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furthermore , the site of lesion could indicate surgical excision to prevent continuous microtraumas [ 13 ] . surgical excision could determine loss of substance due to the dimension of the nevus that could not be easily directly repaired . the foreskin is a good autologous full - thickness skin graft in several conditions . the authors report the use of foreskin as skin graft to repair a loss of substance due to excision of an interdigital nevus of the foot . a four - year - old boy presented a 2 cm 1.5 cm congenital compound nevus entirely covering the plantar surface of the second finger of his left foot ( figure 1 ) . paediatric dermatologist 's indication was a radical excision because of the site and the dimension of this melanocytic lesion . primary closure of the skin defect secondary to radical excision of the lesion was not indicated because of the large loss of substance and the risk of retractive scar . then we performed circumcision and a radical excision of the nevus ( figure 3(a ) ) ; foreskin , trimmed in a rectangular shape ( figure 2 ) , was sutured into the residual defect ( figure 3(b ) ) . , the patient has normal use of the foot finger with no evidence of contracture ( figure 4 ) . congenital melanocytic nevus is a frequent condition in childhood ( 0,21% ) [ 1 , 2 ] . the role of these lesions in increasing incidence of cutaneous melanoma is discussed and the prophylactic removal of all congenital melanocytic nevi is not supported : however , the most congenital melanocytic nevi are removed on preventing criteria . the selective excision of suspicious nevi is indicated when the features of a possible malignancy are faced . these features can include change in size or colour , irregular borders , or development of ulcerations . other features that can justify excision are site and extension of the lesion , multinodular aspect , and the presence of other risk factors ( immunodeficiency , dysplastic nevus syndrome , and xeroderma pigmentosa ) . excision of larger lesions require the use of local plasty , free tissue skin graft , or even the prior use of a tissue expander .graft should be harvested from hairless areas where the skin is redundant ( groin , volar wrist crease , volar elbow crease , and ulnar side of the hypothenar eminence ) . foreskin as a source of skin graft most often been used in urethral reconstruction for congenital or acquired penile defects [ 5 , 6 ] , in burn reconstruction , most commonly for eyelid resurfacing , and in syndactyly repair [ 9 , 10 ] . newborn circumcision remains controversial ; this procedure has potential medical advantages ( decreased risk of cancer of the penis and urinary tract infections ) as well as disadvantages and risks ( bleeding , infection , meatitis , and scarred phimosis ) . in italy , neonatal circumcision is not routinely performed ; this intervention is electively carried out until three years of age to repair congenital phimosis and at all ages in cases of scarred phimosis , recurrent balanoposthitis , and urinary infections . therefore foreskin is frequently available as tissue graft in paediatric population . in our case , dimension and site ( difficult to control ) of melanocytic foreskin was available because the boy was also affected by congenital phimosis , so we did not look for another source of skin graft . the most common problem reported after the use of prepuce as donor skin is hyperpigmentation . in our case , hyperpigmentation was not a contraindication for the use of foreskin as skin graft because the lesion was hidden localizated . foreskin provides a skin of good elastic quality avoiding secondary retraction with a favourable rate of graft intake . therefore , this source of graft gives the advantage of the absence of scar prejudice at the donor site .

we report a four - year - old boy with a nevus covering all the plantar side of his second finger on the left foot . he was also affected by congenital phimosis . surgical excision of the nevus was indicated , but the skin defect would have been too large to be directly closed . the foreskin was taken as a full - thickness skin graft to cover the cutaneous defect of the finger . the graft intake was favourable and provided a functional repair with good aesthetic characteristic .

patients with coronary artery disease ( cad ) and diabetes have higher mortality and morbidity than patients without diabetes . data from studies such as the uk prospective diabetes study suggest that very good glycemic control is associated with fewer cardiovascular events . hypoglycemia is a very common side effect of insulin therapy and , to a lesser extent , of treatment with sulfonylureas . risk factors for severe hypoglycemia include age , duration of diabetes , strict glycemic control , sleep , impaired awareness of hypoglycemia , renal impairment , c - peptide negativity and previous history of severe hypoglycemia . acute hypoglycemia provokes pronounced physiological responses , the important consequences of which are to maintain the supply of glucose to brain and promote hepatic production of glucose . hypoglycemia and the rapid changes in blood glucose have been shown to increase counter - regulatory hormones such as epinephrine and nor - epinephrine , which may induce vasoconstriction and platelet aggregation , thereby precipitating myocardial ischemia . autonomic activation , principally of the sympatho - adrenal system , results in end - organ stimulation and the profuse release of epinephrine which precipitates hemodynamic changes like tachycardia , increased peripheral systolic blood pressure , decreased central blood pressure and increased myocardial contractility with an increased ejection fraction . the increased activity of sympathetic nervous system and secretion of other hormones and peptides such as the potent vasoconstrictor endothelin have pronounced effects on intravascular coagulability and viscosity . increased plasma viscosity occurs during hypoglycemia because of an increase in erythrocyte concentration , while coagulation is promoted by platelet activation and an increment in factor viii and von - willebrand factor . endothelial functions may be compromised during hypoglycemia because of an increase in c - reactive protein , mobilization and activation of neutrophils and platelet activation . the catecholamine - induced increased myocardial contractility may induce ischemia in the myocardium in patients with cad . the greater oxygen demand is not met because of not only the rigid vessels , but also endothelial dysfunction with failure to vasodilate . several studies have shown that the hypoglycemia is associated with a significant lengthening of the corrected qt interval ( qtc ) in subjects with and without diabetes . these changes are likely seen because of increased catecholamine release during hypoglycemia , and qtc prolongation , in particular , could lead to a high risk of ventricular tachycardia and sudden death . hyperinsulinemia and increased secretion of catecholamines may lead to hypokalemia during hypoglycemia , thus potentiating cardiac repolarizing abnormalities . effects of antecedent hypoglycemia on cardiac autonomic regulation may contribute to the occurrence of adverse cardiac events . abnormalities in high - frequency and low - frequency heart rate variability have been associated with hypoglycemia and increased catecholamine release . however , other studies did not find any associations between heart rate variability , hypoglycemia and increased catecholamine release . episodes of hypoglycemia have been found to be associated with rise in inflammatory cytokines including interleukin ( il)-6 , il-8 , tumor necrosis factor ( tnf)- , c - reactive protein and endothelin-1 . these inflammatory cytokines result in endothelial injury and abnormalities in coagulation , resulting in rise of cardiovascular events . inflammatory cytokines like il-1 have also been shown to increase the severity of hypoglycemia , thus perpetuating a positive feedback cycle . vessel wall stiffness was found to be increased during hypoglycemia in patients with type-1 diabetes of longer duration than those with shorter duration of diabetes . thus , hypoglycemia may increase the risk of cardiovascular events , especially in subsets of patients with longer duration of diabetes . inflammation and endothelial dysfunction could potentially be the aggravating factors that contribute to increased cardiovascular risk with severe hypoglycemia , especially in the subset of patients with pre - existing cardiovascular disease , diabetes , and severe autonomic neuropathy . a direct relationship between hypoglycemia and fatal cardiovascular event is difficult to demonstrate as blood glucose and cardiac monitoring are seldom performed simultaneously . in the accord study , excess of deaths was noted in the intensive treatment arm , which led to discontinuation of study . in the smaller study of veterans with type-2 diabetes , veterans affairs diabetes trial ( vadt ) , ecg changes , including ectopic activity , flattening of t - wave , st depression , ventricular tachycardia , and atrial fibrillation , have been reported in cases of low plasma glucose . sudden death during sleep has been described in patients with type-1 diabetes , the mechanism being a significant cardiac arrhythmia induced by nocturnal hypoglycemia . many of these patients have no evidence of severe hypoglycemia - induced neuronal damage at autopsy , implying that a cardiac arrhythmia had been triggered by hypoglycemia , resulting in sudden death . despite the high frequency of nocturnal hypoglycemia in young patients with type-1 diabetes , sudden nocturnal death ( dead in bed syndrome ) is rare . evidence is accumulating that severe hypoglycemia can provoke adverse cardiovascular outcomes such as myocardial ischemia or cardiac arrhythmia . episodes of severe hypoglycemia are common during intensive therapy in type-1 and type-2 diabetes in the out - patient as well as in - patient setting . larger clinical trials are required to look specifically at the association between hypoglycemia and cardiovascular events and to determine the mechanism further . the challenge to the physicians is to lower blood glucose to normal values to decrease the risk for long - term complications and at the same time minimize hypoglycemia and hypoglycemia - associated morbidity and mortality .

hypoglycemia is a very common side effect of insulin therapy and , to a lesser extent , of treatment with oral hypoglycemic agents . severe hypoglycemia can precipitate adverse cardiovascular outcomes such as myocardial ischemia and cardiac arrhythmia . these are mainly secondary to autonomic activation which results in hemodynamic changes , vasoconstriction and rise in intravascular coagulability and viscosity .

aniridia is a rare bilateral congenital malformation of iris due to neuro - ectodermal developmental defect secondary to a mutation in pax6 gene located on chromosome 11p13 . the incidence of aniridia is estimated to be 1 in 60,000 - 100,000 live births.1 it exists in both sporadic and familial form . it may occur in isolation or associated with number of syndromes such as wagr ( wilm 's tumor , bilateral aniridia , genitourinary abnormalities and mental retardation ) . the pax6 mutations cause a spectrum of ocular malformations , of which aniridia is the major sign . other ocular defect that may be associated includes microcornea , corneal opacity , glaucoma , cataract , subluxated lens , foveal and optic nerve hypoplasia . we report two cases of familial total aniridia associated with microcornea , high myopia and aphakia . a 40-year - old male patient presented with marked dimness of vision for last 1 year . his elder brother ( only sibling ) had visual acuity of 6/6 with -1.5 dioptre sphere ( dsph ) without any other ocular abnormalities . ocular examination revealed best corrected visual acuity in right eye ( re ) counting finger at 3 feet and 3/60 in left eye ( le ) with + 7.0 dsph . slit lamp examination showed bilateral total aniridia with superiorly subluxated cataractous lens in the re [ figure 1 ] and aphakia in the le . the radius of corneal curvature was 8.8 mm in the re and 8.6 mm in the le . the refractive power of cornea of the re in dioptre ( d ) was 39.0 in both vertical and horizontal meridian . the anterior chamber depth in the re was 1.54 mm and in the le was 1.48 . the axial length was 25.57 mm and 25.34 mm in the re and le respectively . gonioscopy of be revealed rudimentary frill of iris tissue in all quadrants and the angle was open . aniridia with superiorly subluxated lens dislocated lens in vitreous a 7-year - old girl , the only child of case i , had poor vision since early childhood . her best corrected visual acuity was 6/36 be with -7.0 dsph and -1.0 dioptre cylinder ( dcyl ) at 90. she had bilateral total aniridia , microcornea , microspherophekic lens [ figure 3 ] and horizontal nystagmus . average corneal diameter of be was 9.5 mm . radius of corneal curvature was 8.6 mm in the re and 8.3 mm in the le . the refractive power of cornea of the re was 38.5d in vertical and 37.5d in horizontal meridian . the diameter of lens was 7.9 mm in be and iop in be with goldmann applanation tonometry was 14 mm hg . gonioscopy of be revealed rudimentary frill of iris tissue in all quadrants and the angle was open . fundus examination of be revealed normal optic disc with foveal hypoplasia [ figure 4 ] . a 40-year - old male patient presented with marked dimness of vision for last 1 year . his elder brother ( only sibling ) had visual acuity of 6/6 with -1.5 dioptre sphere ( dsph ) without any other ocular abnormalities . ocular examination revealed best corrected visual acuity in right eye ( re ) counting finger at 3 feet and 3/60 in left eye ( le ) with + 7.0 dsph . slit lamp examination showed bilateral total aniridia with superiorly subluxated cataractous lens in the re [ figure 1 ] and aphakia in the le . the radius of corneal curvature was 8.8 mm in the re and 8.6 mm in the le . the refractive power of cornea of the re in dioptre ( d ) was 39.0 in both vertical and horizontal meridian . the anterior chamber depth in the re was 1.54 mm and in the le was 1.48 . the axial length was 25.57 mm and 25.34 mm in the re and le respectively . gonioscopy of be revealed rudimentary frill of iris tissue in all quadrants and the angle was open . a 7-year - old girl , the only child of case i , had poor vision since early childhood . her best corrected visual acuity was 6/36 be with -7.0 dsph and -1.0 dioptre cylinder ( dcyl ) at 90. she had bilateral total aniridia , microcornea , microspherophekic lens [ figure 3 ] and horizontal nystagmus . average corneal diameter of be was 9.5 mm . radius of corneal curvature was 8.6 mm in the re and 8.3 mm in the le . the refractive power of cornea of the re was 38.5d in vertical and 37.5d in horizontal meridian . the diameter of lens was 7.9 mm in be and iop in be with goldmann applanation tonometry was 14 mm hg . gonioscopy of be revealed rudimentary frill of iris tissue in all quadrants and the angle was open . fundus examination of be revealed normal optic disc with foveal hypoplasia [ figure 4 ] . aniridia is a rare congenital abnormality transmitted as an autosomal dominant trait with 85 - 90% penetrance.2 microcornea may rarely be associated with aniridia.34 high myopia is again a very rare association with aniridia and microcornea.5 both these abnormalities were present in our case . aniridia and lenticular abnormalities like cataract and subluxated lens may co - exist.134 ] case i had subluxated cataractous lens in the re and aphakia in the le due to spontaneously dislocated lens in vitreous cavity in the le . to the best of our knowledge , in the literature of aniridia association of aphakia aniridia may be associated with defects in angle of anterior chamber and glaucoma , but the findings were not present in our cases . foveal hypoplasia that may be associated with aniridia was present in our case.2 none of the patients was mentally retarded and thorough systemic examination was normal . our cases highlight the rare association of congenital aniridia with microcornea , aphakia and high myopia .

aniridia is a rare congenital malformation that may be associated with various ocular and systemic manifestations . we describe two cases of familial total aniridia associated with microcornea , high myopia and dislocated lens . no systemic abnormality was noted in any of the cases .

radical cystectomy ( rc ) is the standard surgical treatment for clinically localized muscle - invasive bladder cancer ( mibc ) . however , nearly 50% of patients with mibc develop metastases and die of bc . such a high failure rate there are several factors to consider while offering perioperative chemotherapy to a patient undergoing rc . the advantages and disadvantages of neoadjuvant chemotherapy ( nac ) and adjuvant chemotherapy ( ac ) are discussed in table 1 . neoadjuvant versus adjuvant chemotherapy for bladder cancer : advantages and disadvantages after a decision is made to administer chemotherapy , to achieve maximum benefit the planned regimen should be initiated and the planned number of cycles must be completed at an optimal dosage . however , due to various reasons chemotherapy is often not initiated or it is initiated but not completed . the aim of our study is to evaluate and compare the likelihood of initiating and completing neoadjuvant and adjuvant chemotherapy in patients with mibc . upon obtaining an institutional review board approval , we performed a retrospective analysis of patients who underwent rc and urinary diversion ( ud ) by a single surgical team between 1992 and 2011 . data on whether nac or ac was recommended , initiated , discontinued , or completed was obtained and analyzed . prior to 2004 , nac was recommended for patients with clinical stage t3 or higher stage , hydronephrosis , extensive lymphovascular invasion ( lvi ) , or prostatic stromal invasion . since 2004 , the typical chemotherapy regimens were gemcitabine plus cisplatin or methotrexate , vinblastine , doxorubicin , and cisplatin ( mvac ) . on occasions , carboplatin was substituted for cisplatin at the discretion of the oncologist . at any given instance , both ac and nac were not recommended to patients who were unlikely to tolerate chemotherapy due to renal impairment or other debilitating morbidities . a total of 363 patients underwent rc and ud and were considered for perioperative chemotherapy141 for nac and 222 for ac . the mean age was 66 years in nac group and 67.5 years in ac group ( p = 0.13 ) . of these patients , 79% were men and 21% were women . completion of chemotherapy was defined as receiving the planned number of cycles predetermined by the medical oncologist . among further , 98 patients ( 78.4% ) completed at least 3 cycles , 20 ( 16% ) completed 2 cycles as planned , and 7 ( 5.6% ) did not complete the planned number of cycles . from the 222 patients recommended to receive ac , 151 ( 68.0% ) initiated the treatment . of patients who initiated ac , 79 ( 52.7% ) completed at least 4 cycles and 72 ( 47.3% ) overall , only 79 ( 35.5% ) of patients who are candidates for ac received the prescribed number of cycles , while 118 ( 83.5% ) of nac candidates received the planned number of cycles . patients who were offered nac were more likely to initiate ( p < 0.001 ) and complete ( p < 0.001 ) chemotherapy [ figure 1 ] . the reason for not initiating nac was patient preference , while for ac the reasons were either patient preference or presence of medical condition or surgical complication interfering with chemotherapy initiation . the reasons for discontinuing nac or ac were patient preference , chemotherapy intolerability , or development of complications [ table 2 ] . initiation and completion rates for nac vs ac reasons for not initiating / completing the chemotherapy there are several difficulties in administering chemotherapy in the adjuvant setting . the typical patient undergoing rc is in his / her sixth or seventh decade of life and has significant associated comorbidities . moreover , rc and ud are associated with complications in approximately 30% of patients . in a study by donat et al . , 83% of patients who experienced complications following rc and ud had significant complications ( grade 2 - 5 , modified clavien system ) , which prevented or delayed the use of adjuvant chemotherapy . the psychological impact of a major surgery may further impede adjuvant therapy , and thus fewer patients receive ac . our study shows that 32% of the patients who were recommended to receive ac rejected the treatment and elected to be monitored instead . patients who undergo a curative treatment for mibc in the form of rc might be more reluctant to receive additional treatment . the associated morbidity brought by such a major surgery alone would interfere with the initiation of ac . on the other hand , patients who were offered nac were more willing to initiate treatment to improve their chance for a cure as well as survival ( 88.6% ) . furthermore , among patients who initiated perioperative chemotherapy , nac patients were more likely to complete the number of planned cycles ( 94.4% ) , while only 52.7% completed the ac treatment . currently , nac followed by rc is an established approach for mibc . supported by several randomized trials , nac is associated with a survival benefit of 6.5% and a lower risk of bc - specific mortality . on the other hand , our study shows that patients are more likely to accept and complete nac , and this further supports the administration of nac for mibc . chemotherapy protocols were not the same throughout the study period as novel chemotherapy regimens with less toxicity became available . however , the protocols offered were the same in the nac and ac setting at a specific time . this study is limited to the likelihood of initiation and completion of nac and ac . patients with mibc are more likely to initiate and complete the planned number of cycles of neoadjuvant chemotherapy as compared to adjuvant chemotherapy . therefore , we advocate administration of chemotherapy in the neoadjuvant setting in patients who are likely to benefit from chemotherapy .

introduction : chemotherapy was shown to improve survival in patients undergoing radical cystectomy ( rc ) for muscle - invasive bladder cancer ( mibc ) . the initiation and completion rates for perioperative chemotherapy are variable . our aim is to compare the likelihood of initiating and completing neoadjuvant ( nac ) and adjuvant chemotherapy ( ac ) in patients who underwent of rc for mibc.materials and methods : we performed a retrospective analysis of patients who underwent rc between 1992 and 2011 . nac was advised for patients with clinical stage t2 , hydronephrosis , extensive lymphovascular invasion ( lvi ) , or prostatic stromal invasion . patients with pt3 or lymph node metastases were considered for ac.results:a total of 363 patients were considered for perioperative chemotherapy . among the 141 patients who were offered nac , 125 ( 88.6% ) initiated nac . a total of 222 were considered for ac , and 151 ( 68.0% ) initiated ac ( p < 0.001 ) . in the nac group , 118 ( 83.5% ) completed planned number of cycles of chemotherapy and 7 ( 5.6% ) did not complete the planned chemotherapy . in the ac group , 79 ( 35.5% ) completed at least four cycles and 72 ( 47.3% ) could not complete the planned cycles ( p < 0.001).conclusions : patients with mibc are more likely to initiate and complete nac than ac .

a 72-year - old man presented with a two - week history of a red painful right eye . he was a soft contact lens wearer and wore his lenses for one month at a time , taking them out at the end of each day and disposing of the lenses at the end of the month . he had no past ocular , medical , or drug history of note . on examination he had an inflamed eye with a central corneal epithelial defect 3.0 mm 3.5 mm in size , with surrounding superficial and midstromal infiltration . a corneal scrape of the right eye was performed for gram staining and cultures were obtained on blood , chocolate , and sabouraud s agar , as well as non - nutrient agar overlaid with escherichia coli . he was started on empirical antimicrobial treatment with topical ofloxacin hourly by day and night . there was no growth of any other organisms , and the topical ofloxacin was discontinued . his treatment was switched to topical , ie , hourly polyhexamethylene biguanide 0.02% , hourly propamidine isethionate 0.1% ( brolene ) , prednisolone 0.5% four times daily , and atropine 1% three times daily . over the following three months , his treatment was slowly tapered to topical , ie , polyhexamethylene biguanide four times daily , brolene four times daily , and prednisolone 0.1% twice daily as his clinical picture improved . at three months after initial diagnosis he complained of the right eye being red and sore again for several days . on examination the gram stain showed gram - positive cocci in chains and he was empirically commenced on topical penicillin hourly , which was added to his prophylactic treatment for acanthamoeba . after 48 hours , the blood agar grew streptococcus viridans which was shown to be sensitive to penicillin . topical penicillin was tapered down to twice daily as his condition improved over the following month . four months after initial diagnosis , he again complained of a red painful right eye for one week . on examination fine debris and hairs were noted in the base of the abscess and were removed by scraping ( figure 1 ) . the penicillin drops were stopped and he was treated with topical ofloxacin initially hourly and then tapered off over one month . five months after initial diagnosis he complained of a one - day history of a painful right eye . fine strands were noted on the surface of a central corneal abscess and were removed during the corneal scrape . he was treated with topical ofloxacin and gentamicin , to which the organism was found to be sensitive . the organism was resistant to ciprofloxacin , chloramphenicol , penicillin , cefuroxime , and fusidic acid . as he improved , fine fibrils were again seen in the healing cornea and were removed and sent for microscopy . this confirmed that the fibrils were synthetic and were not observed in previous / present cultures , and presumed to be derived from tissues which the patient was constantly using to wipe his eye . the cornea continued to heal and topical treatment for acanthamoeba ( including steroids and ofloxacin ) were continued and tailed off over a further three months . in all episodes of infection , antibiotic sensitivities were determined using disc diffusion susceptibility testing . in all episodes of reinfection , scraping for acanthamoeba was performed to exclude reactivation of the initial infection . this patient had acanthamoeba keratitis secondary to contact lens wear and then developed three episodes of bacterial keratitis . microbial keratitis results from the interaction of a broad spectrum of pathogens and a diverse range of host responses . recurrence is rare in the absence of predisposing factors , such as contact lens wear , ocular surface and corneal disease , corneal anesthesia , exposure , trauma , or previous corneal surgery.1 when suspected , corneal scraping is mandatory in order to provide material for a microbiological diagnosis , debride necrotic tissue , and enhance antibiotic penetration . corneal biopsy may be necessary in some cases of recurrence . the initial bacterial infection in this patient presumably was facilitated by the underlying acanthamoeba keratitis and the use of corticosteroids.2,3 the presence of synthetic fibrils on the ulcer base presumably contributed to the infection recurrences by acting as a nidus for organisms and by interfering with corneal healing . it is noteworthy that the cornea finally healed with no further infections once the patient was instructed not to wipe his eye with a paper tissue . unfortunately it was not possible to obtain cultures directly from the tissue fibrils to confirm their direct association with the infective organisms . the first two bacterial infections were due to virulent organisms , such as pseudomonas and streptococcus . the culture of stenotrophomonas maltophilia from the third corneal abscess was feasible because this is an opportunistic organism and infections typically occur in patients with compromised ocular surface and trauma.4,5 it has been reported previously in patients following penetrating keratoplasty.3,6,7 this is the first case report of acanthamoeba keratitis occurring in a contact lens wearer with preceding protozoal and bacterial keratitis . the characteristically resistant antibiogram of s. maltophilia may limit the therapeutic options . fortunately in this patient treatment

recurrence of microbial keratitis in the presence of protozoal infection is very rare and infrequently reported unless predisposing factors are present . the association of recurrent microbial keratitis and synthetic microfibrils has never previously been reported to our knowledge . this single interventional case study describes the clinical course and treatment of a contact lens wearer who was treated for acanthamoeba keratitis with superinfection from bacterial organisms in the presence of synthetic microfibrils . the presence of synthetic fibrils on a corneal ulcer base may act as a nidus for pathological organisms and interfere with normal corneal healing . this may result in infection recurrence and the growth of resistant opportunistic organisms .

pilomatricoma usually appears in the first two decadesusually , an asymptomatic lesion with an occasional inflammation . pilomatricoma usually appears in the first two decades usually , an asymptomatic lesion with an occasional inflammation . usually , they present as a solitary nodule / cystic lesion distributed on head , neck , and upper trunk area . sometimes , there is associated inflammation . a 36-year - old obese , hypothyroid female patient presented in surgery outpatient department of a tertiary care medical college of eastern bihar with a bluish - red , nodular lesion over the upper back [ figure 1 ] . she gave a history of 68 months duration and complained of some sticky material coming out from the lesion intermittently whenever there was pain and redness . she was treated outside with intermittent antibiotic and anti - inflammatory agents with an apparent resolution of the symptoms followed by recurrence in a few weeks . on examination , we found a violaceous nodulo - cystic lesion on the upper back with a size of 2 cm 1.5 cm ; the center of the lesion was eroded , and there was a visible yellow - colored cheesy discharge from that area [ figure 2 ] . depending on the site , the nodulo - cystic nature of the lesion , the recurrent history of inflammation and discharge , a provisional diagnosis of the ruptured epidermal cyst was placed . the patient was managed conservatively till discharge subsided , and the epithelialization of central area was complete . nodule covered with yellow cheesy discharge and crust in scanner view , the epidermis was acanthotic . in the dermis , there was a nodular basaloid cell proliferation extending from papillary to reticular dermis [ figure 3 ] . there was also the proliferation of squamoid cells with the disappearance of nucleus leaving behind a shadow ( ghost cells / shadow cells ) [ figures 4 and 5 ] . the dermis was covered by mononuclear inflammatory infiltrate along with the formation of foreign body type giant cells [ figures 6 and 7 ] . in view of these findings h and e ( scanner view ) : basaloid cells h and e ( scanner view ) : ghost cells ghost cells with nuclear shadow , ( h and e , 40 ) stromal inflammation , ( h and e , 10 ) foreign body giant cells , ( h and e , 40 ) in scanner view , the epidermis was acanthotic . in the dermis , there was a nodular basaloid cell proliferation extending from papillary to reticular dermis [ figure 3 ] . there was also the proliferation of squamoid cells with the disappearance of nucleus leaving behind a shadow ( ghost cells / shadow cells ) [ figures 4 and 5 ] . the dermis was covered by mononuclear inflammatory infiltrate along with the formation of foreign body type giant cells [ figures 6 and 7 ] . in view of these findings h and e ( scanner view ) : basaloid cells h and e ( scanner view ) : ghost cells ghost cells with nuclear shadow , ( h and e , 40 ) stromal inflammation , ( h and e , 10 ) foreign body giant cells , ( h and e , 40 ) in scanner view , the epidermis was acanthotic . in the dermis , there was a nodular basaloid cell proliferation extending from papillary to reticular dermis [ figure 3 ] . there was also the proliferation of squamoid cells with the disappearance of nucleus leaving behind a shadow ( ghost cells / shadow cells ) [ figures 4 and 5 ] . the dermis was covered by mononuclear inflammatory infiltrate along with the formation of foreign body type giant cells [ figures 6 and 7 ] . in view of these findings h and e ( scanner view ) : basaloid cells h and e ( scanner view ) : ghost cells ghost cells with nuclear shadow , ( h and e , 40 ) stromal inflammation , ( h and e , 10 ) foreign body giant cells , ( h and e , 40 ) usually , they appear in early age , the average age of onset being childhood to adolescence . they usually present as a solitary firm nodule or cyst distributed on any nonglabrous area , but mostly restricted to head - neck and upper trunk . sometimes , the lesion shows an inflammatory sign . in histology , usually there is a cyst formation with matrical keratinization . there are two kinds of cells ; outer basaloid matrical cells and central squamatized cells with the remnant of nuclear outline ( shadow or ghost cells ) . in the early stage , particularly , in the elderly population , the basaloid cells predominate , and these tumors are called proliferating pilomatricoma . the treatment is basically surgical excision ; recurrence has been reported following the surgical excision . in our case , the pilomatricoma appeared at 36 years of age , and clinically mimicked ruptured epidermal cyst . the goal of this presentation is to highlight the fact that pilomatricoma may appear late in life and matrical cyst of pilomatricoma can rupture and give rise to a clinical picture mimicking ruptured epidermal cyst .

pilomatricoma is a benign tumor arising from the hair matrical cells . most pilomatricomas appear in the first two decades of life as a solitary skin to a bluish colored nodule on head - neck area with an occasional sign of inflammation . here , we present a case of pilomatricoma which appeared at 36 years of age with a history of recurrent inflammation and discharge mimicking ruptured epidermal cyst .

the potential use of the affected upper extremity of children with hemiplegia often fails due to learned non - use phenomenon . constraint - induced movement therapy ( cimt ) is one of the treatment strategies which utilizes the principles of neural plasticity to help acquire motor skills of the affected upper extremity.[27 ] this therapeutic approach involves constraining of the unaffected upper extremity using sling , plaster cast , mitt or splints and intensive training of the affected upper extremity with task - specific , goal - oriented activities by reinforcement ( shaping technique ) . a five - year - old female child presented with right hemiplegia with the history of delayed motor milestones and limited motor skills of the right upper extremity since birth . the objective details of the cause of hemiplegia could not be established as there were no medical records available . presently , she has achieved the highest level of functional independence ( able to walk and run independently ) . she displayed no voluntary effort to initiate any motor skills of the right upper extremity unless verbally prompted , even otherwise initiating only minimal response suggesting learned non - use phenomenon . the following criteria were considered for use of cimt in this child ( adapted from cochrane review study ) . observed learned non - use of affected upper extremity.a possible movement of at least 10 extension at metacarpophalangeal and inter - phalangeal joints and 20 extension at wrist of the affected upper extremity.no cognitive impairment and shall cooperate with treatment . observed learned non - use of affected upper extremity . a possible movement of at least 10 extension at metacarpophalangeal and inter - phalangeal joints and 20 extension at wrist of the affected upper extremity . no cognitive impairment and shall cooperate with treatment . the quality of upper extremity skills test ( quest ) is a criterion - referenced measure that evaluates the quality of upper extremity function in four domains : dissociated movements ( 19 items with one level of response for each item ) , grasps ( six items with three to five levels of response for each item ) , weight - bearing ( five items with six levels of response for each item ) and protective extension ( three items with six levels of response for each item ) . it is designed to be used with children who exhibit neuromotor dysfunction with spasticity and has been validated with children 18 months to eight years of age . the data collected during the neuro developmental therapy / casting study by law et al . , were used to analyze the validity and responsiveness of the quest . the parents of the child were counseled for the treatment approach that could improve the motor skills of the affected right upper extremity . the parents were interested and were keen in subjecting their child to this treatment approach and gave informed consent . the pre - intervention assessments of the right upper extremity motor skills were measured using quest . the unaffected left upper extremity was constrained with posterior slab plaster cast extending above the elbow to the interphalangeal joints of fingers and supported with a sling . the affected right upper extremity was then subjected to task - specific goal - oriented activities that aimed to improve reaching , grasps , manipulation and release of the object using the arm and hand . the activities were encouraged using play way method and reinforcements using visual ( postural mirror ) and verbal feedback . the therapy session usually lasted for one hour a day for five days a week . the parents were instructed to constantly encourage the same activities that were carried out during the treatment session in daily activities . at the end of the two - week period , post - intervention assessment of the right upper extremity was done using quest . as there was an incremental response in the outcome measure , the investigators convinced the parent to continue the treatment for another week . at the end of three weeks , once again post - intervention assessment was done . the quality of upper extremity skills test ( quest ) is a criterion - referenced measure that evaluates the quality of upper extremity function in four domains : dissociated movements ( 19 items with one level of response for each item ) , grasps ( six items with three to five levels of response for each item ) , weight - bearing ( five items with six levels of response for each item ) and protective extension ( three items with six levels of response for each item ) . it is designed to be used with children who exhibit neuromotor dysfunction with spasticity and has been validated with children 18 months to eight years of age . the data collected during the neuro developmental therapy / casting study by law et al . , were used to analyze the validity and responsiveness of the quest . the parents of the child were counseled for the treatment approach that could improve the motor skills of the affected right upper extremity . the parents were interested and were keen in subjecting their child to this treatment approach and gave informed consent . the pre - intervention assessments of the right upper extremity motor skills were measured using quest . the unaffected left upper extremity was constrained with posterior slab plaster cast extending above the elbow to the interphalangeal joints of fingers and supported with a sling . the affected right upper extremity was then subjected to task - specific goal - oriented activities that aimed to improve reaching , grasps , manipulation and release of the object using the arm and hand . the activities were encouraged using play way method and reinforcements using visual ( postural mirror ) and verbal feedback . the therapy session usually lasted for one hour a day for five days a week . the parents were instructed to constantly encourage the same activities that were carried out during the treatment session in daily activities . at the end of the two - week period , post - intervention assessment of the right upper extremity was done using quest . as there was an incremental response in the outcome measure , the investigators convinced the parent to continue the treatment for another week . at the end of three weeks as the quest measure analyzes the quality of motor skills of both the right and left extremity , it may be noted that the pre - intervention percentage score was 53.04% indicating full percentage score of unaffected left upper extremity and marginal percentage score of affected right upper extremity [ table 1 ] . following intervention , increments in percentage score by 26.79% and 07.51% were observed at the end of two weeks and three weeks respectively which should be attributed to the improvements in the quality of motor skills of the affected right upper extremity . it may also be noted that the grasp percentage score was greater than other domains indicating better improvement in fine motor skills than gross motor skills . the main concern for the parents in the use of cimt for improving the motor skills of the affected upper extremity in infantile hemiplegia was the fact that it restricts the use of the unaffected extremity . the success of the use of cimt in infantile hemiplegia depends on the parents , their proper understanding of the concept of the approach and their deep motivation in carrying out home exercises . the increments in percentage scores observed in this case report are attributed to the increased demands for the use of the affected upper extremity while constraining the unaffected upper extremity through task - specific goal - oriented activities that were reinforced with visual and verbal feedback . the observation of this case report indicates that the use of cimt could reverse the learned non - use phenomenon of the affected upper extremity in infantile hemiplegia and thus reduce disability to greater extent .

infantile hemiplegia is one of the clinical forms of cerebral palsy that refers to impaired motor function of one half of the body owing to contralateral brain damage due to prenatal , perinatal and postnatal causes amongst which vascular lesion is the most common causative factor . we report here the effects of constraint - induced movement therapy in a five - year - old female child with infantile hemiplegia on improvement of upper extremity motor skills .

in modern society , more people spend long periods working at computers , and experience increased work - related neck and back discomfort1 . remaining seated for long periods can cause problems for the spine , circulation , muscles , and joints2 . prolonged sitting is a risk factor for low back pain ( lbp)2 . osullivan et al.2 showed that decreased trunk muscle endurance is associated with habitually adopting a passive sitting posture and reduced activity levels . according to recent findings , the form of resting and the work configuration are important to muscle activation and recovery of the circulation4 , 5 . the purpose of this study was to examine the effects of a relaxation chair and resting with simple range of motion ( rom ) exercises during computer work , and low back muscle strengthening exercises after computer work on pain and the flexion - relaxation ( fr ) ratio of one computer worker with lbp . the subject of this study was a 37-year - old male who complained of severe lbp pain at the l4 level . ethics approval was obtained from the yonsei university faculty of health science human ethics committee . he did not have back pain in the morning but did have it in the evening after working on a computer all day . when he performed forward flexion in the standing position with his knees fully extended , he experienced pain in his lower back with a visual analogue scale ( vas ) score of 7 . , he rested three times for approximately 10 min each at irregular intervals . the rest of the time , he was performing internet searches or shopping on a computer . in the study , he worked on a computer for 5 h each day for 3 days and followed a different program each day . the first day ( session 1 ) , he spent 10 min in a relaxation chair resting after each hour of computer work . the second day ( session 2 ) , he spent 10 min performing simple trunk rom exercises ( trunk flexion , extension , lateral bending , and rotation ) while standing after each hour of computer work . the third day ( session 3 ) , he performed low back muscle strengthening exercises for 20 min after 5 h of using the computer . the exercises consisted of a bridging exercise in the supine position ( three sets of ten repetitions ) and hip extension exercises in the quadruped position with knee extension ( left and right leg , five sets each of ten repetitions ) . this study measured the fr ratio and vas score when he performed forward flexion in the standing position with his knees fully extended , before and after each 5-h work session . electromyography ( emg ) signals were preamplified by a preamplifier placed close to the electrodes , and sent to the data acquisition unit of an mp150 system ( biopac systems , santa barbara , ca , usa ) , which amplified and sampled the emg input at 1000 hz . the emg signals were band - stop filtered at 60 hz , and the root mean square ( rms ) values were calculated . the subject was required to stand comfortably , bend forward slowly with his arms dangling freely ( bending period ) , and then hold this position for 3 s ( hanging period ) . the fr ratio ( % ) was calculated as the ratio of the rms activity in the hanging period to that in the bending period . in session 1 , the fr ratios before and after work were 19% and 38% ( + 19% ) , respectively . the respective vas scores before and after work were 5 and 8 ( + 3 ) . in session 2 , the fr ratios before and after work were 18% and 21% ( + 3% ) , respectively . the respective vas scores were 5 and 6 ( + 1 ) . in session 3 , the fr ratios before and after work were 22% and 29% ( + 7% ) , respectively , and the vas scores were 5 and 6 ( + 1 ) . the fr response is reported to occur in the lumbar region of more than 90% of healthy people who do not have lbp8 . in individuals with lbp , the trunk muscle activation pattern is altered , and the lower back muscles are strongly activated over a long period8 . it is necessary to evaluate the fr response for lbp . mathieu and forin9 suggested that if the erector spinae ( es ) muscle activity during the hanging period is less than 10% of the muscle activity during the bending period , it indicates the presence of fr . in session 1 , the fr ratio ( + 19% ) and vas score ( + 3 ) increased to the greatest degree . therefore , the relaxation chair was not effective for muscle activation and recovery of the circulation . in session 2 , the fr ratio increased by 3% and the vas score increased by + 1 . in session 3 , the fr ratio was increased by 7% and the vas score increased by + 1 . therefore , after computer work , the back muscles require exercises to prevent lbp . there was no difference in pain between sessions 2 and 3 . however , difference in fr ratio was the lower in session 2 than session 3 . the fr ratio is known to be the more sensitive indicator for prediction of lbp8 , 9 . also , session 3 required additional time after work , and it also required exercise with a high load when compared with session 2 . so , this study suggests that session 2 ( regular trunk rom exercises after each hour of computer work ) was more effective than session 3 ( strengthening exercises after work ) . however , this report has a limitation . the carryover effects could have an influence on the results . this study suggests that it is more effective to perform regular , passive exercises to prevent lbp in computer users . it also suggests that poor resting is a risk factor for a change in the muscle pattern .

[ purpose ] this study examined the effects of a relaxation chair and resting with simple range of motion exercises during computer work and low back muscle strengthening exercises after computer work on pain and the flexion - relaxation ( fr ) ratio of one computer worker with lbp . [ subjects ] the subject of this study was a 37 year - old male who complained of severe lbp pain at the l4 level . [ methods ] in the study , the subject worked on a computer for 5 h each day for 3 days and followed a different program each day . [ results ] in session 1 , the fr ratios before and after work were 19% and 38% ( + 19% ) , respectively . the respective vas scores before and after work were 5 and 8 ( + 3 ) . in session 2 , the fr ratios before and after work were 18% and 21% ( + 3% ) , respectively . the respective vas scores were 5 and 6 ( + 1 ) . in session 3 , the fr ratios before and work were 22% and 29% ( + 7% ) , respectively , and the vas scores were 5 and 6 ( + 1 ) . [ conclusion ] this study suggests that it is more effective to perform regular , passive exercises to prevent lbp in computer users .

systemic chemotherapy can lead to a variety of ocular complications , such as cicatricial ectropion , nasolacrimal duct stenosis , conjunctivitis , keratitis , cataract , macular edema , retinopathy , and optic neuropathy ( 1 , 2 ) . although bulbar perforation with orbital cellulitis has been reported in an immunocompromised patient , corneal perforation has not been documented in patients undergoing systemic chemotherapy ( 3 ) . we report a case of corneal perforation with preseptal cellulitis in a patient treated with systemic chemotherapy for acute lymphocytic leukemia ( all ) . a 17-yr - old female patient undergoing systemic chemotherapy for all was referred to our hospital due to swelling and pain of the right upper lid for two days . laboratory examination showed leukocytes 40 cells/l , erythrocytes 3.610 cells/l , hemoglobin 10.8 g / dl , hematocrit 30.6% , and thrombocytes 3310 cells/l . the patients received induction chemotherapy ( vincristine , prednisolone , daunorubicin , and l - asparaginase ) . there were no abnormal findings , other than diffuse swelling of the right upper eyelid . artificial tear eye drops were used for the treatment of superficial punctate erosions . on the 12th day , spontaneous bloody and purulent discharge from the upper palpebral conjunctiva occurred in the right eye . systemic antibiotics ( meropenem ) were maintained per the antibiotic sensitivity test . on the 16th day , periorbital swelling decreased , however , corneal melting and perforation with iris prolapse was noted in the right eye ( fig . seven months after surgery , visual acuity in the right eye was 20/300 , and intraocular pressure was 14 mmhg . ocular complications associated with systemic chemotherapy can be divided into complications in the adnexa , anterior segment , and posterior segment . previously reported complications in the anterior segment include conjunctival injection , conjunctivitis , corneal edema , keratitis , and corneal opacity ( 1 , 2 ) . anticancer agents such as cytosine arabinoside , 5-flurouracil , carmustine , deoxycoformycin , and tamoxifen have been known to cause corneal toxicity ( 1 , 2 , 4 , 5 ) . in our case , vincristine , prednisolone , daunorubicin , and l - asparaginase were used for combination chemotherapy . to the best of our knowledge , corneal toxicity associated with these anticancer agents has not been reported . serratia marcescens , a motile , gram - negative coccobacillus , is an emerging opportunistic pathogen noted for causing urinary , respiratory , blood stream , and ocular infections ( 6 ) . preseptal cellulitis in the present case was caused by s. marcescens because the patient was immunocompromised . extracellular protease produced by s. marcescens is considered a major corneal destructive factor . in an experimental study of rabbit cornea , serratia protease preparations have caused rapid and extensive liquefactive corneal necrosis , descemetocele formation , and corneal perforation ( 7 ) . it is possible that s. marcescens in the purulent discharge directly invaded the cornea with superficial punctate erosions and caused corneal melting and perforation in the immunocompromised patient . this hypothesis is supported by two facts : first , s. marcescens is a virulent organism . second , corneal melting and perforation occurred in the area in direct contact with the infected upper palpebral conjunctiva . in conclusion , physicians should consider the possibility of serious ocular complications , such as severe bacterial keratitis or corneal perforation in cases of preseptal cellulitis caused by a virulent organism , particularly in immunocompromised patients .

we report a case of corneal perforation with preseptal cellulitis in a patient with acute lymphocytic leukemia ( all ) . a 17-yr - old female patient who was undergoing combination chemotherapy for all was referred due to upper lid swelling and pain in the right eye for 2 days . visual acuity in the right eye was 20/20 . initial examination showed no abnormal findings , other than swelling of the right upper eyelid . computed tomography showed a finding of preseptal cellulitis . microbiologic study of bloody and purulent discharge revealed serratia marcescens . corneal melting and perforation with iris prolapse were detected in the right eye on the 16th day . emergent tectonic keratoplasty was performed . seven months after surgery , visual acuity in the right eye was 20/300 , and the corneal graft was stable .

cervical spine spondylolysis is the most commonly caused by high energy trauma to the upper cervical spine in the form of a " hangman 's " fracture of c2 vertebrae7 ) . however , congenital cervical spine spondylolysis is discovered by incidental radiographic finding , neck pain after minor trauma , a clunking or clicking neck and rarely neurologic compromise1 - 3,6,8,10 ) . this rare condition potentially signifies an unstable cervical spine . we will report a case of congenital isthmic defect of cervical spine and describe a cause of cervical isthmic defect , diagnostic radiographic finding and appropriate treatment of cervical spondylolysis or spondylolithesis a 23-year - old female suffered from a hyperextension injury to cervical spine after a traffic accident . following this injury , she experienced mild to moderate degree of nuchal and back pain . plain radiograph of the cervical spine showed bilateral pars interarticularis defect at the c6 level with cleft - bow - tie configuration , hypoplastic pedicle , grade i spondylolisthesis of c6 on c7 and spinal bifida at c6 ( fig . 1 ) . computed tomography of the c6 vertebrae showed bilateral corticated defects of the c6 articular mass , bilateral hypoplastic pedicles , lamina and spinal bifida of c6 and apparent neural foraminal enlargement at c6 - 7 ( fig . the magnetic resonance image of spine showed grade i spondylolisthesis c6 - 7 , degenerative change of c6 - 7 disc and acute compressed fracture at 4 thoracic vertebrae . she was subjected to controlled flexion - extension views of the cervical spine , which confirmed stability of the segment with less than 10 degrees of anterior angulations ( fig . she was treated with the semihard - orthosis brace immobilization for 8 weeks followed by repeated lateral cervical spine radiography . after 10 months to injury , she was symptom free with full range of motion of cervical spine . reported cervical spondylolysis are incidentally discovered except the " hangman 's " fracture1 - 3,6,8,9,10 ) . cervical spondylolysis is less prevalent than lumbar spondylolysis , only about 100 cases have been reported in the literature worldwide , mainly in adults1 ) . there is often no clear uniform mechanism of the injury , or the force causing the injury is often not strong enough to explain all of observed cervical anomaly . incidentally discovered cervical spondylolysis could combine with another anomaly like spinal bifida , dysplastic lamina or transverse process ( table 1 ) . the etiology of cervical spondylolysis is unknown , but congenital basis is asserted because no history of trauma is elicited in the majority of cases2,3,5 ) . another basis is nonunion of vertebra after birth , infantile fracture or repetitive microtrauma1,6,9 ) . the vertebral body will articulate with the vertebral arch at the neurocentral joints at birth , with fusion occurring between the ages of 5 and 8 years5,7 ) . if congenital defect is invalid , problem of ossification or chondrification might occur during vulnerable period from birth to fusion at neurocentral joint . proposed that this condition was caused by repetitive microtrauma resulting in stress fractures to the pars region similar to the process proposed in lumbar spondylolysis5 ) . they further proposed c6 to be the commonest site to be affected as , this being a transitional vertebra , it was subjected to stress more often . some of these theories can be put together and it may be suggested that a dysplastic spine is more susceptible to trauma or stress and leads to spondylolysis6 ) . we could clarify that our case was associated with congenital basis because of well corticated margin in defected area combined with another congenital lesion and no trauma history . congenital cervical spondylolysis , an embryologic developmental defect , occurs the most commonly at c61,2,6,9,10 ) . the overall frequency is unknown , and most cases are detected incidentally ( table 1 ) conventional radiolographic findings of cervical spondylolysis included a deformed articular mass , hypoplastic or dysplastic pedicle , spinal bifida and anterolisthesis1 - 3,6,8 - 10 ) . the computed tomography is the most useful image modality , because it shows the exact bony structures and three dimensional images . the magnetic resonance image can aid identification of combined soft tissue damage , cord injury and bony contusion at trauma lesion . however , magnetic resonance image is not useful for diagnosis of cervical spondylolysis . in our case , cervical magnetic resonance image showed acute compressive fracture at 4 thoracic vertebrae and degenerative disc change . misdiagnosis occurred for traumatic unilateral interfacet dislocation , a chronic nonunited articular mass fracture or congenitally absent pedicle4,10 ) . for example , unilateral interfacet dislocation is characterized by spondylolisthesis greater than 3 mm , rotation without a superimposed articular mass , and misalignment of the spinous process4 ) . the cervical spondylolysis can cause variable symptoms like neck pain , muscle spasm or neurologic deficit . symptomatic cervical spondylolysis need adequate treatment because previously existing cervical spondylolysis is vulnerable to trauma . the treatment of cervical spondylolysis depends on instability . if the cervical spine is stable , conservative approach is desirable for treating symptomatic cervical spondylolysis . our case was stable but needed hard orthosis because of the fracture of 4 thoracic vertebrae .

we report a case of rare cervical isthmic spondylolisthesis of c6 - 7 combined occult spinal bifida at c6 , and review the radiologic finding , different diagnosis and treatment . a 23-year old female presented nuchal , back pain after traffic accident . radiologic finding showed the 6th cervical isthmic defect , spondylolisthesis and dysplasia . the patient was conservatively treated about 8 weeks , and 10 months after injury , she was symptom free with full range of motion of cervical spine and she was followed up . cervical spondylolysis is a very rare condition . this clinical importance is vulnerable to trauma . for whatever reasons , symptomatic patients need to be treated by conservative or surgical option .

acute myocarditis is associated with various electrocardiogram ( ecg ) alterations , including st - t changes , q - waves , atrioventricular- and intraventricular - conduction delays , atrial and ventricular tachyarrhythmia , and low voltage . fulminant myocarditis with ventricular arrhythmia or atrioventricular block is associated with a high mortality rate . extracorporeal membrane oxygenation ( ecmo ) is instituted for the management of life threatening pulmonary or cardiac failure , when no other forms of treatment are likely to be successful . we describe life salvage of ecmo in a patient with cardiogenic shock and malignant arrhythmia caused by acute fulminant myocarditis . a 53-year - old korean - chinese woman was brought into the emergency department of our hospital by an ambulance , with complaints of progressive chest pain and dyspnea . she had a high fever , cough , and general fatigue over the three days . upon arrival , she was fully conscious and had signs of cold sweating , cyanosis and general pallor . the initial creatine kinase ( ck ) , ck - mb and troponin - i levels were 626 u / l , 47.27 ng / ml , > 50 ng / ml , respecitvely . emergent coronary angiogram was performed because of low blood pressure ( 88/56 mm hg ) , st - segment depression in the precordial leads of ecg ( fig . coronary angiographic findings were normal , and an intra - aortic balloon pump ( iabp ) was immediately inserted for hemodynamic support ( fig . 2 ) . on the basis of prodromal symptoms , elevated cardiac enzymes and normal coronary angiographic findings with ventricular wall motion abnormality , we contemplated acute decompensated heart failure due to clinical suspicion acute myocarditis . she was admitted to the coronary care unit , and mechanical ventilation and inotropic agents were started . we decided to support the patient with ecmo , which was inserted in a veno - arterial configuration by cannulation on both femoral vessels . we were able to reduce the amount of inotropic agents using mechanical circulatory support . however , ecg showed wide qrs tachycardia with left bundle branch block ( fig . 3a ) . the serum creatinine level was elevated , and the amount of urination was decreased . because of low blood pressure and the lack of diuretic effect , continuous renal replacement therapy was started using the ecmo circuit on day 1 . ventricular wall movement was gradually decreased , and ecg showed little or no electrical activity ( figs . 1d and 3b ) . on day 2 , iabp had to be removed because of its malfunction . ecg showed little or no electrical activity . on day 5 , ecg revealed weak electrical activities ( fig . mechanical ventilator was weaned off on day 18 , and ecmo was weaned off on day 20 . she underwent cardiac magnetic resonance imaging after making sure that she was medically stable , which showed diffuse wall thinning , decreased wall motion and delayed enhancement of diffuse ventricular wall sparing of the infero - septal wall ( fig . , she was complicated with ischemic insult in the lower extremities , hepatitis with jaundice , and acute tubular necrosis . on day 76 , she was discharged from the hospital . fulminant myocarditis is an inflammatory process that occurs in the myocardium and has a fatal course due to the rapid development into acute heart failure or cardiogenic shock.1 ) fulminant myocarditis is associated with various ecg alterations , including st - t changes , q - waves , atrioventricular- and intraventricular - conduction delays , atrial and ventricular tachyarrhythmia , and low voltage.2 ) our case demonstrated various alterations of ecg , which are some of the characteristics that are observed in the fulminant myocarditis . at first , ecg showed st segmental changes associated with myocardial ischemia , then ecg showed various manifestations , such as ventricular tachycardia , atrial fibrillation with left bundle branch block , and complete atrioventricular block . it also displayed very little electrical activity on the whole leads , resembling an ecg after arrest . low voltage on ecg can be observed in obesity , lung emphysema , pericardial effusion , pulmonary edema , hypothyroidism and amyloidosis , however low voltage on precordial leads , which is less common than on limb leads , implies cardiac tamponade or post - defibrillation state.3 ) low voltage ecg in our case was probably due to pulmonary edema , pericardial effusion and edema of the ventricular wall . patients presented with syncope , new ventricular arrhythmia or atrioventricular block , ejection fraction of less than 40 percent , and lack of response to usual care within 1 to 2 weeks are associated with a high mortality rate in acute myocarditis.4)5 ) extracorporeal membrane oxygenation is instituted for providing emergent circulatory support to patients with cardiogenic shock . extracorporeal membrane oxygenation is not only useful for acute decompensated heart failure with low cardiac output , but also for life - threatening arrhythmia.6 ) it provides a bridge - to - recovery or decision regarding either heart transplantation or ventricular assist device . its adaptation can physiologically reduce wall stress , decrease cytokine activation , and improve myocardial contractility . however , its use is limited by complications such as thromboembolic events.7 ) it is reported that patients with ischemic insult in the lower extremities or multi - organ failure due to thromboembolic events have poorer prognosis.6 ) long term mechanical circulatory support such as implantable left ventricular assist devices is better treatment modality for advanced heart failure patients with nyha functional class iii b or iv , intermacs level 1 to 5 as a bridge to recovery or bridge to transplantation.8 ) unfortunately the device is not yet commercially abailable in korea . in summary , this patient had multiple factors associated with high mortality , such as low cardiac output and malignant arrhythmia . in addition , ischemic insults in the lower extremities and multi - organ failure ( lung , kidney and liver ) occurred during intensive therapy . however , early adaptation of mechanical circulatory support , such as iabp and ecmo , combined with intensive medical therapy could save our patient with high - risk fulminant myocarditis .

clinical features of acute myocarditis range from a subclinical state to a fulminant state . fulminant myocarditis with ventricular arrhythmia or atrioventricular block is associated with a high mortality rate . in cases in which aggressive medical therapy for fulminant myocarditis is not likely to be successful , intensive and emergency mechanical circulatory support , such as extracorporeal membrane oxygenation ( ecmo ) or intra - aortic balloon pump , should be considered . we report life salvage of acute fulminant myocarditis in a 53-year - old woman presented with malignant arrhythmia and cardiogenic shock supported by ecmo .

acute disseminated encephalomyelitis ( adem ) is an autoimmune inflammatory disorder of the central nervous system ( cns ) . etiopathogenesis is thought to be immune mediated , because in up to three - fourths of the cases ; it follows an antecedent infection or immunization . currently for adem , magnetic resonance imaging ( mri ) is the imaging modality of choice to demonstrate lesions in white matter of brain . there are no specific biomarkers available currently to diagnose adem ; hence , diagnosis is made after excluding clinical and laboratory findings and suggestive neuroradiological features of other disease . a 4-year - old boy was brought to our hospital with complaints of slurring of speech and difficulty in swallowing and fever for 2 days . he was evaluated at emergency and his gcs was 10/15 ( e4v2m4 ) . on examination , he was in altered sensorium ; tone was increased in upper and lower limb with brisk deep tendon reflexes . he was started on with iv antibiotic ( ceftriaxone ) , acyclovir and supportive measures . prior to this he had fever for 5 days , 8 days back along with mild cough and cold . on day 4 of fever child had one episode of seizure . mri of brain was done which was unremarkable except for mildly dilated lateral and third ventricle . he was managed with iv ceftriaxione and iv acyclovir iv phenytoin and other supportive measures . two days later patient developed slurring of speech and difficulty in swallowing with fever for which he was admitted in our hospital . initial investigations revealed hb - 11.8 gm / dl , tlc -15200/mcl , platelet - 3.13 lac . / l , typhi dot , widal test , blood c / s and malarial antigen test was negative . mri of brain was done which showed inhomogeneous area of increased signal in t2 weighted images in basal ganglia , pons , midbrain , medulla , thalami , and frontal cortex [ figures 13 ] . lumbar puncture revealed csf cell count was 3 cells ( 100% lymphocytes ) , protein - 32 mg / dl and glucose - 56 mg / dl ( capillary blood glucose was 86 mg / dl ) ldh - 12 u / l . csf bacterial antigen test causing encephalitis ( streptococcus group b , h. influenzae b , s. pneumoniae , n. meningitidis , e. coli k1 ) , herpes igm test and herpes pcr were negative . child was started on iv methylprednisolone ( 500 mg ) pulse therapy for 3 days , following which he was started on oral steroid therapy . gradually his sensorium became better . by day 5 , he started responding to commands . on day 6 he was discharged and at the time of discharge he was conscious oriented with gcs of e4 v5 m6 . he was seen on follow up after 7 days and neurological examination revealed alert child with mild slurring of speech . mri t2 weighted image showing areas of hyper intensity involving brainstem mri t2 weighted image showing areas of hyper intensity involving caudate nucleus and thalamus mri t2 weighted image showing areas of hyper intensity involving white matter the annual incidence of adem is reported to be 0.40.8 per 100,000 and the disease more commonly affects children and young adults in winter / spring . most of the case are reported post - exanthematous infection or vaccination . it may have abrupt , acute , or may evolve over a period of few days . adem typically presents as a monophasic illness but sometimes may have a biphasic or multiphasic course depending on the neuraxis affected . characteristic clinical features include sudden onset multifocal neurologic disturbances such as visual field defects , aphasia , motor and sensory deficits , ataxia , movement disorders , a depressed level of consciousness , focal or generalized seizures , and psychosis . as in our case , child presented with slurring of speech with difficulty in swallowing , altered sensorium and seizure . csf is usually normal , but sometimes mild elevation of protein with lymphocytic pleocytosis can be found . markers such as oligoclonal immunological bands , igg or myelin basic protein ( mbp ) are sometimes detectable , but not diagnostic . the electroencephalogram ( eeg ) often shows non - specific features of an encephalopathic process , and visual evoked potential ( vep ) responses may be delayed . in the absence of specific biologic markers , the diagnosis of adem is based on the clinical and radiologic features . in our case csf and vep studies were normal . with the wider use of mri , adem mri t2 enhancing images shows disseminated multifocal lesions in the white matter , basal ganglia , thalamus , and brainstem consistent with edema , inflammation , and demyelination . sometimes during initial course of disease we may find a normal mri brain . initial mri scan in our case had no evidence of adem but later mri revealed finding suggestive of adem . however , the recovery is incomplete in patients with adem not receiving any form of immunomodulatory treatment . most of the literature is in consensus with the use of high - dose intravenous methyl prednisolone , intravenous immunoglobulin ( ivig ) , and plasmapharesis as various modality of treatment . intravenous methyl prednisolone is the first - line drug ( 1030 mg / kg / day , up to a maximum of 1 g / day ) for 35 days followed by oral corticosteroid treatment continued with gradual tapering over 6 weeks to reduce the risk of relapses . intravenous immunoglobulin ( ivig ) ( 0.4 gm / kg / day for 5 days ) is another option . either plasma exchange or ivig , could be the second - line treatment , when corticosteroids fail . due to lack of any pathognomonic clinical feature or specific biomarker few differential diagnoses first priority should be to rule out infective causes of meningoencephalitis after ruling out infective causes demyelinating inflammatory process should be looked for . the outcome of adem is generally good , with 5789% of children making a full recovery . adem is considered to be monophasic illness but relapse may occur and if it represents same acute monophasic immune process , the term multiphasic disseminated encephalomyelitis ( mdem ) is used . but it must be differentiated from second attack of multiple sclerosis which may take months to years and more commone in older age groups . patient presenting with optic neuritis , ocular lesions , oligoclonal bands in csf examination , disseminated in space and time and periventricular lesion in mri goes in favor of multiple sclerosis ( ms ) . it is very necessary to differentiate adem / mdem from ms as early institution of therapy may alter course of ms .

acute disseminated encephalomyelitis ( adem ) is a rare disease of central nervous system with myriads of presentation . it is a diagnosis of exclusion and relies on neuroimaging which may be normal at the onset . it is a diagnostic challenge at its first attack . here we present a case of adem which initially presented with atypical feature and normal neuroimaging but later turned out to be a case of adem . early diagnosis and treatment holds the key for favorable outcome .

colonic necrosis has been described as a rare complication after the administration of kayexalate [ 1 , 2 ] . in this case study , we present a case of calcium polystyrene sulfonate - induced colonic necrosis and perforation to remind clinicians of this rare , but dangerous , toxicity associated with this commonly used medication . a 78-year - old woman with stage 4 chronic kidney disease ( ckd ) due to chronic pyelonephritis , and a right hypoplastic kidney was presented to our emergency department with a 2-day history of abdominal pain . her medical history included epilepsy that was treated with carbamazepine , hypertension and hyperlipidaemia , for which she received carvedilol and atorvastatin . because of the persistent hyperkalaemia , she was treated with calcium polystyrene sulfonate at 30 g / day ( anti - potassium granule ; assos drug , istanbul , turkey ) . as a result of the ckd , she was prescribed calcitriol for a mineral and bone disorder , darbepoetin alpha for anaemia and sodium hydrogen carbonate for acidosis . upon initial physical examination , her abdomen was non - distended with normoactive bowel sounds , but there was diffuse tenderness in her abdomen . significant laboratory values at the time of admission included sodium 138 meq / l , potassium 4.6 meq / l , ph 7.20 , bicarbonate 13 meq / l , blood urea nitrogen 71 mg / dl , creatinine 2.6 mg / dl , leukocytes 15 500/mm , eosinophils 110/mm , haematocrit 32.7% , platelets 221 000/mm and c - reactive protein 10.9 mg / dl . on the second day of hospitalization , the abdominal pain worsened and free air under the diaphragm was found on abdominal radiography . her sigmoid colon was found to be necrotic and perforated . a biopsy was performed and the perforated segment was repaired by primary closure . the surface of the deep ulcer contained necroinflammatory debris and various sized fragments of basophilic crystalloid material with angulated margins on microscopic examination ( figure 1a and b ) . also , there were no features of chronic colitis , including inflammatory bowel disease or chronic ischaemic colitis . ( a ) the basophilic crystalloid material in the necroinflammatory background of the ulcer surface ( haematoxylin and eosin , original magnification , 100 ) . ( b ) the basophilic crystalloid material in the necroinflammatory background of the ulcer surface ( haematoxylin and eosin , original magnification , 400 ) . sodium polystyrene sulfonate can also bind intraluminal calcium , leading to constipation , fecal impaction and subsequent bowel obstruction or perforation . gerstman et al . reported a 0.27% overall incidence , with a higher incidence ( 1.8% ) during the postoperative period . sorbitol enemas , along with experimental evidence suggesting that the necrosis was due to sorbitol rather than the kayexalate in presence of uraemia . extensive transmural necrosis was noted in rats receiving enemas of sorbitol or kayexalate in sorbitol in both the uraemic and non - uraemic groups . as in this case report , renal failure may be an important facilitating factor in the pathogenesis of the necrosis . in contrast to the experimental data , our case was treated with calcium polystyrene sulfonate orally , not rectally . rashid et al . noted that kayexalate in sorbitol given as an enema or orally to treat hyperkalaemia has been reported to induce intestinal necrosis in uraemic patients . they studied clinical and pathologic features of 15 patients and observed kayexalate crystals in tissue specimens from surgical resections and endoscopic biopsies . one possibility is elevated renin levels , commonly seen in renal insufficiency , that predispose the patient to non - occlusive mesenteric ischaemia via angiotensin - mediated vasoconstriction . one gram of kayexalate possesses a theoretical in vitro exchange capacity of 23.1 meq of potassium and in vivo capacity of 1 meq . emmett et al . reported that in vivo potassium - binding capacity may be lower than previously estimated , more on the order of 0.40.8 meq / g of kayexalate resin . in contrast to other minor digestive complications associated with kayexalate treatment , colonic perforation results in significant morbidity and mortality . as a result , potassium exchange resins may , although rarely , induce a colonic perforation , and this diagnosis should be considered in a patient treated as such in case of acute abdomen . the clinicians must be aware of the possible rare and serious complications of potassium exchange resins .

sodium or calcium polystyrene sulfonate ( kayexalate or analog ) is an ion - exchange resin commonly used to treat hyperkalaemia in patients with chronic kidney disease . it is known to cause digestive complications , such as nausea , vomiting and constipation . although rare , colonic necrosis and perforation are very severe complications associated with the medication . in this case report , we present a case of calcium polystyrene sulfonate - induced colonic necrosis and perforation to remind clinicians of this rare , but dangerous , toxicity associated with this commonly used medication .

development of spontaneous pneumopericardium is a very rare complication of tuberculosis with coexisting human immunodeficiency virus ( hiv ) infection . to the best of our knowledge only three cases of pneumopericardium with pulmonary tuberculosis concomitant with hiv infection[13 ] have been reported so far . a 30-year - old hiv - positive male smoker on antiretroviral therapy ( art ) presented with complaints of cough with expectoration and fever for two and a half months . cough was moderate in intensity and was present throughout the day with worsening at night . patient had intermittent fever with an evening rise . there were associated complaints of decreased appetite and loss of weight . bp was 110/70 mm of hg and temperature was 99.2f with a respiratory rate of 20/min . a thin hyper - lucent line was present lining the lateral cardiac borders suggesting possibility of pneumopericardium [ figure 1 ] . the air or gas in x - ray chest did not rise above the upper level of pericardium in standing erect position differentiating it from pneumothorax and pneumomediastinum . computed tomography ( ct ) scan of the chest confirmed the presence of pneumopericardium [ figure 2 ] . it also showed a well - defined thick walled cavity with necrotic areas in apicoposterior segment of left upper lobe and bilateral infiltration with minimal bronchiectatic changes [ figure 3 ] . x - ray chest pa view showing patchy infiltration in both the upper zones with pneumopericardium ct scan showing bilateral infiltration with pneumopericardium ct scan showing cavitary lesion on the left side with pneumopericardium barium swallow fluoroscopy ruled out any esophagopericardial fistula . the patient did not have any symptoms related to pneumopericardium and its detection was purely incidental . patient was started on anti - tubercular treatment ( att ) as per the guidelines of revised national tuberculosis control program . pneumopericardium was first described by bricheteau in 1844 who named bruit de moulin ( water wheel sound ) associated with pneumopericardium . pneumopericardium occurs typically because of the breech of the pericardium by traumatic or nontraumatic causes . in adults , 60% of pneumopericardium results from trauma either sharp or blunt . iatrogenic factors include thoracocentesis , thoracic surgery , endotracheal intubation , sternal bone marrow puncture , or positive pressure mechanical ventilation . noniatrogenic causes can be underlying disease processes like infected fluid or gas producing organisms in the pericardial sac , fistulous communication between pericardium and other air containing structures such as bronchus , esophagus , or stomach and amebic liver abscess . other causes also include foreign body aspiration , physical exertion , parturition , severe cough , acute asthma , cocaine inhalation , chlorine gas exposure , and forceful emesis . the possible mechanism of pneumopericardium in this case could either be a severe bout of cough or a fistulous communication of pericardium with an infected contiguous organ . the rise in intra - alveolar pressure above atmospheric pressure due to increased bout of coughing may lead to rupture of alveoli and the released air moves to hilar area , mediastinum , and through pericardial reflections on the pulmonary vessels in the pericardial cavity . the parietal pericardium is reflected on the visceral pericardium near ostia of the pulmonary veins , the weakest histological area . nectrotizing pulmonary process like tuberculosis in the setting of hiv could have resulted in the fistulous communication between the lung and pericardium leading to pneumopericardium in this patient . pneumopericardium may be symptomatic or asymptomatic depending upon the quantity of air in the pericardium . the patient with a small pneumopericardium may be asymptomatic and the cardiac examination may be normal . it may only be diagnosed incidentally on a chest radiograph and the gas usually does not rise above the upper limit of the pericardium in the erect position , which differentiates the pneumopericardium from pneumomediastinum as in this patient . in large pneumopericardium the patient may be symptomatic and the note may be tympanic and heart sounds may be metallic . this complication is thought to be caused by a ball valve mechanism preventing air from leaving the pericardial space . pneumopericardium unlike pneumothorax often does not require any specific treatment and is usually self - limiting as in the present case . treatment is required in cases of large and symptomatic pneumopericardium or in patients of tension pneumopericardium , which can be a needle aspiration or tube decompression . oxygen therapy in high concentrations as in management of pneumothorax can also be helpful in absorption of air .

pneumopericardium is defined as a collection of air or gas in the pericardial cavity . it is a rare entity and spontaneous pneumopericardium is even rarer . it is a rare complication of tuberculosis and human immunodeficiency virus and just three cases have been reported so far .

caudal regression syndrome ( crs ) is a rare disorder of distal spinal segments affecting the development of the spinal cord , with attendant sequelae . the exact etiology is elusive , though maternal diabetes , genetic factors , and hypoperfusion might play roles . we report late presentation of crs in a 9 year old with scoliotic deformity with an asymptomatic cervical syrinx , in absence of any systemic abnormality . a 9-year - old girl was referred in view of a lateral deformity of dorso lumbar spine to explore the possibility of an underlying skeletal dysplasia . there was no history of trauma , associated weakness of any part of body , bowel and bladder involvement or protruding mass over the spine . child was born of third degree consanguious mating at full term by normal vaginal delivery at home . according to mother she was of normal intelligence with intelligence quotient ( iq ) of 105 on developmental assessment scales for indian infants ( dasii ) score . clinical examination revealed short stature with short trunk , the height being less than 3 centile with upper to lower segment ratio 0.9 . examination revealed multiple hypo pigmented patches over the left ear lobule , left angle of mouth , and mid dorsum of right leg . additional finding was evidence of dysgenesis at lumbo sacral region with abnormal orientation of sacrum [ figure 1 ] . magnetic resonance imaging ( mri ) spine revealed butterfly vertebrae at d10 level , the first three sacral segments were hypoplastic , distal sacrum and coccyx were absent . a syrinx was present in cervico - thoracic region opposite c5-t1 vertebrae [ figure 2 ] . x - ray showing lumbosacral dysgenesis with abnormal orientation of sacrum saggital neuroimaging shows rudimentary disc at l1-l2 , l3-l4 , visualization of only s1 and s2 segments and non - visualization of distal sacral segments and coccyx crs is a rare congenital malformation characterized by varying degrees of developmental failure first described by duhmel in 1964 to explain the spectrum of sacrococcygeal malformations . the developmental defects include the lower extremities , the lumbar spine , the coccygeal and thoracic vertebrae , and corresponding segments of spinal cord . a male to female ratio of 2.7:1 has been reported . however , upto 22% cases of crs are associated with diabetes mellitus in the mother , diabetic women being 200 times to 400 times more likely to have a child with crs . group 1 has blunt spinal cord termination above l1 , and is the most severely affected . group 2 has less severe dysgenesis with low - lying tapered spinal cord and tethered cord , which may be caused by tight filum , lipoma , etc . , thus , the best diagnostic clue for crs is dysgenetic lumbosacral vertebrae and abnormal distal spinal cord . the latter may explain or at least aggravate the scoliosis that was the only clinical sign in this child . the sacral dysgenesis below s2 suggests this is a group 2 case , therefore an associated tethering mechanism should be suspected . alternatively , probably the scoliosis may be explained by vertebral anomalies at d10 , l1 - 2 and l3 - 4 instead of a tethering mechanism , it is unlikely that a child with spinal cord tethering associated with a crs would merely present with a scoliosis . on the other hand , it is even more unlikely to presume there would be a scoliosis without an associated tethering mechanism , unless the scoliosis is related to the malaligned dysgenetic vertebra as seen in our case . the exact etiology of crs at embryonic level is thought to result from defect in induction of caudal elements in the embryo before the 4 week of gestation . the insult occurs at midposterior axis mesoderm , causing the absence of the development of the mesoblastic caudal bud . the proximity and interdependence of developing caudal neurons , spinal , hindgut and mesonephric elements involved in closure of the neural tube result , in the constellation of neural , distal vertebral , anorectal , renal , and genital abnormalities . the attenuation of bone morphogenetic protein signaling at the posterior primitive streak of embryos leads to the caudal dysmorphogenesis including the cloaca and fusion of both hind limbs . hedgehog - responding cells derived from peri - cloacal mesenchyme contribute to the urogenital / reproductive organs . these findings indicate the existence of developmental programs for the coordinated organogenesis of urogenital / reproductive tissues based on growth factor function and crosstalk . interestingly , structures that are developmentally separate from these caudal elements such as the brain , proximal spine and spinal cord , are generally spared by crs . the consequences of the disruption after the maturation of spinal cord 's caudal portion ensue after the 4 week of gestation are different . this results in motor deficits and neurologic impairment , varying from incontinence of urine and feces to complete neurologic loss . understandably , most children affected by crs- except the very mild cases - would have some problem with genito - urinary and/or anorectal anatomy and function while our case was surprisingly asymptomatic till 7 years of age . short trunk , late onset scoliosis and absence of anomaly of any other system are the unusual manifestations of our case . the presence of an asymptomatic cervical syrinx in association with crs in our case is also intriguing . the overall radiological incidence of syringomyelia in patients with distal spinal abnormalities has varied from 19% to 22.5% in asymptomatic group to 48.5% in symptomatic group . if one were to consider the most accepted theories concerning the pathogenesis of syringomyelia in children with crs , chiari ii malformation , hydrocephalus and tethered cord favour the formation of a cavitating lesion of the spinal cord . in fact , a sizeable spina bifida population , screened with cranial and spinal ultrasound in infancy , showed the absence of syringomyelia . the syrinx may start after the 1 year of life , and remain asymptomatic for a variable amount of time . in our child it was interesting to note the presence of segmental vitiligo in our patient with crs , which might be a chance assoction . neurosurgical consultation taken advocated stringent follow - up , but decided against prophylactic untethering in absence of neurological symptoms . given the fact that genetic and environmental causes may produce a very similar phenotype , a sharp division between syndrome and association is nearly impossible and makes counselling for recurrence risk challenging . the term caudal regression is probably incorrect since more than caudal structures are involved and nothing regressed that was previously present .

we report late presentation of caudal regression syndrome in a 9 year old presenting with a scoliotic deformity . she in addition had an asymptomatic cervical syrinx and vitiligo . we discuss the reasons for this unusual constellation of symptomatology present in our case .

it is caused by trauma , stress , sepsis , adrenal tumors , anticoagulation , hemorrhagic disorders and pregnancy . idiopathic adrenal hematoma. the symptoms vary from subclinical to clinical , such as nausea , abdominal pain , fever and hypotension due to circulatory collapse . adrenal tumors associated with hemorrhage primarily include pheochromocytomas , adrenocortical cancers and metastatic lesions from other organs . from a clinical perspective , whether the lesion is benign or malignant is an important issue but is difficult to determine prior to surgery . a 59-year - old japanese man was referred to our hospital for evaluation of a 7.0 cm mass in the posterior segment of the liver or right adrenal gland in november 2009 . this mass was discovered when abdominal ultrasonography was obtained to evaluate a weight loss of 8 kg from may to november 2009 . his body temperature was 37.0c , his blood pressure was 124/73 mm hg , and his pulse was regular at 67/min . hormonal examination revealed a slightly elevated metanephrine level ( 0.35 mg / day ; normal range 0.040.19 ) . tumor marker levels , including serum -fetoprotein , carcinoembryonic antigen and carbohydrate antigen 19 - 9 were all within normal ranges . only pivka - ii was slightly elevated at 53 mau / ml ( normal < 37 ) . abdominal computed tomography ( ct ) demonstrated a well - demarcated , 7.0 cm lesion in either the posterior segment of the liver or in the right adrenal gland . the peripheral region was slightly enhanced and the central part was hypovascular , indicating central necrosis ( fig . there was no evidence of lymph node enlargement or distant metastasis . on magnetic resonance imaging ( mri ) , , the mass showed arterial enhancement and a ct filling defect in the right superior and inferior adrenal arteries and arterial portography , respectively ( fig . i - meta - iodo - benzylguanidine ( mibg ) scintigraphy demonstrated no aberrant accumulation in the mass , which ruled out the possibility of a pheochromocytoma . for the purposes of securing a definite diagnosis and treatment , an open right adrenalectomy was planned for march 2010 . the resected specimen was 7.0 5.5 cm in size , and the cut surface of the mass was heterogeneous and dark red and white in appearance ( fig . hematoxylin - eosin staining revealed hemorrhage , necrosis and hemosiderin deposit with normal adrenal tissue . the patient had an uneventful postoperative course and was discharged home on postoperative day 12 . small adrenal branches from the three main adrenal arteries form a subcapsular plexus , and the gland is drained by relatively few venules . for example , stress increases adrenal vascularity and increases adrenal venous pressure due to vasoconstriction , resulting in intraglandular hemorrhage . idiopathic adrenal hematomas are difficult to diagnose prior to surgery . in japan , koizumi et al . described 14 cases of idiopathic adrenal hematomas from 1983 to 2010 . of these 14 cases , 13 had been suspected to be adrenal tumors including malignant lesions preoperatively , but were then found to be idiopathic adrenal hematomas on pathological examination after surgery . only 1 patient was diagnosed with an idiopathic adrenal hematoma without surgery . the adrenal hemorrhage was not suspected to be caused by an adrenal tumor on the basis of ct , hormonal assay and mibg results , and this mass spontaneously regressed after 2 weeks . although imaging modalities such as ct and mri are helpful in detecting adrenal hemorrhage , it is difficult to determine whether the hemorrhage is associated with tumors or not . adrenal hemorrhage associated with pheochromocytoma ( 48% ) was the most frequently observed situation , and the second most frequently observed was a malignant lesion ( 20% ) such as adrenocortical cancer or metastasis from another organ . hematomas derived from pseudocysts or adenomas comprised 17% of all cases . therefore , even if pheochromocytoma is ruled out by hormonal evaluations and imaging studies , the possibility that the mass may be a malignant lesion remains approximately 50% . furthermore , the size of adrenal incidentalomas , which are adrenal gland masses discovered serendipitously on imaging , is an important factor in differentiating benign tumors from malignant lesions . nieman recommended routine surgical resection for adrenal incidentalomas > 4 cm in diameter without a clear - cut diagnosis . in our case , metastatic liver tumors , hemorrhaged hepatic cellular carcinoma with central necrosis and adrenal tumor were raised as differential diagnoses at the time of the initial consultation because of ring enhancement with hypovascularity on enhanced ct and a slightly elevated pivka - ii level . subsequently , abdominal angiography revealed the origin of the mass to be the right adrenal gland . although vital signs , laboratory data and mibg scintigraphy ruled out the possibility of a pheochromocytoma , the possibility of another malignancy could not be completely ruled out . therefore , surgery was strongly recommended for a definite diagnosis as well as for treatment . the reason for the patient 's weight loss and the increase in the pivka - ii in our case was not clear , but may have been related to exhaustion during the summer season from may until november that kept him from eating sufficiently . in fact , his weight loss ceased after he consulted our hospital , and the pivka - ii value also normalized prior to surgery . in conclusion , we report herein a case of idiopathic adrenal hematoma . an accurate diagnosis of idiopathic adrenal hemorrhage is quite difficult to make prior to surgery . some imaging modalities are useful in generating a differential diagnosis , but if the potential for malignancy is not excluded , surgical resection should be taken into consideration .

we report herein a case of idiopathic adrenal hematoma . a 59-year - old japanese man was referred to our hospital for evaluation of a 7.0 cm mass in the right upper abdominal cavity . the tumor was suspected to originate from either the posterior segment of the liver or the right adrenal gland . his chief complaint was weight loss of 8 kg over the previous 6 months . he had no past medical history and took no medications , including no anticoagulants . laboratory data were almost normal except for a slight elevation of pivka - ii . the origin of the tumor was found to be the adrenal gland , as angiography revealed the blood supply to the mass to derive from the right superior and inferior adrenal arteries . a fine needle biopsy of the lesion was unable to confirm the diagnosis . open right adrenalectomy was performed . the histopathological findings of the surgical specimen revealed a hematoma with normal adrenal tissue . in the absence of any obvious etiology , the diagnosis was idiopathic adrenal hematoma .

congenital scrotal anomalies are unusual and include penoscrotal transposition , bifid scrotum , ectopic scrotum , and accessory scrotum ( as ) . among these , as is the least frequent , with only 42 cases reported in the english literature . as is characterized by additional scrotal tissue lacking a testis , besides a normally developed scrotum . various associated anomalies have been reported . in particular , contiguous subcutaneous tumor is the most frequently associated abnormality and is reported to be related to the etiology of as . although prenatal screening techniques have advanced , most reported cases of congenital perineal mass have been identified after birth . a 28-year - old woman was referred to our hospital for the evaluation of a fetal perineal mass at a gestational age of 31 weeks . prenatal ultrasonography and magnetic resonance imaging ( mri ) showed a mass of 1.0 1.2 cm located posterior to the scrotum in a male fetus . 1 ) the likely diagnosis was lipoma and the mass maintained a stable appearance until delivery . the male newborn was delivered vaginally at 38 weeks of gestation and his body weight was 2208 g. there were no specific symptoms after birth . arrowheads indicate a 1.0 1.2 cm mass located posterior to the scrotum . on physical examination , the soft peduncular mass , measuring 2.0 cm in diameter , was attached to a midperineal skin tag . there was also a rugged pigmented swelling on the mass , measuring 0.7 cm in diameter , which resembled the scrotum . the perineal mass showed high signal intensity on t1- and t2- weighted images and the signal intensity was suppressed on fat - suppressed t1-weighted images . mri revealed no associated abnormalities of the intraabdominal organs , musculoskeletal system , or genitourinary system . a. a soft peduncular mass with a rugged and pigmented swelling is located posterior to the normally developed scrotum . c. the mass is attached at the midperineum with a skin tag . the preoperative diagnosis was as with perineal lipoma , and we completely excised the mass under general anesthesia at one month of age . the postoperative course was uneventful , and the patient was discharged on the day after the operation . there was no recurrence or functional sequelae within a follow - up period of six months . a histological examination revealed that the peduncular mass consisted of mature adipose tissue . in this case , it was difficult to distinguish between lipoma and normal adipose tissue pathologically . however , our clinical diagnosis was lipoma because the peduncular mass was separated from normal perineal region by the skin tag . the rugged swelling on the peduncular mass showed smooth muscle fibers in the subcutaneous layer , which represented the tunica dartos . 3 ) the swelling was definitively diagnosed as as . histological examination . b. the peduncular mass smooth muscle fibers in the subcutaneous layer of the rugged swelling represent the tunica dartos . these swellings appear at four weeks of gestation and migrate to the caudal portion after 12 weeks of gestation . abnormal migration or early division of the labioscrotal swellings is possibly related to the etiology of congenital scrotal anomalies . the least frequent congenital scrotal anomaly is as , characterized by additional scrotal tissue without a testis , besides a normally developed scrotum . ( table ) characteristics of accessory scrotum various anomalies associated with as have been reported . in particular , contiguous subcutaneous tumor has a high incidence ( 72.5% ) of association with as . histologically , one case of subcutaneous tumor was lipoblastoma , three cases were hamartoma , and the others were consistent with lipoma . ( table ) it is assumed that the contiguous subcutaneous tumor is related to the etiology of as . sule hypothesized that as develops when intervening mesenchymal tissue ( i.e. , the developing subcutaneous tumor ) disrupts the continuity of the developing caudal labioscrotal swelling . however , the complete etiology of as is not explained by this hypothesis because as can occur with no contiguous tumor . takayasu hypothesized that as develops from the early division and teratoid growth of pluripotential labioscrotal tissue elements . however , two cases associated with skeletal abnormalities were located in the pubic area , and one case was located on the distal penile shaft . our case was detected at a gestational age of 31 weeks , and the other two reported cases were detected at 24 weeks and 32 weeks of gestation . a congenital perineal mass is unusual in itself , and most reported cases have been diagnosed after birth . however , detection is possible with careful prenatal screening . the differential diagnosis of a fetal perineal mass includes lipoma , lipoblastoma , infantile hemangioma , hamartoma , and choristoma . if a fetal perineal mass is detected during the antenatal period , it is important to look for any associated congenital anomalies . the prognoses of surgically treated patients are good , and only one has died , from an associated anomaly before surgery . the reported ages at surgery range from four days to 46 years ( median , nine months ) , and three adult cases are recorded in the literature . our patient was operated upon in the neonatal period because the mass was considered to be excisable without complications and the associated subcutaneous tumor had a low probability of malignancy . although a fetal perineal mass is difficult to diagnose , it can be detected with careful prenatal screening . many ass are associated with contiguous subcutaneous tumors , which are assumed to be related to the etiology of as .

abstractwe report a case of accessory scrotum ( as ) in the perineal region with peduncular lipoma , diagnosed prenatally . a male fetus of 31 weeks gestation was referred to our department with a perineal mass . prenatal ultrasonography and magnetic resonance imaging showed a mass of 1.0 1.2 cm located posterior to the scrotum . no other abnormalities were noted during pregnancy . the patient was delivered vaginally at 38 weeks of gestation . on physical examination , a soft peduncular mass with a rugged and pigmented swelling was located between the normally developed scrotum and the anus . there were no specific symptoms or any other associated congenital anomalies . we completely excised the mass at one month of age . a histological examination revealed lipoma , with tissue suggestive of scrotum , so a definite diagnosis of as was made . as is a rare congenital anomaly of the scrotum . we review the literature .

the recognition that elevated intracranial pressure ( icp ) is transmitted through the optic nerve and its sheath has been known for many years . this physiological process is the basis for the physical exam finding of papilledema on fundoscopic examination . recently , interest has turned to measurement of the optic nerve sheath diameter ( onsd ) through non - invasive imaging technologies to provide surrogate markers for early elevated icp . in this issue of critical care , geeraerts and colleagues present their research correlating magnetic resonance imaging ( mri ) measurements of onsd with icp . in a retrospective review of 38 patients with traumatic brain injury requiring both invasive icp monitoring and mri , they found a significant positive relationship between onsd measured by mri and icp ( r = 0.71 ) . the best cut - off value to detect an icp > 20 cm h2o based on a receiver operating characteristic curve was found to be onsd = 5.82 mm with a sensitivity of 90% and a specificity of 92% . the optic nerve is surrounded by cerebrospinal fluid ( csf ) , which is contiguous with intracranial csf . increased icp is transmitted through this subarachnoid space causing distention of the dural optic nerve sheath , especially the retrobulbar segment . the optic nerve and its surrounding sheath can be imaged and measured on mri using a fat - suppressed t2-weighted sequence . mri has been used to demonstrate increased onsd in idiopathic intracranial hypertension , and interestingly , decreased onsd in csf hypotension . the onsd has also been shown on mri to decrease after drainage of subdural hematomas . the research presented by geeraerts and colleagues is unique in its comparison of onsd with simultaneous direct measurements of icp through invasive monitoring . their findings generally correlate with a growing body of research using bedside ultrasound measurements of onsd to detected elevated icp . original research with lumbar intrathecal infusions performed by hansen and helmke demonstrated rapid changes in the onsd with alteration of csf pressures . in emergency department patients with traumatic brain injury , the onsd correlates with signs of elevated icp on computed tomography scans . more recently , researches have compared bedside ultrasound measurements of onsd to invasive icp [ 11 - 13 ] . while there is some variation in the optimal cut - off value , the correlation between onsd and icp remains consistent . in their current article , geeraets and colleagues provide further evidence of this physiological relationship and an intriguing possibility for non - invasive assessment of icp using mri . the obvious drawbacks to mri include its expense , long acquisition times , need for patient transport , and limited availability . however , some research has shown that mri may provide more precise measurements then ultrasound . geeraerts and colleagues used a conventional t2 sequence with relatively large slice thickness and interslice spacing , resulting in an overall feasibility of measuring the onsd in 95% of patients . greater accuracy and reliability would be expected in coronal t2 slices with thinner slices . as mri becomes more accessible and faster , non - invasive mri measurements may prove to be useful in certain clinical settings and as a potential reference standard for further research . continued research with larger studies is required to confirm the precision and accuracy of mri measurements of onsd , as well as the optimal measurement technique . additionally , the time course of onsd distention and reduction needs to be further delineated . currently , non - invasive assessments of icp do not obviate the need for invasive icp monitoring . invasive monitoring detects minute to minute variations in icp and , in the case of intraventricular drains , can also be therapeutic . however , non - invasive screening tests may be useful in select populations who would not otherwise require invasive monitoring and could undergo mri scans , such as patients with liver failure , meningitis , stroke , and moderate traumatic brain injury . in summary , the study by geeraerts and colleagues adds to a growing body of research demonstrating a correlation between increased onsd and elevated icp . by demonstrating the correlation of mri measurements of the onsd with invasive icp monitoring , they illustrate the potential of yet another non - invasive method to screen for elevated icp . while this technique will not replace invasive icp monitoring , it may be useful in select patient populations that would not otherwise have invasive monitoring but are at high risk for elevated icp . further research is required before we can use measurements of the onsd to predict exact values of icp , but it may be useful as a screening test to estimate the probability of elevated icp . csf : cerebrospinal fluid ; icp : intracranial pressure ; mri : magnetic resonance imaging ; onsd : optic nerve sheath diameter .

the current gold standard for the diagnosis of elevated intracranial pressure ( icp ) remains invasive monitoring . given that invasive monitoring is not always available or clinically feasible , there is growing interest in non - invasive methods of assessing icp using diagnostic modalities such as ultrasound or magnetic resonance imaging ( mri ) . increased icp is transmitted through the cerebrospinal fluid surrounding the optic nerve , causing distention of the optic nerve sheath diameter ( onsd ) . in this issue of critical care , geeraerts and colleagues describe a non - invasive method of diagnosing elevated icp using mri to measure the onsd . they report a positive correlation between measurements of the onsd on mri and invasive icp measurements . if the findings of this study can be replicated in larger populations , this technique may be a useful non - invasive screening test for elevated icp in select populations .

children spend from 31 to 60% of their school day either writing or performing other fine motor tasks2 . the preparatory skills for writing are coordination of multiple joints , visual perception , vision - motor integration , and proprioception3 . together with stereognosis , proprioception allows smooth joint movement when vision is impaired or absent5 . children with poor proprioception have problems with handwriting legibility because their grip on a pen is too strong or too weak4 . generally , proprioception can be measured with joint position sense ( jps ) and kinesthetic sense ( ks)6 . while there are many studies investigating children s handwriting , quantitative studies on the association between handwriting and proprioception involving jps and ks are lacking7 . the aim of this study was to investigate the association between proprioception , involving jps and ks , and handwriting legibility in children . nineteen healthy children ( 15 boys and 4 girls ) with an average age of 9.7 0.36 years participated in this study . prior to the study , the children and their parents were informed about the purpose of the study and the general procedures to be undertaken . all children and the study was approved by the kaya university of human health science studies committee . the investigator moved the child s right arm passively through 80 of flexion at the elbow ( from 30 to 110 ) . the children were then directed to repeat this motion 10 times following a metronome set at 1 s intervals . their movements were recorded by a compact measuring system ( cms ) 10 for 3d motion analysis ( using the winarm software zebris medical gmbh , germany ) . cms markers were placed at the greater tubercle and lateral epicondyle of the right humerus and at the right wrist . angles of deviation from the targeted range of flexion were analyzed using matlab version 2014a ( the match works inc . , 2014 ) . ks was measured by the kinesthesia item in the sensory integration and praxis tests ( sipt , wps , torrance , ca , usa ) . the investigator moved a finger of the subject passively along a line from beginning to end , allowing the child to learn the line direction and distance . then , blindfolded and after one trial , the children were directed to trace five lines for each hand . only the results of the right hand were analyzed in this study . the investigator then measured the distance between the test endpoints and the real endpoints of the lines . legibility was evaluated using form , alignment , space , size , and slope8 . the legibility score was calculated as the ratio of the number of clearly written words to the total number of words ( score for legibility ( % ) = number of letters that received 5 points/30 100 ) . all data were analyzed using ibm spss statistics 20.0 ( ibm corp . , armonk , ny , usa ) . spearman s rank correlation was used to determine the relationships between legibility of handwriting and jps and ks , with significance defined as p<0.05 . values obtained for jps and ks in relation to handwriting legibility are shown in table 1table 1.handwriting legibility and proprioception test results ( n=19)mean sdjoint position sense 110 ( degree)22.530 10.063joint position sense 30 ( degree)14.256 10.408kinesthetic sense ( cm)2.044 0.703handwriting legibility ( % ) 33.521 15.852*standard deviation . there was no correlation between writing legibility and jps at either 30 or 110 of elbow flexion ( p>0.05 ) . a high ks correlated significantly with legible handwriting ( p<0.05 , table 2table 2.correlation between handwriting legibility and proprioception ( n=19)handwriting legibility ( % ) joint position sense 110 ( degree)0.016joint position sense 30 ( degree)0.009kinesthetic sense ( cm)0.370*p<0.05 ) . this study investigated the association between the legibility of handwriting and jps and ks in young children . children aged between 7 and 8 years are expected to be proficient in building up their speed of handwriting , ensuring consistency in size and proportions of letters as well as the spacing between letters and words9 . the average age of the participants in this study was 9.7 years old . our results showed that a highly accurate ks was associated with higher legibility scores . ks provides ongoing error information and memory storage to be recalled when writing is repeated . a high ks leads to programmed error correction , and the upgraded program generates better writing legibility . the results of this study support the hypothesis that ks reinforces the linkage between visual and motor control required for clear handwriting15 . we studies jps at the elbow joint because proprioception at the elbow joint is necessary for performing fine manipulative tasks including handwriting16 . studies have reported that proprioception at the wrist joint17 and finger joints18 influences handwriting legibility . we looked at children within a limited age range between 9 and 10 years of age . further , we did not control for other factors that affect writing legibility , such as fine motor control and visual - motor integration19,20,21 . finally , although handwriting quality is measured in terms of legibility and speed , this study investigated only writing legibility .

[ purpose ] this study investigated the association between proprioception , including joint position sense and kinetic sense , and handwriting legibility in healthy children . [ subjects and methods ] assessment of joint position sense , kinetic sense , and handwriting legibility was conducted for 19 healthy children . joint position sense was assessed by asking the children to flex their right elbow between 30 to 110 while blindfolded . the range of elbow movement was analyzed with compact measuring system 10 for 3d motion analysis . kinetic sense was assessed using the sensory integration and praxis test . the children were directed to write 30 words from the korean alphabet , and the legibility of their handwriting was scored for form , alignment , space , size , and shape . to analyze the data , descriptive statistics and spearman correlation analysis were conducted using ibm spss statistics 20.0 . [ results ] there was significant negative correlation between handwriting legibility and kinetic sense . a significant correlation between handwriting legibility and joint position sense was not found . [ conclusion ] this study showed that a higher kinetic sense was associated with better legibility of handwriting . further work is needed to determine the association of handwriting legibility and speed with joint position sense of the elbow , wrist , and fingers .

pectus excavatum ( pe ) , characterized by posterior displacement of the sternum and cartilaginous - rib attachments , is one of the most common congenital chest wall deformities.1)2 ) the physiological impact of pe varies . symptomatic patients frequently complain of dyspnea with exertion , progressive loss of endurance , tachycardia , palpitations and chest discomfort.3 - 5 ) in a critical location , even small depressions of the chest can create significant cardiac dysfunction when the right heart and pulmonary outflow tract are compressed to varying degrees by the depressed sternum.5 - 7 ) an index of severity can be calculated by measuring the inner width of the chest ( at the lowest level of the pectus defect ) and dividing it by the distance between the posterior surface of the sternum ( at the lowest part of the defect ) and the anterior surface of the spine ( with normal being approximately 2.52).2)3 ) in general , an index of 3.2 or greater is considered severe however symptoms do not necessarily correlate with severity of index.2 - 4 ) echocardiography plays a significant role in the evaluation of patients with pe . the degree of right heart compression however is often difficult to assess by transthoracic echo ( tte ) especially with severe deformities that prevent the operator from obtaining the normal transthoracic views.7 - 9 ) a 32-year - old woman presented to out - patient clinic for further evaluation of a 1-year history of progressive chest pain , fatigue , dizziness , paroxysmal tachycardia and dyspnea with exertion . with even mild exertional effort she experienced sharp , " stabbing " chest pain along the left lower and mid - sternum . a 12-lead electrocardiogram demonstrated a right - bundle branch block with left posterior fascicular block . she had a history of severe pe ( index of > 4 ) for which she had undergone operative repair 18 months prior by an open resection of cartilage attachments and sternal " flip " as described by hawkins & colleagues.10 ) prior to her first correction , she had some dyspnea with exertion , however , cardiac work - up , including echocardiogram had been reported to not show any abnormalities . she noted onset of her current symptoms approximately 6 months after her pe repair . a subsequent operation with superficial anterior remodeling of the chest wall cartilage on the left side of the sternum had failed to relieve her progression of pain and symptoms . she exhibited post - operative abnormal remodeling of the chest wall with residual as well as some recurrent pe . the sternum protruded anterior with bilateral depression of the costal - sternal attachments creating a " wave - like " appearance ( fig . close attention to the anteroposterior planes ( best seen from the apical four - chamber views ) clearly demonstrated extrinsic compression and deformation of the lower mid - right ventricle ( rv ) by the chest wall which is more obvious during diastole ( fig . 3b ) . biplane and live 3-d images of the preoperative transesophageal echocardiogram ( tee ) improved the visualization and localization of extrinsic compression of the right ventricle ( fig . tricuspid valve prolapse is seen likely due to partial compression of the rv resulting in distortion of the tricuspid annulus ( fig . open revision of her chest wall deformity was performed with placement of two stainless steel support bars ( lorentz surgical , jacksonville , fl , usa ) and a trabecular metal implant ( zimmer , inc . , minneapolis , mn , usa ) which elevated the sternum and depressed regions 3 - 4 centimeters anterior to the rv with good cosmetic results ( fig . symptomatic pe patients often present for cardiovascular evaluation with tte being the most commonly utilized diagnostic modality . documenting the effects of a depressed chest wall on the right heart and outflow tract as well as any associated interference with diastolic filling is critical for decision making in patient treatment and the need for potential surgical intervention . modifying standard views such as biplane transthoracic and transesophageal views may be necessary in some patients due to limitations from the abnormal anatomy of the deformed anterior chest wall . in some cases , rv compression is often difficult to assess by tte especially with severe deformities that prevent the operator from obtaining the normal transthoracic window views . technical tips for assessment of the rv include 1 ) narrowing the 2-d sector width to optimize only rv structures ; 2 ) use of harmonic imaging and adjustment of gain and compression for good contrast and endocrinal edge detection ; 3 ) making all measurements at end - diastole or the frame demonstrating the largest chamber dimension ; and 4 ) for 2-d measurements , obtain all acquisitions during quiet respiration or full - expiration . apical four - chamber views when seen clearly can usually visualize any extrinsic compression to the rv of the heart . use of all three possible traditional acoustic windows ( parasternal short - axis , apical and subcostal ) may be necessary . in an effort to minimize foreshortening of the rv , the transducer can be positioned down an intercostal space and laterally until the rv apex is clearly seen . in some patients a transesophageal and transgastric view may be necessary to better evaluate the right heart chambers and rv outflow obstruction . many patients with pe have associated alterations in rv morphology and function . assessment for localized sacculation of the rv wall , global dilation of the ventricle , prominent trabeculae , and/or hypertrophy of the moderator band is important . identifying abnormalities , including mitral valve prolapse and aortic root measurements are especially critical in suspected or confirmed cases of marfan syndrome . resolution of mitral valve prolapse with release of the chest wall entrapment is seen in more than half of patients after pe correction.7)11)12 ) more importantly , many patients improve their symptoms such as exertional dyspnea and chest pain which may be related to extrinsic compression of the rv and reduced preload.4)5)7 ) further studies are needed to better understand the pathophyiology of symptoms in relation to cardiac and chest wall function in patients with pe . in conclusion , echocardiographic evaluation is critical in patients with symptomatic pe to assess the degree of rv compression . we presented a case of a pe in a patient with symptoms including severe chest pain with exertion that was diagnosed with the expertise of echocardiography and subsequently surgically corrected . subtle abnormalities in cardiac structure and chest wall compression may result in debilitating symptoms in a small population of patients.2 - 4 ) when astutely performed , echocardiography can accurately provide clinically relevant information about cardiac size and any hemodynamic compromise in patients with pe .

pectus excavatum exists as varying anatomic deformities and compression of the right heart by the chest wall can lead to patient symptoms including dyspnea and chest pain with exertion . echocardiography can be difficult but is critical to the evaluation and diagnosis of this patient population . modifying standard views such as biplane transthoracic and 3-d transesophageal views may be necessary in some patients due to limitations from the abnormal anatomy of the deformed anterior chest wall . apical four - chamber views when seen clearly can usually visualize any extrinsic compression to the right ventricle of the heart .

the adenoid cystic carcinoma ( acc ) is a relatively rare epithelial tumor of the salivary glands . it accounts for about 5 - 10% of all salivary gland neoplasms , representing 2 - 4% of malignant occurrences of the head and neck area . approximately , 31% of lesions affect minor salivary glands , particularly the palate , although they can also be observed in the sub - mandibular and parotid glands . the frequency reported in the tongue is 19.8% , with 85% observed at the base of the tongue . we report one such rare case of tongue neoplasm which turned out to be acc in a middle aged lady . a 45-year - old - female patient presented with an asymptomatic growth of the tongue , which was perceived just 2 weeks before consultation . the intra - oral examination at that time revealed a mass in the dorsum of the tongue with light pain to pressure , without any evidence of cervical lymphadenopathy . the mass was firm , same color as that of the surrounding mucosa and asymptomatic otherwise [ figure 1 ] . as a pre - operative assessment of the lesion , a fine needle aspiration was done and the smear revealed a salivary neoplasm consisting of well delineated , tightly cohesive clusters of basaloid cells surrounding mucoid , hyaline globules , or clear spaces also forming honeycomb ( cribriform ) pattern [ figure 2 ] . at places dense aggregates of monomorphic small cells with uniform round to oval hyperchromatic nuclei and scanty cytoplasm were seen . smears also showed individual tumor cells with high n : c ratio and nuclear moulding . macroscopically , the mass had firm consistency with an irregular form and surface , brown color and measured 2.5 1.5 1.0 cm . the histopathologic study revealed multiple pseudocystic spaces of variable sizes surrounded by cuboidal cells with scarce cytoplasm and oval nuclei , filled with eosinophilic material and hence was consistent with the diagnosis of acc [ figure 3 ] . however , there was no evidence of perineural infiltration on serial sections . clinical photograph showing a swelling on the dorsum of the tongue cytological smears show well - delineated clusters of basaloid cells surrounding hyaline globules with uniform round to oval hyperchromatic nuclei and scanty cytoplasm ( papanicolaou stain , 400 ) histopathological section showing multiple pseudocystic cavities of variable size composed of cuboidal cells with scarce cytoplasm and oval nuclei ( h and e , 400 ) minor salivary gland neoplasms occur less commonly than the major salivary gland tumors and tongue is a relatively uncommon site for salivary gland neoplasms acc is a malignant neoplasm that originates in both the minor and major salivary glands , characterized by slow growth , diffuse invasion , and potential to produce distant metastases , mainly to the lungs and bones . it is an infrequent lesion , as it represents approximately 1 - 2% of all malignant neoplasms of the head and neck , and up to 10 - 15% of all malignant salivary gland neoplasms . the most common intra - oral site for minor salivary gland tumors is the hard palate , followed by the base of the tongue where up to 96% of all tumors are malignant , and acc represents 30% of them . on the other hand , one of the least frequent sites of presentation for acc is the mobile tongue , as several authors have reported an incidence of only approximately 3% of the cases . , analyzed 178 cases of salivary gland tumors , out of which only six cases were located on the tongue . cytologically , cribriform variety of acc can be diagnosed by hypercellular smears composed of clusters of small , relatively monomorphic epithelial cells with hyperchromatic nuclei . these appear bright magenta in may - grunwald giemsa mgg stains and pale blue with papanicolaou stain . finger - like process of similar material can also be found in between the groups of cells in tubular variety . the solid variant of acc also exhibits the same material and the cells resemble that of small cells of anaplastic carcinoma . the globules of amorphous material surrounded by the monomorphic hyperchromatic cells was a clue to the diagnosis of acc in our case , but since the hyaline globules are also found in other tumors like basal cell adenoma , pleomorphic adenoma , polymorphous low grade adenocarcinoma , epithelial myoepithelial carcinoma etc . it is important to distinguish the adenomas from acc because of the conservative mode of management in case of adenomas . we ruled out the adenomas because of the nature of the globules and the cytological morphology . unlike the adenomas , the hyaline globules were dense and stained intensely with mgg and the cells were relatively monomorphic , hyperchromatic with coarse chromatin and irregular nuclear membrane like that of acc . of three histologic variants - tubular , cribriform and solid ; in our case cribriform pattern was the dominant one without any evidence of perineural infiltration . the main factors associated with patient survival were tumor location , clinical stage , and the observed histologic variable . conversely , spiro et al . , have not found histologic classification to be of any benefit , and deny a correlation between microscopic appearance and prognosis . due to the slow growth pattern of the tumor however , due to local recurrence and late metastasis , surgery remains the mainstay of management with or without radiotherapy .

adenoid cystic carcinoma is a relatively rare epithelial tumor of the salivary glands accounting for about 5 - 10% of all salivary gland neoplasms . approximately , 31% of salivary gland neoplasms affect minor salivary glands particularly the palate . it involves tongue in only 19.8% of cases and even rarely the dorsum of the tongue . we report such a rare case that affected dorsum of the tongue in a 45-year - old - female patient .

it has been proven to be very effective and became a standard of care in the treatment of most cases with severe prolapsus . sacropexy is a surgical repair technique that restores pelvic anatomy by attaching synthetic graft material into the vagina and sacrum . erosion of vaginal wall or bowel can be seen as a long term complication . in this paper , we present a patient suffering from mesh migration into the rectum after abdominal sacral colpopexy . a 69-year - old woman was admitted to the hospital with a complaint of sensation of fullness and a feeling of a foreign material protruding during defecation . she had been diagnosed with uterine prolapsus and stress incontinence in 2008 and underwent total abdominal hysterectomy , bilateral salpingo - oophorectomy and sacral colpopexy with prolene mesh . the patient was admitted to our clinic five years after the procedure with complaints mentioned above . there was a foreign material palpated in rectum with digital examination . prolene mesh was detected in sacral region but resection of the mesh could not be conducted because of high levels of adhesions in that region . genital prolapse or genital hernia is described as the protrusion of pelvic organs along the vagina . it is one of the common gynecological conditions that affect the quality of life in women . it may be seen in up to 50% of multipara women , and its incidence increases with age . high rates of recurrence with traditional techniques led to the development of new surgical techniques . the use of synthetic mesh has become more popular surgical approach in cystocele and rectocele repair . mesh migration is a well - known clinical pathology and have been reported in literature . yolen and grossman suggested that intra - abdominal foreign bodies ( like mesh ) transmigrate into the small or large bowel by triggering an inflammatory reaction . persistent inflammatory reaction causes an opening into a hollow organ assisted by the peristaltic movement of the bowel . insufficient fixation of a mesh is another factor for migration of synthetic materials in some patients . larger pores greater than 75 nm permit the migration of macrophage and leukocyte migration and reduce the infection rate . large pores also improves flexibility of the mesh and cause tissue ingrowths and healthy collagen deposition . complications reported after sacropexy include ileus , intraoperative vessel injury , ureter injuries , recurrent descensus and mesh tearing . the use of a mesh as a graft material results in higher success rates but also causes a higher number of complications , such as mesh erosions or chronic infections . taoka reported a case of rectal migration of mesh in a 64-year - old woman who presented with a recto - cutaneous fistula 11 months after a tension - free vaginal ( tvm ) repair ; the patient was treated by removal of the infected mesh and closure of the rectal wall defect under cover of a temporary colostomy . by contrast with the troublesome symptoms reported in such patients in the literature , the only presenting complaint of our patient was protrusion of foreign material from the rectum . in conclusion , mesh migration is a serious complication after sacral colpopexy . sometimes surgical resection of migrated mesh with laparotomy can be difficult due to dense adhesions . scu was responsible for writing , conception and design of the study ; ob contributed toward analysis and interpretation of data ; nas , oa , aa- performed acquisition of data ; bk drafted the manuscript .

introductionpelvic organ prolapse ( pop ) is a common gynecological problem . repair with synthetic materials such as prolene mesh has become a popular approach in prolapsus surgery . migration of synthetic materials can cause serious complications.presentation of casea 69-year - old woman was admitted to the hospital with a complaint of sensation of fullness and a feeling of a foreign material protruding during defecation . the patient underwent exploratory laparotomy . prolene mesh was detected in sacral region but resection of the mesh could not be conducted because of dense adhesions causing frozen pelvis . the migrated prolene mesh was resected transanally.discussiongenital prolapse or genital hernia is described as the protrusion of pelvic organs along the vagina . it is one of the common gynecological conditions that affect the quality of life in women . mesh migration is a well - known clinical pathology.conclusionmesh migration is a serious complication after sacral colpopexy . surgical resection of migrated mesh can be difficult due to dense adhesions .

hemangiomas are categorized as racemose , capillary , cavernous , and venous , according to the size of their vascular spaces . cavernous and venous hemangiomas have low blood flow , because they lack arterial or capillary components . histopathologically , venous hemangiomas contain dilated vessels with thick , fibrous walls , whereas cavernous hemangiomas have capillary - sized vessels lined by flat endothelial cells . we report the first case of a venous hemangioma of the pps and discuss its typical radiographic findings . a 49-year - old female patient was referred for evaluation of swelling in the right submandibular region . she had swelling for the first time 3 weeks earlier , and it had not improved after medical treatment . clinical examination revealed a soft mass in the right submandibular region and a bulge at the right lateral pharyngeal wall . a contrast - enhanced computed tomography ( ct ) scan revealed a cystic lesion , 4.63.0 cm , with contrast non - enhancement in the parapharyngeal space ( pps ) . mri showed a well - circumscribed non - enhancing mass with high signal intensity on t2-weighted images ( fig . the cystic lesion of the pps was surgically removed using a transcervical approach . during surgery the lesion bled profusely , but the mass was bluntly dissected from the surrounding structures . the surgical specimen , measuring 4.34.52 cm , showed a pale - to - dark brown soft cut surface with a blood - filled spongy vascular lesion . microscopic examination revealed blood - filled sinusoidal spaces with large irregular lumens and thick walls lined by endothelial cells ( fig . postoperative recovery was uneventful , and there has been no evidence of cranial nerve palsy or tumor recurrence after one year . pps has a complex anatomy and close proximity to vital anatomical structures , with which it may become involved by various pathological processes . presenting symptoms of pps tumors may be attributed to the size of the mass and compression of neighboring structures ( 1 ) . we had difficulty with the preoperative diagnosis , due to the absence of typical symptoms or signs of a vascular lesion in the pps , such as pulsation or bruit . fine - needle aspiration cytology ( fnac ) was also not helpful in reaching a diagnosis . although an exact tissue characterization of hemangioma in the pps could not be made on fnac , contrast - enhanced ct and magnetic resonance imaging ( mri ) should be included in differentiating these lesions from other tumors of the pps . in general , mr imaging is superior to ct imaging in its ability to ascertain the soft tissue characteristics of pps tumors ( 2 ) . changes in the blood flow dynamics within a hemangioma result in thrombus formation and phleboliths ( 3 ) . phleboliths are calcified nodules that can be regarded as a characteristic property of venous or cavernous hemangiomas . ct with contrast is an excellent imaging technique for revealing phleboliths . although mri is not sensitive for the detection of small amounts of calcification , large amounts of calcification show discrete low signal intensity on all pulse sequences mri can produce high signal intensities , representing the blood , as well as focal heterogeneities , representing areas of thrombosis , fibrosis , or calcification ( 4 ) . although mri is very useful for the detection of vascular lesions , the detectability of phleboliths on ct images is superior to that by mri . some reports have shown that plain x - ray films may also reveal calcified lesions . when imaging shows calcification in the pps , the differential diagnosis may include pleomorphic adenoma of the deep lobe of the parotid gland and metastatic thyroid carcinoma of the pps . pleomorphic adenoma with foci of chondroid or osteoid stroma can demonstrate opacities on imaging , but usually shows minute , scattered flecks . additionally , in cases of metastatic thyroid carcinoma of the pps , large flocculent calcification is seen on ct images ( 5 ) . venous hemangioma can not be clinically or radiologically differentiated from cavernous hemangioma , because of their similar characteristics . however , perfusion and blood pool scintigraphy has been demonstrated to have high sensitivity for detecting head and neck hemangioma , and can also differentiate between cavernous and venous hemangiomas ( 6 ) . they tend to be larger and less well circumscribed , and show no tendency to regress ( 7 ) . attention needs to be given to the tumor location , extent , growth rate , and accessibility as well as the patient 's age and esthetic concerns . the excision of large tumors in the pps can be challenging , given the risk for severe hemorrhage and nerve injury . a surgical approach should be chosen according to the tumor size and location , its relationship to the great vessels , and any suspicion of malignancy . generally , hemangiomas in the pps , including venous hemangioma , can be resected by using a transcervical approach . profuse bleeding often occurs during surgery , but after all of the pathology has been removed , the bleeding will cease . when surgery is impossible , the use of corticosteroids , cryotherapy , feeding vessel ligation , embolization , and fibrosing agents can be considered as alternatives ( 8) . in summary , we present the first reported case of venous hemangioma occurring in the parapharyngeal space . considering that a presumptive diagnosis of most pps tumors can be made based on imaging studies , preoperative imaging findings are very important in approaching these lesions . it may be that multiple , spotty , calcific nodules , 13 mm in size , within a cystic lesion on ct images indicate the pathognomonic finding of hemangioma in the pps .

a hemangioma of the parapharyngeal space ( pps ) is an extremely rare tumor and is responsible for 0.5 - 1% of all tumors occurring in the pps . we report a case of pps venous hemangioma in a 49-year - old woman presenting with diffuse swelling in the submandibular region . a preoperative computed tomography ( ct ) scan showed a cystic mass with multiple calcifications in the pps . the calcific nodules were round and about 2 mm in diameter . the hemangioma was completely resected via a transcervical approach . during surgery , we found several calcific nodules , which represented phleoboliths or areas of thrombosis with dystrophic calcification . despite its rarity , a venous hemangioma of the pps should be considered in a differential diagnosis when a cystic mass with calcification is found by ct scan . to our knowledge , this is the first reported case of a pps venous hemangioma ; we describe its pathognomonic findings on imaging .

verruciform xanthoma ( vx ) , which has almost similar histologic features , is also a rare lesion usually found on the oral mucosa or the genital area . it is presumably associated with the inflammatory response to mucosal damage.1 however , xanthoma and vx of the esophagus are extremely rare . since the first report by remmele and engelsing2 only 13 cases of esophageal xanthoma have been reported,1,2,3,4,5,6,7,8,9 and since the report by herrera - goepfert et al.,10 only four cases of vx of the esophagus have been reported.10,11,12,13 the etiologies of both lesions are not understood . we describe herein a new case , including a review of all reported cases of xanthoma and vx of the esophagus . a 70-year - old man with an unremarkable medical history was hospitalized with a complaint of epigastric pain . serum total cholesterol , triglyceride , high density lipoprotein cholesterol , and low density lipoprotein cholesterol levels were 151 , 215 , 33 , and 102 mg / dl , respectively . multiple shallow gastric ulcers and a duodenal ulcer were detected and suspected to be the cause of the pain . besides the ulcers , in the upper esophagus 20 cm from the incisors , a 3-mm yellowish granular elevated mucosal lesion was found and a biopsy was performed ( fig . 1 ) . microscopically , large round cells were aggregated in the lamina propria immediately beneath the squamous epithelium . the etiologies are different , as xanthoma is caused by hyperlipidemia and vx arises presumably as a result of an inflammatory response to continuous mucosal damage.1 however , the etiologies of the two lesions arising in the esophagus are not understood . the characteristics of all reported cases of xanthoma and vx of the esophagus are summarized in table 1 . fourteen cases of xanthoma and four cases of vx of the esophagus have been reported . however , some reports loosely stratified vx into esophageal xanthoma , whereas others have excluded it.6,8 in terms of clinical data , both diseases were found predominantly in men than in women : 9 versus 3 in xanthoma and 3 versus 1 in vx . the median age was 59 years ( range , 37 to 74 ) in xanthoma and . the predominant location was the lower esophagus for xanthoma ( lower , 7 ; middle , 2 ; upper , 3 ) , whereas vx was not reported in the lower esophagus ( upper , 2 ; middle , 2 ) . the median size was not different : 3 mm ( range , 2 to 10 ) for xanthoma and 4 mm ( range , 3 to 20 ) for vx . the associated medical conditions were diverse ; however , two patients with malignant tumors were included in each group : hepatocellular carcinoma and ileocecal lymphoma in xanthoma , and gastric cancer and multifocal cancer ( cancer of the glottis , liver , and trachea ) in vx , although there was no definite association . vx is characterized by its histologic features , including papillomatosis , acanthosis , and hyperparakeratosis.11 also , the external morphology is verrucoid . nevertheless , findings of large round foam cells in the lamina propria under the squamous epithelium are the same as those in xanthoma . it is difficult to differentiate between the two lesions on the basis of gross examination when they arise on the esophagus . exophytic and verrucoid features seen in vx of the skin were not observed in the esophagus because most of the reported cases were small in size.10,12 considering that xanthoma and vx are nonneoplastic lesions , differentiating between them could be a waste of effort . however , these lesions have to be grossly distinguished from ectopic sebaceous glands and small subepithelial tumors such as carcinoid and granular cell tumor because most of the reported esophageal xanthomas are yellowish or white mucosal elevated lesions . in terms of microscopic findings , signet ring cell carcinoma , which contains round cells with abundant cytoplasm , while signet ring cell carcinoma has an eccentrically located nucleus because of the intracellularly abundant mucin , xanthoma has a centrally located and small nucleus . positive immunohistochemical staining for cd68 , which indicates a histiocytic origin , is another characteristic finding of xanthoma.5 moreover , esophageal cancer and ectopic sebaceous glands do not commonly stain with lugol 's solution ; thus , endoscopists need to be aware of these lesions for the differential diagnosis.6,14 with more case reports of esophageal xanthoma and vx of the esophagus , the characteristics of both lesions will be more clearly elucidated .

xanthoma is an uncommon nonneoplastic lesion resulting from the accumulation of histiocytes . it predominantly shows cutaneous manifestations associated with dyslipidemia . however , xanthoma of the esophagus is extremely rare . to the best of our knowledge , only 14 cases have been reported thus far . the clinical significance of this lesion has not been established . however , this lesion should be distinguished grossly from ectopic sebaceous glands and small subepithelial tumors such as carcinoid and granular cell tumor . moreover , signet ring cell carcinoma , which contains round cells with abundant cytoplasm and has similar histologic features to xanthoma , should be distinguished microscopically .

the sizing of pda for device implantation is based on the automatic calculations of dimension by the inbuilt sizing software in the cardiac catheterisation laboratory and many experienced interventionists believe their eyeball measurements more . the estimations can be erroneous if the calibrations are not proper , leading to catastrophic embolisation of the device , needing emergency surgical removal as was needed in our patient . calibration by comparing with the fluoroscopic images of measured metallic sizing devices placed outside body can be misleading because of magnification errors.1 in order to circumvent these problems we suggest some novel ways which take care of magnification errors and avoid the need for aortic root angiography . an eight year child with echocardiographically documented 6 mm patent ductus arteriosus ( pda ) was planned for device closure . femoral venous access was obtained using 8f sidearm sheath and the artery cannulated with a 6f sidearm sheath . a 6f pigtail catheter was advanced through the femoral arterial sheath into the descending aorta at the mouth of the pda and an angiogram obtained in a lateral projection delineating the ductus ( figure 1 ) . the minimum diameter at the aortic end was 6 mm and a 8 - 10 mm pda device was selected for deployment . a 7f swan - ganz catheter was advanced into the the pulmonary artery through venous route and a 0.35 " terumo wire advanced through the pda into the descending aorta .the terumo wire was exchanged with an 0.35 " extra stiff amplatzer wire and the swan - ganz catheter with the pda delivery system . an 8 - 10 mm size pda device was released across the ductus and after confirming the proper position by repeat angiogram which showed a small residual shunt flow which may be expected . angiogram in lateral plane showing a small residual left to right shunt with pda device in place before unscrewing of the stylet after a short while , the device suddenly embolised into the left pulmonary artery ( figure 2 ) . the patient had a brief episode of mild hypotension which improved spontaneously and his oxygen saturations remained static . an attempt was made to retrieve the device percutaneously by a snare and later by a bioptome but the trial was given up as the device tended to go more distally , in which case even surgical removal would be difficult . the patient was discussed with the cardiac surgeon who shifted the patient for emergency surgery . after considerable debate surgeon decided for a left thoracotomy hoping to avoid cardiopulmonary bypass and closed the ductus . later the left pulmonary artery was separated from the surrounding tissues and umbilical tape passed around it proximally for control of bleeding . a 1.5 cm longitudinal incision was made distal to the umbilical tape which was kept under traction and a long curved forceps passed toward the pulmonary hilum and the device retrieved . cine image in lateral plane showing device embolized into left pulmonary artery soon after its release from stylet . showing the pda device being retrieved from left pulmonary artery via left thoracotomy without cardiopulmonary bypass . in order to prevent such eventualities we tried to devise some alternative ways of sizing a pda .the first involves measuring a fully inflated tyshak pulmonary balloon outside the human body with a caliper and then inflating the balloon to the same pressure in the right ventricular outflow tract passed over a 0.35 " extra - stiff wire , and repeating the measurements , and using the same calibration factor for sizing of pda visualized by aortic arch angiogram . previously , some radio opaque sizing devices have been used placing them outside human body which can have magnification errors if not placed exactly at the level of heart.2 the second method involves advancing a tyshak balloon 2 - 4 mm bigger than the echocardiographic dimension across the pda and inflating the balloon with dilute dye under low pressure without dilating the ductus so that it forms an impression of the ductus which is recorded in a cine mode in multiple planes , and the plane delineating the exact anatomy of the ductus is used for measurements after proper calibrations as mentioned above without the need for aortic arch angiogram ( figures 4 and 5 ) . showing measurement of diameter of inflated tyshak balloon by a caliper after inflating it with fixed quantity of contrast . angiogram in lateral plane showing an inflated tyshak balloon across the pda making an impression of the ductus . technological advances have made nonsurgical closure of the pda a simple and a routine percutaneous intervention . the use of the amplatzer device occluder ( ado ) has further simplified the method and improved the results with minimal complications . however there are situations where complications are encountered and surgical help is required to ameliorate them . faella and colleagues reported 15 procedure related complications in 316 patients which included hemolysis , left pulmonary artery artery stenosis , device protrusion into aorta causing coarctation , device misplacement and one death following device embolisation.2 late embolisation of the device to the left pulmonary artery has been reported with impaired left pulmonary perfusion six months after implantation requiring surgical removal . m vavuranakis et al reported severe hemolysis with jaundice , anemia and hemogloginura on the second day following deployment of smaller sized coil due to improper sizing which needed removal and replacement by an ado after repeat sizing using balloon tipped catheter.3 the complications can be reduced by proper expertise and optimal sizing of the ductus . sizing of atrial septal defect ( asd ) by inflating a balloon across the asd till a circumferential waist is created and measuring the waist ( stretched balloon diameter ) and also inflating the balloon outside by same amount of dye across sized rings , is a standard method.45 using a similar method for the pda is not routine since angiographic visualization is usually adequate . however making an impression of the ductus by inflating a balloon across it can give a detailed anatomy about the length and breadth of the ductus in multiple planes without the need for multiple dye injection and may help in the selection of proper sized device .

nonsurgical closure of patent ductus arteriosus ( pda ) using a duct occluder placed percutaneously is currently the first line of therapy and the success rate is quite high . several devices are currently available . an eight year child underwent device closure of the ductus . however after deployment of the device it , became dislodged into the left pulmonary artery . several attempts at catheter retrieval failed . the child underwent successful surgical removal of the device without cardiopulmonary bypass .

soft tissue coverage for wounds remains a difficult management problem for patients sustaining traumatic injury and burns . there are several methods to achieve wound coverage secondary healing , primary suturing , skin grafting , and flap surgeries as described in reconstruction ladder . a skin graft is the most commonly used modality for coverage of wounds in reconstructive plastic surgery . the skin graft needs to undergo various stages of healing for a good take on the recipient bed . there are different methods employed to secure the graft to recipient bed for a few days with a basic idea to ensure that the graft is not elevated off the bed by formation of haematoma / seroma under it . repeated tie over dressings are required in situations where the dressing needs to be changed more frequently as in cases of infected raw area , bleeding tendency , patients on anticoagulant drugs , and in convex areas of body such as buttocks , breast , and the scalp , where the dressing is difficult to secure . in this novel method , a sterile sample container was cut at its upper part [ figures 1 and 2 ] . the skin graft was applied on the raw area and fixed with skin staplers and tie over sutures . once paraffin gauze and adequate padding is applied on the raw area , the tie over threads were passed from inside out of the container [ figures 3 and 4 ] and pulled at the appropriate tension to keep the dressing in place . the lid of the container was tightened to complete the dressing ensuring that the graft was maintained in close approximation with the wound surface . sterile plastic container upper part of the sterile container is cut tie over threads being passed from inside out the lid is tightened over the dressing the dressing can be changed repeatedly with sterile precautions depending on the requirement , as an outpatient procedure by unscrewing the lid [ figures 5 - 8 ] which can then be easily reapplied . the procedure can be used on wounds of any size by changing the size of the sterile container chosen . post - toilet mastectomy raw area covered with the skin graft the tie over dressing applied in ot tie over dressing repeated in an outpatient department we have used this method in eight cases of the post toilet mastectomy raw areas with very good results [ figures 9 and 10 ] . post - toilet mastectomy raw area skin graft once applied has to be covered with petrolatum gauze to avoid its separation from the wound bed at the time of change of dressing . an ideal method of graft fixation should be simple , rapid , repeatable , able to be performed in the outpatient department , prevent hematoma or seroma formation , soak the exudates well , and allow the graft bed to be inspected easily . there are multiple methods of securing dressings over the skin graft , some of them can be applied only once and some can be repeated . the dressings that can be applied only once are like foam , hydro cellular dressing ( highly absorbent and can be easily changed ) , negative pressure therapy dressing ( stabilises the graft , increases the vascularity of bed , takes away toxic chemicals ) , and gas bag ( transparent , can see graft and monitor any haematoma ) . these traditional methods can stabilize the graft till the first dressing post operatively . in some contaminated wounds , the dressing needs to be removed earlier , especially if there is drainage or foul smell . this approach may also be proper for graft , used to cover defects of some anatomical regions with increases risk of contamination , such as perineal , axillary , and genital or it can be used in areas where base of wound is difficult to immobilize like breast / pectoral region . repeated tie over dressings can be done by keeping interrupted sutures long to be used as tie over dressings . these ties over dressing can be made of sutures or rubber bands . when taking a tie over stitch , both the threads can be left long and only one thread is tied at a time , the other thread is left long for next time . these techniques are difficult for small dressings especially the bra hooks ; the silk loops method is very cumbersome and takes long time to do . the novel method being discussed has a very small learning curve and is very fast . it hardly takes 5 min in the hands of a plastic surgeon to complete the dressing . this dressing technique maintains the advantage of conventional tie over dressing with rapidity and repeativity . good graft take can be expected whether split - thickness or full - thickness with appropriate methods of stabilisation . we recommend a novel , low cost , simple , rapid method of graft fixation that can be used repeatedly and can be applied to a wound of any size .

tie overdressing is commonly used to secure the graft against the raw surface and prevent loss due to of hematoma or seroma . a conventional tie over dressing with silk sutures , is a useful method of securing the graft to raw area . refixation is difficult when repeated tie over dressings are needed . we assessed a low cost repeated tie over dressing method using sterile sample collection containers and silk suture threads in eight patients . after the graft is applied on the bed , tie - over stitches are taken , and paraffin gauze is applied over with adequate padding ; the tie over sutures are passed through the container and the lid is tightened over it to complete the dressing . the lid can be unscrewed easily at any time to inspect the graft and can be easily reapplied in the outpatient department . the skin graft take in all the patients was complete without any seroma or hematoma . a novel and low - cost tie over dressing that enables simple fixation of the dressing , to maintain proper position of grafts that require repeated fixation is reported here .

we tested a structured relaxation program on nine patients with a diagnosis of schizophrenia suffering from akathisia . all patients were rated on barnes akathisia scale ( bas ) before the relaxation program , immediately after and again one week later . the mean bas score was before the relaxation 3.3 which reduced to 1.4 immediately after to finally 1.0 a week later . a wilcoxon signed ranks test revealed a significant reduction in bas score from baseline to endpoint ( p = 0.026 ; z = 2.232 ) and a highly significant reduction from baseline to follow - up ( p = 0.008 ; z = 2.636 ) . although the study has a number of limitations the relaxation program appears to be a promising alternative to traditional treatment of akathisia . the patients appreciated the relaxation session but none of them managed to carry it out on their own without professional encouragement . akathisia ( inability to sit still ) is a movement disorder characterized by objective movements and restlessness and/or distress , which is common among patients under - going treatment with psychotropic drugs . it is generally agreed that the syndrome of akathisia comprises both an objective and a subjective component.1 although one of the main motivating factors for the development of second generation antipsychotics was to reduce the incidence and severity of treatment - emergent akathisia and other extrapyramidal side effects , these remain problematic and not uncommon:2 furthermore , akathisia can also be associated with antidepressant treatment.3 although often extremely upsetting , the widely held view of an association between akathisia and suicidal behavior has not been proven in larger methodologically sound studies,4 and is mainly based on case reports . it is nevertheless a distressing problem that can reduce quality of life and may impede treatment compliance in some patients undergoing pharmacological treatment . in an attempt to make sense of the condition some patients develop a dysfunctional interpretation of the stressful symptoms.5 there is no general agreement on how to diagnose akathisia , and this has hampered both research and clinical practice.6 if akathisia is recognized , current treatment options include reducing the daily dosage or withdrawal of the implicated medication , or the addition of other medications . the most widely used pharmacological interventions include anticholinergic drugs , beta - blockers , and benzodiazepines , but the evidence base to support these interventions is limited and treatment may entail risks such as development of hypotension , tolerance , and dependence.7 in fact the only small , randomized controlled trial on biperiden showed no difference compared to saline water in the treatment of akathisia.8 some of the most important differential diagnoses of akathisia are the presence of agitation and anxiety symptoms , and these can be helped significantly with structured relaxation programs.9 however to our knowledge a relaxation approach has not been examined in patients experiencing akathisia associated with antipsychotic drugs , therefore the aim of this short report is to investigate whether a relaxation approach would reduce akathisia in patients suffering from this syndrome . nine patients with a primary diagnosis of schizophrenia and currently experiencing distressing akathisia ( measured on the barnes akathisia scale [ bas ] ) were invited to participate in a structured relaxation program , lasting 12 minutes and consisting of breathing ( four exercises ) and tension - relaxation ( six exercises ) . the patients were selected specifically because they suffered from akathisia all participants were receiving , or had recently received , pharmacological interventions for akathisia such as benzodiazepines , beta blockers , or procyclidine ( see table 1 ) leading to an unsatisfactorily response . however , the responsible clinicians had been reluctant to lower the dose of antipsychotic medication due to fears of deterioration of psychotic symptoms . one patient ( number 9 ) underwent a repeat of the relaxation program with tl during the week before the final follow - up assessment . all patients were rated ( by lkh ) using the bas,10 before the intervention ( baseline , bas median , 3 ) , after the relaxation session ( endpoint , bas median 2 ) , and again one week later ( follow - up bas median , 2 ) . the short follow - up period was decided on to minimize the risk of changes to the medication regime that could have made the patients unsuitable for the study . all patients were given a written version of the program following the sessions , but according to self - reports , none managed to undertake the program on their own in the week before follow - up . there were no changes to medication regimes in any patient , during the study period . a wilcoxon signed rank test showed a significant reduction in bas score from baseline to endpoint ( p = 0.026 , z = 2.232 ) and a highly significant reduction from baseline to follow - up ( p = 0.008 ; z = 2.636 ) . no significant difference was however found between endpoint and follow - up ( p = 0.257 ; z = 1.134 ) . these findings are promising , although it is possible that the benefits were at least partly due to factors such as increased attention and greater contact with other patients and staff . other limitations in this report include the small sample size , preliminary pilot nature of the data , use of a potentially biased ( lkh ) rater , and the uneven completion of the relaxation program in some patients . although the number of patients was limited , all but one patient appeared to have benefited from the relaxation program . the approach was received enthusiastically by the participating patients , but no patient managed to perform the program on their own without professional intervention . the structured relaxation program appeared to be well accepted and to be associated with a notable reduction in the severity of symptoms of akathisia in this group of patients with chronic schizophrenia , treated with antipsychotic drugs . the pathophysiology underlying akathisia is not fully understood , and current treatment approaches are less than ideal . it is important to develop evidence - based alternatives to current interventions that are feasible in routine clinical practice . the mechanism underlying the reduction in symptoms of akathisia with the structured relaxation program is uncertain but biofeedback methods may be at least partly responsible for the beneficial effect . biofeedback is a process that enables a person to change physiological activity ( eg , heart rate , muscle activity ) . it is conceivable that the relaxation program improved the patients ability to minimize the restlessness characteristic of akathisia . the sustained effect at 1-week follow - up is intriguing and warrants further study , possibly comparing structured relaxation with current pharmacological treatment options , such as use of beta - blockers or benzodiazepines in an open label clinical trial .

purposeakathisia remains a common side effect especially from antipsychotic medication . if the condition is diagnosed the management options are limited.subjects/methodologywe tested a structured relaxation program on nine patients with a diagnosis of schizophrenia suffering from akathisia . all patients were rated on barnes akathisia scale ( bas ) before the relaxation program , immediately after and again one week later.resultsthe mean bas score was before the relaxation 3.3 which reduced to 1.4 immediately after to finally 1.0 a week later . a wilcoxon signed ranks test revealed a significant reduction in bas score from baseline to endpoint ( p = 0.026 ; z = 2.232 ) and a highly significant reduction from baseline to follow - up ( p = 0.008 ; z = 2.636).discussionalthough the study has a number of limitations the relaxation program appears to be a promising alternative to traditional treatment of akathisia . the patients appreciated the relaxation session but none of them managed to carry it out on their own without professional encouragement . the findings in this case series warrant further investigation with larger numbers of patients .

since 2006 , surveillance physicians have listed and collected blood from those patients encephalitis , defined as fever or history of fever with axillary temperature > 38.5c ( 101.3f ) and altered mental status , new onset of seizures , or new neurologic deficit in patients admitted to 3 nipah surveillance hospitals : rajshahi , rangpur , and faridpur medical college hospitals . the institute for epidemiology disease control and research and us centers for disease control and prevention tested serum with an igm - capture enzyme immunoassay to detect niv igm , and we defined laboratory - confirmed niv encephalitis as niv igm in serum . during december 2012march 2013 , surveillance physicians interviewed accompanying caregivers of all hospitalized patients whose illness met the encephalitis case definition on admission in the inpatient ward . study physicians asked about patients consumption of raw or fermented date palm sap and contact with other persons with fever and altered mental status in the month before illness onset ; if caregivers were unaware of the patient s exposures , study physicians asked them phone the patient s friends and colleagues about exposures . hospital physicians used personal protection equipment and provided it to caregivers of each patient with encephalitis and a history of these exposures . as part of subsequent epidemiologic studies , we also conducted detailed case investigations at each niv encephalitis case - patient s household . we interviewed surviving patients directly , or appropriate proxies among family , friends and relatives for patients who died , about their exposures to encephalitis patients or to fresh or fermented date palm sap before illness . we calculated the sensitivity , specificity , positive predictive value ( ppv ) , and negative predictive value ( npv ) of the screening questions asked on admissions to hospitals by comparing with the niv igm results . we repeated the calculations for patients hospitalized during january and february , when the prevalence of niv encephalitis is highest . we compared the answers provided by caregivers during patient hospitalization with those provided during interviews in the community as part of our epidemiologic studies . icddr , b s ethical review committee reviewed and approved the protocol for niv surveillance and case investigation . they collected and tested blood samples from 328 ( 91% ) patients for niv igm . seventeen ( 5% ) had niv igm ( table 1 ) , of whom 15 ( 88% ) niv encephalitis case - patients were identified during january and february 2013 . of the 17 confirmed case - patients , family caregivers of 14 reported either a history of drinking raw or fermented date palm sap or contact with other persons with fever and altered mental status in the month before illness onset . therefore , the sensitivity of the screening questions was 82% , specificity was 86% , ppv was 24% and npv was 99% ( table 2 ) . the sensitivity during january february was 93% , specificity was 82% , ppv was 37% , and npv was 99% . * drinking raw or fermented date palm sap or having contact with encephalitis patients in month before illness onset . at admission , 3 ( 18% ) niv encephalitis case - patients had no reported history of drinking raw or fermented date palm sap or of contact with persons who had encephalitis ( table 1 ) . however , during the epidemiologic investigations in the community , family members of 2 case - patients reported that the patients drank fermented date palm sap in the month before illness onset . of the 14 niv encephalitis case - patients who , at admission , had reported 1 of the risk exposures , results were consistent with exposures reported during the epidemiologic investigation . screening patients with possible encephalitis at the time they seek hospital care regarding recent exposure to date palm sap and to other patients with encephalitis demonstrated high sensitivity and specificity for detecting niv encephalitis , particularly during peak months of niv encephalitis incidence . the high npv of the screening questions suggests that focusing infection control efforts toward patients with these exposures is an efficient use of scarce resources to prevent transmission . although three fourths of encephalitis patients had reported histories of exposure , they possibly could have had other infections , including other bat - borne viruses , that were transmitted through similar routes or could have lacked niv igm , despite having niv infection ( 13 ) . alternatively , recent consumption of date palm sap by these patients might have been purely coincidental because this practice is common in bangladesh during this season , but nipah infection is rare . for 2 niv encephalitis case - patients , caregivers did not report a history of drinking fermented date palm sap during hospital interview , but this behavior was reported in later community investigations . because 90% of bangladeshis are muslim , and consumption of alcohol is prohibited by islam ( 14 ) and illegal in bangladesh , patients might be reluctant to report drinking traditional liquor made of fermented date palm sap . therefore , caregivers should be asked about socially stigmatized behaviors privately and confidentially to increase the odds that these stigmatized behaviors are reported . exposure - based screening can detect patients at high risk for niv encephalitis in low - income , resource - constrained settings , such as bangladesh . we deployed screening questions on admission to inpatient wards but screening earlier , at triage in emergency wards , could further reduce risk . surveillance for other diseases with well - described exposures that put healthcare workers at risk , such as ebola virus infection , and where laboratory diagnosis is limited or delayed could also deploy this approach .

we measured the performance of exposure screening questions to identify nipah virus encephalitis in hospitalized encephalitis patients during the 201213 nipah virus season in bangladesh . the sensitivity ( 93% ) , specificity ( 82% ) , positive predictive value ( 37% ) , and negative predictive value ( 99% ) results suggested that screening questions could more quickly identify persons with nipah virus encephalitis .

parasitic infections are increasing worldwide due to rapid urbanization of cities , global warming , and international traveling . the diagnosis of parasitic diseases of the respiratory system is challenging because clinical manifestation and radiological findings are not specific . the larvae can cause airway inflammation , whereas migration of the mature adult worm may cause mechanical obstruction . we present a case of interstitial pneumonitis caused by parasitic infestation , which was diagnosed on transbronchial lung biopsy ( tblb ) . a 54-year - old female presented with a history of exertional breathlessness and dry cough for two months . she was afebrile with spo2 of 91% on room air and bilateral crepitations on respiratory examination . l ( 46.4% ) , fvc of 1.45 l ( 45.2% ) , and fev1/fvc 83% with reduction in dlco 1.46 mmol / min / kpa ( 18% ) and corrected value with alveolar volume 0.49 mmol / min / kpa ( 30% ) . high - resolution computed tomography ( hrct ) of chest showed multiple ill - defined nodular opacities in both lung fields showing centrilobular distribution associated with ground glass opacities bilaterally . fine reticular densities were seen at places with segments of traction bronchiectasis [ figure 1 ] . total serum ige was 145 iu / ml and specific ige for pigeon droppings ( < 0.10 tblb showed thickened alveolar septa with mild lymphoplasmacytic inflammatory infiltrate in the interstitium with cross - sections of calcified parasitic larvae with foreign body giant cell reaction around it [ figure 2 ] . travel history revealed frequent travels in the himalayan belts yearly for 20 years during which she had frequent episodes of diarrhea . she was given albendazole 400 mg for three days and oral corticosteroids for 12 weeks . bronchoalveolar lavage ( bal ) for tuberculosis culture at 6 weeks was negative . at 6 weeks high - resolution computed tomography chest showing multiple ill - defined centrilobular opacities with ground glass opacities bilaterally before treatment histopathology image of transbronchial lung biopsy showing calcified larva with thickened alveolar septa with mild lymphoplasmacytic inflammatory infiltrate in the interstitium high - resolution computed tomography chest showing remarkable improvement after treatment diffuse lung diseases can be further divided into transient pulmonary infiltrates and alveolar or interstitial lung diseases . ascariasis , anchylostomiasis , and toxocariasis usually cause transient pulmonary infiltrates , whereas schistosomiasis , strongyloidiasis , and tropical pulmonary eosinophilia can cause diffuse interstitial changes , as in the current case . strongyloidiasis causes reticulonodular opacities because of secondary infection , hemorrhage , inflammatory pneumonitis , and bacterial abscess formation . schistosomiasis eggs that are not passed into bladder or intestinal lumen are the main cause of chronic lung diseases causing granulomatous reaction and fibrosis . tropical pulmonary eosinophilia typically results from a hypersensitivity reaction to wuchereria bancrofti and brugia malayi . tropical pulmonary eosinophilia is an immunological response to microfilariae rather than acute infection , which usually present as reticulonodular opacities . the diagnosis of parasitic infections is difficult on chest radiography or hrct of chest because of nonspecific presentations . bronchoscopy may be helpful for diagnosis of parasitic infestation by variety of ways like direct visualization , bal , brushing , and tblb . parasitic lung diseases can also show microscopic pulmonary calcification on lung biopsy . in the current report , transbronchial lung biopsy revealed showed thickened alveolar septa with mild lymphoplasmacytic inflammatory infiltrate in the interstitium with cross - sections of calcified parasitic larvae . tblb is performed for obtaining tissue specimen from peripheral lung masses and focal or diffuse lung infiltrates . the technique is useful in patients with suspected lung cancer , fungal and mycobacterial lung infections , unexplained infiltrates in immunocompromised hosts and in patients with suspected pulmonary sarcoidosis , lymphangitic carcinomatosis , and in selected cases of pulmonary langerhan 's cell histiocytosis , lymphangioleiomyomatosis , and cryptogenic organizing pneumonia . with increasing travels and worldwide migration , parasitic infections should be considered in differential diagnosis of interstitial lung diseases particularly in the evaluation of diffuse lung infiltrates . bronchoscopy and transbronchial lung biopsy can be useful in the diagnosis of diffuse lung infiltrates .

parasite infections are increasing worldwide due to increasing migration and traveling . parasitic infections can affect lungs and present as a focal or diffuse lung diseases . high index of suspicion and detailed history are most important . we present a case of interstitial pneumonitis caused by parasite infestation , which was diagnosed on transbronchial lung biopsy .

ventriculoperitoneal shunt ( vps ) is a well - established cerebrospinal fluid ( csf ) diversion procedure in cases of hydrocephalus . there are a lot of complications associated with this procedure and shunt extrusion via different natural orifices is one of them . transanal and per abdomen shunt extrusion are well documented in literature while shunt extrusion via urinary bladder , vagina , uterus , gall bladder , urethra , and scrotum are sporadically reported in the literature . the distal end of shunt erodes through the wall of hollow viscera , approaches their lumen , and protrudes outside through natural orifices . the distal end of shunt protrusion depends on stiffness of shunt tip , thickness of the wall of hollow viscera , condition of the visceral wall , underlying infection , fixity of viscera , and the operative hand of the surgeon . the incidence of perforation is inversely related to the mobility of gut , and colon is the most frequently perforated viscus due to its immobility . a 10-month - old male child presented with protrusion of shunt tubing from the anus , incidentally noticed by his parents . the patient had a history of excessive cry along with extrusion of shunt tube through anal orifice [ figure 1a ] . the abdomen was soft , nontender , and bowel sounds were audible on abdominal auscultation . his developmental milestones were delayed , and rests of the neurological examinations were within normal limit . x - ray abdomen erect imaging showed no pneumoperitoneum [ figure 1b ] . computed tomography head showed dilated lateral and third ventricles . the patient was taken up for the removal of previous shunt assembly and placement of new one . at the time of distal end removal , lower end of shunt had retracted back into rectum ; hence , we planned to remove that via incision at abdominal insertion point . two incisions were given , one at the cranial end of shunt tubing , and another one at the abdominal insertion site . the shunt was divided at the abdominal insertion point , and cranial part was pulled out from cranial incision while abdominal part was pulled through the abdominal incision . the shunt tube had perforated the appendix and entering through the perforation [ figure 2a and b ] . an urgent gastrosurgery opinion was taken and urgent laparotomy via extension of same incision was performed by the gastrosurgeon . postoperative period was uneventful , and patient discharged from hospital after removal of stitches and is under follow - up . ( a ) protrusion of the lower end of the shunt through anus , ( b ) a radiograph of a patient showing the course of the lower end of the shunt through a large intestine ( a ) an intraoperative photograph showing the appendix coming out of the abdominal incision while pulling the lower end of the shunt which was perforating the appendix at the tip ( black arrow ) , ( b ) the perforated appendix during appendicectomy vps is most commonly used and universally accepted surgical procedure for the management of hydrocephalus in children . a number of complications associated with this procedure are mentioned in literature with abdominal complications accounting for 1030% of all . bowel perforation by the tip of the catheter and extrusion of the shunt through external orifices is one of them . although this is a rare complication and have incidence rate of 0.10.7% only . the first case of anal extrusion of distal vps was reported by wilson and bertan in 1966 . the duration between shunt surgery and bowel perforation was found to be minimum in infants and was related with the age of the patient . the interval between shunt insertion to the protrusion of catheter from anus ranges from 2 to 20 months with an average of 6.1 months . in our case , this period was 6 months . nonenteric visceral perforation has also been sporadically reported in the literature and that includes urinary bladder , urethra , scrotum , vagina , gall bladder , and uterus . factors associated with perforation of gut are foreign body reaction , stiff tip of shunt , thin bowel wall in pediatric patients , abdominal infections , silicon allergy , use of trocar for insertion of peritoneal end , previous abdominal surgery , and chronic wear and tear produced by tip of the shunt . many patients do not present with significant abdominal symptoms because the fibrous tract formed at the perforated site usually seals the perforation and prevents spillage of fecal matter into the peritoneum , which would otherwise lead to peritonitis . hence , the correct diagnosis may be delayed until a very later stage at which gram - negative or anaerobic meningitis , encephalitis or ventriculitis has been fully established , leading to significant morbidity and/or mortality to the patients . mechanism of shunt extrusion is not well - established but most accepted hypothesis is that after bowel perforation shunt tubing propels outside with sequential peristaltic movement of the gut . in the cases of shunt extrusion through anal opening , most common site of bowel perforation is colon ( 70% ) while in cases of oral extrusion most common site is the stomach . early diagnosis , adequate clinical , radiological and biochemical evaluation , and prompt treatment are the key to successful treatment . the standard method of treatment is the removal of the extruded shunt system , control of infection , improvement of general condition followed by csf diversion procedure . the different available alternatives are laparotomy with the revision of the peritoneal end of shunt , conventional exploratory laparotomy and repair of bowel perforation , endoscopic localization of enterotomy site and removal of shunt , shunt removal , external diversion of csf and use of antibiotics , and later on replacement of vps when no infection are seen . in uncomplicated cases of shunt extrusion , shunt tubing can be removed via extruding orifice and bowel perforation can be managed conservatively while in complicated cases shunt removal is advised via a laparotomy . appendicitis after ventriculoperitoneal shunting is a known complication but to best of our knowledge shunt extrusion via appendicular perforation is not documented in literature yet . in such cases , shunt removal via extruding orifice may be quite difficult . theoretically , there are high possibilities of associated appendicitis which can result in the development of frank peritonitis or appendicular abscess after shunt removal through extruding orifice . so in our opinion , such type of cases whether complicated or not should be placed in special category and shunt removal should be done via laparotomy and followed by prophylactic appendectomy . . such type of case should be consider in special category in which shunt removal without proper surgical exploration and added appendicectomy may be quite difficult and hazardous for the patient . optimal management plan in such type of cases either uncomplicated or complicated is laparotomy and removal of the shunt with added appendicectomy .

perforation of abdominal viscera and protrusion of the distal end of ventriculoperitoneal shunt ( vps ) through natural orifice is well known but rare complication . we report a case of a transanal protrusion of distal end of vps through appendix perforation without any symptomatology of prior appendicitis . to the best of our knowledge , no case of such kind has been reported in literature yet . the management plan of these patients should be looked in a different way because they may have underlying inflammation of the appendix and distal end of shunt removal should be done by proper surgical exploration followed by added appendicectomy .

recently , new oral anticoagulants have been approved as alternatives to warfarin for patients with atrial fibrillation . rivaroxaban is one of the novel anticoagulants , which is an oxazolidinone derivative and inhibits both free factor xa and factor xa bound with the prothrombinase complex . it is a highly selective direct factor xa inhibitor with oral bioavailability and rapid onset of action . there are some advantages of the new agents compared with warfarin including rapid anticoagulation after an oral dose and lack of dietary or drug - drug interaction . however , there are no specific antidotes for the anticoagulant effect of rivaroxaban in the event of a major bleeding , unlike warfarin . we present a case of spontaneous rectus sheath hematoma ( rsh ) during rivaroxaban therapy for atrial fibrillation in an elderly female patient . a 75-year - old woman presented to the emergency department with the complaints of fatigue and abdominal pain after coughing . the patient had been started on new oral anticoagulant agent rivaroxaban therapy for nonvalvular atrial fibrillation for 3 days . the dose of 20 mg / day rivaroxaban was started because the creatinin clearance of the patient was above 50 ml / min . she had a blood pressure of 70/40 mmhg and an irregular heart rate of 115 beats / min on admission . the patient had no history of any trauma or surgery ; she reported that the symptoms started after vigorous coughing . blood analyses revealed leukocytosis ( 26.5 k / ul ) accompanied by severe anemia ( 5.4 g / dl ) . platelet counts were within normal ranges and her international normalized ratio ( inr ) was 1.48 . her abdominal x - ray was normal and the stool occult blood test was negative . after the first treatment , the patient was transferred to the intensive care unit ( icu ) . repeated abdominal examination in the icu revealed increased tenderness and a palpable mass on the left side of the umbilicus . noncontrast abdominal computerized tomography scan showed a left - sided rsh , 102 45 mm in size [ figure 1 ] . a specific antidote for rivaroxaban is not available then the patient was treated with fluid resuscitation and packed red blood cells . computed tomography scan of the abdomen shows a left - sided rectus sheath hematoma ( arrow ) rivaroxaban is an oral anticoagulant agent that directly inhibits factor xa and interrupts both the intrinsic and extrinsic pathway of the coagulation cascade . rivaroxaban is currently indicated for use in patients for atrial fibrillation and prophylaxis of deep venous thrombosis . it does not require inr monitoring like warfarin . with the increasing use of the new anticoagulant agents like rivaroxaban in atrial fibrillation , bleeding complications due to these agents there are no specific antidotes for the anticoagulant effect of rivaroxaban and other new oral anticogulants unlike warfarin , thus the management of the bleeding complications include support and observation . currently , no available specific antidote exists for the management of rivaroxaban - associated bleeding events , but supporting therapy is useful which are likely to be effective for the majority of patients because of the short half - lives of these agents . recent studies showed that rivaroxaban was noninferior to warfarin for the primary endpoint of stroke and systemic embolism . there was no reduction in rates of mortality or ischemic stroke , but a significant reduction in hemorrhagic stroke and intracranial hemorrhage . the primary safety endpoint was the composite of major and clinically relevant nonmajor bleeding , which was not significantly different between rivaroxaban and warfarin but , with rivaroxaban , there was a significant reduction in fatal bleeding , as well as an increase in gastrointestinal bleeds and bleeds requiring transfusion . the main causes of the rsh include anticoagulant therapy , hematological disorders , trauma , excessive physical exercise , coughing , sneezing , and pregnancy . especially in elderly patients the risk of rsh may be increased due to the impaired functional status and weakened rectus muscle . early recognition , rapid assessment and treatment are important to reduce the complications such as hemodynamic instability , abdominal compartment syndrome , multiorgan dysfunction and even death . the treatment of such a hematoma includes transfusion with packed red blood cells and supporting therapy based on regularly monitoring of hemoglobin levels . rivaroxaban has a mean terminal half - life of 7 - 11 h so in bleeding events supporting therapies are likely to be effective for the majority of patients . several studies have shown that prothrombin complex concentrate may be useful in reversing the effects of rivaroxaban . other possible measures include the use of recombinant factor viia to reduce bleeding or the use of activated charcoal to reduce absorption in cases of overdose . several factors are reported which increase the risk of patients developing hemorrhage while receiving rivaroxaban , these include advanced age , hypertension , history of hepatic / renal disease , previous stroke , coagulopathy , concomitant use of antiplatelet agents and alcohol consumption . jaeger et al . have reported a 61-year - old female patient who developed a spontaneous spinal epidural hematoma after being treated by rivaroxaban . boland et al . also reported acute onset severe gastrointestinal tract hemorrhage in a postoperative patient taking rivaroxaban after total hip arthroplasty . in our patient , there was no other medication except rivaroxaban that could cause the hematoma . based on naranjo 's scale , a score of 7 showed that the rivaroxaban was the probable cause of the rsh . several case reports of muscle hematoma due to the antiplatelet and anticoagulant agents have been reported previously , but this is the first reported case of spontaneous rsh due to the rivaroxaban .

rivaroxaban is an oral anticoagulant agent that directly inhibits factor xa and interrupts both the intrinsic and extrinsic pathway of the coagulation cascade and is currently indicated for use in patients for atrial fibrillation and prophylaxis of deep venous thrombosis . the present case reports of spontaneous rectus sheath hematoma during rivaroxaban therapy for atrial fibrillation in a 75-year - old woman .

neuromyelitis optica ( nmo ) or devic 's disease is a rare inflammatory , demyelinating disease of the central nervous system ( cns ) that predominantly targets the optic nerves and spinal cord 1 . the disease was first described in 1870 by albutt and 24 years later , devic described the clinical characteristics of nmo the discovery of a specific nmo immunoglobulin ( nmoigg ) opened a new era in the classification and understanding of the pathogenesis of nmo 3 . nmoigg binds to aquaporin4 , which is the main channel that regulates water homeostasis in the cns . diagnostic criteria for nmo with aquaporin4 antibodies ( aqp4ab ) requires at least one core clinical characteristic , a positive test for aqp4ab using best available detection method ( cellbased assay recommended ) and exclusion of alternative diagnoses 1 . the core clinical characteristics are , for example , optic neuritis , acute myelitis , acute brainstem syndrome , symptomatic narcolepsy , or symptomatic cerebral syndrome with typical nmo brain lesions 1 . neuromyelitis optica must be distinguished from other demyelinating diseases , for example , multiple sclerosis . the presence of aqp4ab differentiates nmo from multiple sclerosis ( ms ) with high specificity 4 . in contrast to typical ms , the clinical events in nmo are usually more severe 5 , 6 . cerebrospinal fluid ( csf ) findings in nmo are also known to differ significantly from those in classical ms . csfrestricted oligoclonal igg bands are absent in most nmo patients . however , pleocytosis is usually mild , and frequently includes neutrophils , eosinophils , activated lymphocytes , and/or plasma cells 6 , 7 . studies carried out in europe , south east and southern asia , the caribbean and cuba suggest that the incidence and prevalence of nmo ranges from 0.050.4 to 0.524.4 per 100,000 , respectively 9 . the disease is mainly sporadic , although a few familial cases have been reported 10 . we describe a case of an unusual and severe course of nmo affecting almost the entire spinal cord and brain . examination on the day of admission revealed normal results as regards ecg , troponin i , and computed tomography ( ct ) of the chest and abdomen . the next day , the patient reported headache and neurological examination showed rightsided hemiparesis and afferent pupillary defect of the left eye suggesting an afferent optic nerve defect . within a few hours , the patient showed a rapid neurological deterioration with progressive tetraplegia and global decline . mri of the spinal cord showed myelitis in the spinal cord segments c2 to th5 ( fig . csf examination revealed polymorphonuclear pleocytosis ( leukocytes 1210 10/l , neutrophils 95% ) and an increased total protein concentration ( 2273 mg / l ) . due to spinal cord mri and csf findings , infectious transverse myelitis could not be excluded and the patient was treated with dexamethasone , acyclovir , ceftriaxone , ampicillin , and levofloxacin . ( a ) sagittal t2fse mri of the spinal cord showing high signal changes . ( b ) axial t2flair brain mri showing high signal changes in thalami , internal capsule , and corpus callosum . ( c ) axial t2flair brain mri showing high signal changes in pons , medulla oblongata , cerebellum , and middle cerebellar peduncle . the next day , the patient 's condition worsened ; she became comatose and had a respiratory failure that required assisted ventilation ; this might be caused by bilateral phrenic nerve involvement as its roots originate from c3 to c5 where the lesion was also seen ( fig . brain mri showed high signal changes in thalami , internal capsule , and corpus callosum ( fig . 1b ) and also similar changes in pons , medulla oblongata , cerebellum , and middle cerebellar peduncle ( fig . treatment with methylprednisolone and immunoglobulin was attempted and as serum aqp4ab was confirmed as positive ( indirect immunofluorescence assay was used , the titer was 19.85 , normal < 10 ) and the patient did not respond to the treatment , the patient was also treated by means of plasmapheresis . histological examination of the cns revealed extensive , sharply limited demyelination and axon defect ( fig . 2a and b ) . the spinal cord was almost entirely affected . only the lumbar area was partly spared . smaller demyelination foci were found in the periventricular area of the hippocampus and in the corpus callosum . demyelination was verified by showing both cd68positive macrophage infiltration and betaapp positivity as signs of axonal damage . ( b ) the axon defect is shown by immunostaining ( brown staining ) of betaamyloid precursor protein ( app ) . this is the first case report of nmo described from finland from the aqp4ab era . there is only one older publication of a finnish nmo patient from the preaqp4antibody era 11 . the patient was referred to hospital with an acuteonset chest pain , which is an unusual first symptom of nmo . demyelination affected almost the entire spinal cord , sparing only partly the lumbar cord , which is unusual at first myelitis . lesions involving the lumbar or sacral spinal cord in addition to the cervical and thoracic portions have been reported only in 11% of patients at first myelitis . previous reports have revealed that 92% of the patients have at least one spinal cord lesion extending over three or more vertebral segments at their first myelitis . it has been reported that seropositive women have more severe clinical attacks than males , as evidenced by high lesion load in the spinal cord and other types of coexisting autoimmunity 6 . brain mri abnormalities are relatively common and may be relatively unique by virtue of localization and configuration 12 , as seen also in our patient . the histopathological findings in the cns , csf , and aqp4ab seropositivity are consistent with neuromyelitis opticatype demyelination , although the disease course of our case was not typical of nmo due to its rapid and severe course . according to hospitalbased observational studies , mortality of nmo ranges from 2.9% to 25% and reported that disease duration at the time of death ranged from 6 months to 23.6 years 6 . in addition , our patient 's histological examination revealed extensive , sharply limited demyelination of the spinal cord and brain in the acute phase of the disease . to the best of our knowledge , the treatment in this particular case was targeted to multiple causes of the symptoms due to the unknown etiology in the beginning of the disease . acute attacks and relapses of nmo are generally treated with intravenous glucocorticoids followed by plasmapheresis for refractory or progressive symptoms 13 , 14 . however , there are no controlled trials evaluating the treatment of nmo , and recommendations are primarily supported by data from observational studies and by the clinical experience of experts . despite several forms of treatment , the patient did not survive .

key clinical messageneuromyelitis optica is a rare inflammatory , demyelinating disease of the central nervous system that predominantly targets the optic nerves and spinal cord . our case represents an unusual and severe course of neuromyelitis optica . despite several forms of treatment , our patient died after a severe and shortterm attack .

juvenile polyposis syndrome , a rare disorder in children , is characterized with multiple hamartomatous polyps in alimentary tract . a variety of manifestations include bleeding , intussusception , or polyp prolapse . in this study , we present an 8-month - old male infant of juvenile polyposis syndrome initially presenting with chronic anemia . to the best of our knowledge , we report a rare case of an 8-month - old male infant who presented with chronic anemia and gastrointestinal bleeding initially . panendoscopy and abdominal computed tomography showed multiple polyposis throughout the entire alimentary tract leading to intussusception . technetium-99m - labeled red blood cell ( rbc ) bleeding scan revealed the possibility of gastrointestinal tract bleeding in the jejunum . histopathological examination on biopsy samples showed peutz - jeghers syndrome was excluded , whereas the diagnosis of juvenile polyposis syndrome was established . however , polyps recurred and occupied the majority of the gastrointestinal tract in 6 months . juvenile polyposis syndrome is an inherited disease , so it is not possible to prevent it . concerning of its poor outcome and high mortality rate , it is important that we should increase awareness and education of the parents at its earliest stages . it has been shown that affected children are susceptible to cancers and fatal medical conditions . the common presentations include anemia , recurrent gastrointestinal bleeding , diarrhea , rectal prolapse , intussusception , protein - losing enteropathy , starvation , and malnutrition . an 8-month - old male infant presented melena and iron deficiency anemia with hematocrit of 10.6% and hemoglobin of 2.9 g / dl . panendoscopy revealed several polypoid lesions with ulceration over the body and the prepyloric area , which were bleeding to touch ( fig . computed tomography depicted numerous nodules throughout the entire alimentary tract , indicating intestinal polyposis ( fig . panendoscopy : at least 6 polypoid lesions over gastric body and prepyloric area with ulceration and touch bleeding . abdominal computed tomography showed multiple nodules within the stomach , small intestine ( including duodenum , jejunum , and ileum ) , and descending colon leading to intussusception . owing to uncorrectable anemia as well as sonography constantly demonstrating intussusceptions ( figs . 3 and 4 ) , laparoscopy - assisted enteroscopic polypectomy and reduction of intussusceptions were performed ( fig . the pathology report described hamartomatous polyps with elongation , tortuosity , and dilatation of the gastric foveae and intestinal mucosal glands . the underlying stroma is characterized by broadband smooth muscle fibers , intermingling with the glands ( fig . technetium-99m - labeled rbc bleeding scan : the possibility of gastrointestinal tract bleeding in the jejunum . -1 polypectomy and reduction of intussusceptions : ( a ) 2 intussusceptions were found , 1 at 20 cm distal to treitz ligament and another 1 at jejunoileal junction . ( b , d ) numerous polyps were found in whole small bowel , especially in jejunum . microscopically , hamartomatous polyps composed of elongation , tortuosity , and dilatation of the gastric foveolae and intestinal mucosal glands . the underlying stroma is characterized by broadband smooth muscle fibers , intermingling with the glands . thereafter , the patient suffered from multiple episodes of anemia , gastrointestinal hemorrhage , rectal prolapsed polyps , symptomatic colic - colic intussusceptions requiring radiologic reductions , protein - losing enteropathy , and immunodeficiency . it was our impression that the immunodeficiency was secondary to the remarkable protein - losing enteropathy and malnutrition . human immunodeficiency virus ( hiv ) infection was excluded due to negative maternal hiv testing during prenatal checkups . it is not a routine in our institution to conduct another hiv examination before 24 months of age in infants with prior negative virologic tests . the serology study showed notably hypoglobulinemia with igg of 115 mg / dl and igm of 33 mg / dl . complement levels were also significantly below the normal limits with c3 of 51 mg / dl and c4 of 7 mg / dl . intravenous immunoglobulin was administered . in 6 months , polyps recurred and occupied the majority of the gastrointestinal tract . juvenile polyps are mostly solitary , influencing approximately 1% of preschool and school - aged children . juvenile polyposis syndrome may extensively affect a large portion of alimentary tract , and be usually related to malignant potential . it has been known with 3 subtypes : diffuse juvenile polyposis of infancy ( < 6 months of age ) , diffuse juvenile polyposis ( 6 months5 years of age ) , and juvenile polyposis coli ( 515 years of age ) . histologically , the polyps in juvenile polyposis syndrome are composed of mucous filled , dilated glands that are often associated with inflammatory cell infiltration . unlike those in peutz - jeghers syndrome , smooth muscle proliferation is rarely seen . nevertheless , the polyps consisted of smooth muscle bands in stroma in this case . peutz - jeghers syndrome was initially suspected . however , the patient had neither relevant family history nor oral pigmented lesions that were highly associated with peutz - jeghers syndrome . peutz - jeghers syndrome was excluded , whereas the diagnosis of juvenile polyposis syndrome was established . it is featured with widespread hamartomatous polyps in the entire gastrointestinal tract in infants < 6 months of age . the initial manifestations of anemia and gastrointestinal bleeding were present at 8 months of age . we presumed that this patient should be categorized to juvenile polyposis of infancy . with regard to treatment , surgery is the mainstay to remove polyps . however , repeated operations are usually required because recurrence is not uncommon . in our case , redo enteroscopic polypectomy was technically difficult as well as might bear much higher risks , so it was abandoned . as nearly the entire small bowel is involved , resection of affected bowels was less considered because it would lead to prominent intestinal failure . the decision was made to perform supportive treatment . to the best of our knowledge ,

abstractbackground : juvenile polyposis syndrome , a rare disorder in children , is characterized with multiple hamartomatous polyps in alimentary tract . a variety of manifestations include bleeding , intussusception , or polyp prolapse . in this study , we present an 8-month - old male infant of juvenile polyposis syndrome initially presenting with chronic anemia . to the best of our knowledge , this is the youngest case reported in the literature.methods:we report a rare case of an 8-month - old male infant who presented with chronic anemia and gastrointestinal bleeding initially . panendoscopy and abdominal computed tomography showed multiple polyposis throughout the entire alimentary tract leading to intussusception . technetium-99m - labeled red blood cell ( rbc ) bleeding scan revealed the possibility of gastrointestinal tract bleeding in the jejunum . histopathological examination on biopsy samples showed peutz - jeghers syndrome was excluded , whereas the diagnosis of juvenile polyposis syndrome was established.results:enteroscopic polypectomy is the mainstay of the treatment . however , polyps recurred and occupied the majority of the gastrointestinal tract in 6 months . supportive management was given . the patient expired for severe sepsis at the age of 18 months.conclusion:juvenile polyposis syndrome is an inherited disease , so it is not possible to prevent it . concerning of its poor outcome and high mortality rate , it is important that we should increase awareness and education of the parents at its earliest stages .

the phylum apicomplexa includes a large group of protozoan parasites responsible for a wide range of animal and human diseases . among the human pathogens are plasmodium falciparum and plasmodium vivax , the major causative agents of human malaria , as well as cryptosporidium parvum and toxoplasma gondii , which are particularly pathogenic in immunocompromised patients . apicomplexa are intracellular obligatory parasites that multiply in a so - called parasitophorous vacuole . although the alterations of the host cell harboring toxoplasma or plasmodium parasites have been extensively documented at the cellular level , still little is known about how the parasite manipulates the host cell at the molecular level , with the notable exception of plasmodium infection of host erythrocytes . so far , most molecular studies on the host - parasite interface have focused on the role of parasite factors that are secreted by the invading parasite , as well as by the resident intracellular parasite , into the host cell . these intracellular parasites are expected to profoundly reorganize the host cell for their own needs to ensure safe growth and persistence , and presumably to deploy the most sophisticated mechanisms to this end . emerging evidence indicates that viruses and bacteria manipulate the microrna ( mirna ) pathways of the host cells they infect . mirnas are the most abundant class of small , non - coding , single - stranded rnas and are involved in regulating gene expression at the post - transcriptional level . in silico target prediction suggests that mirnas may control up to 30% of the translation of the human transcriptome . as such , they govern a variety of fundamental cell functions , including cell proliferation and apoptosis , and are key regulators of cell metabolism . when homeostatic conditions are disrupted - for example , when cells encounter micro - organisms - these regulatory pathways might also contribute to host cell responses / defenses ( i.e. , the inflammatory response ) against the foreign bodies . for example , cell infection by mammalian viruses might be counteracted by cellular mirnas that target either the virus itself , as in the case of the rhabdoviral vesicular stomatitis virus , or a host factor critical to the virus , as for the lentiviral hiv . conversely , mirnas can also act in favor of the micro - organism , either when it is pathogen - encoded ( e.g. , mammalian virus - encoded mirnas ) or when the micro - organism subverts host mirnas to its own benefit . effectors from the bacteria pseudomonas syringae have been recently shown to suppress transcriptional activation of some mirnas generated upon sensing of pamps ( pathogen - associated molecular patterns ) by arabidopsis . recent data have begun to show how two apicomplexan parasites , cryptosporidium and toxoplasma , are able to target mirnas in the host cell to alter the cellular environment in ways that favor their intracellular development . cryptosporidium is able to trigger the down - regulation of let-7i ( a mirna with complementarity to toll - like receptor ( tlr)-4 mrna ) in the host cell , leading to the up - regulation of tlr4 , a key pathogen recognition molecule that plays a central role in epithelial innate immunity to cryptosporidium infection . various studies have further substantiated the ability of cryptosporidium to alter mirna expression in cholangiocytes [ 9 - 11 ] . it is emerging from these studies that following cryptosporidium infection , specific mirna cluster genes are activated by the binding of the nf-b ( nuclear factor - kappa b ) p65 subunit to their promoter , and that inhibition of these mirnas increases parasite burden . these results mirror those showing differential alterations in mature mirna expression profiles in primary human fibroblast cells following toxoplasma infection . zeiner et al . showed that toxoplasma infection specifically increased the transcription of the mir-17/92 loci by two- to three - fold in human fibroblasts . the effect is apparently a specific response to toxoplasma infection since levels of the mature mir-17/92-derived mirnas remained unchanged upon infection by the closely related parasite neospora caninum . microarray data comparing the mirna profiles of cells infected by toxoplasma or cryptosporidium or treated with lipopolysaccharide ( lps ) have revealed several important findings . for example , mir-155/bic is up - regulated upon toxoplasma infection but remains unaffected or is down - regulated when exposed to cryptosporidium or lps , respectively . of note , mir-155 has an important role in the mammalian immune system , regulating , at least in part , cytokine production . two other mirnas , mir-198 and mir-320 , are both up - regulated upon toxoplasma infection whereas they are down - regulated after cryptosporidium infection and unaffected after lps stimulation . these data point to specific modifications of host mirna profiles upon cell infection by apicomplexa parasites . obviously , any change in the host cell mirna pattern might indicate either a defense mechanism by the cell or a subversion strategy by the parasite , two processes that can be differentiated by evaluating the consequences on parasite growth of disruption or over - expression of the target mirna pathway . given the propensity of apicomplexan parasites to co - opt cellular pathways and activities for their benefit , it is perhaps not surprising that these parasites could also reshape their cellular environment by reprogramming the host s rna interference machinery . specific host mirnas could either counteract the intracellular growth of parasites or facilitate it , the two possibilities being not mutually exclusive and depending on the physiological context . these findings open an exciting opportunity to pursue a deep understanding of how the host proteome can be reprogrammed dynamically and reversibly upon apicomplexa infection . an additional line of research should explore the upstream regulatory mechanisms , that is , how the parasites directly interfere with rna silencing pathways and , more specifically , the parasite effectors that are involved in the process . as discussed above , in response to cryptosporidium and toxoplasma infections , the expression of specific mirna genes is altered at the transcriptional level . mirna are generated through the concerted action of multi - subunit complexes that promote the sequential cleavage , export , and loading of mirna into silencing complexes . an increasing number of reports suggest that , beyond the transcriptional control of genes that code cluster mirnas , each of these steps serves as a potential point of regulation , and therefore adds additional complexity to mirna - dependent gene regulation . could parasite regulators of host mirnas be ribonucleic acids ? unlike in cryptosporidium and plasmodium species , the toxoplasma genome encodes elaborate rna silencing machinery that generates endogenous small silencing rnas , including specific mirnas . thus , an attractive hypothesis is that toxoplasma has the potential to secrete its own mirnas to hijack the host cell mirna defense pathway , similar to what some viruses are able to do . it is known that apicomplexan parasites inject various molecules into the host cell resulting in extensive remodeling of the host cell gene expression profile and metabolic pathways [ 17 - 19 ] . the expression of host mirnas can also be altered in response to parasite recognition by cell surface tlrs . both intrinsic and extrinsic acting factors could then interfere with target host mirnas , at any point of the processing of the pri - mirnas and biogenesis of the mirnas - transcription , processing , or export .

rna silencing plays an important role in development through the action of micrornas , which fine tune the expression of a large portion of the genome . it is also very important in innate immune responses , especially in antiviral and antibacterial defenses in plants , insects , and animals . two recent papers now indicate that apicomplexan parasites display the ability to interfere with host microrna populations .

a 60-year - old male patient , with a known history of arterial hypertension and type-2 diabetes mellitus , was referred to the outpatient echocardiography laboratory to undergo stress echocardiography because of recent episodes of chest pain occurring on mild exertion . he was on drug therapy with angiotensin - receptor blocker , thiazide diuretic , nondihydropyridine calcium antagonist , and dronedarone , which had been started because of recent episodes of paroxysmal atrial fibrillation and discontinued 2 days before the stress test . the patient received a standard protocol of high dipyridamole infusion in two doses ( 0.56 mg / kg and 0.28 mg / kg ) followed by atropine administration ( 1 mg in four 0.25 mg doses ) . at rest , no ischemic abnormalities were observed on electrocardiogram ( ecg ) and transthoracic echocardiography ( tte ) [ figure 1 and videos 13 ] . after completion of dipyridamole infusion , the patient complained a mild chest discomfort , without any significant ecg changes and any apparent wall - motion abnormalities on tte [ videos 46 ] . after atropine injection , a worsening of the anginal symptoms combined with a descending st - depression in v3 occurred ; despite the absence of relevant echocardiographic changes , two - dimensional ( 2d ) strain analysis showed lower longitudinal strain of the anterior interventricular septum from rest to peak dose [ figure 2 ] . afterward , as recommended , aminophylline was administered ; interestingly , a more pronounced st - depression and deep inverted t - waves in v2v4 appeared [ figure 3 ] . the patient was admitted to the cardiology department and underwent coronary angiography from the radial access , which revealed a long myocardial bridge ( mb ) of the left anterior descending ( lad ) artery with systolic milking [ figures 4 , 5 and videos 79 ] . the patient was , thereafter , treated with a beta - blocker and discharged without symptoms and ischemic abnormalities on rest ecg . electrocardiogram at rest two - dimensional strain analysis showing global longitudinal strain at rest ( upper panel ) and at peak dose of dipyridamole ( lower panel ) electrocardiogram after injection of atropine and aminophylline coronary angiography images in diastole ( left ) and systole ( right ) showing myocardial bridge of the left anterior descending artery with systolic milking angiographic images did not change after intracoronary nitroglycerine administration this case depicts a clinical scenario of positive dipyridamole stress test in a patient affected by mb of the lad . although mb has been classically deemed a benign coronary artery abnormality , it has been recently related to acute myocardial infarction and sudden cardiac death . moreover , mb has been also associated with endothelial dysfunction , early atherosclerosis , and coronary vasospasm . in particular , a worsening of systolic coronary narrowing of mb has been found when using vasodilator agents , such as nitroglycerine , which are usually not administered in these patients . in our case , dipyridamole provoked chest pain associated with minor ecg ischemic changes , such as an only one - lead ( v3 ) st - depression ; these abnormalities worsened after the administration of atropine and later , aminophylline , likely because of drug - induced positive inotropic and chronotropic effects . atropine might also have determined myocardial ischemia through a paradoxical coronary vasoconstriction induced by acetylcholine as observed in the presence of endothelial dysfunction and mb . this case has the following interesting implications : ( 1 ) the ability to detect mb also using a vasodilator stress test ( and not only dobutamine echocardiography or exercise test ) , particularly with the addition of atropine injection , ( 2 ) the utility of 2d strain analysis in confirming subtle regional wall - motion abnormalities , and ( 3 ) peculiar diagnostic features , on stress echocardiography , suggesting mb rather than obstructive coronary artery disease . in particular , the observed ecg and echocardiographic ischemic changes , although suggestive of a lad disease , appeared to be late occurring and less extended than usually observed in patients with stable hemodynamic lad obstruction , which should have determined more pronounced ecg and wall - motion abnormalities . indeed mb , differently from a fixed coronary obstruction , is a dynamic stenotic lesion , requiring a consistent increase in heart rate and myocardial contractility to provoke myocardial ischemia . hence , mb could have been clearly unmasked only whenever positive inotropic and chronotropic agents had been added to the vasodilator stress caused by dipyridamole . thus , the finding of worsening ischemic abnormalities after the administration of atropine ad aminophylline , during dipyridamole stress echocardiography , may represent a particular diagnostic feature of mb .

a 60-year - old male patient was submitted to dipyridamole - atropine stress echocardiography ( dse ) for chest pain during exertion . at rest , no electrocardiographic ( ecg ) and transthoracic echocardiographic ( tte ) abnormalities were observed . after dipyridamole infusion , the patient complained a mild chest discomfort , without ecg changes and tte wall - motion abnormalities . subsequently , worsening of the anginal symptoms combined with descending st - depression and t - negative waves occurred after atropine and unexpectedly , aminophylline administration . coronary angiography was performed showing a myocardial bridge ( mb ) of the left anterior descending artery . the occurrence , during dse , of worsening ischemic abnormalities after atropine and aminophylline administration may be a particular diagnostic feature of mb .

giant cell tumour is the commonest benign bone tumour arising at the epiphyseometaphyseal regions of long bones . around the knee there are very few similar cases reported worldwide and it is the purpose of this report to describe the management of such a case . a 17 year old girl presented with swelling of ankle and pain while walking for six months . radiographs were suggestive of a giant cell tumour , computerised tomography revealed cortical break , en block resection was done with ipsilateral proximal fibula used in reconstruction of ankle mortise . giant cell tumour of long bones are common but those involving the distal fibula are exceedingly rare . the management of such tumours with high recurrence rates can be easily accomplished by en block resection and reconstruction of the ankle mortise with proximal fibula ensuring good range of motion of the joint post operatively . first described by sir astley cooper in the year 1818 , giant cell tumour of bone or osteoclastoma is the commonest benign bone tumour encountered by an orthopaedic surgeon . it is characterised radiographically as a lytic lesion occurring in the ends of bones and has well known propensity for local recurrence after surgical management . current treatment modalities including a meticulous curettage with extension of tumour removal using high speed burrs and adjuvant local therapy has significantly lowered the recurrence rates to less than 10% from 60% in the past with curettage alone . the commonest age is the 3rd or the 4th decade with a slight female predominance . the other less common infrequent sites are sacrum , distal tibia , proximal humerus , proximal femur and proximal fibula . involvement of distal fibula by benign aggressive and malignant tumors usually necessitates resection of the involved segment of fibula . the incidence of giant cell tumour of distal fibula was found to be less than 1% of 1182 cases . schajowicz , in his series of 362 cases has reported only a single case affecting the lower end of the fibula ( 0.28% ) . a seventeen year old girl presented with swelling around the right ankle for six months associated with pain while walking and restriction on squatting . the swelling was six by four by two centimetres in size , firm to hard in consistency , no tenderness on deep palpation . [ fig 1 ] clinical picture showing swelling and radiographs showing expansile lesion with soap bubble appearance . anteroposterior and lateral radiographs were taken which showed single epiphyseal expansile lesion with soap bubble appearance . magnetic resonance imaging could not be done as the facility was not available then in our government hospital and patient s financial background prevented us getting an imaging from private centres . all routine haematological investigations were found to be normal and chest radiograph was also found to be normal . an excisional biopsy was planned with reconstruction using the proximal end of the ipsilateral fibula . under pneumatic tourniquet without exsanguination an en bloc excision of the lateral malleolus with lower third of the fibula the level of resection of distal fibula was determined by the computerised tomography , clinical intra operative findings and by pre operative radiographs . the proximal fibula was reversed with head of fibula incorporating into the ankle mortise and fixed to the remaining fibula using plate and screws . meticulous haemostasis was achieved after release of the tourniquet , and the wounds were closed in layers . patient kept non weight bearing for three months and full weight bearing at six months after the removal of screws . patient was followed up and at the end of one year patient had full range of motions with mild restriction of dorsiflexion of the affected ankle [ figs 3 ] . b- fixation of the reversed proximal end of fibula and incorporation into the ankle mortise . d - photomicrograph of the specimen showing multinucleate giant cells suggestive of giant cell tumor six month follow up showing good radiological union ( a ) and clinical photograph showing good dorsiflexion and plantar flexion ( b , c ) . the proximal fibula can be sacrificed for the purposes of reconstruction as is recommended for lower end fibula and distal radius . giant cell tumour of the bone has an unpredictable behaviour , not always related to radiographic or histological appearance . many earlier studies had shown very high local recurrence rates after curettage and bone grafting . the use of modern imaging techniques and extended curettage through the use of power burrs and local adjuvants have improved outcome with reduced recurrence rates . phenol , liquid nitrogen , bone cement , hydrogen peroxide , zinc chloride and more recently , argon beam cauterization have been employed as local adjuvants . chemical or physical agents work by inducing an additional circumferential area of necrosis to extend the curettage . in distal fibular resection without reconstruction , soft - tissue reinforcement , even when it is possible , can not fully compensate for the loss of stability . resection of the lateral ankle can cause a varus instability or a collapse into valgus . this technique of ankle resection and reconstruction has provided good oncological and functional results and recommended in young active patients requiring resection of distal fibula . giant cell tumour of long bones are common but those involving the distal fibula are exceedingly rare . the management of such tumours with high recurrence rates can be easily accomplished by en block resection and reconstruction of the ankle mortise with proximal fibula ensuring good range of motion of the joint post operatively . resection arthrodesis which was the method primarily employed for bone tumours involving ankle can now be replaced with ankle reconstruction . distal fibula gct being an extremely rare entity and its management not been described , reconstruction of ankle with proximal giant cell tumour of distal fibula are extremely rare and such benign tumours with high recurrence rates with the evidence of medial cortical break should be managed by an en block resection and reconstruction of the ankle mortise and the preferable method would be by the usage of proximal fibula graft . this method produced no recurrence and ensured good range of motions and can effectively replace resection arthrodesis as management in cases which require resection of lateral malleolus .

introduction : giant cell tumour is the commonest benign bone tumour arising at the epiphyseometaphyseal regions of long bones . around the knee is commonest site followed by distal radius . a giant cell tumour of the distal fibula is extremely rare . we report here a case of giant cell tumour of distal fibula . there are very few similar cases reported worldwide and it is the purpose of this report to describe the management of such a case.case report : a 17 year old girl presented with swelling of ankle and pain while walking for six months . radiographs were suggestive of a giant cell tumour , computerised tomography revealed cortical break , en block resection was done with ipsilateral proximal fibula used in reconstruction of ankle mortise.conclusion:giant cell tumour of long bones are common but those involving the distal fibula are exceedingly rare . the management of such tumours with high recurrence rates can be easily accomplished by en block resection and reconstruction of the ankle mortise with proximal fibula ensuring good range of motion of the joint post operatively .

a retrospective review of clinical records of late postpartum eclampsia patients who had presented with acute onset of visual and/or neurological symptoms to the emergency department of a medical college hospital in south india between april 2009 and may 2013 was done . all patients had underwent mri with diffusion weighted imaging ( dwi ) and apparent diffusion coefficient ( adc ) mapping to differentiate vasogenic from cytotoxic cerebral edema . patient records were analyzed for presence of visual symptoms , peak systolic and diastolic blood pressures , associated neurological symptoms , location of mri lesions and time taken for recovery . the mr images were graded for extent and severity of cortex and white matter vasogenic edema , degree of confluence , mass effect and ventricular distortion on a scale from 1 to 5 . statistical analysis was performed using chi - square test and a p value of less than 0.05 was considered to be statistically significant . clinical and imaging findings are summarized in table 1 . clinical profile of patients with pres the 10 patients ranged in age from 21 to 32 years ( average-26 3.49 years ) . mean peak systolic and diastolic blood pressures were 144 21.71 and 93 9.19 mm of hg , respectively . bilateral visual loss was noted in five patients of whom three patients were able to perceive hand movements and two patients had only perception of light . the most common abnormality noted on mri included bilateral symmetrical hyperintensities on t2-weighted images and fluid attenuated inversion recovery ( flair ) sequences in the parieto - occipital regions [ figs . 1 and 2 ] . dwi showed high signal intensity with no areas of restricted diffusion while adc mapping did not show corresponding low signal intensity thus suggesting vasogenic edema . axial mr image ( fluid attenuated inversion recovery ) showing confluent edema in subcortical and deep white matter ( grade 4 ) seen as hyperintensities in bilateral parieto - occipital lobes ( patient 6 ) axial mr image ( fluid attenuation inversion recovery ) showing edema in subcortical white matter ( grade 2 ) seen as hyperintensities in bilateral occipital lobes ( patient 9 ) five patients had additional abnormalities in the cerebellar hemispheres and three patients had involvement of frontal lobes . average time taken for visual recovery was 2.6 1.34 days . follow - up mri in four patients after 1 month revealed complete resolution of these abnormalities . in the remaining , clinical symptoms and signs follow - up scans were not performed due to financial constraints . the extent of cerebral edema in patients with and without visual loss was compared and results summarized in table 2 . posterior reversible encephalopathy syndrome ( pres ) is a rare neurotoxic state that presents with altered mental status , headache , seizures , and visual disturbances along with neuroimaging features of vasogenic edema involving the posterior cerebral circulation . although several cases of pres have been reported in neurology and neuroradiology literature , exposure in ophthalmic literature has been rather limited . the causes of pres are diverse and include pre - eclampsia , eclampsia , renal insufficiency , solid organ transplantation and immunosuppressive therapy . hypertensive encephalopathy is said to be the cause of this syndrome which has been demonstrated by various clinical and experimental studies . patients with hypertensive encephalopathy have the same clinical signs as those with pres and they also have rapid resolution of clinical and imaging abnormalities once the blood pressure is lowered . the most widely accepted theory states that sudden elevation of blood pressure causes failure of autoregulation in the cerebral blood vessels leading to hyperperfusion , breakdown of blood brain barrier , and vasogenic edema . the posterior circulation is preferentially affected since it has less sympathetic innervation than the carotid circulation , thus rendering it less able to adjust to blood pressure fluctuations . however , this theory is not comprehensive because pres can affect normotensives . high degree of suspicion is required in these patients of late postpartum eclampsia because it occurs between 48 hours postpartum and 1 month after delivery frequently in women who have had a normal pregnancy and delivery and no signs of a pre - eclamptic syndrome . alternative theory implicates endothelial dysfunction as the cause for occurrence of pres in eclampsia and sepsis . this is supported by the fact that elevations in markers of endothelial dysfunction such as lactate dehydrogenase and abnormal red blood cell fragmentation have been reported in pres . vasospasm with subsequent ischemia has also been hypothesized to cause loss of integrity of the blood brain barrier in eclampsia . although reversible by definition early recognition and prompt treatment is essential to prevent secondary complications like intracerebral hemorrhage and infarction . a large retrospective study of pres in diverse clinical situations revealed the occurrence of visual symptoms in 20% and headache in 26% while seizures was observed in 74% of cases . the visual disturbances reported with pres include cortical blindness , visual neglect , homonymous hemianopia and blurred vision . cortical visual loss was observed in five of our patients ( 50% ) while the remaining denied any visual disturbances . our findings are in line with liman et al . , who observed that visual disturbances like cortical blindness , blurred vision and hemianopia are more common in eclampsia related pres ( 50% ) . patients with pres due to other etiologies more often present with severe symptoms like altered mental status or neurological deficit and lesser visual disturbances ( 27.8% ) . roth et al . , have also reported a higher percentage of disturbed vision in pre - eclampsia - eclampsia - related pres . this could be because of the younger age and lesser associated comorbidities in these patients as compared to patients with pres due to other etiologies . all patients ( with / without visual loss ) demonstrated bilateral symmetrical hyperintensities in the parieto - occipital regions on t2-weighted images . this feature along with predominant involvement of the white matter distinguishes this syndrome from bilateral posterior cerebral artery territory infarction . although several studies have reported visual symptoms of varying degrees in pres , to the best of our knowledge no study has attempted to correlate the visual symptoms with imaging abnormalities . the limitations of our study include its retrospective design , and smaller sample size of postpartum eclampsia patients . we conclude that there is a higher prevalence of cortical visual loss in patients with pres associated with late postpartum eclampsia . patients with higher degree of vasogenic edema of the posterior cerebral white matter might present with visual loss . ophthalmologists should be aware of this clinical entity since it is reversible and readily treated by controlling the blood pressure . further prospective studies comprising larger sample size and different etiologies of pres are warranted in this regard .

the purpose of this study was to determine the prevalence of visual disturbances in patients with posterior reversible encephalopathy syndrome ( pres ) associated with late postpartum eclampsia . we retrospectively reviewed the clinical records of late postpartum eclampsia patients with features of pres for the presence of visual disturbances and location of radiological abnormalities . we found a higher prevalence of cortical visual loss in patients with pres associated with late postpartum eclampsia . bilateral symmetrical vasogenic edema of the parieto - occipital lobe was the most common magnetic resonance imaging ( mri ) abnormality noted . no significant differences were observed in the extent of edema in patients with and without visual loss .

institutional review board approval was obtained from the wake forest university , and informed consent forms were completed for all participants . items for four concepts identified in the literature were glucose control , well - being and side - effects , lifestyle burden , and treatment complexity and convenience and were evaluated in a series of five focus groups made up of five to eight patients drawn from an evaluation study of community diabetes clinics in north carolina ( 11 ) . participants were male and female and white and nonwhite with simple and complex medication regimens and a1c levels that ranged from well controlled to uncontrolled . the resulting 35-item prototype instrument was administered by mail to a convenience sample of 75 patients ( the exploratory sample ) , who were treated with diabetes medications at our study community - care site , to assess item reliability , mean and distribution , redundancy or uniqueness , skewness , and construct validity . also examined were item correlations with a1c level , the multidimensional diabetes questionnaire ( 12 ) lifestyle interference scale , the medical outcomes studies ( mos ) health worries scale score ( 13 ) , and global items assessing extent that blood glucose has been unacceptably high or low . an item performance score was constructed ( 0 , weak ; 1 , moderate ; or 2 , ideal performance ) to guide item retention . fifty - five ( 73% ) patients completed the survey , and nine items were removed based on skewness or redundancy ( r > 0.75 ) with other items . in the initial test sample , patients of a large family - medicine practice treated for diabetes with a recent a1c value within the last 3 months ( the evaluation sample ) were invited to complete the study survey packet including the revised 26-item instrument and validation instrument described above . medication complexity was assessed using a score of 0 or 1 ( no / yes ) for common diabetes medications and a score of 0 or 2 ( no / yes ) for insulin , a more demanding regimen . self - reported adherence to medications was by recall of skipped or missed doses over the last 10 days . packets were mailed to patients with instructions and a voucher for a 25 usd gift certificate . exploratory factor analysis ( efa ) of the dmsat items was conducted using sas ( version 8 ; sas , cary , nc ) to assess whether the common factor model was appropriate ( 14 ) based on kaiser 's sampling adequacy , scree plot , and model fit . discriminant validity of the dmsat was examined by comparing means across levels of a1c ( < 8% and 8% ) , treatment complexity ( low and high ) , self - reported adherence , and mos health worries . for the final test sample , another sample of patients from our community diabetes care clinics ( 11 ) and from an academic medical center was recruited to conduct and evaluate confirmatory factor analysis of the dmsat and confirm validity in the evaluation sample , 194 ( 63% ) of 307 eligible patients returned the survey packet ; of these , 140 reported current medication use . participants had a mean age of 63 years , and most had completed high school ( 77% ) and had been diagnosed with diabetes at least 5 years previously ( 61% ) . one - third ( 2939% ) were taking one , two , or three medications for diabetes , with 16% taking insulin ; 14% had a recent a1c > 8.0% , and 19% rated their adherence to their medication regimen in the last 10 days as less than complete . ten items displayed high inter - item correlations ( > 0.75 ) and were removed . initial factor analysis of the reduced 16-item questionnaire identified a four - factor structure consistent with our domains of lifestyle , medical control , convenience , and well - being and explained 75% of the total variance . as shown in table 1 , dmsat scales and total score discriminated ( p < 0.05 ) between high and low levels of treatment complexity , self - rated glucose control , mos health worries scale score , and clinical value for recent a1c ( < 8% vs. 8% ) in the expected direction . correlation of the dmsat scores with continuous a1c values was 0.24 ( p = 0.0049 ) . in the final , confirmatory sample , as shown in table 1 , dmsat scales and total scores discriminated between validity groups as in the previous sample and were highly correlated with the dtsq ( r = 0.68 ; p < 0.001 ) . unlike the dmsat , the dtsq total score did not discriminate between levels of treatment complexity and clinical a1c value . the dmsat is intended as a brief measure of diabetes medication treatment satisfaction and discriminates between important correlates of patient management . it performed as well as the dtsq in detecting self - rated glucose control and health worries but showed superior properties in correspondence with treatment complexity and a1c . note that appraisals of cost of medications or specific side effects that may be caused by diabetes or its treatment , such as diminished sexual functioning , bloating , or weight gain , are not separately assessed and may require assessment elsewhere . , we believe that the 16-item dmsat offers a comprehensive assessment of satisfaction with diabetes therapy and may aid in individualizing patient diabetes treatment .

objective to develop and test a patient questionnaire on treatment satisfaction with diabetes regimens.research design and methods survey items were developed from community clinic focus groups , pretested in patients with diabetes , and examined in two samples of treated patients.resultssixteen items performed well in assessing treatment experiences : ease and convenience , lifestyle burdens , well - being , and medical control . construct validity was supported by associations ( p < 0.05 ) with treatment complexity , self - rated glucose control , health worries , and a1c . internal consistency ranged from 0.89 to 0.95.conclusionsthe diabetes medication satisfaction tool offers a comprehensive assessment of patient acceptability , with diabetes therapy useful for individualizing therapeutic decision making .

the incidence of infective endocarditis ( ie ) remained relatively stable from 1950 through 2000 at about 3.6 to 7.0 cases per 100,000 patient - years.1 in selected areas , the incidence may rise because of the concentration of populations at uniquely high risk of infection , specifically intravenous ( iv ) drug abusers . the risk of ie among iv drug abusers , 2 to 5% per patient - years , is several - fold greater than that for patients with rheumatic heart disease or prosthetic valves . ie is located on the tricuspid valve in 46 to 58% of patients with iv drug abuse . staphylococcus aureus causes more than 50% of ie occurring in iv drug abusers overall and 60 to 70% of infection involving the tricuspid valve . the clinical manifestation of ie in iv drug abusers depends on the valve involved and , to a lesser degree , on the infecting organism . the sensitivity of transthoracic echocardiography ( tte ) for the detection of vegetations in patients with native valve endocarditis ( nve ) is approximately 65% , whereas that of transesophageal echocardiography ( tee ) in these patients is 85 to 95%.2 tee is the preferred approach in patients in whom tte is technically suboptimal and is the procedure of choice for imaging the pulmonic valve.3 when initial tee is negative and the clinical suspicion of ie remains , repeating tee within 7 to 10 days is advocated.4 perivalvular abscess or intracardiac fistula formation occurs in 10 to 14% of patients with nve.6 persistent , otherwise unexplained , fever despite appropriate antimicrobial therapy in patients with ie suggests infection extending beyond the valve leaflet . tee is superior to tte for detecting invasive infection in patients with nve and prosthetic valve endocarditis ( pve ) . cardiac surgery should be considered to debride abscesses , allowing the eradication of uncontrolled infection , and to reconstruct cardiac structures , restoring homodynamic and alleviating congestive heart failure . a 21-year - old man , who was an iv drug abuser , presented with fever and dyspnea . tte showed highly mobile , large vegetation on the anterior leaflet of the tricuspid valve and no vegetation on the aortic valve , mitral valve , and pulmonic valve . the patient was admitted to the cardiology ward for antibiotic therapy and received vancomycin and gentamycin . the blood culture of the patient became positive for staphylococcus areus 3 times in 24-hour intervals . despite the antibiotic therapy for 10 days tee revealed severe aortic insufficiency , moderate tricuspid regurgitation , and an echo - lucent space between the tricuspid and aortic valves ( figures 1 ) . color doppler demonstrated a flow within the echo - lucent space and a connection between that and the left ventricle , suggesting a perivalvular abscess of the tricuspid valve opening in the left ventricle ( figure 2 ) . after consultation with a cardiac surgeon , the patient was transferred to the operating room , where he unfortunately expired due to severe bleeding and disseminated intravascular coagulation . ie is a serious complication of iv drug abuse , with a reported mortality of 5 to 10%.1 endocarditis in iv drug abusers commonly involves the tricuspid valve , and staphylococcus aureus is the most common causative organism . dyspnea , cough , and chest pain are the common complaints of iv drug users . this is likely related to the predominance of tricuspid valve endocarditis in this group and secondary embolic showering of the pulmonary vasculature . within a week after the initiation of effective antimicrobial therapy , almost 70% of patients with nve or pve are afebrile and 90% have defervesced by the end of the second week of treatment.5 persistence or recurrence of fever more than 10 days after the initiation of antibiotic therapy identifies patients with increased mortality rates and with complications of infection or therapy.5 patients with a prolonged or recurrent fever should be evaluated for intracardiac complications . perivalvular infection beyond the valve leaflet results in abscesses in the annulus or adjacent structures , intracardiac fistulas , and purulent pericarditis . periannular extension is common , occurring in 10 to 40% of all native valve ie and complicates aortic valve endocarditis more commonly than mitral or tricuspid valve endocarditis.3 intra - cardiac fistulas are rarely seen and they are estimated to account for < 1% of all cases of ie.6 fistulization of the paravalvular abscess has been found in 6 to 9% of all cases.7 perivalvular abscess and intracardiac fistula of the tricuspid valve is very rare . ie is a lethal disease if not treated aggressively with parental antibiotics , often in combination with surgery . cardiac surgery should be considered in patients with perivalvular abscess and intracardiac fistula to debride abscesses and to reconstruct cardiac structures , restoring homodynamic and alleviating congestive heart failure .

infective endocarditis is a serious complication of intravenous ( iv ) drug abuse , with a reported mortality of 5 to 10% . a 21-year - old man , who was an intravenous drug abuser , presented with fever and dyspnea . transthoracic echocardiography showed a highly mobile , large vegetation on the anterior leaflet of the tricuspid valve . despite antibiotic therapy for ten days , the patient remained febrile . transesophageal echocardiography revealed severe aortic regurgitation and an echo - lucent space between the tricuspid and aortic valves . color doppler demonstrated a flow within the echo - lucent space and a connection between that and the left ventricle , suggesting a perivalvular abscess of the tricuspid valve opening in the left ventricle . the patient was transferred to the operating room , where he unfortunately expired .

six hundred officers were recruited from the police departments of new york , ny , and oakland and san jose , calif , usa . fifty - two percent of the sample was caucasian . the mean age was 36,50 years ( standard deviation [ sd])=6.96 ) . years in the police force everaged 12.37 ( sd=6.78 ) . number of exposure to critical incidents ranged from 2 to 670 ( mean [ m]= 171.27 , sd=130.93).the incident selected for completing the questionnaires had occured on average 6.50 ( sd= 5.11 ) years ago . the peritraumatic distress scale ( pds ) was used to assess emotional , cognitive , and physical reactions occuring during a critical incident and immediatly after . dissociation at the time of the incident was measured with the peritraumatic dissociative experience questionnaire ( pdeq).the impact of event scale - revised ( ies - r ) was used to measure ptsd symptoms in the last 7 days . the mississippi scale ( mcs ) was used to measure ptsd and associated symptoms since the critical incident . we conducted a cronbach alpha reliability analysis and an oblique principal factor analysis with promax rotation on the items of the pds . two series of hierarchical multiple regression analyses were conducted using sociodemographics ( gender , ethnicity , years of service ) , exposure , the pdeq and pds as predictors of either the mcs or the ies - r six hundred officers were recruited from the police departments of new york , ny , and oakland and san jose , calif , usa . fifty - two percent of the sample was caucasian . the mean age was 36,50 years ( standard deviation [ sd])=6.96 ) . years in the police force everaged 12.37 ( sd=6.78 ) . number of exposure to critical incidents ranged from 2 to 670 ( mean [ m]= 171.27 , sd=130.93).the incident selected for completing the questionnaires had occured on average 6.50 ( sd= 5.11 ) years ago . the peritraumatic distress scale ( pds ) was used to assess emotional , cognitive , and physical reactions occuring during a critical incident and immediatly after . dissociation at the time of the incident was measured with the peritraumatic dissociative experience questionnaire ( pdeq).the impact of event scale - revised ( ies - r ) was used to measure ptsd symptoms in the last 7 days . the mississippi scale ( mcs ) was used to measure ptsd and associated symptoms since the critical incident . we conducted a cronbach alpha reliability analysis and an oblique principal factor analysis with promax rotation on the items of the pds . two series of hierarchical multiple regression analyses were conducted using sociodemographics ( gender , ethnicity , years of service ) , exposure , the pdeq and pds as predictors of either the mcs or the ies - r the pds scores ranged from 0.10 to 3.57 and the mean was 1.37 ( sd=0.56 ) . the scale was internally consistent ( =0.80 ) and showed strong convergent validity with the pdeq , r(599)=0.55 , p<0.001 . the pds factor solution is presented in table i items defining factor 1 included dysphoric emotions such as helplessness , sadness and grief , frustration and anger , and horror . factor 2 was mostly defined by items related to loss of safety and arousal , such as being afraid , thinking one might die , and having intense bodily reactions ( sweating , shaking , heart - pounding ) . items loading on factor 3 were related to the loss of positive beliefs about the self and others , such as thinking that one had done all he or she could during the critical incident , not felling prepared by one 's experience , and not believing tha others understood . we labeled the factors negative emotions , perceived life threat and bodily arousal , and appraisal . the sum of communality estimates was 7.58 , explaining 38% of the communality estimates was 7.58 , explaining 38% of the total variance and 93% of trace . intercorrelations among the pds factors were low , ranging from -0.25 to 0.12 ( p<0.05 ) . the low pds factor intercorrelation coupled with correlations of 0.17 to 0.42 ( p<0.001 ) with the outcome measures ( ies - r and mcs ) suggest that various forms of peritraumatic distress , as captured by the pds , can lead to the development of ptsd symptoms . two stepwise regression analyses ( not fully reported here ) were conducted . in predicting the mcs and ies - r , the pdeq , entered in the second step , explained 20% and 16% of unique variance on the mcs and ies - r , respectively . entering the pds in step 3 explained 11% and 8% unique variance on the mcs and ies - r , respectively . we repeated this set of analyses with the inclusion order of the pdeq and pds reversed . entered in the second step , the pds explained 29% and 17% of unique variance on the mcs and ies - r , respectively . entered in the third step , the pdeq explained 3% of unique variance on both the mcs and the ies - r . the items and factors of the pds provide insight as to what some of the salient peritraumatic dimensions may be , in addition to peritraumatic dissociation . in this study , the pds explained a significant amount of variance over and above peritraumatic dissociation which is currently considered among the mos powerful predictors of ptsd symptoms . test - retest data for the pds is currently being gathered as well as data from individuals not working in the police . in future , it would be useful to investigate prospectively the power of the pds in predicting ptsd diagnosis rather than symptoms , as well as other trauma - related disorders .

posttraumatic stress disorder ( ptsd ) occurs when significant intrusion , avoidance , and hyperarousal symptoms are manifest for at least 1 month following exposure to a traumatic event , with at least 1 month following exposure to a traumatic event , with at least 1 month elapsed betwenn the event and the diagnossis ( diagnostic and statistical manual of mental disorders , 4th edition , 1994 [ dsm - iv]).1 however , such symptoms are not necessarily manifest in the immediate aftermath of the trauman,2 nor does their intial presence strongly predict who will develop ptsd.3 one immediate response to trauma which has been convicingly linked to ptsd symptoms is peritraumatic dissociation.4 in this poster , we briefly introduce a new scale assessing immediate responses distinct from peritraumatic dissociation , and we examine its power to predict ptsd symptoms .

gastrointestinal perforations constitute one of the commonest surgical emergency encountered by surgeons [ 1 , 2 ] . management of these patients continues to be highly demanding despite the advances made in diagnosis and surgical therapy . the etiological spectrum of perforation peritonitis in india differs significantly from its western counter parts [ 35 ] . our study was carried out to highlight the spectrum of perforation peritonitis ( diffuse ) in a single unit at lok nayak hospital , a tertiary care hospital in delhi . the retrospective study was conducted at the department of surgery , maulana azad medical college and associated lok nayak hospital , delhi , from may 2010 to june 2013 . the study population included 400 patients of perforation peritonitis ( diffuse or localized ) presenting to the surgical emergency of lok nayak hospital , delhi , who underwent exploratory laparotomy . cases were studied with respect to clinical features at the time of presentation , comorbid conditions , radiological investigations , operative findings , and postoperative course . after establishing the clinical diagnosis of perforation peritonitis on performing exploratory laparotomy , the operative findings were noted and the source of peritonitis was found and managed accordingly . all patients were then treated in the postoperative ward initially under the cover of parenteral broad spectrum antibiotics and fluids ; orals were started on the appearance of bowel sounds . 98% patients presented with the history of abdominal pain , 62.5% with altered bowel habit , 41.5% with nausea and vomiting , and 28% with abdominal distention . 15% patients had positive history of nsaid intake for more than 6 months ( table 1 ) . in our study , the commonly associated comorbidity was chronic obstructive pulmonary disease followed by renal disease , diabetes , and hypertension . multiple air fluid levels on abdominal x - ray in erect position were present in 28% patients . electrolyte imbalances included hyponatremia in 21% , hypokalemia in 19% and elevated serum creatinine in 18% patients . most of the patients were operated within 24 hours of presentation under the cover of broad spectrum antibiotics after adequate resuscitation and correction of electrolyte imbalances . the commonest cause of perforation peritonitis in our study was gastroduodenal perforation due to acid peptic disease ( 45% ) followed by appendicitis ( 18.5% ) , typhoid fever ( 12% ) , tuberculosis ( 10% ) , and trauma ( 9% ) , ( table 2 ) . patients of peptic ulcer perforation usually had a short history of pain starting in epigastrium followed by generalized tenderness . 175 such were managed by an omental pedicle repair , in the other 4 cases a feeding jejunostomy was also done due to the large size of the perforation . 8% of these patients were managed by a limited resection with ileo - ascending anastomosis due to associated unhealthy caecum . patients of typhoid perforation had an initial history of high grade fever prior to abdominal complaints . of the 40 patients of tubercular perforation , 60% had previous history of tuberculosis and 50% of these patients took antitubercular therapy for < 6 months . in cases of traumatic perforation , the most common site was jejunum ( 49% ) followed by ileum ( 42% ) . the most commonly performed procedure was omental pedicle closure of peptic ulcer perforation ( 43.75% ) , followed by exteriorization of the gut in the form of ileostomy or colostomy ( 22.5% ) . appendectomy was the third most common procedure ( 17% ) , ( table 2 ) . the most common complication was wound infection followed by dyselectrolytaemia , abdominal collection , and respiratory complications . the morbidity rate was higher in the patients with intestinal perforation ( 58% ) than those with gastroduodenal perforation ( 32% ) . factors involved in death included septicemia due to fecal peritonitis , respiratory complications , pulmonary embolism , and late presentation . it is commonly seen in a younger age group in the tropical countries ( mean age in our study was 37.8 years ) as compared to the studies in the west [ 79 ] . more commonly the perforations involve the proximal part of the gastrointestinal tract ; [ 1013 ] this being in contrast to studies from the western countries , where perforations are common in the distal part [ 1416 ] . etiological factors also show a wide geographical variation . according to a study from india , infections formed the most common cause of perforation peritonitis , around 50% cases in this study were due to typhoid . in our study 22% of the cases were due to typhoid and tuberculosis . in contrast to this , noon et al . from texas in their study reported only 2.7% cases due to infections . also studies from the west have shown that around 1520% cases are due to malignancy [ 19 , 20 ] , this being in stark contrast to our study where malignancy was ascertained to be the cause of perforation peritonitis in only 3% of the cases . this shows that malignancy is not a common cause of perforation peritonitis in our setup as compared to our western counterparts . adequate preoperative resuscitation ( with fluids , etc . ) , correction of electrolyte imbalances followed by an early surgical intervention , to remove the source of infection and stop further contamination , is imperative for good outcomes minimizing morbidity and mortality . peptic ulcer perforation , perforating appendicitis , typhoid , and tubercular perforations are the major causes of gastrointestinal perforations . early surgical intervention under the cover of broad spectrum antibiotics preceded by adequate aggressive resuscitation and correction of electrolyte imbalances is imperative for good outcomes minimizing morbidity and mortality .

background . perforation peritonitis is the one of the commonest emergency encountered by surgeons . the aim of this paper is to provide an overview of the spectrum of perforation peritonitis managed in a single unit of a tertiary care hospital in delhi . methods . a retrospective study was carried out between may 2010 and june 2013 in a single unit of the department of surgery , lok nayak hospital , delhi . it included 400 patients of perforation peritonitis ( diffuse or localized ) who were studied retrospectively in terms of cause , site of perforation , surgical treatment , complications , and mortality . only those patients who underwent exploratory laparotomy for management of perforation peritonitis were included . results . the commonest cause of perforation peritonitis included 179 cases of peptic ulcer disease ( 150 duodenal ulcers and 29 gastric ulcers ) followed by appendicitis ( 74 cases ) , typhoid fever ( 48 cases ) , tuberculosis ( 40 cases ) , and trauma ( 31 ) . the overall mortality was 7% . conclusions . perforation peritonitis in india has a different spectrum as compared to the western countries . peptic ulcer perforation , perforating appendicitis , typhoid , and tubercular perforations are the major causes of gastrointestinal perforations . early surgical intervention under the cover of broad spectrum antibiotics preceded by adequate aggressive resuscitation and correction of electrolyte imbalances is imperative for good outcomes minimizing morbidity and mortality .

the term isotopic response connotes the occurrence of a new skin disorder at the site of another , unrelated , and already healed skin disease . since the introduction of this phenomenon in dermatology , a variety of dermatoses we hereby describe a young girl who developed lesions of lichen planus pemphigoides ( lpp ) over preexisting atrophic scars , an example of wolf 's isotopic response . to the best of our knowledge , such a clinical presentation of lichen planus ( lp ) pemphigoides is not documented yet in the english literature . an 18-year - old girl presented with multiple itchy hyperpigmented papules and plaques , along with intermittent blisters over the lower limbs and buttocks for last 3 months [ figures 1 and 2 ] . past history was significant for painful ulcers over the lower limbs and buttocks 6 years back . the diagnosis of pyoderma gangrenosum was made ( as evident from documents available with patient ) , and she was treated for a year , resulting in complete resolution of ulcers . the bulla was noted over both lichenified plaques and otherwise normal appearing skin of scars . interestingly , most of the lichenified plaques and bullae were found over preexisting scars only [ figure 3 ] . skin biopsy was done from multiple sites , for both histopathological examination and direct immunofluorescence ( dif ) . the histological features of lichenified plaque were orthokeratotic hyperkeratosis , acanthosis , wedge shaped hypergranulosis , basal layer degeneration and band - like of infiltrate of lymphocytes in the papillary dermis ; thus corroborating with a diagnosis of lp [ figure 4a c ] . histopathology of bulla showed a subepidermal bulla with eosinophilic infiltrate ; which were suggestive of bullous pemphigoid ( bp ) [ figure 5a c ] . the scar tissue was characterized histologically by the presence of abundant collagen fibers without any features of inflammation [ figure 6 ] . dif from plaque showed a linear pattern of deposition of igg and c3 along the basement membrane zone [ figure 7 ] . dif from the perilesional skin surrounding a bulla showed linear igg and c3 along basement membrane zone ; a feature suggestive of bp [ figure 8 ] . thus , in conjunction with the clinical , histopathological and dif findings , the final diagnosis for our case was lp pemhigoides developing over preexisting scars , as an isotopic phenomenon . atrophic scars with overlying lichenoid papules and plaques ; and tense bulla on lower limbs atrophic scars with overlying lichenoid papules and plaques on buttocks close - up of lesions on the leg . lesions are restricted to area of scar only histopathology from plaque showing hyperkeratosis , hypergranulosis , saw toothing of rete ridges , band - like infiltration in upper dermis ( a ) ( h and e , 40 ) and ( b ) ( h and e , 100 ) with basal layer degeneration ( c ) ( h and e , 400 ) histopathology from bulla showing partial loss of epidermis including basal layer ( a ) ( h and e , 40 ) and ( b ) ( h and e , 100 ) and plenty of eosinophils in split ( c ) ( h and e , 400 ) histopathology from scar showing unremarkable epidermis , increased collagen in dermis and no inflammatory cells ( h and e , 40 ) direct immunofluorescence from plaque showing linear deposition of ig g and c 3 along basement membrane zone direct immunofluorescence from perilesional skin of bulla showing linear deposition of ig g and c3 along basement membrane zone lpp is an uncommon variant of bp , clinically manifested with bullous lesions over papules and plaques of lp , as well as on the uninvolved skin . it was first described by kaposi in 1892 as a classical case of lp complicated by extensive bullous dermatosis ; which was termed as lpp . it can be precipitated by certain medications like cinnarizine , captopril , ramipril , psoralen and ultraviolet a ( puva ) , simvastatin , and antituberculous medications . igg autoantibodies against both bpag2 and bpag1 are thought to play a pivotal role in the development of the dermatosis . the widely accepted mechanism proposed is that lp or sometimes , puva causes damage to basal cells leading to unmasking of hidden antigens ; thus paving the pathway for the formation of antibodies and development of lesions of bp . a report described the correlation of levels of antibodies against bpag2 with the disease activity in lpp . the salient points of difference between lpp and bullous lp have been summarized in table 1 . dif testing show a smooth linear distribution of immunoreactants ( c3 , igg , igm , and iga ) along the basement membrane zone of dermoepidermal junction . however , a unique 200 kd antigen has been recognized on the epidermal side of salt split skin of patients with lpp and thus , lpp may represent a clinically distinct entity and not merely , a co - existence of lp and bp . effective treatment modalities include topical and systemic steroids , tetracycline , nicotinamide , dapsone , isotretinoin , methotrexate and other immunosuppressive drugs . this could be explained by the hypothesis that lpp has a unique antigenic target and thus , represents a distinct entity from bp . lpp has been reported in association with keratoacanthoma and colon adenocarcinoma . a unilateral presentation has also been reported . the occurrence of a different or unrelated dermatological disease at the site of the healed disease is termed as an isotopic phenomenon . the proposed etiologies of isotopic response include viral , immunologic , neural , vascular and locus minoris resistentiae ( a site of lessened resistance ) . however , development of lpp over preexisting scars is extremely unique and to the best of our knowledge , is not described in the english literature . it is a well - known fact that bulla formation in lpp is mediated by immunological damage against bp antigen along basement membrane zone . the predilection of the blisters to develop on the lower extremities could be explained by sluggish circulation in those regions . in our case , scars involving the dermis and subcutaneous tissue with largely unaffected epidermis might have altered the blood circulation in that particular area . this could explain the preferential deposition of immunoglobulins in the scar area and subsequent bulla formation . wolf 's isotopic response in lpp has probably not been described earlier in the medical literature . wolf 's isotopic response in lpp has probably not been described earlier in the medical literature . wolf 's isotopic response in lpp has probably not been described earlier in the medical literature .

an 18-year - old girl presented with multiple itchy hyperpigmented papules and plaques , along with tense blisters over the lower limbs and buttocks for last 3 months . these papules , plaques , and bullae were mostly localized to preexisting scars . the histopathological findings from papule and bulla were consistent with lichen planus ( lp ) and bullous pemphigoid , respectively . direct immunofluorescence ( dif ) of perilesional skin around bulla showed linear deposition of igg and c3 . considering clinical , histopathological and dif findings , diagnosis of lp pemphigoides ( lpp ) was made . the preferential localization of lpp lesions over preexisting scars was a very interesting finding in our case an extremely rare instance of the isotopic phenomenon .

. the baff / april axis plays an important role in the pathogenesis of various autoimmune diseases including sle ; inhibitors of this axis are therefore useful in treatment of these disordersresults from bliss-52 and bliss-76 trials have shown belimumab to be efficacious in treating patients with sleserious adverse effects noted with belimumab such as progressive multifocal leukoencephalopathy , depression , suicidal tendency , and malignancy are to be watched for . the baff / april axis plays an important role in the pathogenesis of various autoimmune diseases including sle ; inhibitors of this axis are therefore useful in treatment of these disorders results from bliss-52 and bliss-76 trials have shown belimumab to be efficacious in treating patients with sle serious adverse effects noted with belimumab such as progressive multifocal leukoencephalopathy , depression , suicidal tendency , and malignancy are to be watched for . . the baff / april axis plays an important role in the pathogenesis of various autoimmune diseases including sle ; inhibitors of this axis are therefore useful in treatment of these disordersresults from bliss-52 and bliss-76 trials have shown belimumab to be efficacious in treating patients with sleserious adverse effects noted with belimumab such as progressive multifocal leukoencephalopathy , depression , suicidal tendency , and malignancy are to be watched for . the baff / april axis plays an important role in the pathogenesis of various autoimmune diseases including sle ; inhibitors of this axis are therefore useful in treatment of these disorders results from bliss-52 and bliss-76 trials have shown belimumab to be efficacious in treating patients with sle serious adverse effects noted with belimumab such as progressive multifocal leukoencephalopathy , depression , suicidal tendency , and malignancy are to be watched for .

belimumab is the only approved biological agent for the treatment of systemic lupus erythematosus ( sle ) . it is a fully humanized igg1 monoclonal antibody directed against soluble b lymphocyte stimulator ( blys ) . it is indicated as an add - on therapy for the treatment of adult patients with active , autoantibody - positive sle , who are receiving standard therapy . belimumab is generally well - tolerated , common adverse effects include infections , infusion reactions , hypersensitivity , headache , nausea , and fatigue . psychiatric events including suicidal tendency , progressive multifocal leukoencephalopathy and malignancies too have been reported . apart from sle , the drug is also being tried for other autoimmune disorders .

cementoblastoma in the current world health organization classification of odontogenic tumor , is in the category of tumors of mesenchyme and/or odontogenic ectomesenchyme with or without odontogenic epithelium . it generally occurs in young persons , comprises < 1 - 6.2% of all odontogenic tumor and is characterized as being attached to the roots , most frequently associated with first permanent molar . the majority of these tumor are radiopaque , but radiolucent tumor may occur in rare instances . histologically , it presents as a well - circumscribed tumor composed of cementum like tissue surrounded by a fibrous capsule . this was a case report of a 16-year - old male patient who reported to the department of oral medicine , gdc , ahmedabad with the chief complaint of swelling and mild pain in the right side of the jaw since 6 months , with a history of extraction of 46 because of pain before 6 months in a private dental clinic . on clinical examination , there was diffuse bony hard swelling present in the right body of mandible with normal overlying skin . intra - orally , there was diffuse bony hard swelling in 46 region with normal overlying mucosa and expanded buccal and lingual cortical plates [ figure 1 ] . adjacent teeth were immobile and undisplaced . radiological examination revealed a well - defined round radiopacity with radiolucent rim in the right body of mandible [ figure 2 ] . he had pre - extraction intraoral periapical radiography ( iopa ) radiograph of 46 region , which showed a well - defined radiopacity surrounded by radiolucent rim attached to roots of 46 [ figure 3 ] . the typical radiographic presentation of radiopacity attached to root with radiolucent rim suggested of benign cementoblastoma with differential diagnosis of hypercementosis , osteoblastoma , periapical cemental dysplasia and condensing osteitis . histopathologically it showed a well - circumscribed tumor composed of cementum like tissue surrounded by a fibrous capsule [ figure 4 ] . intra - oral view showing diffuse swelling in right lower quadrant with missing 46 and expansion of buccal and lingual cortical plates right lateral oblique of mandible showing well defined radiopacity surrounded by radiolucent zone in body of mandible with missing 46 pre - extraction radiograph showing radiopaque lesion attached to roots of 46 microphotograph showing sheet of cementum like tissue with intervening loose fibrovascular connective tissue stroma the male to female ratio of the prevalence has been reported to be 2.1:1 with a mean age of 20.7 years . benign cementoblastoma is also reported in the maxillary sinus and associated with deciduous and unerupted permanent tooth and multiple teeth . clinical sign and the radiographic appearance of benign cementoblastoma is well - defined radiopacity with radiolucent zone . though it is a benign tumor , but some cases reported in the literature exhibited signs of local aggressiveness and destruction , including bony expansion , erosion of cortical plates , displacement of adjacent teeth , maxillary sinus involvement , invasion of the pulp chamber and root canals and extension to and incorporation of adjacent teeth . expansion , pain and erosion or perforation of bony cortex were seen in a higher percentage of recurrent tumors , but were also seen in non - recurrent tumors . the differential diagnosis for a periapical radiopacity include cementoma , osteoblastoma , periapical cemental dysplasia , condensing osteitis and hypercementosis . histologically , this tumor presents sheets of cementum like tissue , which may contain a large number of reversal lines with active cementoblasts . cementoblastoma and osteoblastoma are essentially identical histologically and the only distinguishing feature is attachment of cementoblastoma to the root of a tooth . histologically , the cementoblast in cementoblastoma may be plump with pleomorphic and hyperchromatic nuclei , however , mitotic figures are not seen in cementoblastoma . in contrast to osteoblastoma , the cementoblastoma is an odontogenic tumor that recapitulates cementum deposition similar to that during root formation in the later stages of odontogenesis . furthermore , the cementoblastoma is continuous with the cemental layer of the apical third of the tooth root and remains separated from bone by a continuation of the periodontal ligament , all of which supports an odontogenic origin . whereas osteoblastoma arises in the medullary cavity of bones . the treatment of choice is complete removal of the lesion with extraction of associated tooth , followed by thorough curettage and peripheral ostectomy . cases have been also reported of endodontically preserving the tooth while surgical removal of benign cementoblastoma is done . we presented a rare case of benign cementoblastoma in mandible and it should be considered in differential diagnosis of bony swellings of mandible . when extraction is attempted in such cases leaving the lesion behind makes the clinical diagnosis difficult . though the patient had pre - extraction records , which helped in formulating the diagnosis .

we present a rare case of radiopaque lesion with radiolucent rim in the right body of mandible , with history of extraction of involved tooth , which made diagnosis confusing unless pre - extraction intraoral periapical radiography radiograph was recovered , finally diagnosed as benign cementoblastoma . it was surgically managed , with no recurrence of the lesion more than 2 years of follow - up . benign cementoblastoma is a rare , benign tumor of odontogenic ectomesenchymal origin , usually associated with roots of first mandibular molar .

the anterior choroidal artery ( achoa ) supplies several important structures including the optic tract , geniculate body , posterior limb of internal capsule , globus pallidus , cerebral peduncle , uncus , amygdala , ventrolateral nucleus of thalamus , and choroid plexus . the vascular injury of achoa may result in severe neurological deficits such as hemiplegia , hemianesthesia , hemianopsia , decreased consciousness , and extrapyramidal symptoms . therefore , complete knowledge about vascular anatomy of achoa is very important before surgical or endovascular manipulations . although several detailed reports about anatomical variations of achoa have been reported , transposition of achoa as to posterior communicating artery ( pcoma ) origin was demonstrated only in 3 reports2 - 4 ) . we report a case with ruptured cerebral aneurysm arising from pcoma , which originated from the internal carotid artery ( ica ) distal to the origin of achoa . a 40-year - old woman presented with sudden onset of headache . computed tomography ( ct ) revealed subarachnoid hemorrhage ( sah ) which was slightly predominant in the left basal and sylvian cisterns . two small aneurysms arose from both distal supraclinoid ica , these were observed on ct angiograms ( fig . cerebral angiography demonstrated a small aneurysm arising from left fetal type of pcoma with suspicion of rupture and the other small aneurysm originating from right pcoma or perforating branch of ica . in this study , left achoa originated from ica proximal to the origin of fetal type of pcoma ( fig . achoa usually arises from the posterolateral wall of ica , a few mm distal to the origin of pcoma , and it provides blood supply to the important neural structures around crural and ambient cisterns . the achoa can have several variations in the course , size , number , branching pattern , and brain region supplied by this artery1 ) . among them , there have been several anomalies related to the origin of achoa including transposition of achoa and pcoma , origin from pcoma , origin from junction of ica and pcoma , and origin from middle cerebral artery4,5 ) . the clinical findings of transposed achoa cases including present one are summarized in table 1 . all of the transpositions of achoa occurred in the left side . among all the cases , our case showed sah due to ruptured aneurysm arising from fetal type of pcoma among multiple cerebral aneurysms founded and achoa originating from ica proximal to pcoma . typically , achoa runs posteromedially within the crural cistern and continues ambient cistern to enter the choroidal fissure . the proximal portion of this artery is cisternal segment and following portion becomes intraventricular segment1 ) . the transposed achoa in the previous cases including ours did not seem to affect the vascular territory or course despite of unusual vascular arrangements . the achoa can be exposed or manipulated in surgical approaches into the pathological lesions around temporal lobe or involving supraclinoid ica . although the achoa aneurysms accounting for 4% of all cerebral aneurysms are not so common , vascular injury of achoa with small caliber supplying the eloquent areas may be critical because of the possibility of significant morbidity and mortality . and , another caution is that achoa do not have significant collateral flows despite a potential anastomosis with posterior choroidal artery3 ) . we tried to preserve pcoma itself and all perforating branches arising from it because of the possibility to compromise the collateral flows of fetal type pcoma . it is notable that careful review of the preoperative angiograms helps to find unexpected vascular anomalies which may impose an additional surgical risk .

the anterior choroidal artery has several kinds of variations . among them , the transposition of anterior choroidal artery and posterior communicating artery origins has been extremely rare . we report a case with cerebral aneurysm arising from posterior communicating artery which origin was distal to the anterior choroidal artery and review the relevant literature .

in recent years , video - assisted intubation devices have flooded theaters , and , to a lesser extent , are also being used in icus . but can such new technology really contribute to making tracheal intubation in the icu less life - threatening , or even more life - saving ? noppens and colleagues , in a study just published in critical care , evaluated the efficacy of the c - mac video laryngoscope for endotracheal intubation in an icu managed by anesthesiologists and compared the results to those obtained during a previous period when conventional direct laryngoscopy was used . in patients with at least one predictor for difficult intubation , poor glottic views ( cormack and lehane grades iii and iv ) were reduced from 38% to 19% , and intubation success on the first attempt increased from 56% with direct laryngoscopy to 79% when the c - mac video laryngoscope was used . even in all patients , the incidence of at least two intubation attempts though this was not statistically significant , halving this incidence may have substantial clinical impact . although the overall intubation success was 100% in either group , successful tube placement in the trachea on the first attempt is crucial in icu patients with poor tolerance to apnea . after implementation of a video laryngoscope , however , intubations were more often performed by less experienced physicians . even if one reason may be that younger physicians are more open to the newer technique , it must be guaranteed that physicians ' experience is not substituted solely by technical equipment . similarly , the use of endoscopic intubation was reduced substantially in the intervention phase ( 4% versus 13% ) , so experience with such devices may decrease , causing problems if video laryngoscopy is not possible or is contraindicated . video laryngoscopy for every patient ? sparing such devices for the really difficult cases ? to answer these questions it needs to be emphasized that all video - assisted and optical intubation devices have different view angles , thus producing different images with particular distortions , and even experts in ' old - fashioned ' airway management need a substantial level of training with a certain device before using it safely and successfully in critical situations and patients . video laryngoscopes , regardless of a particular brand or device , can not be used intuitively - they require expert skills and routines to be tamed and turned into life - saving tools . tube placement can turn out to be difficult and even impossible , despite the excellent glottic exposure video laryngoscopes most often provide . the reason is that the operator 's eye is no longer in a straight - optical and anatomical - line with the larynx , but at the tip of the laryngoscope blade . optical and anatomical axes divert , and tube placement is sometimes difficult or fails . becoming alert to these potential problems associated with video laryngoscopy and constantly improving one 's technical skills to overcome such pitfalls can only be achieved when such devices are used on a daily basis and in routine patients . apart from the primary aim of the study - evaluating video laryngoscopy - it needs to be highlighted that noppens and colleagues routinely used capnography to verify correct tube placement after intubation . capnography has become a mandatory requirement in any operating room , but its use in the icu seems still to be somewhat dowdy . as of 2010 , only 25% of icus in the uk and the republic of ireland used capnography to confirm correct tube placement after intubation . such refusal may produce catastrophic outcomes : data collected from all uk national health service hospitals over a period of one year in 2008/2009 revealed that 61% of adverse events during airway management in the icu resulted in death or permanent brain damage . in 74% of such fatal cases no capnography was used ! doubtless , video laryngoscopy is a helpful tool in the hands of experienced physicians , but should not be considered a bailout procedure for beginners in airway management . and as attractive and simple video laryngoscopy appears , we must always remind ourselves to maintain our skills in endoscopic intubation . both authors receive continuous material support for clinical studies from karl storz gmbh & co. kg , the manufacturer of the c - mac video laryngoscope .

the c - mac video laryngoscope substantially reduced poor glottic views and increased intubation success in icu patients with at least one predictor for difficult intubation . however , all video - assisted and optical intubation devices have different view angles , thus producing different images with particular distortion , and even experts in ' old - fashioned ' airway management need a substantial level of training with a certain device before using it safely and successfully in critical situations and patients . video laryngoscopes , regardless of a particular brand or device , can not be used intuitively - they require expert skills and routines to be turned into a life - saving airway management tool .

septic shock is consistently the most common causative factor identified for acute kidney injury ( aki ) in critical illness , and has been associated with nearly 50% of cases internationally . despite advances in our understanding of the pathophysiology of septic aki , treatment aimed at reversing or preventing septic aki remains primarily based on supportive haemodynamic management . in the previous issue of critical care , legrand and colleagues examine the association between haemodynamic targets of resuscitation ( cardiac output , mean or diastolic blood pressure , central venous pressure ( cvp ) and central venous oxygen saturation ) and development or persistence of aki in a single centre study of patients with septic shock admitted to a surgical icu . the authors found that only higher cvp and low diastolic blood pressure were associated with increased risk of development of new aki , or persistence of renal dysfunction present at icu admission . in these patients importantly , the association between cvp and aki remained when potentially confounding effects of positive fluid balance and higher positive end - expiratory pressure were accounted for in a multivariable analysis . so , a 5 mmhg increase in cvp predicted 2.7-fold odds of new or persistent aki . furthermore , when the association between different levels of mean cvp and aki was examined in a non - parametric logistic regression , there was a trend for higher cvp to be associated with worse renal outcome for all levels of cvp from 4 mmhg upward , so that a cvp of 15 mmhg was associated with an approximately 80% risk of new or persistent aki , compared to approximately 30% at a cvp of 6 mmhg . these findings are important because current surviving sepsis campaign guidelines recommend fluid resuscitation of patients with sepsis - induced tissue hypoperfusion to target a cvp of 8 to 12 mmhg ( or 12 to 15 mmhg in mechanically ventilated patients ) within 6 hours of presentation . in patients with sepsis - induced hypotension the rationale for cvp targeted fluid resuscitation is to ensure ' adequate cardiac preload and hence maintain cardiac output and organ perfusion . however , absolute levels or changes in cvp poorly predict cardiovascular response as sepsis - induced hypotension is multi - factorial , related to changes in myocardial performance , vascular tone , regional blood flow distribution , venous reservoir capacity and capillary permeability . in contrast , elevated cvp will cause a direct and predictable increase of renal venous pressure that , experimentally , has been associated with elevated renal interstitial and intra - tubular pressure . resultant renal venous congestion can reduce renal blood flow ( lower trans - renal pressure gradient ) and decrease the pressure gradient for ultrafiltration ( higher intra - tubular pressure ) . in septic shock , . aggressive fluid resuscitation beyond reversal of hypovolaemia to arbitrary cvp targets could result in increased venous congestion without substantial benefit to forward renal perfusion , thus predisposing to renal dysfunction . the association between elevated cvp and renal dysfunction has been made previously in the setting of chronic cardiac failure . now legrand and colleagues have provided evidence that such an association is observed in septic shock and its treatment . their findings are also in accord with secondary analysis of the vasopressin in septic shock trial , which reported that a more positive fluid balance and elevated cvp were associated with increased mortality in patients with septic shock even when accounting for differences in baseline illness severity and demographics . although these observational findings are hypothesis - generating rather than confirmatory , they add weight to the literature suggesting the adverse effects of fluid overload in critical illness and aki in particular , effects that may be mediated in part by venous congestion causing impaired organ function . it is now important to determine whether uncritical pursuit of cvp targets such as those advocated by the surviving sepsis campaign might , in some patients , add insult to sepsis - induced organ injury . prospective data are required to evaluate the true contribution of raised venous pressure to renal and other organ dysfunction and to determine better endpoints for fluid resuscitation in sepsis . rajkumar rajendram bsc mbbs mrcp frca fficm , specialty registrar in anaesthesia , royal free hospital , london . john r prowle ma msc md mrcp fficm , consultant in renal medicine and intensive care , the royal london hospital , london .

in critical illness , septic shock is a contributing factor in nearly half of all cases of acute kidney injury ( aki ) . traditional approaches to prevention of organ dysfunction in early sepsis have focused on prevention of hypoperfusion by optimisation of systemic haemodynamics , primarily by fluid resuscitation . fluid administration to a target central venous pressure ( cvp ) of 8 to 12 mmhg ( 12 to 15 mmhg in mechanically ventilated patients ) is currently recommended for the early resuscitation of septic shock . however , in the previous issue of critical care , legrand and colleagues report that higher cvp in the first 24 hours of icu admission with septic shock was associated with increased risk for development or persistence of aki over the next 5 days . this study highlights a potential association between venous congestion and the development of septic aki , suggesting that cvp - targeted fluid resuscitation in septic shock might not be beneficial for renal function .

one hundred forty - seven clinical isolates of m. tuberculosis were obtained from the molecular mycobacteriology laboratory , department of microbiology , faculty of medicine , siriraj hospital , mahidol university , thailand , and the t-2 project from 1997 to 2001 ( table ) . these strains were isolated from 74 cerebrospinal fluid ( csf ) samples and 73 sputum samples from 147 different patients . dna from these isolates was isolated by an enzymatic method and submitted for genotyping by performing the is6110 restriction fragment length polymorphism with the standard method ( 12 ) and for sequencing the pks15/1 region ( 8) . * csf , cerebrospinal fluid . using the genotyping results , we categorized m. tuberculosis isolates into beijing , single - banded , few - banded ( 25 bands ) , nonthaburi , and heterogeneous with > 5 bands ( table and figure 1 ) , as recently reported ( 13,14 ) . all m. tuberculosis genotypes were sequenced around the junction of pks15 and pks1 ( corresponding to the m. tuberculosis h37rv sequence ) to determine whether they contained an intact pks15/1 or separated pks15 and pks1 . unexpectedly , the results showed that the 7-bp insertion of pks15 that causes a frameshift mutation resulting in an intact pks15/1 was found in most strains of all genotypes , except the heterogeneous group with > 5 bands ( table and figure 2 ) . r indicates the m. tuberculosis mt 14323 strain used as the positive control for is6110 typing . sequence alignment of region corresponding to the 3 portion of pks15 and 5 portion of pks1 in various mycobacterium tuberculosis genotypes . b ) m. tuberculosis strains isolated from sputum . letters in brackets refer to is6110 restriction fragment length polymorphism patterns : bj , beijing ; sb , single banded ; fb , 25 bands ; nb , nonthaburi ; h , heterogeneous . the 7-bp insertion is shown in boldface , and the start codon of the pks1 gene is underlined . the intact pks15/1 has been shown to be responsible for the production of phenolic glycolipids and is seemingly found in m. tuberculosis w - beijing family , but it was not found in m. tuberculosis cdc1551 and h37rv ( 8) . previous studies suggested that pgls produced by the m. tuberculosis w - beijing family were associated with the hypervirulent phenotype by inhibiting the innate immune response ( 6,7 ) . the intact pks15/1 has also been shown to be nonpolymorphic in the w - beijing family ; it was found in all 102 w - beijing strains tested ( 15 ) . from this observation , we hypothesized that if the ability to produce pgls is among the factors that make this family more virulent than others , the intact pks15/1 should be absent in strains other than the w - beijing family . our results showed that the 7-bp insertion of the pks15/1 was not only present in the w - beijing family but also in other m. tuberculosis genotypes . although almost all beijing strains contain the intact pks15/1 ( 97% ) , 38.5%100% of strains of other genotypes also contain it . these strains could , therefore , produce pgls and cause both pulmonary and disseminated diseases as the w - beijing strains do . our results showed no significant difference in the percentage of m. tuberculosis isolates with an intact pks15/1 gene between csf isolates ( 65 [ 87.8% ] of 74 ) and sputum isolates ( 62 [ 84.9% ] of 73 ) . the hypothesis that the hypervirulence of the w - beijing family is solely attributable to pks15/1 is still inconclusive . this family may have only recently been transmitted globally and may have had more chances to cause infections and disease than other families . although pgls are involved in the hypervirulence of the pgl - producing strains , they are not a unique characteristic of the w - beijing family . if w - beijing strains are more virulent than others , other virulence determinants besides pgls must be responsible for the hypervirulent phenotype .

to determine whether intact pks15/1 is unique to the w - beijing family , we investigated 147 mycobacterium tuberculosis strains with different is6110 genotypes . intact pks15/1 was found in 87.8% of cerebrospinal fluid and 84.9% of sputum isolates . it was found not only in w - beijing strains ( 97% ) but also in other genotypes ( 38.5%100% ) .

cholelithiasis presented as chronic right back pain in the case discussed in this report . according to the clinical practice guidelines , gallbladder lithiasis is a common condition . around two - thirds of the cases are asymptomatic , with the most frequent clinical presentation being the presence of attacks of acute abdominal pain . however , chronic right back pain persisting for months as a main symptom is uncommon . this report presents a patient with right back and subscapular pain for 9 months , demonstrating both biliary lithiasis and chronic cholecystitis . it is important to take into account the atypical manifestations of a prevalent illness as well as the limitations of the diagnostic tests , since the presence of gallstones can easily be missed or misinterpreted and delay in diagnosis may lead to complications which could worsen the prognosis of the patient . a 47-year - old male presented with a history of chronic neck pain that had started 10 years back . he underwent a magnetic resonance ( mr ) which showed cervical canal stenosis of degenerative etiology and also had an episode to self - limited abdominal spasmodic pain that appeared 10 years ago , considered as being functional by the gastroenterologist . abdominal ultrasonography was performed twice , and both times it was normal . one and a half years ago , the patient had right subscapular and paralumbar back pain at the t10l2 level for 9 months . he described it as a permanent pain , which persisted throughout the day , with very intense exacerbations , especially in the evening and at night . these exacerbations usually lasted for 4 - 6 h , worsening on inhalation and sometimes radiating to subcostal and right hemithorax . the only feature of note on physical examination was the palpation of painful spinal processes from t10 to l2 . blood analysis showed the level of gamma - glutamyl traspeptidase to be 79 u / l , while all the other laboratory parameters were normal . abdominal computed tomography ( ct ) showed an extensive hepatic steatosis and a moderately relaxed gallbladder , with no stones observed within thin walls [ figure 1 ] . abdominal ct : gallbladder without gallstones despite the regular use of analgesics and the rehabilitation treatment that followed , the patient presented a torpid evolution . due to the pain in the right paralumbar area , an abdominal ultrasonography was performed , which showed moderate hepatic steatosis and a gallbladder with multiple inner calculi [ figure 2 ] . on diagnosis of cholelithiasis , a cholecystectomy was carried out , which revealed a gallbladder with a large number of yellow lithiasic structures , thereby confirming an anatomopathological diagnosis of chronic cholecystitis [ figure 3 ] . the previous pain completely disappeared after surgery and the patient remained asymptomatic 7 months later . back pain affects 80% of adults at some point of their life and occurs at all ages . the first objective is to rule out a visceral disorder or a serious potentially life - threatening condition , which occurs in 2.7% of the cases . back pain may be the manifestation of thoracic involvement as in esophageal disease , pleurisy , aortic aneurysm , or coronary heart disease , or an abdominal process , such as gastrointestinal ulcer , gastric cancer , pancreatic cancer , pancreatitis , or biliary pathology . only one - third of the cases of gallstones are symptomatic and give an indication for surgery . the most frequent symptomatic manifestation is episodic upper abdominal pain called biliary colic , with complications such as cholecystitis , acute pancreatitis , cholangitis , or choledocholithiasis being less frequent . characteristically , this pain is severe and located in the epigastrium and/or the right upper quadrant . the pain may radiate to the upper back or the right scapula in 60% of the cases and might be associated with nausea or vomiting , usually occurring in the late evening or at night , in 80% of the cases . one study carried out to characterize the presentation in cholelithiasis reported that all patients described pain in the right upper quadrant of the abdomen , including the epigastrium . the pain was located in the right subcostal area ( 20% ) and epigastrium ( 14% ) , which radiated to the back in 63% . in fact , chronic cholecystitis is thought to be a result of a delay in diagnosis . the test of choice to diagnose cholelithiasis is transabdominal ultrasound , with a sensitivity and specificity greater than 95% for detecting gallstones larger than 4 mm . abdominal ct scan has a very low sensitivity and gallstones may be visible due to most being isodense . microlithiasis ( stones <3 mm in diameter ) is not detectable by transabdominal ultrasonography ; however , it may cause complications such as acute pancreatitis . in some patients the diagnosis can be confirmed by the detection of biliary crystals by microscopic examination of bile collected from the duodenum . in patients with a clinical history of biliary colic with negative transabdominal ultrasonography , endoscopic ultrasonography is an important diagnostic tool , since it can detect microlithiasis in a proportion of patients ranging from 41 to 78% . patients with pain and a negative abdominal ultrasound show an endoscopic ultrasound and/or a positive analysis of the bile . most of these patients show positive results on cholecystectomy , with resolution of abdominal pain and improvement in their quality of life . non - vertebral causes such as the presence of gallstones should be considered in patients with back pain . physicians should be aware of atypical manifestations of common diseases , so as to avoid diagnostic delays . gallbladder disorders should be taken into account in patients with dull , undefined right back pain , despite a normal physical examination . abdominal ultrasound should be chosen as the first diagnostic procedure when considering gallstones , since ct scans have a very low sensitivity for their detection . in a patient with a typical clinical report of biliary colic and a negative abdominal ultrasound , this was the case of our patient in whom the abdominal ultrasonography was normal 5 years earlier , thereby making correct differential diagnosis crucial .

chronic right back pain is a symptom in both biliary lithiasis and chronic cholecystitis . ten percent of the population in the world suffers from biliary lithiasis . only 20% are symptomatic . the first diagnostic test of choice is an abdominal ultrasound . when a suggestive clinical sign of biliary colic with negative abdominal ultrasound is identified , we should consider the option of carrying out an endoscopic ultrasound in order to rule out microlithiasis . the case discussed in the report presented with chronic right back pain , which is an atypical manifestation of biliary lithiasis and chronic cholecystitis . it is important to know about the atypical manifestations of the prevalent illnesses as well as the limits of the diagnostic tests , in order to avoid diagnostic delays which may cause complications that could worsen a patient 's prognosis . this case should contribute to the medical knowledge and must have educational value or highlight the need for a change in clinical practice , especially in primary care .

spending hours in plastic surgery opd in a government medical college and dealing with the kind of queries posed to us by patients and often having to see delayed and neglected patients made us question and wonder on various views that people have of plastic surgery . most of the patients had no idea of the range of our services and had reached us after having visited a series of generalists and eventually being referred to us . hence , we undertook this study with an aim to ascertain current awareness among the medical students and residents ( the future practitioners ) about the surgical procedures that a plastic surgeon performs . this study was conducted in four medical colleges chosen from four zones of india : king george medical college ( kgmu ) , lucknow ; all india institute of medical sciences ( aiims ) , new delhi ; king edward memorial ( kem ) hospital , mumbai ; and stanley medical college ( smc ) , chennai . the questionnaire had 35 surgical situations , whereby the respondents were to refer a patient to any department or departments where a particular problem can be managed . a total of 2000 questionnaires were distributed and 1552 responses were received . there were 440 respondents from kgmu , 312 from aiims , 418 from kem , and 382 from smc . the respondents were categorized into four groups : mbbs students , interns , junior residents , and senior residents . the y - axis reveals percentage of referral to plastic surgeons and x - axis shows the group studied . facial fractures , craniofacial surgery , temporomandibular joint dysfunction cleft lip palate , burns liposuction , rhinoplasty , hair grafting wound cover , pressure ulcer , scalp avulsion peripheral nerve injury , hand anomalies and trauma it was observed [ table 1 ] that there was a good deal of awareness ( > 60% ) regarding the role of plastic surgeons in dealing with burn injuries , rhinoplasty , liposuction , hair grafting and cleft lip and palate . there was a moderate degree of awareness ( 4060% ) regarding wound management and pressure ulcer , being managed by plastic surgeons . only 1530% respondents were aware that craniofacial anomalies , congenital hand anomalies , facial fractures , scalp avulsion , hand injuries and peripheral nerve injuries are reconstructed by us . there was a poor knowledge ( < 15% ) about brachial plexus surgery and temporomandibular joint ( tmj ) ankylosis being managed by plastic surgeons . table 1 is an ascending order depiction of the awareness regarding treatment of various conditions by plastic surgeons . the findings of our study are similar to those of previous studies on different population and socioeconomic groups which show the public to be poorly informed about plastic surgery . in his paper perception of plastic surgery in the society , the author concludes that indian public associates plastic surgeons with cosmetic and burn surgeries . the knowledge is also limited among medical students and nurses and the source of their knowledge is magazines and newspapers . in their study perception and reality a study of public and professional perceptions of plastic surgery conducted in the british public population , medical students and general practitioners , plastic surgery was associated with reconstruction for trauma and cancer and procedures with a strong aesthetic element by all the three groups and hand was associated with orthopaedic surgeons . in a study conducted in philadelphia in the usa , among consecutive patients visiting a primary care facility , medical students , and primary care physicians , the authors demonstrate that public perception of the plastic and reconstructive surgeon is limited and grossly underestimates this specialty . although the plastic surgeons are associated with reconstructive surgery , they are not necessarily identified as primary surgeons for procedures fundamental to this specialty . many people who do not come into contact with plastic surgery in their hospitals are under the popular misconception that plastic surgeons spend their time fixing noses and altering the size of breasts . it is not really surgery since it can be done in the office and is not covered by insurance . the media is also little informed about the versatility of plastic surgeons and ends up building false expectation in public and wrong images of plastic surgeons as scavengers for money . in their paper plastic surgery in the cinema , the authors concluded that films usually incorporate cosmetic surgery and burns in the screenplay . also , plastic surgeons are depicted to be living in affluent localities , making a lot of money . patients visiting plastic surgeons are also shown to be ultra rich.[710 ] this often deters many patients from primarily consulting us . patients visiting the department late in the course of disease process have often revealed that they had feared to visit our department fearing the expenditure they would incur . although the findings of this paper may not surprise practicing plastic surgeons , it does quantify a reality we all share that various works of our specialty are not easily identified by public and medical professionals . we have made an attempt to reason out the cause for the same in our country and suggest remedial measures . also , the commonly followed textbook of general surgery in the undergraduate curriculum discusses mostly burns and cleft lip and palate under plastic surgery section . to add to these , the lectures devoted to plastic surgical topics are few and often delivered by general surgeons who are less equipped to spread awareness among medicos . hence , plastic surgery department must be developed in all colleges , and medical students and surgical residents must be rotated for at least a week or two as part of their training programme . this is a long - term goal , and till it is in place , a few committed and reverent teachers in the society may come forward to deliver planned lectures to mbbs students in colleges where the department is lacking . this can be put forward by the association of plastic surgeons of india ( apsi ) as part of expansion of plastic surgery . we should also develop methods to educate the general practitioners because they are the people who reach out to public at large . our primary aim then should be to educate and sensitise the media regarding the role of plastic surgeon even in day - to - day life of a common man . the contribution of prominent members of plastic surgery society will be more than sufficient to open the eyes of media regarding various subspecialities . apsi may invite media personnel on an awareness trip to well - established centres to see a variety of procedures and the necessity of plastic surgeon in trauma centres , burn care , as reconstructive surgeons in trauma , congenital and oncological surgeries besides their role as an aesthetic surgeon . we should encourage media personnel to project a real picture to the general public and publicise the various conditions in which plastic surgeons play a role . at the village level , this may be done by plays and distributing pamphlets and posters showing preoperative and postoperative photos . regular monitoring of progress in the endeavour to spread awareness can be conducted yearly , and necessary intervention may be added as and when required welcoming newer ideas for the same . our study clearly shows that knowledge about the field of plastic surgery is highly lacking and lot needs to be done to spread awareness . the best way to describe plastic surgeons is that we are a problem solving specialty . the amazing developments made in our field must be made available and accessible to the mass and a collective part played by plastic surgeons can go a long way in making our specialty really known to even layman as to it being a necessity rather than a privilege .

the field of plastic surgery , while being famous for aesthetic surgery , also includes craniofacial surgery , hand surgery , burn surgery , microsurgery , reconstructive plastic surgery and paediatric plastic surgery . the magnanimous progress in these areas , though a hot topic in conferences , remains cryptic to the layman and also to generalists who are and will remain to be the most important referral source of these patients.[1 ] hence , it becomes the duty of plastic surgeons themselves to spread awareness regarding their chosen field of endeavour .

restoration of structure and function using autologous free fat grafts has always been a challenge . the advent and refinement of liposuction and lipoinjection techniques , abundant donor - tissue availability , and relative ease of harvesting have made autologous fat an attractive material for use as soft - tissue filler . fat is harvested using either ( 1 ) syringe aspiration or ( 2 ) liposuction aspiration,(1 ) performed with a liposuction pump ( byron medical , tucson , ariz . ) . a standard coleman aspirator cannula ( byron medical ) with a curettage tip ( 500 mm mercury).(2 ) syringe aspiration is tedious if the amount of fat required is substantial . when the liposuction aspiration using a liposuction pump is performed the whole suction system , and the container needs to be kept sterile . ( figure 1 ) we have devised a novel method of lipoaspiration to overcome these problems . the above picture helps us to compare the three modalities of lipoaspiration and also helps us to note the advantage of our technique materials required 20 - 50 cc disposable syringe , a piece of 2 2 cm polypropylene mesh , lipoaspiration suction pump ( figure 2a ) . ( a ) materials required for the procedure , ( b ) connected syringe mesh system , ( c ) close - up view of the mesh in liposuction aspiration , a suction pump is used . in our technique , a piece of polypropylene mesh is kept at one end of the lipoaspiration syringe , which is then connected to the suction pump . ( figure 2b & 2c ) as one syringe fills , it is replaced by another one until the required amount of fat is obtained . these syringes are then centrifuged and the emulsified fat thus obtained is used for grafting ( video 1 ) . by using a polypropylene mesh in our technique , we are able to separate the transfusate from the harvested fat during harvesting itself in our experience , the fat graft obtained by this method is found to be concentrated and has fewer contaminants . this method also has the added advantage of having a short processing time , from harvesting to grafting . fat grafting remains unpredictable , and various anecdotal harvesting and preparation techniques have been advocated . our method is a more economical and an easier way to extract sterile emulsified fat . as it is a simple method , it can be practiced even in day care centers . hence , we recommend our technique as a reliable and routine method for extracting sterile emulsified fat in an economical way .

background : successful restoration of structure and function using autologous free fat grafts has remained elusive . review of literature shows that various harvesting and preparation techniques have been suggested . the goal of these techniques is to obtain greater adipocyte cell survival and consequently more reliable clinical results.materials and methods : in our technique , a piece of mesh is kept at one end of the lipoaspiration syringe , which is then connected to the suction pump . as one syringe fills , it is replaced by another one until the required amount of fat is obtained.results:by using a polypropylene mesh in our technique , we can separate the transfusate from the harvested fat graft during harvesting itself . the fat graft thus obtained is dense and concentrated , with fewer impurities.conclusion:hence , we recommend our technique as a reliable method for extracting sterile emulsified fat in an economical way .

turner syndrome ( ts ) is caused by partial or complete monosomy of the x chromosome . only a few cases of ts with ischemic stroke have been reported [ 2 , 3 , 4 , 5 ] . in previously reported cases , various arteriopathies affecting the cerebral arteries such as fibromuscular dysplasia , hypoplasia , moyamoya syndrome , and , we report a case of ts with multiple embolic infarcts caused by a cryptogenic embolism . a 28-year - old woman with ts was referred to our hospital because of abnormal findings on brain magnetic resonance imaging ( mri ) . she was diagnosed with ts [ karyotype : 45 , x/46 , x , + mar ] at the age of 16 years due to amenorrhea . she denied alcohol , tobacco , or drug use and had no family history of neurologic disorders . the patient was afebrile with a blood pressure of 180/130 mm hg and a heart rate of 89 beats / min . she was obese with a body mass index of 27.3 kg / m ( 160 cm height , 70 kg weight ) . she visited hospital due to sudden - onset severe headache with high blood pressure ( 240/140 mm hg ) . she underwent brain computed tomography and mri at the referring hospital for differential diagnosis of thunderclap headache including hypertensive intracranial hemorrhage , subarachnoid hemorrhage , arterial dissection , or reversible cerebral vasoconstriction syndrome . brain computed tomography was unremarkable , but diffusion - weighted imaging showed restricted diffusion in multiple lesions involving multiple vascular territories ( fig 1a ) . cerebral magnetic resonance angiography ( mra ) ( fig 1b ) and carotid sonography demonstrated no extracranial or intracranial arterial stenosis . laboratory tests revealed diabetes mellitus ( fasting plasma glucose 219 mg / dl , hemoglobin a1c 11.0% ) and hyperlipidemia ( low - density lipoprotein 189 mg / dl ) . transthoracic echocardiography revealed normal valvular structure and function with a normal left ventricular ejection fraction . patent foramen ovale , atrial septal aneurysm , or any aortic pathologies including aortic atheroma were not detected in transesophageal echocardiography . the results of the hypercoagulability panel , vasculitis panel , and cerebrospinal fluid examination were all negative . aspirin was administered , and medications for hypertension , hyperlipidemia , and diabetes were also started . within few hours after blood pressure control , the patient was discharged from the hospital without any symptoms of stroke , and recurrence was not observed in the 2 years after discharge . cerebrovascular arteriopathies such as fibromuscular dysplasia , congenital hypoplasia , moyamoya syndrome , and premature atherosclerosis have been shown to be causes of ischemic stroke in previous case reports . in this case furthermore , our patient showed multiple lesions in different vascular territories that were strongly suggestive of embolic stroke . atrial fibrillation ( af ) could also be considered a potential source of embolic stroke in ts , as p - wave dispersion , a potential substrate for af , is increased in ts . the classical risk factors for developing af include hypertension , diabetes mellitus , and valvular disease , and these are more common in ts than in the general population . however , we could not completely exclude the possibility of paroxysmal af , as holter monitoring has a low yield for af detection ( 35% ) . other possible cardio - aortic sources of embolism include intracardiac shunts , thrombi , calcifications / vegetations in the mitral valve , or aortic atheroma plaque . we sought to identify possible cardio - aortic sources on transthoracic echocardiography , transesophageal echocardiography , and transcranial doppler shunt test ; however , all tests were negative . some case reports have described deep venous thrombosis and portal vein thrombosis in ts [ 11 , 12 ] . these factors include fibrinogen , d - dimer , factor viii , von willebrand factor , and proteins c and s . we also considered the possibility of hypercoagulability as a cause of stroke in this case ; however , levels of d - dimer , fibrinogen , protein c and s were normal . the embolic source of stroke in our patient remains unclear ; however , this is the first report of a ts patient with an embolic stroke pattern . our case suggests another possible mechanism of stroke in ts that is different from previous case reports . ischemic stroke in ts could be due to embolism as well as various cerebral arteriopathies . further investigation is needed to more fully understand the various mechanisms of ischemic stroke in ts . ischemic stroke in ts could be due to embolism as well as various cerebral arteriopathies , as documented in previous reports . further studies are needed to determine the extent of the underlying mechanisms of ischemic stroke in ts . this case report has been approved by the institutional review board of the inha university hospital .

only a few cases of turner syndrome ( ts ) with ischemic stroke have been reported . various arteriopathies of the cerebral arteries , including fibromuscular dysplasia , congenital hypoplasia , moyamoya syndrome , and premature atherosclerosis have been assumed to be the cause of ischemic stroke in ts . there has been no case report of a ts patient presenting with an embolic stroke pattern without any cerebral arteriopathy . a 28-year - old woman with ts was referred to our hospital because of abnormal brain magnetic resonance imaging ( mri ) findings . she underwent brain mri at the referring hospital because she experienced sudden - onset diffuse headache . diffusion - weighted imaging revealed multiple acute embolic infarcts in different vascular territories . intracranial and extracranial arterial disease was not detected on cerebral magnetic resonance angiography and carotid sonography . embolic source workups , including transthoracic and transesophageal echocardiography , holter monitoring , and transcranial doppler shunt study , were all negative . hypercoagulability and vasculitis panels were also negative . our patient was diagnosed with cryptogenic embolic stroke . this is the first report of a ts patient with an embolic stroke pattern . our case shows that ischemic stroke in ts could be due to embolism as well as the various cerebral arteriopathies documented in previous reports .

we wish to report some corrections to our study , none of which alters the interpretation of the data or the conclusions drawn . after publication , we noticed that one of the micro - array hybridizations ( on sample nb11 ) was performed on the same patient 's material as another hybridization ( sample nb4 ; see table 1 ; a corrected version of table 5 ) . as this error leads to an incorrect subclassification of the patients into the ' favourable ' and ' unfavourable ' neuroblastoma subgroups , we would like to exclude this data point from the differential expression analysis of favorable versus unfavorable neuroblastoma given under the heading ' differential expression analysis of favorable and unfavorable neuroblastoma ' in the results section of . careful reanalysis after exclusion of nb11 did not lead to important changes in the generated gene lists and conclusions ; the changes are given in the corrected paragraph and table 2 ( a corrected version of table 4 ) , and the additional data files 1 and 2 ( corrected versions of additional data files 2 and 3 ) available online with this article . we also noticed that sample nb1 is stage 1 instead of stage 4s and that sample nb2 was not localized to the adrenals ( see table 1 ) . so far , most published microarray studies on neuroblastomas mainly compared favorable with unfavorable neuroblastomas in order to identify prognostic markers or pathways that are involved in these clearly different neuroblastoma tumor types . in order to add value to such an analysis , we contrasted similar differentially expressed gene lists with the normal neuroblast expression profile ( additional data file 1 ) . in a first step , we compared the differentially expressed genes between these two tumor types with published prognostic gene lists . we found that 23 of the 193 genes on our list were previously reported , including the well established markers mycn , ntrk1 , and cd44 ( see nbgs analysis in additional data file 2 ) . subsequently , we looked for the corresponding gene expression levels of the differentially expressed genes in the normal counterpart cells , aiming to select neuroblastoma candidate genes . of the 100 genes that are more highly expressed in favorable tumors ( compared to unfavorable ) 41 also have a significant differential expression ( either higher or lower ) compared to neuroblasts , whereas 43 of the 93 genes that are more highly expressed in unfavorable tumors exhibit differential expression compared to the neuroblasts ( table 2 ) . from this analysis , a few putative positional tumor suppressor candidates emerge : cdc42 on 1p36 , cacna2d3 on 3p21 and dlk1 on 14q . the latter two genes are of particular interest because they are highly expressed in neuroblasts and favorable neuroblastomas and their expression is significantly lower in unfavorable neuroblastomas . among the genes that are more highly expressed in unfavorable neurob - lastomas than in favorable ones and neuroblasts , the proven oncogenic transcription factor mycn emerges ( and putative downstream genes kifap3 , ophn1 , rgs7 , ascl1 , odc1 , twist1 and tyms , according to nbgs ) , as well as several other genes that have been identified or studied in the context of neuroblastoma such as alk and prame , and positional candidates on 17q including birc5 and rnu2 . so far , most published microarray studies on neuroblastomas mainly compared favorable with unfavorable neuroblastomas in order to identify prognostic markers or pathways that are involved in these clearly different neuroblastoma tumor types . in order to add value to such an analysis , we contrasted similar differentially expressed gene lists with the normal neuroblast expression profile ( additional data file 1 ) . in a first step , we compared the differentially expressed genes between these two tumor types with published prognostic gene lists . we found that 23 of the 193 genes on our list were previously reported , including the well established markers mycn , ntrk1 , and cd44 ( see nbgs analysis in additional data file 2 ) . subsequently , we looked for the corresponding gene expression levels of the differentially expressed genes in the normal counterpart cells , aiming to select neuroblastoma candidate genes . of the 100 genes that are more highly expressed in favorable tumors ( compared to unfavorable ) 41 also have a significant differential expression ( either higher or lower ) compared to neuroblasts , whereas 43 of the 93 genes that are more highly expressed in unfavorable tumors exhibit differential expression compared to the neuroblasts ( table 2 ) . from this analysis , a few putative positional tumor suppressor candidates emerge : cdc42 on 1p36 , cacna2d3 on 3p21 and dlk1 on 14q . the latter two genes are of particular interest because they are highly expressed in neuroblasts and favorable neuroblastomas and their expression is significantly lower in unfavorable neuroblastomas . among the genes that are more highly expressed in unfavorable neurob - lastomas than in favorable ones and neuroblasts , the proven oncogenic transcription factor mycn emerges ( and putative downstream genes kifap3 , ophn1 , rgs7 , ascl1 , odc1 , twist1 and tyms , according to nbgs ) , as well as several other genes that have been identified or studied in the context of neuroblastoma such as alk and prame , and positional candidates on 17q including birc5 and rnu2 . additional data files 1 and 2 containing the corrected data available online with this article . corrected version of additional data file 2 . corrected version of additional data file 3 . clinical and genetic data of carefully selected neuroblastoma samples that were included in this study samples were subdivided into favorable or unfavorable type based on mycn amplification , ploidy and age at diagnosis . nd , not determined or unknown . genes that are differentially expressed in favorable vs unfavorable neuroblastoma genes that are differentially expressed compared with neuroblasts among the differentially expressed genes in favorable neuroblastoma ( nb ) vs unfavorable nb , with an indication of the number of neuroblastoma microarray studies in which these genes were found through nbgs analysis .

a correction to human fetal neuroblast and neuroblastoma transcriptome analysis confirms neuroblast origin and highlights neuroblastoma candidate genes by k de preter , j vandesompele , p heimann , n yigit , s beckman , a schramm , a eggert , rl stallings , y benoit , m renard , a de paepe , g laureys , s phlman and f speleman . genome biology 2006 7:r84

pulmonary arteriovenous malformation ( pavm ) , first described in 1897 by churton , is characterized by abnormal communications between pulmonary veins and arteries , and is known to disturb the filtering action of pulmonary capillaries , causing thromboembolic event in systemic circulation . most of the pavms have no symptoms , and are detected as abnormal shadow in the chest . conclusive diagnosis is generally made by means of pulmonary arteriography or three - dimensional computed tomography ( 3d - ct ) angiography . while hypervascular lesions can mimic pavm , we observed a rare case of a lung granuloma mimicking pavm , and performed video - assisted thoracic surgery ( vats ) . a 76-year - old woman , who was otherwise symptom - free , was admitted to our hospital because an abnormal shadow was detected in the left lung field on her chest x - ray . chest computed tomography ( ct ) revealed a 20 mm 14 mm nodule with well - defined margins and smooth contours in the left upper segment ( fig . an enhanced solitary lung nodule , connected with linear structures suggestive of feeding artery and drainage vein , was revealed by contrast - enhanced 3d - ct ( fig . arterial blood gas analysis showed hypoxemia , with arterial oxygen pressure ( pao2 ) 56 mmhg in room air . first , the patient was placed in the right lateral decubitus position , and the left lung was deflated . intraoperatively , elastic hard nodule was palpable in the left upper segment and bruit was not convincing . the nodule was nontortuous in shape , covered with the visceral pleura , and neither the feeding artery nor the drainage vein was detected . histopathological findings revealed multiple foci of coagulative necrosis surrounded by epithelioid cell granuloma containing langhans - type multinucleated giant cells , involving the medium - sized blood vessels in the pulmonary parenchyma . the lung granuloma was difficult to be preoperatively distinguished from pavm , because hypervascular lesion such , as inflammatory changes can present as strongly enhanced nodules after injection of contrast material . the patient had an uneventful postoperative course and was discharged 14 days after the operation . causes of acquired or secondary pavm include chest trauma , thoracic surgery , hepatic cirrhosis , infections ( actinomycosis , schistomiasis ) , metastatic carcinoma and systemic disease . twenty eight percent of cases are considered to have no symptoms . it was reported that when a single isolated pulmonary arteriovenous malformation is 2 cm or smaller , no symptoms appear . generally , when pavm is 2 cm or more , symptoms such as breathing difficulty , cyanosis , hypoxemia , finger clubbing and polycythemia occur , and the rate of occurrence of severe complications , such as rupture of the malformation , hemoptysis , cerebral infarction and cerebral abscess , is approximately 30% . the patient in the present case was asymptomatic and had no history of chest trauma , surgery , infection or systemic disease . abnormal vascular structures , such as pavm are not convincing in histopathological findings . coincidentally , the inflammatory granuloma of the lung looked almost identical to pavm because of the nontortuous shape with linear structures suggestive of feeding artery and drainage vein . the resection of lung granuloma is highly successful for an isolated malformation , and mortality rate is low in this surgery . embolization is an appropriate treatment modality in multiple pavm for which surgery is not suitable . complications of embolization include pleuritic chest pain , pulmonary infection , air embolism , migration of coils and paradoxical embolism . although pulmonary angiography or contrast - enhanced 3d - ct has been the standard diagnostic tool for pavm , it has limitations in detecting pavms presenting as small nodules and enhanced nodules . in our case , since the enhanced lung nodule size was 22 mm in diameter , it mimicked pavm . in this patient , it was difficult to distinguish the lung granuloma from pavm , preoperatively . though there is a report which shows lung cancer mimicking pavm , to our knowledge , this is the first report of a lung granuloma mimicking pavm . in conclusion , clinicians should pay particular attention to enhanced nodules to rule out a variety of disorders , including neoplasm , infection , inflammatory and vascular abnormality when pavm was suspected . for the purpose of not only diagnosis but also for safety in treatment of pavm , the surgical approach seems appropriate . 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 . yoshinobu ichiki : study design , data collections , data analysis , writing ; junji kawasaki : data collections ; takayuki hamatsu : data collections ; taketoshi suehiro : data collections ; makiko koike : data collections ; fumihiro tanaka : study design , data collections , data analysis ; keizo sugimachi : data collections .

introductionwhile hypervascular lesions in the lung are known to mimic pulmonary arteriovenous malformation ( pavm ) , here we report a rare case of lung granuloma mimicking pavm , on which video - assisted thoracic surgery ( vats ) was performed.presentation of casea 76-year - old woman without any symptom was admitted to our hospital because of abnormal shadow in the left lung field on chest x - ray . a 20 mm 14 mm nodule with well - defined margins and smooth contours in the left upper segment was detected in her chest computed tomography ( ct ) . contrast - enhanced three - dimensional ct ( 3d - ct ) revealed an enhanced solitary lung nodule , which was connected with linear structures suggestive of feeding artery and drainage vein . thus , we made a preoperative diagnosis of pavm by performing partial pulmonary resection by vats . intraoperatively , elastic hard nodule was palpable in the left upper segment and bruit was not convincing . histopathological findings revealed multiple foci of coagulative necrosis surrounded by epithelioid cell granuloma containing langhans - type multinucleated giant cells , involving the medium - sized blood vessels in the pulmonary parenchyma . abnormal vascular structures , such as pavm were not convincing . based on these findings , a diagnosis of left lung granuloma was made.discussionpreoperatively , it was difficult to distinguish the left lung granuloma from pavm , because hypervascular lesion , such as inflammatory changes can present as strongly enhanced nodules after injection of contrast material.conclusionsurgical approach seems appropriate , not only for the purpose of diagnosis , but also for the safety in treatment of a pavm .

the treatment of patients with multiple trauma requires a different approach to that of patients with regular trauma because they are threatened not only by the injuries themselves , but also by the metabolic disruptions that follow.1 delay in surgery , blunt trauma , extensive soft tissue damage , and combined orthopedic and vascular injuries have been associated with an increased risk of amputation , while associated nerve and bone injuries with extensive soft tissue damage are risk factors for a poor outcome.2 acute renal failure is the main cause of death in patients with war wounds and trauma of the extremities . it would be helpful to minimize mortality in these patients by managing shock in a timely manner and taking the decision to amputate appropriately and promptly.3 severe head injury is known to be a major determinant of mortality in patients with multiple injuries , but other injuries also contribute to the clinical outcome.4,5 different mechanisms of injury , such as motor vehicle crashes , falls , or pedestrians being struck by a motor vehicle , impart varying degrees of force and energy transfer that may impact outcomes ; this was found to predict mortality and functional impairment independently at hospital discharge.6 acute lower extremity compartment syndrome is a devastating complication that often presents silently in critically injured patients.7 patients who underwent delayed fasciotomy had twice the rate of major amputation and a three - fold higher mortality rate.8 the aim of this study was to evaluate if injuries of the extremities are associated with a higher one - month mortality rate than other types of associated trauma . this prospective , observational , cohort study was carried out in the regional emergency center of hospital de base after prior approval by the ethics research committee of the so jos do rio preto medical school . the emergency department follows a systematic pathway to provide initial assistance to accident victims using the atls ( advanced trauma life support ) protocol . all live accident victims treated in the emergency department from july 2004 to june 2005 were included . patients who were dead on arrival and not submitted to any type of inhospital resuscitation procedure were not included in the study . accident victims were allocated to two groups , ie , those with severe injuries to the extremities or pelvis ( abbreviated injury scale [ ais ] 34 ) and those without injuries or with minor injuries to the extremities ( ais 02 ) . the fisher s exact test and relative risk were used for statistical analysis , and an alpha error of 5% ( p 0.05 ) was considered statistically significant . a total of 3489 patients were evaluated in this study ; 3244 ( 92.98% ) did not have severe trauma of the extremities ( ais 02 ) , 34 ( 1.05% ) of whom died . severe injuries of the extremities ( ais 34 ) occurred in 245 ( 7.02% ) of the patients , with 13 ( 5.31% ) dying ( fisher s exact test : p = 0.001 , relative risk 5.063 , 95% confidence interval [ ci ] : 2.7079.467 , table 1 ) . table 2 shows the age , type of injury sustained , and time until death after trauma for the patients who died . of the 245 patients with ais 34 , 71 ( 28.98% ) were women and 174 ( 71.02% ) were men , with the mean age of the men being 40.1 20.5 years and of the women 60.2 23.6 years . this study assessed whether severe injuries of the extremities affect the overall mortality rate in accident victims . the death rate was found to be higher for patients with ais 34 than in those without injuries or with minor injuries to the limbs . thus , these data serve as a warning in respect to increased risk of death in orthopedic patients compared with general trauma patients . when the severity of specific injuries in accident victims is reported in the literature , head trauma is cited as one of the main causes of death.5,6 however , there are few data in the literature about the association between death and injuries to the extremities . one study reported that the mortality rate in accident victims with extremity injuries was higher in pedestrians struck by motor vehicles ( 20% ) , and for those with head injuries , it was higher for motor cycle crash victims ( 16%).6 the first phase of management for these patients aims to control bleeding , by surgical intervention if necessary , and to prevent further wound contamination . the second phase consists of resuscitation in the intensive care unit , and the third phase aims at definitive repair of the injuries sustained.9 pelvic injuries represent a thorny and stubborn therapeutic challenge . rapid diagnosis and effective treatment ( damage control ) of these injuries play a key role in the patient s survival , inasmuch as the mortality of multiply injured patients with pelvic ring disruption remains high ( 20%35%).10 the preclinical management of patients with multiple trauma influences the prognosis regarding mortality and morbidity . diagnostic overview , protection of vital functions in the special circumstance of shock , immobilization of the spine , and treatment of isolated injuries are an essential part of preclinical management.11 the type of trauma is known to influence the mortality rate.12 in this study , all the patients received specialized pre - hospital assistance , and were treated in a regional trauma reference center . in spite of all the care given at the scene of the accident through to discharge from hospital , trauma of the extremities was a significant cause of death . accident victims with injuries of the extremities are at higher risk for death than those with other types of trauma .

background : the aim of this study was to evaluate one - month hospital mortality in victims with injuries of the extremities.methods:all accident victims admitted to the hospital de base in so jos do rio preto , brazil , during the period from july 2004 to june 2005 , were evaluated in an observational study . patients were classified using the abbreviated injury scale ( ais ) . patients with severe injuries of the extremities ( ais 34 ) were compared with those without injuries or with minor extremity injuries ( ais 02).results : a total of 3489 accident victims were evaluated ; 3244 ( 92.98% ) did not suffer injuries or had minor injuries of the extremities ( ais 02 ) and 245 ( 7.02% ) had severe injuries ( ais 34 ) . of the 245 patients with ais 34 extremity injuries , 13 ( 5.31% ) patients died , and of those without severe injuries to the extremities , 34 ( 1.05% ) died ( fisher s exact test p = 0.0000 , relative risk 5.063 , 95% confidence interval [ ci ] : 2.7079.467).conclusion : patients with injuries of the extremities are at greater risk of death than accident victims with other types of trauma .

in recent years , coupling of theoretical and experimental approaches in the study of protein folding has resulted in providing fruitful clues . experimental and computational protein design provides vital clues to understand the protein folding process , and it is of considerable interest in the area of protein science to engineer proteins with novel folds and desired functions . the field of protein design has a unique history where researchers from diverse discipline come together to explore novel catalytic , pharmaceutical , structural , and sensing properties of amino acids in proteins . interestingly , a designed eleven amino acid sequence folded as a helix in one position and as a sheet in another position in the protein sequence . this work has enabled to explore the role of nonlocal interactions in the formation of secondary structure . subsequently , helices were transmuted into sheets to understand the conformation change phenomenon and illustrate that not all the amino acids play an equal role in specifying a fold . utilizing the knowledge offered by several protein design groups , kuhlman et al . in 2003 have computationally designed a 93-residue / protein called top7 and found that the protein could be experimentally folded and extremely stable . this pioneering work has enabled further research to understand the contribution of each amino acid residue in a protein to adopt a certain fold . hence , emphasizing that protein design could be a powerful experiment to understand the processes that underlie conformational plasticity in proteins . explored how two proteins with almost similar amino acid sequences change their fold and function . following the contribution of various theoretical and experimental protein science research groups , several such engineered proteins with selective nevertheless , the design of such a pair of proteins with high sequence identity with completely different topologies can be viewed as a challenge to the well - accepted paradigm that similar sequences always tend to fold into similar three - dimensional structures . an analysis of the literature reveals that the design of two highly identical proteins with different folds and functions is challenging and time bound as shown in [ table 1 ] . streptococcus protein g contains two types of domains ( ga and gb ) that bind to serum proteins in blood . the natural versions of ga and gb domains share no significant sequence homology and have different folds , 3 and 4 + , respectively . from the above two parent proteins , high - identity versions of ga and gb were synthesized . interestingly , small and critical differences in the sequences of the two proteins determine the topology of the protein early on the folding pathway . in addition , two proteins named ga88 ( pdb i d : 2jws ) and gb88 ( pdb i d : 2jwu ) by mutation experiments from the streptococcus protein g with 88% sequence identity adopt different structures and functions and these proteins are valuable tools to understand the contribution of residues to adopt a particular fold . these two proteins vary only at seven positions out of 56 amino acids , which are shown in [ figure 1 ] . this design has made a breakthrough in the field of protein science and contradicts the general statement that following this , we have carried out computational sequence and structural analysis on these two designed proteins . we have performed secondary structure prediction of these two proteins and observed that the methods such as multivariate linear regression combiner can predict some regions as extended structures for the helical protein sequence ga , which gave us a clue that there may be structural plasticity at the region of first 15 residues , which are identical in the both proteins . we also discovered some patterns in the nonidentical positions of two proteins with a rare combination of residues that are not present in any publicly available sequence databases . by analyzing the structures of the two designed proteins , we predicted nucleation sites at various positions in the sequence , which may start or terminate secondary structural elements ( helix , sheet , and coil ) . we also observed drastic difference in the surrounding environment of nonidentical residues ( 7 out of 56 ) and difference in interaction energy . by observing the structural plasticity at the amino and carboxyl terminal of the sequences of two designed proteins and the influence of surrounding environment of each residue , we concluded that early on during the process of folding , both proteins may choose different energetically favorable pathways to attain the different folds . literature review of design of two proteins with high sequence identity adopting different folds sequence , dictionary of secondary structure of proteins assigned secondary structures and tertiary structures of pair of homologous heteromorphs ( the seven residues that vary in both sequences are indicated in rectangular boxes ) other researchers have characterized the folding of these two proteins using biophysical and computational experiments . they also indicated that the final native structures of these proteins were dictated very early along the folding pathway by performing equilibrium unfolding of ga88 and gb88 , folding and unfolding kinetics and molecular dynamics simulations experiments . concurrently , energy calculations were performed on the two designed proteins in a vacuum , which indicated that current computer modeling / simulations experiments can not explain why two highly similar sequences fold into different structures . however , it was suggested that improved modeling / simulations tools should be developed to predict the pair of sequences with different structures , which differ , by only few residues . in a recent study , folding and unfolding kinetics experiments performed on these two designed proteins indicated a detectable residual structure in the denatured state of gb88 whereas the denatured state of ga88 is unstructured . interestingly , they explored these two proteins by value analysis based on 132 site directed mutants and concluded that the protein 's topology is committed very early along the folding pathway . based on the above studies , we suggest that , along with the suitable protein design experiments , better theoretical models including folding simulations coupled with structure prediction and sequence search in databases can shed light on the phenomenon of protein folding and conformation switching which may ultimately lead us to understand the contribution of each amino acid in these proteins to adopt a specific fold .

in our study , we have concluded that two proteins with 88% homology choose different energetically favorable pathways in the very early stage of the folding process to attain their native folds . subsequent reports from other investigators by performing folding and unfolding kinetics experiments concur with our findings . we herewith discuss the key papers revealing computational and experimental analysis of two designed proteins with similar sequence distant folds . further we suggest that the theoretical / computational analysis of protein sequences and structures along with the relevant experiments provide a better understanding of the relationship between protein sequence , folding , and structure .

primary tumors of the trachea are rare and account for 0.1% of all malignancies . in adults , approximately 90% of the primary tracheal tumors are malignant , whereas in children , the majority of these tumors are benign [ 2 , 3 ] . in a retrospective study by webb et al . , 55.4% of the patients were male and 77.3% of them were smokers . the frequency of this rare disease has been estimated to be 0.10.4% of all malignancies , with an annual incidence of 2.6 new cases per million per year . primary tracheal tumors can arise from the respiratory epithelium , salivary glands and mesenchymal structure of the trachea . squamous cell carcinoma ( scc ) and adenoid cystic carcinoma ( acc ) make up about 71.6% of all adult primary tracheal tumors . the remaining portion ( 28.4% ) scc occurs predominantly in men in the sixth and seventh decades , whereas acc is equally distributed between the sexes and peaks in incidence in the fourth and fifth decades . in contrast to acc , scc ( the most common type of tracheal tumors ) has a more aggressive course . median survival time ranges from 6 to 14 months , and tumor disappearance is dependent on whether the primary lesion is resected . sccs spread to the lymph nodes and 1020% of the patients will have distant metastasis at presentation . carcinoids , lymphomas , granular cell tumors and small cell carcinomas have variable prognoses ; yet they seem to behave better than sccs , adenocarcinomas or sarcomas . the majority of the tracheal neoplasms are primary in nature , and other primary sites that can metastasize to the trachea are the lung and esophagus , the latter being the most common . patients with surgically resectable primary tracheal tumors have a better prognosis than those with tumors that can not be resected . preoperative radiation therapy has been attempted in some patients , yet the most compelling evidence for adjuvant radiation therapy comes from postoperative cases . the present study reports a rare case of primary tracheal malignancy treated with surgery and adjuvant radiation therapy . our 60-year - old diabetic and hypertensive male patient had a 1-month history of cough associated with marked respiratory distress and occasional dyspnea in the right lateral lying position . the patient consulted a chest physician and underwent bronchoscopy , which showed a polypoid growth in the trachea extending to the larynx ( fig . a computed tomography ( ct ) scan revealed irregular soft tissue thickening in the region of the trachea extending up to the adjacent larynx with no soft tissue calcification , and there was no definite lymphadenopathy in the cervical region and no involvement of the underlying bone . a repeat ct scan of the neck ( fig . 2 ) showed evidence of an ill - defined , irregular , nodular , infiltrating soft tissue growth arising from the posterior tracheal wall , bridging the lumen and reaching up to the anterior wall , with an infiltration of about 2.2 1.8 cm . the location was opposite the c7-d1 level involving the 5th or 6th tracheal ring , approximately 7 cm proximal to the carina with no significant evidence of cervical and mediastinal lymphadenopathy . excision of the tracheal growth through a cervical transverse incision was performed , and a tracheostomy tube was inserted . soon after surgery , the cough subsided and the patient attended square hospital , dhaka , bangladesh , for postoperative irradiation . ninety - five percent of the planning target volume ( ptv ) covered 97.3% of the prescribed dose . the minimum and maximum dose received by the ptv was 50.05 and 63.68 gy , respectively . 3 ) . three multileaf collimator fields ( left anterior oblique , right anterior oblique and posterior ) were used for three - dimensional conformal radiation therapy ( 3dcrt ) . complete response was documented , and the patient is still alive without any evidence of disease during 30 months of follow - up . primary malignant tumors of the trachea are uncommon , and therefore limited data supporting a standard management are available . the vast majority of the primary tracheal tumors in adults are malignant and most of them usually diagnosed at a later stage , which is due to a delayed presentation of specific symptoms like cough , dyspnea and hemoptysis . it is clear that patients who can be resected have a better prognosis than those who can not , prompting the recommendation of surgical resection for most primary tracheal tumors . all resected patients need postsurgical irradiation , regardless of tumor burden , margin status , histology or nodal status . grillo and mathisen experienced a median survival of 34 months in scc and 118 months in acc patients who underwent a combined modality like surgery and radiation therapy . attained a 61-month median survival using a combined surgery and radiation therapy in their study . . showed that 73% of the patients with a 5-year survival underwent radiation therapy postoperatively . the role of external beam radiation therapy as an adjuvant to surgical resection is better established . grillo and mathisen advocated maximal ( 4,5006,500 cgy ) radiation therapy after scc and acc resection because of the close margins necessary for resection and the high likelihood of local recurrence . modern ct - based 3dcrt or intensity - modulated radiation therapy theoretically allows higher and safer doses delivered to the trachea . for postoperative cases , all patients with this tumor in the postoperative or definitive setting should be treated with the 3dcrt technique . an intraluminal boost technique after external beam radiation therapy may decrease the risk of late side effects . there are many studies showing the benefit of postoperative irradiation therapy in primary tracheal malignancies such as sccs and accs . however , little experience has been shared in the case of primary adenocarcinomas of the trachea , the tumor type described in our report . the prognosis of patients with malignant tumors of the trachea remains gloomy , and long - term median survival of tracheal adenocarcinoma patients undergoing combined modalities is unknown to us . primary adenocarcinoma of the trachea needs to be diagnosed at an early stage , and combined multimodal approaches may be explored to attain an extended median survival . none of the authors has any conflicts of interest regarding the content of this article .

primary adenocarcinoma of the trachea is extremely rare and a standard treatment does not exist due to nonavailability of evidence - based randomized control studies . this paper reports the case of a 60-year - old male , who presented with cough and occasional respiratory distress . bronchoscopic examination and a computed tomography scan revealed a soft tissue mass in the trachea arising from the posterior tracheal wall . cytological examination and immunochemistry confirmed primary adenocarcinoma of the trachea . excision of the tumor followed by three - dimensional conformal radiation therapy was performed , and a dose of 56 gy was delivered to the primary site . two and a half years after treatment , the patient has no clinical or radiological evidence of the disease , and no late complication has occurred .

antiphospholipid syndrome ( aps ) is an autoimmune disease characterized by thrombosis and morbidity , specifically in pregnancy , due to antiphospholipid antibodies . about half of the cases of aps occur as a primary disorder , while the rest arise in association with other autoimmune diseases , such as systemic lupus erythematosus ( sle ) . some diseases , such as pulmonary thrombosis and pulmonary hypertension , are known to be complicated by aps ; however , aps with pleural effusion is extremely rare . here , we present a case of aps complicated by unilateral pleural effusion that responded well to oral corticosteroid therapy . a 75-year - old japanese man was admitted to our hospital for spreading erythema on his trunk and extremities , as well as dyspnea . one year prior to admission , he visited us with a 1-year history of erythema and purpura on his legs , accompanied by intermittent fever . results of laboratory examinations for antiphospholipid antibodies , lupus anticoagulant ( using the phospholipid neutralization test ) , and anticardiolipin antibody had been positive 12 weeks apart . in addition , he showed positive antinuclear antibody ( 1:80 , homogeneous pattern ) , but was negative for anti - dsdna antibody , anti - sm antibody , anti - rnp antibody , anti - ss - a antibody , anti - ss - b antibody , antitopoisomerase i antibody , and anticentromere antibody . mpo - anca , pr3-anca , and cryoglobulin were negative . given the diagnosis of aps , we initiated combination therapy with aspirin ( 100 mg daily ) and warfarin ( target international normalized ratio , 2.03.0 ) , but the skin lesions continued to gradually worsen . violaceous erythema , purpura , and pigmentation were widely noted on his trunk and extremities ( fig . 1 ) ; they were associated with low platelets ( 93,000/l ) and elevated partial thromboplastin time ( 48.4 s ) . a biopsy specimen revealed marked thrombosis in the dermal and subcutaneous small vessels ( fig . 2 ) . there were interface changes of the dermo - epidermal junction and mild inflammatory infiltrates in the perivascular area of the dermis , but mucin deposition and thickening of the basal layer of the epidermis were not apparent . in addition , a chest x - ray and computed tomography demonstrated a large pleural effusion in the left lung ( fig . 3 ) , without evidence of large vessel thrombus . electrocardiogram and echocardiogram were normal . despite serial thoracenteses , effusion recurred . bacterial and fungal cultures , as well as cytology analyses for malignant cells , were all negative . after excluding infectious diseases , malignancies , pulmonary thrombosis , and heart failure , we added oral prednisolone ( 30 mg daily ) to his prior anticoagulant regimen . the skin lesions and the pleural effusion improved rapidly , eventually disappearing without complication ( fig . 4 ) . on follow - up clinical examinations , no symptoms related to sle or other collagen diseases were noted . common causes of pleural effusion include malignancies , infectious diseases , pulmonary embolism , collagen vascular disease , and heart failure . aps - related pleural effusion has rarely been reported , and those cases that have been reported appeared to be complications of accompanying pulmonary embolism , sle , or catastrophic aps [ 4 , 5 , 6 ] . pleuritis , which can induce pleural effusion , is the most common pleuropulmonary manifestation of sle . in the present case , after excluding these differential diagnoses , aps was determined to be the direct cause of the pleural effusion . however , a strong possibility still exists that the pleural effusion may be associated with occult collagen vascular disease , particularly sle or lupus - like disease ( lld ) heretofore undiagnosed . a long - term follow - up study in 128 patients with primary aps demonstrated that 11 patients ( 8% ) developed sle , while 6 ( 5% ) developed lld during a median follow - up period of 8.2 years ( range , 114 years ) . the results of this study suggest that the pleural effusion may be attributed to a coexisting condition like lld , although our patient has not fulfilled american college of rheumatology diagnostic sle criteria to date . this may be supported by the fact that oral corticosteroid therapy was a remarkably effective treatment of the pleural effusion that had previously been unsuccessfully treated by anticoagulant therapy and repeated drainage . corticosteroids and immunosuppressants continue to be the treatment of choice for severe sle complications , including pleural effusion . furthermore , the clinical manifestations of primary aps and aps associated with sle are similar , which makes it more difficult to differentiate these diseases . as pleural effusion can be life - threatening corticosteroids might be an effective choice of treatment for intractable pleural effusion in aps patients .

antiphospholipid syndrome ( aps ) with pleural effusion is extremely rare . a 75-year - old man was admitted to our hospital for spreading erythema on his trunk and extremities , as well as dyspnea . one year before admission , he had visited us with a 1-year history of erythema and purpura on his legs and occasional fever . given the diagnosis of aps , we initiated a combination therapy of aspirin and warfarin , but the skin lesions had gradually worsened . a biopsy specimen revealed marked thrombosis in the dermal and subcutaneous small vessels . in addition , chest x - ray and computed tomography demonstrated a large pleural effusion in the left lung . he underwent repeated drainage of the pleural effusion but the effusion recurred . we added oral prednisolone 30 mg daily to his prior anticoagulant therapy . the skin lesions and pleural effusion rapidly improved and disappeared without any complication . corticosteroids might be a choice of treatment for intractable pleural effusion in aps patients .

a girl weighing 3,190 g was delivered by a caesarean section at 38 weeks and five days of gestation . at the time of birth , a systolic murmur was noted during a physical examination , and transthoracic echocardiography revealed a peri - membranous ventricular septal defect ( vsd ) with a septal aneurysm , a small patent foramen ovale ( pfo ) , and a small right - sided patent ductus arteriosus ( pda ) from the innominate artery . the vsd was measured as having a diameter of 3.5 mm and a shunt flow less than 2.5 m / sec . due to the presence of neonatal hyperbilirubinemia , echoencephalography was conducted , and no abnormalities were found . five months later , the patient was referred to konkuk university medical center for vsd and pda . her body weight was 6,700 g ( 25th percentile ) , and her height was 61.6 cm ( 10th percentile ) . transthoracic echocardiography revealed a vsd approximately 6 mm in size with a minimal aneurysm , a left - sided pda 3.6 mm in diameter from the right aortic arch and an aberrant left subclavian artery . a subsequent computed tomographic scan demonstrated isolation of the left subclavian artery with a right aortic arch , a left pda , and a vsd ( fig . the intraoperative findings were a perimembranous vsd , a pfo , a mildly patent right ductus arteriosus , and isolation of the left subclavian artery connected to the left pulmonary artery via a left pda . the left subclavian artery was disconnected from the left pulmonary artery and reimplanted to the left common carotid artery by end - to - side anastomosis with monofilament polypropylene 6 - 0 sutures ( fig . ductus arteriosus is usually located on the left side , between the descending aorta and the junction of the main pulmonary artery and left pulmonary artery . however , ductus arteriosus may also be present on the right side or , very rarely , may occur bilaterally in association with aortic arch anomalies or conotruncal anomalies . in such aortic arch anomalies , isolation of the left subclavian artery with right aortic arch is also uncommon . here , isolation refers to the fact that the left subclavian artery connects to the pulmonary artery via either the ligamentum arteriosum or a patent ductus arteriosus without any connection to the aorta . isolation of the left subclavian artery with a right aortic arch is known to be commonly associated with congenital heart disease , but may also occur with normal intracardiac anatomy , although few such cases have been described . isolation of the left subclavian artery with a right aortic arch may be related to the 22q11 deletion . bilateral ductus arteriosus and isolation of the left subclavian artery with a right aortic arch can be explained through the hypothetical double aortic arch plan suggested by edward . regression takes place on two levels in the double aortic arch plan : on one level , regression occurs between the left common carotid artery and the left subclavian artery ; and on the other level , regression occurs at the left dorsal aortic root distal to the left ductus arteriosus . and then right ductus arteriosus remains persistent , left ductus arteriosus connects the left subclavian artery to the left pulmonary artery ( fig . however , the right ductus arteriosus regressed , and only the left ductus arteriosus remained patent . if the left ductus arteriosus is patent , blood may be supplied to the left subclavian artery via the left ductus arteriosus . if the left ductus arteriosus regresses , the blood supply to the left subclavian artery may involve a mediastinal , thoracic anastomosis , or vertebral pathway . isolation of the left subclavian artery usually presents with no apparent symptoms in neonates , but it may present with congenital pulmonary steal syndrome , subclavian steal syndrome , or may even present in adults with late symptoms due to sporadic progression . hayabuchi et al . reported the case of a three - month - old girl with cerebral atrophy and an underdeveloped left arm . reported the case of a 15-year - old boy with an underdeveloped left arm . due to these symptoms and signs , the therapeutic management of isolation of the left subclavian artery remains controversial , especially when it is associated with complicated congenital heart disease . some authors have suggested that adequate collateral circulation must be ensured , meaning that reconstruction of the isolated subclavian artery is optional , regardless of the symptoms and signs . successful results have been reported after ligation or device closure of the pda and ligation of the left subclavian artery . however , reconstruction of the left subclavian artery due to pulmonary steal syndrome after right pda closure in bilateral pda has been reported . in one report , ischemic symptoms in the left arm and vertebrobasilar insufficiency occurred years after ligation of the left subclavian artery . hokari et al . reported that a man with peutz - jeghers syndrome presented with his first vertigo attacks due to subclavian steal syndrome at 29 years of age . our patient presented with no symptoms and signs related to subclavian or pulmonary steal syndrome , and had shown normal findings on an echoencephalography study conducted at our medical center due to neonatal hyperbilirubinemia . however , brain computed tomography angiography performed after surgery revealed hypoplasia of the left vertebral artery . we suggest that this hypoplasia would have led to vertebrobasilar insufficiency or underdevelopment of the left arm without surgical reconstruction . since surgical reconstruction of the isolated left subclavian artery leads to antegrade flow in the left subclavian artery , it can prevent hypoplasia of the left vertebral artery and subclavian / pulmonary steal syndrome . our case shows that early surgical reconstruction is reasonable , regardless of the symptoms , in cases of isolation of the left subclavian artery .

right aortic arch with isolation of the left subclavian artery is a rare anomaly . the incidence of bilateral ductus arteriosus is sporadic , and a right aortic arch with isolation of the left subclavian artery in association with bilateral ductus arteriosus is therefore extremely rare . since the symptoms and signs of isolation of the left subclavian artery can include the absence or underdevelopment of the left arm , subclavian steal syndrome , or pulmonary artery steal syndrome , the proper therapeutic approach is controversial . we report a case in which surgical reconstruction was used to treat isolation of the left subclavian artery with right aortic arch in association with bilateral ductus arteriosus and a ventricular septal defect .

the head and neck posture of an individual can influence soft - tissue relationships in the cervical and shoulder region1 , 2 . a common concern in the modern workplace is upper extremity disorders arising from overhead work , which is associated with neck and shoulder disorders and pain3 . long - term overhead working postures result in strain and fatigue of the shoulder muscles because arm elevation is associated with shoulder muscle fatigue4 , 5 . previous studies have focused on risk factor analysis and the development of therapeutic exercises for overhead work - related disorders rather than prevention6 , 7 . some studies have been performed on postural ergonomic interventions including working techniques for overhead work6 , 7 . however , we found that few studies have focused on protective ergonomic devices for overhead workers . therefore , this study investigated a new neck support tying ( nst ) method that used a thera - band for the prevention of neck and shoulder pain in workers performing overhead work . the new nst method supports the neck during hyperextension and prevents excessive upward rotation of the scapula during overhead work . the purpose of the present study was to investigate the effect of this nst method on cervical rom and shoulder pain after overhead work . the subjects were divided into two groups as follows : a control group consisting of 7 males without nst , and a nst group consisting of 7 males with nst . the initial cervical rom and initial ppts of the ut and mt were not significantly different between the two groups . the initial values of cervical flexion , extension , and right and left lateral flexion in the control group were 63.44.2 , 72.86.0 , 53.92.9 , and 51.35.6 degrees , respectively . the initial values for cervical flexion , extension , and right and left lateral flexion in the nst group were 62.35.1 , 72.53.9 , 53.33.0 , and 52.22.4 degrees , respectively . all participants gave their informed , written consent according to the protocol approved by the human ethics committee of the yonsei university faculty of health science . this study examined a new nst method that uses a thera - band for the prevention of neck and shoulder pain in workers performing overhead work . for the nst method , we used the grey thera - band ( 60 cm length ) which was applied as follows . the midpoint of the thera - band supported the posterior aspect of the neck , and both ends of the thera - band were passed under both axillae , and tied behind the back . the nst provided support for neck hyperextension and prevented excessive upward scapular rotation during overhead work . cervical flexion , extension , and right and left lateral flexion were measured with a cervical range of motion ( crom ) instrument ( performance attainment associates , st . a dolorimeter pressure algometer ( fabrication enterprises , white plains , ny , usa ) was used to measure the pressure pain threshold ( ppt ) of the right side upper trapezius ( ut ) and the lower trapezius ( lt ) muscles . a 1-cm rubber plate delivers pressure from the probe to the body , and the pressure is read from a needle gauge . all subjects performed one trial of overhead work with their arms over their heads for 15 min . the overhead work was performed at a height of 25 cm above each subject 's head . differences in cervical rom and ppt between the nst and control groups after the overhead work were tested with the independent t - test using the spss statistical package ( version 18.0 ; spss , chicago , il , usa ) . the cervical flexion , extension , and lateral flexion angles of the nst group were significantly larger than those of the control group ( p < 0.05 ) . the cervical flexion , extension , and right and left lateral flexion of control group were 50.48.2 , 64.711.3 , 41.77.9 , 43.29.2 degrees , respectively . the cervical flexion , extension , and right and left lateral flexion of nst group were 61.511.2 , 69.46.9 , 48.75.6 , 49.86.7 degrees , respectively . the ppt of ut of the nst group ( 7.21.8 lb ) was significantly higher than those of the control group ( 6.32.0 lb ) ( p < 0.05 ) . the ppt of mt of the nst group ( 5.81.4 lb ) was significantly higher than those of the control group ( 5.01.2 lb ) ( p < 0.05 ) . repeated and sustained working with elevated arms is known to lead to neck and shoulder pain8 . this study proposed a new neck support tying method using thera - band and investigated its effect on cervical rom and shoulder pain after overhead work . reductions in rom have implications for the safety and efficiency of functional activities , and lead to a loss of corrective or protective reactions1 , 9 . rom losses can occur from inactivity and structural changes of the tissues in the cervical spine , and result in an increase in connective - tissue density , shortening of collagen tissue , and muscle fibrosis1 , 9 . in this study , the cervical flexion , extension , and lateral flexion angles of the nst group shoulder forward flexion with scapular upward rotation requires the activation of the upper trapezius , and overstretches the middle trapezius through scapular protraction3 , 5 , 7 . the ppts of the ut and mt were significantly lower in the nst group than those of the control group . these results indicate that the nst supported the neck and prevented excessive scapular elevation and upward rotation during overhead work . the thera - band , which provides varied resistance through the range of movement , has been used for rehabilitation in combination with therapeutic exercise10 . it is light and portable , has low resistance , and can be adjusted to accommodate various situations11 . the nst method prevented rom reduction and pain in the cervical and shoulder regions . the nst method can be easily and simply applied using a thera - band and is also inexpensive . we suggest that industrial workers could use the nst method when performing overhead work .

[ purpose ] this study proposed a new neck support tying ( nst ) method using thera - band for the prevention of neck and shoulder pain in workers doing overhead work . the purpose of this study was to investigate the effect of the new nst method using thera - band on cervical rom and shoulder pain after overhead work . [ subjects ] fourteen male subjects were recruited . [ methods ] this study measured the cervical rom and pressure pain threshold ( ppt ) of the upper and middle trapezius ( ut and mt ) muscles after the control and nst groups had performed overhead work . [ results ] the cervical flexion , extension , and lateral flexion angles of the nst group were significantly larger than those of the control group . the ppts of ut and mt of the nst group were significantly higher than those of the control group [ conclusion ] the nst prevented rom reduction and pain in the cervical and shoulder regions .

in this issue of critical care , laporta and coworkers review a multidisciplinary working group 's analysis of a case study on the causes of and solutions for staff turnover in an intensive care unit ( icu ) setting . this issue is of profound significance to health care leaders in western countries because the workforce is shrinking as a result of impending baby boomer retirements and , as the population ages , the demand for intensive care services will grow considerably . these demographic factors are further compounded by the fact that the complexity of care provided in the icu demands professionals who are highly trained and skilled . in this environment , turnover can be costly to the organization because of the significant expenses associated with recruiting and training workers . there are many well documented reasons for staff turnover in the intensive care setting that are highlighted by laporta and coworkers as core reasons . these core reasons include job dissatisfaction due to inflexible scheduling practises , insufficient opportunity for professional development , as well as a lack of collaborative decision making around clinical and practice issues . the authors discuss that data on icu turnover comes from nursing literature and that this research may be applicable to other health care professionals . however , it is important not to assume that reasons for turnover are the same among different groups of health care providers and that staff turnover is something to be avoided at all costs . for example , misra - hebert and coworkers state that one contributor to physician turnover is conflict between the physician 's and organization 's philosophy and goals . physician turnover in this case may be beneficial both to the physician and organization if the two parties can not reconcile their differences and the conflict impacts on the ability of both parties to move forward . there are other important reasons for turnover that should be considered by icu leaders , and these include burnout and generational diversity . burnout is a prevalent phenomenon in icus , and the nursing literature suggests that issues such as moral distress when engaging in futile care contributes to burnout . in the medical literature causes of physician burnout include volume of work , increased expectations of the public , lack of sleep and the possibility of being sued . the consequence of burnout is that there is a negative impact on quality of care and staff morale , which can ultimately cause turnover . for example , gunderson indicates that physicians who are dissatisfied may engage in inappropriate prescribing patterns . neuhauser , furthermore , discusses how environments with rigid systems and attitudes among the leadership will decrease staff morale because staff desire flexible policies and autonomy in decision - making . the generational diversity found in the icu environment can also be a source of turnover of staff . it is well documented that generation x ( born in 19651980 ) and the millennial generation ( born in 19802000 ) have a strong desire for more balanced work life than veterans ( born in 19251945 ) and baby boomers ( born in 19461964 ) . research conducted by lorin and coworkers on internal medicine residents of the millenial generation showed that although 41% considered a fellowship in critical care , only 3.4% chose this training because of lack of leisure time and stress levels among faculty and fellows . clearly , it is important for leaders to be attuned to these generational differences when developing recruitment and retention plans and redesigning the workplace environment . the review from laporta and coworkers also highlights the importance of icu leadership working with frontline staff to create a vision and strategy that addresses the core reasons for turnover . it is essential that this vision be aligned with the vision , mission and values , and strategic plan of the health care organization . furthermore , the team should assess whether their hospital is highly reputable , has high patient satisfaction , and sufficient resources and equipment to provide care . all of these components are signs of a positive work environment , and leadership can build on these attributes to recruit and retain staff . the other key factor in this process is the use of a team work approach . team work training in the areas of conflict resolution , learning styles and giving feedback will help the staff to work together to create and achieve an inspiring vision . although the financial and human resource investments required to engage in this process are considerable , there is substantial evidence in the literature that highly functioning , satisfied teams lead to more efficient patient care and better outcomes . staff turnover is a critical issue that icu leaders need to understand and address in their unit settings . attention to this issue with a systematic , evidence - based approach that focuses on team work and collaboration will not only improve retention but will also make the icu a highly competitive and desirable place to work .

this commentary discusses laporta and coworkers analysis of a case study on the causes of and solutions for staff turnover in an intensive care setting . staff turnover is a significant issue for health care leaders due to the shrinking workforce in western countries and an increased demand for intensive care services as the population ages . the commentary considers reasons for turnover such as burnout and generational diversity , and highlights the importance of a team work approach to address the issue of turnover .

implant - supported overdenture prostheses can be divided into bar overdentures and single attachment overdentures . single attachment elements for overdentures include single retentive anchors , single magnet anchors , and individually cast telescopic copings.1 among these , telescopic copings have the benefit of implant splinting found in bar overdentures and the retrievability of single attachment overdentures . however , this method is typically fabricated using gold materials , so it is not an economical treatment option . also , if an inner crown will be worn , it is difficult to maintain appropriate retentive forces . in this case report , a telescopic implant - supported overdenture prosthesis was made using a new material , polyaryletherketone ( paek ) based polymer ( pekkton ivory , cendres + mtaux sa , biel / bienne , switzerland ) . it shares benefits of typical telescopic coping , in additional to being highly economical , wear resistant , and light in weight compared to conventional implant overdenture prostheses . 1 ) presented to the department of prosthodontics at chonnam national university dental hospital . after clinical and radiographic examinations after maxillary teeth extractions and use of provisional maxillary complete denture for six months , six small diameter implant fixtures ( 3.0 10.0 mm usii , osstem implant co. ltd . , seoul , korea ) the definitive prosthesis was planned as a telescopic overdenture using paek based polymer . after a making definitive impression by polyvinylsiloxane ( honigum , dmg , hamburg , germany ) , a polymer telescopic abutment and an outer overdenture frame were fabricated with consideration of the patient 's vertical dimension ( fig . telescopic abutment and framework design were laid out by cad software ( exocad dental cad , exocad gmbh , darmstadt , germany ) . the milling machine ( s1 , vhf camfacture ag , ammerbuch , germany ) made the final framework and abutment according to the design . polymer abutment and titanium link were sandblasted by 110 um grit aluminum oxide , and bonded with primer ( sr link , ivoclar vivadent , schaan , liechtenstein ) and bonding agent ( multilink n , ivoclar vivadent , schaan , leichtenstein ) . after that , the definitive prosthesis was made by autopolymerized pour - type resin ( press lt , retec , rosbach , germany ) ( fig . 4 ) ; the design and weight of the prosthesis were adjusted to achieve acceptable esthetics and phonetics . after 6 months , there were no problems with alveolar bone around the implant fixtures and retention of the overdenture prosthesis . however , no treatment modality meets all criteria for successful treatment , and conventional overdenture material can sometimes be limited by economic , functional , and technical considerations . now , many new prosthetic materials are available to overcome these limitations , and as in this case , a new polymer can be used to make telescopic crowns and frameworks to obtain satisfactory results . paek based polymer , pekkton ivory , as used in this case , is a member of the high performance semi - crystalline thermoplastic resin group , recognized for its keto and ether group ratio . paek has good dimensional stability at high temperature , high chemical and mechanical resistance against wear , and high tensile , fatigue and flexural strength , making it an attractive material with expanded uses in medicine and dentistry.3 however , peek ( polyetheretherketone ) , a conventional paek - based polymer , can not be used as a permanent material due to its relatively weak physical properties . a new material , pekkton , is mainly composed of pekk ( polyetherketoneketone ) ; its molecular structure has an added ketone to the structure of peek with and has a wide range of uses due to its amorphous and crystalline structure . pekk reveals up to 80% greater compressive strengths than peek , so this polymer may be used in permanent prostheses according to the manufacturer.4 thus , this new polymer can be considered to have greater strength than peek and have greater esthetics titanium , higher resin bond strength than zirconia , and a lighter weight ( 1.4 g / cm ) than metal . therefore , this material is found to be mechanically suitable for fpd frameworks , milled overdenture bars , clasps , telescopic crowns , and other applications . despite the reportedly good bond strength , bonding between the titanium link and the telescopic abutment is still very sensitive , with the risk of fracture of the thin abutment wall due to connecting titanium link ; hence more studies are needed on such cases . she reported satisfaction with its strength and esthetics , and no negative symptoms . due to the lack of evidence on the long term retentive capabilities of this material , due to its functional and economic advantages , paek based polymer is a good alternative material to conventional materials and methods in the fabrication of implant overdenture .

although many prosthetic materials exist for fabrication of implant - supported telescopic overdentures , available materials have not been thoroughly evaluated from a functional standpoint . this case report describes the use of polyaryletherketone ( paek ) based polymer for an implant - supported telescopic overdenture , a seldom used material in dentistry . this material is lighter than traditional materials , can accommodate changes in retentive forces , and is an easily retrievable by cad / cam fabrication . this case highlights the possibility of using new polymer materials for implant - supported telescopic overdentures .

xanthogranulomatous pyelonephritis ( xgp ) is a rare , distinct and aggressive form of chronic infectious pyelonephritis . it accounts for lesser than 1% of chronic pyelonephritis . though common in fifth to sixth decade complications can occur in the form of psoas abscess , nephro cutaneous fistula , enterocolonic fistula , paranephric abscess and sepsis . it is essential to suspect and diagnose this condition early to prevent the morbidity and mortality . owing to its rarity and clinical curiosity a 75-year - old man presented with difficulty in micturition since 15 days , fever , abdominal and flank pain and burning micturition since 7 days . x - ray , ultrasonography ( usg ) and computed tomography ( ct ) scan abdomen findings include pyonephrosis and cortical atrophy in the right kidney . intravenous urography ( ivu ) revealed non - excretory right kidney . left kidney showed normal excretion resected specimen was yellowish lobulated renal mass measuring 13 8 6 cm with ureter [ figure 1a ] . cut section showed dilated pelvis , calyces and cortical atrophy due to extensive destruction of renal parenchyma , which were covered with thick purulent material [ figure 1b ] . histopathology revealed atrophic and dilated renal tubules showing thyroidisation and sclerosed glomeruli and interstitial fibrosis [ figure 2 ] . many areas showed histiocytes with abundant foamy cytoplasm , lymphoplasmacytic inflammatory cells with foci of polymorph nuclear leukocytes [ figure 3 and inset ] ziehl nelsen stain was negative for acid fast bacilli . ( a ) yellowish lobulated renal mass with ureter ; ( b ) dilated pelvis , calyces and cortical atrophy covered with thick purulent material atrophic and dilated renal tubules showing thyroidisation , sclerosed glomeruli and fibrosis . inset show giant cells and cholesterol clefts ( h and e , 400 ) foamy histiocytes , inflammatory cells . xgp is a severe chronic renal inflammatory condition leading to focal or diffuse kidney destruction . it may be due to defect in degradation of bacteria in the macrophages especially when associated with infection and obstruction by stones . three forms of xgp are recognised : diffuse is characterized by diffuse involvement of kidney , segmental by segmental involvement and focal - is located within the cortex . it is often misdiagnosed pre - operatively as pyelonephritis , tuberculosis , perinephric abscess and renal cell carcinoma ( rcc ) . symptoms include flank or abdominal pain , fever , palpable mass , gross hematuria , pyuria , dysuria and weight loss . in our case , symptoms were attributed to bph . other organisms include staphylococcus aureus , group b streptococcus , candida , klebsiella and bacteroides . elevation of serum creatinine and bun in our case can be attributed to impaired renal function . non - functioning , ct scan is the main stay of diagnostic imaging for xgp . xgp has been shown to be associated with transitional cell carcinoma of renal pelvis and rcc . they have even been reported in renal allograph . in diffuse or advanced stage xgp , nephrectomy is the treatment option . focal or segmental xgp if diagnosed early pre - operatively can be treated with antibiotics . pre- and post - operative broad spectrum antibiotics and symptomatic management are also key factors for successful management and better prognosis . we presented this unusual case of elderly male to stress the importance of through evaluation of renal function who gave a history of recurrent urinary tract obstruction and infection . chronic renal infection and obstruction are two common etiological factors for xgp . in all patients of prostatic enlargement , renal function must be assessed for the extent of damage . in non - functioning kidney

xanthogranulomatous pyelonephritis ( xgp ) is rare and aggressive form of chronic infectious pyelonephritis . no single clinical or radiological feature is diagnostic of xgp . a 75-year - old man with prostatic enlargement presented with difficulty and burning micturition fever , abdominal and flank pain . x - ray , ultrasonography and computed tomography scan diagnosis was right kidney pyonephrosis . intravenous urography revealed non - excretory right kidney . right nephrectomy was done . histological diagnosis of xgp was made . in all patients of prostatic enlargement , renal function must be assessed for the extent of damage . surgery is the treatment choice in most cases . pre- and post - operative antibiotics are key factors for successful management and better prognosis .

synovial chondromatosis is a disease with unknown etiology , originating from synovia and characterized by the presence of metaplastic cartilaginous nodules in the synovial cavities , bursa or tendon sheaths . the disease is commonly seen in men and between the 3rd and 5th decades of life . although the exact etiology is not known the knee , hip and elbow joints are frequently reported to be involved by the condition . however , shoulder and ankle joints are involved extremely rarely . the disease is classified in 3 stages and evaluated according to following criteria : the early stage with intrasynovial differentiation without loose bodies , the transitional stage by intrasynovial cartilaginous nodules with loose bodies and late stage with multiple loose bodies . the treatment decision is made according to the patient 's age , symptoms and the disease stage . the main advantages of the arthroscopic approaches are decreased morbidity , synchronous visualization and treatment feature for intra and extra articular pathologies . the hypertrophic synovia and multiple loose bodies are typical arthroscopic findings . in this case report , we presented an arthroscopically managed adult patient with anteriorly localized right ankle chondromatosis and discussed the potential benefits of arthroscopic surgery . a twenty - eight year old male patient was admitted to our hospital with decreased range of motion , swelling and increased pain during movement in the right ankle joint . he had no history of trauma , systemic inflammatory disease or family history of bone or joint diseases . the physical examination revealed that he had mild tenderness around the anterior ankle joint on palpation with palpable loose bodies . multiple nodules 39 mm in diameter with calcifications were located at the anterior aspect of the right ankle on the plain anteroposterior and lateral x - ray images ( fig . 1 ) . magnetic resonance imaging ( mri ) revealed multiple calcified well - circumscribed loose bodies at the same location and synovitis in the ankle joint ( fig . the laboratory tests were within the normal limits and the patient was scheduled for arthroscopic surgery with the diagnosis of anterior impingement syndrome due to right ankle synovial chondromatosis . the ankle joint was entered via anteromedial and anterolateral arthroscopic portals during spinal anesthesia and tourniquet application . multiple loose bodies and hypertrophic synovia around the anterior ankle joint were seen ( fig . 3 ) . arthroscopic partial synovectomy and excision of loose bodies were performed ( fig . the drain was removed in the 1st postoperative day and the active and passive range of motion exercises was started . the patient was allowed partial weight bearing with crutches and at the 2nd week he was mobilized with full weight . there were multiple cartilaginous loose bodies , with the biggest and smallest dimensions of 0.9 cm 0.7 cm 0.5 cm and 0.4 cm 0.3 cm 0.2 cm in the permanent pathology report respectively ( fig . the patient 's dorsiflexion and plantar flexion degrees were 25 and 30 , respectively , at the end of the 11th postoperative month . no complications were diagnosed in the follow - up period with no recurrence on the plain x - ray images and mri . trauma , degenerating joint diseases , osteochondritis dissecans , rhomatoid arthritis and tuberculosis arthritis are examples of the secondary form . our case was evaluated in the primary synovial osteochondromatosis group due to the absence of previous trauma or inflammatory pathologies . complaints of pain , swelling of the joint ( especially after physical activity ) with or without accompanying pain , decreased range of motion , palpable mass , locking paresthesias and joint clicking are main symptoms and signs in patients with synovial chondromatosis . the suspected diagnosis was confirmed by the appropriate radiologic investigations and pathologic examination after history taking and physical evaluation . the calcified form synovial osteochondromatosis could be seen in the anteroposterior and lateral plain x - ray images . mri investigation enabled the diagnosis of the disease in the early stage , the exact localization of the disease and intrinsic property of chondroid tissue . the tenosynovial giant cell tumor , calcifying aponeurotic fibroma , periosteal chondroma , osteocartilaginous loose bodies and soft tissue chondrosarcoma must be kept in mind in the differential diagnosis . the disease is slowly progressive and is considered to be a self - limiting condition . in the early stages of the disease and in asymptomatic patients , treatment can be planned conservatively with frequent follow - up visits . degenerative changes could occur in the later stages of the disease in patients without appropriate therapy . although the classical treatment approach for ankle joint chondromatosis is open surgery , arthroscopic surgery is rarely encountered in the literature . some important advantages of arthroscopic surgery are wide visualization areas , easy access to difficult to reach areas , lower morbidity , no need for casting and immobilization , early rehabilitation and quick recovery period . however , there is the possibility of limited synovectomy and residual loose bodies . arthroscopic surgery enables wide regional visualization , lowers morbidity , promotes early rehabilitation , shortens the recovery period and decreases the immobilization period . 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 upon request . nevres hurriyet aydogan , ahmet ozmeric , onur kocadal , murad pepe , talip kara took part in surgical procedure , literature search , preparation of the paper and review . serap gozel took part in pathological investigation.key learning pointssynovial chondromatosis is a disease originating from synovia and characterized by the presence of metaplastic cartilaginous nodules.pain , swelling of the joint especially after physical activity and decreased range of motion are main symptoms.treatment is arranged according to the patient 's complaints , age and disease stage.there are many advantages of arthroscopic surgery in selected patients . synovial chondromatosis is a disease originating from synovia and characterized by the presence of metaplastic cartilaginous nodules.pain , swelling of the joint especially after physical activity and decreased range of motion are main symptoms.treatment is arranged according to the patient 's complaints , age and disease stage.there are many advantages of arthroscopic surgery in selected patients . synovial chondromatosis is a disease originating from synovia and characterized by the presence of metaplastic cartilaginous nodules . pain , swelling of the joint especially after physical activity and decreased range of motion are main symptoms .

introductionsynovial chondromatosis is characterized by the presence of metaplastic cartilage nodules originating from the synovia , bursa and tendon sheaths . although it is extremely rare in the ankle joint , malignant transformation is possible . the choice of treatment is usually open surgery for excision of loose bodies and synovectomy . limited data is available concerning arthroscopic approaches.presentation of casea 28-year - old male patient was evaluated for pain and swelling of the right ankle joint . based on the findings of physical examination and radiographic investigations , arthroscopic surgery was performed due to ankle impingement syndrome . a diagnosis of synovial osteochondromatosis was made following the pathological survey.discussionsynovial chondromatosis is slowly progressive and is considered to be a self - limiting situation . treatment strategies are decided on according to the patient 's complaints , age and disease stage . open or arthroscopic surgery . can be performed . some advantages of arthroscopic surgery are wide visualization areas , easy access to areas difficult to reach , lower morbidity , no necessity for casting and immobilization , early rehabilitation and quick recovery period.conclusionin conclusion , arthroscopic management can be successful in selected patients with synovial osteochondromatosis localized to the ankle joint .

the incidence of bladder forming a part of an inguinal hernia is 14% . with correct knowledge of anatomy and careful dissection , injuries to the bladder during hernia repair we hereby report a case where the patient first presented with a scrotal abscess and vesicocutaneous fistula after surgical repair of sliding hernia . a 65-year - old morbidly obese man underwent mesh hernioplasty for large right inguinal hernia . after removing the urethral catheter , he developed gradually increasing right scrotal swelling with fever . thereafter , he developed continuous urine leakage from the site of incision and drainage , figure 1 . we carried out a cystogram via the urethral catheter that revealed a fistulous communication between bladder and scrotal skin , figure 1 . , cystoscopy revealed normal anterior and posterior urethra , non - obstructing prostatic lobes and a defect in the anterior bladder wall with no evidence of mesh erosion . almost the whole of the bladder was lying in the right scrotum and densely adherent to the right testis and cord structures and mesh . there was a fistulous opening at the dome of the bladder wall well away from the mesh . our main concerns were inguinal hernia repair and creation of extraperitoneal space to reposition the bladder in the normal position , which was not possible without performing right high inguinal orchiectomy . hence , we performed right high inguinal orchiectomy and removal of mesh and extraperitoneal space was made to reposition the urinary bladder to its normal position . fistula opening was repaired in two layers and the bladder was put on continuous drainage via 20 french urethral catheter , figure 2 . post - operatively at 2 weeks , there was no urinary leak on cystogram and the urethral catheter was removed and normal voiding was restored . scars of previous surgery with vesicocutaneous fistula and cystogram showing contrast in the left hemiscrotum the entire urinary bladder lying in the scrotum , with the bladder re - positioned into the normal position levine coined the term scrotal cystocoele in 1951 for inguinoscrotal herniation of the bladder . urinary bladder herniations are usually diagnosed at the time of inguinal herniorraphy and are commonly repaired through the same incision . they are sometimes found incidentally during the evaluation of a patient with lower urinary tract symptoms and associated inguinal hernias . two - stage micturition is the classical symptom , with the second stage facilitated by some form of external pressure on the bladder . the para - peritoneal type is the most common type and the extra - peritoneal type is the least common . because imaging all patients with large hernias may not be cost - effective , imaging studies are performed only when bladder herniation is suspected . the diagnostic triad of lateral displacement of the distal one - third of the ureter , small asymmetric bladder and incomplete visualization of the bladder base on an intravenous urogram has been described by reardon and lowman . iatrogenic injury to the bladder during hernia repair can be due to multiple factors , such as an inexperienced surgeon in the early part of the learning curve or an obese patient with large hernial sac with unrecognized bladder component . in our patient , there could have been an injury to the bladder that was not recognized at the time of hernia repair , which led to subsequent scrotal abscess formation resulting in a vesicocutaneous fistula . if unrecognized , these usually present immediately after catheter removal , but presentation can sometimes be delayed in case the fistula is very small and there is no infravesical obstruction . management includes immediate repair in case it is recognized intraoperatively . in case of unrecognized injury and with delayed presentation , the first step is to put a wide caliber per urethral catheter followed by thorough evaluation with urine culture examination and cystogram . a small fistula can be healed with only continuous bladder drainage with per urethral catheter or preferably suprapubic cystostomy , provided lower tract infravesical obstruction has been ruled out . a larger fistula needs open surgical management . careful dissection is needed in the extraperitoneal space while separating the sac from cord structures as the bladder forms a part of the posterior wall of the sac . after completing bladder dissection , the fistula is repaired in two to three layers and an adequate space is created in the extraperitoneal plane to reposition the bladder . sometimes , large hernias could be treated by resection of the herniated bladder as described by thomas and gomella . in our case , the whole bladder was lying in the scrotum and extensive adhesions were present between the cord structures and the bladder . high inguinal orchiectomy was performed to create space for the bladder and for proper closure of the inguinal canal . urinary bladder rarely forms a part of an inguinal hernia and , with the correct knowledge of anatomy and careful dissection injuries to the bladder during hernia repair , surgery can be prevented . surgically creation of an extraperitoneal space for bladder repositioning is of paramount importance , sometimes needing inguinal orchiectomy in the elderly .

sliding inguinal hernias are usually direct inguinal hernias containing various abdominal viscera . the incidence of bladder forming a part of an inguinal hernia , called as scrotal cystocele , is 14% . the risk of bladder injury is as high as 12% when repairing this type of hernia . this case report emphasizes this aspect in a 65-year - old man who presented with urinary leak through the scrotal wound following right inguinal hernia repair .

holt - oram syndrome ( hos ) is an autosomal dominant condition with complete penetrance . manifested in 1:1 , 00 , 000 live births and characterized by forelimb deformities , congenital heart disease and/or cardiac conduction abnormalities . it is linked to a single - gene tbx5 protein - producing mutation with gene map locus 12q24 and is the most commonly occurring heart - hand syndrome . congenital cardiac and upper limb malformations frequently occur together and are classified as heart hand syndromes . the most common among the heart hand disorders is hos , which is characterized by cardiac septation defects and preaxial radial ray abnormalities . this condition with a high rate ( 3085% ) of new non - familial cases was first described by holt and oram in 1960 in a 4-generation family with atrial septal defects ( asd ) and thumb abnormalities . the most common cardiac disorder is an ostium secundum asd , followed by ventricular septal defect ( vsd ) and ostium primum asd . electrocardiogram ( ecg ) abnormalities such as various degrees of atrioventricular ( av ) block have also been reported . a full term female neonate born out of a nonconsanguineous marriage by cesarean section ( indication - previous cesarean section with polyhydramnios ) to a 25-year - old ( weight 58 kg , height 155 cm ) booked g3p1l1a1 with unremarkable antenatal history . family history revealed that the father has radial ray deformity of left upper limb without any cardiac anomaly . physical examination revealed an active baby weighing 2790 g and length of 49 cm , heart rate of 146/min , blood pressure of 70/30 mm of hg , respiratory rate of 40/min , and systemic oxygen saturation of right upper limb being 83% in room air and that of right lower limb being 74% in room air [ figure 1 ] . on musculoskeletal examination , left upper limb shortening was noticed with absent radius bone , radial flexion deformity of the wrist and also absent thumb [ figure 2 ] . no obvious deformities were noticed elsewhere . on cardio - vascular system examination , the pansystolic murmur of grade iii at the mitral and left parasternal area was heard picture showing the baby of holt - oram syndrome left upper limb showing radial ray deformity with absent thumb right hand showing triphalangeal thumb on further investigation , chest x - ray showed normal thoracic situs with cardiomegaly , plain radiograph of both upper limbs revealed absent radius on left side with absent carpal bones and absent first metacarpal bone and phalanges ( thumb ) , right side showing absent carpal bones and triphalangeal thumb [ figure 4 ] . plain radiograph showing the bony deformities of the upper limb with cardiomegaly the baby developed cyanosis couple of hours after delivery , following which an ecg was done which was normal and a 2d echocardiography was done which revealed severe aortic atresia with hypoplastic arch , large perimembranous vsd and asd as well [ figure 5 ] . the neonate was referred to a cardiac center for further management , however due to lack of resources the baby died on day 4 of life . holt - oram syndrome is an autosomal dominant disorder characterized by distinctive malformation of bones of the upper limbs and abnormalities of the heart . cardinal manifestations of hos are dysplasia of upper limb that ranges from minor findings including hypoplasia of thumb , clinodactyly , brachydactyly , triphalangeal thumbs , carpal bone dysmorphism , shortness of ulna , shortness of humerus , aplasia of radius to phocomelia and cardiac abnormalities . although bilateral , left side is often affected more significantly . in a study of 98 subjects with hypoplastic thumbs , 16% proved to be the cases of hos . there are many well described heart - hand syndromes characterized by deformities of the radial ray and congenital heart defects such as thrombocytopenia absent radius syndrome , roberts syndrome , thalidomide embryopathy , and fanconi anemia . the unique feature that helps to differentiate these from hos is that the radial aplasia is associated with hypoplasia / absence of the thumb without any hematological abnormalities and there is often a family history of heart and limb defects . the associated congenital heart defects are the most important determining factors in morbidity and mortality in these patients . other cardiac associations include pulmonary stenosis , mitral valve prolapse and arrhythmias in the form of atrioventricular blocks . more complex cardiac lesions such as tetralogy of fallot , endocardial cushion defects , and total anomalous pulmonary venous return are observed in 18% of subjects with hos . the association with aortic atresia is extremely rare . as per best of our knowledge , till date there were no cases reported in the literature having a description of hos with aortic atresia . the electrocardiographic abnormalities such as variable degree of av blocks have also been reported , but in our case no conduction defects were noted . 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 .

holt - oram syndrome ( hos ) is a rare autosomal dominant disorder that causes abnormalities of the upper limbs and heart . it is seen in 1:1 , 00 , 000 live births . it is linked to a single - gene tbx5 protein - producing mutation with gene map locus 12q24 . most commonly it is characterized by the cardiac septation defects and pre - axial radial ray abnormalities . we are reporting a case of hos with aortic atresia which is a rare association .

proteus syndrome ( ps ) is a rare and sporadic disorder that causes postnatal overgrowth of tissues in a mosaic pattern . the complications of ps include , progressive skeletal deformities , invasive lipomas , benign and malignant tumors , and deep venous thrombosis with pulmonary embolism . we report a rare case of ps that presented with hypertrophy of index and middle finger without any other abnormalities or complications . incidentally we noticed that he had enlarged index and middle fingers of both hands and thumb of right hand [ figure 1 ] . on probing patient revealed that it was present since childhood with onset around the age of 5 years and gradual progression over years to the present size . no similar tissue growth in other parts of the body and there was no one in the family with similar features . on examination hypertrophy of index and middle finger of both the hand ( a , b , c ) and thumb of the right hand ( c ) laboratory investigations revealed normal renal and liver function tests . his x - ray of hands showed hyperostosis of involved fingers [ figure 2 ] . x - ray hands showing hyperostosis of both index and middle fingure ( a , b ) and thumb of right hand ( b ) proteus who had the ability to change his shape and was proposed by wiedemann , et al . in 1983 . happle , et al . in 1987 hypothesized that the syndrome might be due to somatic alteration of a gene leading to mosaic effects that would be lethal if the mutation were carried in nonmosaic fashion . the dysregulated tissue growth in mosaic pattern results in various phenotypic presentations and hence the clinical manifestations of ps are highly variable . the tissue overgrowth is usually absent or mild at birth and progressive in nature but usually appears to plateau after adolescence . the disproportionate overgrowth of tissue is usually asymmetrical and involves the arms , legs , hands , feet , and digits . characteristic manifestations include hyperostoses , often near epiphyses with associated impaired mobility and cerebriform connective tissue nevus seen most commonly on plantar surface . other findings are lipomas , epidermal nevi and capillary vascular malformations [ table 1 ] . criteria for the diagnosis of proteus syndrome there is no specific molecular marker , or laboratory test , for the diagnosis of ps . the diagnosis is mainly based on history , clinical examination and imaging studies . because of its variable presentation , ps may be confused with other conditions . the two disorders most commonly confused with ps are klippel - trenaunay syndrome and hemihyperplasia / lipomatosis syndrome . the important points in ps that help in the differential diagnosis are : differential diagnosis of proteus syndrome sporadic and progressive nature of tissue overgrowththe absent or mild tissue growth at birthabsence of bone tumor , enchondromasabsence of specific gene mutations differentiates from neurofibromatosisabsence of familial inheritance ( postzygotic somatic mutation of genes ) sporadic and progressive nature of tissue overgrowth the absent or mild tissue growth at birth absence of bone tumor , enchondromas absence of specific gene mutations differentiates from neurofibromatosis absence of familial inheritance ( postzygotic somatic mutation of genes ) there are no effective treatment modalities for ps . the patients should be followed up regularly for development of complications and their management . the management is also challenging because of progressive nature of tissue growth . both benign and malignant two relatively common tumors include cystadenomas of the ovary and monomorphic adenomas of the parotid gland . presented with macrodactyly of index and middle finger of both the hands and thumb of right hand ( one criteria of category b ) and he met all the three general criteria . his skeletal survey was normal except for hyperostosis of index and middle finger and there were no associated complications except for the limitation of his affected finger movements . although the patient did not satisfy the proposed criteria [ table 1 ] , a literature search revealed that out of the 205 cases reported 90 satisfied the criteria highlighting the variability in clinical presentation in cases of ps . our patient was managed as a case of inferior wall mi and is presently on anti - ischemic medications with no new complications . in conclusion , ps is a very rare and highly variable , progressive tissue overgrowth disorder . patients should be kept under regular follow - up for the development of complications and their management .

proteus syndrome ( ps ) is a rare hamartomatous disorder characterized by various cutaneous and subcutaneous lesions , including vascular malformations , lipomas , hyperpigmentation , and several types of nevi . partial gigantism with limb or digital overgrowth is pathognomonic of ps . we report a rare case of ps in a 50-year - old man who presented with inferior wall myocardial infarction and was incidentally detected to have hypertrophy of index and middle fingers of both the hands .

craniopharyngioma is an uncommon tumor of the nervous system ; it is well - known to recur even several years after surgery . we are here with reporting a case of craniopharyngioma which recurred at a site removed from the original site 5 years after surgery and radiotherapy . a 4-year - old girl was admitted for progressive deterioration of vision of 2 months duration . in addition there was no history of endocrinopathy , fits or any symptom of raised intracranial pressure . on examination , visual acuity was questionable perception of light on the right side and counting fingers at 3 m distance on the left . imaging revealed a solid and cystic craniopharyngioma in the sellar - suprasellar region [ figure 1a and b ] . she underwent a right frontotemporal craniotomy and transsylvian exploration and almost total excision of the tumor . postoperative mr showed a tiny residual tumor adherent to the pons [ figure 2a and b ] . ( a and b ) showing the preoperative images ( before first surgery ) ( a and b ) images after first surgery showing no residual tumour in the primary site but a small fragment adherent to the pons she was given a course of radiotherapy for this residue 54 gy in 30 fractions . follow - up imaging at the end of 2 years did not reveal any residual tumor [ figure 3 ] . two years after surgery and radiotherapy no recurrence imaging was being done periodically to check for recurrence . the 5-year surveillance imaging showed a recurrence in the right sylvian fissure along the route taken during the first surgery . there was no evidence of tumor in the sellar - suprasellar area [ figure 4a and b ] . she underwent reexploration by the same route , and a tiny fragment densely adherent to the middle cerebral artery was left behind . ( a and b ) showing remote recurrence five years after first surgery and radiotherapy . although craniopharyngiomas are benign tumors they are known to recur even after years and even after the administration of radiotherapy , recurrence rates ranging from 25% to 70% . recurrences at a site removed from the original site are very rare < 25 cases have been reported . these ectopic recurrences are not to be misinterpreted as ectopic primary occurrences since craniopharyngioma can occur anywhere along the obliterated rathke 's pouch . these ectopic recurrences may occur along the surgical pathway or at a site , not along the surgical pathway . the cells of the tumor may get implanted and may subsequently metamorphose into a fresh neoplasm . these tumor cells may in turn give rise to the regrowth of the tumor . the usual time to recurrence is around 4 years . but why this has to be a peculiarity of craniopharyngiomas can not be explained . another way the tumor may get seeded at a distant site the evidence for this is strong since tumor cells have been observed in the csf . although most recurrences are along the surgical corridor an instance where the recurrence has occurred in the spine has been recorded . when the transsphenoidal route is used , the csf spaces are not violated this may explain the absence of recurrences after transsphenoidal route . it is not surprising to observe that the histologic examination of the primary and recurrent lesions are the same . recurrences rates are said to be low after total surgical excision . but recurrences even decades after a quiescent period are well - known . it can be assumed that radiotherapy would have sterilized the surgical corridor and ectopic recurrences will not occur . but this was not the case in our patient , and in the few that have been reported . total excision is not an assurance that recurrences at ectopic or primary site will not occur . certain measures have been proposed to minimize these ectopic recurrences , protecting the operative field with patties to prevent seeding , emptying the cyst prior to removal of tumor , thorough irrigation of the field before dural closure . probably , a higher mib-1 index and expression of p53 may predispose to these recurrences . but eternal vigilance and regular imaging are mandatory to detect recurrences . another point worthy of note is why this phenomenon is not seen with respect to other benign tumors like meningiomas or even malignant tumors .

the aim was to present a rare case of recurrent craniopharyngioma remote from the primary site of origin . a young girl was operated for sellar region craniopharyngioma . for a small residual tumor , she underwent radiotherapy . follow - up imaging did not reveal any residual tumor or recurrence . surveillance magnetic resonance imaging after 5 years revealed a recurrence in the right sylvian fissure . this tumor was totally excised . recurrence of craniopharyngioma is well - known , but recurrence at a site remote from the original site after radiotherapy is extremely rare . one such case is being presented .

nearest neighbor approaches were developed to predict the folding stabilities of nucleic acid secondary structures ( 1 ) . these parameter sets utilize empirical rules , generally derived from optical melting experimental data , as the basis of the predictions . for rna , rules exist for predicting both free energy and enthalpy change of watson crick helices , gu pairs and loops ( 25 ) . parameters for dna have also been assembled for predicting watson crick pair free energy and enthalpy change and free energy changes of loops ( 6,7 ) . these parameter sets are the basis of computer programs that predict low free energy secondary structures . such programs include mfold / unafold ( 8,9 ) , the vienna rna package ( 10 ) , rna structure ( 2 ) , rnasoft ( 11 ) and sfold ( 12 ) . additional approaches that use statistical learning of parameters for rna folding have also used the rules from the nearest neighbor methods and derived new parameter values ( 13,14 ) . nearest neighbor parameter sets include both a set of rules , called either equations or features , for predicting stability and a set of parameter values used by the equations ( 14 ) . for rna , separate rules exist for predicting stabilities of helices , hairpin loops , small internal loops , large internal loops , bulge loops , multibranch loops , exterior loops and pseudoknots . given the number of rules and constraints on the length of journal publications , it is difficult to assemble all the parameters in one publication and provide meaningful tutorials for using the parameters . this is a barrier to software development for novel algorithms that could take advantage of the parameters . for example , many software packages that use rna parameters still implement the set of parameters assembled in 1999 ( 4 ) , in spite of the fact the rna parameters were updated in 2004 ( 2 ) based on experimental results . the nearest neighbor database ( nndb ) is a web - based tool for assembling and archiving complete nearest neighbor sets , including rules and values . currently , the 1999 and 2004 sets of rna folding parameters are provided ( 25 ) . the nndb is built using a set of static html , specifically xhtml 1.0 transitional pages with a page hierarchy shown in figure 1 . text is encoded in unicode ( utf-8 ) to facilitate display of equations in pages with diverse browsers running on diverse operating systems . the top - level page provides access to a help page , available parameter sets and a page of references to rna optical melting experiments . additionally , links provide downloading of the whole database in either zip or gzipped tar format . the help page introduces the purpose of the database and defines basic terms , including the set of structural features defined by secondary structures . for example , figure 2 , from the help page , shows an rna secondary structure that illustrates the loop features covered by nearest neighbor parameter sets . the basic equations for utilizing the parameters to extrapolate folding free energy changes to temperatures other than 37c and to predict melting temperatures are also provided . this figure illustrates the page hierarchy by following the linked pages down through the 1999 parameters and down to the hairpin loop pages . note that there are five example calculations for hairpin loops to illustrate the separate sequence - dependent rules that are used depending on the specific loop . figure 2.an rna secondary structure illustrating the types of features included in nearest neighbor parameter sets . internal loops have nucleotides not in canonical pairs on each of two strands , but bulge loops have nucleotides not in canonical pairs on only one strand . formally , a pseudoknot occurs when there are at least two pairs , with indices i paired to j and i paired to j , that satisfy the condition i < i < j < j. the pseudoknot helix is often considered to be composed of the fewest pairs that need to be removed to relieve the pseudoknot ( 19 ) . in this structure , this figure illustrates the page hierarchy by following the linked pages down through the 1999 parameters and down to the hairpin loop pages . note that there are five example calculations for hairpin loops to illustrate the separate sequence - dependent rules that are used depending on the specific loop . an rna secondary structure illustrating the types of features included in nearest neighbor parameter sets . internal loops have nucleotides not in canonical pairs on each of two strands , but bulge loops have nucleotides not in canonical pairs on only one strand . formally , a pseudoknot occurs when there are at least two pairs , with indices i paired to j and i paired to j , that satisfy the condition i < i < j < j. the pseudoknot helix is often considered to be composed of the fewest pairs that need to be removed to relieve the pseudoknot ( 19 ) . in this structure , the tan nucleotides are in pairs that could be removed to relieve the pseudoknot . for each set of parameters , for example , the 1999 rna rules predict only folding free energy changes ( 4 ) , but the 2004 rules can be used to predict both folding free energy and enthalpy changes ( 2,5 ) . for each structural feature , a page defines the basic equations and provides links to parameter values ( in plain text and html ) , references and tutorial pages ( e.g. figure 3 ) . the number of tutorials varies from feature to feature ; the set of tutorials is designed to cover each type of rule that can be encountered in practice . crick helix parameters are covered with two tutorials , one for self - complementary and one for non - self - complementary strands . these two tutorials also demonstrate the difference in the calculation when there are terminal au base pairs , which receive a free energy and enthalpy change penalty ( 3 ) , because the self - complementary duplex example has two terminal au pairs and the non - self - complementary case has no terminal au pairs . this tutorial demonstrates the prediction of folding free energy change for a hairpin loop of six unpaired nucleotides using the 2004 parameters ( 2,3 ) . an example tutorial from the database . this tutorial demonstrates the prediction of folding free energy change for a hairpin loop of six unpaired nucleotides using the 2004 parameters ( 2,3 ) . individual pages above the level of value tables have top banner , a left navigation bar that allows the user to navigate back up the hierarchy to any level above and a bottom bar with the date of last editing . for pages edited after the database has gone online , previous versions of the page are available using this bottom content bar . to facilitate indexing by search engines , all pages have a descriptive title , including the set of parameters to which it belongs ( if applicable ) . the first release of the nndb contains the rna folding rules assembled in 1999 and 2004 ( 25 ) . these rules represent the most recent set of parameters and a prior set that is widely used in software packages . because folding rules are derived to work as a set , the two versions of rules and values should not be mixed and the website hierarchy reinforces this . it is anticipated , for example , that additional pages will be written to include nearest neighbors for dna folding ( 6,7 ) and for predicting rna pseudoknot stabilities ( 1518 ) . additionally , the values derived from the re - estimation of the values of the 1999 parameter set using the set of known rna secondary structures will also be included ( 14 ) . the nndb is designed to provide a convenient location for assembling parameter sets for predicting the stability of nucleic acid secondary structures . it is modular in design , which facilitates its future expansion to contain additional parameter sets . furthermore , the web format makes it feasible to provide extensive tutorials for utilizing the parameters , which is generally not possible in print . the creation of the nndb was supported by united states national institutes of health grants gm076485 to d.h.m . and gm22939 to d.h.t . funding for open access charge : united states national institutes of health .

the nearest neighbor database ( nndb , http://rna.urmc.rochester.edu/nndb ) is a web - based resource for disseminating parameter sets for predicting nucleic acid secondary structure stabilities . for each set of parameters , the database includes the set of rules with descriptive text , sequence - dependent parameters in plain text and html , literature references to experiments and usage tutorials . the initial release covers parameters for predicting rna folding free energy and enthalpy changes .

mucocele of the appendix ( collection of mucus within the appendiceal lumen ) is a rare lesion , found in only 0.2% to 0.3% of 43,000 appendectomies reviewed . currently , the assessment of pelvic masses relies heavily on usg as the primary diagnostic tool . in such cases , clinical findings and other investigative modalities are warranted to aid the diagnostic process . in spite of extensive preoperative investigations , the diagnosis may still remain elusive and may only be made at the time of surgery . some regard this lesion as benign , a result of obstruction of the proximal lumen by fibrosis ; others believe it to be a neoplasm of the appendix . is the method of choice in the management of simple mucocele and for cystadenoma with an intact base . several studies ( mostly case reports ) on laparoscopic resection of mucocele have been reported . a 60-year - old female presented with pain in lower part of abdomen and palpable tender lump in the right ileac fossa . ultrasound of the abdomen reports a cystic mass of size 12 15 cm with thin internal septations in the right adnexa . the pneumoperitoneum was created with veress needle using carbon dioxide and the pressure was kept at 11 mmhg . a 0 telescope was introduced through the umbilical port for the complete examination of the abdomen . diagnostic laparoscopy revealed approximately 14 15 cm large bluish mucocele of the appendix with omental adhesions . two 5-mm ports were placed in the supra pubic area below the pubic hair line as the working port . the mucocele of the appendix was isolated after separating the mesoappendix from it with the help of bipolar cautery . following this , mucocele of the appendix [ figure 1 ] was retrieved out in a plastic bag through the umbilical port . hemostasis was obtained and a suction drain left in situ which was removed when non - productive . cut section showed appendix was filled with mucin - like material [ figure 2 ] . she was started orally after 4 hours of operation and solid food on the next day . appendicular lump from the distal portion of appendix after removal the appendicular lump filled with mucinus material mucocele of the appendix is a descriptive term for an appendix distended by mucus , secondary to mucinous cystadenoma ( 63% ) , mucosal hyperplasia ( 25% ) , mucinous cystadenocarcinoma ( 11% ) , and retention cyst . clinical presentation may include right lower quadrant pain , change in bowel habits , per rectal bleeding , or a palpable mass . approximately 23 - 50% of patients are asymptomatic , with the lesions being discovered incidentally during surgery , radiological evaluations , or endoscopic procedures . the preoperative clinical diagnosis of appendiceal mucoceles can therefore be difficult because of this lack of clinical symptomotology . the initial detection of the lesion may be facilitated by radiological , sonographic , or endoscopic means . on barium enema , the lesion may be seen as a sharply outlined sub - mucosal or extrinsic mass indenting the cecum and laterally displacing it . purely cystic lesions with anechoic fluid , hypoechoic masses with fine internal echoes as well as complex hyperechoic masses can be seen depending on the contents . ct of the abdomen usually shows a cystic well - encapslated mass sometimes with mural calcification , in the expected location of the appendix . it may be causing extrinsic pressure on the cecal wall without any surrounding inflammatory reaction . colonoscopic findings include the volcano sign , the appendiceal orifice seen in the center of a firm mound covered by normal mucosa or a yellowish , lipoma - like submucosal mass . in our case , usg was unable to provide a preoperative diagnosis . in our case , the decision for excision of the appendiceal mucocele was made as a result of diagnostic laparoscopy and a need to rule out malignancy . therefore mucocele of the appendix can mimic an adnexal mass and prove to be a diagnostic challenge . in a woman presenting with right iliac fossa mass and with clinical features not indicative of gynecological pathology , an appendiceal origin should be considered in the differential diagnosis . surgery is the treatment of choice and should be done early as tumor can not be ruled out as the causative factor for the mucocele . pre - operative diagnosis is important to avoid unintended rupture and the development of pseudomyxoma peritonei during surgery . however , laparoscopic dissection , grasping of the appendix specimen , pneumoperitoneum , or transport of the specimen through the abdominal wall might contribute to peritoneal dissemination of a tumor , if present . these setbacks can be avoided by taking precautions like using bowel holding graspers ( non - traumatic ) to handle the mucocele and using a non - permeable bag to deliver the specimen out of the port . mucocele of the appendix can mimic an adnexal mass and prove to be a diagnostic challenge . laparoscopic resection of mucocele of the appendix is feasible in spite of the danger of malignancy , provided necessary precautions are taken .

mucocele of the appendix is an aseptic dilatation secondary to obstruction . the preoperative clinical diagnosis of appendiceal mucoceles can therefore be difficult because of this lack of clinical symptomotology . surgical excision is the treatment of choice in benign mucocele . we report a case presenting to the surgeons where initial clinical findings and investigations suggested a cyst in the right adnexa . diagnostic laparoscopy revealed mucocele of the appendix and laparoscopic appendicectomy was done .

reactivation of latent tuberculosis ( tb ) is a serious hindrance to continuation of therapy . we present here a case of pleural tb in a patient on infliximab for ankylosing spondylitis . a 36-year - old male presented to our hospital in 2006 with low back ache inflammatory type with symmetric joint pains involving large joints such as shoulder joints , hip joints , knee joints , and small joints such as metacarpophalangeal joints , elbow joints , and metatarsophalangeal joints . there was associated early morning stiffness for over an hour . on examination , synovitis was present in peripheral joints with restriction of movement in all joints . modified schober 's test was positive , and the chest expansion ( <3 cm ) was restricted . on evaluation , he had anemia and elevated erythrocyte sedimentation rate ( esr ) ( 46 mm / h ) and c - reactive protein ( crp ) ( 18 mg / l ) . imaging revealed kyphoscoliosis of thoracic spine , syndesmophytes at multiple levels giving the appearance of bamboo spine [ figure 1 ] . diffuse ossification of interspinous and paraspinal ligaments with fusion of thoracic and lower cervical vertebra was present . radiograph showing bamboo spine in view of hla - b27 , more than 2 spa features and typical radiological features , ankylosing spondylitis was considered . he was started on nonsteroidal anti - inflammatory drugs ( nsaids ) and dose escalated for symptomatic relief . in view of persisting symptoms and elevated esr and crp in spite of optimal nsaids , following three doses of infliximab and 10 months after initiation of therapy , patient came with complaints of fever and cough for 1 week . on examination , breath sounds were reduced over the right side , and there was dullness on percussion . diagnostic thoracocentesis showed lymphocytic ( total leukocyte count - 4800 cells / cumm , lymphocytes - 90% ) exudative type of effusion with high adenosine deaminase ( ada ) ( 114 iu / l ) . according to light 's criteria ( effusion protein - 6 g / dl , serum protein - 3.37 g / dl , effusion lactate dehydrogenase ( ldh ) - 575 ankylosing spondylitis is a chronic , systemic , inflammatory disease that affects primarily the sacroiliac joints and spine . it is a spondyloarthropathy with a prevalence of 0.1%0.4% globally . data from india are sparse . its more commonly seen among males under 30 years of age . diagnosis is made after thorough clinical examination and radiography . infliximab is one such biologic which acts by inhibiting a pro - inflammatory cytokine tnf- and reducing inflammation . target - related adverse effects with tnf inhibitors are infections , opportunistic infections , malignancies , demyelinating conditions , hematologic abnormalities , congestive heart failure , autoantibodies ( antinuclear antibody and anti - double - stranded dna ) , hepatotoxicity , dermatologic reactions , and lupus - like syndromes , whereas the agent - related adverse effects are administration reactions and immunogenicity . tnf- is a cytokine that plays an important role in the mediation of inflammation and immune regulation . they are required for inflammatory response against intracellular organisms . in experimental models , fungal and bacterial infections pneumocystis carinii and histoplasma capsulatum are some of the fungal pathogens , whereas the bacterial agents are listeria monocytogene , mycobacterium tuberculosis , and mycobacterium avium . upper and lower respiratory tract infections are the most commonly seen ones . there was also an increased risk of serious infections compared with controls ( 3.6% vs. 1.7% ) . registries of rheumatoid arthritis patients have shown that the relative risk for infection ( 3.34.1 ) as well as serious infection ( 2.72.8 ) was significantly higher among patients receiving tnf inhibitors . to conclude , severity of disease , use of other medications such as corticosteroids , and the presence of comorbidities also contribute to infections in addition to tnf inhibitors alone . opportunistic infections following tnf inhibitor therapy include disseminated m. tuberculosis . in a study of seventy cases of tb following infliximab therapy , thirty were pulmonary tb and of forty were extrapulmonary disease , only two were cases of pleural tb . around a quarter of the cases were disseminated disease . the majority of cases of tb were observed within a median period of 12 weeks after initiation of therapy and is likely due to reactivation of latent tb . a study by grover et al . in india has shown a high incidence of tb ( 21% ) following biologic therapy . it was also seen that among those who received low doses of infliximab ( 3 mg / kg body weight ) did not develop tb . another study by malaviya et al . has found a lower incidence ( 9.4% ) of tb among those on tnf inhibitors . the rate of development of active tb among rheumatoid arthritis patients on anti - tnf therapy has dropped by 83% with the help of screening . suppressing the action of tnf- can help in relieving the symptoms of ankylosing spondylitis by reducing the inflammatory process , but at the same time , it weakens immune response to microbes such as tubercle bacilli . hence , meticulous screening and close monitoring of patients on infliximab for any symptoms and signs of tb are important as there is a risk even though the screening tests have come out be negative .

we present a case of pleural tuberculosis ( tb ) in a patient on infliximab for ankylosing spondylitis . a 36-year - old male presented to our hospital with low back ache of inflammatory type along with multiple symmetric inflammatory type of joint pain . further clinical examination , laboratory and radiological investigations were suggestive of ankylosing spondylitis . he was initially treated with nonsteroidal anti - inflammatory drugs but citing poor response it was decided to initiate biologic therapy using infliximab ( antitumor necrosis factor - alpha ) . mantoux test and chest radiograph were done before the therapy to rule out tb . following three doses of infliximab , patient came with complaints of fever and cough for 1 week . on investigation , it was found to be a case of pulmonary tb . this shows the importance of close monitoring of patient for tb among patients on infliximab even though the screening test has come out to be negative .

myoepithelial carcinoma ( mc ) is a rare tumor with an incidence of 0.2% of all salivary gland tumors . most of the reported cases of mc arise in the parotid gland ( 4875% ) , followed by minor salivary glands , and the submandibular gland . the first case was described by higashiyama et al . , in 1998 . since then , only seven cases have been reported in literature . a mentally retarded 13-year - old girl , with a history of congenital hypothyroidism and cystic lymphangioma in the left dorsal region , operated in 2004 , had consulted for cough and dyspnea in september 2010 . physical examination showed a decrease in vesicular breath sounds at the basal areas of chest bilaterally , without fever . pulmonary computed tomography ( ct ) showed bilateral pleural masses measuring 6.3 cm and 7.4 cm at the right and left bases , respectively , with lymph node metastases [ figure 2 ] . ( a ) bilateral pulmonary opacities ( b ) disappearance of pulmonary opacities pulmonary computed tomography ( ct ) : bilateral pulmonary masses a histopathological study of the left pleural biopsy revealed a monomorphic proliferation of round cells with clear cytoplasm and a weak , mitotic hyperchromatic oval nucleus [ figure 3 ] . there were some cohesive layers , separated by bands of sclerosis ; the stroma was sparse with foci of necrosis . the immunohistochemistry ( ihc ) study was focally positive for vimentin , cd99 , and ps100 . the abdominal ultrasound , bone scan , and metaiodobenzylguanidine ( mibg ) scintigraphy were normal . the histopathological and the ihc review at the institut bergoni in france concluded the diagnosis of myoepithelial carcinoma of the soft tissues with intermediate malignancy . ihc was positive for pancytokeratin ( ae1/ae3 ) and ps100 , and negative for ema , cd34 , desmin , and cd99 . the progression was marked by the disappearance of the pulmonary opacities [ figure 1 ] . they include mucoepidermoid carcinoma , adenoid cystic carcinoma , acinic cell carcinoma , oncocytoma , epithelial it arises from the submucosal bronchial glands of the lower respiratory tract . in the world health organization classification , published in 2004 , mc was cited as being synonymous with epithelial myoepithelial carcinoma . as mc and epithelial myoepithelial carcinoma of the salivary gland are distinguished by the presence or absence of ductal cells , their pulmonary counterparts must also be differentiated . myoepithelial tumors are low - grade lesions without recurrences or metastasis described after resection , whereas , the rate of metastasis in mc is high , as seen in our case . our patient represents the first pediatric case , described in the literature , having primitive pulmonary mc . the tumor was peripheral and bilateral , measuring 6 cm and 7 cm , which was in agreement with the literature . in fact , the size of the mc ranged from 15 mm to 130 mm ( mean 50.7 mm ) [ table 1 ] . characteristics of pulmonary mc in our case , the histopathological study oriented to the diagnosis of a primitive neuroectodermal tumor or neuroblastoma . the pathological review , with further ihc analysis in france , had concluded the diagnosis of mc . the following markers were found to be useful in myoepithelial carcinoma : cytokeratins ( ae1/ae3 ) and vimentin ( reported to be positive in neoplastic myoepithelial cells and negative in normal myoepithelial cells ) . other variable markers , such as , ps100 , calponin , smooth muscle actin ( sma ) , muscle - specific actin ( msa ) , smooth muscle myosin , and p63 protein , could be positive . however , neoplastic transformation of myoepithelial cells could result in a loss or a modification of their smooth muscle phenotype . in our case , although the eight patients reported in literature were treated with optimal surgery , metastases were reported in seven of them ( 87.5% ) ( contralateral lung , forearm , liver , and brain ) . it was significant that a patient who had not developed metastasis , had the lowest tumor mitotic rate of 5/10 hpfs . this fact reasoned that the mitotic rate could be an important prognostic factor of the clinical outcome and survival in primary myoepithelial carcinoma of the lung . given the rarity of these tumors , recommendations regarding chemotherapy or radiation , either pre- or postoperatively , are difficult to formulate . the originality of our case is the disappearance of the pulmonary opacity spontaneously , without any treatment . our case represents , to the best of our knowledge , the first pediatric case having primitive pulmonary mc . the histopathological study familiarizes the diagnosis , but a further ihc study is needed to confirm the diagnosis and to eliminate other etiologies . surgery represents the main treatment for the operable forms . to the best of our knowledge , we have reported the first case , with spontaneous regression of this tumor , without any treatment .

primary myoepithelial carcinoma ( mc ) of the lung is exceedingly rare . we report here , to the best of our knowledge , the first pediatric case having primitive pulmonary mc . the originality of our case was the disappearance of the pulmonary opacity spontaneously , without any treatment . the difficulties in our case were the diagnosis of this rare entity and its subsequent treatment . in fact , given the rarity of these tumors , recommendations regarding chemotherapy or radiation , were difficult to formulate .

amisulpride came into the indian market a few years back with hypes and hopes in the management of schizophrenia . its broad spectrum effectiveness with lower chances of extrapyramidal symptoms ( eps ) and metabolic syndrome did help psychiatrists to treat schizophrenia and related disorders more effectively . although this antipsychotic does not block serotonin receptors at all , it is a high - affinity and highly selective d3/d2 receptor antagonist with atypical properties . its selective affinity for dopamine receptors in the limbic structures , but not in the striatum , leads to a low risk of extrapyramidal side effects . all available reports suggest that chance of eps is very less with amisulpride at doses < 400 mg / day . however , there are sporadic reports of drug - induced eps including dystonia and akathisia even in patients receiving low doses of amisulpride . here a 30-year - old male with schizophrenia for the past 10 years now presented with predominantly negative symptoms . he was on olanzapine 15 mg / day for more than 6 months without much improvement . hence , amisulpride was instituted with a starting dose of 50 mg / day with a gradual increment up to 300 mg / day within 14 days . the patient came after 14 days to the casualty with features of parkinsonian syndrome such as slowed gait , mild rigidity , salivation , and bradykinesia . he was hospitalized , amisulpride was immediately stopped , and trihexyphenidyl 4 mg / day was given to manage the side effect . his eps gradually subsided and for negative symptoms , clozapine was introduced at a small dose of 25 mg / day and gradually increased to 200 mg / day over a period of 10 days . at the time of discharge , on the 14 day , he was free from parkinsonian symptoms . subsequent follow - up showed no parkinsonian symptoms and he had modest improvement in negative symptoms . a 48-year - old male with schizophrenia for the last 20 years was treated with various antipsychotics without much improvement . since the last 6 months , he was on olanzapine 15 mg / day . as there was no significant improvement , his olanzapine dose was gradually tapered and stopped over a period of 14 days and was started on amisulpride 100 mg / day and was increased to 200 mg / day over a period of 3 weeks . the patient returned on the 24 day with severe parkinsonian symptoms . in this patient also , there was no prior history of parkinsonism . we managed him with injection promethazine 25 mg intramuscular bid first 3 days along with trihexyphenidyl 2 mg bid after stopping amisulpride . after 7 days , parkinsonian symptoms improved considerably and clozapine was introduced at a dose of 25 mg / day which was subsequently increased to 100 mg / day on the 10 day and the patient was discharged . since the discovery that clozapine induces fewer eps and is more effective for negative symptoms than conventional antipsychotics for the treatment of schizophrenia , psychopharmacological research has focused on the development of drugs that block central 5-ht2 receptors more than d2 receptors . combined 5-ht2/d2 receptor antagonism is the most current explanation for the so - called atypical profile of some antipsychotics . amisulpride at low doses binds selectively to dopamine d2 , d3 autoreceptors , thereby enhancing dopaminergic transmission and thus might be effective for negative symptoms . it has no affinity for d1 , d4 , and d5 receptor subtypes . at higher doses , it blocks postsynaptic receptors , thus inhibiting dopaminergic hyperactivity . at the same time , amisulpride has greater specificity for the limbic system and thus has low incidence of eps . amisulpride binds more loosely than dopamine to the dopamine d2 receptor and is rapidly dissociated from the dopamine d2 receptor . low - dose therapy with amisulpride is associated with a significantly lower blockade of striatal dopamine d2 receptors than is seen during high - dose treatment . however , a significant striatal d2 blockade was demonstrated at therapeutically effective dose ranges , and a good relationship between the degree of striatal dopamine d2 receptor occupancy and the amisulpride plasma concentration or the administered dose was shown . in general , asians are slow metabolizers . low body weight and slow metabolism may increase the plasma concentration of drugs causing side effects . reported a low postsynaptic d2 occupancy in the striatum at low doses of amisulpride ( 50100 mg / day ) . it has also been suggested that extrastriatal binding could mediate the effect on negative symptoms . the probable causes of eps with low doses of amisulpride could be that it blocks postsynaptic d2 receptors significantly in striatum without much effect in the mesolimbic pathway . , the lower incidence of eps which is claimed by western researchers as well as pharmaceutical companies should be studied well in the indian context . we should at least keep this side effect in our minds while starting or increasing the doses .

amisulpride , recently introduced atypical antipsychotic , is well - known for its broad spectrum effectiveness and lower profile for extrapyramidal side effects ( eps ) . its selective affinity for dopamine receptors in the limbic structures , but not in the striatum , leads to a low risk of extrapyramidal side effects . here , we report two cases of eps associated with lower dose of amisulpride . the proposed mechanism for its causation is also discussed . authors invite more studies , specifically from the indian context to find out the incidence of eps and other associated side effects .

thirty patients with zygomatic complex fractures were treated with one point fixation [ figures 13 ] . preoperative peripheral nerve stimulation x - ray preoperative computed tomography scan under general anesthesia , nasoendotracheal intubation was done . the incision can be made from anterior to posterior or from medial to lateral and should extend through mucosa , submucosa , and any buccinators muscle fibers [ figure 4 ] . rowe 's zygomatic elevator was then inserted behind the infra temporal surface of the zygoma , and bone was reduced into its correct anatomical position using superior , lateral and anterior force . an audible click and fullness of the cheek together with palpation for normal contour of the zygomatic bone and orbital rim gave an idea about the adequacy of the reduction . one hand over the side of the face was used to assist in the reduction . a four hole plate with a gap was fixed with 4 mm 2.5 mm screws on the zygomatic buttress [ figure 5 ] . immediate post operative immediate peripheral nerve stimulation x - ray six months postoperative and peripheral nerve stimulation x - ray for all the patients , immediate postoperative and 6 months postoperative peripheral nerve stimulation x - rays were taken , and the x - rays review successful reduction . none of the patients complained of any paresthesia , bony movements or pain in the frontozygomatic or zygomatic buttress region . since intraoral approach was used , all the patients had an aesthetic facial profile without any unsightly scars . the integrity of the zygoma bone is critical in maintaining normal facial width and prominence of the cheek . the zygomatic bone is a major contributor to the orbit and plays an important role in protecting the eyes . zygomatic bone alone is rarely involved in fractures ; usually its articulating surfaces which are maxilla , temporal , frontal and sphenoid bones are also involved . the fractured fragments of a tripod or tetrapod zygomatic complex fracture near these suture lines needs to be restabilized by open reduction followed by fixation . studies suggest that two point gives a considerable stabilization , and three point fixation gives the maximum stabilization . however other studies suggest that one point fixation for zymatic complex fractures gives an excellent results considering the esthetics and stabilization for simple tripod fractures without any comminution of the zygomatic bone or the lateral orbital wall one point fixation with a single mini plate in the frontozygomatic area through the lateral eyebrow incision have been suggested by many authors . i n these cases it was found that when a tripod fracture without any comminution or mild or no displacement can be stabilized very well with a single point fixation in the frontozygomatic area without any complications of diplopia or enopthalmos . however , zygoma provides the attachment point for muscles of mastication and facial animation , but amongst these , it is the masseter that provides the most significant intrinsic deforming force on the zygomatic body and arch . the integrity of zygomatic buttress is necessary for withstanding the contraction force of the masseter muscle . in 2002 fujioka et al . in vivo studies successfully proved that one point fixation at the zygomaticomaxillary complex gives three point alignment and sufficient rigidity when the fractures are not comminuted . in 2011 kim et al . found out that lateral eyebrow incision for mini plate fixation at the frontozygomatic area led to unaesthetic scar and few patients underwent plate removal through a second surgical re - entry through the existing scar of the lateral eyebrow incision which further enhanced the unsightly scars and compromised facial esthetics . since the skin over the lateral eyebrow region is thin there are more chances of palapation of the mini plates after fixation , and it may lead to pain . as early as in 1994 tarabichi et al . proved that in vitro studies are misleading regarding the mini plate fixation along the orbital margins and successfully applied transsinus reduction through anterior comminuted sinus wall . in 2012 kim et al . successfully reduced the zygomatic complex fractured fragments through intraoral approach and gained sufficient rigidity and excellent esthetics with one point fixation at the zygomatic buttress region . we also found that one point fixation with a single mini plate at the zygomatic buttress through intraoral incision provided excellent stability and esthetics in the selected cases of simple zygomatic complex fractures without any comminution of the zygoma or the lateral orbital rim without or with minimal displacement and none of our patient complained of pain or palpation or bony movements in the postoperative study period of 6 months rather they were happy to get operated without any unaesthetic facial scars .

for decades , facial beauty and esthetics have been one of the most important quests of the human race . the lateral prominence and convexity of the zygomatic bone makes it the most important bone for providing the aesthetic facial look and sets up the facial width but at the same time this prominence and convexity makes this bone more vulnerable to injury . zygomatic complex fractures or tripod fractures are the second most common fractures after nasal fractures among facial injuries . several studies have been undertaken regarding the reduction and fixation of zygomatic fractures with mini plates and screws . in 2002 fujioka et al in vivo studies successfully proved that one point fixation at the zygomaticomaxillary complex gives three point alignment and sufficient rigidity when the fractures are not comminuted . in this article , 30 cases have been reviewed with one point fixation of zygomatic complex tripod fractures at the zygomatic buttress through keen 's intraoral approach along with advantages and disadvantages .

sotos syndrome is a dysmorphic syndrome characterized by early overgrowth , developmental delay , advanced bone age and characteristic craniofacial appearance . sotos syndrome results from mutation involving the nuclear receptor set - domain - containing protein ( nsd1 ) gene , located on chromosome 5q . the mutational mechanism can be a point mutation in the nsd1 gene or a microdeletion that includes nsd1 . fluorescence in situ hybridization ( fish ) did not detect microdeletion of 5q35 in this patient . nsd1 gene mutations are also found in weaver syndrome where camptodactyly is a common feature . this report describes camptodactyly for the first time in a girl with sotos syndrome and provides further evidence that sotos and weaver syndrome are allelic disorders . we describe a two and half years old girl born of non - consanguineous tamilian parents . milestones were delayed with head holding at 6 months , walking at 2 years and delayed speech . family history was unremarkable . at two and a half years she weighed 15 kg , height was 97 cm and head circumference was 52.5 cm ( all above 90 centile ) . she had a high forehead with frontal bossing , dolichocephaly , large ears , pre - auricular pits , down - slanting palpebral fissures , high arched palate , pointed chin and pectus carinatum [ figure 1 ] . she had three caf au lait spots distributed on the chest and trunk , large hands and camptodactyly of the left hand [ figure 2 ] . fish studies carried out using probe rp11 - 265k23 did not reveal a microdeletion of the 5q35 region . face showing the facial features of sotos syndrome left hand showing camptodactly a clinical diagnosis of sotos syndrome was made based on the criteria comprising of rapid early growth , advanced bone age , developmental delay and characteristic facial appearance . camptodactyly in sotos syndrome has not been previously described in literature to the best of our knowledge . a high frequency of congenital heart defects has been reported in patients with intragenic mutations of the nsd1 gene and phenotypic overlap with other overgrowth syndromes , in particular with weaver syndrome is seen . though this sotos patient is likely to have a point mutation of the nsd1 gene , this could not be confirmed due to lack of facilities . sotos syndrome was first recognized as a distinct clinical syndrome in new england in 1964 . the diagnosis is based on the clinical criteria of rapid early growth ( pre and post natal ) , advanced bone age , developmental delay and characteristic facial appearance . growth is rapid in the first years of life but final height may not be excessive . intellectually , the iq ranges from 21 to 103 with a mean of 74 and almost half of affected children achieve normal schooling . behavioral issues are common and are one of the key areas that can influence the outcome . hypotonia is usually present from birth and although this improves during childhood , subtle evidence may remain even in adults . congenital heart disease is not very common in this condition and overall incidence of cardiac defects is approximately 8% . an association of sotos syndrome with tumor development was documented over 30 years ago and has been a point of debate ever since . gorlin et al . estimated a risk of 3.9% of benign or malignant tumors in sotos syndrome . handicaps in sotos syndrome are fewer than previously believed and tend to improve with age . in patients with sotos syndrome harboring a chromosomal translocation kurotaki et al . isolated the nuclear receptor set - domain - containing protein ( nsd1 ) gene from the 5q35 breakpoint . added noted a large difference between japanese and non - japanese patients in the frequency of microdeletions , which occurred in 49 ( 52% ) of the 95 japanese but in only 1 ( 6% ) of the 17 non - japanese . there was a strong correlation between presence of an nsd1 alteration and clinical phenotype , in that 28 of 37 ( 76% ) patients with typical sotos or sotos - like phenotype had nsd1 mutations or deletions . studied nsd1 gene in a series of typical sotos patients ( 23/39 ) , sotos - like patients ( lacking one major criteria , 10/39 ) and weaver patients ( 6/39 ) . they conclude that nsd1 mutations account for most cases of sotos syndrome and a significant number of weaver syndrome cases . comparing the clinical phenotype of children carrying either a deletion or a mutation , rio et al . failed to detect distinctive features except for the severity of mental retardation . they reported , 4/6 children carrying a nsd1 deletion were extremely severely mentally retarded with no language at all , major delay in motor milestones and autistic features . by contrast , in patients carrying nsd1 mutations , mental retardation was usually mild to moderate with verbal skills being more affected . the major differential diagnoses for sotos syndrome are other conditions with overgrowth including beckwith - weidemann , weaver , nevo and simpson golabi behmel syndromes . . however nsd1 gene mutations have been found in beckwith - weidemann syndrome , weaver syndrome and the 11p15 abnormalities seen in beckwith - wiedemann syndrome have been found in some cases of sotos syndrome . camptodactly has not been previously described in literature to the best of our knowledge with sotos syndrome but is seen in weaver syndrome where nsd1 mutations have been described . these could be due to , allelic heterogeneity , effect of other modifying genes ethnic background and nutritional status adding to the overall expression of a syndrome . microdeletions of chromosome 5 were not detected in our case suggesting a likely point mutation in the nsd1 gene and further evidence of that weaver and sotos syndrome are allelic . further delineation of the phenotype with molecular studies will provide correct genotype - phenotype correlations .

we describe a girl with sotos syndrome presenting at two and a half years age with developmental delay . she has camptodactyly which has not previously been reported in sotos syndrome but is a common finding in weaver syndrome . both these conditions have been reported to have nsd1 gene mutations . this report is consistent with the conditions being allelic .

in march 2013 , cases infected with a novel reassortant avian - origin influenza a ( h7n9 ) virus emerged in china and had high mortality . that month , a patient with h7n9 influenza was admitted to our hospital , and daily lung ultrasound was performed . a 54-year - old woman , who ran a convenience store beside a poultry market , complained of cough and high fever for 4 days . her temperature was 38.6c , and she had a heart rate of 113 beats per minute and a respiratory rate of 26 breaths per minute . her white blood cell count was 2.7 10/l , and neutrophil , lymphocyte , and monocyte levels were 72.4% , 22% , and 5.2% , respectively . her partial pressure of oxygen in arterial blood was 72 mm hg , and her fraction of inspiratory oxygen ( fio2 ) was 40% . avian - origin influenza a ( h7n9 ) virus was confirmed from the pharyngeal swabs by real - time reverse transcriptase - polymerase chain reaction . treatment with oseltamivir ( 150-mg capsule taken by mouth twice a day ) was initiated , and she was admitted to an isolated room in the infectious diseases department . bothell , wa , usa ) with c60 convex probe ( 2 to 5 mhz ) was performed , and the lung ultrasound score ( lus ) was recorded , and both effectively reflected the progression of pneumonia ( figure 1 ) . dynamic changes of chest computed tomography ( ct ) , radiography , and lung ultrasound in a patient with h7n9 influenza . ( a - c ) ct showed pneumonia in the left upper lung , with partial consolidation on admission . ( d - f ) lung ultrasound corresponding to ct in ( a - c ) showed multiple abutting b2 lines , and some regions presented a tissue pattern ( arrow ) . ( g - i ) ct on day 6 after admission showed that the pneumonia was partially absorbed . ( j - l ) lung ultrasound corresponding to ct in ( g - i ) showed that the number of b lines was obviously decreased , and the consolidation disappeared . ( m - o ) ct on day 9 after admission showed that the size of the lesion was obviously reduced . ( p - r ) lung ultrasound corresponding to ct in ( m - o ) indicated that only the a line and few b lines were visible . ( s - u ) chest radiography on days 1 , 3 , and 6 after admission showed no obvious change of the pneumonia . ( v ) dynamic changes of lung ultrasound score ( lus ) ( total of 48 for normal lung ) . the onset of h7n9 influenza in this case was manifested by hyperpyrexia and flu - like symptoms and progressed to lobar pneumonia 4 days later . chest radiograph is the routine tool for assessment of pneumonia , but its sensitivity and accuracy were not so good . ct is regarded as the gold standard , but its application is limited in cases with this new emerging virus since strict protection should be followed to avoid person - to - person transmission . ultrasound has many advantages , including convenience , rapidness , non - invasiveness , availability for repeated examination , and absence of radiation . the right lung presented an a line with few isolated b lines , whereas the left lung presented multiple abutting b lines and consolidation . when the patient improved , both b lines and the area of consolidation were decreased and the lus was synchronously increased . this case highlights that ultrasound can be an adjutant to chest radiography and ct in caring for patients with h7n9 influenza . written informed consent was obtained from the patient for publication of this letter and accompanying images .

h7n9 influenza is a new emerging infection and has high mortality . both chest radiography and computed tomography ( ct ) had some limitations in assessing such patients . we performed daily lung ultrasound in a patient with h7n9 influenza . lung ultrasound and lung ultrasound score showed high consistency with ct and the progression of pneumonia . ultrasound can be adjutant to chest radiography and ct in caring for patients with h7n9 influenza .

a 23-year - old engineering graduate presented with primary palmoplantar hyperhidrosis , for which he was advised an alternate day schedule of tap water iontophoresis . on his next visit , he presented with a very simple iontophoresis device that he devised on his own . the device was constructed with a rechargeable 12 volt battery , two aluminum trays and copper wires , and connecting clamps [ figure 1 ] . hence , using his engineering background he constructed this simple device based on basic mechanism behind iontophoresis . he followed an alternate day schedule of 20 min utes immersion for initial 4 weeks , followed by once a week for next 8 weeks . he achieved an excellent reduction in palmoplantar sweating without any adverse effect , within 3 months of starting iontophoresis . a simple user - made iontophoresis device iontophoresis is defined as passing of an ionized substance through intact skin by application of direct current ( dc ) . tap water iontophoresis is a reliable and effective method for the treatment of palmar and plantar hyperhidrosis , when practiced with appropriate technique and timing . many dermatologists consider simple tap water iontophoresis to be first line therapy for primary focal palmar and plantar hyperhidrosis . the mechanism of production of anhidrosis is not completely understood ; however , obstruction of sweat duct has been suggested as a possible cause . few brands of iontophoresis devices are commercially available ; however , they are expensive and are not readily available . commercially available construction of an iontophoresis device has been described by levit , in which output of the 115 volt isolation transformer is rectified by the full wave selenium rectifier and then filtered by the choke and capacitors , and the potentiometer acts as a voltage divider . levit had suggested that such devices can be constructed at home , and circuit diagram could be found in his original report , but we feel that the procedure for assembling such a device will be difficult for a layperson . for tap water iontophoresis , patients are instructed to apply petroleum jelly with a cotton swab to cover any cuts over the treated area before the session . the trays should be filled with tap water , then , the affected areas should be immersed in the tap water . a monday - wednesday - friday schedule should be followed until the condition improves ; subsequently , the treatment should be tapered once a week for 8 weeks and then once a month for maintenance . additionally , the patient should wear rubber or plastic footwear and should keep himself from directly touching the floor . burning and pin pricking sensations are very common and erythema and vesiculation are transient ; topical corticosteroids cream can be applied for persistent erythema and vesiculation . pregnant women , people with pacemaker or metal implants , cardiac conditions , or epilepsy are contradictions for the use of iontophoresis machine . once a home device is obtained and the patient has received adequate education and training , the maintenance cost and effort are minimal for the patient and health care provider alike . iontophoresis machines basically produce a voltage sufficient to drive a dc of 15 - 20 ma through the hands of patients . an ampere - meter could be used to measure the output current of such user - made devices and can upgrade the voltage of the battery , provided the output is low in terms of current . simple user - made devices such as this one would make the process of iontophoresis very easy , safe , and cost - effective . there are no conflicts of interest .

iontophoresis is defined as passing of an ionized substance through intact skin by application of direct electric current . tap water iontophoresis is reliable and effective method for treatment of palmar and plantar hyperhydrosis when practiced with appropriate technique and timing . one of the major setback for using iontophoresis is that the apparatus is expensive and is not readily available . a simple user - made iontophoresis device have been described here , which could be easily constructed and used at home .

a 61-year male presented to the emergency department with a history of road traffic accident . he arrived hemodynamically stable with a blood pressure of 126/76 mmhg and a heart rate of 78 beats per minute . on plain radiograph ( fig . 1a ) anteroposterior and two judet 45 oblique view1 ) and computed tomography ( ct ) scan of pelvis ( fig . 1b ) , the findings revealed both column fracture of acetabulum without hip dislocation , but no presence of femoral head fracture or onfh . buttress plating through ilioinguinal approach was performed using a reconstruction plate , which was supplemented by a compact hand plate . the patient was transfused 8 units of whole blood , 3 units of fresh frozen plasma and 8 units of packed red blood cells . intra - operative hb was 9.7 gm% ; the average mean arterial pressure was 91.82 mmhg during the operative procedure . post - operatively the patient was transfused 2 units of whole blood and 1 unit of fresh frozen plasma . the hb postoperatively was 10.3 gm% , the patient was shifted to intensive care unit for a day , later was transferred to the ward . the post - operative x - ray ( fig . 2a ) and ct scan revealed an acceptable reduction of the fracture fragments and a concentric hip . sitting up was performed on the first postoperative day ; the patient subsequently began formal physical therapy and active range of motion exercises . partial toe touch weight bearing ( 20 to 30 lb ; 9 to 13.6 kg ) with a walker was maintained for 6 - 8 weeks . progression to full weight bearing was started on the basis of the follow - up radiographs . the patient 's 4-month postoperative x - ray revealed a radiolucent lesion in the superolateral part of femoral head , crescent sign , and sclerosis . 2b ) showed collapse and sclerosis , findings consistent with onfh . a ct scan ( fig . on was diagnosed only when the radiographic findings provided a clear differentiation from wear of the femoral head2 ) . the joint pain increased due to the onfh , we performed a total hip replacement ( fig . 3b ) 12 months after the index surgery . late complications of acetabulum fractures include heterotopic ossification and onfh , which are present in less than 10% of the population3 ) . the incidence of onfh is known to be high in transverse and posterior wall fractures associated with posterior dislocation6 ) . on also occurs in conjunction with approximately 3% of anterior hip dislocations and in more than 13% of posterior hip dislocations . in a recent meta analysis of 3,670 surgically treated displaced acetabular fractures the incidence of onfh showed an overall incidence of 5.6%3 ) , suggesting that it is grossly overestimated and that most of the observed changes in the head of the femur are probably due to osteoarthritis5 ) . onfh is caused by inadequate blood supply to the affected segment of the subchondral bone . when posterior surgical approaches have been used , on rates as high as 42% within the first year after surgery have been reported7 ) . many systemic conditions are associated with on , but 25% of all cases are described as idiopathic and can contributes as a cause9 ) . trauma is one of the most common causes of on , interruption of the blood supply to the affected segment of the bone being the cause of ischemia . in this case the exact cause of onfh eludes us , especially in the absence of any patient related predisposing risk factors , except presence of fracture without hip dislocation and subsequent intervention by an ilio - inguinal approach . a probable theory of etiology could be the intra - operative hypovolaemia , low mean arterial pressure , causing compromised flow to the femoral head being so as to act as the final blow . alteration of the blood supply to vital organs during hypovolaemia is well established . with mean arterial pressure usually in the range of 50 to 60 mmhg , the flow to the femoral head is potentially compromised10 ) so as to act in an accumulative stress theory , as suggested by kenzora and glimcher9 ) . it is questionable as to whether this alone would be enough to explain the development of on .

osteonecrosis in isolated fractures of the acetabulum without dislocation of hip seems to be a known complication , but to our knowledge it has not been reported adequately . the causative nature of post - traumatic femoral head osteonecrosis has not been studied critically . the pathophysiology of osteonecrosis in this case also eludes us . striking evidence points towards the intra - operative blood loss and low mean arterial pressure possibly leading to hypo - perfusion of femoral head leading to osteonecrosis . fractures of the acetabulum pose a difficult problem for the patient and the surgeon because of possible complications . thus any surgeon involved in surgery for fractures of the acetabulum should be aware of the possibility of this potential complication . here is a 61-year male , who sustained a complex fracture of the acetabulum without hip dislocation , subsequently was treated surgically with internal fixation using an anterior approach , 10 months after surgery patient developed osteonecrosis of the femoral head .

pericardiocentesis is an invasive procedure which is usually performed in a patient who has pericardial effusion to resolve the pressure in the pericardial sac . in 1653 , riolanus ( 1 ) first described as a trephination of the sternum to relieve fluid surrounding the heart . due to frequent complications this procedure was out of interest until ultrasound guided technique emerged ( 2 ) . herein , we report a case of iatrogenic tension pneumopericardium , which exhibit impending cardiac arrest . a 70-year - old male presented with severe dyspnea and general weakness on march 8 , 2013 . he was referred from a local hospital for pericardiocentesis and further work - up due to a large pericardial effusion . he had a past medical history of ischemic stroke 15 years prior to his visit to the hospital and of syncope a day before his visit to the hospital . the vital signs of the patient in the emergency room ( er ) were as follows : blood pressure of 163/93 , heart rate of 135 beats per minute , respiratory rate of 30 breaths per minute , body temperature of 37.0c , and oxygen saturation of 91% . the patient had distended neck veins and muffled heart sounds but did not have low blood pressure . echocardiography was performed by an emergency physician , and a right ventricle ( rv ) free wall collapse in was observed in the diastolic phase . an emergency pericardiocentesis was indicated , and it was performed by a subxiphoid approach . approximately 700 ml of serous effusion was drained through a catheter ; the patient s heart rate dropped to 110 and his blood pressure was maintained above 130 . a water - sealed chest tube bottle was connected at the end of the catheter for further drainage . the patient was transferred to a computerized tomography ( ct ) room for a chest ct to examine the cause of his pericardial effusion . before he left after the patient returned to the er from the ct room , his heart rate fell to 30 , and he exhibited impending cardiac arrest . an intravenous dose of 0.5 mg atropine was given , and the patient was hydrated with crystalloid solution . the patient s chest ct confirmed tension pneumopericardium , and imaging showed that pericardial air was compressing the right ventricle and that the catheter tip was placed behind the left ventricle ( lv ) ( fig . 1 ) . approximated 500 ml of pericardial air was evacuated rapidly through the previously implanted catheter , and the patient s vital sign became stable . the patient was admitted to the intensive care unit and subsequently transferred to a long - term care hospital . a contrast - enhanced computed tomography scan of the chest . ( a ) axial view showed the air compressing the right ventricle ( arrows ) and tip of the catheter inside pericardium ( arrow head ) . pneumopericardium is defined as the presence of air inside the pericardial space . in 1910 , wenkebach first described the x - ray findings of pneumopericardium , and in 1967 , cimmino ( 6 ) described the diagnostic features of pneumopericardium . in a review of the literature , toledo et al . ( 7 ) classified the etiology of pneumopericardium into four categories : iatrogenic , pericarditis , fistula formation between the pericardium and an adjacent air - containing organ , and trauma . ( 8) reported that trauma and positive pressure ventilation are the major causes of pneumopericardium . iatrogenic cases of pneumopericardium associated with various procedures , such as thoracentesis , paracentesis , poststernal bone marrow aspiration , postcauterization of esophageal webs , pericardiocentesis , radiofrequency ablation , and pacemaker insertion , have been reported ( 7910 ) . if the patient has tension pneumopericardium , physical examination may show tachycardia , distended neck veins , and hypotension . in 1844 , bricheteau was the first to describe the mill wheel murmur in auscultation , which is a characteristic churning or splashing auscultatory sound due to blood mixing with air in the pericardial sac . a chest x - ray showing air surrounding the heart and the small heart sign pericardial air can be mistaken for mediastinal air , but the air in the pericardial sac does not rise above the pericardial reflection of the proximal great vessels . also a chest x - ray taken from the decubitus position may show a shift of air if the air is in the pericardium , but mediastinal air does not shift ( 12 ) . a small amount of air in the pericardial sac is indistinguishable by plain chest x - ray but can be detected by ct scan . bedside echocardiography in the er is an alternative method for detecting air in the pericardial sac , but a large amount of air may hinder the penetration of the ultrasound beam , so diagnosis by this method may be challenging . the needle should be placed 1 cm inferior to the left xiphocostal angle at a 30-degree angle to the skin . it should be aimed toward the left shoulder and advanced while maintaining negative pressure ( 13 ) . after the insertion of the catheter , a drainage tube is connected to a water sealed device . in our case , a conventional chest tube bottle was used , and during transportation to the ct room , the drainage device was not clamped . , the water in the bottom of the bottle could have swung back and forth . originally , the tube connected to the catheter would have been sealed with water , but this swinging motion would have provided the opportunity for air to enter the catheter and , subsequently , the pericardial sac . iatrogenic pneumopericardium is rarely reported after pericardiocentesis , but it can lead to tension pneumopericardium , which is a life threatening condition . physicians should be aware of this serious complication of pericardiocentesis and take extra precautions in handling drainage devices because this iatrogenic complication can lead to cardiac arrest and a medical dispute .

pneumopericardium is defined as the presence of air inside the pericardial space . usually , it is reported as a complication of blunt or penetrating chest trauma , but rare iatrogenic and spontaneous cases have been reported . pneumopericardium is relatively stable if it does not generate a tension effect on the heart . however , it may progress to tension pneumopericardium , which requires immediate pericardial aspiration . we report a case of iatrogenic pneumopericardium occurred in a 70-year - old man who presented dyspnea at emergency department . the patient underwent pericardiocentesis for cardiac tamponade due to large pericardial effusion , and iatrogenic tension pneumopericardium occurred due to misuse of the drainage device . after evacuating the pericardial air through the previously implanted catheter , the patient became stable . we report this case to increase the awareness of this fatal condition and to help increase the use of precautions against the development of this condition during emergency procedures .

she was referred to saitama hospital due to severe headache and nausea on october 2008 . brain mri detected a 1.5 cm abscess mass with extensive edema in the right frontal lobe . we performed intensive therapy using some antibiotics that included cefotaxime and meropenem and depressants for intracranial pressure for six weeks . there was a good prognosis for the woman and her fetus without any sign of neurological abnormalities . early medical intervention is required before it is too late for brain abscess in pregnancy . brain abscess caused by bacterial infection has extremely low incidence , and a high mortality rate of 30% . it causes poor prognosis for both mother and fetus , regardless of the state of pregnancy . unlike non - pregnant women a 24-year - old woman who lived in saitama , japan had three pregnancies , two childbirths , body mass index ( bmi ) of 22.3 , and unremarkable past medical and family histories . she also had an uneventful first trimester , but developed a fever of > 39c at 22nd week , 1st day of pregnancy . because of prolonged headache and nausea , she was referred to our hospital in saitama for complete physical examination on october 2008 . on admission , she had blood pressure of 103/51 mmhg , heart rate of 100 beats per min ( bpm ) , body temperature of 39.0c , mild stiffness in the neck , and cold extremities . however , brain computed tomography ( ct ) for the prolonged headache revealed a 1.5 cm mass in the right frontal lobe , while hematological analysis showed an elevated white blood cell count of 12,400 cells/l ( neutrophils , 87.7% ) . cerebrospinal fluid findings were positive for gram - positive bacteria , an increased cell count ( especially for neutrophils ) of 2,332 cells/l , and a low glucose concentration of 30 mg / dl . brain mri revealed a 1.5 cm mass with a high intensity signal inside and a low intensity t2 signal on the margin in the deep white matter of the right frontal lobe . based on the above findings and a high intensity zone surrounding the mass on diffusion - weighted images , she was immediately placed on intensive therapy with concurrent administration of antibiotics cefotaxime ( 2 g / day ) and meropenem ( 3 g / day ) , as well as glycerin 20 g / day to reduce intracranial pressure . table 1 shows a list of examinations performed in search of causal factors , while the results show the isolation of methicillin - sensitive staphylococcus aureus ( mssa ) from the throat . on the other hand , she had no dental problems . because of unremarkable upper gastrointestinal endoscopy findings and a negative fecal occult blood test result , the possibility of brain metastasis of a malignant tumor was ruled out . after six weeks of intensive therapy with concurrent administration of two antibiotics and glycerin , the headache and nausea disappeared along with a reduction in the number of white blood cells . subsequent brain mri at 28th week , 4th day of pregnancy showed no enlargement of the abscess and disappearance of the surrounding edema , with no indication of puncture drainage . at this point , she was switched to oral administration of amoxicillin 750 mg / day for four weeks and was discharged at 29th week , 3rd day of pregnancy . body temperature slowed down after the day 7 causes of brain abscess mssa : methicillin - sensitive staphylococcus aureus , cns : coaglese negative staphylococcus she vaginally delivered a 2,890 g girl baby at 38th week , 5th day of pregnancy , with no abnormalities . no neurological abnormalities were evident during a five - year follow - up observation conducted over the phone . mri findings at the 22nd and 28th week of pregnancy are shown in figure 2 . a : axial image at 22ne week of pregnancy shows the large right frontal abscess with severe edema . b : axial image at 28th week of pregnancy shows no enlargement of the abscess and disappearance of the surrounding edema despite the extremely low incidence , brain abscess caused by bacterial infection has a high mortality rate of 30% and is therefore a disease with poor prognosis for both mother and fetus , regardless of the state of pregnancy . although we listed previous reports on brain abscess during pregnancy ( table 2 ) , it should be noted that the number is extremely small ( 16 ) . approximately , 7% of the previous cases were related to dental treatment ( 7 ) , but no dental abnormalities were observed in the present case . during the pregnancy , maternal immunity is reduced due to a hormonal imbalance , and according to lanciers et al . , 26.6% of pregnant women , as opposed to 11.0% of non - pregnant women , are significantly infected with helicobacter pylori ( 8) . it goes without saying that organisms with low pathogenicity under normal circumstances can cause serious infection during pregnancy . in this case , the clear source of infection was not identified . it seems that the pregnant woman whose immunity was diminished is vulnerable to mssa , which was extremely rare and considered as a serious case . brain abacess in pregnancy ( literature review ) the symptoms of brain abscess include headache , nausea , and localized neurological abnormalities ( 9 ) . headache is the most common symptom , occurring in 75% of pregnant women , followed by 67% of neurological abnormalities and 58% of altered consciousness ( 10 ) . although no adverse effects of mri have been reported ( 11 , 12 ) , the ct should be avoided as much as possible because there are some problems about the degree of radiation exposure in pregnant women . therefore , mri may be a safer and is a highly sensitive diagnostic imaging modality for use in pregnancy ( 13 ) . yet , because of potential thermal tissue damage due to the high magnetic field , the national radiological protection board recommends that pregnant women avoid mri examination during the first trimester . to treat a brain abscess , it is necessary to select antibiotics capable of effectively crossing the blood brain barrier and their sensitivity should be proven in bacterial culture . the use of steroidal drug is also recommended to prevent an increase in intracranial pressure and the development of brain edema ( 13 ) . however , because intensive therapy for a brain abscess with antibiotics and steroidal drugs takes somewhere between six to eight weeks , its effect on the fetus is a huge concern . betamethasone and dexamethasone , which are transported via the placenta , should be avoided because they may affect the development of the fetal central nervous system . furthermore , the early administration of antiepileptic drugs is recommended because 70% of patients with a brain abscess develop epilepsy ( 13 ) . even infection by vulnerable bacteria becomes serious and early treatment intervention is desirable because immunity power diminishes during the pregnancy . our treatment obtained ethics approval from the regional ethics committee responsible for human experimentation and conformed to the provisions of the declaration of helsinki .

introductionbrain abscess in pregnancy is very rare , which mostly progresses to neurological abnormalities.case presentationthe patient is a 24-year - old pregnant woman . she was referred to saitama hospital due to severe headache and nausea on october 2008 . brain mri detected a 1.5 cm abscess mass with extensive edema in the right frontal lobe . we performed intensive therapy using some antibiotics that included cefotaxime and meropenem and depressants for intracranial pressure for six weeks . there was a good prognosis for the woman and her fetus without any sign of neurological abnormalities.conclusionearly medical intervention is required before it is too late for brain abscess in pregnancy .

caustic esophageal injury in infants is a devastating insult to the gastrointestinal tract and will often require major reconstructive surgery to replace the damaged esophagus . esophageal replacement with colonic interposition has been utilized since dale and sherman performed the first retrosternal colonic interposition in 1955 . up to 80% of patients with colonic interposition endoscopic dilation is relatively safe and effective for the initial treatment of anastomotic strictures , but surgical management is indicated in refractory cases . when surgery is required , graft revision utilizing both a thoracotomy and laparotomy is common . we report a case of cologastric stricture treated with resection and reconstruction of the anastomosis solely through an abdominal approach , which can offer less morbidity and mortality . a 31-year - old male developed a caustic esophageal injury after ingestion of an alkaline solution when he was 2 years old . he required emergent esophagectomy , proximal gastrectomy and reconstruction with a colonic interposition graft from the cervical esophagus to the stomach . the patient also had a pyloric stricture , for which a gastrojejunostomy was performed . over the next three decades , he required frequent hospital admissions for abdominal pain and dysphagia , and had multiple endoscopic dilations performed for a severe cologastric anastomotic stricture ( fig . the patient had severe malnourishment , with a body mass index of 14 kg / m . he had a jejunostomy feeding tube for nutritional support , which had been removed due to abdominal pain a few months prior to presentation . endoscopic evaluation revealed the severe cologastric stricture , severe inflammation just proximal to the cologastric anastomosis , and significant ulcerative disease at his gastrojejunal anastomosis . the patient underwent resection of the cologastric anastomosis and the gastrojejunal anastomosis with ulcerated stomach , neo - cologastric anastomosis and neo - gastrojejunal anastomosis via a transabdominal approach without a thoracotomy . intraoperative endoscopy was utilized during the case to ensure that the cologastric anastomotic stricture was entirely resected . figure 2:preoperative cologastric and pyloric strictures ( a ) and post - operative changes including new cologastric , gastrojejunal , duodenojejunal anastomoses ( b ) . preoperative cologastric and pyloric strictures ( a ) and post - operative changes including new cologastric , gastrojejunal , duodenojejunal anastomoses ( b ) . the patient tolerated the procedure well and was transferred to the intensive care unit . on post - operative day 2 , he was transferred to the floor . his post - operative course was remarkable for development of an intra - abdominal fluid collection , which required percutaneous drainage , but was otherwise uncomplicated . severe esophageal damage may require resection with creation of a neo - esophagus . because these operations occur in children , complications in regards to the colonic interposition graft classically , a transthoracic approach has been used to resect the entire colonic graft . in this situation , a repeat colon graft may be needed , which carries high morbidity and mortality . in particular , the majority of the proximal colon graft , which was functional , was able to be spared . given our patient 's preoperative nutritional status , he would be at a predisposed risk for wound healing complications . via transabdominal approach , to the best of our knowledge , this is the first written report of using a completely transabdominal approach for revision of the colonic graft . our patient had previously been refused surgery by multiple surgeons . if this relatively less complex surgical management was used , perhaps he would have had definitive treatment much earlier . our patient did well with our transabdominal approach given the chronicity of his symptoms . in light of his clinical dilemma of continued non - operative versus operative intervention , his symptoms were relieved immediately . this surgical approach should be considered in patients with history of colonic interposition requiring reoperation for complications of the colonic graft .

a 31-year - old gentleman who had undergone an emergent esophagectomy and reconstruction with a colon interposition graft , presented with a long - standing cologastric stricture . he had undergone multiple attempts at endoscopic dilation over multiple decades with little symptomatic relief . he underwent a resection and reconstruction of the anastomosis entirely through an abdominal approach . he did well from surgery and experienced complete symptomatic relief immediately . complications of colon interposition grafts can occasionally be treated using an abdominal incision only .

sternoclavicular joint septic arthritis ( ssa ) and its clinical presentation are infrequently seen and often difficult to manage . presenting symptoms of ssa can vary , with chest and shoulder pain being the most common clinical features . after thorough literature search , no cases have yet been reported on ssa leading to vocal cord palsy . vocal cord palsy is an important sign of thoracic and head and neck pathology that is caused by an extremely wide set of pathology . a 67-year - old gentleman presented to the emergency department with a 3-week history of worsening dysphagia and hoarse voice . routine examination of the patient in the emergency department revealed that he was haemodynamically stable and apyrexial and exhibited tenderness in the left anterior neck . examination by the otolaryngology team demonstrated no evidence of cervical lymphadenopathy but tenderness of the lower left anterior triangle , as well as evident swelling , erythema and mild bruising of the anterior chest wall . on questioning the patient regarding this , he revealed that he burnt his chest using a hot water bottle 3 weeks previously and he also admitted to having stiffness and pain in the left shoulder over this same period . indirect laryngoscopy with flexible nasendoscopy revealed non - discrete swelling / oedema of the left pharayngeal wall and reduced mobility of the left vocal cord . routine haematological investigation revealed a white cell count of 18.6 10/l , c - reactive protein of 288 mg / l and platelets of 499 10/l . no other haematological abnormality was noted on admission . with a working differential of parapharyngeal space infection and possible malignancy , the patient was referred for a computed tomography ( ct ) scan of the neck and thorax with contrast . the patient was also started empirically on intravenous co - amoxiclav as treatment for neck space collection . ct imaging , performed 24 h after admission , revealed no evidence of malignancy or indeed any paraphayrngeal space collection . few small lymph nodes were noted on the left side of the neck , but were deemed to be reactive in nature , and left vocal cord palsy was evident ( fig . 1 ) . the key finding was that of a left sternoclavicular joint collection and closely associated superficial anterior chest wall , soft tissue swelling and oedema ( fig . 2 ) . this inflammatory process was also noted deep to the manubrium and sternum and extending somewhat into the mediastinum with evident enlarged mediastinal lymph nodes ( fig . the ct findings were in keeping with ssa with associated superficial and deep tissue inflammation and oedema . with no other cause found , the vocal cord palsy was attributed to the inflammation within the mediastinum , which in turn was caused by superficial burn from hot water bottle use . findings : left vocal cord palsy indicated by the para - median position of the left vocal cord in comparison with the right . findings : left sternoclavicular joint collection and closely associated superficial anterior chest wall , soft tissue swelling and oedema . findings : soft tissue oedema of the chest seen retrosternally ( marker a ) and superficially on the left anterior chest ( marker b ) as well as evidence of mediastinal lymph node enlargement . computed tomography . a 67-year - old male with ssa . findings : left vocal cord palsy indicated by the para - median position of the left vocal cord in comparison with the right . findings : left sternoclavicular joint collection and closely associated superficial anterior chest wall , soft tissue swelling and oedema . computed tomography . a 67-year - old male with ssa . findings : soft tissue oedema of the chest seen retrosternally ( marker a ) and superficially on the left anterior chest ( marker b ) as well as evidence of mediastinal lymph node enlargement . the antibiotic regimen for the patient was converted to intravenous benzylpenicillin ( 1.2 g four times per day ) and flucloxacillin ( 1 g four times per day ) . response to antibiotic therapy was limited in the first few days of admission with little change in haematological inflammatory markers . after discussion with the microbiologist , the flucloxacillin was increased to 2 g four times a day on the fourth day of admission . the patient made slow but positive improvements over the course of the next 8 days while on intravenous antibiotics , after which he was successfully discharged . he continued on oral antibiotics and on outpatient review 3 weeks after his admission , his external swelling , erythema and voice had returned to normal . repeat ct performed 3 months following discharge demonstrated complete resolution of the deep and superficial inflammatory process as well as the mediastinal lymph nodes . vocal cord palsy can be due to weakness in one or both vocal cords , and diagnosis is made when reduced mobility is evident by laryngoscope examination . in a review of 117 cases , benninger et al . attributed the following as the most common causes of vocal cord palsy : surgical trauma ( 44% ) , malignancies ( 17% ) , endotracheal intubation ( 15% ) , neurological disease ( 12% ) and idiopathic causes ( 12% ) . furthermore , a review of 389 vocal cord palsy cases by holinger et al . makes no reference to the cause being related to a septic sternoclavicular joint . explanations for this unusual presentation of ssa seen in this case include tracking infection from the septic sternoclavicular joint , resulting in a vocal cord palsy due to reactive mediastinal lymphadenopathy . spreading infectious sequeale of ssa significant contributing factors to these outcomes are the methicillin - resistant staphylococcus aureus ( mrsa ) strains , which are becoming increasingly prevalent . a literature review of 180 cases shows mediastinitis as a clinical feature in up to 13% of patients with ssa . reports of fibrosing mediastinitis and descending necrotizing mediastinitis leading to vocal cord palsy have been documented . the mild reactive mediastinal inflammation seen in the presented case has not been presented in the literature as a cause of vocal cord palsy . therefore , as mentioned previously , it is most likely explained by mediastinal lymphadenopathy , which is a known cause of left vocal cord palsy in malignancy and atypical infections such as tuberculosis . it has been shown that if radiological findings indicate a limited extent of disease , then medical therapy alone should be sufficient . this empirical antibiotic coverage should be active against s. aureus , the most common infective agent in ssa . joint resection would only be indicated if extensive bony destruction , chest wall phlegmon or abscess , retrosternal abscess , mediastinitis or pleural extension is evident on ct / mri imaging . it is clear from the haematological and radiological findings , as well as the response to treatment , that all the presenting features of this patient were as a result of the septic focus in the sternoclavicular joint .

sternoclavicular joint septic arthritis ( ssa ) is rare and often difficult to manage condition . the sternoclavicular joint is an unusual site of septic arthritis in healthy persons , but may be commonly involved in intravenous drug users , primary or secondary immunosuppressive disorders , infections or the presence of infected central lines . after thorough literature search , no cases have yet been reported on ssa leading to vocal cord palsy . the following case describes a male patient who presented to hospital with left vocal cord palsy and symptoms consistent with aero - digestive tract malignancy . radiological examination and subsequent response to treatment demonstrated the only causative pathology to be an ipsilateral septic sternoclavicular joint .

a 60-year - old male patient was referred to an otorhinolaryngology clinic due to a lump on the left side of his jaw , which had grown in 2 months . ultrasound sonography test examination revealed a cystic mass that was 2417 mm in size with smooth contours . multiple echogenic and reactive lymph nodes with partially visible hila were visualized in the neighboring upper jugular chain , with the largest being 1610 mm in size . following a neck magnetic resonance imaging and a preliminary diagnosis of wt , left superficial parotidectomy materials were sent for pathologic examination in two pieces , which were 53.22 cm and 4.531.2 cm in size . cross section analysis showed an off white - yellowish , well - contoured nodular tumor with a bleeding center of 42.52.2 cm . microscopic examination indicated that the tumor had epithelial components with basaloid and oncocytic columns of cells neighboring lymphoid components ( fig . in addition to the lymphoid follicles with distinct germinal centers , infiltration of large neoplastic cells with bizarre and extremely atypical morphology was seen in the lymphoid component ( figs . 2 , 3 ) . 4b ) , leukocyte common antigen , igg , cd138 , mum1 , and focal positivity for kappa . staining for lambda , igm , iga , cd3 , cd5 , cd10 , cd15 , cd56 , epithelial membrane antigen , bcl2 , bcl6 , cyclind1 , s100 , pancytokeratin , cytokeratin 20 , human melanoma black 45 , actin , and desmin were negative . latent epstein - barr virus ( ebv ) was shown to be negative in tumor cells by using ebv - encoded rna chromogenic in situ hybridization . due to these findings , the patient was diagnosed with " wt and cd30 positive diffuse large b - cell lymphoma in the parotid gland . " following the lymphoma diagnosis , a full body screen was performed . in addition to these findings , the left suprarenal gland showed two nodular mass lesions , which were assessed as likely adenomas ; however , this preliminary diagnosis was not confirmed by histopathology . the patient was stage 3a and received six courses of rituximab , cyclophosphamide , doxorubicin , vincristine , and prednisone ( r - chop ) therapy . during 6-month follow - up , the patient was free of disease . wt is the second most common type of salivary gland tumor . in 10 - 15% of cases , it is bilateral , and it accounts for 70% of all bilateral salivary gland tumors.2 the male / female ratio is 1.6/1 , and it typically develops in the 6th and 7th decades . smoking increases the risk of developing wt.5 microscopically the tumors are typically composed of proliferative epithelial components accompanied by lymphoid stroma with lymphoid follicles that have distinct germinal centers . histogenesis of the lymphoid stroma in wt has been a topic of discussion for many years . lymphoid stroma can arise as a cell response to epithelial neoplasms or as a normal lymph node due to residue held by the epithelial neoplasm.5,6 the most widely accepted hypothesis suggests that wt is a neoplasm that develops in the heterotopic salivary gland ductus within or around the parotid lymph nodes.7 transformation to carcinoma in wt is a well - known phenomenon ; however , the development of lymphomas from wts is very rare.4,8 although some cases contain a normal residual lymphoid component , in others cases the lymphoid component contains entirely neoplastic lymphoid cells.4 in the present case , non - neoplastic lymphoid tissue was also present in the neighboring areas . the pathogenesis of malignant transformation of wt remains unclear ; however , exposure to radiation is of particular interest , as the relationship between previous radiotherapy and lymphomas arising from wts has been determined by some authors.4,5,9 chronic immune sialadenitis is thought to play an important role , independent of the presence of sjgren syndrome symptoms.4,7,10 in this case , there was no history of radiotherapy or sialadenitis , but a history of smoking may have provoked the development of wt . saxena et al.1 state that because the lymphoid stroma of wt is part of the systemic lymphoid tissue , in patients with lymphomatous spread of wt , disseminated disease is present during the staging either at the time of the diagnosis or after . in the present case , with screening techniques , lymphadenopathies of a pathologic size were found in the inguinal and iliac regions . some researchers suggested that although the relationship between wt and lymphoma could be coincidental , it might also be of a pathogenic nature . according to the latter statement , a single agent can affect different tissues or one tumor could trigger the formation of another . from this point of view , the epithelial component is a continuous antigenic stimulator for the lymphoid component , which provides the stimulus for the development of lymphoma.1,6,8 according to this theory , the frequently observed reactive follicular hyperplasia in wt may be histological evidence of chronic antigen stimulation.1 it has been suggested that the lymphomas seen with wt are typically non - hodgkin lymphomas ; however , there are a few cases reporting hodgkin 's lymphomas.11,12 the majority of non - hodgkin 's lymphomas in wt are follicular lymphomas . dlbcl , small lymphocytic lymphoma , extranodal marginal zone lymphoma of mucosa associated lymphoid tissue , and mantle cell lymphoma have also been reported.4,6,8,9 a small number of t - cell lymphomas such as peripheric t - cell lymphoma and t - cell lymphoblastic lymphoma have also been described in wt.4,8,13 in summary , malignant lymphomas in wt are very rare . the presented case is a diffuse large b - cell lymphoma expressing cd30 positivity . to the best of our knowledge this is the first case in literature describing dlbcl with expression of cd30 in wt .

warthin 's tumor is the second most common type of salivary gland tumor . microscopically , warthin 's tumor displays a proliferative epithelial component and lymphoid stroma . carcinomas arising from the epithelial component are well known , but malignant transformations of the lymphoid stroma are rare . when they do occur , they are most commonly b - cell type non - hodgkin lymphomas . a 60-year - old male patient underwent surgical resection of a parotid mass . after superficial parotidectomy , microscopic examination indicated that the tumor was of epithelial components with basaloid and oncocytic columns of cells neighboring lymphoid components . in addition to the lymphoid follicles with distinct germinal centers , there were large , bizarre and extremely atypical neoplastic cells seen in the lymphoid component . large neoplastic cells were diffusely cd20 and cd30 positive . the patient was diagnosed with " warthin 's tumor and diffuse large b - cell lymphoma with expression of cd30 . " the histopathologic and clinical features are discussed along with a review of the literature .

renal transplantation rates are low among patients highly sensitized to human leukocyte antigen ( hla ) because of the high rate of antibody - mediated rejection and subsequent graft loss . it was recently reported , however , that preoperative desensitization using an anti - cd 20 antibody ( rituximab ) and intravenous immunoglobulin improved transplantation rates in patients highly sensitized to hla . in contrast , the significance of a positive lymphocytotoxic crossmatch in living donor liver transplantation ( ldlt ) is controversial . successful ldlt using a liver graft in which the lymphocytotoxic crossmatch was highly positive is reported . the recipient was a 41-year - old woman with end - stage liver disease due to alcoholic liver cirrhosis ( model for end - stage liver disease score 21 ) . at the age of 20 , she was gravida one , para one . she was considered a candidate for liver transplantation because of repeated episodes of encephalopathy . because of the severe shortage of cadaveric donor grafts in japan , we planned an ldlt , and her husband was willing to donate his partial liver . the abo blood type was identical , but the t lymphocytotoxic crossmatch titer was over 10,000 and the b lymphocytotoxic crossmatch titer was 128 ( complement method with the dilution technique according to the standard national institutes of health technique ) . in addition , an examination of anti - hla antibodies using fluorescent microspheres revealed that the recipient had donor specific antibodies ( b51 and b52 ) . after obtaining written informed consent from the patient and donor and the approval of the intra - institutional committee , we proceeded to the preoperative preparations . for preoperative desensitization , the patient was first infused with rituximab 2 weeks before the scheduled surgery ( due to a catheter - associated infection , however , the operation was postponed and ldlt was performed 21 days after initiation of the rituximab therapy ) . as the antibody to hepatitis b core antigen was positive , entecavir ( 0.5 mg / day ) was administered for 3 weeks preoperatively to prevent a possible hepatitis b virus breakthrough . on postoperative days 1 and 4 , 20 mg of anti - cd25 antibody ( basiliximab ) was administered in addition to the routine methylprednisolone and tacrolimus , as we were anxious about hyperacute rejection . besides , mycophenolate mofetil ( mmf ; 2,000 mg / day ) was started on postoperative day 7 . the postoperative course was uneventful except for an episode of mild acute cellular rejection ( banff score 3 ) on postoperative day 27 , which responded promptly to steroid recycle therapy . the liver biopsy specimen obtained at the time of the acute rejection showed mild infiltration of lymphocytes in the portal area and around the bile ducts . one year after the ldlt , the lymphocytotoxic crossmatch remained negative and the patient has been well with good graft function.fig . acr acute cellular rejection , alt alanine aminotransferase , mmf mycophenolate mofetil , mp methylprednisolone , pe plasma exchange , tb total bilirubin , pod postoperative day the clinical profile of the present patient . acr acute cellular rejection , alt alanine aminotransferase , mmf mycophenolate mofetil , mp methylprednisolone , pe plasma exchange , tb total bilirubin , pod postoperative day the impact of a lymphocytotoxic crossmatch - positive liver graft on acute cellular rejection and graft survival remains controversial , both in deceased donor liver transplantation [ 3 , 4 ] and in ldlt [ 57 ] . some institutions have reported significantly unfavorable outcomes in ldlt recipients with a positive lymphocytotoxic crossmatch [ 6 , 7 ] . in contrast , our previous results showed that if the titer is low ( no more than 32 ) , a positive lymphocytotoxic crossmatch does not adversely affect the graft or survival in patients without desensitization . although the significance of a quantitative assessment of the lymphocytotoxic crossmatch has not been reported , the high titer in our present patient led to the need for perioperative desensitization to prevent early graft loss due to antibody - mediated rejection . after considering the results in the present patient , we have settled the indication criteria for preoperative desensitization therapy at the titer of 1,000 ( t lymphocyte crossmatch ) . in this patient , therefore , we applied preoperative desensitization using rituximab and plasmapheresis to reduce the high titer of preformed antibodies and b lymphocytes . as a result , the lymphocytotoxic crossmatch was negative after the 3rd plasmapheresis , and negativity was sustained thereafter . preoperative desensitization using rituximab was introduced in abo - incompatible ldlt in 2003 and has dramatically improved the outcomes of abo - incompatible ldlt . the appropriate dosage of rituximab is still controversial , but many previous studies have reported the administration of 375 mg / m of rituximab 13 weeks before the transplant . following these successful cases , we planned the administration of 375 mg / m ( 500 mg / body ) of rituximab 2 weeks before the operation [ 8 , 9 ] . splenectomy is also considered to be effective to reduce antibody production , as the spleen is the site of antibody production . after the operation , the suppression of t - cell function to prevent the initiation of t - cell - mediated antibody production was regarded as indispensable . we have routinely used tacrolimus and steroid as an immunosuppressive regimen , and in this particular patient , we added basiliximab ( postoperative days [ pods ] 1 and 4 ) and mmf . mild acute cellular rejection occurred about 3 weeks after the ldlt , but response to the steroid recycle therapy was prompt , and the lymphocytotoxic crossmatch was negative during this episode . in summary , we report a successful ldlt using a lymphocytotoxic crossmatch highly positive graft . perioperative desensitization using plasmapheresis and rituximab may provide significant benefits for reducing anti - hla antibodies .

we describe a successful living donor liver transplantation ( ldlt ) using a lymphocytotoxic crossmatch highly positive graft . a 41-year - old woman with alcoholic liver cirrhosis was referred as a potential candidate for ldlt , and her husband was willing to donate his partial liver . as the t - lymphocytotoxic crossmatch titer was over 10,000 , the patient was first infused with rituximab for preoperative desensitization , and then five rounds of plasmapheresis were performed . after the third plasmapheresis , the lymphocytotoxic crossmatch test was negative . a left liver graft including the caudate lobe was implanted , and anti - cd25 antibody ( basiliximab ) was administered on postoperative days 1 and 4 . the postoperative course was uneventful except for an episode of mild acute cellular rejection on postoperative day 27 . although the impact of a lymphocytotoxic crossmatch - positive liver graft on acute cellular rejection and graft survival in ldlt remains controversial , perioperative desensitization may provide benefits when using a highly sensitized liver graft .