely rare case of acquired nasolacrimal duct obstruction caused by oncocytic carcinoma . a 64-year - old man presented to the hospital complaining of epiphora and left - side nasal obstruction . ophthalmic and otolaryngology examination revealed a left lacrimal duct obstruction caused by a mass in the left nasal cavity and lacrimal drainage system . the mass was removed and confirmed as an oncocytic carcinoma . nine months after surgery , without adjuvant radiotherapy , a left orbital mass was observed and the patient underwent reoperation . the mass proved to be recurrent oncocytic carcinoma . the patient underwent adjuvant radiotherapy to eradicate any residual tumor and the patient remains tumor - free one year post - radiotherapy .

about 86000 deaths related to drowning had been reported in india ( in 2000 ) , with a mortality rate of 8.5% . the lack of sufficient education in drowning prevention and resuscitation has resulted in thousands of unnecessary deaths . here we report a case of secondary pneumomediastinum , bilateral pneumothorax and acute respiratory distress syndrome ( ards ) in a male with near drowning that recovered with conservative therapy . this 24-year - old college student was brought to the emergency with history of drowning in a fresh water pond . he had been waist - deep in the pond drinking alcohol , while his friends who were swimming had been unaware when he had slipped into the water . he was immediately rescued when his absence was noted and taken to a nearby hospital within thirty minutes . en route , they had noticed that he was barely conscious , able to follow a few commands and had shallow breathing . he was nasally intubated and brought to our institution about three hours later . on examination he had tachycardia 130/min , tachypnea 26/min and systolic blood pressure of 60 mm hg . bilateral air entry was reduced albeit with crackles basally , and he was awake , obeying commands with no focal neurological deficits . his investigations were : hemoglobin 21.4 g / dl , haematocrit 60 , urea 10.7 mmol / l , creatinine 176.6 mol / l , potassium 2.6 meq / l , metabolic acidosis , sinus tachycardia on electrocardiogram , and bilateral lung infiltrates , bilateral pneumothorax and pneumomediastinum on chest radiography ( figure 1a ) . his central venous pressure was 6 cm of saline following which fluids and inotropes ( dopamine and dobutamine ) were administered . the patient also had upper gastrointestinal ( gi ) bleeding following which he was transfused two units of red blood cells ( rbcs ) . on the second day subcutaneous emphysema over neck was noticed and a computed tomography of the chest was performed to rule out chest trauma . echocardiography was normal ; a left sided intercostal chest tube was placed and removed on day 4 of admission . inotropes were tapered and stopped on the third day . his renal function improved with fluid management and he was discharged on day 10 with the presence of a small pneumothorax on the right side . on a follow up visit two weeks later his chest x - ray ( cxr ) was normal and he remained symptom free . figure 1a ) chest x - ray ( cxr ) at admission showing bilateral pneumothorax , pneumomediastinum ; b ) computed tomography ( ct ) thorax revealing pneumothorax , pneumomediastinum , crazy pavement pattern of collapsed lung segments ; c ) cxr with bilateral pneumothorax and pneumomediastinum reduced markedly following chest tube insertion . drowning is suffocation arising from submersion in a liquid medium , generally water and near drowning is at least temporary survival following drowning that enables a patient to obtain health care . near drowning most such incidents are under - reported , although up to half a million deaths occur worldwide due to drowning . ninety percent of near drowning incidents are wet drowning wherein small to large amounts of fluid are aspirated into the lungs along with temporary laryngospasm . wet drowning with fresh or sea water generally disrupts surfactant lining and hence alveolar collapse , adult respiratory distress syndrome , hypoxemia and cerebral hypoxia occur . drug intoxications , arrhythmias , depression , epilepsy , head injuries and accidental falls are common causes of near drowning . about 2025% of young adult ( especially males ) deaths associated with water recreation activities are seen with alcohol use as in our patient . serum sodium may be altered depending on whether fresh or seawater was aspirated , but our patient 's sodium was normal . hemoconcentration is generally seen in sea water drowning , but our patient who drowned in a pond presented with elevated hematocrit and low central venous pressure ( cvp ) . his upper gi bleeding probably contributed towards his contracted intravascular volume and shock . computed tomography ( ct ) findings of near drowning including pneumomediastinum , crazy pavement appearance , groundglassing as reported in a study pneumomediastinum is presence of free air in the mediastinum , that originates from the alveolar space or airways . pneumomediastinum had been reported initially in 1819 and has been described in the setting of labor and child birth , reactive airway diseases , surgeries to the neck and tonsils , blunt chest trauma and illicit drug inhalation . apart from barotrauma , coughing and breath holding during the drowning process that possibly occurred in our patient , can also lead to pneumomediastinum due to elevation of intraalveolar pressures and subsequent alveolar rupture . most cases of pneumomediastinum can be managed conservatively . due to presence of pneumothorax and haemodynamic instability , factors governing prognosis in drowning incidents include time submerged , timely and quality resuscitation , water temperature , depth of water and nature of the water . complications arising from submersion include aspiration , chest trauma , arrhythmias , pulmonary edema , dehydration , dyselectrolytemia , renal failure and rhabdomyolysis . ct chest revealed no trauma , while hypokalemia , renal failure and pulmonary edema were seen in our patient . good prognostic indicators in the emergency include gcs>5/15 , short submersion time and spontaneous respiration and cardiac activity all of which were observed in our patient . in conclusion , we report a case of pneumomediastinum in near drowning that followed slipping into the water in an intoxicated state which has not been previously reported . presence of shock , upper gi bleeding , pneumomediastinum , pneumothorax were not poor prognostic indicators when the sensorium was normal and could be treated conservatively .

we report pneumomediastinum , bilateral pneumothorax and acute respiratory distress syndrome in a victim of near drowning who was intoxicated and did not have thoracic or neck trauma . chest radiograph revealed the above findings , later confirmed by computed tomography . he was in shock and also had gastrointestinal ( gi ) bleeding and renal dysfunction . with adequate resuscitative measures including fluids , blood transfusions , intercostal tube drainage and mechanical ventilation he made a complete recovery . good prognostic indicators in near drowning patients include higher glasgow coma scale , short submersion time and quick resuscitative measures even in the presence of serious cardiorespiratory or hemodynamic compromise .

therefore , thoracic radiculopathy due to degenerative root canal stenosis is a rare entity . here , we present a patient with right t2 nerve root canal stenosis with pain in the right axilla and posteromedial arm . he had undergone c56 anterior fusion for cervical spondylosis 5 years prior . a 60-year - old man presented with 3 months of progressive pain in the right axilla and posteromedial upper extremity . examination revealed no sensory disturbance , no abnormal tendon - jerk reflexes and progression of muscle weakness . roentgenograms showed scoliosis with a right cervicothoracic curvature with a cobb angle of 17 degrees before his anterior fusion ( fig . in addition , computed tomography ( ct ) scanning revealed bone spurs of the c67 facets ( fig . magnetic resonance ( mr ) imaging showed a decrease in intervertebral height , but the nerve root canal was preserved at the c67 level . ct images also showed osteophytes of the superior costal facet and articular process at the t23 level ( fig . ( a ) cervical frontal view before anterior fusion . ( b ) cervical frontal view 5 years after anterior fusion . ( a ) thoracic sagittal views of ct image before cervical anterior fusion at right t2 nerve root canals . preoperative multi - planar ct images ( b d ) and reconstructed 3d images showing right c67 and t23 nerve root canal stenosis caused by bone spurs of facets and vertebral bodies ( f and h ) . mr imaging of protruded disks at the t23 level ( e ) . postoperative ct shows the decompression of the right t23 thoracic nerve root canal ( g ) . ( a ) cervical frontal view before anterior fusion . ( b ) cervical frontal view 5 years after anterior fusion . ( a ) thoracic sagittal views of ct image before cervical anterior fusion at right t2 nerve root canals . preoperative multi - planar ct images ( b d ) and reconstructed 3d images showing right c67 and t23 nerve root canal stenosis caused by bone spurs of facets and vertebral bodies ( f and h ) . mr imaging of protruded disks at the t23 level ( e ) . postoperative ct shows the decompression of the right t23 thoracic nerve root canal ( g ) . the width and height of the nerve root canal on the right measured 6.4 mm and 4.2 mm at c7 and 5.2 mm and 5.1 mm at t2 , respectively . the right c7 and t2 nerve root canals were narrow compared to left side ( left c7 width 8.0 mm , height 8.2 mm ; left t2 width 9.9 mm , height 10.5 mm ) . from c5t4 , mean width was 9.4 1.8 mm and mean height was 9.2 2.4 mm . before anterior cervical fusion , the width and height of the nerve root canal on the right at t2 had been 7.4 mm and 5.0 mm and on the left had been 8.6 mm and 12.2 mm , respectively ( fig . these findings indicate that the nerve root canals on the right at c7 and t2 were degenerative . blood tests , electrocardiogram , electromyogram , somatosensory evoked potential ( ssep ) and head mr imaging were all normal . this patient displayed no c7 radiculopathy and had neither delay nor disappearance of ssep in the median nerve distribution . the causes of thoracic radiculopathy include bulging disc , herniated disc , bone spur , spinal canal stenosis or foraminal stenosis . in this patient , osteophytes of the intervertebral joint and the superior costal facet at t23 , and a bulging disc on the right at t23 caused right t2 foraminal stenosis . asymmetric bone degeneration , such as disc - endplate degeneration , plays a biomechanical role in the progression of scoliosis . it has been commonly suggested that a spine with scoliosis bears a greater load on the concave side and that this asymmetrical loading causes asymmetrical growth and progression of the deformity , and has been also suggested that the risk of curve progression is cobb angle > 30 degrees , not simply advancing age . adjacent - segment disease after cervical spine arthrodesis sometimes occurs at the high thoracic levels , generally occurring in the segment adjacent to the spinal fusion , not in a remote segment . in this case after the cervical anterior fusion , cervicothoracic scoliosis , osteophytes and disc degeneration had been exacerbated . these findings suggest that the mechanical stress after c56 anterior fusion burdened not only the adjacent c67 segment but also the right t23 segment at the apex of the scoliotic curve on the concave side . degenerative changes occurred at t23 , which was likely subjected to mechanical stress under asymmetrical loading for 5 years after surgery , resulting in asymmetrical growth and progression of deformity . we considered cervical angina in this patient s differential diagnosis due to adjacent - segment degeneration after c56 anterior fusion . walter et al . described the clinical characteristics of cervical angina : a history of cervical radiculopathy , neck pain or pain induced by cervical range of motion . in this case , the t2 nerve roots innervate the upper chest , axilla and posteromedial upper extremity areas , which correlated anatomically with symptoms in this case . however , a c7 nerve root block could have been performed prior to surgery to further clarify the differential diagnosis . t2 radiculopathy is rare but should be considered in the differential diagnosis of chest pain , when cardiac ischemia or cervical angina have been ruled out . surgeons should pay attention not only to adjacent - segment disease but also to segmental degeneration at the apex of a scoliotic deformity after cervical anterior fusion .

thoracic radiculopathy is a rare entity . symptomatic adjacent - segment disease after anterior cervical fusion occurs commonly in the lower cervical spine segment . we describe the clinical presentation and treatment of t2 radiculopathy after c56 anterior fusion . a 60-year - old man presented with the right axillary pain for 3 months . he had undergone c56 anterior fusion for cervical spondylosis 5 years prior . computed tomography ( ct ) and magnetic resonance images showed t23 degenerative disease . c56 anterior fusion exacerbated the t23 segment involved in the patient s scoliotic deformity . after 2 months of conservative treatment , we decompressed the t2 foramen via t23 hemilaminectomy and partial facet resection . after the surgery , his symptoms disappeared . t2 radiculopathy is rare but should be considered in the differential diagnosis of chest pain . surgeons should pay attention not only to adjacent - segment disease but also to segmental degeneration at the apex of a scoliotic deformity after cervical anterior fusion .

coronoid hyperplasia is a rare condition which is macroscopically characterized by an increase in the dimensions of the coronoid process , resulting from an abnormal bony elongation of histologically normal bone . the most obvious clinical feature of this condition is reduced mouth opening associated with facial asymmetry . in addition to restricted opening , protrusion and lateral excursions may also be affected . the cause of restricted mandibular movement is widely accepted to be an impingement of elongated coronoid process to the posterior aspect of the zygomatic bone , or to the inside of the zygomatic arch , at the mouth opening . unilateral coronoid hyperplasia is usually due to a trauma or a pathologic condition and is associated with facial asymmetry , more frequently seen in women with histologic chondromatous or neoplastic changes most similar to ostechondroma . however , others consider enlargement of the coronoid process to be a hyperplastic process rather than a true neoplasm . the primary clinical feature of coronoid enlargement is limitation of opening . shira and lister reported the appearance of bony enlargement of the zygoma on the affected side as demonstrated on a submentovertex radiograph . a thorough clinical history should include information about the onset and progression of pain and other subjective symptoms . in the case of coronoid hyperplasia , ct also allows surgical planning due to its accuracy to detect coronoid process volume and morphology . the following is a report of a case of unilateral coronoid enlargement with restricted opening as well as an obvious zygomatic asymmetry . a 15-year - old male with no medical history of interest reported to the out - patient department of government college of dentistry , indore , with the chief complaint of facial asymmetry [ figure 1 ] . preoperative clinical photograph of the patient , revealing facial asymmetry the patient mentioned asymptomatic difficulty in opening his mouth . on examination , he was found to have a reduced mouth opening , with limited lateral and protrusive jaw movements and no hypertrophy of the masseter muscles [ figure 2 ] . reduced mouth opening of the patient a ct scan was carried out with three - dimensional reconstruction ( 3d ct ) , showing unilateral hyperplasia of the coronoid process and contact between said process and the zygomatic arch , limiting mouth opening- movement [ figures 3 and 4 ] . 3d ct reconstruction of face revealing facial asymmetry on left side of face large anteroposterior extent of left mandibular coronoid process as seen in three - dimensional ct reconstruction image due to massive anteroposterior extent of coronoid , transzygomatic approach was considered . a hemicoronal incision ending in a preauricular extension up to the lobule of the ear was made through the skin , subcutaneous tissue , and galea . in the temporal region , this incision was up to superficial layer of the temporal fascia . at the root of the zygomatic arch , hemicoronal incision was taken and zygomatic arch was exposed two plates were adapted , bridging the osteotomy site on the zygomatic arch . the arch was osteotomized , temporalis muscle was stripped off the coronoid , and the process exposed adequately . an osteotomy cut was taken on the coronoid and the enlarged coronoid stump was removed [ figure 6 ] . enlarged coronoid process mass removed zygomatic arch was repositioned in its proper anatomic position and fixation was performed with titanium plates [ figure 7 ] . repositioned zygomatic arch in its proper anatomic position and fixation done with titanium plates the wound was then closed in layers . the patient reported after 6 months with adequate mouth opening and marked reduction in facial asymmetry [ figures 8 and 9 ] . marked reduction in facial asymmetry seen after 6 months postoperatively increase in mouth opening appreciated 6 months postoperatively elongation of the coronoid process of the mandible was described for the first time in 1853 by von langenbeckin . in 1899 , jacob described a synovial joint formation between an elongated coronoid process and the homolateral zygomatic bone . several approaches are advised by different authors for performing coronoidectomy , such as intraoral approach , submandibular approach , and coronal approach . since the coronoid was very large anteroposteriorly , we planned a transzygomatic approach . it can be difficult to establish the best time to administer treatment in infants or preadolescents , although most authors agree that except in patients with very severe limitation of the mouth opening , it is best to perform the operation once the growth process has finished in order to avoid recurrence , deformity , or even restricted movement . active physiotherapy is to be commenced immediately after surgery and continued for at least 6 months for satisfactory results . postoperative cases of limitation of mouth opening caused by fibrosis , secondary to incorrect reorganization of a hematoma at the site of operation , and even recurrence in the growth of the coronoid process have been described . coronoid process hyperplasia as one the causes of mandibular hypomobility is largely underdiagnosed as it is a very rare entity , but a thorough clinical and radiological examination can help to rationalize the line of management and the ultimate clinical outcome . in addition to limited mouth opening , facial asymmetry was also a clinical finding . in case of massive anteroposterior extension of the coronoid , coronoidectomy with transzygomatic approach followed by active immediate physiotherapy

coronoid hyperplasia is a rare disorder , which effectively is a mechanical problem associated with limited mouth opening . in some cases , enlarged coronoid pushes the zygoma forward causing facial asymmetry . this article looks at the case of a 15-year - old boy reporting in opd of government college of dentistry , indore , with chief complaint of facial asymmetry . transzygomatic approach was taken to get proper access to the enlarged coronoid . coronoidectomy was performed which significantly reduced facial asymmetry . postoperative healing was uneventful and recurrence was not reported .

it has four types based on its content ; trichobezoar consists of hair , phytobezoar comprises undigested fruit and vegetable fibers , lactobezoar has milk and pharmacobezoar contains medications . the main cause of trichobezoar formation is the resistance of human hair to peristalsis and digestion . the continuous ingestion of hair together with food particles increases its volume producing symptoms such as abdominal pain , abdominal distension and vomiting . untreated cases may result in serious complications like gastric erosion and ulceration , gastric and small bowel perforation , gastric outlet obstruction , pancreatitis , obstructive jaundice , protein - losing enteropathy , intussusception and death . herein we aimed to present an interesting case of this rare entity diagnosed with sonography , computed tomography and endoscopy and subsequently treated with laparotomy . a 18 years old , unmarried female presented with large and hard upper abdominal mass . she complained from dull abdominal pain , anorexia and weight loss for last 4 years . her previous medical and surgical records were insignificant . on physical examination the patient looked pale with lean body mass . a giant , firm and hard mass was palpated in the epigastric and right hypochondriac regions . usg demonstrated a large area of abnormal thick echogenicity with dirty posterior shadowing extending from the left sub diaphragmatic area to the right sub hepatic region ( fig . , the exact size , internal texture and extent of the mass lesion could not be assessed with usg . the patient subsequently underwent contrast enhanced abdominal computed tomography ( ct ) examination with 128 slice siemens scanner . the ct images revealed a huge , well defined , multi - layered , heterogeneous , solid appearing , non - enhancing mass in the gastric lumen extending from the gastric fundus to the pyloric canal . 2b ) the large mass lesion measured 27 10 15 cm in size . review of the bony skeleton revealed incidental finding of left sided untreated severe developmental dysplasia of the hip joint . endoscopy revealed a collection of large black and hard hair ball occupying entire lumen of stomach from fundus up to pylorus . the scope passed with difficulty in the space between the hair ball and stomach to the pyloric region ( fig . endoscopy confirmed the absence of trichobezoar s extension into duodenum through pyloric canal ( fig . an attempt was made to remove the huge mass , but due to its large size and hard nature , we failed to excise and remove it . anterior wall of the stomach was incised parallel to the greater curvature nearly from fundus to pyloric area . the abdominal incision was closed in two layers with 1.0 absorbable suture and skin was closed with stapler . the postoperative course was uneventful and the patient was discharged on 8th post - operative day in stable condition . the patient 's parents were advised a psychiatric consultation for patient to avoid recurrence of her condition . swain reported human trichobezoars in a post mortem case for the first time in 1854 and the first surgical removal was performed by schonbern in 1883 . debakey and oshner revealed that 90% of trichobezoars occur in females and 80% of these cases occur before 30 years of age . trichobezoars are predominantly found in young females with psychiatric disorders however they can also be found in postsurgical cases ; post - partial gastrectomy , post - vagotomy , patients with diabetes mellitus related gastroparesis , guillainebarre s syndrome , myotonic dystrophy , hypothyroidism and cretinism . trichobezoars are most commonly found in the stomach but in approximately 10 percent of cases they extend into small bowel and even into colon , . as hair is indigestible in gastrointestinal tract , it accumulates within the alimentary tract mostly in the stomach . due to the denaturation of proteins in acidic gastric juice , the ingested hairs become black colored irrespective of their original color . the hair together with ingested food and trapped air increases the volume of the mass and gives the mottled appearance to the trichobezoar visible on ct . the mass may enlarge and cause gastric outlet obstruction or it may extend distally into the bowel loops . extension of the bezoar from the stomach into the jejunum or further into bowel is referred to as rapunzel syndrome . more than 80% of the patients with trichobezoars present with palpable abdominal mass . approximately 80% of patients have epigastric discomfort , up to 70% complain of abdominal pain , 65% complain of nausea and vomiting , 38% have asthenia with weight loss and 33% of patients have intestinal transit problems like diarrhea or constipation . untreated cases can result in harmful complications such as upper gastrointestinal hemorrhage , gastric or duodenal perforation , peritonitis , sub - phrenic abscess , digestive fistula , acute pancreatitis and obstructive jaundice . usg used as the first line imaging modality for the evaluation of intra - abdominal masses provides limited information regarding trichobezoar . this is because of high echogenicity of hair and multiple acoustic reflections of trapped air bubbles . upper gastrointestinal series may show a large filling defect in the stomach , occasionally extending into the small bowel . it can depict an intraluminal , well - demarcated , heterogeneous , non - enhancing mass with mottled appearance due to the entrapped air among the hair ball . no specific advantage of magnetic resonance imaging over computed tomography is described in the literature in detection of trichobezoar . upper gastrointestinal endoscopy can not only visualize the mass , but also determine its extension beyond pylorus ( as per our case ) and in suspicious cases , sample of the mass can be also be taken . once diagnosed , trichobezoar needs immediate surgery as it can ensue in a number of harmful events . the treatment approaches include endoscopic removal ( which may involve fragmentation using electrohydraulic lithotripsy , tripod forceps ) , chemical dissolution , laparoscopic removal or laparotomy depending on the size and site of trichobezoar . what leads to recurrences of this condition is the negligence paid to the major triggering factor which is the psychiatric condition of the patients . prognosis of trichobezoars is quite good only if psychiatric follow - up is strictly maintained , , therefore psychiatric follow - up should be made a compulsory part of the management . trichobezoars should be suspected in young females with long standing upper abdominal masses ; as the possibility of malignancy is not very common in this age group . while ultrasound is inconclusive , trichobezoar can be accurately diagnosed with computed tomography . authors present the diagnostic work up and laparotomy approach to a huge trichobezoar in an 18 years old girl . the article has got approval of exemption from ethical review committee of french medical institute for children , as case reports are exempted from ethical review according to this institute s policy . written informed consent was obtained from the patient for publication of this case report and accompanying images . a copy of the written consent is available for review by the editor - in - chief of this journal on request . dr hidayatullah hamidi : the main concept of paper , providing usg and ct images and communication between authors .

highlightstrichobezoar is a rare clinical entity in which a ball of hair amasses within the alimentary tract , mainly in stomach.trichobezoars should be suspected in young females with long standing upper abdominal masses ; as the possibility of malignancy is not very common in this age group.while ultrasound is inconclusive , trichobezoar can be accurately diagnosed with computed tomography.convenient approach of treatment should be selected depending on the size and site of trichobezoars followed by psychiatric consultations in order to prevent its relapses .

science and technology have its worth due to continuous evolution of new concepts or advanced modification of existing knowledge throughout the planet . the worth has its establishment due to the dependency variable of humans that exists in today s era . majority of people are dependent on science and technology for integration of their routine activities . one of the branches of technology that has gained maximum popularity in recent times is information technology ( it ) . the installation of such a founding was into place by advent of telecommunications , a branch of it . over the period of years , with due progress and evolution such a device is carrying the property of being compact , easy to store and use , serving purpose of communication and possessing all properties that a stable unit can have , has made its popularity reach peak . cellular phones , what we understand is become a part of everyone s life without which there may be chances that a person s daily activity will be hampered . among the population , moreover , their dependency is justifiable due to their noble profession that requires immediate access in cases of emergencies . within the domains in hcps , dentistry is accepted as an operative field by what the dental surgeon needs infection control to maximum since routine involvement in procedures . due to their procedural participation , they act in similarity to surgeons and hence correspond to call as a dental surgeon . the access to cellular phones during the procedures in addition , there is available literature that speaks about mobile phones as a source of infection1,2 and its risk of contamination with infection transmission if protocols are violated.3 thus , these phones can be regarded as a habitat for microorganisms to breed since its basis to be a transporter for nosocomial infection amongst other professionals , patients , and community . however , the literature available speaks of hospital based environment and not a climate describing clinical dentistry . very few studies express its relevance in clinical dental surroundings , especially in dental school by what consideration was given only to dental faculty and trainees , specified as chief operator.4 however , dental nurses also form a significant group of hcps in a dental environment , whereby their involvement can not be a mark of ignore . thus , dental nurses mobile phones also need microbiological investigation to evaluate its entanglement with the risk that operative dental professionals carry as notified . hence , the current study deals with the evaluation of cellular phones for potential risk of nosocomial infection among dental operators and auxiliary staff in a dental school . of the available faculty , students and interns termed as dental operators in comprehension ; 50 individuals were randomly selected from the available list . a total of 50 dental auxiliary staff - dental nurses were also selected on randomization as above mentioned . each participant s mobile phone was first cleaned with 70% isopropyl alcohol swab . following the cleansing protocol , the mobile phones were then swabbed aseptically by rotating damp cotton swabs with sterile normal saline over three sites where hands came into contact with the phone being the lateral ends and keypad ( figure 1 ) . cultures were done on sheep blood agar and mcconkey s agar plates and then incubated at 37c for 48 h ( figure 2 ) . all plates were examined for visible growth ( figure 3 ) . based on colonial morphology , gram - stain , pigmentation and different biochemical reactions , sabouraud dextrose agar media was used for fungi species . swab streaking on agar plates . the collected data was analyzed using spss ( statistical package for social sciences ) version 17.0 ( ibm statistics , chicago , illinois , usa ) . 50% of dental professionals had shown active bacterial and fungal growth in which 35% ( n=35 ) were dental operators and 15 % ( n=15 ) were dental nurses . 95% gram - positive organisms , 2% gram - negative organisms , and 3% fungi were identified growths on cellular phones . thus , the predominant of all growths was gram - positive organisms . among the gram - positive organism , the traces of escherichia coli and aspergillus species were also noticed ( table 1 ) . it was presumed in the study that the mobile phones would carry some bacterial and fungal species and hence was thoroughly decontaminated with an alcohol swab . also after thorough decontamination this is in differing to the study done by singh et al.4 whereby following decontamination the bacterial load was significantly reduced to 87% . cellular phones of dental operators have shown maximum bacterial and fungal growth in comparison to dental nurses . the effect of notification can be rationalized with the direct involvement of operators when compared to dental nurses in treatment protocol . however , there was positive growth on phones of dental nurses that can not be ignored . it has the capacity to the extent even at the community level whereby the patients , professionals and other members of population interact . although , s. epidermis was found to be a predominant gram - positive bacteria , but is usually nonpathogenic as compared to s. aureus reported second in the investigation which can be both community and hospital acquired with high potential for transmission and infection . however , s. epidermis is also a matter of concern in a certain condition where the patient is immune - compromised.5 s. aureus values are in concurrence to the values seen in the study by singh s et al.4 this study also evaluated the fungal growth that was although in traces , but present with marginal excess when compared to gram - negative bacteria . however , the existence of gram - negative bacteria and fungi seems to be insignificant as compared to the gram - positive bacterial growth determined . thus , it can be concluded that the cellular phones of dental operators as compared to auxiliaries can act as a potential source of nosocomial infection . the investigators at this moment recommend implementing few standard infection control protocols before and after the procedure to establish infection free area relevant to the cellular phones . the protocols may include the use of disinfection barrier system or regular cleansing with an effective disinfectant . further randomized control trials are recommended on the subject with multiple institutions and clinical settings to have an effective picture and conclusion over the concern .

background : this study evaluates cellular phones for potential risk of nosocomial infection amongst dental operators and auxiliary staff in a dental school.materials and methods : each participant s mobile phone was first cleaned with 70% isopropyl alcohol swab . following the cleansing protocol , the partakers were asked to make a short phone call . the mobile phones were then washed aseptically by rotating damp cotton swabs with sterile normal saline . bacterial growth was identified on sheep blood agar and mcconkey s agar plates . sabouraud dextrose agar media was used for fungi species . descriptive statistics was established with the data statistically explored with spss version 17.0.results:about 50% of dental professionals had shown active bacterial and fungal growth in which 35% ( n=35 ) were dental operators and 15% ( n=15 ) were dental nurses . 53% gram - positive organisms , 2% gram - negative organisms , and 3% fungi were identified growths on cellular phones.conclusion:thus , it can be concluded that the cellular phones of dental operators as compared to auxiliaries can act as a potential source of nosocomial infection .

the proband , a 12-year - old caucasian male , was referred to the congenital cataract section of the federal university of sao paulo , brazil , complaining of progressive loss of vision . slit - lamp examination revealed a total cataract in the right eye , and a nuclear and posterior subcapsular cataract in the left eye . goldmann tonometry was 11 mmhg and 10 mmhg , respectively , in the right and left eyes . fundus examination of the left eye showed a myopic fundus with vitreous detachment and myopic macular scarring . vitreous opacities resembling the presence of posterior fetal vasculature were detected in the right eye ( figure 1 ) . the axial length was 27.8 mm and 27.5 mm , respectively , in the right and left eyes . a prior familial genetic study had been performed at another institution before the referral of the proband to our service . the familial genetic diagnosis was knobloch syndrome type i. the parents and one proband s sister had a normal eye examination without systemic features . the proband , as part of the present examination , also underwent a genetic study , which disclosed the same genetic diagnosis ( knobloch syndrome type i ) . phacoemulsification in the right eye was performed under general anesthesia , given the patient s age . there was no vitreous prolapse and it was possible to implant an intraocular lens within the capsular bag . at one - week follow - up , slit - lamp examination showed an inflammatory reaction in the anterior vitreous and anterior chamber , despite use of topical and systemic steroids . two months after surgery , visual acuity had improved to 20/200 in the right eye , and the patient switched to a left exotropia . a progressive posterior capsular opacity was detected in the visual axis , and a neodymium yag laser capsulotomy was performed in the right eye . one year after surgery , dislocation of the intraocular lens was observed by slit - lamp examination . ultrasound biomicroscopy showed a displaced intraocular lens behind the ciliary body at 78 clock hours . the intraocular lens haptic was dislocated to the inferior temporal quadrant ( figure 2 ) . a scleral intraocular lens fixation and anterior vitrectomy were performed to reposition the intraocular lens . one year later , a phacoemulsification was performed in the patient s left eye due to considerable progression of the pre - existing lens opacity to a total cataract . a peroperative posterior capsular rupture occurred , as had previously happened during the patient s right eye surgery . at this time , there was no capsular support to perform a safe intraocular lens implantation , and the left eye was left aphakic . visual acuity with best corrected refraction the visual axis remained clear in both eyes , with final best corrected visual acuity of 20/200 , leading to a better quality of life for the patient . this case report describes the outcomes of cataract surgery in a patient with knobloch syndrome type i , an autosomal recessive disease affecting collagen xviii . it was first described by knobloch and layer in 1971.1 the pattern of inheritance is related to consanguinity . there are ocular and systemic clinical features.2,3 ocular abnormalities include high myopia , vitreoretinal degeneration and opacities , retinal detachment , macular abnormalities , and cataract.4 ocular abnormalities are present in all cases reported in the literature , with great variability of clinical manifestations , due to the large number of mutations that occur in the gene . there are reports of cases of knobloch syndrome associated with the persistence of primary vitreous.1,5 there is no previous report of cataract surgery in knobloch syndrome , according to a recent pubmed search using knobloch syndrome and cataract as keywords . collagen xvii is responsible for a proteoglycan component of basement membranes , including those of the iris , retina , and vitreous . it plays a role in eye development and maintenance of visual function.6,7 planning cataract surgery in patients with knobloch syndrome requires attention to the modified structure of the collagen , probably causing alterations and weakness of the zonula and lens capsule.5 an intense inflammatory response may also occur after surgery , especially in children.8 knobloch syndrome should be monitored due to the risk of complications during or after phacoemulsification surgery . genetic analysis in a specialized laboratory is necessary to confirm the diagnosis of knobloch syndrome .

knobloch syndrome is an autosomal recessive disorder associated with early - onset ocular abnormalities and central nervous system malformations . ocular abnormalities are usually severe , and include high myopia , vitreoretinal degeneration , retinal detachment , macular abnormalities , and cataract . the most frequent systemic changes are midline malformations of the brain , ventricular dilation , and occipital encephalocele . cognitive delay may occur . we report a case of cataract in a child with knobloch syndrome . cataract surgery and follow - up are described .

a.t . , a 43-year - old nigerian lady , presented to the eye clinic of the university college hospital , ibadan with gradual blurring of vision in both eyes for 4 years . ocular examination showed unaided visual acuity of 6/12 ou corrected to 6/9 in both eyes . dilated fundoscopy showed a well circumscribed cystic fovea lesion with a fluid level and an atrophic center in the right eye , while the left eye showed a scar in the macula ( fig . 1 and fig . the patient was prescribed with spectacles , counseled and placed on close observation with amslers grid for the development of choroidal neovascular membrane that will require urgent treatment . this is probably the first case of best macular dystrophy to be reported in ibadan , nigeria and possibly sub - saharan africa to the best of the author 's knowledge . it has also been reported in caucasians and asians , the other cases seen in african americans were associated with sickle cell trait . the pathophysiology of best 's disease is explained by abnormality in the retinal pigment epithelium ( rpe ) with resultant abnormal ionic transport leading to the accumulation of lipofuscin in the rpe cells and sub - rpe space in the macular area . the vitelliruptive stage may herald visual deterioration which becomes worse in the atrophic stage due to the presence of choroidal neovascular membrane . our patient presented with the early stages of the disease , hence the good visual acuity . the patient will be under observation and follow - up so as to detect changes amenable to treatment . choroidal neovascular membrane ( cnvm ) from best 's disease has been reported to respond well to intravitreal antivegf [ 7 , 8 ] . fundus flourescein angiography is essential in confirming the presence of cnvm and should be done when patient presents with sudden deterioration in vision . if a cnvm develops , then a corresponding area of hyperfluorescence with leakage will be found . since the disease is an autosomal dominant disorder , other family members will benefit from regular fundus examinations , and amslers grid is a valuable tool for monitoring central vision . an electrophysiologic test such as electrooculogram ( eog ) is specific for confirming the presence of the disease in relatives even in the absence of clinical signs and symptoms . a severe decrease occurs in light response , reflected by an arden ( light - peak / dark - trough ) ratio of 1.11.5 ( the normal arden ratio is 1.8 ) . the full - field electroretinogram ( erg ) result is normal in best 's disease . a focal erg or multifocal erg , concentrating on macular function , reveals abnormal function corresponding to the area of anatomical disruption . the pathophysiology of best 's disease is explained by abnormality in the retinal pigment epithelium ( rpe ) with resultant abnormal ionic transport leading to the accumulation of lipofuscin in the rpe cells and sub - rpe space in the macular area . the vitelliruptive stage may herald visual deterioration which becomes worse in the atrophic stage due to the presence of choroidal neovascular membrane . our patient presented with the early stages of the disease , hence the good visual acuity . the patient will be under observation and follow - up so as to detect changes amenable to treatment . choroidal neovascular membrane ( cnvm ) from best 's disease has been reported to respond well to intravitreal antivegf [ 7 , 8 ] . fundus flourescein angiography is essential in confirming the presence of cnvm and should be done when patient presents with sudden deterioration in vision . if a cnvm develops , then a corresponding area of hyperfluorescence with leakage will be found . since the disease is an autosomal dominant disorder , other family members will benefit from regular fundus examinations , and amslers grid is a valuable tool for monitoring central vision . an electrophysiologic test such as electrooculogram ( eog ) is specific for confirming the presence of the disease in relatives even in the absence of clinical signs and symptoms . a severe decrease occurs in light response , reflected by an arden ( light - peak / dark - trough ) ratio of 1.11.5 ( the normal arden ratio is 1.8 ) . the full - field electroretinogram ( erg ) result is normal in best 's disease . a focal erg or multifocal erg , concentrating on macular function , reveals abnormal function corresponding to the area of anatomical disruption . this is a report of best macular dystrophy in ibadan , sub - saharan africa .

best macular dystrophy is reported to be rare in africans . it is a hereditary disease that starts in childhood and progresses through some stages before visual symptoms occur . this case report presents a 43-year - old nigerian with the disease and stresses the importance of regular eye exams of patients and relatives to detect changes such as choroidal neovascular membrane amenable to treatment .

extramammary paget 's disease ( empd ) is a marginated plaque resembling paget 's disease but occurring in anogenital area , axilla , or most commonly on the vulva . we report a case in 62-year - old menopausal woman with lesions occurring on the perineal area . a 62-year - old postmenopausal married woman presented with slow - growing extremely pruritic plaque on the perineal skin . it initially started as a small pruritic papule which enlarged gradually to the present size in 3 years . on the basis of clinical presentation , she was diagnosed as a case of lichen simplex chronicus and started on topical medications and antihistamines . there was no response to any topical / systemic steroids , antibiotics , and antifungals . dermatological examination revealed approximately a 7 8 cm well - marginated , hypertrophic , and verrucous plaque in the perineal region . [ figure 1 ] at places grayish crusts with shallow erosions and a purulent discharge was seen . lesion on perineal area erosions and crusts over the plaque a part of the plaque was excised for histopathological evaluation . hematoxylin and eosin staining of the biopsy from the lesion showed large round cells with ample pale - staining cytoplasm , pleomorphic nuclei , and occasional prominent nucleoli , indicative of paget cells . inflammatory infiltrate with invasion of paget 's cells into the dermis at places was seen [ figure 3 ] . cells were positive for pas lying singly and in clusters also seen infiltrating along the skin appendages [ figure 4 ] . paget 's cells in h and e stain 10 paget 's cells in pas stain 40 gynaecological examination and mammography was normal . ultrasonography of abdomen - pelvis , magnetic resonance imaging and computerized tomography scan of the abdomen did not reveal any intraabdominal mass or retroperitoneal lymph nodes . the patient was then referred to an oncosurgeon who performed a surgical excision considering the premalignant potential of the disease . empd is a considered as uncommon cutaneous intraepithelial adenocarcinoma involving the epidermis and extending into the dermis . empd of the external genitalia and perineal skin may be associated with bladder , urethra , prostate cancer , or colorectal neoplasia . due to such associations , a complete investigation for an underlying carcinoma should accompany every confirmed diagnosis of empd . the patients present with pruritus , bleeding , oozing , tenderness , painful burning sensation , or hypopigmented / eczematous lesions . three patterns of empd are accepted : ( 1 ) in situ epithelial form without carcinoma and excellent prognosis , ( 2 ) epithelial form with adnexal carcinoma , and ( 3 ) with visceral malignancy . the characteristic histology of empd is paget 's cells , having abundant pale staining cytoplasm and large atypical nuclei with distinct intraepithelial mucus - secreting neoplastic proliferative cells . staining of paget 's cells immunohistochemical assessment can be done by markers like epithelial membrane antigen , carcinoembryonic antigen ( cea ) and gcdfp-15 . differential diagnosis includes neurodermatitis , periorificial tuberculosis , lichen simplex , psoriasis , lichen planus , mycosis fungoides , bowen 's disease and seborrheic dermatitis . histopathologically , the differential diagnosis includes pagetoid bowen 's disease and pagetoid malignant melanoma in situ . due to lack of knowledge about the disease and no specific guidelines for diagnosis radiotherapy , medical therapy with 5-fu , bleomycin , and co2 laser ablation are other modes of treatment . imiquimod used topically for a localized lesion of empd has shown promising results with 8 - 16 weeks of therapy with lifelong follow - up . moh 's surgery is most effective and has a recurrence rate of 8 - 26% . topical therapy helps only in few localized cases and there is a relapse rate of 50% . associated internal malignancy or a cea with dermal and lymphatic infiltrate of empd have worse prognosis . to conclude , an uncommon condition like empd should also be kept in mind when an elderly female presents with severe pruritic or nonspecific perineal lesion not responding to routine treatment . a differential diagnosis of empd should be considered for a severely pruritic perineal lesion unresponsive to routine treatment .

extramammary paget 's disease is a marginated plaque resembling paget 's disease but occurring in anogenital area , axilla or most commonly on the vulva . a 62-year - old postmenopausal woman presented with extremely pruritic plaque on the perineal skin which progressed gradually over 3 years and did not respond to any topical / systemic steroids , antibiotics , and antifungals . examination revealed 7 8 cm . hypertrophic , verrucous plaque with erosions , and crusts at places . there was no evidence of visceral malignancy . biopsy showed paget 's cells which were positive for periodic acid schiff and alcian blue stain . surgical excision was done considering the premalignant potential .

lipomas are benign , encapsulated tumours of mesenchymal origin , arising from the adipose tissue . superficial , simple lipomas can grow for many years without causing any functional problems , and they rarely reach very large dimensions . the surgical excision of giant occipitocervical lipomas is necessary for aesthetic concerns , pain and limitation of neck motion , especially in the recumbent position . a 52-year - old male patient was admitted to our clinic due to swelling at the back of his head . the history revealed that the swelling had been present for 10 years ; it had grown gradually from the size of a small hazelnut . during the patient 's general physical examination , a large , mobile mass of 10 10 cm was found , localized in the left occipitocervical region . the patient magnetic resonance imaging ( mri ) revealed a mass of 10 10 cm in the left occipitocervical region that had adhered to the adjacent bone structure , causing a thickening of the scalp ; however , there was no bone defect or destruction . the patient was operated on , and the subcutaneous mass was totally excised [ figure 1 ] . during the pathological examination the excised soft tissue mass was classified as a lipoma . ( a ) axial and ( b ) sagittal t2-weighted mr imaging shows the large lipoma ; ( c ) the lipoma in the occipitocervical region ; ( d ) the resected lipoma ; ( e ) picture of the completely resected lipoma a 56-year - old male patient was admitted to our clinic due to a swelling at the back of the head . the physical examination revealed a giant , painful mass covering the entire left occipital region . an mri showed a 11 10 cm mass , with a smooth , lobulated contour , and subcutaneous localization in the occipital region extending halfway down the neck , hyperintense on the t1-t2-weighted sequences with a thin septal contrast enhancement after contrast administration , which was adherent to the surrounding muscle tissue and bone . the pathological examination of the mass revealed a tumour covered with a thin , fibrous , connective tissue capsule that consisted of thin vascular structures formed by mature adipocytes of large vacuolated cytoplasm . ( a ) axial and ( b ) sagittal t2-weighted mr imaging shows the giant lipoma ; ( c ) the lipoma in the occipital region ; ( d ) the capsule of the lipoma after skin incision ; ( e ) the resected lipoma peeled off from the surrounding tissue and removed together with the capsule ; ( f ) picture of the completely resected lipoma after anaesthesia and intubation , the patient was placed in a prone position . following this , the capsule of the mass was reached via an elliptical skin incision about half the size of the mass . with careful dissection of the subcutaneous tissues surrounding the capsule , the skin was primarily closed . to prevent the accumulation of blood in the potential space and enhance early adhesion , a 52-year - old male patient was admitted to our clinic due to swelling at the back of his head . the history revealed that the swelling had been present for 10 years ; it had grown gradually from the size of a small hazelnut . during the patient 's general physical examination , a large , mobile mass of 10 10 cm was found , localized in the left occipitocervical region . the patient magnetic resonance imaging ( mri ) revealed a mass of 10 10 cm in the left occipitocervical region that had adhered to the adjacent bone structure , causing a thickening of the scalp ; however , there was no bone defect or destruction . the patient was operated on , and the subcutaneous mass was totally excised [ figure 1 ] . during the pathological examination the excised soft tissue mass was classified as a lipoma . ( a ) axial and ( b ) sagittal t2-weighted mr imaging shows the large lipoma ; ( c ) the lipoma in the occipitocervical region ; ( d ) the resected lipoma ; ( e ) picture of the completely resected lipoma a 56-year - old male patient was admitted to our clinic due to a swelling at the back of the head . the physical examination revealed a giant , painful mass covering the entire left occipital region . an mri showed a 11 10 cm mass , with a smooth , lobulated contour , and subcutaneous localization in the occipital region extending halfway down the neck , hyperintense on the t1-t2-weighted sequences with a thin septal contrast enhancement after contrast administration , which was adherent to the surrounding muscle tissue and bone . the pathological examination of the mass revealed a tumour covered with a thin , fibrous , connective tissue capsule that consisted of thin vascular structures formed by mature adipocytes of large vacuolated cytoplasm . ( a ) axial and ( b ) sagittal t2-weighted mr imaging shows the giant lipoma ; ( c ) the lipoma in the occipital region ; ( d ) the capsule of the lipoma after skin incision ; ( e ) the resected lipoma peeled off from the surrounding tissue and removed together with the capsule ; ( f ) picture of the completely resected lipoma after anaesthesia and intubation , the patient was placed in a prone position . following this , the capsule of the mass was reached via an elliptical skin incision about half the size of the mass . with careful dissection of the subcutaneous tissues surrounding the capsule , the skin was primarily closed . to prevent the accumulation of blood in the potential space and enhance early adhesion , lipoma is a benign tumour of mesenchymal origin that is rarely seen in the head and neck region . although the recurrence rate is not high , resection may be difficult due to the large size and adherence to the surrounding tissues . especially in our second case , resection was difficult due to the hard capsule , which was adherent to the occipital bone . lipomas are usually asymptomatic , but they can cause pain when they are large and press on the nerves . the two patients in our study were admitted to the hospital due to pain and cosmetic concerns . lipomas tend to locate in the trunk , shoulders , posterior neck , and axilla . approximately 80% are in the form of a single lesion , and they are generally seen in women . on the other hand , in addition to a physical examination , radiological assessment is necessary for the evaluation of the nature of the lipoma and surgical planning . connection with the surrounding tissues and bone can be evaluated by computed tomography ( ct ) and magnetic resonance imaging ( mri ) . in the case of suspicious certain positions of the body and/or some daily physical activities may be restricted due to the size of the tumour . lipomas that are large , painful , and cause cosmetic problems should be operated on following good preoperative planning .

lipomas are capsulated benign tumours that are commonly found in all body parts . a lipoma is a well - defined mesenchymal tumour that arises from the adipose tissue . although giant lipomas are rare in the head and neck regions , when they are located here , they are most commonly found in the subcutaneous posterior neck area . recurrence as well as invasion is very rare after total surgical excision . in this article , we present two rare cases of giant lipomas in the posterior occipitocervical region , which is an exceptional location .

sarcoidosis is a multi - system disorder of unknown etiology which can involve any organ of the body , more commonly affecting the lymph nodes , lungs , liver , spleen , skin , eyes , and bones . diagnosis of this condition is made primarily by histopathology wherein characteristic noncaseating epitheloid granulomas are observed in all or most of the affected tissues . hence , it has been described as a great mimicker in dermatology . though sarcoidosis may affect the skin of various areas of the body , rarer still is the incidence of sarcoidosis of scalp without affection of other cutaneous sites . sarcoidosis of the scalp may lead to cicatricial alopecia , a distressing complication for the patient . we report here a case of a middle - aged female who presented with scarring alopecia of the scalp as the only cutaneous manifestation of sarcoidosis . a 43-year - old woman presented with a complaint of loss of scalp hair for preceding 7 months . the condition had started insidiously and slowly progressed to involve almost the whole of the scalp . there was no history of exposure to radiation or intake of any medications . on clinical examination we observed patches of scarring alopecia , which had become confluent to progressively involve a major portion of the vertex of the scalp [ figures 1 and 2 ] . normal hair bearing skin was present among the patches and toward the margins of the scalp [ figure 3 ] . no other cutaneous findings suggestive of any other dermatologic condition were found at any other cutaneous site . a provisional diagnosis of cicatricial alopecia was made , and a punch biopsy was done to differentiate between the common causes of ciatricial alopecia such as lichen planopilaris , discoid lupus erythematosus , psudopelade of brocq , folliculitis decalvans , dissecting cellulitis of the scalp frontal fibrosing alopecia , and acne keloidalis . a punch biopsy specimen obtained from the affected area for histopathology showed multiple naked epitheloid cell granuloma in the dermis [ figure 4 ] . a number of giant cells of langhans and foreign body type were seen [ figure 5 ] . on the basis of these histological findings , a diagnosis of sarcoidosis of scalp was made . no abnormalities were found on the chest roentgenogram , but serum calcium levels were mildly elevated . patches of scarring alopecia on the scalp showing dryness and scaling lesions over temporal and frontal areas normal hair - bearing lesions at the occipital area noncaseating epitheloid cell granuloma in the dermis with a complete absence of any adnexal structure ( h and e , 100 ) granuloma showing langhan 's giant cell ( h and e , 400 ) based on the histological finding of naked epithelioid granulomas with multinucleated giant cells of the langhasns type , a diagnosis of sarcoidsis was confirmed . the patient was treated with systemic corticosteroids at a dose of 30 mg prednisolone / day gradually tapered over a period of 3 months . follow - up up to 1 year showed no regrowth of hair occurred though the treatment successfully controlled any further hair loss . cutaneous lesions were observed in 20 - 30% of cases of sarcoidosis and may be specific or nonspecific . specific lesions exhibit noncaseating granulomas on histopathological examination . however , nonspecific lesions are devoid of such granulomas . the commonest specific skin lesions observed clinically are reddish yellow maculopapules , plaques and subcutaneous nodules , but innumerable variations such as lupus pernio and scar sarcoidosis may occur . hence , the diagnosis of the condition is often challenging . sarcoidosis of the scalp resulting in alopecia is a rare cutaneous manifestation . while reporting two cases of sarcidosis induced alopecias house et al . observed that a thorough literature search in pubmed revealed reports of 39 published cases till 2012 . later one more case has been reported from mexico followed by one from japan . in most of these cases , the alopecia was cicatricial in nature though a few cases with nonscarring alopecia have also been reported . however , diffuse alopecia has also been reported , though rarely.a syrian patient with cicatrical alopecia due to sarcoidosis was found to have severely disfiguring lesions in the scalp , though the number of lesions were few . the disease shows a strong gender bias with 33 of the 38 cases ( where details were available ) occurring in females . racially , most of the reported cases were seen in afrcans or afro - americans . histologically , sarcoidosis can be distinguished from other causes of cicatricial alopecia by the presence of naked epithelioid cell granulomas . since our patient presented with scarring alopecia of 7 months duration discoid lupus erythematosus and lichen planopilaris the absence of lichenoid features , follicular plugging , vacuolar degeneration of basal cell layer and perivascular and periappendageal infiltrates helped in excluding lichen planopilaris or discoid lupus erythematosus as possible causes histologically . folliculitis decalvans and dissecting cellulitis were not considered as candidates for diagnosis because of the absence of suppurative folliculitis , abscesses , and sinus tracts . though systemic involvement particularly of the thorax has been found to occur in most of the cases of scalp sarcoidosis reported thus far , we did not find any evidence of underlying systemic involvement in our case other than a mildly raised serum calcium level . earlier reports of therapy of sarcoidosis with alopecia with corticosteroids ( topical and intralesional and systemic ) antimalarials and methotrexate have shown poor to moderate response . there are case reports of therapy with systemic corticosteroids where the progress of the disease has been arrested without any new hair growth . in our case , too , systemic corticosteroids were able to check the progress of the disease . the lack of improvement in the existing patches of cicatricial alopecia , in spite of therapy , was but expected as the hair follicles had been permanently destroyed by the disease process . not much is known about the overall prognosis of scalp sarcoidosis primarily because of the small number of reported cases . it might be possible that cases of alopecia due to sarcoidosis are often misdiagnosed in the absence of proper histopathological evaluation . this is unfortunate as recognition of the cutaneous lesions of sarcoidosis which are easily accessible for histopathology , can provide a clue to the underlying systemic disease and help in proper management of the patient . sarcoidosis of scalp has been reported earlier from india . however , to the best of our knowledge our case is the first report of cicatricial alopecia due to scalp sarcoidosis from this country . this report also emphasizes the e role of histopathology as an important diagnostic tool in the evaluation of cases of both scarring and nonscarring alopecia presenting to the dermatologist .

sarcoidosis affects different systems of the body including the skin where it can affect various cutaneous sites . among these sites , the scalp is a very unusual location for lesions of sarcoidosis . sarcoidosis of the scalp can very rarely be accompanied by cicatricial alopecia . we report here a rare case of sarcoidosis of scalp with cicatricial alopecia . to the best of our knowledge , this is the first such report from india .

hormonal changes , especially related to the menstrual cycle , have a great impact on the occurrence of migraines in women . the use of menstrual or non - menstrual migraines has been one way to stratify migraines without aura according to the appendix criteria of the international classification of headache disorders , second version ( ichd-2 ) . menstrual migraines ( mm ) are further classified into pure menstrual migraines ( pmm ) and menstrually related migraines ( mrm ) , which affect 1014 % and over 50 % of female migraineurs , respectively . women having migraines without aura encounter mm more frequently than those having migraines with aura . although a mrm sufferer may experience a migraine with aura outside the menstrual period , mm attacks almost invariably occur without aura . however , we diagnosed a female patient whose pmm with aura exclusively occurred at the onset of menstruation in the peri - menopausal years . from our literature review , we believe this is the first formal report of pmm with aura . a 51-year - old woman had been healthy and denied head trauma , coffee or alcohol consumption , and contraceptive drug use history . however , she had suffered from monthly episodic moderate headaches since age 46 and they always occurred on the first menstrual day . she always experienced sensory aura as hypoesthesia at her left hand and gradually extended to whole upper limb within 2030 min . it disappeared also from her hand and then completely remission in another 2030 min . after remission of the sensory aura , headache ensued immediately and was described as holocranial , throbbing , and outward expanding in nature . associated symptoms included photophobia , nausea , and occasional vomiting without phonophobia . the headache usually lasted for 6 h despite taking 500 mg acetaminophen from local clinic . she began menopause half a year ago , and did not receive hormone replacement therapy . her headache pattern then changed , with the headache still occurring regularly at intervals of once per month , but each attack lasted only for one - half to 2 h. she no longer experienced aura or premonitory symptoms . physical activities did not exacerbate her pain , so she could work or ambulate as usual . due to the atypical presentation of mm , she came to visit us and further evaluations were arranged for her . as the current headache simply recurred each month and did not affect her much , 500 mg acetaminophen was still recommended for acute symptomatic relief . we reported a peri - menopausal woman whose late - onset pmm was associated exactly with sensory aura . previous epidemiologic study of migraine with typical aura showed 99 % in the form of visual aura . in addition , she also had a longer period of premonitory symptoms and post - dromal euphoria . although mm is usually more severe and resistant to treatment , our patient s headache had a relatively good response to acetaminophen 500 mg . mm tends to be associated with migraine without aura . in a pilot study of 55 migraineurs , macgregor et al she advocated new appendix criteria for the ichd-2 , which classified female migraine without aura into pmm , mrm , and non - mm . however , some studies showed opposing results . in a retrospective clinic - based study , migraine with aura attack only in the menstrual period had a prevalence similar to migraine without aura ( 4 % vs. 3 % ) , but granella et al . attributed this result to selection bias from clinics because migraine with aura drove patients to visit clinics another questionnaire - based study showed menstruation may be a trigger of migraine with aura , but did not clearly describe pmm or mrm . in conclusion , several epidemiological studies mention migraine with aura occurring menstrually , but clinical details are usually lacking and one can not be sure if these papers refer to pmm or instead to mrm . the pathophysiology of mm is associated with falling estrogen levels in the late luteal / early follicular phase of the menstrual cycle . however , in the peri - menopause years , the disordered and often unpredictable hormonal fluctuations may worsen previous migraines , or even contribute to the new onset or rebound of subsided migraines . in our case , although the patient s pre - menopause life was uneventful , estrogen concentrations fluctuated chaotically during the peri - menopausal years , exposing her to migraine with aura during the onset of each menstruation . it seems convincing that the decreased and less fluctuated estrogen level after menopause had abated the typical aura and premonitory symptom , and even changed the headache pattern after menopause . normally , the relationship of migraine attacks with menstrual cycle is ascertained by a headache diary . since our patient had regular occurrence of attacks on the first day of menses only , a diagnosis of pmm by history can be acceptable . the lack of a headache diary , however , should be underlined as a limit of our case report . in conclusion , we reported an unusual case of a female patient who had pmm with a distinct and regular aura .

hormonal changes related to the menstrual cycle have a great impact on migraines in women . menstrual migraine attacks are almost invariably without aura . categorizing migraines into menstrual or non - menstrual types is one way to stratify migraines without aura according to the appendix criteria of the international classification of headache disorders . we report a peri - menopausal woman whose sensory aura exclusively heralded menstrual migraine . a 51-year - old woman had suffered from monthly episodic headaches since the age of 46 . before a headache , and within 1 h on the first day of her menstruation , she always experienced numbness in her entire left upper limb . after the sensory aura , migrainous headaches occurred with nausea and photophobia . in the postmenopausal period , she no longer had sensory aura , and her headache pattern changed and became less severe . her physical and neurologic exams as well as electroencephalography , brain magnetic resonance imaging , and conventional angiography were all normal . she fulfilled the diagnosis of pure menstrual migraine with typical sensory aura . to our knowledge , this is the first formal case report of pure menstrual migraine with aura .

a 70-year - old japanese man presented at our hospital with an asymptomatic , 20 30 mm irregularly shaped blackish , flat elevated plaque with a gray nodule in the periphery on his left lower leg ( fig . the lesion had been present for 10 years and had recently enlarged , associated with bleeding . dermoscopy revealed leaf - like areas , large blue - gray ovoid nests and multiple blue - gray globules ( fig . basal cell carcinoma and malignant melanoma were listed as suspected diagnoses , and we performed an excisional biopsy of the tumor . histopathological examination of the tumor revealed infundibular structures of the outer hair sheath in the center of the plaque , which consisted of three distinct parts ( fig . 2a ) . the first part showed circumscribed massive aggregation of basophilic basaloid cells containing abundant melanin granules with peripheral palisading and retraction spaces ( fig . this part of the tumor exhibited trichilemmal keratinization with squamous eddies , which were surrounded by a fibrous stroma ( fig . the third part showed reticular aggregation of basaloid cells with small infundibular cystic structures in the papillary dermis ( fig . the stromal part showed no fibrosis and abundant mucin deposition staining positive with alcian blue ( fig . immunohistochemical staining revealed diffuse positivity for anti - bcl-2 in the first part ( fig . 3b ) , positivity only at the periphery of tumor nests in the second part ( fig . microscopic examination of the tumor showed that it consisted of three distinct histological types : massive aggregation of basophilic cells was diagnosed as solid - type basal cell carcinoma , massive aggregation of clear cells with trichilemmal keratinization was diagnosed as proliferating trichilemmal tumor , and reticular small aggregations of basophilic basaloid cells in the papillary dermis were diagnosed as infundibulocystic basal cell carcinoma . basal cell carcinoma with follicular differentiation in 1987 by tozawa and ackerman , and later in 1990 the tumor was proposed as a variant of basal cell carcinoma showing differentiation to infundibular cyst - like structures by walsh and ackerman . clinically , the tumor presents as small papules / nodules , most commonly on the face . characteristic histopathological features of the tumor are small symmetrical , circumscribed aggregations of basaloid cells containing numerous infundibular cyst - like structures . infundibulocystic basal cell carcinoma is different from trichoepithelioma in that there are no follicular bulbs and papillae , no stromal fibrosis around the tumor , reticulated pattern of neoplastic cells , and abundant epithelial mucin . in the present case , anti - bcl-2 immunohistochemical staining was diffusely positive in portions of typical basal cell carcinoma and infundibulocystic basal cell carcinoma . , bcl-2 is present within basal keratinocytes , mesenchymal cells of follicular papilla , clear cells of eccrine glands , melanocytes and lymphocytes . anti - bcl-2 staining is useful for distinguishing between trichoepitheliomas and basal cell carcinoma , the former staining positively in the periphery of tumor nests , whereas the latter shows diffuse and widespread staining in all tumor nests . basal cell carcinoma is a neoplasm proposed to be derived from epithelial germ cells , and infundibulocystic basal cell carcinoma is derived from the infundibulum of the hair follicle , whereas proliferating trichilemmal tumor is derived from the hair follicle isthmus . there have been four reports of basal cell carcinoma arising with trichilemmoma without a pre - existing sebaceous nevus . taken together , the present findings suggest that basal cell carcinoma may be derived from hair germ cells , including the outer hair sheath and hair follicular infundibulum . the authors report no conflicts of interest . there were no funding sources for this work .

a 70-year - old japanese man presented at our hospital with an asymptomatic , blackish , irregularly shaped plaque with a gray nodule in the periphery on his left lower leg . the lesion had been present for 10 years and had recently enlarged , associated with bleeding . histopathologically , the tumor consisted of three distinct parts : the first part showed massive aggregation of basophilic basaloid cells with peripheral palisading and abundant melanin granules , and was diagnosed as solid - type basal cell carcinoma . the second part showed aggregation of clear cells with squamous eddies , and was diagnosed as proliferating trichilemmal tumor . the third part showed reticular aggregation of basaloid cells with infundibular cysts in the papillary dermis , and was diagnosed as infundibulocystic basal cell carcinoma . we diagnosed this tumor as basal cell carcinoma with various forms of hair follicle differentiation , including differentiation into the outer root sheath .

tracheostomy is one of the most frequent procedures done in an intensive care unit ( icu ) . more than 100,000 tracheostomies are performed annually in the us ( healthcare cost and utilization project 2007 ) . the reason for tracheostomies may be diverse , but the patients tend to have a long length of stay . due to large differences between hospital resource consumption and reimbursement , the health care financing administration added new diagnosis - related groups in 1987 , with heavy weights given to tracheostomy patients . surprisingly , there is a paucity of studies addressing the multifaceted care of these difficult patients to minimize length of stay and complications once the patient leaves the icu . as reported in the previous issue of critical care , garrubba and colleagues culled the literature and found only three studies [ 2 - 4 ] assessing the impact of a multidisciplinary team ( mdt ) on outcome of tracheostomy patients on the ward . all studies compared results with historical controls , and despite the problems intrinsic in cohort studies , the consistent observation was decreased time to decannulation [ 2 - 4 ] , and two of the three studies revealed decreased length of stay . specific outcomes and complications pertinent to tracheostomy patients were notably absent in these studies , although the implication is improved patient care . one study reported improvement in nursing compliance of tracheostomy care plan after institution of mdt , and death and code blues were less common ( albeit not statistically significant ) in the other two studies . in regard to tracheostomy patients , there are multiple variables that may impact clinical outcome , complications , or length of stay or all three . some of these considerations are the following : 1 . early versus late timing of tracheostomy 2 . the surgical technique itself : percutaneous versus open surgery 3 . choice of size and type of tube : double versus single cannula and size of tube in relation to the patient to provide the best function with least airway injury 4 . the best practical method to assess swallowing and prevent aspiration 5 . provision of pulse oximetry monitoring for higher - risk patients in a stepdown unit 8 . preventive measures to avoid tube obstruction such as hydration , humidification of airway , and suctioning of secretions 9 . factors leading to inadvertent decannulation ( such as underlying mental status ) and the best way of securing tracheostomy tubes ( suturing versus tie ) 10 . psychosocial well - being of patients with earlier speech therapy and effective swallowing leading to better communication , less isolation , and improved nutritional support 11 . the background of the physician may not be as important as their interest in these patients and the participation of the respiratory therapist , speech pathologist , clinical nurse specialist , physiotherapist , and dietitian . tobin and santamaria reorganized the existing staff to provide coordinated care without additional costs . resource expenditure on more personnel during times of health care cost crisis may be offset by the decreased length of stay and avoidance of catastrophic events . this lesson may be learned from other specialties in which utilization of case managers for a specific group of patients ( such as trauma victims ) may lead to decreased hospital days and improved care and is currently the standard of practice in trauma centers . it is probable that many institutions already have a modification of the mdt or a stepdown unit prompted by some catastrophic event of tracheostomy patients or both . a philosophical question is whether we can ethically design a prospective randomized trial in which the control group does not receive the benefits of multi - disciplinary care . the concept of mdt may be a common sense issue in which patient benefits and decreased length of stay occur due to small increments of coordinated efforts without a large - scale prospective randomized trial to ' prove ' that mdt works . recent studies advocate earlier performance of tracheostomies to decrease icu length of stay , and the number of tracheostomies performed may increase in the future , making this topic even more important .

patients requiring tracheostomies tend to have a longer length of stay due to their underlying disease . after a thorough literature search , garrubba and colleagues found only three studies assessing the impact of multidisciplinary teams ( mdts ) on tracheostomy patients on the ward . one consistent observation was the decreased time to decannulation after institution of mdt care when compared with historical controls . although a large prospective randomized trial is desirable before mdt is recommended , many institutions may have already formed a team approach to provide coordinated care resulting in improved outcome and length of stay .

lichen planus pigmentosus has been described as a condition of unknown etiology which clinically differs from the classic lichen planus by exhibiting dark brown macules and/or papules , mottled or reticulated hyperpigmentation and a longer clinical course . the most common sites of involvement are the face and neck and the flexural folds [ 1 , 2 ] . the most common pattern of pigmentation is diffuse , whereas less common patterns include reticular , blotchy , unilateral linear and perifollicular patterns . however , there has been no previous report of lichen planus pigmentosus with bilateral linear distribution . we report a first case of lichen planus pigmentosus with bilateral linear distribution associated with hepatitis c virus infection . a 33-year - old male presented with itchy brown - gray patches which first appeared on the dorsum of both hands and then spread proximally to both forearms and arms 2 years before consultation . he had no history of contact with any chemicals , plants , or perfumes and was not taking any medications . physical examination showed bilateral linear streaks of brown - gray patches with violaceous hue on both arms and forearms ( fig . 1 , fig . a skin biopsy was obtained from the right forearm and the left arm and stained with hematoxylin - eosin . it consisted of compact orthokeratosis , epidermal atrophy and focal hypergranulosis with vacuolar alteration of the basal cell layer and band - like infiltration of lymphocytes and melanophages with incontinence of pigment ( fig . routine laboratory findings were normal , except for an elevation of serum aspartate aminotransferase and serum alanine aminotransferase . the serology for hepatitis c virus was positive , while the serology for hepatitis b virus was negative . the lesion improved after sun avoidance and treatment of hepatitis c virus infection with a combination of interferon and ribavirin . lichen planus pigmentosus clinically differs from the classic lichen planus by exhibiting dark brown macules and/or papules , mottled or reticulated hyperpigmentation and a longer clinical course without scalp , nail , or mucosal involvement . it is most common in sun - exposed areas such as the face and neck and the flexural folds , including axillary , inguinal , and submammary regions . some authors observed a striking predominance of lesions in intertriginous locations , among which axillae are the most common ; thus , they proposed the designation lichen planus pigmentosus inversus . the most common pattern of pigmentation is diffuse , whereas less common patterns include reticular , blotchy , unilateral linear and perifollicular patterns . the characteristic histopathological findings are atrophic epidermis with vacuolar alteration of the basal cell layer , and scarce lymphohistiocytic or lichenoid infiltrates in the dermis with pigmentary incontinence and presence of melanophages . although lichen planus pigmentosus was first described by bhutani et al . in indians , it is also a common pigmentary disorder among other populations [ 5 , 6 ] . hepatitis c virus , mustard oil , amla oil , henna and hair dye could be precipitating factors in predisposed individuals . the association between hepatitis c virus and lichen planus has been extensively reviewed and the results suggest that lichen planus , mainly the oral type , is significantly associated with hepatitis c virus infection in certain geographic areas . regarding lichen planus pigmentosus , the prevalence of positive serology for hepatitis c virus is 60.6% in one study . however , it would be difficult to conclude that there is a positive association between the two conditions in regions with a high prevalence of hepatitis c virus infection . considerations in the differential diagnosis include linear ashy dermatosis , lichen striatus , linear and whorled nevoid hypermelanosis , and incontinentia pigmenti . most of these can be differentiated by clinical findings and histopathology . in conclusion , we report a case of lichen planus pigmentosus and hepatitis c virus infection . the linear bilateral involvement was interesting because it is the first reported case of this distribution . we propose that serology for hepatitis c virus and liver function test should be carried out in patients with lichen planus pigmentosus .

lichen planus pigmentosus is a rare subtype of lichen planus . we report a first case of lichen planus pigmentosus with bilateral linear distribution associated with hepatitis c virus infection . the lesion was improved after sun avoidance and treatment of hepatitis c virus infection with a combination of interferon and ribavirin . this case stresses the importance of screening for hepatitis c virus infection as lichen planus pigmentosus can be an associated condition .

even rarer is a transverse sacral fracture associated with an unstable pelvic ring injury ( a malgaigne pelvic fracture ) . they are difficult to diagnose as they develop after severe traumas and the accompanying clinical symptoms are masked by other pathologies . unstable pelvic injuries characterized by the detachment of the sacroiliac complex leads to continuous pain and functional limitations as well as early morbidity and mortality with severe late outcomes . achieving anatomical reduction with stabilization and fusion is of great importance for sacroiliac dislocations . the malgaigne fracture was defined by a famous french anatomist and orthopedic surgeon in the 1800s . the malgaigne fracture develops as a fracture of both pubic rami and the posterior region of the sacroiliac complex . namely , it is a fracture of both the anterior and posterior regions of the pelvis . the usually associated sacral fracture of the malgaigne fracture is unilateral and vertical through the sacral ala or transforaminal region ( denis type 1 or 2 ) . this case report introduces to the literature the unusual upper sacral ( s1/s2 ) transverse fracture with a kyphotic displacement and potential nerve root compression in a pediatric patient . a 12-year - old girl was admitted to the emergency room after having suffered from a fall . she fell from a height of approximately 12 m on her left leg then hit her hip on the ground vertically . she did not have any neurological deficits or incontinence and her abdominal examination revealed normal findings . x - rays revealed a detachment in the right sacroiliac joint and the symphysis pubis ( figure 1 ) . right sacroiliac displacement ( white arrow ) , displacement of symphysis pubis ( black arrow ) . lumbar computed tomography ( ct ) revealed a zone 3 sacral fracture according to the denis classification , a subtype 2 fracture according to the roy - camille classification , an s1s2 listhesis , and a right sacroiliac joint dislocation . ( a ) s1s2 listhesis image in the sagittal plane . ( b ) s1s2 listhesis image in the axial plane with a right sacroiliac joint dislocation . the patient was placed on the operating table in the prone position under general anesthesia after the preoperative procedures had been completed . bilateral iliac wing screws were placed and both iliac wings were attached to the system through a connector ( figure 3 ) . the patient , who could not be mobilized early due to the left talus fracture , was mobilized with crutches so that the left leg could be in a plaster cast for 1 week postoperatively . she did not have any complaints on the control examination 3 months later and she was able to do her daily activities . as in adults , sacral fractures during childhood develop as a result of high energy traumas . the rate of sacral fractures that accompanies spinal trauma has been reported as 30% in a study conducted in adults . the rate of sacral fractures is 0.6% , and that of pelvic fractures is 4.76% in all spinal axis traumas in childhood . many classification systems have been proposed for sacral fractures ; however , there is no universally accepted classification system . the classification recommended by denis et al is a very useful system and is commonly used today . according to denis , the sacral ala is affected in zone 1 , sacral foramina are affected in zone 2 , and the sacral canal is affected in zone 3 . it has been reported that fractures exceeding this limitation or classification are known to be defined as zone fractures . according to this classification , gibbons et al reported the rate of zone 1 fractures as 6% , zone 2 fractures as 28% , and zone 3 fractures , including vertical fractures , as 58% . they reported neurological deficits occur at a rate of 57% in zone 3 fractures that accompany transverse fractures . type 1 was defined as a simple flexion deformity of the sacrum , type 2 as a partial translation and hyperkyphotic , and type 3 is a complete translation with no fracture overlap . our case was a zone 3 fracture according to denis , a type 2 fracture according to the roy - camille classification , and there was no neurological deficit . transverse fractures occur less frequently and all sacral fractures in adults are reported in varying rates between 5% and 16% . it has been reported that sacral fractures may coexist with spinal vertebral fractures in different localizations , usually with pelvic fractures and large extremity fractures . a sacroiliac joint dislocation accompanied the vertical type sacral fracture in our case . we considered that the absence of neurological deficits was due to the elastic structure of the bone in the developmental period . the importance of the mechanical stability role provided by the sacrum and pelvis bone ring require that meticulous care be provided to patients with pelvic trauma . vertical shear fractures , known as malgaigne fractures , develop as a result of high energy traumas . sacroiliac joint dislocations are pathologies that develop as a result of vertical loading ; almost all of these develop as the result of falls in the standing position . in malgaigne fractures , the sacrospinous and sacrotuberous ligaments are also torn in addition to the posterior sacroiliac complex . anterior and posterior lesions develop in this fracture . additionally , an impairment of the symphysis pubis or superior inferior rami , fracture in the ilium , dislocation or fracture in the sacroiliac joint , and fractures in the lumbar vertebrae 4 and 5 are also observed . there was a detachment in the symphysis pubis , a sacroiliac dislocation , and a sacral fracture in our case . however , these fractures may be overlooked in x - rays due to the complexity of the region . comparative studies have shown that all current sacroiliac fixation methods have close stability with regard to biomechanical properties . another result of these studies is that no methods can reach the stability of the intact pelvis . s1 sacroiliac fixation with an iliac screw and transpedicular screw , anatomical reduction of the sacroiliac complex , and stabilization are the most commonly preferred techniques . in our case , an appropriate load distribution was provided with lumbosacroiliac fixation through bilateral l5s1s2 transpedicular screws and bilateral iliac wing screws . neural decompression was performed with an s1s2 total laminectomy , and the compression on the neural tissue in the sacral region was eliminated . it is very difficult to make treatment generalizations due to the limited number of cases of sacral fractures in childhood and malgaigne fractures . sacral fractures should be suspected in the presence of ecchymosis and pain in the sacral region , pelvic unstability , radiculopathy , and urinary / anal incontinence .

abstractsacrum fractures are rare pathologies seen after spinal traumas . the incidence of a sacral fracture after trauma is 0.6% in childhood . a malgaigne fracture is composed of fractures and dislocations of the anterior and posterior regions of the pelvis . this is the first reported case of malgaigne fracture during childhood.a 12-year - old girl was admitted to our emergency room after having suffered a fall . radiological tests revealed a zone 3 sacral fracture according to the denis scoring , a subtype 2 sacral fracture according to the roy - camille classification , and a detachment in the symphysis pubis . appropriate load distribution through a bilateral l5s1s2 transpedicular screw and a bilateral iliac wing screw , as well as neural decompression were performed together with an s1s2 total laminectomy.it is very difficult to make a generalization for treatment of sacral fractures and malgaigne fractures in childhood due to the small number of patients . each patient should be individualized and lumbosacroiliac instability should be treated .

according to the international agency for research on cancer ( iarc ) , leather tanning and processing is not classifiable as to its carcinogenicity to humans , although the production process involves exposure to numerous chemicals , for some of which there is evidence of carcinogenicity in humans . however , in 1981 , iarc had reported an increased risk for bladder cancer in the only study available at the time . tannery workers have been known from previous studies to have the potential for exposure to numerous known or suspected occupational carcinogens including hexavalent chromium salts , arsenic , organic solvents ( benzene , formaldehyde , butyl acetate , ethanol , acetaacetate , toluene and acetone ) . the two major sources of chromium particulates in the tannery work environment are chromium chemicals used in the tannery work environment are chromium chemicals used in the tanning process in the form of baychrom and cr ( oh)so4 . chromium , which is a basic tanning agent , is available in trivalent form as chromium sulphate and in inorganic form and in the protein bound form that is known as leather dust . the leather dust produced by mechanical operations the exposures within the leather tanning industry have been suggested by some investigators[35 ] to result in the development of a variety of specific cancers including lung , bladder , kidney , pancreatic oral cavity , nasal and soft tissue sarcoma and skin along with dermatitis , ulcers , perforation of the nasal septum , respiratory illnesses . lung cancer : epidemiologic studies of tanners have shown an increased risk for cancer of the lung.[612 ] a review of cancer risk among tanners in italy was published and indicated an excess of lung cancer . increased mortality from lung cancer has also been reported earlier . an excess of lung cancer was observed in a case control study in the us , but these results were not supported by other studies . chromium and arsenicals were mentioned as possible contributors to the lung cancer excess . on the contrary , opined that the excess of lung cancer among tannery workers may partially be explained by cigarette smoking and not alone occupational exposure in the tannery industry as smoking is strongly correlated with cigarette smoking.[1617 ] pancreatic cancer : a three - fold statistically significant excess in pancreatic cancer mortality was noted in a swedish case referent study ; a 50% increase in pancreatic cancer was also noted in another study of three swedish tannery industries[910 ] and in an italian tannery . despite the excess risk of pancreatic cancer , no specific environmental agent was identified and dietary factors were considered as a possibility . these women were employed in the dyeing , stuffing and decorative process where they were basically exposed to organic solvents , formaldehyde , aniline and azo dyes . similar findings have also been reported who reported that the majority of the workers who died of pancreatic cancer had high exposure to formaldehyde and high dye and solvent exposure . high mortality form of pancreatic cancer is also shown in earlier studies showed the strong ability of proteins of leather dust to bind inorganic cr iii compound and facilitate their entrance into gastro intestinal tract . skin cancer and melanoma : one study in ussr showed skin carcinogenic effect of some synthetic fats used under leather stuffing . the workers exposed to leather dyes and fats may initiate skin cancer by direct impact . the females working in the dyeing , stuffing and decorating processes suffered from melanoma of skin and nonmelanoma skin cancer . high mortality of melanoma and skin cancers in females occupied in dyeing , stuffing and decorative workshops was observed in an recent study . kidney cancer : an excess of kidney cancer among tannery workers has been suggested by an earlier report . the dyes and pigments were mentioned as possible causative factors . buccal cavity and pharynx cancers : the workers in the tanning and liming workshops where exposure of leather dust was not high suffered from buccal cavity and pharynx cancers . pancreatic cancer : majority of workers who died with pancreatic cancer were either driers ( high formaldehyde exposure ) or painters ( high dye - direct black 3 azo dye and solvents ) . in these conditions they presumed that azo dyes and/or formaldehyde could initiate dna mutation and chromium iii promote pancreatic cancer growth . bladder cancer : the analysis of recent literature data mainly based on case control studies showed the prevalence of bladder cancer in the leather industry . recent reports show one case of bladder cancer in man who was occupied in tanning and liming workshop . they pointed out gender difference in the prevalence of bladder cancer as women had much lower risk to develop bladder cancer than males . a study of the chinese leather tanning industry showed a statistical significance excess morbidity from bladder cancer among tanners were exposed to benzidine - based dyes , which increased with duration of exposure . a similar result was reported in her study of tuscan tannery industry and in her review of other studies among workers in leather finishing operation . however , earlier report suggest that in his mortality study bladder cancer risk was not elevated . sinonasal cancer : an excess risk of sinonasal cancer among leather tannery workers was observed in a case control study in italy . one case of nasal cancer was also reported earlier . however , iarc reported that there was no evidence of an increased risk of nasal cancer in the leather tanning and processing industry . testicular cancer : an excess risk of testicular cancer incidence was observed among leather tanners from the finishing department of the tannery and were exposed to dimethylformamide ( dmf ) , a substance known to cause testicular damage . a statistically significant odds ratio of 7.2 for testicular cancer was observed among leather tanners . soft tissue sarcoma : two separate tannery studies indicated a significantly increased risk of this rare cancer . both investigators suggested that chlorophenols used in the pretanning operation and in the tanning process which have produced these tumors .

work in leather tanning involves exposure to a wide range of chemicals . some of these are carcinogens or suspected carcinogens . increased risks for a number of cancers have been reported among the tannery workers . in the present review , a detailed account of lung cancer , testicular cancer , soft tissue sarcoma , pancreatic cancer , bladder cancer among tannery workers is mentioned .

situs inversus is a rare condition with a frequency of about one in 10,000 of the normal population . however , the association of duodenal atresia with situs inversus is very rare , with just about 20 cases reported in the literature so far . reverse double - bubble sign as a means of identifying this condition , abdominal sonography for confirmation and the importance of keeping the mirror anatomy in mind while carrying out the surgery . a 1.9-kg preterm ( 36 weeks ) male child , born by caesarean section to a 2 gravida mother , was seen on day 2 of life . the antenatal ultrasonogram at 32 weeks of gestation had revealed findings suggestive of duodenal atresia with left ventricular hypoplasia . the infantogram showed double - bubble sign with gastric shadow in the right and cardiac shadow normally placed [ figure 1 ] . the abdominal sonography confirmed situs inversus with the liver on the left side and the spleen on the right side . an echocardiography revealed situs solitus with hypoplastic left ventricle , double - outlet right ventricle , small atrial septal defect and small ventricular septal defect . reverse double - bubble the child underwent laparotomy on the 4 day of life . the child had type 1 duodenal atresia with situs inversus abdominus . a side - to - side duodeno - duodenostomy was performed . postoperatively , the baby required ventilatory support for a day and parenteral nutrition for 7 days . a contrast study confirmed a patent anastomosis without any leak . the parents were advised to visit a higher cardiac center for the management of congenital cardiac anomalies . among the known congenital anomalies associated with duodenal atresia , the more common ones are down 's syndrome , cardiac anomalies , annular pancreas and malrotation . situs inversus abdominus , also called abdominal heterotaxia or isolated levocardia , is characterized by inversion of the abdominal organs but with a normally located left - sided heart . it is quite an uncommon condition , with a frequency ranging from one in 4,000 to one in 20,000 live births . the association of abdominal situs inversus with congenital duodenal obstruction is very rare , with only about 20 cases reported so far in the literature . the majority of them , 10 cases , had either annular pancreas or duodenal web . preduodenal portal vein , duodenal stenosis and complete atresia have also been reported singly or jointly . the embryological explanation for the occurrence of duodenal atresia is the theory of failure to recanalise in the 12 week of intrauterine life from the solid cord stage . this can lead to duodenal stenosis , a web with or without central aperture or a complete atresia . however , when associated with duodenal atresia , it is detected in the newborn period . the diagnosis can be made with a plain radiograph of the chest and abdomen , which reveals reverse double - bubble . in a case of situs inversus abdominus , one is likely to think , as we did initially in this case , of wrong labeling of the side on the radiograph , as it is the more common occurrence . an ultrasonography of the abdomen would clinch the condition and an echocardiogram is necessary to confirm the position of the cardia and detect any associated anomalies . the treatment does not differ whether or not duodenal atresia is associated with situs inversus . the association of duodenal atresia with situs inversus abdominus is extremely rare . with prompt recognition of the condition the outcome is no different from the duodenal atresia without situs inversus , as the prognosis depends on the gravity of the associated cardiac anomalies .

we report a rare association of duodenal atresia with situs inversus abdominus in a newborn . the infantogram revealed reverse double - bubble sign without dextrocardia . the sonography and echocardiography confirmed the diagnosis of situs inversus abdominus with multiple cardiac anomalies . laparotomy and a duodenoduodenostomy were carried out .

vignon and colleagues prospectively assessed the ability of transesophageal echocardiography ( tee ) to predict paop higher than 18 mmhg in mechanically ventilated patients with an inserted pulmonary artery catheter . in a first group , they analyzed simple doppler variables derived from transmitral flow ( tmf ) and pulmonary venous flow ( pvf ) and performed the usual measurements and calculations ( maximal velocity and velocity time integral of e ( the maximal velocity of early diastolic tmf ) and a ( the maximal velocity of late diastolic tmf ) wave , e / a ratio , e wave deceleration time ( edt ) , maximal velocity and velocity time integral of s ( the maximal systolic pvf velocity ) and d ( the maximal diastolic pvf velocity ) wave , s / d ratio , atrial filling fraction and systolic fraction of pulmonary venous flow ( sfpvf ) ) . the correlations between doppler variables and pulmonary artery occlusion pressure ( paop ) were better in patients with depressed left ventricular ( lv ) systolic function than in those with normal lv systolic function . paop could be predicted by e / a > 1.4 , edt > 100 ms , atrial filling fraction > 31% and sfpvf > 44% , with similar sensitivity and specificity and acceptable positive and negative predictive values . in a second group these cutoff values were prospectively evaluated for prediction of paop higher than 18 mmhg . additionally , they measured maximal early diastolic velocity of lateral mitral annulus by tissue doppler ( ea ) and color m - mode doppler flow propagation velocity ( vp ) . an e / ea ratio < 8 and an e / vp ratio < 1.7 were predictive for paop > 18 mmhg , but the use of these additional variables did not improve the correct estimation of paop . elevated paop reflects an increase of lv end - diastolic pressure due to lv diastolic and/or systolic dysfunction / failure . paop less than 18 mmhg , if measured , supports criteria for the definition of acute respiratory distress syndrome and acute lung injury . clinical and radiological estimation of paop is uncertain in cardiac patients and almost impossible in intensive care unit patients [ 2 - 5 ] . paop measurement by pulmonary artery catheter is , for various reasons , not commonly used in cardiac failure and critically ill patients . on the other hand , tee and transthoracic echocardiography ( tte ) are increasingly used for diagnostic and hemodynamic assessment and in critically ill patients , allowing noninvasive estimation of paop by doppler technique . the first group includes relatively simple variables ( e , a , e / a , edt , sfpvf ) derived from analysis of diastolic tmf and pvf . the second group includes ea and vp ; both variables are preload independent and are used to correct the e velocity for relaxation changes ( e / ea and e / vp ratio ) . use of tte was limited because of inadequate visibility ; many patients had to be excluded because of inadequate doppler signal recordings . technical improvements and the use of harmonic imaging now allow measurement of tmf and pvf in the majority of patients , but tee is still frequently used , especially in mechanically ventilated critically ill patients . tmf and pvf variables measured by tte are accurate for the estimation of lv filling pressure and cardiac index in patients with depressed cardiac function and heart failure , but in patients with normal systolic lv function tissue doppler derived variables show better correlation with paop [ 9 - 11 ] . in patients who have undergone cardiac surgery and in critically ill patients , tee - derived sfpvf and e / ea correlate well with left atrial pressure and paop [ 12 - 14 ] . the study by vignon and coworkers shows that in patients with acute lung injury , simple doppler variables derived from tmf and pvf by tee predicted elevated paop better than atrial filling fraction and edt and that the use of additional and more advanced variables ( ea and vp ) did not improve the accuracy of prediction . an important practical limitation of the study is the fact that 20% of patients could not be studied because of cardiac problems , and that in a further 10% of patients , some variables could not be recorded . concerning the study , the following questions should be considered . despite the fact that paop is not transmural pressure and does not accurately reflect preload and volume responsiveness , it is still used as a supportive criterion for the diagnosis of acute respiratory distress syndrome and heart failure . noninvasive estimation of paop is feasible by using tte / tee - derived simple doppler variables , but not in every patient . despite technological improvements in past years , adequate doppler tracing can not be obtained by tte in many critically ill patients . . it would be interesting to compare tte and tee simultaneously for paop estimation in a large group of critically ill patients . besides this , in a certain subset of patients , noninvasive estimation of paop is not possible and invasive measurement of paop , if needed , is still necessary . taking into account that tte or tee should be performed in the majority of intensive care unit patients for initial hemo - dynamic assessment , the systematic estimation of paop by simple analysis of tmf and pvf would undoubtedly increase the overall quality of this . the use of additional variables ( ea , vp ) , which are routinely not measured in the intensive care unit setting , is not necessary for paop estimation in patients with impaired global systolic lv function , but can improve its estimation in patients with normal systolic function and diastolic dysfunction / failure . a = maximal velocity of late diastolic tmf ; d = maximal diastolic pvf velocity ; e = maximal velocity of early diastolic tmf ; ea = tissue doppler diastolic velocity of mitral annulus ; edt = e wave deceleration time ; lv = left ventricular ; paop = pulmonary artery occlusion pressure ; pvf = pulmonary venous flow ; s = maximal systolic pvf velocity ; sfpvf = systolic fraction of pvf ; tee = transesophageal echocardiography ; tmf = transmitral flow ; tte = transthoracic echocardoigraphy ; vp = color m - mode doppler flow propagation velocity .

the measurement of pulmonary artery occlusion pressure ( paop ) is important for estimation of left ventricular filling pressure and for distinction between cardiac and non - cardiac etiology of pulmonary edema . clinical assessment of paop , which relies on physical signs of pulmonary congestion , is uncertain . reliable paop measurement can be performed by pulmonary artery catheter , but it is possible also by the use of echocardiography . several doppler variables show acceptable correlation with paop and can be used for its estimation in cardiac and critically ill patients . noninvasive paop estimation should probably become an integral part of transthoracic and transesophageal echocardiographic evaluation in critically ill patients . however , the limitations of both methods should be taken into consideration , and in specific patients invasive paop measurement is still unavoidable , if the exact value of paop is needed .

the prevalence of diabetes mellitus is increasing , in part due to aging , obesity , and lower levels of physical activity . the findings of siegelaar and colleagues presented in the previous issue of critical care challenge current beliefs but are also relevant to all . advanced diabetes is associated with end organ damage that is likely to impact upon critically ill patients - nephropathy , autonomic neuropathy , and small - vessel and large - vessel disease . thus many believe that diabetes is associated with increased mortality and morbidity in patients admitted to an intensive care unit ( icu ) . siegelaar and colleagues use meta - analysis techniques to examine the relationship between mortality and diabetes mellitus in patients admitted to an icu . pooling data from 141 studies and nearly 12.5 million patients , they demonstrate that , outside the cardiothoracic icu , diabetes mellitus is not associated with an increased risk of mortality . their findings were consistent whether mortality was considered at icu discharge , hospital discharge , or 28/30 days . the value of the study lies in the large number of patients included , sourced from a broad range of publications most of which were not specific to patients with diabetes mellitus . moreover , the forest plots demonstrate consistency between studies . however , unmeasured con - founders may influence their results . whilst a sensitivity analysis using risk - adjusted mortality from five studies encompassing ~15% of the patients demonstrates a similar result , for example , patients with diabetes mellitus may receive inhibitors of the renin - angiotensin system , hmg - coa reductase inhibitors , peroxisome proliferator - activated receptor gamma agonists , and aspirin . all of these treatments have been postulated to affect outcome in critically ill patients [ 4 - 6 ] . the study is limited also by considering diabetes mellitus as a homogeneous entity rather than a diagnosis that encompasses two different pathophysiologies and widely ranging therapies and qualities of glycaemic control . the authors ' results are mirrored in current risk - adjustment models . in the cardiothoracic setting , the presence of diabetes mellitus has been used as a risk factor for perioperative mortality in the parsonnet score but not the euroscore tools . cardiac revascularisation of patients with diabetes mellitus is complicated by poorer targets and microvascular disease . by contrast , diabetes mellitus does not form part of standard icu risk - prediction models such as the acute physiology and chronic health evaluation score and the simplified acute physiology score [ 9 - 12 ] . why does the presence of diabetes mellitus not matter outside the cardiothoracic icu ? as the authors point out , the higher incidence of sepsis in patients with diabetes mellitus may imply a protective effect . insulin is anti - inflammatory and switches cells to preferentially metabolise glucose rather than free fatty acids . alternatively , patients with diabetes mellitus may be taking protective medications or have become accustomed to mild hyperglycaemia , a prooxidant status , or increased levels of advanced glycated end products - all implicated in the pathogenesis of systemic inflammation . since few would have cited diabetes mellitus as a contributory reason for defining an icu admission as futile , siegelaar and colleagues ' study does not change our clinical practice . nevertheless , the suggestion of a potential protective effect for diabetes mellitus merits further investigation . unpicking this hypothesis may reveal new therapeutic strategies that we can exploit in other patient populations .

diabetes mellitus is increasingly prevalent and associated with significant end organ damage that one may presume to impact upon critical illness . however , siegelaar and colleagues present data that suggest , excepting those patients admitted to a cardiac intensive care unit , the presence of diabetes mellitus is not associated with increased mortality in critically ill patients . it is not possible to unpick how unmeasured parameters such as glycaemic control , the nature of whether type i or type ii , or concomitant drug therapy confound the results . nevertheless , the results are consistent with many risk - adjustment models used in the critically ill , and clinical practice that tolerates mild hyperglycaemia . is it even possible that diabetes mellitus is protective ?

a foreign body in the rectum is always a challenging diagnostic and management dilemma that begins with the initial evaluation in the emergency department and continues through the postextraction period . usually , it is a result of pathological sexual activities performed by the patient themselves , or by other people , the latter being a criminal act ; however , sometimes , a foreign body is swallowed passes through the gastrointestinal tract and is held up in the rectum . most of them are excreted ( 8090% ) , some require endoscopic removal ( 1020% ) , but 1% require surgical intervention . one of the most common problems encountered in the management of rectal foreign bodies ( rfb ) is the delay in presentation , as many patients are embarrassed and reluctant to seek medical care . most of these patients present to the emergency room after efforts to remove the object at home that may lead to a perforation or significant bleeding from the rectum . hence , a stepwise approach that includes diagnosis , removal and postextraction evaluation is essential . a single incision laparoscopic surgery ( sils ) port with a shape adapted to the anatomy of the anus was used to remove a rectal foreign body , for the first time , in korea , our case was done almost in a similar fashion and as far as we know the first to be reported in france and the third case worldwide . a 70-year - old male with the history of psychiatric disorders presented to our emergency department after insertion of a cap of a bibber ( nursing baby bottle ) into his rectum . he attempted to remove it by himself but did not succeed . upon presentation , the patient was stable , abdomen soft no peritoneal signs and digital rectal examination ( dre ) was done with failure even to palpate the foreign body with the tip of the finger . the patient was taken to the operating room under general anesthesia , underwent rectosigmoidoscopy that revealed no damage to the rectum and the cap to be impacted in the rectum at 12 cm from the anal verge ( fig . 1 ) . accordingly , a nonsurgical technique was attempted ; manual and endoscopic attempts were not effective . so the patient was put in a lithotomy position ; after manual dilatation of the anus , a sils port was introduced into the anal canal ( fig . 2 ) and the rectum was extended by continuous co2 insufflation up to 10 mm hg . a zero degree 5-mm laparoscope was then introduced , and under direct vision , the foreign body was removed using two laparoscopic grasping forceps and gently extracted from the rectum ( fig . 3 ) . a postextraction evaluation using the sils port showed small superficial mucosa ulcerations with no serious damage to the wall of the rectum . two days later a follow - up rectosigmoidoscope was done and was normal except for the superficial injuries that were seen before . rfbs present to the modern surgeon with a difficult management dilemma , as the type of object , host anatomy , time from insertion , associated injuries and amount of local contamination may vary widely . management of these patients may be challenging , as presentation is usually delayed after multiple attempts at removal by the patients themselves have proved unsuccessful . cohen and sackier showed a trend with 78% of cases linked to anal eroticism and 10% to assault . rfb may be high- or low lying depending on their location relative to the rectosigmoid junction . generally , low - lying rectal foreign objects can be easily reached by the examining finger , whereas the ones above the rectosigmoid junction are difficult to reach . generally , low - lying rfb that are palpable on dre may be extracted in the emergency department . however , objects that are above the sacral curve and rectosigmoid junction are difficult to visualize and remove and may be unreachable even with rigid proctosigmoidoscopy . fifty - five percentage of cases above the rectosigmoid junction require surgery , whereas only 24% required surgery when an object present in the rectum . the basic principles of therapeutic intervention include transanal removal under the appropriate anesthesia , selection of patients for surgical retrieval , proctosigmoidoscopy following retrieval to assess the extent of damage and inpatient observation to rule out possible complications [ 4 , 6 , 7 ] . an anoscope or sigmoidoscope should be utilized to remove the foreign body under direct vision where possible to avoid iatrogenic injury . there are varying techniques of transanal foreign body retrieval described in the literature including the use of polypectomy snares , forceps , inflated foley balloon catheters , obstetric forceps , vacuum extractors , achalasia or dilation balloons and even plaster casts [ 9 , 10 ] . a new approach to the management of high - lying rectosigmoid foreign bodies using the transanal sils port technique with conventional laparoscopic instruments allows the direct vision and identifying the nature of foreign body , assessment of the rectal wall and the extent of injury if present . another important aspect of this method is the replacement of the rectosigmoidoscope which is mandatory for postextraction evaluation of the distal bowel wall . as a conclusion , in the treatment of rfbs , the aim is to use the simplest possible method while protecting the integrity of the intestine . outcome was excellent for this new method , which we think is simple and safe to use and can be considered as an alternative to surgical approach and even as a standard of care for removal of high - lying foreign body in order to avoid surgery .

a foreign body in the rectum is not a very common surgical emergency case . in the treatment of rectal foreign bodies , the aim is to use the simplest possible method while protecting the integrity of the intestine . many removal techniques have been described in the literature . here we report a case in which a transanal technique using a single incision laparoscopic surgery port was successfully used .

rhizomelic chondrodysplasia punctata ( rcdp ) is a very rare disease . it impairs the normal development of many parts of the body . the features of this disorder include bony abnormalities , severe mental retardation , joint contractures , cataract and recurrent respiratory infections and breathing problems . seizures and distinctive facial features including prominent forehead , depressed nasal bridge and small nose is also associated with this pathology . our aim is to discuss current knowledge on etiopathogenesis as well as radiological and clinical symptoms of diseases associated with rcdp . 5 yrs old male child presented with chest infection and periarticular swelling of all the small and large joints . he was further investigated in the form of clinical , biochemical and radiological assessment which stated that the patient was suffering from rcdp . though this is not curable , management of rcdp is symptomatic and supportive and may include physiotherapy and orthopedic procedures ( in later stages ) to improve function . rhizomelic chondrodysplasia punctata type 1 ( rcdp1 ) is an inherited disease characterized by skeletal abnormalities , growth retardation , severe mental retardation , cataracts and has decreased life expectancy . it impairs the normal development of many parts of the body . these affected individuals have a specific bone abnormality called chondrodysplasia punctata , which shows scattered calcifications at the end of the long bones thus affecting their growth . people with rhizomelic chondrodysplasia punctata often develop joint deformities ( contractures ) that makes the joints stiff and painful . this condition is considered as a developmental brain disorder characterized by systemic shortening of the proximal bones ( i.e. rhizomelia ) , seizures , recurrent respiratory tract infections , and congenital cataracts . 3 genetic subtypes has been reported of which rcdp type 1 , caused by mutations in the peroxisomal biogenesis factor 7 gene , is the most common type . a five years male child presented to the paediatric opd with complaints of recurrent upper respiratory tract infection . on examination he had swelling of the joints for which the child was referred to our opd . examination revealed that the swelling was non - tender , bony hard and did not show any effusion . thorough examination with proper history taking was done which revealed that the child was born of a 3rd degree consanguineous marriage at home after 38 weeks of gestation from the third pregnancy of a healthy 26- year - old mother and 30 yrs old father . currently at 5 yrs of age , weight is 10.2 kgs , length of upper extremities ( 41cms ) and that of lower extremities ( 36.5 cms ) , height is 81 cms , head circumference is 47 cms , chest circumference is 49 cms . the patient had history of upper respiratory tract infection frequently ( once a month or once in 2 months ) . clinically , the child had congenital cataract in left eye and early cataract changes in right eye ( fig 1 ) . facial features showed prominent forehead , widely set eyes and a sunken face ( fig 2 ) . the patient can speak sentence , can run , can dress and undress himself . at 1.5 yrs of age weight by age and height by age was less than 3rd percentile ( fig 4 ) . normal weight should have been around 18 kgs ( weight is 10.2 kgs ) and normal height around 115 cms ( height is 81cms ) . the radiological findings of the patient were compatible with cdp with punctate calcifications in the epiphyses and metaphyseal dysplasia ( fig 5,6,7 ) . it is generally associated with recurrent respiratory infections and breathing problems which may be life threatening . because of their severe health problems , most children with this do not survive long . it is rare for affected child to live past age 10 . the milder form of this disease is characterized milder degrees of rhizomelia along with growth and mental retardation . distinctive facial features such as prominent forehead , widely set eyes , sunken appearance of face , small nose . additionally , almost all affected individuals present with cataract . an individual who inherits one copy of a pex7 gene mutation is a carrier and do not have related health problems . an individual who inherits mutations from each parent , is expected to be affected with rcdp . thus it shows that rcdp is an inherited disease but results only when the genes are inherited from both the parents . if mother and father both are carriers , the chances of getting affected for a child is 25% in each pregnancy ; therefore , it is of utmost importance that the reproductive partner of a carrier be offered testing . a negative does not eliminate the possibility of inheritance of the gene mutation by the child . there is no cure for rcdp . unfortunately , most children do not survive after age of 10 . diagnosis was done mainly on the basis of clinical examination and investigations such as x - rays . we concluded that , management of rcdp is symptomatic and supportive and may include physiotherapy and orthopedic procedures ( in later stages ) to improve function . physiotherapists and occupational therapists can help relieve the symptoms of a child s unusual skeletal development and cataract can be removed surgically .

introduction : rhizomelic chondrodysplasia punctata ( rcdp ) is a very rare disease . it impairs the normal development of many parts of the body . the features of this disorder include bony abnormalities , severe mental retardation , joint contractures , cataract and recurrent respiratory infections and breathing problems . seizures and distinctive facial features including prominent forehead , depressed nasal bridge and small nose is also associated with this pathology . being rare , this is very difficult to diagnose when presented at opd . proper history and meticulous examination is extremely necessary . our aim is to discuss current knowledge on etiopathogenesis as well as radiological and clinical symptoms of diseases associated with rcdp.case report:5 yrs old male child presented with chest infection and periarticular swelling of all the small and large joints . the patient was walking with limp . history elicited that the child was born of a consanguineous marriage . the child was delivered at home . birth weight was 2.4 kgs . he repeatedly had upper respiratory tract infections and was taking treatment for the same . he was further investigated in the form of clinical , biochemical and radiological assessment which stated that the patient was suffering from rcdp.conclusion:this is a rare presentation . though this is not curable , management of rcdp is symptomatic and supportive and may include physiotherapy and orthopedic procedures ( in later stages ) to improve function . the child may also undergo cataract surgery to improve vision .

foreign bodies are frequently ingested by young children , mostly by ambulatory toddlers who explore their surroundings orally . almost 40 percent1 of the ingestion of the foreign bodies are unwitnessed and 50 percent2 are asymptomatic . depending on the size , shape , content and nature , most ingested small objects on reaching the gastrointestinal tract pass through spontaneously3 within 2 - 6 days , but may take up to four weeks . while serious complications such as bowel obstruction or perforation can occur , gastric outlet obstruction by the ingestion of a foreign body is uncommon.45 we report a case of a toddler presenting with persistent vomiting caused by an ingested foreign body obstructing the gastric outlet . a plain radiograph of the abdomen did not reveal the presence of a foreign body ; however , abdominal ultrasonography detected the obstructing foreign object which was extracted by upper gastroscopy . a 19-month old toddler presented with a one- week history of persistent non - bilious vomiting and intolerance of oral fluids and solids . there was no history of diarrhea , choking or the witnessing of the ingestion of a foreign body . physical examination revealed a distended abdomen with increased bowel sounds , but no tenderness and no organomegaly . the vomiting ceased with intravenous hydration and keeping the patient on nil by mouth . as he was restarted on clear oral fluids , the vomiting recurred . flat film of the abdomen showed paucity of gas in the gut which was reported as normal . the suspicion of a faint radio - density projecting over the stomach was seen retrospectively following the results of the abdominal sonography ( figure 1 ) . abdominal radiograph showed a relative paucity of gas in the gut . the faint radio - density ( arrow ) which represented the foreign body an abdominal ultrasound done showed a small echogenic structure localized to the region of the pyloris . the structure was obstructing the gastric outlet ( figure 2 ) as evidenced by a distended fluid filled stomach . abdominal sonogram demonstrated the presence of a small echogenic structure casting an acoustic shadow localized to the region of the pylorus . the structure appeared to be creating a gastric outlet obstruction as evidenced by a massively distended fluid filled stomach . the procedure revealed a normal but hugely dilated stomach with a funnel - shaped plastic foreign body impacting and completely obstructing the pylorus ( figure 3 ) . the patient was discharged home 24 hours following gastroscopy on a regular diet and ranitidine for mild esophageal erosions . the specimen was examined by the pathologist who confirmed a pink funnel - shaped structure measuring 2.322 centimeters . similar plastic objects are typically fixed to balloons as handles and are readily available to children . on upper gastroscopy , the stomach looked normal but hugely dilated with a foreign body partly impacted in the pyloric channel . the major part of the foreign body was lodged in the stomach extracted specimen was identified as a plastic funnel - shaped structure , pink in color measuring 2.322 cm with bite marks . once past the lower esophageal sphincter ( les ) , the majority of regularly - shaped smooth foreign bodies usually pass spontaneously through the gastrointestinal tract within 4 - 6 days , but may take up to 4 weeks . while 80% of ingested foreign bodies pass through spontaneously , 10 - 20% require intervention and 1% need surgical removal.6 conventional radiography will identify most radio - opaque metallic objects , but may not detect radiolucent objects such as plastic objects , wood , glass , fish or chicken bones . hence , the diagnosis of radiolucent foreign bodies in the gastrointestinal tract can be challenging . oral contrast studies have been utilized to locate ingested non - opaque foreign bodies which appear as filling defects.3 however , oral contrast studies have the potential risk of aspiration in cases of a high grade obstruction or poor control of secretions . they are to be avoided if esophageal perforation is suspected . if perforation is suspected and a study is necessary , an isotonic contrast agent may be used , whereas gastrograffin and barium are contraindicated . sonography , unlike conventional radiography , is not dependent on radiographic density and does not involve ionizing radiation . despite its ease of use and relative low cost , sonography has been considered a poor imaging modality to detect abdominal foreign bodies as an air - filled bowel is difficult to visualize . the foreign object elicits its own primary signs by casting an echogenic mass with a dense acoustic shadow . the secondary signs due to tissue edema or hemorrhage result in a hypoechoic area surrounding the foreign body . distension of the stomach or the bowel segments is a further important secondary sign of an obstructing foreign body . ginsburg et al12 demonstrated that sonography is the most reliable method of detecting non - opaque soft tissue foreign bodies . caspi et al13 revealed that sonography is effective in detecting foreign bodies in vaginal orifices . isaac14 reported the detection of a ball point pen by abdominal ultrsonography in a 4-year - old female . asad et al15 diagnosed a case of appendicitis due to an accidentally ingested endodental file which lodged in the appendix . the graded compression technique was used in abdominal sonography to detect the precise location of the missing object . in a 5-year - old boy , ileal perforation occurring secondary to an ingested fragment of a skewer was identified by rathaus et al16 by using abdominal sonography . the abdominal sonogram revealed the presence of a hyperechoic straight - line foreign body embedded within a hypoechoic inflammatory mass . an earlier case of gastrointestinal perforation due to a swallowed toothpick was recognized sonographically by chau et al.17 in a review of 23 cases of abdominal foreign bodies diagnosed sonographically , kuznetsov et al18 concluded that ultrasonic examination provided the maximal information about sizes , structure , location of foreign abdominal bodies , their depth of location and relationship with abdominal organs . this case report adds to the increasing evidence that the skilled use of ultrasonography , a noninvasive painless diagnostic modality , can detect abdominal foreign bodies . ingestion of foreign bodies should be considered in the evaluation of persistent vomiting in a toddler . some objects may not be radio - opaque and may be difficult to find by plain radiographic views . this report , along with the literature review , shows that abdominal ultrasonography is preferred to plain film19 and oral contrast studies in the diagnosis of suspected foreign body ingestion .

the diagnosis of unsuspected foreign body ingestion is a common problem in children . we describe a toddler who presented with persistent vomiting and dehydration . a plain radiograph of the abdomen did not reveal a foreign body . however , abdominal ultrasonography promptly identified a funnel - shaped foreign body obstructing the gastric outlet . this was extracted by upper endoscopy . a recent review of the literature shows increasing evidence that abdominal ultrasonography is an equally complementary diagnostic modality for ingested foreign bodies in children .

sit - to - stand ( sts ) is a frequently performed movement in independent living . dall and kerr ( 2010 ) reported that sts is the most frequent movement of sedentary workers1 . if the sts is disturbed by improper technique or factors restraining the human musculoskeletal system , repetitive movement of sts might contribute to prevalence of musculoskeletal dysfunction2 , 3 . general usage of waist belt is to emphasize a slender waist as well as to hold pants at the waist level . the wearing tight garments could be a risk factor for digestive problems , increasing the incidence of bowel discomfort4 . however , the influence of a general waist belt on the musculoskeletal system has not yet been investigated . we hypothesized that wearing a tight - fitting waist belt , which is commonly done in daily lives , influences the human musculoskeletal system during sts , forcing the abdominal muscles to work together differently , creating different functional synergies . the purpose of this study was to determine the effects of the tightness of a general waist belt on pelvic kinematics during the sts . this study subjects were 12 healthy male students aged 2027 years ( 23.85.7 years , meansd ) , whose height and weight were 175.45.2 cm and 66.12.1 kg , respectively . subjects with conditions that might have affected trunk mobility , such as injury or neurological deficits of the hip and lower extremities , during the previous one year , were excluded from the study . this study was approved by the inje university faculty of health sciences human ethics committee . the general waist belt used in this study was made of layers of leather and had an adjustable buckle ; the width and thickness of the belt were 3 cm and 3 mm , respectively . the tightness of the belt was adjusted using the circumference of each subject s waist . there were three belt conditions in this study : no belt , the belt tightened to 100% of the patient s waist circumference , and the belt tightened to 90% of the patient s waist circumference . data were collected at a sampling rate of 100 hz with a motion - capture system ( vicon mx , oxford metrics , oxford , uk ) that consisted of eight infrared cameras . sixteen reflective markers were attached to the lower body according to the plug - in - gait marker set ( oxford metrics ) using double - sided tape . the software used for kinematic data collection was nexus 1.4.1 ( oxford metrics ) and the data were analyzed with polygon 3.1 software ( oxford metrics ) . the experimental protocol required the completion of two sts trials for each of the three belt conditions . each subject was asked to stand up at a self - selected speed from the seated position with an erect - spine posture . the phase of the sts commenced when the right pelvis was flexed at least 0.1 anteriorly , and ended when the right pelvic angle was maintained for at least three frames ( 0.03 s ) in the standing posture , or when the reduction in the right pelvic angle had stopped . the sts movement cycle for time normalization is expressed as sts from movement onset , 0% to end , 100% . the change in pelvic angle was calculated as the difference between the maximal pelvic flexion angle and the initial pelvic angle . the subjects were given 3 min of practices and 1 min of rest prior to each data acquisition trial . for the analysis , we used a within subject design , and one - way repeated - measures anova was conducted to test for differences in pelvic kinematic values during the maneuver . significant main differences appearing in pairwise multiple comparison , were treated with the bonferroni correction to identify specific differences . significance was accepted for values of p<0.05 , and spss version 12.0 ( spss , chicago , il , usa ) was used for statistical analyses . the change in the anterior tilt of the pelvic angle differed significantly with the tightness of the waist belt during the sts maneuver . the change in the anterior tilt of the pelvis was significantly greater in the 90% waist belt condition ( 36.6 6.0 ) than in the no - belt condition ( 33.3 6.1 ) , but it did not differ significantly between the no - belt condition and 100% waist belt condition ( 33.1 4.8 ) ( p<0.05 ) . theoretically , sts is the initiation of flexion of the trunk and hips to bring the center of mass forward , followed by symmetrical extension of the lower - limb joints and trunk extension to raise the body mass in a vertical direction over the feet6 . wearing a tight waist belt a possible explanation for this is that the tight waist belt might have increased abdominal pressure by narrowing the waist circumference , which might have decreased the space within the abdominal cavity . elevated abdominal pressure has been shown to cause multi - directional stiffness of the spine7 , 8 . tightness of a waist belt might restrict forward movement of the center of mass , increasing pelvic inclination through a compensatory mechanism . this mechanical change would increase the lever arm between weight and fixed foot necessitating a greater force for extension of the knee joint . habitual sts while wearing a tight belt may nterrupt normal lumbo - pelvic coordination as well as increase the load on the knee joint , contributing to muscle imbalance . increased abdominal pressure due to a tight waist belt might counteract the required contraction of the erector spinae . a previous study , using a wide belt , reported that the intramuscular pressure on the erector spinae influenced spinal stiffness separately from muscle activation9 . although a narrow belt did not completely restrict trunk movement , pressure elevated by the tightness contributed to spinal stiffness . our results indicate that wearing a tight waist belt may contribute to the development of excessive anterior pelvic tilt . however , the present study did not conduct against specific skeletal structure , and was conducted with young adult males with a normal body mass index . the effects of tight waist belts need to be investigated in further studies with female subjects and subjects with obesity .

[ purpose ] the purpose of the present study was to evaluate the effect of a tight waist belt on the human musculoskeletal system by assessing sagittal pelvic kinematic data during the sit - to - stand ( sts ) maneuver . [ subjects ] twelve asymptomatic males were recruited and three belt conditions were used during the sts . sagittal kinematic data of pelvic motion were collected using a 3d motion - capture device [ results ] the changes of the anterior pelvic tilt during the sts were significantly greater in the tight waist - belt condition than in the no - belt condition . [ conclusion ] the results of this study show that wearing a tight waist belt increases anterior pelvic motion .

fabry disease ( fd ) is a rare x - linked disorder resulting from a deficient activity of the lysosomal enzyme -glycosidase a ( -gal a ) that leads to progressive accumulation of glycosphingolipids in several tissues and organs . in the classical form of the disease , renal involvement commonly ensues in the second to third decades of life with overt proteinuria ( > 300 mg / day ) rapidly leading to chronic kidney disease ( ckd ) and end - stage renal disease ( esrd ) . cardiac and renal atypical variants of the disease with different degrees of organ impairment and the pattern of disease have been described in males . in females , the disease presentation may range from a completely asymptomatic status to the classic form of fd . nonetheless , to the best of our knowledge , isolated proteinuria as the first sign of the disease is rare and has been described only in males . enzyme replacement therapy ( ert ) with agalsidase has been demonstrated to be safe and effective in the improvement of several signs and symptoms of fd in both genders , and it is accepted that the early establishment of ert might prevent organ damage . thus , ert is recommended in the presence of symptoms or organ involvement . however , due to the variable presentation and the slower progression of the disease in female patients , the questions of how to detect organ involvement and when to start therapy remain open . vv , a 20-year - old female was referred to our clinic in 2006 for a family screening programme due to one uncle , the mother and sister being diagnosed with fd [ point mutation i354k ( c1061t > a ) of the exon 7 ] . at that time , vv was completely asymptomatic for fabry disease ; leucocyte -gal a activity was 19.5 nmol / mg prot / h ( normal values 1850 nmol / mg prot / h ) , while molecular analysis showed the presence of the pathologic genotype , confirming the heterozygous status of the patient . at this time , clinical evaluation and periodic laboratory tests were unremarkable and no sign of fd could be detected . two years later in the first evaluation , urine analysis showed evidence of isolated persistent microalbuminuria ( 303 mg / l ; normal value < 30 mg / l ) without overt proteinuria or abnormalities in the urinary electrolytes . all laboratory tests were in the normal range including serum creatinine and creatinine clearance ( 0.7 mg / dl and 92 ml / min , respectively ) . similarly , a thorough physical examination of the patient could not elicit any sign of fd or any other disease . specifically , body mass index , blood pressure and heart rate were 20.5 kg / m , 120/70 mmhg and 68 bpm , respectively . audiometric evaluation did not show any evidence of hearing loss . magnetic resonance imaging ( mri ) of the brain was negative for infarctual encephalopathy . finally , both sweat test and electromyography were normal , with no evidence of sensitive - motor abnormalities . due to the presence of isolated and persistent microalbuminuria light microscopy revealed numerous vacuolated cells in both glomerular tuft and tubular cells ; myelin osmiophilic bodies were also present in glomeruli or between tubules ( figure 1a ) . direct immunofluorescent study showed negative staining for iga , igm , igg , c1q , c3 and c4 . zebra bodies in podocytes , epithelial and endothelial cells of the glomerular tuft ( figure 1b ) . thus , the renal biopsy confirmed that the patient presented fd with the sole involvement of the kidney . considering the aggressive course of the disease in the family , the optimal blood pressure control and what was suggested by the international guidelines for fd diagnosis and treatment , we started ert with agalsidase beta ( fabrazyme - genzyme corp . ) at a standard dose of 1 mg / kg every 2 weeks , in the hope to provide vv with an aetiological rather than antiproteinuric treatment . after 6 months of treatment , the microalbuminuria returned to the normal range ( 27 mg / l ) , while no other signs or symptoms of fd could be detected . renal biopsy of an asymptomatic fabry female patient presenting with isolated microalbuminuria ( 303 g / l , 24 h urine collection ) . light microscopy ( panel a , left ) shows numerous vacuolated cells in glomerular tufts ( upper panel ) and tubuli ( lower panel ) . tem ( panel b , right ) shows osmiophilic zebra bodies in podocytes and epithelial cells ( upper panel ) and endothelial cells ( lower panel ) . several studies have demonstrated that heterozygous patients , due to the lyon phenomenon , may present different phenotypes ranging from the complete absence of symptoms of the classical form of fabry disease . to the best of our knowledge , this is the first case reported of an adult heterozygous female in whom isolated microalbuminuria is the first and only presenting sign of both fd and renal involvement . the presentation of fd with a prevalent renal impairment ( so - called renal variant ) is rare and documented in male patients only . nonetheless , microalbuminuria has been recently described as a preliminary sign of renal impairment in symptomatic young female patients . it is the authors opinion that the present case report should question the watch and wait approach and pose several questions on what should be looked at as early signs of fd and when to start ert . indeed , the early detection of renal damage appears of paramount importance since the presence of proteinuria and/or ckd at the time of the start of ert are independent predictors of poor outcome . however , our case should suggest that microalbuminuria might be regarded as an early sign of fd nephropathy . furthermore , the disappearance of microalbuminuria after 6 months of ert without antiproteinuric agents [ such as angiotensin - converting enzyme inhibitors ( ace - i ) or angiotensin receptor blockers ( arbs ) ] should deserve attention since it might suggest that early intervention might also reverse fd nephropathy . this finding might be of importance if we consider data from the fabry registry that seem to corroborate the notion that females with fd are undertreated , as only 33.8% of females currently included in the fabry registry are on ert . in summary , this case suggests that the kidney may be the first , and sometimes only , organ damaged by fd in heterozygous female patients and that isolated and persistent microalbuminuria instead of proteinuria should be regarded as an early marker of fd nephropathy and an indication for ert . renal variant, as well as how to detect organ damage and when to start an early treatment in female fd patients .

fabry disease ( fd ) is a rare x - linked disorder characterized by low or absent activity of the lysosomal enzyme -glycosidase - a that leads to progressive accumulation of glycosphingolipids in different organs and tissues . clinical manifestations vary from classic to atypical forms characterized by one prevalent organ involvement , and a renal variant has been described in men but not in women . however , little is known about renal manifestation in females affected by fd . we herein report a case of a 22-year - old female with isolated and persistent microalbuminuria as the only sign of fd . in light of the importance of early recognition and treatment of fd organ damage , this case should call for future studies to determine how to assess organ damage , investigate the existence of a renal variant in fd female patients and determine when best to start enzyme replacement therapy ( ert ) .

complications during percutaneous coronary intervention ( pci ) include coronary artery complications ( perforation , distal embolization , side branch occlusion , and stent thrombosis ) and vascular complications ( puncture site hematoma , retroperitoneal bleeding , and atheroembolism ) . among them , stent entrapment with resulting stent deployment failure is rare but can cause fatal conditions such as stent thrombosis or myocardial infarction . although many methods have been used for stent retrieval , there are few reports of the use of a snare via a contralateral femoral approach . here we report one case of half - degloved stent entrapment that occurred during pci and that was successfully treated by a snare via a contralateral approach . an 80-year - old male was admitted owing to recurrent chest pain for elective pci of a chronic total occlusion ( cto ) in a left circumflex ( lcx ) lesion . six months previously , the patient had undergone emergent pci in the left anterior descending artery ( lad ) owing to unstable angina . follow - up coronary angiography revealed no significant in - stent restenosis in the lad and cto in the lcx ( fig . a 7-fr jl4 guiding catheter was engaged and predilation with a 2.020-mm balloon was done . after ballooning , we attempted to insert the 2.530-mm resolute - integrity ( medtronic , minneapolis , mn , usa ) stent into the lcx , but the stent failed to cross the cto lesion in the lcx . we used the deep intubation technique , but the stent could not cross the lesion entirely because the lesion was severely calcified and angulated . while attempting to push the stent , we noticed that the stent was stuck within the lesion . after a struggle , the guiding catheter was taken out but we observed that the stent was half - degloved off the balloon ( fig . because of the risk of stent loss and embolization , we decided to pull out the catheter and stent strut by use of an amplatz gooseneck snare ( ev3 , plymouth , mn , usa ) . we pushed down the guiding catheter and stent strut together to the iliac bifurcation level . the left femoral artery was punctured and we used a 6fr balkin sheath to grab the stent with the snare and both the stent strut and the balloon catheter were removed successfully by the snare ( fig . we completed the procedure and decided on medical therapy instead of pci for the lcx lesion . two days later , the patient was discharged and there have been no adverse events during 3 months of clinical follow - up . entrapment of coronary angioplasty devices , especially the stent strut , is rare but is a serious complication of pci for which cardiac surgery is sometimes required . calcified , long lesions and angulated lesions may predispose to entrapment of the stent strut . the incidence of stent dislodgement or migration ranges from 1.4% to 8%.1 - 4 to prevent stent dislodgement or migration , predilatation with a balloon catheter or use of stents with a smaller profile and good tractability are recommended in case of severe angulated or calcified lesions , as in this patient.5 a variety of stent retrieval methods have been described in the literature , such as using a snare device , a multipurpose basket , a variety of forceps , an angioguard ( a distal protection device ) , a simple balloon and just crushing of the stent into the vessel wall by a balloon.6 - 10 however , using a balloon or a snare to rescue an entrapped stent in a half - degloved state may contribute to stent loss ; to prevent stent loss , it would be safer to approach both sides by inserting the snare via another vascular route . in this case , we pushed down the stent strut and guiding catheter together to the iliac bifurcation level . after that , the contralateral femoral artery was punctured and both stent strut and balloon catheter were removed successfully by a snare with the aid of a 6fr balkin sheath . we think that this technique is safer than other methods in this situation to prevent stent loss and embolization . however , to avoid serious complications , interventional cardiologists should keep in mind the various complications during pci and should understand the lesion anatomy and characteristics in their daily practice .

although stent entrapment is a rare event during percutaneous coronary intervention , stent entrapment can cause stent breakage or loss , which results in fatal complications such as stent embolism or acute myocardial infarction . we report one case of stent entrapment that was successfully treated by a snare via a contralateral transfemoral approach .

the gonadotropin releasing hormone ( gnrh)-dependent activation of the hypothalamic - pituitary - gonadal axis leads to central precocious puberty ( cpp ) . the extrapituitary secretion of gonadotropins or secretion of gonadal steroids independent of pulsatile gnrh stimulation may lead to pseudoprecocious puberty , or gnrh - independent sexual precocity . incomplete isosexual precocity is a consequence of premature increased sex hormone secretion , iatrogenic exposure of gonadal steroids , mccune albright syndrome , juvenile hypothyroidism in either sex , and , in boys , rarely hcg- or lh - secreting tumors . van wyk - grumbach syndrome ( vwgs ) is characterized by juvenile hypothyroidism , delayed bone age , and isosexual precocious puberty with reversal to a prepubertal state following thyroid hormone replacement therapy . we report a girl with long - standing , untreated hypothyroidism who presented with precocious puberty . a girl aged 10 years and 9 months presented with progressive breast enlargement and menarche at 7 years of age . she was born of nonconsanguineous marriage at full - term , normal vaginal delivery , and was first in the birth order . there was no history of headache , vomiting , visual symptoms , and gelastic episodes . her appetite was normal and she did not have excessive somnolence , cold intolerance , or constipation . her height was 114 cm ( < 3centile , target height of 165.2 cm ) and weight was 25 kg . as per tanner 's staging , her sexual maturation score was b 4 and p3 for breast and pubic hair , respectively , and axillary hair were present . her iq was 76 ( low average ) with a verbal iq of 73 and performance iq of 78 . she had normocytic normochromic anemia with a hemoglobin level of 9.8 g / dl ( normal 1214 g / dl ) . hormonal investigations revealed tsh > 150 iu / ml ( 0.35 5.5 ) , t3 47.5 pg / ml ( 60181 ) , t4 1.0 ng / ml ( 4.512.6 ) , through radioimmunoassay , and fsh 6.78 miu / ml ( 0.32.0 ) , lh < 0.07 miu / ml ( 0.16.0 ) and prolactin 185.8 ng / ml ( 2.8 - 29.2 ) , through electrochemiluminescence immunoassay . her radiological investigations revealed a bone age of 7 years ( greulich and pyle 's atlas ) . ultrasonography of the pelvis showed a uterine size of 5.4 3.2 3.6 cm with enlarged multicystic ovaries ( right ovary measuring 7.9 4.6 cm and left ovary 4.2 3.9 cm ; figure 1 ) . the mri scan of sella revealed a sellar mass with suprasellar extensions of 1.7 1.6 1.2 cm size [ figure 2 ] . the radioiodine nuclear thyroid scan did not reveal any functioning thyroid tissue in the neck or chest . during follow - up repeat ultrasonography revealed complete regression of ovarian cysts with normal sized ovaries at 1 year . the presence of precocious puberty and enlarged ovaries suggested an estrogen - secreting ovarian tumor in the present case . but the finding of a delayed bone age in the patient with precocious puberty narrowed the differential diagnosis to long - standing hypothyroidism . high circulating levels of tsh along with prepubertal lh levels suggested van wyk grumbach syndrome . in girls , the condition usually presents with vaginal bleeding , and uncommonly with breast development or galactorrhea . despite an early stage of puberty , there is lack of pubic hair . the salient diagnostic features include long - standing hypothyroidism , high levels of tsh , isosexual precocity with lack of public and axillary hair growth , and delayed bone age . sella turcica enlargement may be seen at times and it has been attributed to thyrotroph hyperplasia . thus , vwgs can be diagnosed nonoperatively , by the recognition of the salient clinical features and appropriate confirmatory endocrine laboratory tests . van wyk and grumbach postulated a lack of specificity in the feedback mechanism leading to an overproduction of multiple hormones . the serum gonadotropin levels in these patients are relatively low for their degree of gonadal stimulation . immunological activity is present but these gonadotropins are biologically inactive in an in vitro assay . thus , elevated gonadotropins alone can not completely explain the gonadal stimulation seen in severe juvenile hypothyroidism . tsh levels are consistently elevated in such patients and the tendency to manifest sexual precocity may be directly related to the severity of tsh elevation . high circulating levels of tsh acting directly on fsh receptors may be the actual mediator of precocity . using recombinant tools , it has been shown that human tsh can interact with the human fsh receptor to stimulate the adenylyl cyclase activity . human recombinant tsh at a dose about 1000-fold greater than hfsh evoked a dose - dependent cyclic amp response in chinese hamster ovary ( cos-7 ) cells transfected with the human fsh receptor thus suggesting that relatively low fsh - like activity of tsh can be clinically significant at very high concentrations of tsh present in severe primary hypothyroidism . a direct effect of severe hypothyroidism on the prepubertal testis , which leads to over proliferation of sertoli cells is responsible for macroorchidism in males . in females , the multicystic ovaries may result from elevated levels of circulating gonadotropins acting on it . it is also possible that increased sensitivity of the ovaries to the circulating gonadotropins could result from the hypothyroid state directly or via increased prolactin . our patient also had multicystic ovaries with normal to low gonadotropins , suggesting that the increased sensitivity of ovaries to gonadotropins may be responsible for it . enlarged pituitary was probably because of thyrotroph hyperplasia . in patients with isosexual pseudo precocity , the presence of palpable adnexal mass would suggest ovarian tumors but in all such cases , the bone age is advanced . hence , the presence of a delayed bone age in patients with precocious puberty is an important clue for the diagnosis of vwgs . although there is little consensus regarding the precise etiopathogenesis of the disorder , the treatment approach is clear . all symptoms subside with thyroxine replacement , the endocrine abnormalities resolve , and even the ovarian cysts decrease in size or altogether disappear , as also in the present case during follow - up .

van wyk - grumbach syndrome ( vwgs ) is characterized by juvenile hypothyroidism , delayed bone age , and isosexual precocious puberty . a 10.7-year - old girl presented with premature menarche and isosexual precocity . she had delayed bone age and multicystic ovaries . high circulating levels of tsh with prepubertal lh confirmed the diagnosis of vwgs . tendency to manifest sexual precocity in vwgs may be directly related to the severity of the tsh elevation . high circulating levels of tsh acting directly on fsh receptors are the actual mediators of precocity .

renal transplantation prolongs survival and improves quality of life for most patients who require renal replacement therapy . . epstein - barr virus ( ebv ) , cytomegalovirus ( cmv ) and varicella zoster virus ( vzv ) can all cause serious illnesses in transplanted patients . the spectrum of illness caused by ebv ranges from an acute infectious mononucleosis - like illness to a highly malignant b cell tumour . we describe a patient who developed a lymphoma after her second transplant in whom long - term remission has been achieved by reduction in immunosuppression and the use of rituximab , a monoclonal antibody with activity against b lymphocytes . this prompted us to test for susceptibility to ebv infection in the scottish adult renal transplant pool . a 38-year - old woman presented with left leg , tiredness , sore throat and sweats , 16 months after a second cadaveric renal graft . positive anti - vca igm and negative ebna igg supported a diagnosis of glandular fever . her underlying diagnosis was focal segmental glomerulosclerosis , an early recurrence of which had led to the loss of her first graft . her second transplant was perfectly matched , but highly sensitized , so she had been given basiliximab as induction therapy followed by prednisolone , tacrolimus and mycophenolate . imaging by ultrasound and ct showed a 6 cm soft tissue mass inferior to the transplanted kidney , encircling the femoral vessels . a diagnosis of monoclonal polymorphic high - grade non - hodgkin 's lymphoma was made by a ct - guided biopsy ( figure 1 ) . the cells in this tumour were confirmed immunohistochemically as lymphocytes of b - cell origin by their cd20 and cd79a positivity . in situ withdrawal of tacrolimus and mycophenolate followed by infusion of rituximab 375 mg / m once weekly for 4 weeks led to a significant reduction in tumour size . when last seen at the clinic 6 years after her initial presentation with post - transplant lymphoproliferative disorder ( ptld ) , serum creatinine was 137 mol / l with the estimated gfr of 38 mls / min and the urine protein : creatinine ratio of 86.5 mg / mmol . this patient 's case prompted us to test for susceptibility to ebv infection in the scottish adult renal transplant pool . we obtained a list of patients who were active on the renal transplant waiting list in july 2007 through the scottish renal registry and uk transplant , and then tested their most recent stored blood for ebv igg viral capsid antigen and cmv igg vca if not already known . nine ( 1.8% ) of these were ebv igg vca negative and one was equivocal . seven ( 78% ) of the nine patients who were ebv - negative were also cmv negative . our survey showed that 1.8% of scottish patients awaiting renal transplantation are susceptible to ebv infection , and therefore , at risk of ptld . this is comparable to population studies showing ebv seronegativity in up to 5% of european adults and also to a small canadian survey showing 2 ebv seronegative patients amongst 40 adult transplant recipients ( 5% ) . the main risk factors for the disease are ebv seronegativity and the degree of immunosuppression . ptld is more common in children than in adults because more children are seronegative , and therefore , susceptible to primary ebv infection at the time of transplantation . the incidence of ptld has increased following the introduction of ciclosporin , tacrolimus and newer immunosuppressive agents such as okt3 . the risk of ptld is also 4-fold greater in ebv - negative recipients if they are cmv negative . this is either because cmv acts as a cofactor in the development of ptld or could simply reflect the level of immunosuppression . milder forms of the disease may respond simply to a reduction in immunosuppression although there is no consensus on which drugs to target first [ 35 ] . some recommend cutting the dose of calcineurin inhibitors by half and stopping antimetabolite drugs while continuing prednisolone at < 10 mg / day . patients with more severe forms of ptld are unlikely to respond to a reduction in immunosuppressive therapy alone . previously , chemotherapy and radiotherapy were used with variable results , but recently it has been shown that treatment with rituximab 375 mg / m by once weekly infusion for 4 weeks may induce complete remission . chemotherapy should now be reserved for patients not responding to antibody treatment . despite these advances in therapy , outcome studies suggest a 5-year patient survival of only 51.4% from time of transplantation in renal patients who develop ptld . what then can be done to prevent the emergence of ptld in high risk ( donor ebv positive , recipient ebv negative ) patients ? serial ebv monitoring , tailoring of immunosuppression and antiviral prophylaxis have all been reported to reduce the incidence of ptld . the american society of transplantation nevertheless recommended in 2006 that donor and recipient ebv status should be ascertained prior to kidney transplantation and that ebv viral load should be checked monthly for at least 1 year thereafter in patients who are ebv seronegative . the purpose of this is to allow early detection of first - time ebv infections . similar recommendations were made for checking cmv status . in patients at risk by virtue of their ebv seronegativity , reducing the overall burden of immunosuppression and the strong association between ebv infection and ptld risk suggests a possible prophylactic role for antiviral therapies , though this remains controversial as these drugs affect replicating viruses only . in ptld the incidence of ptld is too low to test antiviral therapies by randomized trial , but a large multicentre , case - control study has suggested up to 83% reduction in risk of ptld depending on the antiviral agent . might also be achieved through the use of immunization against ebv prior to the commencement of immunosuppression . a vaccine using the gp350 ebv envelope protein is currently in phase i / ii clinical trials in the uk .

we report a case of high - grade non - hodgkin 's lymphoma following epstein - barr virus ( ebv ) infection in a 38-year - old renal transplant recipient who was successfully treated with rituximab and remains alive 6 years later with reasonable graft function . we subsequently undertook a survey showing that 1.8% of the scottish adult transplant pool are susceptible to ebv infection . though a vaccine for ebv is currently not yet available , routine screening of potential renal transplant recipients for ebv should help identify those at increased risk of post - transplant lymhoproliferative disorder ( ptld ) , while tailoring of immunosuppression and antiviral prophylaxis with ganciclovir may help reduce the emergence of this potentially life - threatening disease .

post - traumatic cardiac pseudoaneurysm ( psa ) is a rare , potentially life - threatening complication following penetrating cardiac injury . . however , in small and stable postinfarct psa selective non - operative management ( snom ) has been practiced . a 38-year - old hispanic male was admitted to los angeles county + university of southern california medical center after sustaining a single stab wound to the left chest . upon arrival the patient was bradycardic with a systolic blood pressure of 50 mmhg , and glasgow coma scale ( gcs ) score of 3 . the patient underwent simultaneous intravenous access , oral intubation and resuscitative left thoracotomy . after evacuation of 400 ml of blood and clot from the chest a pericardial wound was noted anterior to the phrenic nerve . four additional sutures were required upon restoration of sinus tachycardia to control an exsanguination in the emergency department as the full extent of the defect was not evident during asystole . residual hemorrhage was present when the patient was taken to the operating room ( or ) for definitive repair . in the or , the cardiac wound was reinforced with four pledgeted polypropylene mattress sutures with no residual hemorrhage . attempted chest closure resulted in severe bradycardia and therefore , temporary closure was obtained with a vacuum - assisted closure device and the patient was transferred to the surgical intensive care unit . in total , 24 units of packed red cells , 10 units of fresh frozen plasma , and 6 units of platelets were transfused . on the second postoperative day the early hospital course was complicated by acute renal failure ( peak creatinine 6.19 mg / dl ) requiring dialysis . the patient was extubated on hospital day ( hd ) 10 with no neurological impairment . on hd 24 a cardiac ultrasound was obtained revealing findings consistent with a pseudoaneurysms ( psa ) of the left ventricle . on hd 25 a magnetic resonance imaging ( mri ) confirmed a psa in the lateral wall of the left ventricle measuring 1.01.6 cm without thrombi [ figure 1 ] . six - month follow - up echocardiogram confirmed normal cardiac function and there was no evidence of progression or leakage from the psa . one - year follow - up mri detected a 30% decrease in size of the psa [ figure 2 ] . one - year follow - up echocardiogram showed overall ejection fraction of 55 - 60% with moderate hypokinesis of the anterolateral wall of the left ventricle without septal or valvular lesions . based on the fact that the patient had a small and non - progressing psa , definitive non - operative management was instituted . patients with penetrating cardiac injuries arriving at the hospital hypotensive with cardiac tamponade can be salvaged only with prompt surgical intervention . isolated cardiac stab wounds in patients with vital signs on admission holds the best prognosis after these life - threatening injuries . not uncommonly , atrial septal defects ( asd ) , ventricular septal defects ( vsd ) , valvular insufficiency , and more rarely , development of a ventricular psa may occur . to diagnose these sequelae a psa is a rare complication resulting from a rupture of the ventricular wall , which has been contained by pericardial adhesions or the pericardial wall . the wall of the psa is composed of fibrous tissue and lacks the integrity of the normal ventricle . to date , very few cases of post - traumatic psa have been reported in the literature[468 ] and the time from injury to diagnosis ranged between 5 days and 25 years . these psa may predispose patients to embolic sequelae , rupture and death and therefore have been subjected to prophylactic aneurysmectomy . alternatively , selective non - operative management ( snom ) of this complication has been documented in asymptomatic postinfarct patients with minor ( < 33 cm ) and non - progressing lesions . snom may also be an option in chronic psas ( > 3months ) . in a review of 66 patients , natarajan and colleagues observed that conservative management of a chronic postinfarct psa was not associated with an increased risk of cardiac rupture or poor prognosis . septic ventricular psa has been reported by utley and colleagues where staphylococcus epidermidis , staphylococcus aureus and salmonella have been observed to be the most common pathogens . in these cases , snom is ill - advised . nevertheless , in our patient , psa was unlikely sequela of an infectious source since multiple blood cultures were negative throughout the hospital stay . taking into consideration the risk of stroke due to the potential embolic source associated with cardiac psa , anticoagulation treatment has been recommended , particularly in those with arrhythmias . our case was extensively discussed with cardiac surgery service resulting in no such recommendations and the patient was discharged without anticoagulation . we present , to the best of our knowledge , the first case of a post - traumatic ventricular psa subjected to snom . our case highlights the importance of rapid surgical intervention in penetrating cardiac trauma and the importance of thorough follow - up to detect post - traumatic complication including ventricular psa . to make this diagnosis , postoperative screening with echocardiogram or mri is mandatory as clinical signs and symptoms of developing psa are generally non - specific or , as seen in this present case , absent . a follow - up repeat cardiac echocardiogram at 3 months is our protocol as early edema and thrombosis may preclude detection of a minor asd , vsd , valvular insufficiency or psa . in this case , due to the long hospital stay and a thorough work - up with multiple mris confirming the psa , no 3-month follow - up echocardiography was performed . the cornerstone of the management of this complication is surgical ; however , there may be very selected instances whereas snom is applicable such as in small and stable psas . the current literature , . nevertheless , postinfarction and post - traumatic psas are different entities . in this critically ill trauma patient with a small and stable psa a trial of nom these patients , however , should be surveyed for any signs of progression of the psa . it is important to emphasize that the long - term complications of residual psa after cardiac trauma is poorly understood . therefore , more studies with longer follow - up would be required to assess the effectiveness of the non - operative management as a treatment option in selected patients . finally , there is no role for non - operative management in enlarging psas or in symptomatic patients . we report the first case of a post - traumatic ventricular psa subjected to snom . a cardiac psa is a rare , potentially life - threatening complication after penetrating cardiac injury . in addition to more commonly occurring cardiac complications such as asd or vsd , psa should be screened for in patients after an emergency cardiac repair . snom may be a treatment option in carefully selected patients with small and stable psas .

post - traumatic cardiac pseudoaneurysm ( psa ) is a rare , potentially life - threatening complication after penetrating cardiac injury . early surgical intervention has been the treatment of choice for this sequela due to the risk of rupture . nevertheless , selective non - operative management ( snom ) has been practiced in patients with postinfarct psa that are small and stable . we report a case of a post - traumatic cardiac psa subjected to snom .

brucellosis is a zoonotic disease of worldwide distribution that mainly affects persons working with domestic animals and animal products . despite being controlled in many developed countries , the disease remains endemic in many parts of the world , including latin america , the middle east , parts of africa , and western asia . the clinical presentation can vary from asymptomatic infection with seroconversion to a full - blown clinical picture of fever , night sweats and joint manifestations ; occasionally , there is hepatic , cardiac , ocular or central nervous system involvement . vascular complications , including arteries and veins , associated with brucella infection have rarely been reported . in this paper , a case of deep venous thrombosis ( dvt ) developing after a diagnosis of acute brucellosis in a young milkman is presented . apparently , this case report is the second case of dvt due to brucellosis in turkey . a 26-year - old man presented to our hospital with a 1-week history of pain and swelling in the right leg . the patient 's medical history included a diagnosis of brucellosis 1 week previously in our hospital , where he had presented with complaints of weakness and fever . the rose - bengal test was positive ; the brucella tube agglutination test result was 1/160 . laboratory analyses revealed the following results : white blood cell count 8,000/l , hemoglobin 14.2 g / dl , platelet count 226,000/l , alanine aminotransferase ( alt ) 69 u / l , aspartate aminotransferase ( ast ) 92 u / l . chest x - ray film disclosed the heart and pulmonary vessels to be of normal size , and electrocardiogram ( ecg ) showed a sinus rhythm . color venous doppler ultrasound of the right lower extremity revealed increased diameter of the right main , deep and superficial femoral veins , the popliteal vein and the cranial segments of the deep crural veins ; the lumens of these vessels were occluded with thrombosis . there was no blood flow in the vascular lumen , and there was no response in the veins to compression . rifampicin 600 mg / day per oral , doxycycline 400 mg / day per oral and heparin sodium 20 000 units / day intravenous in divided doses were administered . he was discharged on day seven of hospitalization with regression of complaints and physical examination findings . on discharge , he was prescribed warfarin 5 mg / day , rifampicin 600 mg / day and doxycycline 100 mg / day per oral . brucellosis is a systemic infection that can involve any organ or organ system of the body , and since many cases go unrecognized , the true incidence of the disease is unknown . the onset may be sudden , over a few days , or gradual over weeks to months , with nonspecific symptoms including fever , lassitude , malaise , headache , backache , and arthralgia . vascular complications , including arteries and veins , are rare in brucellosis . in a review of articles , reported cases included abdominal artery thrombosis , portal vein thrombosis and cerebral vein thrombosis [ 2 , 411 ] . the disease is mainly transmitted to humans through the ingestion of raw milk or non - pasteurized cheese contaminated with one of the four brucella species pathogenic to humans . species causing vascular complications have been identified as b. melitensis , b. abortus and b. suis . endothelial damage , hypercoagulability , and stasis have been recognized for over 150 years to be the three elements associated with venous thromboembolism ( vte ) , with often two of these three factors needed for its initiation . therefore , its etiology is multifactorial , with each component having different impacts on different patients . brucella infections may cause dvt due to the following mechanisms : invasion of the surrounding tissue by the infectious agent , inflammation caused by the infectious process in the surrounding tissue , direct damage to the endothelium caused by the infectious agent , and temporary hypercoagulopathy caused by the infectious agent [ 5 , 10 ] . in our patient , there are no abnormal results for the etiological factors that cause thrombosis such as protein c , protein s and anticardiolipin igg and igm . we believe that dvt in our patient was associated with brucella infection , as there were no risk factors for venous thrombosis such as immobilization , history of previous dvt , recent surgical intervention or local tissue trauma . early detection and appropriate treatment of brucellosis are crucial measures to prevent complications of the disease . patients presenting with symptoms of dvt or similar vascular pathologies should be assessed for infectious agents , particularly in those coming from brucella - endemic areas .

brucellosis is a zoonotic disease common in developing countries . vascular complications , including arterial and venous , associated with brucella infection have rarely been reported . a case of deep venous thrombosis ( dvt ) developing after a diagnosis of acute brucellosis in a young milkman is presented . a 26-year - old man presented with pain in the right leg . the patient 's medical history included a diagnosis of brucellosis in our hospital where he had presented with complaints of weakness and fever . peripheral venous doppler ultrasound showed dvt , and the patient was treated with anticoagulants . the patient was discharged with warfarin therapy and anti - brucellosis treatment . although rare , some infectious agents may cause vascular pathologies . patients presenting with symptoms of dvt or similar vascular pathologies should be assessed for infectious agents , particularly in those coming from brucella - endemic areas .

rib osteomyelitis is rare and usually occurs in patients with predisposing factors such as pneumonia and/or lung empyema , chest trauma , chronic granulomatous disease and neutropenia , sickle cell disease , thalassemia or disseminated fungal infections , , , . we report here the first case of early sternum and rib osteomyelitis occurred during breast implant infection managed in an inter - regional referral center for bone / joint infections in the south of france . in july 2014 , a 59-year - old women was admitted in into the infectious disease department of the university hospital in marseille for swollen , erythematous , painful breasts with purulent discharge from surgical wound of left breast prosthesis implantation . fifteen months after weight - loss surgery and advanced diet per bariatric surgery nutritional procedure for morbid obesity ( body mass index 41 kg / m ) , she has significant loss of 43 kg ( body mass index weight 20 kg / m ) . four weeks before her admission , she underwent breast prosthesis implantation , mastopexy and arm lifting surgery in private clinics in the region . one week after surgery , she presented with thoracic pain and purulent discharge from surgical wound . the laboratory investigations revealed high c - reactive protein levels ( 74 mg / l ; normal values 5 mg / l ) , elevated leukocyte count ( 12,000 l , predominantly neutrophil granulocytes ) , low hemoglobin concentration ( 10 breast prosthesis implant have been removed and intravenous antimicrobial treatment of amikacin and ceftazidime was begun . fifteen day later , she had been admitted in our unit for persistent of swelling , reddish , painful left breast and purulent discharge from surgical wound of left breast implant . her body temperature was 37 c , her pulse was 74 beats / min , and her blood pressure was 127/70 mmhg . laboratory investigations revealed normal values for c - reactive protein ( 1 mg / l ; normal values 5 mg / l ) and normal leukocyte count ( 5000 l ) , low hemoglobin concentration ( 10 g / l ) , and normal platelet count ( 300,000 l ) . magnetic resonance imaging ( mri ) has shown an inflammation of anterior chest wall in the left side with sternal osteitis ( fig . she was treated by surgical lavage with debridement , a fistulectomy with costal - chondral resection of infected tissue . a removal of 3rd and 4th left ribs and a partial sternal resection was performed ( fig . analyses for s. aureus and m. tuberculosis by pcr and 16s rna testing were negative . breast implants are implemented both for breast enlargement and for breast reconstruction , e.g. , in women who have undergone mastectomy for breast cancer . the prevalence of infectious complications from breast implants is less than 5% overall but is higher in the case of patients with a previous history of breast cancer treated with radiotherapy and/or immediate reconstructive surgery after mastectomy , . recently , we have reported the first 3 cases of chronic rib osteomyelitis as a complication of chronic breast implant infection which was occurred in 8% of breast implant infections managed in our center from 2008 to 2012 . all of chronic rib osteomyelitis cases reported in our previous study were diagnosed during the surgical breast implant removal without prior diagnostic imaging study . the case reported here is remarkable because the diagnosis of early sternum and rib osteomyelitis was identified by mri and confirmed by in intra - operatively finding at 4 weeks after breast prosthesis implantation . sternal and rib osteomyelitis after breast implantation was a poorly recognized complication , only four cases of osteomyelitis of the rib after breast implantation were reported by including in this case . we supposed that the periprosthetic compartment was primary infected with p. aeruginosa and the infection has spread secondary the ribs and sternum by direct continuity . in this case , round breast implants in silicon with macro - textured surface were inserted in the retro - muscular space at the level of the periosteum in the mammary groove at the same time of mastopexy . three previous cases , round breast implants in silicon with macro - textured surface were inserted in the retro - muscular space at the level of the periosteum through inframammary fold . patient was positioned supine with both arms outstretched on arm boards and breast implants were inserted under general anesthesia . surgical wounds were disinfected every 2 days by povidone iodine and covered with fatty gauze dressings ( jelonet , smith and nephew ltd . ) . pre - surgical preparation and overall skin care was in conformity as recommendation of the french society of hospital hygiene guideline ( http://nosobase.chu-lyon.fr/recommandations/sfhh/2013_gestion_preoperatoire_sf2h.pdf ) . to date , two cases of osteomyelitis of rib after breast prosthesis implantation caused by s. aureus , 1 case of osteomyelitis of sternum and rib after breast prosthesis implantation caused by p. aeruginosa ( this case ) , and one cases of osteomyelitis of rib after breast prosthesis implantation caused by both bacteria have been reported . in general , s. aureus and m. tuberculosis were frequently involved in osteomyelitis of rib , . however , 8 of 37 cases ( 22% ) of breast implant infection managed in our centers over 4.5 years of experience were caused by p. aeruginosa ( data not shown ) . sternum and rib osteomyelitis after breast prosthesis implantation should be considered and investigated by early imaging study which is necessary to adjust the management strategy . in this case , the infection was successfully managed with surgical removal of the 3rd and 4th left ribs and a partial sternal resection was followed by prolong antibiotic treatment . no apparent relapse was observed at the three - month post - antibiotic follow - up . this study was approved by the institutional research ethics board , and a written informed consent form was signed by the patient . the authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in this manuscript .

sternum and rib osteomyelitis complicated from breast implant infection is rare . we report a case of early sternum and rib osteomyelitis occurred during breast implant infection managed in an inter - regional referral center for bone / joint infections in the south of france .

lipoid proteinosis , also known as hyalinosis cutis et mucosae ( omim 247100 ) , was first described by urbach and wiethe in 1929 . since then , over 250 cases of this autosomal recessive disorder have been described . it is characterized by the deposition of an amorphous hyaline substance ( glycoprotein ) in the mucous membrane and skin . hoarseness and thickening of vocal cords are the most characteristic symptoms present from infancy or early childhood.3 , 4 another classic and most easily recognizable sign is the beaded eyelid papules . the other cutaneous changes may include waxy , yellow papules and nodules with generalized skin thickening . the mucosa of the pharynx , tongue , soft palate , tonsils and lips is also infiltrated.6 , 7 other features may include epilepsy and calcification in the temporal lobes or hippocampi . since suturing is difficult because of rigid mucosa in these patients , the use of cryosurgery seems to offer advantages for removing oral nodular lesions . therefore , cryosurgery ( n2o ) was performed to remove and reshape the lip lesions in a case of this rare syndrome . the patient was a 24-year - old female from shahrekord , central iran , who sought medical care at the department of oral medicine , isfahan university of medical sciences in 2003 . the patient was completely edentulous and showed the classic signs of lipoid proteinosis ( figure 1 ) . during oral examination , the patient had six sisters and one brother , and two of her sisters ( 25 and 11 years old ) suffered from the same syndrome . the results of complete blood analysis and routine urinalysis were all within the normal limits . the biopsy of lip lesions was performed with a scalpel under local anesthesia and examined by a pathologist to confirm the diagnosis . suturing after biopsy was difficult because the mucosa was rigid and the sutures cut into it . the patient suffered from some nodules on upper lip . after applying local anesthetic spray on the site , cryosurgery ( n2o , c 502 t , emd co ; tehran , iran ) was performed ( 63c for one minute ) , as this treatment has been used for similar superficial lesions . the surgery was done in two sessions ; half of the lip was involved in each ( figure 2a , b ) . the superficial layers were fallen by day 3 . after the operation , the patient had pain for about a week with a visual analogue scale of up to five . follow up , no new nodules were formed ; however , some fissures were created on her lip ( figure 2c ) . after the treatments , the appearance and the emotional state of this young female had improved . lipoid proteinosis is a rare autosomal recessive disorder accompanied by hoarseness as well as infiltration and thickening of the skin and certain mucous membranes . recently , lipoid proteinosis was mapped to 1q21 and pathogenetic loss - of - function mutations were identified in the extracellular matrix protein 1 ( ecm1 ) gene.2 , 4 some treatments for this disorder have already been reported . a case of lipoid proteinosis was successfully treated with dimethyl sulfoxide , however , in three other cases , it showed no beneficial effect . retinoids have shown encouraging results in cutaneous lesions in previous studies.12 , 13d - penicillamine had a favorable effect in a patient with this syndrome . microlaryngoscopy and dissection of the vocal cords , dermabrasion , chemical skin peeling and blepharoplasty were performed in some cases.5 , 1 lasers are used in many fields of medicine and dentistry . carbon dioxide laser surgery of thickened vocal cords and beaded eyelid papules has proved to be helpful in some studies.2 , 1 nd : yag and er : yag lasers were applied to remove oral lesions in one case of this syndrome . cryosurgery is the use of very low temperatures to destroy tissue . immediately following cryosurgery , tissues are virtually indistinguishable from the normal , but latent damage is produced which progresses to severe destruction of the tissue . it is a safe , effective method of treatment for many benign soft tissue lesions in the oral cavity . as suturing was difficult in this patient and mucosa was rigid , the use of cryosurgery seemed to offer some advantages in removing oral lesions in this case . it seems that surgical methods like cryosurgery and laser1 , 1 have some advantages over conventional surgery for removing oral lesions of lipoid proteinosis syndrome as there is no bleeding and no need to suture , and it is more comfortable for the patient . the described approach is considered an early report of n2o cryosurgery being used in removal and reshaping of lip lesions of this rare syndrome .

lipoid proteinosis is a rare hereditary metabolic disorder transmitted as an autosomal recessive trait . it is characterized by the deposition of an amorphous hyaline - like material ( glycoprotein ) in the mucous membranes , skin and various internal organs . cryosurgery ( n2o ) was applied to remove and reshape the lip lesions in a case of this rare syndrome . the patient was a 24-year - old female . cryosurgery ( 63c n2o for one minute ) was performed to re - shape some areas of the upper lip . the lips were softer and had better esthetics after treatment . the use of cryosurgery offers advantages over surgery in reshaping of the lip lesions in this syndrome , since suturing is not feasible in rigid mucosa of these patients .

a 43-year - old woman presented with recurrent paroxysmal involuntary movements of her right upper limb and quadriparesis . she had four previous relapsing and progressive episodes presenting as right upper limb monoparesis , quadriparesis , and paresthesias during three years . the previous weakness resolved incompletely with steroid treatment . on admission , her wide right forearm - flinging movements , which were occasionally provoked by left knee flexion , lying on the bed , the attacks involved flexion of the right elbow , repeated flexion and extension of the ipsilateral wrist . her only medication was baclofen , which she had been taking for the last three years . she had a quadriparesis ( grade 34 , medical research council ) , worse on the right limbs and distal muscles . rough touch , pain , and temperature sensation were slightly decreased on all extremities , but there were no deep sensory abnormalities . laboratory studies including routine chemistries , various serological markers related with connective tissue diseases , thyroid function tests , and serum and urine copper levels were all normal . csf examination showed mild elevation of protein ( 80.5 mg / dl ) and igg index ( 0.70 ) , but oligoclonal bands and nmo - igg antibodies were negative . median and posterior tibial nerve somatosensory evoked potentials showed no n13 , n20 and p37 . mri revealed diffuse swelling and heterogeneous enhancement of spinal cord from the cervicomedullary junction to c7 ( figure 1 ) . six months after the disappearance of involuntary movement , she developed decreased visual acuity with prolonged latencies of p100 components in visual evoked potentials . the occurrence of ballism - chorea and dystonia implies development of lesions involving the basal ganglia and their afferent or efferent connections , such as thalamus , subthalamic nucleus , basal ganglia , and putamen . however , the unique feature of this patient is choreic movement without responsible brain lesions . ballism has never been reported in spinal cord lesions whereas chorea has been seen very rarely.4 it was uncertain whether she exhibited a pure ballism or chorea but presumably it is not important . while ballism and chorea are distinguishable based upon the type and distribution of movements , they may represent two different symptoms or a spectrum of the same basic disease processes.5 why a patient with a spinal cord lesion develops chorea but not ballism is unknown . ballism , a coarse proximal movement , could evolve into more distal and lower amplitude chorea . athetosis , which is considered a slow form of chorea , often evolves further into dystonia.5 why movement disorders occur in only a minority of patients with myelopathy is also unknown . one mechanism involves the posterolateral cervical cord , the projections from supraspinal centers on the ia inhibitory neurons , and other interneurons concerned with reciprocal inhibition.13 disruption of the somatosensory pathways or motor cortex to the striatum also may produce abnormal movement without sensory loss.1 for our purposes , considering paroxysmal chorea and dystonia together as involuntary movements , we can infer the effect of provocative movements in this patient from the results of previous studies . paroxysmal chorea could be easily provoked by movement , either passive or volitional , and by tactile stimulation.4 it often crosses to the contralateral trunk or limbs . the so called trigger zone was found at either the same spinal segment or below the clinically estimated segment . although this patient had relapsing - remitting weakness , it did not fulfill the conditions of definite nmo when the involuntary movements occurred . however , she must have had nmo , considering the visual symptoms afterwards . despite the lack of lesions located appropriately for the involuntary movements , however , we could not evaluate the effectiveness of such therapy because our patient was treated with steroid and clonazepam . our case suggests that ballism - chorea could present in a spinal cord lesion without involvement of striatum and deep sensory abnormalities . thus , acute exacerbation of recurrent myelopathy in nmo may be associated with transient movement disorders .

movement disorders secondary to intrinsic spinal cord disease are rare . paroxysmal chorea has not yet been reported in the neuromyelitis optica ( nmo ) . we report a 43-year - old woman with relapsing - remitting cervical myelopathy who developed paroxysmal chorea during clinical exacerbation of nmo . mri scan of the cervical spine revealed a long segmental enhancing lesion , but brain mri did not show any responsible abnormalities . acute exacerbation of recurrent myelopathy in nmo may be associated with transient movement disorder .

atomic bomb survivors are known to be at a high risk of developing various malignancies containing sarcomas . we report a case of dedifferentiated liposarcoma in the retroperitoneum in an atomic bomb survivor . a 76-year - old japanese man was admitted to kosei - nenkin hospital ( osaka , japan ) in november 2006 ; his chief complaint was a 10-kg loss in body weight over 3 months prior to admission . he had undergone appendectomy at age 10 and percutaneous transluminal coronary angioplasty at age 70 . at the age of 16 , he had been exposed to radiation from the atomic bomb at hiroshima towards the end of the second world war . physical examination showed a 10-cm mass palpable on the right side of the abdomen , but no peripheral lymphadenopathy was observed . serum white blood cell count and c - reactive protein were elevated to 9,200/l and 4.94 mg / dl , respectively . carcinoembryonic antigen , carbohydrate antigen 19 - 9 , cancer antigen 125 and alpha - fetoprotein showed no abnormalities . abdominal computed tomography ( ct ) showed two 10-cm masses in the right side of the retroperitoneal space . dynamic magnetic resonance imaging ( mri ) showed two 10-cm masses ( tumors 1 and 2 ) and another 5-cm mass ( tumor 3 ) in the retroperitoneum ( fig . 1 ) . tumor 1 at the inferior pole of the kidney appeared as a ring - enhanced mass in the late phase . a t1-weighted image showed a low signal intensity in tumors 1 and 2 but a high intensity in tumor 3 . a t2-weighted image showed a high intensity in tumors 2 and 3 and a slightly high intensity at the center of tumor 1 . a positron emission tomography ( pet ) scan using fluorodeoxyglucose revealed slight uptake ( suvmax : early phase , 2.4 ; late phase , 2.8 ) at the border of tumor 1 , and slight homogeneous uptake ( suvmax : early phase , 2.5 ; late phase , 2.2 ) in tumor 2 , suggestive of liposarcoma . we were unable to establish tumor diagnosis , but we ascertained that these tumors might be malignant . we found that tumors 1 and 2 were present in the retroperitoneum , adjacent to the ascending colon . tumor 3 was also present in the retroperitoneum and was located between the duodenum and the transverse mesocolon . macroscopically , the cut surface of tumor 1 ( 13 10 5 cm , weight 460 g ) and tumor 2 ( 12 8 6 cm , weight 460 g ) was brown , glistening , and mucoid . that of tumor 3 ( 6 4 4 cm , weight 60 g ) was yellowish ( fig . microscopically , spindle fibroblast - like cells were arranged in a storiform pattern in tumors 1 and 2 . the final histopathological diagnosis of tumors 1 and 2 was malignant fibrous histiocytoma ( mfh ) of the retroperitoneum . most parts of tumor 3 were composed of well - differentiated fat cells , which varied in size and shape , and a few lipoblasts were found , which had irregularly shaped nuclei and lipid droplets in their cytoplasm ( fig . he was not administered chemotherapy or radiation therapy because of his heart disease and was discharged on the 14th postoperative day . it is now 13 months after surgery , and the patient is doing well without any evidence of recurrence or metastasis . mfh was first described as malignant histiocytoma and fibrous xanthoma by ozello et al . in 1963 , and it was reported as malignant fibrous xanthoma by o'brien and stout of the same group in 1964 . weiss and enzinger pointed out that mfh was the most common sarcoma in adults , and the concept of mfh arose based on their study conducted in 1978 . mfh is classified as a group of soft tissue sarcomas composed of both histiocytic and fibrous elements arranged in a storiform pattern and accompanied by pleomorphic giant cells , xanthoma cells , and inflammatory cells . however , coindre et al . showed that in 17 of 25 cases in whom mfh in the retroperitoneum was initially diagnosed , dedifferentiated liposarcoma was the final diagnosis . dedifferentiated liposarcomas have components of both well - differentiated liposarcoma and nonlipogenic sarcoma . they mainly occur in the retroperitoneal space , and the most common pattern in the dedifferentiated areas is that of high - grade pleomorphic mfh or storiform fibroblastic mfh . nowadays , many pathologists recognize that most so - called mfhs located in the retroperitoneum are dedifferentiated liposarcomas . in our case , the immunohistochemical staining of tumors 1 and 2 yielded positive results for vimentin and 1-antichymotrypsin , and the tumors were diagnosed as mfh . tumor 3 was diagnosed as well - differentiated liposarcoma because most of it was composed of fatty tissue and few lipoblasts were observed . by the presence of the liposarcoma most area of tumor 3 was composed of adipocytes , which was the reason why tumor 3 was not detected in the preoperative pet scan . several studies showed that radiation therapy increases the relative risk of subsequent sarcomas [ 6 , 7 ] . described the case of radiation - induced liposarcoma which showed dedifferentiation toward pleomorphic malignant fibrous histiocytoma . atomic bomb survivors are known to have a high risk of developing various malignant and benign tumors . a significantly high risk for cancers of the digestive system , breast , gastrointestinal tract , lung , skin , liver , ovary , urinary bladder , and other organs has been demonstrated in such persons . the incidence of non - cancer diseases such as liver cirrhosis and myocardial infarction is also increased . . demonstrated that the incidence of radiation - induced malignant tumors was 38% in atomic bomb survivors with irradiation doses ranging from 1 to 2 sv , and it was 59% in survivors exposed to doses exceeding 2 sv . kamada et al . reported that persons within 500 m from the hypocenter were exposed to irradiation doses ranging from 1.1 to 6.9 sv . the present patient was exposed to radiation within 500 m from the hypocenter , and the irradiation dose was estimated to be more than 1.1 sv . we postulate that in this case , radiation from the atomic bomb may have played an important role in the development of the sarcomas .

a 76-year - old japanese man was admitted to kosei - nenkin hospital ( osaka , japan ) in november 2006 ; his chief complaint was a 10-kg loss in body weight over 3 months prior to admission . abdominal computed tomography ( ct ) and dynamic magnetic resonance imaging ( mri ) showed three masses in the retroperitoneum . the patient subsequently underwent surgery . the final histopathological diagnosis of tumors 1 and 2 was malignant fibrous histiocytoma of the retroperitoneum , and tumor 3 was a well - differentiated liposarcoma . by the presence of the liposarcoma , tumor 1 and 2 were thought to be the dedifferentiated areas of liposarcomas . at the age of 16 , the patient had been exposed to radiation from the atomic bomb at hiroshima towards the end of the second world war . we postulate that in this case , radiation from the atomic bomb may have played an important role in the development of the sarcomas .

the general warm - up has a potentially positive effect on short - term performance1 . this may be because of reduction in joint stiffness2 , increased neurotransmission3 , and differences in the relationship between power and acceleration4 . in addition , disruption of stable links between actin and myosin after warm - up may decrease muscle stiffness and affect short - term performance5 . in - depth investigation of the effects of a general warm - up on respiratory muscle activity has recently been performed , and the therapeutic and benefical effects were noted by the researchers6 . accordingly , we hypothesized that respiratory muscle warm - up may positively affect anaerobic power , and investigated this hypothesis in the present study . , they were familiarized with the maximal inspiratory pressure ( mip ) , wingate anaerobic tests , and respiratory warm - up . during their second and third visits , a wingate anaerobic power test with general warm - up as the control trial ( can ) and a wingate anaerobic power test with general and respiratory warm - up as the experimental trial ( ean ) , were randomly performed . the trials were applied at the same time each day ( between 16:00 and 20:00 h ) . a total of 30 field hockey players ( age , 20.5 2.0 years ; height , 179.3 6.9 cm ; weight , 73.7 12.7 kg ) voluntarily participated in the present study . approval was obtained from ondokuz mayis university clinical research ethical committee ( om kaek 2014/635 ) . for the general warm - up , low - intensity aerobic running for 10 min and dynamic stretching for 5 min were performed by the subjects . for respiratory warm - up , an inspiratory muscle training device ( powerbreathe classic , imt technologies ltd two sets of 30 inspirations were performed at an intensity of 40% of mip with a 2-min rest between each set7 . mip was measured with a respiratory pressure meter8 ( microrpm , carefusion micro medical , kent , uk ) . anaerobic power testing was performed with a cycle ergometer ( 894e peak bike , monark exercise ab , vansbro , sweden ) . data analysis was performed using a statistical program ( spss for windows , version 16.0 , 2008 , spss inc . , significant changes in peak power ( can=767.0 162.9 w , ean=835.1 175.1 w , percent difference=8.9% ) , relative peak power ( can=10.4 1.4 w / kg , ean=11.4 2.0 w / kg , percent difference=9.6% ) , and time to peak ( can=3.9 1.7 s , ean=2.8 1.4 s , percent difference= 28.8% ) were observed between the can and ean trials . previous studies showed that general warm - up may positively affect anaerobic power and performance1,2,3,4,5 . however , respiratory warm - up may affect short - term performance in a different way . in particular , when considered as part of an anaerobic energy system , respiratory warm - up may not have an impact at a physiologic level . an increase may occur with rising core temperature induced by respiratory warm - up . previous research examined the effects of respiratory warm - up on 100-m swimming performance in 15 subjects . , investigated respiratory warm - up and rowing performance in seven male and seven female rowers , and found higher power output during a rowing test after respiratory warm - up7 . studied intermittent sprint performance , and showed that respiratory warm - up resulted in higher power output values than a general warm - up11 . in conclusion , anaerobic power ( peak power ) significantly improved after respiratory warm - up , and peaked faster .

[ purpose ] the aim of the present study was to examine the effects of respiratory muscle warm - up on anaerobic power . [ subjects and methods ] thirty male field hockey players ( age , 20.5 2.0 years ) each participated in a control ( can ) trial and an experimental ( ean ) trial . the ean trial involved respiratory muscle warm - up , while the can trial did not . anaerobic power was measured using the wingate protocol . paired sample t - tests were used to compare the ean and can trials . [ results ] there were significant increases in peak power and relative peak power , and decreases in the time to peak after the ean trial by 8.9% , 9.6% , and 28.8% respectively . [ conclusion ] respiratory muscle warm - up may positively affect anaerobic power due to faster attainment of peak power .

spinal epidural hemangiomas have been reported in the literature , but most of them were cavernous type hemangiomas that enable a preoperative differential diagnosis with relative ease . the limited number of spinal epidural hemangiomas and few radiological findings make an exact diagnosis difficult prior to surgery . a high vascularization of spinal epidural hemangiomas may result in an unexpected surgical situation in the case of preoperative misinterpretation . in the current case , a 51-year - old woman presented with a 3-week history of lower back pain with right anterior thigh numbness . the patient had a magnetic resonance imaging ( mri ) at a local hospital which revealed a lesion at the l3 level , located in the ventral epidural space and connected with l3/4 protruded disc material that demonstrated a heterogeneous signal at t2 weighted images and an iso- to low signal at t1 weighted image ( t1-wi ) . 1 ) . based on a presumed diagnosis of a ruptured disc with possible sequestration or granulation tissue formation , the patient underwent surgery . a right hemilamincetomy of l3 was performed , and a retracting thecal sac revealed a highly engorged vascular structure . near - infrared indocyanine green videoangiography ( icg - va ) showed a delayed mass filling . histological examination revealed a vascular lesion composed of small to medium sized veins with irregular calibers , which is consistent with an arteriovenous hemangioma ( fig . the typical symptoms of epidural lesions other than disc herniation of the lumbar region are low back pain or radiculopathy which are indistinguishable from the clinical symptoms of disc herniation diseases . moreover , spinal epidural hemangiomas are very rare . also , spinal epidural hemangiomas constitute approximately 4% of all epidural tumors and 12% of all intraspinal hemangiomas4 ) . the differential diagnosis for spinal epidural hemangiomas before surgery included schwannoma , lymphoma , meningioma , angiolipoma , disk herniation , synovial cysts , granulomatous infection , pure epidural hematoma , and extramedullary hematopoiesis9,12 ) . the cavernous type displays histologically with large number of sinusoidal channels in collagenous tissue7 ) , whereas the arteriovenous type shows with a cluster of abnormal arteries and veins and vessel walls containing elastin , and smooth muscle5 ) . a complete surgical en bloc removal is the treatment of choice for spinal epidural hemangiomas with mass effect because of the excessive vascularity of hemangioma , piece to piece resection should be avoided6 ) . an indocyanine green videoangiography can help surgeons understand the vasculature surrounding the mass and facilitate the en bloc removal of the hemangioma . fluorescence angiography with indocyanine green provides real - time information regarding the patency of vessels . an icg - va enhances the flow direction delineation capability , flow velocity and sequence of dye filling in different components of complex spinal vascular lesions3 ) . due to the high vascularization of hemangiomas , the incomplete surgical removal of a spinal hemagioma because of diffuse bleeding or minimal exposure during disk surgery might result in the persistence of clinical symptoms or recurrence . reoperation for remnant or recurrent spinal hemangioma is very difficult due to peridural or periradicular adhesion and unclear margins ; as a result , complete resection can not be guaranteed . therefore , proper preoperative planning and complete resection during the operation is essential . for this , but , like this case , an angiography may not always confirm the diagnosis of an artriovenous hemangioma . clinicians should be aware that an angiography can not provide conclusive evidence of the presence of an antriovenous hemangioma . if spinal epidural hemangiomas are unexpected encountered during surgery , an icg - va can be helpful to diagnose and surgery . this technique provides accurate information about the flow dynamics through the anatomy of vascular lesions in real time10,11,13,14 ) . further study is required to recognize and to provide a differential diagnosis of spinal epidural hemangiomas . if the lesion is like a ruptured disc in mri , we should consider spinal epidural hemangioma as one of differential diagnosis .

a spinal epidural hemangioma is rare . in this case , a 51 year - old female patient had low back pain and right thigh numbness . she was initially misdiagnosed as having a ruptured disc with possible sequestration of granulation tissue formation due to the limited number of spinal epidural hemangiomas and little - known radiological findings . because there are no effective diagnostic tools to verify the hemangioma , more effort should be put into preoperative imaging tests to avoid misdiagnosis and poor decisions ) .

a 40-year - old homosexual man with history of adpkd was admitted to palermo university hospital in july 2012 complaining of high fever , headache , and general malaise . physical examination showed oral candidiasis , seborrheic dermatitis , skin lesions on the trunk compatible with kaposi s sarcoma and hepatosplenomegaly . blood tests on admission showed severe renal failure with a creatinine clearance of 18 ml / min . the abdomen ct scan showed bilateral multiple renal cysts that were replacing the parenchyma ( fig.1 ) . hiv infection was diagnosed by enzyme - linked immunosorbent assay ( elisa ) and confirmed with western blot assay . cd4 + lymphocyte t - count was 13 cells / mm ( 1.6% ) , with cd4/cd8 ratio of 0.02 ; hiv - rna viral load was 936.000 copies per ml . the patient promptly started haart with daily darunavir / ritonavir 800/100 mg and abacavir / lamivudine 600/300 mg , and prophylaxis for opportunistic infections with trimethoprim / sulfamethoxazole 160/800 mg daily and azithromycin 1200 mg once a week . after one month into haart , the hiv - rna load had markedly decreased to less than 250 cpm and cd4 + count had raised to 83 cells / mm ( 4.9% ) . during the follow up , the laboratory tests showed a descending trend of creatinine clearance values and metabolic acidosis . in june 2014 hiv rna level was undetectable and cd4 + count was 187 cells / mm ( 17% ) and , in anticipation of hemodialysis , haart with abacavir / lamivudine was replaced with raltegravir 400 mg twice a day due to raltegravir being removed by hemodialysis only in minimal quantities . the patient started hemodialysis three times a week in october 2014 and it was not possible to place him on the transplant list because of a cd4 + cell count under 200 cells / mm despite undetectable hiv rna level . though virologically suppressed , the patient has not yet been transplanted because his cd4 + count remains below 200 cells / mm . esrd is a serious complication of chronic hiv infection that carries significant morbidity and mortality . initially , hiv infection was considered an absolute contraindication for transplantation . since 1996 when haart became widely available and the prognosis of hiv infection dramatically improved , many transplant programs have reevaluated the policies regarding the exclusion of patients with hiv infection . nowadays , kidney transplant has become a viable alternative for hiv infected individuals with esrd since it is associated with better quality of life , fewer medical complications , longer survival and lower cost than chronic dialysis treatment , , . obviously , current indications and contraindications for transplantation also apply to hiv - infected patients . in addition , a cd4 + cell count above 200 cells / mm is required for all organs ( with the exception of liver , that has a lower requirement of 100 cells / mm ) , as well as an undetectable hiv rna level , and a stable potent antiretroviral regimen for at least three months . haart allows the reconstitution of immune functions in most treated hiv patients , but sometimes discrepant responses may occur , including failure to achieve a significant increase in circulating cd4 + t cells despite undetectable plasma viral loads , with a substantially increased long - term mortality for all causes of death . the relevance of this case is to focus on the condition of a viro - immunological dissociation which in fact does not allow the inclusion in the transplant list . our patient has not yet been transplanted because , although he remains virologically suppressed , his cd4 + count remain below 200 cells / mm3 . indeed , lymphopenia frequently occurs in hemodialysis patients waiting for kidney transplantation ; it could be related to an increased turnover of lymphocytes , to a disturbance in lymphocyte homeostasis due to uremia , and/or to increased peripheral lymphocyte apoptosis associated with an activation stimulus . in fact , esrd is associated with premature aging of the t - cell system , and even if the consequence of esrd related accelerated immunesenescence are mostly unknown , it is reasonable that may worsen the immunological status of the hiv infected patient . for all these reasons we believe that lymphopenia should not contraindicate kidney transplantation in selected hiv infected patients with a cd4 + count < 200 cells / mm if hiv rna level is undetectable and the patient is doing a stable potent antiretroviral regimen . several studies have shown that with sustained suppression of viral replication , pneumocystis jiroveci pneumonia prophylaxis may not be necessary , regardless of cd4 + t - cell count , . furthermore it is known that hiv infection is associated with a two- to threefold increased risk of rejection following kidney transplant and that the administration of antithymocite globulin ( atg ) is used to reduce the risk of rejection to that of hiv negative recipients even if it increase the risk of infective complications , . a randomized clinical trial should be designed to investigate whether patients with a cd4 count < 200 cells / mm and undetectable hiv rna level might benefit from kidney transplantation and whether atg induction should or not be administered in these cases . cc , mt , and cg developed the idea of the study , participated in its design and coordination and helped to draft the manuscript . cc , mt , cg and am contributed to the acquisition and interpretation of data . a copy of the written consent is available for review by the editor - in - chief of this journal .

highlightsautosomal dominant polycystic kidney disease is the most common among inherited cystic kidney diseases.patients with hiv infection are at risk of developing acute kidney injury and chronic kidney disease.we provide the first report of hiv infection in a patient with polycystic kidney disease.lymphopenia should not contraindicate kidney transplantation in patients with hiv infection .