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peripheral facial nerve palsy ( fnp ) can result from idiopathic ( primary ) or detectable ( secondary ) causes . secondary causes include systemic viral or other infections , trauma , ischemia , tumor , and extrinsic compression.1 however , secondary fnp by external compression has rarely been reported.2,3,4,5 therapeutic endoscopy lasts significantly longer than diagnostic endoscopy , and therefore , the left facial nerve may be compressed by the pillow and mouth piece band during the procedure ( fig . 1 ) . we encountered a very rare complication of peripheral fnp during therapeutic endoscopy . , the mouth piece band should not be too tight and a soft pillow should be replaced with the hard pillow currently used . the endoscopy showed a 3-cm , slightly elevated , discolored lesion at the greater curvature side of the antrum , so a biopsy was performed . our treatment plan consisted of endoscopic submucosal dissection ( esd ) using an insulation - tipped knife ( kd-610l ; olympus , tokyo , japan ) . initially , the endoscopist administered 2.5 mg ( 0.03 mg / kg ) of midazolam and 50 mg of propofol intravenously . depending on the consciousness state of the patient , the endoscopist added 20 mg of propofol . on the first day , the esd procedure lasted 40 minutes , and no complications were observed . on the next day , the patient complained of left facial weakness . on physical examination , the patient showed inability to wrinkle the forehead , eyebrow sagging , disappearance of the nasolabial fold , and drooling at the left corner of the mouth ( fig . 3 ) . symptoms and signs suggestive of viral infections such as parotid gland pain , cough , myalgia , febricity , and skin vesicle lesion were not present . furthermore , there were no findings of mass or stroke on magnetic resonance imaging ( mri ) . therefore , direct compression by the pillow and the mouthpiece band during endoscopy was suggested as the cause of peripheral fnp . following 2 weeks of conservative treatment , the fnp completely resolved , and the patient remained without recurrence at the follow - up . peripheral fnp may arise from a detectable cause ( secondary fnp ) or from an idiopathic cause ( primary fnp ) . secondary peripheral fnp is generally less prevalent than primary fnp ( 25% vs. 75%).6 secondary fnp caused by external compression has rarely been reported.2,3,4 the facial nerve is a primary motor nerve with a small sensorial component ; it emerges at the stylomastoid foramen , turns anterolaterally , and passes through the parotid gland . five groups of the nerve emerge from the parotid gland and are distributed to the facial muscles in a complex pattern.7 hence , the facial nerve can be subject to extrinsic compression , at the parotid gland level by the mouth piece band and pillow during endoscopy in the left lateral decubitus position . in addition , because therapeutic endoscopy requires longer than diagnostic endoscopy , it is a possible risk factor for secondary peripheral fnp by extrinsic compression . a brain mri , exhaustive medical history , and careful physical examinations are required to confirm causality . in our case , the brain mri was inconclusive , and physical examination that was suggestive of viral infection revealed no small blisters or vesicles on the external hearing disturbances . the patient developed fnp following esd but rapidly , fully recovered with only conservative treatment . although treatment of fnp is still controversial , empirically antiviral agents and corticosteroids have been successfully administered.6,8,9,10,11 in this case , clinical findings that are suggestive of viral infection were non - existent , and corticosteroid treatment was not prescribed due to bleeding risk . previous reports on peripheral fnp caused by external compression showed complete recovery within few days or months using only conservative treatment without special treatment in most cases.2,3,4 the patient in our case presented had rapid and complete recovery with conservative treatment without any antiviral agents or corticosteroids . the prognosis of fnp is relatively favorable , with approximately 80% to 85% of patients recovering spontaneously and completely , and only 15% to 20% of patients experiencing some permanent nerve damage.1 prognosis of fnp should be based on the clinical assessment using nerve conduction studies 2 to 3 weeks following diagnosis.1 long - term sequelae of fnp may include weakness , contractures , facial spasms , synkinesis , decreased tears , or psychosocial effects.6 in conclusion , we report the first case of peripheral fnp developed following therapeutic endoscopy . there are two points arising from this case report that we would like to highlight . first , physicians should be aware of the possibility of development of peripheral fnp following therapeutic endoscopy and should inform patients of the possible side effects and that complete recovery is possible with adequate treatment . second , the mouth piece band should not be too tight , and the hard pillow should be replaced with a soft pillow during endoscopic procedure . further studies are required to determine the extent to which the strength or duration of the compression may contribute to fnp .

peripheral facial nerve palsy ( fnp ) is a mononeuropathy that affects the peripheral part of the facial nerve . primary causes of peripheral fnp remain largely unknown , but detectable causes include systemic infections ( viral and others ) , trauma , ischemia , tumor , and extrinsic compression . peripheral fnp in relation to extrinsic compression has rarely been described in case reports . here , we report a case of a 71-year - old man who was diagnosed with peripheral fnp following endoscopic submucosal dissection . this case is the first report of the development of peripheral fnp in a patient undergoing therapeutic endoscopy . we emphasize the fact that physicians should be attentive to the development of peripheral fnp following therapeutic endoscopy .

aplasia cutis is a condition in which localized or generalized areas of skin are absent at birth . bullous aplasia cutis congenita is a subtype rarely seen in its initial form ; in most cases , it is found as a flat scar once the bullae have already reabsorbed . in some cases , there is underlying neural tube defects . there are only 17 cases reported in the literature including the one discussed in this paper . a 2-day - old male infant presented to our dermatology service with a 2-cm tense blister filled with serous liquid located on the vertex of the head [ figure 1 ] . the diagnosis of bullous aplasia cutis congenita was made , and we recommended conservative management . till now the child has no developmental alterations . aplasia cutis congenita is a rare condition characterized by the absence of skin and in some cases underlying structures such as bone or dura ( 20%30% of patients ) . it can present as a localized congenital defect with familial incidence ( usually on the scalp ) or be widespread with less familial incidence . it has been associated with dysgenic hydrocephaly , extensive unilateral linear epidermal nevus , hemangiomas , and multiple defects . bullous or membranous aplasia cutis congenita is a clinical subtype of this condition , with extremely few cases reported in the literature ( 17 ) , either because of underreporting or to it being so rare . the lesions are cystic or bullous at birth and eventually transform into an atrophic , flat scar covered by a thin epithelium , which is how doctors most commonly find aplasia cutis . many present with a collarette of dark , coarse hair around the defect on the vertex ( collar sign ) . it probably is not attributable to a single cause but to a combination of genetic factors , teratogens , compromised vasculature to the skin , and trauma . the increased incidence of lesions on the vertex is considered to be the result of a point of maximum tensile forces during rapid brain growth , inducing disruption of the overlying skin at 1018 weeks of gestation . some authors have proposed that membranous aplasia cutis congenita is an incomplete type of neural tube defect and may be derived from a similar embryological defect . first , the collar sign that is a relatively specific marker for cranial neural tube closure defects . second , the thin epithelial covering resembles that of encephaloceles and meningoceles both clinically and histologically . finally , membranous posterior cranial closure sites in neural tube defects resemble the findings in membranous aplasia cutis . the diagnosis is primarily clinical and a biopsy is not needed nor suggested routinely , but characteristically there is an absence of epidermis and dermis and decreased subcutaneous tissue as deep as muscle and fascia . healed lesions often demonstrate flattened epidermis , proliferation of fibroblasts , newly formed capillaries , and complete absence of adnexal structures . the diagnosis can be suspected in utero with elevated amniotic fluid alpha - fetal protein levels , positive acetylcholinesterase , and normal ultrasound findings . in all patients with aplasia cutis congenita , a complete obstetric and family history should be obtained , as well as a medical examination guided by the pattern of the lesions . management remains controversial , because the location , size , and depth of the defect must be considered . good results are obtained with conservative treatment in a defect up to 47 cm in size ( silver sulfadiazine or antibiotic ointments ) . bullous aplasia cutis congenita is an extremely rare or much under - reported type of aplasia cutis . it is hypothesized to be a form fruste of a neural tube defect . some cases present with a dark hair collar sign or an altered hair pattern surrounding the lesion , which can be even more indicative of an underlying neural tube defect .

aplasia cutis congenita is a rare condition characterized by the absence of skin and sometimes other underlying structures such as bone or dura . it can be a part of various syndromes and can be associated with multiple genetic diseases , malformation patterns , or a combination of all . it is even considered as a form frustre of a neural tube defect in several literatures . bullous aplasia cutis congenita is a clinical subtype of the condition , with extremely few cases reported in the literature . it presents as a cystic or bullous lesion at birth , which eventually transforms into an atrophic , flat scar covered by a thin epithelium . some cases present with a dark collar hair sign around the lesion , which can be even more indicative of an underlying neural tube defect . management remains controversial and depends on the characteristics of the lesion , but conservative treatment is usually chosen .

while aortic valve replacement ( avr ) is the treatment of choice for several aortic valve disorders ; it is important to recognize some complications post this surgical procedure . most importantly , these include valve dehiscence , conduction abnormalities , thrombosis , infective endocarditis , fistulas , and left ventricular outflow tract ( lvot ) pseudoaneurysms , among others . current imaging modalities such as echocardiography , computed tomography ( ct ) , and magnetic resonance imaging have allowed us to detect these potential complications during follow - up of postoperative patients . in this case report , we present the case of a patient with three previous surgical bioprosthetic avrs who presented to our institution for an additional surgical opinion . a 45-year - old nigerian female was seen in our emergency department complaining of chest pain . her history was remarkable for having three bioprosthetic avr procedures in the preceding year at another institution , the most recent being 6 months prior to the presentation . the initial indication for avr was aortic regurgitation and her previous valve surgeries were noted to be complicated by endocarditis . her past medical history is remarkable for childhood malarial and typhoid infections ; all completely treated . she complained of dyspnea on exertion , chest discomfort , orthopnea , and the need for supplemental oxygen at home ; therefore , she requested a second opinion regarding any remaining surgical options to treat her disabling symptoms . during her hospital course , an echocardiogram was obtained that demonstrated the sewing ring to be positioned higher than it normally is above the sinuses of valsalva [ figure 1 , videos 3 and 4 ] . a small mass was also noted on the posterior portion of the sewing ring with a fibrinous strand going to the base of the native aortic annulus [ figure 1 ] . a mild perivalvular leak was also noted [ figure 2 and video 5 ] . more interestingly , there appeared to be a 2 cm perforation in the lvot and a large clear space in the lateral pericardial wall consistent with a pseudoaneurysm [ figure 3 ] . contrast was used to opacify the chambers and the pseudoaneurysm is clearly seen [ figure 4 , videos 1 , 2 and 6 ] . rheumatology , allergy and immunology , as well as infectious diseases services were consulted to assist with management of this case and determine if there was any underlying process contributing to her bioprosthetic avr failure and development of the postsurgical pseudoaneurysm . they were unable to demonstrate any underlying systemic process as contributing to her cardiac disease . parasternal long view showing the sewing ring to be positioned higher than it normally is above the sinuses of valsalva ( arrow ) and a small mass was also noted on the posterior portion of the sewing ring with a fibrinous strand going to the base of the native aortic annulus . lv = left ventricle , la = left atrium parasternal long view showing mild perivalvular leak apical 4-chamber showing 2 cm perforation in the left ventricular outflow tract and a large clear space in the lateral pericardial wall consistent with a pseudoaneurysm . rv = right ventricle , psa = pseudoaneurysm apical 4-chamber with contrast demonstrating the pseudoaneurysm clearly the high - velocity blood flow in the lvot enters the tear formed by the suture and a pseudoaneurysm develops . barbetseas and colleagues used echocardiography for evaluation of aortic aneurysms after avr . even though transthoracic echocardiography clearly documented the presence of the pseudoaneurysm that formed postoperatively in our case ; cardiac ct was ordered for confirmatory purposes to reach a final therapeutic decision . in our patient 's case ,

we present a case of a pseudoaneurysm arising from the left ventricular outflow tract / aortic root as a complication of aortic valve surgery . a 45-year - old nigerian female presented to our institution 's emergency department with chest discomfort . she had three bioprosthetic aortic valve replacements in the preceding year at an outside institution for aortic regurgitation and wanted a second opinion on remaining surgical options . the learning points relevant to this case are as follows : ( 1 ) recognizing potential complications postmultiple valve surgeries , ( 2 ) screening patients for chronic infections and rheumatologic conditions that can contribute to failed valve surgeries .

the most common organisms causing mucormycosis belong to the genera rhizopus , lichtheimia , and mucor . physicians should maintain a high index of suspicion while encountering nonmycobacterial cavitary lesions not responding to antibiotic therapy . a 44-year - old man presented to us with the chief complaints of on and off fever , cough with expectoration , dyspnea , new york heart association grade 2 and two episodes of hemoptysis since 2 months . on asking leading questions , he admitted of having a right sided pleuritic chest pain . there was no history of diabetes mellitus , hypertension , or any other chronic illness . physical examination revealed temperature of 101.0f , pulse 104/min , respiratory rate 28 cycles / min , with pallor and grade 1 clubbing . respiratory system examination revealed cavernous bronchial breathing , crackles , and egophony at the right lung base . chest radiograph showed nonhomogeneous opacity of the right lower zone with a large cavity in lower and mid zone [ figure 1 ] . high resolution computed tomography thorax revealed a well - defined thick walled lesion with air fluid level and multiple air foci within it in the apical and posterior segment of the right lower lobe and lateral segment of the right middle lobe . posteriorly , the lesion abutted the posterior chest wall and anteriorly , it extended to the hila and was surrounded by ground glass opacity [ figure 2 ] . chest x - ray posterior - anterior view showing a non - homogeneous opacity of the right lower zone with large cavity high resolution computed tomography thorax showing a well - defined thick walled lesion with air fluid level and multiple air foci within it in the apical and posterior segment of right lower lobe and lateral segment of right middle lobe lower and mid zone histopathologic examination revealed evidence of mycotic infection in the segmental bronchioles with peribronchial destruction at places infiltrating into the lung connective tissue [ figure 3 ] . the mycotic elements were broad , branching aseptate hyphae belonging to zygomycetes family suggestive of mucormycosis . fungal culture of the exudate inoculated on to sabourad 's dextrose agar ( sda ) media yielded white cottony colonies with no reverse pigmentation in 7 days . treatment was started with amphotericin b deoxycholate 1.5 mg / kg / day for 6 weeks . h and e stained slide ( 40 ) showing pleomorphic , irregular , broad , branching aseptate hyphae with characteristic budding at right angles suggestive of mucormycosis mucormycosis agents being angioinvasive have a potential to cause infarction and necrosis of the affected tissues . diagnosis of pulmonary mucormycosis can be challenging because of its rarity . on chest imaging , pulmonary mucormycosis may present with focal consolidation , lung masses , pleural effusions , cavities , or multiple nodules . effective management requires a 3-pronged combination of medical and surgical modalities along with the correction of the predisposing underlying condition(s ) . amphotericin b or its newer lipid formulation liposomal amphotericin - b along with the extensive surgical debridement to remove the necrotic tissue , remains the mainstay of therapy .

cavitary lung lesions have a specific array of differential diagnosis . among rare causes is mucormycosis that should not be overlooked . a high index of suspicion is necessary for a correct diagnosis and aggressive management . it usually occurs in immunosupressed patients . it is a life - threatening , rapidly progressive , and angioinvasive fungal infection . we present a case of pulmonary mucormycosis presenting as a cavity in an immunocompetent middle aged male .

trichotillomania is a common impulse control disorder seen in both children and adults characterized by the intense urge to pull out one 's hair which then results in hair loss over the scalp , eyebrows , and/or pubic areas . pulling is often caused or preceded by stress and anxiety which is then relieved after the hair pulling process . there is also a sense of guilt , ridicule , and shame associated with the disorder and patients do not often present on their own for treatment . sometimes , patients may have trichotillomania like symptoms that may extend to them pulling hair from sofa fibers , carpets , soft toys , and even pet animals though its documentation is not as common as the disorder itself . patients with trichotillomania often have insight that they have a disorder and may present to dermatological clinics seeking treatment for alopecia which is patchy and may appear to be alopecia areata . on close scrutiny , however , it may be detected that the alopecia is often due to trichotillomania rather than alopecia areata . here , we present a case of a patient who had trichotillomania and recovered well but then started pulling her child 's hair and in fact brought the child for treatment claiming nonawareness with regards to the cause of the hair loss . a 36-year - old woman presented to the psychiatry outpatient department with symptoms of hair pulling . she was brought by her husband who claimed that his wife used to pull her hair when anxious or worried . on questioning the patient , she revealed having an anxious nature and would worry unnecessarily about routine daily activities . when her worries would increase , she would develop an incessant urge to pull her hair and initially started pulling eyebrow hair , later moving to the scalp . she would have an intense urge that would make her do so , and she would experience a sense of relief after the act . she had similar hair pulling behavior when she was in school , and it would usually happen prior to either a stage performance or school examinations . it resurfaced in the past 6 months when her son who was in second grade had academic difficulties , and she would worry about his academics and education . she had good insight and realized that she has a problem that needed treatment and wanted a cure for her woes . she was started on fluoxetine in the dose of 20 mg / day which was increased weekly by 20 mg and over a period of 3 weeks ; we reached a dose of 60 mg / day . she was also psycho - educated about trichotillomania and was taught thought stopping and habit reversal to encounter her intense urges to pull hair . she responded well to the treatment and claimed nearly 100% improvement in a period of 4 weeks . she did not follow - up thereafter . within a period of 3 months post recovery , she brought her 8-year - old son to our outpatient department as a patient , claiming that he too exhibited trichotillomania and that he used to pull his eyebrow hair . the child was assessed and on repeated questioning vehemently denied hair pulling while his mother insisted on the same . the child 's father was called upon in the absence of the mother and it was during the same session that the child tearfully told us that his own mother used to pull his hair from the eyebrows as a punishment for his bad performance in school . he said she used to do this whenever she felt like and it was only when his eyebrows look damaged and the school authorities complained about the same to her , that she brought him for treatment to us claiming that he was the patient as she needed documentation to the school that he was ill and was undergoing treatment . when we confronted the mother , in the presence of her husband threatening her police and legal action , in view of physically abusing her own child , she then broke down and admitted that she did pull her child 's hair as she had intense hair pulling urges from time to time . supportive counseling was provided to the family , and the school authorities were briefed and asked to report to us if they suspected further hair loss in the child . in child and adolescent psychiatry , we come across a condition called factitious disorder by proxy or munchausen 's disorder by proxy where a child may present to the medical or pediatric unit with medical conditions which may not have a cause and may , in fact , be due to the wrongdoing of a parent . trichotillomania by proxy as a variant of munchausen or factitious disorder by proxy where a parent pulls the child 's hair as a means to satisfy her hair pulling urges and brings the child for treatment claiming that the child has trichotillomania . this is an important consideration for pediatricians , dermatologists , trichologists , and child psychiatrists when assessing childhood onset trichotillomania particularly when parents too have or admit to a history of trichotillomania . this case study brings to the fore an important yet rare presentation of trichotillomania that needs a detailed analysis .

trichotillomania is a disorder of an impulsive hair pulling that occurs in both adults and children alike . trichotillomania is seen in children and often has other psychiatric comorbidity . here , we present an interesting case of a mother who had trichotillomania and recovered with treatment following which she resorted to pulling hair of her child and brought her child for treatment saying that the child too had trichotillomania and that we should help the child recover like her . after interviewing the child , it was revealed that it was , in fact , the mother who used to pull the child 's hair as a release for her hair - pulling urges .

in 1987 , researchers hunting for cytotoxic cell surface molecules isolated a cdna from activated cd8 t cells and called it cytotoxic t cell antigen ( ctla)-4 ( 2 ) . genetic studies provided a clue that connected ctla-4 to t cell costimulation : ctla-4 and cd28 both mapped to the same chromosomal neighborhood and shared a high degree of sequence similarity ( 3 ) . this information caught the attention of jeffrey ledbetter and peter linsley at the bristol - myers squibb research institute ( seattle , wa ) . at the time , their group was studying b7-cd28 interactions using a soluble version of cd28 that lacked transmembrane and intracellular domains . the soluble cd28 protein worked just as well as the cell - attached version in binding b7 ( 4 ) . the team hedged their bets on the similarities between ctla-4 and cd28 and tested whether ctla-4 also bound b7 . soluble ctla-4 turned out to bind b7 with 20-fold higher avidity than the soluble cd28 protein , establishing ctla-4 as a second receptor for b7 . the group published these results in the journal of experimental medicine in 1991 ( 5 ) . it now seemed that ctla-4 was a third switch in what was previously thought to be a two - switch circuit for t cell activation . but whether it promoted or jammed the circuit was a contentious issue for several more years . the debate arose because in vitro assays of costimulation - dependent t cell proliferation offered multiple interpretations . but when soluble ctla-4 was added to the mix , this proliferation was strongly inhibited ( 5 ) . one interpretation of this finding was that the soluble ctla-4 blocked the binding of b7 to the ctla-4 on the t cells . in this model , this conclusion was supported when the group showed that antibodies to ctla-4 enhanced cd28-stimulated proliferation ( 6 ) . a second interpretation , however , was that soluble ctla-4 might block proliferation by gumming up b7 's interaction with cd28 . and the ctla-4 antibody might enhance proliferation not because it stimulates clta-4 's proliferative power , but because it blocks ctla-4 's negative signal . this alternative role for ctla-4 was supported by the work of james allison and his team at the university of california ( berkeley , ca ) , which was also published in the jem ( 7 ) . this group studied cross - talk among tcr , cd28 , and ctla-4 by cross - linking these receptors . t cells proliferated when the tcr was linked to cd28 , but not with ctla-4 . t cell proliferation was greatly reduced when all three receptors were cross - linked simultaneously , suggesting that ctla-4 inhibits cd28 costimulation . the generation of ctla-4 knock - out mice finally put the conflict to rest . these animals develop a fatal t cell proliferative disorder , as their t cells lack the brakes to hold them in check ( 8) . one benefit of enhancing t cell responses via ctla-4 blockade is the strengthening of antitumor immunity . allison 's team found that tumor - transplanted mice injected with antibodies that block ctla-4 activity rejected several different types of tumors and had long - lasting antitumor immunity ( 9 ) . human anti - ctla-4 mabs are now in phase iii clinical trials against melanoma and renal carcinomas . the t cell inhibiting soluble ctla-4 originally defined by linsley and ledbetter is now used to treat autoimmune diseases such as rheumatoid arthritis .

ctla-4 was first identified in 1991 as a second receptor for the t cell costimulation ligand b7 . uncertainties about its biological function plagued the early years after its discovery until 1995 , when it was confirmed to be an inhibitor of t cell responses . ctla-4 has since scored in the clinic as a target for antitumor therapy and as a soluble inhibitor of autoimmunity .

extramammary paget 's disease ( empd ) is a rare cutaneous , intraepithelial adenocarcinoma involving primarily the epidermis but occasionally extending into the underlying dermis which accounts for < 1% of carcinomas in vulva and majority of the patients are postmenopausal females . it presents with a long - standing history of pruritic , erythematous , scaly , or velvety patches . the most frequent site is the vulva , but perineal , scrotal , perianal , and penile skin are also common areas . the cancer cells in the neoplasm usually stay in situ and only rarely invade into the dermis to be metastatic through the lymphatic system . a 55-year - old postmenopausal female presented with history of itchy and gradually progressive reddish lesion on genitals for 3 years . cutaneous examination revealed a well - defined , moist erythematous plaque of size 15 cm 10 cm with multiple erosions involving bilateral labia majora and clitoris [ figure 1 ] . on full body examination , no other lesion or lymphadenopathy was observed . on investigations , a well - defined erythematous plaque the clinical diagnosis of empd was confirmed by a 3 mm punch biopsy which showed cells within the basal layer of epidermis having nuclear enlargement with atypia , prominent nucleoli , and well - defined ample cytoplasm . in addition , a focus of squamous cell compression and moderate inflammatory cell infiltrate was seen in the upper dermis [ figure 2 ] . our patient was advised to undergo excisional surgery , but she refused and was lost to follow - up . in 1874 , sir james paget first described mammary paget 's disease and in 1901 , dubreiuhl reported the first case of vulvar empd . vulvar paget 's is a rare disease and typically presents as a red , velvety , pruritic skin rash of the vulva region which closely mimics a multitude of other , more common conditions . as a result paget 's disease can be found in both men and women ; however , it is most commonly seen in postmenopausal caucasian females . typically , involved sites are the vulvar , perianal , scrotal and penile regions ; rare sites include the thighs , buttocks , axilla , eyelids and external ear canal . the lesions can be white to red , scaling or macerated , can appear infiltrated , eroded or look like an ulcerated plaque . due to disease 's relatively low incidence and since many cases are unreported , its true incidence remains unknown . the clinical differential diagnosis for empd includes psoriasis , contact dermatitis , fungal infections , lichen sclerosus , intraepithelial neoplasia and melanoma . the nonspecific clinical findings often lead to misdiagnosis and an average of 1 year can pass before a biopsy is taken and definitive diagnosis is made . it is characterized microscopically by the presence of specific tumor cells called paget 's cells . paget cells are large cells with pale clear cytoplasm , large round hyperchromatic nuclei which tend to form clusters or solid nests . paget 's cells have intracellular mucopolysaccharides , with empd having a greater amount of mucin as compared to mpd . as a result , cells frequently show positive staining for periodic acid - schiff and diastase resistance , mucicarmine , alcian blue at ph 2.5 , and colloidal iron . surgery remains the treatment of choice for empd with recurrence rate of up to 44% with wide local excision . other modalities include mohs micrographic surgical excision , radiotherapy , topical agents including 5-fluorouracil , bleomycin , imiquimod and photodynamic therapy . this case is being reported for its rarity and to stress on the fact that an early biopsy should be advised to avoid misdiagnosing empd .

extramammary paget 's disease is a rare cutaneous , intraepithelial adenocarcinoma involving primarily the epidermis but occasionally extending into the underlying dermis . the condition typically presents as a red , velvety , pruritic skin rash of the vulva region which closely mimics a multitude of other , more common conditions . as a result , vulvar paget 's disease is frequently misdiagnosed , leading to an often lengthy lag time ( an average of about 23 years ) between the onset of symptoms and diagnosis .

in a recent article for critical care link and colleagues reported that the reversible platelet glycoprotein iib / iiia antagonist tirofiban may prevent platelet activation and preserves platelet numbers during continuous venovenous haemodialysis in patients with cardiogenic shock . acute kidney failure is a frequent complication in the critically ill , particularly so in patients with severe sepsis / septic shock or with acute myocardial infarction / cardiogenic shock . often this complication will entail the need for renal replacement therapy until kidney function recovers . contact of blood with artificial surfaces of extracorporeal systems , however , may lead to platelet activation , to formation of platelet monocyte aggregates , and to induction of inflammation . in the majority of cases , extracorporeal therapy thus requires an effective anticoagulation strategy that , in turn , may put the patient at risk of bleeding complications . especially during continuous renal replacement therapy ( crrt ) , platelet dysfunction may occur with increased activation and aggregation and ultimately with platelet loss . in recent years , glycoprotein iib / iiia antagonists have found their way into clinical routine , serving as powerful receptor blockers in the final stage of platelet activation ; for example , in patients with acute coronary syndromes and percutaneous transluminal angioplasty [ 4 - 6 ] . these antagonists have also been proposed as a pharmacological strategy to prevent platelet loss during extracorporeal circulation , especially in cardiac surgery ( platelet anaesthesia ) ; however , their potential role in extracorporeal renal replacement therapies is unclear at present . in their pilot study link and colleagues randomly assigned 40 patients with cardiogenic shock and acute kidney failure requiring crrt to two groups , either receiving unfractionated heparin ( ufh ) ( n = 20 ) or receiving a combined anticoagulation with ufh and tirofiban ( n = 20 ) . they found in the group receiving only ufh that the percentage of platelet monocyte aggregates was significantly increased ( p < 0.001 ) and the platelet number was significantly decreased ( p < 0.001 ) . in contrast , platelet monocyte aggregates and the decrement in platelet numbers were significantly reduced under combined therapy ( p < 0.001 ) . there were no significant differences between the groups regarding the efficacy of crrt , the haemofilter lifespan , or bleeding events . platelet transfusions were only necessary in three patients of the ufh group ( p = 0.016 ) . the authors conclude that , in patients with cardiogenic shock and acute kidney injury requiring crrt , the use of tirofiban in addition to ufh prevents platelet loss and platelet monocyte interaction and may preserve platelet function . whilst these findings are of interest and are of potential clinical relevance , caution needs to be exercised in their interpretation . as is also reflected in the present study , patients with acute coronary syndromes and cardiogenic shock will often be subjected to a variety of specific ( pharmacological and nonpharmacological ) interventions that may impact on platelet function and/or number . this includes the administration of anticoagulants ( heparins , heparinoids , thrombin inhibitors ) , antiplatelet agents ( glycoprotein iib / iiia antagonists , acetylsalicylic acid , thienopyridine ) , and catecholamines , as well as treatment with intraaortic counterpulsation . in a fairly small and heterogeneous cohort the same holds true for the potential influence of the type and mode of the extracorporeal treatment and materials , and , last but not least , for the metabolic control / uraemic state of the patient . moreover , the response of individual patients to acetylsalicylic acid and/or thienopyridin may vary considerably up to the point of the new and not completely understood phenomenon of thienopyridine nonresponders , an issue that will also have to be carefully considered in a subsequent study . the platelet numbers in the ufh therapy group were more than halved , dropping from an average of ( 216 64.3 ) 10/l to as low as ( 87.3 41.1 ) 10/l within 4 days three patients with platelet counts < 20 10/l even requiring platelet transfusions . given the fact that the authors used modern polysulphone capillary haemofilters and employed a continuous venovenous haemodialysis regime that reduces transmembrane pressures and thus reduces shear stress compared with postdilution continuous venovenous haemofiltration , the observed extent of platelet loss seems unusually large and remains unexplained . in summary , the study by link and colleagues raises the important question of whether platelet anaesthesia with tirofiban prevents platelet activation and loss during crrt . the data presented indicate a significantly reduced platelet loss with additional glycoprotein iib / iiia antagonist therapy compared with ufh therapy alone . owing to the small sample size , however , the potential impact of additional treatment variables could not be clarified . a substantially larger , adequately powered study

link and colleagues present a pilot study investigating platelet function and platelet numbers in patients with cardiogenic shock and acute kidney failure undergoing continuous venovenous haemodialysis . their data indicate a significantly reduced platelet loss with combined therapy of unfractionated heparin plus tirofiban , the glycoprotein iib / iiia antagonist , compared with unfractionated heparin therapy alone . owing to the small sample size , however , the potential impact of additional treatment variables ( antiplatelet agents , intraaortic counterpulsation ) could not be clarified . a substantially larger , adequately powered study is therefore called for to establish the potential clinical relevance of these findings .

a 56-year - old man was hospitalized because of pain and numbness in his left hand since he slipped a week earlier . , we found that the motor and sensory functions of his left hand were normal . his laboratory data , including anti nuclear antibody , anti - neutrophil cytoplasmic antibody , and rheumatoid arthritis factor , were within normal limits . however , the laboratory analysis showed decreased protein c and s concentrations ( protein c antigen , 57% ; protein s antigen , 19% ) . the diagnostic work - up to determine the degree of lesion included a computed tomography ( ct ) angiography , which showed an intraluminal lesion of the left upper extremities vessel . in the ct scan , coincidently , we stumbled across a mass lesion on the aortic arch . for the evaluation of the incidental mass lesion , we performed a chest ct scan and transthoracic echocardiography . the ascending aorta and the aortic arch had intact intima and a normal size ( fig . 2 ) . in transthoracic echocardiography , we found a floating mass in the lesser curvature of the aortic arch ( fig . 3 ) . we decided to surgically remove this floating mass because of the risk of peripheral embolization , including thrombectomy for the brachial and radial artery occlusion of the left arm . a median sternotomy was performed , a venous cannula was inserted in the ra auricle , extracorporeal circulation was begun , and the central temperature was decreased to 25. the patient was then in total circulatory arrest . an incision was made in the aortic arch , and the 3.0-cm intraaortic mass was completely removed ( fig . the mass had no definite stalk , and its attachment site in the aorta was relatively normal . we also removed the thrombus of the left upper extremities through the brachial artery . in the postoperative peripheral angiography , one week later , the patient recovered without complications and was discharged on the regimen of warfarin . aortic thrombi , however , are another important cause of arterial thromboembolism . factors related to an arterial thrombus are arteriosclerosis , arterial dissection , trauma , malignant tumor , and hemostatic disorder . in this case , the patient had protein c and s deficiency , which induced a hypercoagulable disorder . the presence of pedunculated thrombi in the aortic arch as in this case is rare . the incidence of embolic events from mobile aortic thrombi is 73% . in this case , the patient had a thrombus in his left arm . they occur more commonly in patients of advanced age and those with several cardiovascular risk factors . in our case the most frequent location of thoracic aorta thrombi is the region of the aortic isthmus and the portion distal to the aortic arch , at the side opposite to the origin of the subclavian artery . ct and echocardiography can be used for the diagnosis of aortic thrombi . in particular , transthoracic and transesophageal echocardiography have high diagnostic accuracy and allow the assessment of the size , morphology , and anchoring site of the thrombus , as well as the characteristics of the aortic wall . further , to determine the cause of the thrombus , we should consider a survey for hypercoagulable disorder . a definite diagnosis requires histological and immunohistochemical studies . in a differential diagnosis with other mass lesions , such as tumors , the treatment of aortic thrombi is considered necessary because of the risk of a massive systemic embolization . thrombolysis can be a possible treatment , but there is a risk of thrombolytic agents selectively lysing the stalk of the lesion , releasing the bulk of the lesion into the systemic blood stream . we believe that in selective patients with acceptable surgery for cardiopulmonary bypass and definite systemic embolic events due to highly mobile aortic thrombi , surgical treatment has been successful . this case is reported in order to inform the readers of a rare case where floating thrombi in the aortic arch of patients with embolization were successfully treated surgically .

floating thrombi in the aortic arch are very rare and an unusual source of systemic embolism . herein , a case of a 3-cm thrombus in the aortic arch is reported . it was a floating , highly mobile thrombus attached to the lesser curvature of the aortic arch . the patients had a hypercoagulable disorder induced by protein c and s deficiency . the thrombus was operatively removed with a favorable outcome .

idiopathic granulomatous mastitis ( igm ) is an uncommon disease which usually arises in premenopausal women shortly after their last childbirth . its etiology is unclear , however , breast - feeding and the use of oral contraceptives could exert an influence in its pathogenesis . very often clinical and radiological findings mimic multifocal breast cancer and the diagnosis is made by histopathology . etiopathogenesis of igm often involves an inflammatory mechanism which could be resolved by the administration of corticosteroids or methotrexate , negating the requirement complete surgical excision . cases of igm are related to the chronic use of selective inhibitors of serotonin reuptake ( ssri ) during antidepressant therapy are underestimated and should be treated with non - surgical therapeutic approaches . the postulated causes have included autoimmune diseases such as granulomatous thyroiditis , granulomatous prostatitis , granulomatous orchitis , immune response to local trauma , local irritants , undetected organisms such as viruses , mycotic , and parasitic infections , hyperprolactinemia , diabetes mellitus , alpha-1 antitrypsin and the use of oral contraceptives . interestingly , it has been reported that antipsychotic therapy can be associated with hyperprolactinemia and that the onset of breast enlargement can occur during chronic antidepressant therapy suggesting a possible side effect of ssri . in particular ssri could exert a perturbation in dopamine secretion , counteracting its role in repressing prolactin gene expression , leading finally to hyperprolactinemia and associated igm . in this regard , it seems worthy of noting the findings about a functional crosstalk between serotonin and dopamine receptors . in our opinion , surgical excision of the lesion is not necessary in cases of antidepressant therapy - related igm , and where possible , a conservative approach based on administration of an antiinflammatory therapy is favored . histopathological confirmation , combined with exclusion of malignancy and other causes of granulomatous disease , is of great importance in guiding clinical decision making and preventing inappropriate and unnecessary treatments . a retrospective study by erhan et al . reviewed delayed wound healing or recurrence after excisional biopsy , or those who have had an incisional biopsy only , if prolactin level was normal , reexcision and oral prednisone usage may be curative . in patients with a high prolactin level and who had recurrence , medical treatment to control prolactin levels this study seems , therefore , to underline an unexpected and underestimated prognostic value of blood prolactin levels , which could exert an interesting role in the recurrence of igm . as hyperprolactinemia can be induced by ssri , whose chronic use can cause gm , in these particular cases the ideal management of patients is not surgical excision , given that lesions are not malignant , but are probably caused by an hypersecretion of prolactin by the pituitary gland related to alteration of serotonin / dopamine metabolism . if this pathogenetic hypothesis is validated it could be reasonable to propose a suspension of ssri therapy to monitor the evolution of breast lesions , which would regress spontaneously , or alternatively , a therapy aimed to control prolactin blood levels and/or to modulate inflammation could be achieved . igm raises important diagnostic and therapeutic dilemmas , as more than 50% of the reported cases are initially mistaken for breast carcinoma . occasionally some patients may be subjected to mastectomy as a consequence of a false positive fine - needle aspiration cytology ( fnac ) result , as has been previously reported . the practice of performing mastectomy merely on the basis of triple assessment ( clinical , mammographic , and fnac findings consistent with malignancy ) , therefore , does not seem to be necessarily justified . in this paper we emphasize the importance of diagnosis of igm , which in the non malignant forms should not be treated surgically . in particular we draw attention to cases of igm related to antidepressant ( ssri ) therapy , postulating that these clinical scenarios must also be approached conservatively .

granulomatous mastitis is a rare benign inflammatory disease of the breast with multiple etiologies such as tuberculosis , sarcoidosis , foreign body reaction , and mycotic and parasitic infections . in contrast , idiopathic granulomatous mastitis ( igm ) is characterized by the presence of chronic granulomatous lobulitis in the absence of an obvious etiology . clinically and radiologically it may mimic breast carcinoma and so awareness of surgeons , pathologists , and radiologists is essential to avoid unnecessary mastectomies . cases of igm are reported during antidepressant therapy in patients also showing high levels of prolactinemia . in these cases , we believe that surgical excision must be avoided being replaced with a conservative management of the pathological condition based on a corticosteroid treatment .

a 66-year - old japanese woman was referred to us with a 6-month history of decreased vision and metamorphopsia in the left eye . visual acuity was 20 / 16 for the right eye and 20 / 32 for the left eye . fundus examination showed mild epiretinal membrane in the macula of the right eye , and an elevated , oval , 1-disc - diameter , reddish - orange lesion accompanied by serous detachment of the neurosensory retina in the macula of the left eye ( fig . during the choroidal arteriolar filling phase of icg angiography ( hra2 ; heidelberg engineering , heidelberg , germany ) , branching vessels ( arterioles ) appeared in blocked fluorescence , and a few dilated choroidal venules with dye leakage manifested in the early choroidal venular filling phase ( fig . the dye proceeded extremely slowly in the choroidal venules , leaking to form polypoidal vessels . notably , the early choroidal venular filling phase revealed pulsatile movements as subtle rhythmic variations in the caliber of the choroidal venule at an a - v crossing ( fig . 1b , yellow arrowhead ) , where the venule ( fig . her left eye was diagnosed as pcv and treated with photodynamic therapy using verteporfin ( visudyne ; novartis ag , basel , switzerland ) according to the standard protocol ( 698-nm laser system , 50 j / mm , 83-s exposure time , 2,650 m spot size ) . within 3 months after treatment , the reddish - orange lesion gradually decreased in size with complete disappearance of the retinal detachment ( fig . the present findings support the hypothesis that pulsations in pcv might occur at a - v crossings or at a - v shunts , as discussed in our previous study . importantly , the pulsations disappeared when dye leakage from the polypoidal vessel ceased after treatment , suggesting a relationship between venous pulsations and polypoidal vessel formation . regarding venous pulsations , spontaneous venous pulsation ( svp ) is well known on the optic disc , where the central retinal vein and artery are fixed to the bundle of the lamina cribrosa by dense collagen and elastic tissues , resulting in the vessel lumen being narrow at this location , and accounting for the greater frequency of occlusion at or near this site . svp is generally believed to be caused by the oscillation of intraocular pressure , which is transmitted to the intraocular central retinal vein across the vessel walls , during the cardiac cycle at a significantly higher level than the pressure in the retrolaminar portion of the central retinal vein . this theory may also explain the mechanism of pulsation in our case : the venous pulsations may be caused by oscillations of intraocular pressure transmitted to the choroidal venule during the cardiac cycle when there is a significant difference in intravenous pressure between both sides of the a - v crossing . the cause of the difference in intravenous pressure may be that the choroidal venule is made unusually narrow by the compression at the a - v crossing . in the retina , branch retinal vein occlusion occurs at the a - v crossing portion , particularly in arteriolar sclerosis : a vein compressed by an artery is occluded , showing stasis and dilation in the portion proximal to the a - v crossing . in the present case , a similar mechanism might contribute to the venous stasis and the increase in intravenous pressure , resulting in dilation of the portion proximal to the a - v crossing , that is , the polypoidal vessel , although occlusion did not occur . consequently , fluctuations in blood flow may occur at an a - v crossing , causing variations in the caliber of the venule . previous clinicopathologic studies revealing a close relationship between pathologic polypoidal choroidal venules and arterioles support this possibility . this study suggests that unusual compression at a - v crossings may make the venule polypoidal , and fluctuations of blood flow and pressure in the venule may cause pulsatile movements in the vessel wall . further studies elucidating the relationships between pulsation , a - v crossings , and polypoidal vessels would be helpful in understanding the pathogenesis of the formation of polypoidal vessels .

it has been reported that pulsations in abnormal vessels are observed on indocyanine green ( icg ) angiography in half of patients with polypoidal choroidal vasculopathy ( pcv ) , although the mechanism of the pulsation is unknown . in this study , we report a case of pcv showing venous pulsations at an arterio - venous ( a - v ) crossing , and discuss a possible mechanism of polypoidal vessel formation and pulsations in pcv . a 66-year - old female presented with a reddish - orange elevated lesion and serous retinal detachment in the macula of her left eye , and was diagnosed as pcv . she was treated with photodynamic therapy ( pdt ) , and followed - up through routine examinations , including icg angiography . icg angiography at presentation showed a branching vascular network and choroidal venules with dye leakage ( polypoidal vessels ) in the left eye . pulsations , supposedly of venous origin , were observed at an a - v crossing in the abnormal vessels . within 3 months after pdt , the polypoidal vessel ceased to leak and the pulsations vanished . the reddish - orange lesion gradually decreased in size with complete disappearance of retinal detachment . this study suggests that an unusual compression at an a - v crossing may make a venule polypoidal , and fluctuations of blood flow and pressure in the venule may cause pulsatile movements of the vessel wall .

acute subdural hemorrhage requires emergent neurosurgery or close observation because of its high mortality rate . a number of cases who showed rapid resolution of acute subdural hemorrhage with neurological improvements have been reported2,6,7 ) . herein , we present a case who showed rapid resolution of acute subdural hemorrhage spontaneously within 2 days after severe head injury . initially , she was stuporous and measured as glasgow coma scale ( gcs ) score of 6 . brain computed tomography ( ct ) revealed an acute subdural hematoma with a thickness of 10 mm in the right frontotemporoparietal region . however , an unexpected neurological improvement was noted within 1 hour following the accident . the patient was obeyed and gcs score was 10 even though intubated . a follow - up brain ct showed a decrease in the thickness of hematoma and improvement of the midline shift ( fig . following the accident , brain ct showed a remarkable reduction of acute subdural hematoma and mass effect . the hemorrhages of subdural space around falx , tentorium , posterior fossa , and upper cervical spinal canal were not found ( fig . usually , it requires emergent neurosurgery for decompression or close observation because of the possibility of irreversible brain damages or abrupt neurological deteriorations . rapid resolution of acute subdural hemorrhage resulting in neurological improvement is uncommon and several reports about that have been documented2,6,7 ) . wen et al.7 ) summarized some characteristics in most patients who showed rapid resolution of acute subdural hemorrhages as followings : transitory coma which last no longer than 12 hours , exclusion of cerebral contusion , widely distributed hematoma with thin width , a band of low density between the hematoma and inner table of the skull on images , a minor or moderate head trauma sustained on insults . in our present case , similar clinical findings were found except for minimal contusion of left temporal lobe . flow of cerebrospinal fluid ( csf ) through the arachnoid membrane tear site and retrograde flow into the subarachnoid space may result in dilution of the hematoma5 ) . a low density band in the subdural hematoma which is seen on the images seems to mean csf that comes from the subarachnoid space through arachnoid membrane tear . redistribution of the hematoma towards another subdural space or extracranial spaces through the skull fracture site can be another acceptable explanation1,4 ) . our present case did not show definite skull fractures or hemorrhages in another subdural space except for minimal cerebral contusion was found . so , it is thought that the dilution of acute subdural hematoma caused by csf flow is more consistent with our case rather than redistribution of hematoma . brain swelling caused by cerebral contusion may contribute the redistribution of hematomas resulting in rapid resolution of subdural hematomas3 ) . however , it is more likely to obstruct csf circulation and would mitigate the dilution and redistribution1 ) . as seen in our case , minimal cerebral contusion dose not seem to influence the obstruction of csf circulation . it is very difficult to predict the rapid resolution of acute subdural hematoma which may undergo prompt neurosurgical treatment . therefore , meticulous observation and concomitant imaging should be required even though some characteristics which may develop rapid resolution of acute subdural hematoma were seen .

acute subdural hematoma is usually a neurological emergency that requires hematoma evacuation or close observation . however , spontaneous resolutions of an acute subdural hematoma without surgical interventions have been reported rarely . we report on a case who showed rapid resolution of an acute subdural hematoma with neurological improvement and review the relevant literatures .

a 42-year - old man with hcv was referred to our hospital in august 2004 because of diarrhea and bloody stool . ten years before admission , he had been diagnosed with total uc endoscopically and histologically . colonoscopic examination revealed reddish and edematous mucosa with multiple erosions through the entire colon ( fig . laboratory data showed thrombocytopenia ( 57,000/mm3 ) , asparate aminotransferase ( ast ) 124 iu / l , alanine aminotransferase ( alt ) 110 iu / l , and hepatitis c virus ( hcv ) rna ( genotype ib ) 757 kiu / ml . after informed consent had been obtained , he was treated with peg - ifn--2a ( 90 g / week ) for improving both colonic and liver inflammation . four weeks after initiation of peg - ifn therapy , his abdominal symptoms gradually subsided . we performed intracellular cytokine assay with peripheral cd4 t cells before and after ifn therapy . the ratio of t - helper ( th ) 1 ( ifn-?)/th 2 ( interleukin ( il)-4 ) increased ( 40.2 ) at 4 weeks after ifn therapy compared to that before ( 27.4 ) ( fig . however , there was no significant difference of il-10 production from cd4 t cells between before and after ifn therapy . he was uneventfully treated with peg - ifn--2a for one year . when last seen in november 2006 , he was still in remission of uc and his laboratory data showed a negative serum hcv - rna , which was suggestive of sustained virological response . inf- , through its immunomodulatory function , could have an impact on pathways in the immune system . therefore , the effect of inf- on the pathophysiology of ibd has been focused , because patients with ibd are characterized by imbalance of the th1/th2 cytokine response . previous reports showed that a treatment with inf- might be a trigger for development of th1-related intestinal disease such as celiac disease and crohn 's disease , because inf- plays an important role in t cell differentiation towards a th1 type of immune response [ 5 , 6 ] . regard , inf- is considered to have beneficial effects in the treatment of diseases characterized by excess th2 cells such as uc . in fact , several clinical trials with ifn seem to be successful in chronic active uc [ 1 , 2 ] . however , the effect of inf- on uc remains controversial , because of some reports on the provoking onset and exacerbation of uc by treatment of inf- [ 7 , 8 , 9 ] . in this case , we evaluated the change of th1/th2 ratio of cd4 t cells before and after inf- therapy . the data on intracellular cytokine assay clearly demonstrated that ifn administration increased th1/th2 ratio of peripheral cd4 t cells , which was suggestive of shifting cytokine profile toward th1 . in addition , our data showed that administration of peg - ifn--2a did not increase il-10 production from peripheral cd4 t cells . thus , alteration of th1/th2 cytokine balance of peripheral cd4 t cells may be a biomarker for type 1 interferon therapy in patients with uc . in the future , ifn therapy could be useful for patients with uc refractory to other conventional therapies by practical application of this biomarker .

a 42-year - old man with chronic hepatitis c and ulcerative colitis ( uc ) was referred to our hospital in august 2004 because of bloody diarrhea . he was clinically and endoscopically diagnosed with flare of uc . after informed consent had been obtained , he was treated with peg - ifn--2a . four weeks after initiation of peg - ifn therapy , his abdominal symptoms gradually subsided . intracellular cytokine assay revealed that the ratio of t - helper ( th ) 1 ( ifn-?)/th 2 ( il-4 ) increased after ifn therapy . three months after starting ifn therapy , colonoscopy revealed a normal mucosal pattern . he was uneventfully treated with peg - ifn--2a for one year . when last seen in november 2006 , he was still in remission of uc . our intracellular cytokine data suggested that alteration of th1/th2 cytokine balance by ifn is one possible mechanisms of reducing intestinal inflammation in patients with uc . in this regard , ifn therapy could be useful for some patients with uc refractory to other conventional therapies .

the presence of pancreatic tissue outside its normal localization and without anatomic and vascular continuity with the normal pancreas is termed heterotopic pancreas ( hp ) ; pancreatic rests , ectopic , aberrant , or accessory pancreas are the other terms to describe the entity . hp may occur throughout the gastrointestinal tract , but has a propensity to affect the stomach , duodenum , jejunum , meckel 's diverticulum , and ileum . less frequently , it is observed in the liver , gallbladder , bile duct system , and papilla of vater . hp is known to be associated with various associated anomalies like malrotation , small intestinal atresias , biliary atresias , congenital diaphragmatic hernias , meckel 's diverticuli , annular pancreas and esophageal atresias . there have been isolated case reports of the presence of hp within the choledochal cyst ( cc ) . a 9-year - old boy presented with features of gastric outlet obstruction for 1 month . he was operated for cc type 1 at the age of 4 months at the same center ; complete excision of cyst with roux - en - y hepaticojejunostomy was done . magnetic resonance cholangio - pancreaticography ( mrcp ) done prior to first surgery had shown pancreaticobiliary malunion ( pbmu ) , cc and dilated pancreatic duct , suggestive of chronic pancreatitis [ figure 1 ] . no annular pancreas or obvious pancreatic rests were noted at the mrcp or subsequent surgery . upper gastrointestinal ( gi ) contrast study revealed partial obstruction of the first part of duodenum [ figure 2 ] . upper gi endoscopy revealed the lumen of first part of the duodenum completely obliterated by a large polypoidal mass ; the endoscope could not be negotiated beyond the first part of duodenum . magnetic resonance cholangio - pancreaticography showing pancreaticobiliary malunion , type i cc and dilated pancreatic duct upper gastrointestinal contrast study showing near - total obstruction of first part of duodenum on exploration , the first part of duodenum was found to be severely inflamed and thickened with near complete obliteration of its lumen . the roux - en - y loop of the previous hepaticojejunostomy was 5 cm away from the duodenal lesion . excision of 2.5 cm of distal stomach , pylorus and first part of duodenum with gastroduodenostomy akin to billroth i procedure was done . histopathologic examination of the entire excised specimen revealed transmural acute on chronic inflammation and diffuse fibrosis . there were submucosal heterotopic pancreatic rests in the excised duodenum , though there were no abnormal cells in the excised pylorus and the stomach [ figure 3 ] . heterotopic pancreatic rests in the submucosa of the duodenal partition ( h and e , 40 ) the proposed theory is that during rotation of foregut in a fetus and fusion of dorsal and ventral parts of pancreas , small islands of pancreatic rests are carried away and continue to develop at this aberrant location . hp is seen in 12% of patients as autopsy findings and 1 in 500 of upper abdominal surgeries . our case was considered to be a type 2 ectopic pancreas , based on heinrich classification . however , they may present with obstruction , lump , gi bleeding , etc . , depending on the size and location of hp . correct preoperative diagnosis is usually not possible and the eventual diagnosis is made on surgical exploration and pathological examination . clinically significant lesions are greater than 1.5 cm in maximum diameter and are adjacent to or directly involve the mucosa . but most of the cases of hp causing gastric outlet syndrome have been described in adults . such an occurrence has been occasionally reported in children and rarely reported in infants and neonates too . radiological investigations like computed tomogram ( ct ) scans , endoscopic ultrasonograms and nuclear scan studies assist in preoperative diagnosis , but with difficulty . upper gi endoscopy , though helpful in certain patients presenting with submucosal nodules , was not helpful in the preoperative diagnosis in our case . therefore , surgery is frequently needed to make a definitive diagnosis and plan further management and also because differential diagnosis includes leiomyomas , lymphomas , carcinoid tumors , gastrointestinal stromal tumors ( gist ) and other malignancies . on review of available literature on hp , we found that all the pathologies known to affect the pancreas can also be seen in hp . hence , we feel that whenever hp is encountered incidentally or because of complication , it warrants excision . keeping this in mind , we performed the excision of the lesion rather than a simple bypass such as posterior gastrojejunostomy .

a 9-year - old boy presented with duodenal pancreatic rest causing obstruction and required surgical intervention . he had been treated at the age of 4 months for a choledochal cyst . both choledochal cyst and heterotopic pancreas are entities that are commonly encountered in children , but the incidental presence of both the entities in the same child , albeit presenting metachronously , is extremely rare .

eccrine angiomatous hamartoma ( eah ) is a benign enlargement of eccrine components , accompanied by abundance of vascular channels . the anomaly is usually asymptomatic , but pain , hypertrichosis , and hyperhidrosis have been reported in a few patients . a 26-year - old female presented with a 2.5 cm 1.5 cm blue - colored , ill - defined swelling over the dorsum of her left hand near the last intertriginous space encroaching over the ring finger . the surface was irregular in texture and she could demonstrate small beads of perspiration on patting the lesion [ figure 1 ] . this swelling was present for the last 2 years and she had sought medical aid as it was painful to touch . the adjoining skin over the 3 metacarpophalangeal joint and proximal interphalangeal joint of the 3 finger showed a skin - colored swelling and it represented an area of similar tumor which had grown slowly since birth . a 4 mm punch biopsy was performed and the histopathology revealed lobulated , unencapsulated structure composed of mature , numerous eccrine glands enmeshed in loose connective tissue [ figure 2 ] . blood vessels could not be demonstrated with the eccrine coils in the specimen [ figure 3 ] . beads of perspiration on eccrine angiomatous hamartoma lobulated structures in the dermis showing eccrine glands ( h and e , 100 ) plentiful eccrine coils without blood vessels ( h and e , 400 ) such angioma was first described by lotzbeck in 1859 and termed as eah by hymann and colleagues in 1968 . clinically , the lesion presents as solitary , bluish , or skin - colored nodules or plaques usually over the extremities as trauma may be an inciting factor . numerous lesions in the same individual can be the result of mosaicism of a gene mutation occurring in the early developmental stage . the tumor is painless , but involvement of nerve fibers by the enlarging eccrine elements can result in tenderness . sudoriparous angiomas , as they are also known as , can display increased eccrine sweat production on exercise or when lightly stroked . it has been suggested that as yet an unclear chemical interaction between the differentiating epithelium and the mesenchyme leads to the abnormal proliferation of eccrine structures . the tumor has to be differentiated from other structures like tufted angiomas , capillary hemangiomas , smooth muscle hamartomas , and dermatofibromas . dilatation of eccrine coils is a constant feature with amplification in their numbers , but associated enlargement of vascular channels may not be present . this modification has been termed apart from closely entwined pilar structures and infiltration of adipose tissue , profuse deposition of mucin in the connective tissue has been detailed in the literature . carcinoembryonic antigen ( cea ) and s-100 protein , which are commonly found in the eccrine sweat apparatus , are found to be diminished . simple surgical procedures like excision have produced excellent results where it was sought for pain or for cosmetic reasons . associations of such eccrine hamartomas are not common . a case with cowden 's syndrome , who had developed a thyroid adenoma , and another with neurofibromatosis are the only two such reports . eah are rare malformations presenting diverse clinical and histological deviations which need differentiation from other complex tumors . they carry a good prognosis and treatment is simple , often providing complete gratification for the sufferer .

hamartomatous , circumscribed swellings of the extremities make an interesting study . presentations are manifold and the naevi are not always present from birth . excessive growth of hairs leads to remarkable appearance of such swellings . a young woman presented to the dermatology department , complaining of tenderness over 4th finger of her left hand . the defect was present for the last couple of years and was typified by visible hyperhidrosis on gentle tapping . counseling of the affected lady made her agree for a skin biopsy . the histopathology revealed it to be of the nature of eccrine angiomatous hamartoma . blood vessels were scarce . eccrine ducts were plentiful without other associated anomalies . the deformity was removed by simple excision with good result .

motor branch or recurrent branch of the median nerve , also called as the thenar branch , innervates the intrinsic muscles of the thumb ( abductor pollicis brevis , opponens pollicis , and the superficial head of the flexor pollicis brevis ) . it has an important role in the movements of the thumb and thus in many activities of the hand . accordingly , hand surgeons pay attention not to injure the motor branch while performing carpal tunnel surgery . herein , a rare anatomic variation regarding the motor branching of the median nerve is being reported . during surgery for releasing the left carpal tunnel in a 62-year - old lady , the thenar muscular branch of the median nerve was observed to originate 2.5 cm above the proximal border of the transverse carpal ligament [ figure 1 ] . it traveled along the medial side of the flexor carpi radialis tendon . by entering the carpal tunnel , the motor branch , along with the median nerve , the medial and lateral divisions of the median nerve were normal in appearance under the carpal tunnel and there were no pseudoganglion formation . intraoperative photograph demonstrating motor branching of the median nerve proximal to the transverse carpal ligament illustration showing the course of motor branching of the median nerve the motor branch of the median nerve was , in relation to the transverse carpal ligament , was found to be of extraligamentous , subligamentous , transligamentous types in 46% , 31% , 23% , respectively in 246 carpal tunnel release operations by lanz . while studying 72 hands of 36 cadavers , reported the classical distal ( extraligamantous - subligamantous ) branching in 86% , and transligamentous motor branching in the rest . while linburg and albright , rockwell reported multiple motor branching anomalies , graham referred to a case with motor branching in the ulnar region . lanz and gruber found upper level median and lateral division branching ( in the distal forearm ) in four cases . eiken et al . reported three similar cases and kessler reported one case with the same anomaly . however , all those reports were of medial and lateral divisions of the median nerve at the upper level . the upper level branching of only the motor branch is reported in the series of olave et al . in one case . likewise in our case , the sensory branch of the median nerve ( medial and lateral divisions ) was inside the carpal tunnel . while releasing carpal tunnels a proper understanding of the anatomic variations of the motor branch is paramount in order not to cause iatrogenic injuries that may compromise hand functions severely .

we present a 62-year - old female patient who had an anatomic variation in the median nerve of the left hand . during surgery for releasing the left carpal tunnel , an abnormally high level of origin of the thenar muscular branch of the median nerve was detected , at 2.5 cm above the proximal border of transverse carpal ligament . it traveled between the medial side of the flexor carpi radialis tendon and median nerve and entered the carpal tunnel . after exiting the carpal tunnel distally , the nerve , was noted to course towards the thenar area . such variations in the median nerve should be kept in mind while performing carpal tunnel release .

a 15-year - old female developed fever , stinging eyes and headache , followed one day later by sudden appearance of flaccid blisters on the face with subsequent dissemination to more than 70% of the whole integument and development of mucosal erosions . thirty days before , she had been operated on the nose and medicated with cefuroxime ( for 8 days ) , paracetamol ( for approx . 10 days ) and metamizol ( for approx . 10 days ) , the latter of which was later identified by lymphocyte transformation test as the most likely causative compound . four days before admission , she had taken acetylsalicylic acid and paracetamol for flu - like symptoms including conjunctival irritation and rhinitis . upon physical evaluation , disseminated flaccid blisters filled with serous liquid were present on 70% of her skin surface . her eye- , mouth- , pharyngeal- and genital mucosae were also affected by erosive lesions . she received intravenous immunoglobulins ( ivigs ) , in total 3 g / kg body weight over three consecutive days , hydration and nutritional support as well as careful wound care . topical therapy included silver - coated dressings on erosions , and after reepithelialization , skin washes with water and wax emulsion were performed , followed by the application of hydrocortisone 0.5% in hydrophilic unguent . from day 1 after onset of ivig therapy onwards , no new development of blisters was observed . during the clinical course , desquamation of dusky areas of skin leaving an intact epidermis below was observed . we noted frequent spontaneous bleeding upon changes of the silver - coated dressings during reepithelialization period in the second week . the remaining detached skin fragments that overlied the progressively regrowing epidermis as of day 7 were progressively shed between day 7 and day 28 . the patient was discharged from the intensive care unit ( icu ) at day 28 after admission , with almost complete skin reepithelialization . at the last follow - up control 6 months later , the patient showed full recovery , except for residual alopecia and dry eyes . to date , no specific treatment with a high evidence level of efficacy has been reported for ten . the standard of care consists of best supportive therapy in an icu setting , and includes hydroelectrolytic and nutritional support as well as regular wound care and the appropriate detection and treatment of infectious complications . whereas best supportive care is an accepted standard of care for ten patients , none of the specific treatment strategies described to date , including ivig ( adequately dosed at 3 g / kg body weight ; there is an apparent dose dependence , as the reduction in mortality appears to be greatest for doses of 3 - 4 g / kg ) , corticosteroids , and infliximab , has shown solid evidence in support of a survival advantage in ten patients . to date , only one prospective , randomized - controlled clinical trial has been reported in ten , demonstrating no therapeutic benefit for thalidomide in the treatment of ten . ivig is a safe therapeutic modality that has been shown in several case series to potentially provide a benefit in ten . controversy as to the exact efficacy does , however , exist . in the recently established guideline for the use of high - dose immunoglobulins in dermatology , early administration of high - dose immunoglobulins ( 3 g / kg ) indeed , several published studies reporting the use of ivig for the treatment of ten have shown this therapy to potentially provide a survival benefit . the studies published to date are unfortunately heterogeneous , and often of limited size , retrospective nature , and noncontrolled . on the other hand , ten is a rare disease , and randomized prospective controlled studies analyzing the effect of ivig are very difficult to perform , and do not exist to date . eight of the 11 studies published to date reporting the use of ivig in ten suggest a positive effect of ivig on mortality if compared to the mortality predicted by scorten where applied , or the 25 - 35% average mortality rate for ten reported in the literature ( table 1 ) . it must be kept in mind , however , that as for many other therapies , the total dose ( in g / kg body weight ) of ivig administered may have an effect on the therapeutic outcome . indeed , a recent publication by trent et al . , analyzing in detail the studies published to date , showed that a dose - response relationship likely exists : per 1-g / kg increase in ivig dose , there was an observed 4.2-fold increase in patient survival . moreover , no mortality was observed amongst the 30 patients that had received 3 g / kg ivig or more . taking into account the lack of a proven specific therapy , the severity of ten , the safety of ivig , and the data to date concerning the efficacy of ivig in ten , we are of the opinion that in the absence of proven superior therapies , ivig therapy should be considered in patients with ten .

toxic epidermal necrolysis is a rare but clinically well - described dermatological pathology . however , clinical pictures of this disorder in text books do not reflect its dynamic evolution . usually , the desquamative post - bullous stage is represented , neglecting the initial bullous stage as well as the skin healing . with one clinical case , we provide a day - after - day illustration of the evolution of a patient suffering from toxic epidermal necrolysis . during one month , a skin area of a limb was regularly photo - documented .

urinary bladder metastasis from solid tumors is rare and represents 2% of all bladder neoplasms . direct extension from the cervix , prostate and colon is not unusual but metastasis from a distant organ is extremely rare . breast cancer represents as a primary site in about 2.5% cases of all metastatic bladder cancer . breast cancer commonly metastasizes to the lung , bone , liver , lymph nodes and skin ; less frequently it involves the brain , adrenal glands , ovary , spleen , pancreas , kidney , thyroid and heart . there are reports on unusual sites of breast cancer metastases ; the urinary bladder and retroperitoneum is considered one of these unusual sites . a 45-year - old premenopausal female patient presented with two months history of recurrent high - grade fever with chills , bilateral pedal edema and oliguria . on examination bilateral pitting pedal edema was present . but due to persistent urinary tract infection and renal failure we removed dj stents and bilateral nephrostomies were kept . contrast enhanced computerized tomography ( cect ) was suggestive of irregular circumferential moderately enhancing thickening of urinary bladder with hypodense mass lesion in the retroperitoneum with bilateral hydroureteronephrosis [ figure 1 ] . cystoscopy showed thick irregular bladder wall with no definitive mass lesion , so random bladder wall biopsies were taken . biopsy was suggestive of undifferentiated adenocarcinoma [ figure 2].immunohistochemistry was positive for cytokeratin , ck 7 and er and negative for ck 20 and pr . contrast enhanced computerized tomography suggestive of irregular circumferential moderately enhancing thickening of urinary bladder histopathological examination of bladder biopsy ( h and e , x 100 ) shows metastatic undifferentiated adenocarcinoma , arrow shows tumor cells subsequently , during a search for primary site of bladder metastasis , we found patient having right breast mass . true - cut biopsy from right breast mass was suggestive of invasive lobular carcinoma of breast ( nuclear grade ii ) . after two cycles of chemotherapy patient started passing urine per urethra and nephrostomy output decreased . after nephrostomy removal patient 's serum creatinine remained static at 1.6 mg% and usg showed no hydronephrosis . metastases to urinary bladder are rare , accounting for less than 2% of all bladder tumors , these are mostly found in advanced stages with peritoneal dissemination . information pertaining to bladder metastases is derived largely from autopsy studies , and known primary sites of origin in descending frequency are gastric cancer , malignant melanoma , breast and lung . potential mechanisms contributing to the appearance of secondary bladder tumors could be due to minute viable tumor emboli that pass through the pulmonary circulation without establishing a lung metastasis and subsequently reach the urinary bladder by hematogenous transport . other possible routes are extension from retroperitoneal involvement or dissemination through the lymphatic or arterial circulation . the relative infrequency of primary adenocarcinoma of the bladder causes the dilemma whether bladder adenocarcinoma represents a primary or secondary process . if the adjacent mucosa contains polypoid formation , brunn 's nests , or glandular or mucous metaplasia , a primary bladder lesion is likely . cytokeratin , ck-7 , ck-18 , ck-19 , ck-20 are useful screening markers for the recognition of epithelial differentiation . other specific markers that are commonly used are er/ pr for endometrial and breast carcinoma , ca 19 - 9 for pancreatobiliary malignancy , prostate specific antigen ( psa ) for prostate , thyroglobulin for thyroid , uroplakin iii for urothelium , and heppar i for hepatocellular . in a retrospective study bates and baithun found 282 secondary urinary bladder metastases in a series of 6289 bladder tumors ( about 4.5% of all bladder tumors detected ) . seven cases of primary breast cancer were found ; bladder metastases were detected post - mortem in six of these seven cases and all of them had metastasized widely . postobstructive renal failure in breast cancer patients can be treated easily by endoureteral catheterization or percutaneous nephrostomy which allows rapid normalization of renal function in most cases and further administration of effective systemic chemotherapy . survival after the onset of distant metastases is relatively short , poulakis et al . in 2001 reported a patient with breast cancer and urinary bladder involvement still alive at five years from diagnosis . hence , appropriate treatment and follow - up may improve the prognosis of patients with bladder metastases .

breast carcinoma is the most common nondermatologic cancer diagnosis in women . common metastatic sites include lymph nodes , lung , liver , and bone . breast carcinoma metastatic to the bladder has been reported only sporadically . most patients were symptomatic breast cancer with evidence of disseminated disease at the time of diagnosis . metastasis usually occurred many years after diagnosis , and the prognosis was poor . we report a case of breast caricinoma metastasizing to the urinary bladder and retroperitoneum , which presented initially with acute renal failure . patient was treated with bilateral per cuteneous nephrostomies and chemotherapy . starting from this clinical case we review the available literature on this issue . patients with breast cancer presenting with urinary symptoms should be examined for possible bladder metastasis .

antibiotics are well known to alter neuronal functioning , with penicillin being the best studied on not only a clinical level but on a basic science level as well . after multiple reports emerged on the probable relation between high dose penicillin and seizure , detailed animal studies emerged that showed toxic doses of penicillin given intravenously could induce myoclonic jerks at the spinal level , and could be abolished by penicillinase administration . given the wide spread use of structurally related antibiotics that share the beta lactam ring structure of penicillin , clinicians need to be aware of the potential for secondary neurotoxic effects . this report describes reversible frequent myoclonic jerks of the extremities during intravenous administration of nafcillin with piperacillin . a 66-year - old male who underwent a repeat knee prosthesis surgery developed pain and swelling at the operative site shortly afterwards with fever and elevation of the white blood count to 13,700 . for the treatment of staphylococcus warneri isolated from the operative site , intravenous antibiotics were given , with vancomycin started initially at a dose of 1.25 gm q8 hours , but then switched to a combination of nafcillin 2 grams 18 hours iv , plus piperacillin / tazobactam 4.5 grams q6h iv . shortly after starting this regimen , the patient noted the onset of random myoclonic jerks of the extremities , which would be an isolated limb but at other times be all four limbs at once as a single isolated myoclonic jerk . in addition , the patient reported something is wrong with my time clock and felt surprised that long periods of time had passed , and also had a spell of getting up to go to the bathroom , and feeling there was a gap in time . approximately 40 random myoclonic jerks were estimated to occur over the course of each day of the six week course of the nafcillin / piperacillin combination by the patient and his wife , who stayed in the room , and noticed random jerks of the extremities also during sleep , which would awaken the patients at times . electrolytes were stable with a sodium of 140 on the first day of myoclonic jerking with potassium normal at 3.8 , calcium normal at 8.9 , and magnesium normal at 2.10 . a non - contrast computed tomography of the head was negative ( figure 1 ) . past medical history was negative for any neurological problems , and there was no personal or family history for seizures or myoclonic jerks . neurological exam revealed the patient to be fully oriented with accurate 4 of 4 complex object recall at 5 minutes , with normal deep tendon reflexes and normal muscle tone and strength . the patient reported complete cessation of the myoclonic jerks after discontinuation of antibiotics , and has been free of this problem afterwards . the presented case clearly illustrates the potential for patients to develop myoclonic jerks as a secondary adverse effects of piperacillin and/or nafcillin . given the underlying pathophysiology of interaction of penicillin with the gamma - aminobutyric acid type a ( gaba - a ) receptor chloride channel , it is likely that these two antibiotics may interact in a similar way , and possibly reduce inward inhibitory chloride fluxes into both spinal and supra - spinal neurons . piperacillin has been noted to not only induce an encephalopathy , but can also produce seizures as shown by lin et al . , who rapidly reversed this by high - flux hemodialysis in a 57 year old end stage renal disease patient who developed 2 generalized tonic - clonic seizures after five 250 mg doses of piperacillin / tazobactam an excellent study by sugimoto et al . defined the pathophysiology of the ability of penicillin to produce seizure activity by reducing chloride currents across the gaba - a receptor gated channel . by inducing a selective mutation of tyrosine to phenylalanine at position 256 within the gene encoding the gaba - a receptor gated channel , this selective mutation of the beta 2 subunit rendered the altered gaba - a receptor insensitive to the pro - convulsant medication picrotoxin . detailed voltage clamp study on frog oocytes transfected with these altered channels showed a dramatic inability for penicillin to reduce inhibitory currents . in summary , clinicians need to be aware of the potential for triggering myoclonic activity with piperacillin and nafcillin . as they are structurally similar to penicillin , these two antibiotics may place patients at risk as well for developing seizure activity . the pathophysiology for these effects appear to be related to reducing inhibitory hyper - polarizing chloride currents across gaba - a receptor gated channels within the brain

a 66-year - old male receiving intravenous piperacillin and nafcillin for a post - surgical wound infection developed intermittent myoclonic jerks of all four extremities that disappeared after discontinuation of these two medications . in addition there was a mild yet definite intermittent encephalopathic effect ; head computed tomography examination as negative and there was no prior history for seizure or myoclonus . these two beta lactam ring antibiotics are structurally similar to penicillin , which is well known to induce not only myoclonus but also seizure activity by reducing the gamma - aminobutyric acid ( gaba ) induced inhibitory currents by inducing an open chloride channel block of the gaba type a receptors within the brain . clinicians need to be fully aware of the potential epileptogenic effects of piperacillin , nafcillin , and related antibiotics .

reed 's syndrome refers to the onset of uterine leiomyomas with cutaneous leiomyomas , the latter occurring segmentally or affecting a particular dermatome , that are classified into type 1 and 2 . type 1 is caused by a novel postzygotic segmental mutation ; type 2 reflects an additional postzygotic loss of heterozygosity of the gene locus responsible for cutaneous leiomyomatosis in an initially heterozygous embryo . loss of heterozygosity is a genetic process when a heterozygous cell becomes homozygous or hemizygous by loosing the corresponding wild - type allele . this phenomenon can be regarded as a precondition for tumor growth in type 2 cases , the segmental manifestation is more distinctive with additional disseminated disease because of a germline mutation with heterozygosity of all somatic cells outside the strongly affected area . a subset of individuals with reed 's syndrome is predisposed to develop papillary renal cell carcinoma . herein , we report such rare occurrence of familial myomatosis cutis et uteri with type 2 segmental variety . a 50-year - old female presented to us with history of developing multiple brown painful lesions which appeared initially over her left leg . they extended to appear insidiously over trunk , chest , and upper limbs about 25 years back which extended to appear insidiously despite excision of the left leg lesion . she also underwent hysterectomy , owing to menorrhagia and multiple uterine fibromas , 25 years ago . dermatological examination revealed multiple tender , hyperpigmented papulonodules over the left lower limb , trunk , right side of the chest , and bilateral upper limbs [ figures 1a , b and 2a , b ] . a clinical suspicion of leiomyomas was confirmed by histopathological findings of a circumscribed tumor in the dermis composed of bundles of smooth muscles cells arranged in an interlacing and whorled pattern , having abundant eosinophilic cytoplasm and elongated nuclei with blunt ends . deep red color with masson 's trichome confirmed smooth muscle fibers [ figure 3a , b ] . gynecological consultation for her daughter 's complaints of menorrhagia revealed uterine fibroids . a diagnosis of familial myomatosis cutis et uteri , segmental type 2 was made . ( a ) unilateral distribution of leiomyomas on the left leg , ( b ) multiple papulonodules on the extensor aspect of the left arm ( a ) multiple papulonodules distributed over the right side of chest , ( b ) multiple leiomyomas distributed over the trunk ( a ) histopathology shows eosinohillic smooth muscles arranged in interlacing and whorled pattern ( h and e , 40 ) , ( b ) histopathology showing red color stained smooth muscle fibers ( masson 's trichome , 40 ) leiomyoma , a rare benign tumor of smooth muscle derived from arrector pili muscle , media of blood vessels , smooth muscle of scrotum , labia majora , and nipples , can be of pilar , genital ( dartoic ) , and angioleiomyomic type . pilar leiomyoma originating from the arrector pili is the most common type of cutaneous leiomyoma and usually occurs in early adult life . it classically presents as a collection of pink , red or dusky brown firm nodules of varying size . the gene that predisposes to multiple pilar leiomyomas has been mapped to chromosome 1q 42.3-q 43 . multiple cutaneous leiomyomas , with inherited predisposition , are linked to uterine leiomyoma ( reed 's syndrome ) , and increased incidence of renal cell carcinoma . type 1 reflects heterozygosity for the underlying mutation , with a clinical picture similar to that in a nonmosaic phenotype . leads to homo- or hemizygosity , with a pronounced segmental manifestation of lesions in the affected segment . though the exact molecular etiopathogenesis of multiple cutaneous leiomyomas is not known , recent studies have demonstrated the involvement of a classical tumor suppressor gene encoding fumarate hydratase , in the pathogenesis of multiple leiomyomas . the lesions are often sensitive to touch , cold , emotional stress , or spontaneous pain . it could be attributed to the local pressure exerted by the tumor on cutaneous nerves . the excitation of these muscles occurs via the sympathetic nervous system resulting in contraction with the influx of calcium ions . hence , nifedipine , a calcium channel blocker , has a role in relieving pain associated with cutaneous leiomyoma . this case of familial cutaneous myomatosis et uteri is reported for its rare occurrence and is rarer for being segmental type 2 variety .

reed 's syndrome or familial myomatosis cutis et uteri , an autosomal dominant inherited condition with incomplete penetrance , is characterized by multiple cutaneous and uterine leiomyomas.[1 ] uterine leiomyomas usually commence earlier compared to that in the general population and cutaneous leiomyomas may precede , follow or occur concurrently . few patients may have associated renal cell carcinoma . herein we report a case of a 50-year - old female with multiple , painful cutaneous leiomyomas and who had undergone hysterectomy owing to large uterine fibroids . her 18-year - old daughter also has uterine fibroids .

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patients commonly present with acute cholinergic crisis , intermediate syndrome , or delayed polyneuropathy . they may also present with pancreatitis or hepatitis . here a 75-year - old homemaker presented to our emergency department following deliberate self - harm consuming around 100 ml chlorpyrifos along with small amount of kerosene . she was known to have systemic hypertension and was not on any treatment for the same . pulse rate was 120 beats / min and irregular . rest of the examination was normal . electrocardiogram showed rate 140/min , electrical alternans , multifocal ventricular ectopics , ventricular reentrant beats , ventricular couplets , and prolonged qt interval ( corrected qt = 512 ms ) . transtubular potassium gradient was 6.85 , and urine analysis revealed inappropriately raised urinary ph ( 7.5 ) with positive urine anion gap all suggestive of distal renal tubular acidosis . however , her cholinergic symptoms subsided , and potassium values started improving once atropinization was attained . blood gas analysis , urine potassium excretion , urine ph , and urine anion gap done were normal with serum potassium 4.5 meq / l . they inhibit acetylcholinesterase enzyme reversibly as well as irreversibly producing excess of acetylcholine in neuromuscular junction . hence , usually , clinical presentation of organophosphates includes excessive muscarinic symptoms such as increased salivation , lacrimation , urination , diarrhea , gastrointestinal cramps , emesis , and nicotinic symptoms such as tachycardia , hypertension , mydriasis , fasciculation , and weakness . rarely may they present with intermediate syndrome or delayed polyneuropathy . . it may be intense sympathetic activity due to nicotinic effect producing tachycardia and hypertension or muscarinic activity producing bradycardia and hypotension . third case , they may present with polymorphic ventricular tachycardia with prolonged qt in early phase of toxicity which is attributed to rise in free fatty acid levels by some authors . however , in all studies , echocardiogram was normal showing no permanent damage to heart . cardiac injury is a strong predictor of mortality in organophosphate poisoning , and direct myocardial injury is maximum in acute early phase of severe organophosphate poisoning . potassium value if <3 meq / l is known to present with multifocal ventricular ectopics , ventricular reentrant beats , ventricular couplets , and prolonged qt interval . in our patient , cardiac arrhythmias may be due to either organophosphate poisoning or hypokalemia or both combined . our patient had severe hypokalemia which was evaluated and detected to be due to distal renal tubular acidosis . transient distal tubular acidosis may be seen in paraproteinemia , medullary sponge kidney , nephrocalcinosis , obstructive uropathy , and autoimmune diseases . there was one reported case where distal tubular acidosis was detected with accidental kerosene ingestion . however , that patient also had concomitant ingestion of metformin in a high dose which produced lactic acidosis and acute renal failure . however , in our case , very minimal amount of kerosene has been ingested which can not explain her tubular acidosis . hence , the same may be attributed to organophosphates itself as it completely improved after atropinization . toluene poisoning presenting with transient distal renal tubular acidosis was reported , which has been attributed to impaired active hydrogen ion transport and hydrogen ion back diffusion . the same mechanism may be the cause of transient distal renal tubular acidosis due to organophosphate poisoning also though conclusive proof is yet to be availed . hence , it is better to know about all atypical presentations and expected complications of its toxicity . although neurological complications are quite well described , many others such as pancreatitis , hepatitis , and cardiac toxicities are seldom accounted . we are presenting this case just to open up possibility of a rare complication of organophosphate poisoning , distal renal tubular acidosis which should be thought of in the presence of persistent hypokalemia refractory to treatment .

renal complications due to organophosphate poisoning are very rare . we are presenting a unique case of transient distal renal tubular acidosis due to organophosphate poisoning , which to the best of our knowledge is the first of its kind . an elderly female after deliberate self - harm with ingestion of chlorpyrifos had multiple ventricular arrhythmias due to hypokalemia secondary to distal renal tubular acidosis which improved completely after treatment .

evaluation of athletic hip pain routinely includes radiographs and magnetic resonance imaging ( mri ) . computed tomography ( ct ) is applied at some centers and in certain clinical scenarios . although mri ( arthrogram ) alone can clearly define changes to labrum or cartilage , osseous pathology may be underappreciated . we present the case of an unrecognized osteoid osteoma in a patient with femoral acetabular impingement ( fai ) , cam morphology , and labral injury to emphasize the need to maintain a high index of clinical suspicion for this atypical cause of hip pain . a 19-year - old male soccer player presented with hip pain unresponsive to non - steroidal anti - inflammatory drugs ( nsaids ) and physical therapy . range of motion demonstrated 10 loss of internal rotation and muscle weakness ( 4+/5 hip flexion ) was present . radiographs revealed cam morphology ( angle = 74 ) with slight acetabular retroversion ( + cranial crossover sign ) ( fig . ( c ) ct of patient s right hip revealing osteoid osteoma at the lesser trochanter . ( d , e ) right hip ct scan in prone position with needle and radiofrequency ablation system at level of the osteoid osteoma . ( c ) ct of patient s right hip revealing osteoid osteoma at the lesser trochanter . ( d , e ) right hip ct scan in prone position with needle and radiofrequency ablation system at level of the osteoid osteoma . incidentally noted was the atypical appearance of the lesser trochanter attributed to lesser trochanter apophysitis . mri arthrogram confirmed labral tear , partial thickness chondral injury with mild iliopsoas tendonitis with bone marrow edema at the lesser trochanter . one year later the patient returned with buttock pain but denied anterior hip or groin pain . repeat radiographs and mri arthrogram revealed well healed labrum and no evidence of heterotopic ossification . ct with 3d reconstruction confirmed extent of bony resection but also clearly defined the osteoid osteoma nidus at the lesser trochanter ( fig . salicylic acid failed to relieve pain and the patient was referred for radiofrequency ablation . under ct guidance , via a posterolateral approach , a 10-gauge bone biopsy needle was advanced to the nidus of the osteoid osteoma and through the reactive bone . radiofrequency ablation was performed at the proximal and distal termini of the nidus and surrounding periosteum using a dfine , inc . post - procedure recovery was uneventful and at 3 months the patient returned to all activities denying further hip pain . our case emphasizes the value of advanced imaging in the setting of any incompletely defined bony changes of the proximal femur or acetabulum . due to limitations of radiographs and mri , bony changes were inaccurately attributed to apophysitis versus osteoid osteoma . we also draw attention to the complete lack of response to nsaids for the entirety of this patient s treatment . recent literature has shown that clinical response to nsaids in osteoid osteoma may be overstated . the lack of response certainly does not exclude osteoid osteoma as the cause of pain [ 3 , 4 ] . the authors report no funding or external sponsors relevant in the authorship and publication of this clinical vignette formal conflict of interest statement : the authors report no conflict of interest or disclosures relevant in the authorship and publication of this clinical vignette .

femoro - acetabular impingement is a common cause of hip pain in young athletes . evaluation typically includes radiographs and magnetic resonance imaging . it is important to appreciate uncommon diagnoses and the role of complimentary imaging . this clinical vignette emphasizes the need complete imaging with ct in select case of atypical hip pain.we present a 19-year old soccer player who underwent seemingly successful arthroscopic fai surgery but returned with pain . computed tomography ( ct ) revealed osteoid osteoma of the lesser trochanter . the lesion was successfully treated with percutaneous ct guided radiofrequency ablation .

there is an increased incidence of major and minor congenital abnormalities in infants born to epileptic mothers ( 6 - 7% compared with 2c3% in the general population ) . sodium valproate is a popular drug because of its broad range of anticonvulsant effects and relative freedom from sedative and behavioral effects . exposure to valproic acid during first trimester can result in the constellation of minor craniofacial anomalies and major organ malformations in human fetuses . here , we report a case of a 3-month - old baby with facial dysmorphism , as a case of fetal valproate syndrome ( fvs ) based on the phenotype and maternal use of valproic acid during the antenatal period . a 3-month - old male child admitted with complaints of breathlessness since 3 days with fever . it was a full - term normal delivery with birth weight of 2.5 kg without any postnatal complications . mother was known case of epilepsy and was controlled on sodium valproate 800 mg / day since 3 years . before delivering this child mother had three spontaneous abortions within first trimester during those 3 years . on admission , the child was in congestive cardiac failure with heart rate-168/min , respiratory rate - 64/min . child had severe failure to thrive with current weight 3.8 kg at 3 months of age despite on exclusive breastfeeding . features of facial dysmorphism [ figure 1 ] such as prominent metopic sutures , trigonocephaly , tall forehead , epicanthal folds , infraorbital groove , and medial deficiency of eyebrows , shallow philtrum , anteverted nares , and broad root of nose , low set ears , thin upper lip , and small mouth were present . broad hands and feet , loose skin [ figure 2 ] , and hypospadias were other features . based on facial dysmorphism , congenital heart disease and hypospadias in the setting of maternal valproic acid consumption during antenatal period , diagnosis of fvs prominent metopic suture fetal valproate syndrome on auscultation , pansystolic murmur with loud pulmonary component of second heart sound were present . two - dimensional echocardiography showed moderate atrial septal defect , ventricular septal defect with tiny patent ductus arteriosus with moderate pulmonary hypertension . ophthal evaluation , ultrasonography abdomen , and x - ray spine were within normal limits . valproic acid crosses the placenta and is present in a higher concentration in the fetus than in the mother . complications of epilepsy and antiepileptic drug treatment , include stillbirths , prematurity , low birth weight , major and minor malformations , and cognitive delay later in life . following several other case reports of the teratogenic effects of vpa , all of which documented similar major and minor anomalies . the facial features seen in fvs are trigonocephaly , tall forehead with bifrontal narrowing , epicanthic folds , infraorbital groove , medial deficiency of eyebrows , flat nasal bridge , broad nasal root , antiverted nares , shallow philtrum , long upper lip and thin vermillion borders , thick lower lip , small downturned mouth . our patient had almost all facial features of fvs that has previously been described in literature . the timing of exposure and the dose of the drug are important in influencing the outcome of pregnancy . first - trimester exposures are more likely to result in malformations as this is the main period of structural development in the fetus . the efficacy of valproic acid as an antiepileptic drug can not be disputed , but the extent of its teratogenic effects can not be under - estimated either . hence , the balance between the therapeutic effects of this drug and its teratogenic effects is critical in the management of women with epilepsy . high - dose folic acid ( 4 mg / day ) is recommended during pregnancy , starting at least 6 weeks preconception and continuing through the first trimester .

antenatal use of anticonvulsant valproic acid can result in a well - recognized cluster of facial dysmorphism , congenital anomalies and neurodevelopmental retardation . in this report , we describe a case with typical features of fetal valproate syndrome ( fvs ) . a 26-year - old female with epilepsy controlled on sodium valproate 800 mg / day since 3 years , gave birth to a male child with characteristic features of fvs . she also had 3 spontaneous first - trimester abortions during those 3 years . sodium valproate , a widely used anticonvulsant and mood regulator , is a well - recognized teratogen that can result in facial dysmorphism , craniosynostosis , neural tube defects , and neurodevelopmental retardation . therefore , we strongly recommend avoidance of valproic acid and supplementation of folic acid during pregnancy .

the female child is six - years - old and comes from fortaleza in the state of ceara in brazil . initial symptoms began six months ago and included fever , asthenia , and weight loss . tests findings include : cbc : normochromic and very microcytic anemia ( hb : 7.1 g ) , leucopenia ( 3,300 wbc ) , and thrombopenia ( 70,000 platelets / mm ) ; esr : 77 mm for first hour ; serology of leishmaniasis : positive ( 1/1600 using immunofluorescence and 5 archs in electrosyneresis ) ; bone marrow biopsy : the medulla is rich , but no leishmania are seen . the patient is given a meglumine antimoniate treatment with increasing doses reaching 60 mg / kg / day after three days . transient hepatic cytolysis emerges with alanine transaminasi ( alt ) and aspartate aminotransferase ( ast ) 1.5 times above upper normal limit . according to the world health organization , leishmaniasis is a poverty - related disease whose public health impact has been , until recently , grossly underestimated . the disease is caused by protozoan parasites belonging to the genus leishmania that are transmitted by the bite of a phlebotomine sandfly . there are approximately two million new cases each year 1.5 million of cutaneous leishmaniasis and 500,000 of visceral leishmaniasis or kala - azar . kala - azar is endemic to south america , east africa , the mediterranean basin , the middle east , india , and china . the cardinal sign of kala - azar is an anarchic type of fever resisting all forms of treatment . the disease always yields splenomegaly and is thought by many to produce the largest spleens in parasitology . contrary to adult manifestations , cutaneous signs such as erythematous , hyper- or hypopigmented papules and nodules of various sizes are rare in children . although not performed in the case reported , protein immunoelectrophoresis reveals hypergammaglobulinemia with an igg peak in patients with kala - azar . serological tests , which are useful for the diagnosis of kala - azar , include elisa ( enzyme linked immunosorbent assay ) , direct agglutination test ( dat ) , indirect fluorescent antibody test ( ifat ) , and the rk39 dipstick test . elisa is widely performed , particularly in epidemiological studies , because it is very simple . anemia secondary to kala - azar is aregenerative because of bone medullar parasitic invasion . even without direct evidence of leishmania , this child s poor health condition coupled with the epidemiological , clinical , and biological presentations called for immediate treatment . in brazil , side - effects of this case s treatment drug of choice , meglumine antimoniate , include the following : i ) intolerance that usually appears after the first injections and consists of fever , chills , cough , myalgia , and/or skin rash . treatment discontinuation is mandatory if such intolerance occurs ; ii ) intoxication resulting in fever , cough , skin rash , polyneuritis , hepatitis , cardiac , and renal signs . alternative treatments for kala - azar include sodium stibogluconate , amphotericin b , liposomal amphotericin b , pentamidine , and paromycin . the cardinal sign of kala - azar is an anarchic type of fever resisting all forms of treatment . the disease always yields splenomegaly and is thought by many to produce the largest spleens in parasitology . contrary to adult manifestations , cutaneous signs such as erythematous , hyper- or hypopigmented papules and nodules of various sizes are rare in children . although not performed in the case reported , protein immunoelectrophoresis reveals hypergammaglobulinemia with an igg peak in patients with kala - azar . serological tests , which are useful for the diagnosis of kala - azar , include elisa ( enzyme linked immunosorbent assay ) , direct agglutination test ( dat ) , indirect fluorescent antibody test ( ifat ) , and the rk39 dipstick test . elisa is widely performed , particularly in epidemiological studies , because it is very simple . anemia secondary to kala - azar is aregenerative because of bone medullar parasitic invasion . even without direct evidence of leishmania , this child s poor health condition coupled with the epidemiological , clinical , and biological presentations called for immediate treatment . in brazil , the etiological agent of kala - azar is leishmania donovani . without treatment , side - effects of this case s treatment drug of choice , meglumine antimoniate , include the following : i ) intolerance that usually appears after the first injections and consists of fever , chills , cough , myalgia , and/or skin rash . treatment discontinuation is mandatory if such intolerance occurs ; ii ) intoxication resulting in fever , cough , skin rash , polyneuritis , hepatitis , cardiac , and renal signs . alternative treatments for kala - azar include sodium stibogluconate , amphotericin b , liposomal amphotericin b , pentamidine , and paromycin .

we report the case of a six - year - old brazilian girl referred for splenomegaly who first presented with fever , asthenia , and weight loss . geographical location , clinical exam , and blood laboratories suggested kala - azar . serology confirmed kala - azar diagnosis , but direct evidence of the parasites was not made . a treatment by meglumine antimoniate is given under hospital surveillance for two weeks . thereupon , the patient is asymptomatic and all tests are normal .

99mtc pertechnetate scintigraphy is routinely used to detect testicular torsion and differentiate this condition from acute epididymo - orchitis . however , differentiation of torsion from hydrocele and testicular or scrotal abscess may be difficult . in this report , we present a 5-year old child in whom epididymitis with hydrocele was misdiagnosed as testicular torsion on scrotal scintigraphy . a 5-year - old boy presented to the pediatric emergency department with a complaint of left scrotal swelling and local tenderness for 7 h. doppler ultrasound ( us ) imaging showed mild hydrocele of the left scrotum with no e / o torsion of the left testis . static images at 5 and 10 min showed a halo - like pooling of tracer in the periphery of the left hemi - scrotum with a photopenic center and normal tracer uptake in the right hemi - scrotum [ figure 1 ] . a scintigraphic diagnosis of mid - phase testicular torsion of the left testis was made and the patient was immediately operated . however , it was discovered intraoperatively that the patient had epididymitis with a hydrocele and not torsion of the left testis . static images at 5 and 10 min show a halo - like pooling of tracer in the periphery of the left hemi - scrotum ( arrow ) with a photopenic centre . normal tracer uptake is seen in the right hemi - scrotum differentiation by scintigraphy between acute torsion , hydrocele , testicular or scrotal abscess , or even inguinal hernia can be difficult , and images must be interpreted in conjunction with clinical findings from scrotal transillumination . in this situation , sonography can provide useful complementary information.[14 ] in a previous study comparing scintigraphy and us in children with scrotal pain , doppler us was able to provide the diagnosis of epididymo - orchitis in cases of false - positive scintigraphy . a peri - testicular hyperemic rim on radionuclide scrotal scintigraphy epididymitis and hydrocele have also been reported to mimic testicular torsion on scintigraphy in adults.[68 ] the present study describes a halo - like appearance on scrotal scintigraphy caused by epididymitis with co - existing hydrocele in a clinical setting , suggestive of testicular torsion in a child .

scintigraphic differentiation between acute torsion , hydrocele and testicular or scrotal abscess can be difficult . doppler sonography may provide useful complimentary information toward diagnosis . the authors describe a 5-year - old child where epididymitis with hydrocele was misdiagnosed as testicular torsion on scrotal scintigraphy .

nemazee had been studying the deletion of autoreactive b cells in transgenic mice whose b cells all expressed receptors specific for a particular mhc molecule . when this transgene was bred onto mice that expressed that mhc protein , as expected , these mice lacked autoreactive b cells in the periphery . but to nemazee 's surprise , a large pool of autoreactive b cells persisted in the bone marrow ( 3 ) . to pinpoint the exact step at which the cells were escaping deletion , nemazee and his team engineered strains in which the autoantigen was targeted to different anatomical locations . expression of autoantigen in just the liver caused a near complete deletion of anti - mhc b cells , which were not replaced by non - autoreactive counterparts . but when autoantigen was also present in the bone marrow , there were many more peripheral b cells that were no longer self - reactive . nemazee discovered that these cells had escaped by switching antibody receptors they had new light chains derived from a rearranged version of the endogenous gene . the alterations took place in immature b cells , as nemazee only detected the rearrangements in bone marrow b cells . light chain replacement was simultaneously demonstrated in a different transgenic model by martin weigert and his team at the fox chase cancer center ( philadelphia , pa ) . while studying lupus , weigert had designed mice that carried a transgene for an anti - dna antibody . younger mice had fewer b cells , suggesting that autoreactive clones had been deleted . but adult mice had normal numbers of b cells . weigert and nemazee published their seminal results in a series of papers in the journal of experimental medicine in 1993 ( 46 ) . the studies were at first controversial , but numerous reports from other groups have since bolstered the case for receptor editing as a major mechanism for tolerance ( 7 ) . it is now estimated that at least 25% of b cells in the repertoire have undergone receptor editing ( 8) . nemazee 's anti - mhc transgenic mouse model has since been modified such that transgenes insert specifically into the heavy and light chain loci ( 9 ) . repairing its receptors rather than throwing away the whole cell might be the most efficient way to generate a diverse , non - self - reactive repertoire .

in 1993 , david nemazee and martin weigert independently showed that autoreactive b cells could proofread , alter , and reexpress modified receptors to become nonautoreactive . this process , called receptor editing , has since gained prominence as the main mechanism of b cell tolerance .

duodenal atresia and stenosis are rare causes of intestinal obstruction in the newborn , with a prevalence of 1 in 5,000 to 10,000 live births , with duodenal stenosis occurring less frequently than duodenal atresia.1 however , these intestinal tract anomalies occur significantly more frequently in patients with down syndrome . approximately 2.5% of infants with down syndrome are ultimately diagnosed with either duodenal atresia or stenosis.2 conversely , 24% of patients with duodenal atresia or stenosis carry a diagnosis of down syndrome.3 duodenal atresia invariably presents within the first few hours to days of life with persistent vomiting after all feeds . in general , the atresia occurs at the level of , or just distal to , the ampulla of vater.4 as a consequence , the emesis is frequently bilious and quickly brought to the attention of the medical provider . on the other hand , recurrent episodes of vomiting and failure to thrive are the most common presenting symptoms of duodenal stenosis.5 we report this case because of its unusual and delayed presentation despite the presence of high - grade stenosis . moreover , few endoscopic images of duodenal stenosis have been published in the literature , suggesting that this finding is uncommon , but providers need to be prepared to encounter this complication . a 5-month - old male infant with down syndrome presented to the emergency department with coffee - ground emesis . two weeks before admission , the patient had a single episode of emesis with dark streaks concerning for blood . he looked well on examination by his pediatrician and was started on lansoprazole with the plan to follow - up at our pediatric gastroenterology clinic . however , coffee - ground emesis recurred , and the infant was referred to the emergency department for evaluation . growth had been appropriate for a child with down syndrome . at the time of the emergency room evaluation , abdominal radiography showed a normal bowel gas pattern . on the day of admission , the patient underwent esophagogastroduodenoscopy using a gif - xp 160 endoscope ( olympus , tokyo , japan ) . there was mild erythema in the distal esophagus , and a dark brown substance was observed in the stomach , consistent with blood exposed to gastric secretions . 1a ) , with a tubular structure containing normal - appearing villous mucosa in the center ( fig . 1b ) . no lumen could be identified in this structure . an upper gastrointestinal series ( fig . 2 ) demonstrated a markedly dilated duodenal bulb and narrowing of the second portion of the duodenum ( arrows ) , confirming the suspicion of duodenal stenosis . intestinal malrotation was also identified at the time of surgery ; and therefore , the patient underwent ladd 's procedure . duodenal stenosis frequently presents with recurrent vomiting and failure to thrive . owing to its chronic and variable presentation it has been reported that the presentation and diagnosis of duodenal stenosis may be delayed to as late as adolescence.6 therefore , pediatric providers should maintain a high index of suspicion for duodenal stenosis , particularly in patients with down syndrome . our patient presented at the age of 5 months , outside of the immediate neonatal period , and with the atypical presenting sign of hematemesis . however , other studies have reported that hematemesis is the primary presenting symptom of duodenal stenosis and atresia in children.7,8 the etiology of hematemesis in these patients is not clear but may be related to esophageal or gastric irritation in the setting of gastric stasis , recurrent reflux , and vomiting . hematemesis has been better described in pyloric stenosis and is secondary to reflux esophagitis in these patients.9 the mechanism of duodenal stenosis may be similar . our patient showed erythema in the distal esophagus and had no evidence of duodenitis ; therefore , esophagitis was considered to be the most likely cause of bleeding . a typical finding of duodenal atresia on abdominal radiography in infants is the " double bubble " sign , which is marked gaseous distention of the proximal duodenum and stomach . duodenal stenosis is classically diagnosed by an upper gastrointestinal series , which demonstrates a dilated stomach and duodenal bulb and a narrowing in the duodenum at the location of stenosis.4 an upper gastrointestinal series was not used as the initial diagnostic modality in our patient because he reported hematemesis without a history of chronic vomiting as the primary presenting sign . diagnosis by endoscopy is unusual , and few endoscopic images of congenital duodenal stenosis have been published in the literature so far . once medically stable , the patients can undergo operative repair with duodenoduodenostomy performed with either side - to - side or diamond - shaped anastomosis . our patient underwent a diamond - shaped duodenoduodenostomy , which allows earlier postoperative feeding and a shorter hospital stay and is therefore the preferred procedure.10 in conclusion , patients with down syndrome are at a higher risk of gastrointestinal anomalies . even high - grade duodenal stenosis can present late and atypically in children , as observed in our patient with hematemesis at 5 months of age . therefore , pediatric gastroenterologists should maintain a high index of suspicion for congenital duodenal stenosis in older infants and children and should be familiar with the endoscopic appearance of this lesion .

duodenal stenosis and duodenal atresia are well - known gastrointestinal anomalies in patients with down syndrome . although duodenal atresia presents early and classically with vomiting in the immediate neonatal period , the presentation of duodenal stenosis can be significantly more subtle and the diagnosis delayed . here , we describe the case of a 5-month - old male infant with down syndrome and delayed presentation of high - grade duodenal stenosis diagnosed endoscopically . pediatric gastroenterologists should include duodenal stenosis in the differential diagnosis of older infants and children with vomiting and should be familiar with the endoscopic appearance of this lesion .

among stage iiia non - small cell lung cancer patients , t4n0 - 1 cases can be good candidates for surgery and have a relatively favorable postoperative prognosis [ 1 , 2 ] . however , most t4 cases with thoracic vertebral invasion may not be operated as complete resection with vertebrectomy is challenging . we report the case of a male patient with primary lung pleomorphic carcinoma invading the thoracic vertebra who underwent complete resection with en bloc total vertebrectomy after embolization of the tumor - feeding vessels . a 35-year - old japanese man presented with a 6-month history of left back pain . chest x - ray and ct revealed that a tumor ( 43 34 mm ) in s1 + 2 of the left lung had invaded the third thoracic vertebra ( fig . an mri revealed that the tumor had invaded the third thoracic vertebra and the third rib ( fig . 1c ) . as no nodal or distant metastasis was identified by whole - body ct and bone scintigram , we planned a primary surgery with a clinical diagnosis of lung cancer invading thoracic vertebrae ( ct4n0m0 , stage iiia ) . to prevent massive bleeding during resection of the tumor with vertebral invasion , arterial embolization was performed prior to surgery , and the first , second , and third intercostal arteries feeding the tumor ( fig . we performed a complete resection consisting of left upper lobe lobectomy and lymph node resection ( nd2a-2 ) , partial resection of the second and third ribs , and total en bloc spondylectomy of the third thoracic vertebra . pathological examination showed that all resection margins were free from malignant cells , and the final pathological diagnosis was pleomorphic carcinoma of the lung ( pt4n0m0 , stage iiia ) . we conducted 3 cycles of adjuvant chemotherapy ( cisplatin , 80 mg / m ; docetaxel , 60 mg / m ) followed by oral administration of s-1 [ 4 , 5 ] and radiation to the primary tumor field . the patient is alive at 14 months after surgery and without any evidence of tumor recurrence . patients with primary lung cancer invading the vertebral column are rarely operated [ 6 , 7 ] as the postoperative survival is poor ( 5-year survival rates , 1620% ) [ 6 , 8 ] . however , when complete resection is achieved , favorable prognosis might be expected for such patients [ 6 , 7 ] . thus , complete resection with vertebrectomy , especially total vertebrectomy , is challenging in the field of lung cancer surgery . in the present case , we adopted surgical treatment for the following reasons : ( 1 ) no nodal or distant metastasis was identified by whole - body ct and bone scintigram , and ( 2 ) complete en bloc tumor resection can be expected by total vertebrectomy of the third thoracic vertebra . prior to surgery , we performed embolization of the tumor - feeding arteries to prevent possible uncontrollable bleeding during vertebretcomy . we performed surgery first because complete resection is expected by this technique without preoperative induction therapy . in addition , life - threatening or extremely unpleasant spinal cord invasion and/or meningitis may occur when chemoradiation therapy does not result in tumor shrinkage . accordingly , we conducted adjuvant chemotherapy and radiation after surgery . as summarized , a careful preoperative evaluation is essential to determine surgical indication , surgical approach , and sequence of multimodality therapy for t4 tumor with vertebral invasion .

we present a case who had left upper lobectomy with total vertebrectomy after arterial embolization in preparation for intraoperative bleeding . a 35-year - old man complained of left back pain . chest ct revealed a tumor in s1 + 2 of the left lung , invading the third thoracic vertebra . as no nodal or distant metastasis was detected , we performed left upper lobectomy and lymph node dissection ( nd2a-2 ) after embolization of the vessels feeding the tumor in order to reduce intraoperative bleeding . in addition , the team of orthopedics performed en bloc resection of the third thoracic vertebra and parts of the left third and fourth ribs . histological examination of the tumor revealed pleomorphic carcinoma ( pt4n0m0 , stage iiia ) .

although priapism has been reported as a complication of malignant tumor metastasis , leukemia , sickle cell disease , embolism and spinal canal stenosis [ 1 , 2 , 3 , 4 , 5 ] , there are few reports of priapism as a complication of colorectal cancer in the absence of penile metastasis . here we report a patient with rectal cancer with no penile metastasis in whom chemotherapy resulted in an improvement of priapism . a 65-year - old man was admitted to our hospital for priapism with tenderness of the penis and dysuria . enhanced abdominal computed tomography demonstrated metastatic tumors in the liver , lung , sacrum ( fig . ultrasonography of the penis revealed no tumor and normal blood flow in the penis vessel . chemotherapy ( folfox4 ; levofolinate 25 mg , fluorouracil 250 mg , oxaliplatin 100 mg ) was administered , and although the primary rectal cancer showed no change ( fig . brain or splenic metastasis and hypercalcemia arising from bone metastasis have been reported as rare complications of colorectal cancer [ 6 , 7 , 8 ] . chemotherapy regimens for metastatic colorectal cancer include fluorouracil , leucovorin , either oxaliplatin or irinotecan , and the vegf inhibitor bevacizumab . priapism is a complex involuntary behavioral response that depends on the integration of vascular , endocrine and neurological mechanisms . stimuli for erection can be classified as psychogenic and reflexogenic . when visceral nerves at the sacrum are stimulated electrically , this results in persistent penile erection . nervi erigentes. a second , thoracolumbar erection centre at the level of t12l1 plays a role in psychogenically mediated erections via sympathetic efferent fibers . disturbances in penile blood flow due to metastasis in the penis have been reported to cause priapism . in this case , the cause of priapism might have been micrometastasis in the penis , metastatic tumors that affected the spinal cord , or continuous stimulation of nervi erigentes by a metastatic tumor . chemotherapy , which reduced the metastatic tumor in the sacrum , may also have reduced the level of nervi erigentes stimulation . the results from this case suggest that chemotherapy can be effective for treating rare complications of rectal cancer such as priapism .

a 65-year - old man was admitted with penile tenderness and dysuria due to priapism . enhanced computed tomography revealed metastatic tumors in the liver , lung , sacrum and lymph nodes . advanced rectal cancer , detected by colonoscopy as a primary tumor , was treated with chemotherapy ( folfox4 ) . although the rectal cancer showed no change , five months of chemotherapy improveid the priapism , suggesting that chemotherapy can improve rare symptoms of rectal cancer .

acromegaly is a syndrome seen in adults due to excessive production of pituitary growth hormone as a result of an adenoma or hyperplasia of the eosinophilic cells of the anterior pituitary gland . the main clinical features of acromegaly are skeletal overgrowth affecting the hands , feet , and skull along with spinal deformities like kyphosis . the interface between psychiatry and endocrinology is well documented and psychiatric problems in various endocrine disorders are well known . psychiatric issues in acromegaly have been addressed sparsely and no systematic studies exist for the same , though anecdotal case reports are present in literature . one case depicts pure depression with acromegaly , while another reports the presence of depression with pathological gambling . other psychiatric symptoms like schneiderian first - rank psychotic symptoms , persecutory delusions , visual and auditory hallucinations , apathy with lack of motivation and mood swings have all been documented in acromegaly . in all the cases , response to various medications used has been excellent bringing about a reduction of symptoms . one study on 12 patients with acromegaly failed to prove a relationship between psychopathology and growth hormone levels , while it did mention a sexual dichotomy with 11 patients being female . a 33-year - old lady from middle socioeconomic class presented with depressed mood , loss of appetite , lack of sleep at night , inability to concentrate on her day - to - day activities , and occasional passive suicidal themes in her thoughts . the patient had a similar episode three years ago and had responded well to sertraline . the patient 's father had a history of depression with psychotic features and during his hallucinatory episodes had committed suicide by jumping into a well . the facial features noted by us were prognathia of the mandible , enlargement of the supro - orbital ridges , enlarged facies , and macroglossia . on testing after endocrine consultation , the patient had a high fasting blood sugar of 185 mg% while post lunch blood sugar was 210mg% . the level of growth hormone was 7.2 ng / ml ( normal 0.044.5 ng / ml ) . a ct scan revealed an enlarged sella tursica and a thickened skull with pituitary enlargement . the patient was started on escitalopram 10 mg / day with zolpidem 10 mg at night for depressive symptoms . the patient was also called weekly for cognitive therapy sessions where suicidal and negative thoughts would be addressed . the patient started responding showing a 20% improvement in the first week itself with 80% symptom reduction at the end of four weeks . the patient was to undergo treatment with the endocrinologist for acromegaly but was noncompliant and did not follow - up after the fifth week . during the five weeks of clinical observation , improvement was 80% with no worsening of acromegaly symptoms . it was difficult to label the depression secondary to her acromegaly as the endocrine features did not bother the patient and were detected by us . a strong family history of depression with psychotic features and suicide of her father may have contributed to her depression . our limited knowledge and lack of data on the relationship between depression and acromegaly makes it very difficult to comment on any associations .

depression is one of the commonest disorders encountered in general hospital psychiatry . acromegaly is a condition with excessive growth hormone secretion that may at times present with oversychopathology . we present the case of a 33-year - old lady with depression and acromegaly that successfully resolved after treatment with escitalopram and cognitive therapy .

we investigated a tbe outbreak , comprising 6 cases , in a mountain region in western austria in july 2008 . the index case occurred in a 43-year - old shepherd who had stayed for 24 days at his alpine pasture ( 1,564 m above sea level ) before he was hospitalized for nonbacterial urethritis and nonspecific influenza - like symptoms ( including pain in the lower abdomen and legs ) , followed by clinical signs of meningitis . tbev infection was confirmed serologically by elisa demonstration of specific immunoglobulin ( ig ) m and igg in serum and cerebrospinal fluid . the patient did not remember a tick bite but had eaten self - made cheese prepared from a mixture of nonpasteurized goat milk and cow milk 811 days before illness onset ; further investigation found 6 additional persons who had eaten the same cheese ( figure ) . for 5 of them , recent tbev infection was serologically proven ( table ) . for 3 of these persons ( 2 men , 44 and 65 years of age ; and 1 woman , 60 years of age ) , similar to the index patient , a typical biphasic course and symptoms of tbe ( nonspecific flu - like symptoms followed by fever , cephalea , meningism , and ataxia after 410 days ) developed and they were hospitalized . the 2 other persons who had eaten the cheese ( female , 37 and 7 years of age ) were clinically asymptomatic . the noninfected person had vomited shortly after eating the cheese because of a gastric banding . time course and series of events of a tick - borne encephalitis ( tbe ) outbreak from cheese made with goat milk . week 0 , transport of goat to high altitude ; , onset of disease ; o i , hospitalization period ; tbev , tick - borne encephalitis virus ; me , meningoencephalitis . * tbev , tick - borne encephalitis virus ; nt , neutralization test ; csf , cerebrospinal fluid ; ig , immunoglobulin ; me , meningioencephalitis ; pos , positive ; bor , borderline ; na , not applicable ; neg , negative . the cheese was prepared from a mixture of fresh milk from 1 goat and 3 cows and was eaten shortly after production . detection of tbev - specific hemagglutination inhibiting ( hi ) and neutralizing antibodies in the goat s serum proved infection in the goat ; the 3 cows were seronegative for tbev . at the time of this investigation ( 1 month after cheese production ) , tbev was already undetectable by pcr in serum and milk of the goat . cheese from the 3 batches produced after the contaminated batch was tbev negative by pcr . the 4 domestic pigs kept at the alpine pasture and fed with the whey and goat milk , however , were seropositive ( tbev hi- and neutralizing antibodies detected ) , which indicated tbev infection , but no clinical signs were observed . serum samples from 105 goats from pastures in the neighborhood also were investigated for tbev - specific antibodies ; all goats were seronegative . our analyses showed that the 6 humans and the 4 pigs were infected through the milk of 1 goat , which had been transported by car from a tbe nonendemic valley to the alp 12 days before production of the tbev - contaminated cheese . experiments have demonstrated that infected domestic animals ( i.e. , goats , sheep , and cows ) can excrete tbev into milk for 37 days , beginning as early as the second or third day postinfection ( 69 ) . in addition , although cheese was produced once or twice each week , only this 1-kg batch of cheese transmitted tbev . therefore , all the evidence indicates that the goat was infected at the alpine pasture at an altitude of 1,564 m. indeed , some ticks were collected from cows that had stayed at this altitude during the entire summer . analyses of these ticks for tbev by pcr , however , yielded only negative results . our findings provide further evidence for the expansion of tbev - endemic regions to higher altitudes in central europe . for example , longitudinal studies in the czech republic , a country with similar climatic and ecologic conditions to those of austria , showed a shift in ixodes ricinus ticks and tbev , from 700 m in 19811983 to 1,100 m altitude in 20012005 ( 10,11 ) . likewise , zeman and benes demonstrated that the maximum altitude at which tbev is found in the czech republic gradually moved upward during 19702000 , corresponding to the rise in temperature during the same period ( 12 ) . in scandinavia , a northward extension of the geographic range of i. ricinus ticks and tbev since the mid-1980s has also been recognized ( 1,1315 ) . climatic changes most likely are the major driving forces for the geographic changes in the distribution of tbev and its main vector , i. ricinus , in europe . this report also emphasizes the efficiency of oral transmission of tbev to humans and to pigs . six of the 7 persons who ate the cheese and all 4 pigs fed residual milk or whey from the same cheese became infected . given the excellent effectiveness of the tbe vaccine ( 2 ) , vaccination probably could have prevented all 6 human cases .

we report transmission of tick - borne encephalitis virus ( tbev ) in july 2008 through nonpasteurized goat milk to 6 humans and 4 domestic pigs in an alpine pasture 1,500 m above sea level . this outbreak indicates the emergence of ticks and tbev at increasing altitudes in central europe and the efficiency of oral transmission of tbev .

neurofibroma is a benign peripheral nerve sheath tumor arising from the schwann cells and perineural fibroblasts . oral involvement is noted in 3.4 - 92% of adults and 40% of children with nf1 . a 28-year - old male patient reported [ figure 1 ] with the chief complaint of a painless swelling in the right upper back tooth region since 3 years . history revealed swelling was of insidious in onset , which increased to attain the present size . family history revealed the patient 's father [ figure 3 ] also had multiple swellings all over the body and face . on general examination , multiple swellings all over the trunk region and arms were observed [ figure 2 ] . solitary well - defined oval shaped swelling [ firm in consistency and nontender on palpation figure 4 ] approximately 4 cm 3 cm was present in the right maxillary posterior region extending from distal aspect of maxillary first premolar to mesial aspect of third molar . . however , multiple , discrete , sessile cutaneous masses which had started appearing since childhood were observed . histopathological examination of incisional biopsy of the oral lesion showed spindle cells with elongated wavy nuclei . the lesional area was separated from the overlying epithelium by mature fibrous connective tissue [ figure 5 ] . cutaneous neurofibroma over the trunk patient 's father with cutaneous neurofibroma gingival neurofibroma h and e stained section of the lesion under , 40 neurofibromatosis type 1 is due to alteration of nf1 gene , which is a tumor suppressor gene located in the long arm of chromosome 17 . the clinical criterion for the diagnosis of nf1 encompasses presence of six or more cafau lait spots ( > 5 mm in children or > 15 mm in adults ) , two or more cutaneous or subcutaneous neurofibromas or one plexiform neurofibroma , freckles in the axilla or groin optic glioma , two or more lisch nodules ( pigmented hamartomas of the iris ) , bony lesion with sphenoid wing dysplasia or bowing of the long bones with or without pseudoarthrosis , and/or first degree relative with nf1 . other features that have been described to occur variably in individuals with nf1 include short stature , large head size , failure to gain weight , precocious puberty , vascular disease including childhood hypertension , neural problems secondary to the spinal cord involvement , headaches , cognitive problems , stroke , brain tumors , and rarely tumors such as pheochromocytoma , and juvenile chronic myeloid leukemia . common sites of the oral solitary neurofibromas include tongue ( 26% ) , buccal mucosa ( 8% ) , alveolar ridge ( 2% ) , labial mucosa ( 8% ) , palate ( 8% ) , gingiva ( 2% ) , nasopharynx , paranasal sinuses , larynx , floor of the mouth and salivary gland . this patient reported with gingival localization of neurofibroma , which is extremely rare and unique . gingival neurofibromas can cause periodontal disease , as tissue growth is an obstacle in carrying out routine oral hygiene measures . oral radiographic findings unique to nf include lengthening , narrowing and rarefaction of coronoid and articular process , deepening of sigmoid notch , an enlarged mandibular canal , mandibular foramen and mental foramen . other findings are shortening of the ramus , notching of the inferior border of the mandible . in the case usually , the prognosis for solitary neurofibroma is extremely good , with only rare instances of recurrence . the present case was kept under observation and was recalled every 3 months for a period of 1 year and showed no signs of recurrence . malignant transformation of neurofibromas with nf1 into neurogenic sarcomas bears a very bad prognosis with a 5 years survival rate of just 15% . long - term review of patients and genetic counseling is recommended owing to the likelihood ( 50% ) of vertical transmission . it is imperative for the general physicians and dermatologists to be aware of the oral manifestations of nf1 considering the risk of malignant transformation and the poor prognosis .

neurofibroma is a benign peripheral nerve sheath tumor and is the most frequent tumor of neural origin . its presence is one of the clinical criteria for the diagnosis of neurofibromatosis type 1 ( nf1 ; a common hereditary disease occurring in one out of every 3000 births ) . the diagnosis can sometimes be made at birth , while in others the diagnosis is made later in life after the appearance of additional clinical criteria . majority of the solitary neurofibromas are sporadic , while a few are associated with nf1 syndrome . oral hard and soft tissue are affected by the tumor ; however , the tongue is the most affected site . gingival neurofibroma is an uncommon oral manifestation of nf . here , we report a rare case of gingival neurofibroma in nf1 patient . one of the most feared complications of nf1 is its transformation into neurofibrosarcoma , which bears a very poor prognosis . treatment of neurofibroma is surgical resection .

chorea may be the manifestation of a wide variety of degenerative , vascular , metabolic , or toxic disorders involving the central nervous system , in which dysfunction of the basal ganglia , particularly of the striatum , is generally assumed to be responsible . we report an index case of generalized chorea secondary to the ingestion of propiconazole toxin ( fungicide ) in a young female . in this case , the patient was a 35-year - old lady who was a known case of primary juvenile myoclonic epilepsy , on treatment with valproate ( 800 mg / day ) for 7 years . she was found in unconscious state at home by the family , who then took her to the emergency department of another facility . in view of prior history of epilepsy , three days later , due to persistent unresponsive neurological condition , she was referred to our tertiary care hospital for further management . at admission , her glasgow coma scale was e2m5v1 , with bilateral small - sized pupils ( 2 mm , reacting ) , heart rate of 64/min , blood pressure 90/60 mmhg on inotropic support with norepinephrine 2 g / kg / min and dopamine 8 g / kg / min . blood biochemistry and metabolic profile were normal with total leukocyte count-12,000/mm , rbs-85 mg / dl , blood and urine culture were sterile . electroencephalogram was done to rule out status epilepticus while the patient was on valproate ( blood level-80 ) and no sedatives . magnetic resonance imaging ( mri ) brain with contrast , and cytological and biochemical analysis of cerebrospinal fluid were normal . on day 3 of her illness when inotropes had been tapered , her heart rate was in the range of 5058/min . bradycardia ( even with inotropic support ) along with small - sized pupils raised the suspicion of intoxication . furthermore , relatives were redirected to look for any evidence of suspicious compound at home . cholinesterase levels were very low ( 134 ) along with an empty bottle of propiconazole toxin ( fungicide ) found at home , confirming the diagnosis of intoxication . however , on the 9 day of her illness , she developed irregular random flowing movements from one part of the body to another , suggesting generalized chorea [ video 1 ] . a detailed family history was not positive for any chorea / choreiform movements suggestive of inherited degenerative disorders . the patient was extensively evaluated for other acquired cause of chorea , but her hematological investigations including peripheral blood film , biochemical , thyroid function , serum valproate levels , and vasculitic workup were negative and repeat mri brain and computed tomography ct abdomen were also normal . she was treated with clonazepam ( 2 mg ) , tetrabenazine ( 75 mg ) , and risperidone ( 2 mg ) with partial improvement in chorea . our case depicts initial diagnostic dilemma , which is frequent problem in intoxication as history is often concealed . hence , insecticide poisonings are relatively a common occurrence with wide spectrum of neurological presentations which may affect the function of the central and peripheral nervous system . clinical manifestations vary greatly , and include movement disorders such as secondary parkinsonism and a wide range of hyperkinetic disorders and even delayed neuropathy . propiconazole is the triazole class of fungicide and is available as an emulsifiable concentrate ready - to - use liquid [ figure 1 ] . it is also known as dmi or demethylation - inhibiting fungicide , due to its binding with and inhibition of 14-alpha - demethylase enzyme . to the best of our knowledge , this is the index case of generalized chorea following intoxication with propiconazole ( fungicide ) . the probable mechanism may be excessive acetylcholine activity in the nigrostriatal system due to inactivation of acetylcholinesterase by propiconazole . within the nigrostriatal network , caudate nucleus and globus pallidus are particularly rich in cholinergic neurons causing less inhibition of pallidothalamic fibers and generalized choreiform movements . chemical structure of propiconazole ( c15h17cl2n3o2 ) the neurological manifestation with propiconazole intoxication is not reported in literature . only few cases of extrapyramidal manifestations following cholinergic intoxication secondary to organophosphate poisoning ( opc ) have been described . in the three cases described by joubert et al . , the extrapyramidal manifestations were limited to choreiform movements . although exact mechanism of action of propiconazole is not clear , but circumstantial evidence , low cholinesterase levels , and response to anticholinergic treatment sufficiently support our diagnosis . with delayed development of generalized chorea as neurological sequelae , propiconazole may be added as another compound in the causal list of toxic chorea widening the spectrum of acquired toxic causes of chorea still further . in a country like ours , where poisonings are frequent exposure to toxic agents

chorea is a rare manifestation of poisoning . we report an index case of a young woman who developed generalized chorea following propiconazole toxin ingestion . as large series on neurological complications of toxic compounds are difficult to be compiled , it is of interest to report our experience . this report adds one more compound to the increasing list of toxic chorea .

a technique of orthotopic rat liver transplantation has been shown in a recent journal of visualized experiments paper . portal vein and infra - hepatic vena cava anastomoses were performed using the quick - linker assisted cuff technique . this technique was chosen among other revisions of the original kamada 's technique , as it allows for easier and quicker anastomoses , results in a better hemodynamic , and can be implemented with a shorter learning curve . the quick - linker technique requires the use of a handle , an approximator and cuffs , all of which can be designed from common laboratory material according to the technique described in the present report . drill a 2 mm hole with a spherical diamond bit , mounted on a high - speed drill . widen the hole with fusiform or conic diamond bits . figures 1 and 2 can be used as stencils . convert the drill into a mini bench grinder and cut off half of one branch under the microscope using the high - speed mini bench grinder . make symmetrical grooves on both sides to allow for the handle to slide in completely . cut a longitudinal slit in the long arm and check that the center of the long arm and a well - positioned handle are on a same axis . make the branch as thin as possible and round off the piece with the low speed rotating brush . take fine - bore polyethylene tubing and round off one edge with an nr 10 scalpel . design a grove 0.5 mm from the edge , and carefully cut out a slope going down to the grove . drill a 2 mm hole with a spherical diamond bit , mounted on a high - speed drill . widen the hole with fusiform or conic diamond bits . figures 1 and 2 can be used as stencils . convert the drill into a mini bench grinder and the approximator is obtained by modifying straight surgical klemmer 's or kocher 's forceps . cut off half of one branch under the microscope using the high - speed mini bench grinder . make symmetrical grooves on both sides to allow for the handle to slide in completely . cut a longitudinal slit in the long arm and check that the center of the long arm and a well - positioned handle are on a same axis . make the branch as thin as possible and round off the piece with the low speed rotating brush . take fine - bore polyethylene tubing and round off one edge with an nr 10 scalpel . design a grove 0.5 mm from the edge , and carefully cut out a slope going down to the grove . this problem was due to the fact that the ring of the handle was too narrow , and it has been solved by setting the narrowest part of the handle ring at 0.5 mm . if printed with a scale 1:1 can be used to guide the handle shaping . click here to view larger figure . if printed with a scale 1:1 can be used to guide the handle shaping . click here to view larger figure . graft 's v.porta should be secured to the central groove , while recipient 's vessel to the lateral one . the present report describes the design and building of the quick - linker system for easier rat liver transplantation . rat liver transplantation is a popular model with 125 to 180 related papers published yearly since the early 1990 's ( www.pubmed.com ) . it allows for a wide range of experiments including the exploration of immunological and graft preservation issues . the use of the " quick - linker " kit allows for easier and quicker anastomoses with guaranteed intima - to - intima contact . a detailed description and discussion of the technical steps of a rat liver transplantation the quick - linker kit can be built at low price from common laboratory material . the main challenge encountered has been related the breakdown of one handle during the final brushing , and that problem has been solved by setting the narrowest part of the handle ring at 0.5 mm ( figures 1 and 2 show appropriate sizes ) . regarding the blade quality , only stainless steel should be considered . although slightly less flexible compared to the average carbon steel blade , stainless steel blades do not develop rust , allowing a lifetime use of the handles . of note , the design of the current handles and cuffs is the result of a process , which has led to the proposed sizes , allowing for the most efficient anastomoses . minimal variations can be tolerated in terms of length , with a usual length of about 4 mm ( figures 3 and 4 ) . regarding the approximator , the selected kocher 's forcep should have handles at least 4 cm from the fulcrum . this allows for a safe closure of the rack outside the animal 's abdomen . overall , the proposed device can allow easier vein anastomoses for the transplantation of liver and other types of grafts in rats .

orthotopic rat liver transplantation is a popular model , which has been shown in a recent jove paper with the use of the " quick - linker " device . this technique allows for easier venous cuff - anatomoses after a reasonable learning curve . the device is composed of two handles , which are carved out from scalpel blades , one approximator , which is obtained by modifying kocher 's forceps , and cuffs designed from fine - bore polyethylene tubing . the whole process can be performed at a low - cost using common laboratory material . the present report provides a step - by - step protocol for the design of the required pieces and includes stencils .

a 46-year - old gentleman presented with sudden onset of loss of vision following blunt trauma to the right eye . the patient had sustained the injury when he was assaulted with a stone . he had undergone cataract surgery with posterior chamber ( pc ) iol in the right eye three months ago elsewhere . on examination his visual acuity was perception of light , accurate projection of rays in the right eye and 20/30 , n6 in the left eye . anterior segment evaluation revealed iol haptic ( " j " type ) extrusion through the scleral tunnel with hyphema , aniridia and posterior capsular remnant in the right eye [ figs 1,2 ] . his intraocular pressure ( lop ) was undetectably low in the right eye and 16mmhg in the left eye . he was diagnosed to have traumatic rd with vitreous hemorrhage with iol extrusion with total aniridia . he was advised iol haptic explantation , vitreoretinal surgery with encircling band and silicone oil . intraoperatively , after clearing the vitreous hemorrhage , the total rd was evident , with two peripheral retinal tears . the breaks were well supported after the application of silicon encircling band ( mira - 240 with a width of 2.5 mm ) . hence c 3 f 8 ( perfluoropropane ) gas was used as a tamponade . . the inferior and nasal 180 posterior capsule was intact and was left behind for a secondary implant later . at six weeks follow - up his best - corrected visual acuity ( bcva ) was 20/120 + 1 , ni0 with aphakic correction . anterior segment evaluation showed aphakia with aniridia and posterior capsular remnant in the right eye [ fig . 3 ] . indirect ophthalmoscopy showed attached retina with epiretinal membrane ( erm ) and mild macular pucker in the right eye [ fig . follow - up bcva was 20/80 ( hazy ) , n8 in the right eye with correction . fundus examination showed attached retina with erm and internal limiting membrane striae in the right eye . optical coherence tomography ( oct ) showed erm and cystoid macular edema ( cme ) . he underwent secondary aniridic pciol ( io care baroda ; optic 10 mm , central 2.25 mm opaque with surrounding 5.5 mm clear , a constant 118.2 ) with erm removal in the right eye under local anesthesia . although a scleral fixated iol was planned , the iol when placed on the inferior capsular rim was found to be stable . the posterior capsule had fibrosed since the injury and hence provided adequate support without the need for scleral fixation . moreover , since the iol was rigid and measured 12.75 mm in length , it was found to be stable in the scleral sulcus . the incision had to be widened to 10 mm to accommodate the iol . at six weeks follow - up his bcva was 20/30 and n6 , with -2.00 diopter cylinder x 170 and lop was 15 mmhg . indirect ophthalmoscopy revealed normal disc , macula and attached retina with good buckle effect [ fig . optical coherence tomography revealed resolution of cme . at six months follow - up the above findings were maintained . these patients often describe discomfort or difficulty in brightly lit areas , such as on sunny days . a history of blunt trauma besides damage to the iris ; tends to be associated with trauma to the angle or zonules . the most common of these are traumatic cataract , retinal detachment and vitreous hemorrhage . both penetrating and blunt injuries can cause traumatic total iridectomy.1 in our case there was significant damage to the iris with a crescent of posterior capsule intact . iridoplasty , colored contact lenses and corneal tattooing have been developed to overcome the visual function impairments that arise from damage to the iris.2,3 however , when significant amounts of iris tissue are damaged or missing , iris repair may be impossible . in these eyes , several ophthalmologists have used this artificial iris implants called aniridic iol in patients with traumatic and congenital aniridia.4 additional procedures along with iol implantation may be required , including cataract surgery , transscleral iol fixation , keratoplasty and vitreoretinal procedures . a combined aniridic iol implantation and vitreoretinal procedure is possible without significant intraoperative complications.5 a black diaphragm iol design allows simultaneous treatment of aniridia and aphakia6 as was the situation in our case wherein the previously implanted iol had extruded through the scleral tunnel and was removed in the first sitting along with retinal detachment surgery . at the second sitting the aniridic iol the patient did not suffer from any of the complications associated with such similar procedures such as , persistent intraocular inflammation , glaucoma , endophthalmitis and residual photophobia . the patient had good visual recovery , with his vision being 20/30 on his last follow - up six months after the surgery . we present a rare case of traumatic aniridia with vitreous hemorrhage and retinal detachment , in which a good visual outcome was achieved after a combined surgery . after a detailed medline search , there were less than five such cases reported in the literature . both the cost factor and surgical time could be reduced with a combined approach giving an excellent cosmetic , visual and surgical outcome .

a 45-year - old man presented with post - traumatic aniridia . we describe the combined surgery done to treat both aniridia and epiretinal membrane simultaneously . a combined aniridia intraocular lens and vitreoretinal surgery was done . the case report highlights the advantage of combined surgery in terms of cost factor and surgical time .

eed is a localized , low - grade form of leukocytoclastic vasculitis of unknown pathogenesis characterized by persistent , symmetrical , red - purple papules , nodules , and plaques affecting the extensor surfaces of the extremities , the ears , trunk and buttocks . dermatitis herpetiformis is an autoimmune blistering disease characterized by granular deposits of immunoglobulin a ( iga ) in dermal papillae . the common link between these two disorders is the deposition of ig a antibodies , underlying gluten sensitivity in both , their association with celiac disease and response to treatment with dapsone . a 50-year - old male presented with firm skin - colored and erythematous nodules over the knuckles , elbows , buttocks , knees , and lower legs which gradually increased in size in since 3 years duration [ figure 1 ] . complete blood picture , liver function tests , urine routine , blood sugar were within normal limits . biopsy of the nodules of the knuckles showed focal parakeratosis and mild spongiosis in the epidermis , perivascular neutrophilic infiltrate in the dermis , neutrophils in the vessel wall and leukocytoclasia [ figure 2 ] . immunofluorescence showed linear iga deposits in the basement membrane zone and weak granular perivascular c3 and fibrinogen deposits [ figure 3 ] . four years ago itchy vesicles over the back and arms were detected in the patient . he was administered dapsone which leads to disappearance of lesions with recurrence after stopping treatment . with the clinical presentation , biopsy and immunofluorescence results , a diagnosis of erythema elevatum diutinum on a background of dermatitis herpetiformis was made . skin - colored nodules over the knuckles neutrophilic vasculitis with leucocytoclasia ( h and e , 400 ) linear iga deposits in the basement membrane zone dermal papillary microabscess ( h and e , 400 ) erythema elevatum diutinum ( eed ) is a rare cutaneous condition that initially presents as leukocytoclastic vasculitis ( lccv ) of the skin and later resolves with fibrosis clinically characterized by persistent red - purple to yellow papules , plaques , and nodules . the symmetrical lesions typically affect the acral surfaces of the body , especially the extensor surfaces of the hands , but other areas such as the buttocks may also be involved . antecedent bacterial infections , collagen vascular diseases , myeloproliferative diseases , human immunodeficiency virus infection , cryoglobulinemia , wegener granulomatosis , crohn disease , systemic fungal diseases , iga paraproteinemia , and other associations have been reported . histopathologically , eed initially presents as a leukocytoclastic vasculitis ( lccv ) with polymorphonuclear neutrophils , extravasated red cells , nuclear dust , and fibrin deposits in the walls of the small arterioles . dermatitis herpetiformis ( dh ) is characterized by chronic , intensely pruritic , polymorphic , vesicles usually appearing on elbows , knees , buttocks , and scalp associated with gluten - sensitive enteropathy there are granular iga deposits in the dermal papillae by the direct immunofluorescence in the perilesional skin of patients with dermatitis herpetiformis . the close association of iga with erythema elevatum diutinum and dermatitis herpetiformis is well known . because of the association with iga , the presence of erythema elevatum diutinum in a patient with chronic dermatitis herpetiformis is not surprising . the iga in the skin may be an epiphenomenon or may represent deposition in the skin along with other immune complexes . aftab mn et al . have reported a case of erythema elevatum diutinum arising in the setting of dermatitis herpetiformis . they concluded that iga immune complexes within the basement membrane zone region may trigger a cascade that eventuates into a blister or the formation of the chronic vasculitis of erythema elevatum diutinum .

erythema elevatum diutinum ( eed ) is a rare skin disease that initially presents as leucocytoclastic vasculitis and later resolves with fibrosis . dermatitis herpetiformis is an autoimmune blistering disease characterized by granular deposits of immunoglobulin a ( iga ) in dermal papillae . we report a rare association of these two disorders .

a 61-year - old woman came to our attention and was hospitalized for the acute onset of fever ( 38c ) , erythroderma , and pustulosis ; the pustules were mainly localized on her shoulders , upper arms , and trunk [ figure 1 ] . in the latter area clinical history revealed that she had been treating a bacterial inguinal intertrigo for 4 days with ciprofloxacin 500 mg tablets twice daily and desloratadine 5 mg tablet once daily . she referred that she had already taken ciprofloxacin in the past for refractory urinary tract infections . laboratory tests showed leukocytosis ( 23,000/ml , nv 390011700 ) , neutrophilia ( 86.4% , nv 39.674.7 ) , increased c - reactive protein ( 188 mg / l , nv < 8) , increased fibrinogen ( 505 mg / l , nv 200400 ) , increased d - dimers ( 4.8 mg / l , nv < 0.5 ) , slightly increased urea ( 41 mg / dl , nv 1538 ) , and decreased glomerular filtration rate ( 66 ml / min , nv > 90 ) . skin biopsy showed a nonfollicular , subcorneal , neutrophilic pustule with eosinophils , histiocytes , mild spongiosis , and moderate papillary dermal edema . erythroderma and pustulosis of our patient at the first day of hospitalization the cutaneous morphology , course , histological findings , and laboratory examinations were consistent with a definite diagnosis of agep according to the validation score of the euroscar study group ; we therefore reported it to italian pharmacovigilance . we immediately stopped the ciprofloxacin and started infusional therapy with methylprednisolone 40 mg once daily and trimeton ( chlorphenamine ) 10 mg twice daily . the rash significantly improved within a few days ; after complete resolution of the lesions , patch tests and lymphocyte proliferation test performed with ciprofloxacin both resulted negative . the most important step of agep diagnosis is to identify the provocative agent : if it is a drug , it must be discontinued immediately , if an infection , immediately treated . commonly , agep is caused by penicillins and cephalosporins , sometimes by fluoroquinolones ( especially norfloxacin and ofloxacin ) . to the best of our knowledge , this is the third reported case of agep caused by ciprofloxacin , supporting two other previous reports . in 2005 , knoell and lynch reported the case of a 63-year - old man with a photoinduced acute exanthematous pustulosis ( an unusual form of agep ) caused by ciprofloxacin and exposure to ultraviolet light . diagnosis was made on the basis of clinical and histological findings . in the same year , hausermann et al . reported another case of ciprofloxacin - induced agep in an 80-year - old woman who also showed a positive patch test to the drug . patch testing with the drugs involved is a useful and noninvasive way to identify the culprit agent of agep but is characterized by low sensitivity , being positive in about 60% of cases . the lymphocyte proliferation test seems promising but likewise has low sensitivity . as in our case , a negative reaction to these tests should not prompt a reconsideration of the diagnosis that remains primarily clinical .

acute generalized exanthematous pustulosis ( agep ) is an uncommon and self - limiting skin rash commonly caused by drugs and is characterized by the acute onset of fever , pustulosis , and neutrophilia from 4 to 10 days after the drug intake . we describe a case of agep in a 61-year - old woman that was hospitalized for the acute onset of fever , erythroderma , and pustulosis . clinical history revealed that she had been treating a bacterial inguinal intertrigo for 4 days with ciprofloxacin 500 mg tablets twice daily and desloratadine 5 mg tablet once daily . to the best of our knowledge , this is the third reported case of agep caused by ciprofloxacin , supporting two other previous reports .

psychocutaneous diseases can present either as primary psychiatric diseases such as dermatitis artefacta and delusions of parasitosis or as secondary psychiatric conditions such as depression , anxiety or social phobia due to chronic dermatoses like psoriasis , atopic dermatitis , alopecia areata . while in the former group , the diagnosis is usually straight forward , in the latter group such as psoriasis , atopic dermatitis etc the associated psychiatric co - morbidity may be missed or overlooked . unless the dermatologist has a special interest in mental health , in a busy practice , the relevant history is not sought . invisible mental disease in addition to the visible skin disease . in chronic skin diseases , patients feel stigmatized especially when lesions are widespread and may also experience depression . even in clinical remission , some may have anxiety about possible relapse in future . the relationship between mind and skin can be understood on the basis of the neuro - immuno - cutaneous - endocrine network . there is a dedicated european society of dermatology and psychiatry that holds a congress biennially . the association for psychocutaneous medicine of north american too holds regular meetings . in india , this subject has received scantattention . case reports and few studies appear from time to time . whilst dermatologists refer cases to psychiatrists , a dedicated liaison clinic is virtually unknown . we discuss the working pattern of the dermatology - psychiatry - clinical psychology liaison clinic set up at manipal in august 2010 . patients with chronic dermatoses such as psoriasis , eczema as well as those with primary psychiatric conditions were recruited by the dermatologist from the general dermatology out patients and referred to the liaison clinic which functions once weekly from 2:30 to 5:00 pm . the psychologist counseled all patients and if stressors were elicited , coping strategies were taught . both specialists discussed the case with the dermatologist and standard dermatologic treatment with or without psychopharmacologic agents and/or psychological interventions were advised . we examined 175 cases ( 117 females ; 58 males ) aged between 10 and 75 years maximum being in the second and third decade . out of 175 , primary dermatological cases constituted 154 ( 88% ) [ table 1 ] and primary psychiatric 21 ( 12% ) [ table 2 ] . the leading primary dermatosis was psoriasis in 40 ( 23% ) while the leading primary psychiatric disease was neurotic excoriations in 6 ( 3% ) patients . out of 92 ( 53% ) who needed psychologic interventions , only 25 ( 27% ) underwent the same . 12 patients ( 48% ) underwent one session while only 3 ( 12% ) had more than five sessions . forty seven patients ( 30% ) with primary dermatosis had an associated psychiatric diagnosis [ table 4 ] the most common being dysthymia in 22 ( 46% ) . the three leading dermatoses with psychiatric co - morbidity were psoriasis , prurigo / generalized pruritus and chronic dermatitis . a major advantage of a combined clinic is the prompt availability of a psychiatrist , dermatologist and a clinical psychologist at a single visit . the stigma of visiting the psychiatry department although has lessened in recent time , stillexists . our clinic functions in an informal manner without any inhibitions for patients about meeting a mental health professional as there are no boards mentioning the designations of the liaison specialists . the number of patients examined can not exceed four or five as detailed interviews are carried out . a level - one dermatologist is a well informed specialist who can treat psychocutaneous diseases but does not bring about psychological change in the patient while a level two dermatologist is one who liaises with a clinical psychologist or psychiatrist bringing about psychological changes . by liaisoning , patient can be treated in a holistic fashion thereby addressing both the psychological and the physical needs . in our study although stress has been implicated in several dermatoses , psoriasis is the one which is mostly associated with . stress induced derangements of epidermal function may precipitate inflammatory dermatoses . nonpharmacologic interventions are stress reducing adjuncts that can enhance the efficacy of standard dermatogic therapies . some of the therapies useful in dermatologic patients are biofeedback , relaxation training , hypnosis and psycho - education . in our study majority of patients were non - compliant with psychological interventions . probably they did not realize the benefit or were not keen on attending the clinical psychology department . this underscores the need for dermatologists to master selected psychological techniques such as relaxation which can easily be taught to patients in the office . 30% of our patients had psychiatric comorbidity which is in agreement with several studies in psychodermatology . in conclusion , dermatologist should liaise with psychiatrists or clinical psychologists when managing psychosomatic dermatoses . in university teaching hospitals dermatologists should familiarize with selected psychopharmacological drugs and simple nonpharmacologic interventions . screening for common psychiatric conditions such as anxiety and depression

psychodermatology is an emerging specialty in dermatology which deals with the interactions between mind and skin . psychocutaneous diseases can be either primary psychiatric or primary cutaneous , with various degrees of associations between psyche and skin . unless the dermatologist cultivates a special interest in this field , many an invisible mental disorder may be missed leading to sub optimal treatment of the visible skin condition . though dermatology psychiatry liaison clinics are common in europe and other western countries , it is just an emerging concept in india . here we describe the working pattern of psychodermatology liaison clinic established in manipal in august 2010 and describe briefly the type of cases attended to .

ventriculoperitoneal ( vp ) shunting for hydrocephalus is a common neurosurgical procedure in our daily practice . migration of distal part of vp shunt into the adjacent organs including bowel tract , thoracic cavity , heart , scrotum , umbilicus , abdominal wall , and urinary bladder is reported . a case of repeated shunt migration in different organs this is the first case of bowel perforation with transanal extrusion continued with bladder migration and urethral extrusion of peritoneal shunt . we report our case of a child with these unusual complications ; treatment was done by a team involving neurosurgeons and urologists . a 4-year - old boy was admitted to the hospital because of abdominal discomfort . on further examination the first operation was undertaken when he was 2 months old because of his congenital hydrocephalus . the patient went back to the hospital when he was 1 year old because of severe headache . computed tomography ( ct ) scan confirmed a large right porencephaly . his abdominal x - ray revealed that the extruded shunt was the right peritoneal catheter [ figure 2 ] . the result was showing 200 cells found with protein 100 mg / dl , glucose 13 mg / dl , and the culture showed micrococcus sp . urological procedure was performed endoscopically through the urethra to explore the site of migration and potential of leakage . it was found that the shunt was penetrated through the bladder wall just above the trigonal area [ figure 3 ] . we decided to treat conservatively by inserting an urethral catheter to keep the bladder decompressed for optimal healing process . extruded shunt was exposed through the external urethral orificium abdominal x - ray shows the extruded ventriculoperitoneal shunt cathether through the urethral orificium during endoscopic urological surgery , migration site just above the triogonal area of the urinary bladder was identified ( blue arrow ) thereafter , intraventricular antibiotic was administered according to the culture results . one month later , culture of csf was performed and it showed a sterile result . bowel perforation was present in 0.1%0.7% of cases in which colon was the most involved organ , and the most common site of extrusion is anus ( 68.9% ) , followed by scrotum ( 22% ) , umbilicus ( 6.9% ) , and vagina ( 3.2% ) . a case of urinary bladder migration as well as urethral extrusion of vp shunt is extremely rare . there have been various theories proposed such as bioreactivity , local inflammation , infection , and surgical error . the location of the bladder makes it highly unlikely site of perforation , as the catheter must pass through the peritoneum into the extraperitoneal space and subsequently perforate the bladder . in this present case , the first migration of the catheter was into the anus , and this is considered as a frequent event of migration . the second migration into the bladder is a rare case . symptom was only abdominal discomfort and it became obvious after the expose through the urethral orifice . repeated case of shunt migration is an interesting case regarding the pathophysiology and management of this patient . with the history of prior bowel perforation by shunt catheter , and cultures of specimens indicating an infection fixation of the tip catheter at the specific site of the peritoneum is facilitated and then , continuous erosion and perforation of the peritoneum into the bladder was done . the trigonal area , which comprises less muscle layer , was the site of migration in this case . there are three main procedures of shunt removal : gentle pulling , endoscopic surgery , and major open surgery . as per earlier reports , catheter removal is mostly done by open abdominal surgery , but in author 's opinion , removal of the catheter could be done with a less invasive method . in our case presenting per - urethtraly , the catheter was gently pulled out through the urethral orifice . the surgery was then followed by urological endoscopic exploration through the urethra to view the size and location of the perforation . it is believed that the perforation site seals off due to the presence of a chronic fibrous sheath around the shunt tract and requires no aggressive surgical intervention . we found the pitfall of this case to be the lack of detailed history of the previous operation because the patient underwent the surgery in another hospital . the repeated migrations also give us some thought of another option of shunting , for example , ventriculo - atrial shunt . bladder migration and transurethral extrusion is an extremely rare complication of vp shunt . to our knowledge , this is the ninth case reported . the presence of previous abdominal complication and repeated organ perforation indicates predisposition factors which must be sought and handled before any further treatment .

bladder migration and transurethral extrusion is an extremely rare complication of ventriculoperitoneal ( vp ) shunt . only eight cases have been reported in the english literature since 1995 . we report a case of a 4-year - old boy with cerebral palsy , hydrocephalus , and vp shunted on both sides who presented with a protruded distal vp shunt from his urethral orifice . the patient was reported for having previous shunt extrusion through the anus . the patient was treated on by a multidisciplinary approach , involving a neurosurgeon and urologist . shunt removal with simple procedure was smoothly achieved without morbidities . he was discharged home in satisfactory condition .

guided cell migration requires the generation of polarized plasma membrane protrusions such as filopodia , lamellipodia , blebs and invadopodia . although these protrusions differ structurally and in their composition , they each contribute to cell movement by pushing the leading edge forward . both axonal growth cones and endothelial tip cells display abundant filopodia protrusions ( fig . 2 ) . their dynamic behavior of extension and retraction is reminiscent of antennae probing their environment and are generally thought to serve as environmental sensors that integrate extracellular signals during directed migration . growth cone morphology , reflected for instance by filopodia number , has been correlated with guidance decisions at choice points and filopodia preferentially contact target cells , sometimes over long distance . in vivo experiments with titrated doses of an inhibitor of f - actin polymerization , cytochalasin b , blocked growth cone filopodia formation in grasshopper ti1 neurons and in xenopus retinal ganglion cell neurons . while pathfinding was disrupted in conditions without filopodia , only minor effects were seen on axonal extension , suggesting that filopodia are dispensable for growth but essential for guidance . similarly , in vitro treatment with cytochalasin b abolished glutamate - induced growth cone turning with only slight effects on neurite extension . however , a little known study on retinal ganglion cells suggests that axon guidance can occur without filopodia . here , the authors demonstrate that growth cones extend few or no filopodia after inhibiting ena / vasp function . while axonal elongation was slowed down , retinal ganglion cell axons devoid of filopodia showed normal trajectories in vivo and growth cone navigation across several choice points was unaffected . axonal growth cones ( a , image courtesy of isabelle brunet ) and endothelial tip cells ( b ) extend long filopodia ( arrowheads ) in the direction of migration . scale bars , 10 m . similar to growth cones , endothelial tip cells at the leading edge of vascular sprouts produce many long filopodia that extend toward the direction of vascular growth during angiogenesis . these filopodia express the vegf - a receptor , vegfr2 , and become misdirected and shorter upon disruption of vegf - a distribution in the mouse retina . as filopodia have been proposed to act as sensors of the extracellular milieu and endothelial cells respond to axon guidance molecules such as slits and roundabouts , netrins and unc5 receptors , semaphorins , plexins and neuropilins , and ephrins and eph receptors , it has been widely assumed that they sense and integrate pro - angiogenic and repulsive cues in tip cells to enable guided migration and stereotypic vessel patterning . however , the role of filopodia in vessel guidance has never been proven nor questioned . recently , work from our laboratory demonstrated that filopodia are not essential for mediating endothelial tip cell guidance . by using low concentrations of latrunculin b ( lat . b ) , which prevents f - actin polymerization , endothelial filopodia formation was abolished in the zebrafish embryo . live microscopy revealed that endothelial tip cells of isvs without filopodia continued to migrate along normal trajectories to form the stereotypic isv pattern and to anastomose with other tip cells . at the low concentrations of lat . b used , tip cells were able to generate lamellipodia that provided the driving force for cell movement although at a decreased velocity . furthermore , the induction of new vascular sprouts toward sources of ectopic vegfa165 ensued in the absence of filopodia . in summary , our study shows that during angiogenesis , endothelial filopodia are dispensable for tip cell guidance . this finding complements that of dwivedy et al . , who showed that filopodia are also not essential for axonal growth cone navigation and challenges the long - standing notion that filopodia are required for guided migration . in fact , filopodia or filopodia - like structures have been ascribed many other functions . these include facilitating cell - cell matching and epithelial sheet adherence during dorsal closure in drosophila , transmitting signals such as delta - notch and sonic hedgehog signaling between non - neighboring cells , inducing cell shape changes required for preimplantation embryonic development by providing tension and positioning nuclei in nurse cells during oogenesis in drosophila . in endothelial cells , we propose that filopodia serve as templates from which lamellipodia emerge and that both protrusive structures coordinate to allow efficient migration and expansion of new vascular sprouts . in addition , tip cell filopodia facilitate the process of anastomosis , a process whereby tip cells meet , fuse and establish new junctions to form a connected vascular network . thus , the mechanism(s ) by which blood vessels are guided by extracellular cues is still unresolved .

filopodia are highly dynamic , rod - like protrusions that are found in abundance at the leading edge of migrating cells such as endothelial tip cells and at axonal growth cones of developing neurons . one proposed function of filopodia is that of an environmental probe , which serves to sense guidance cues during neuronal pathfinding and blood vessel patterning . however , recent studies show that tissue guidance occurs unhindered in the absence of filopodia , suggesting a dispensability of filopodia in this process . here , we discuss evidence that support as well as dispute the role of filopodia in guiding the formation of stereotypic neuronal and blood vessel patterns .

a 44-year - old man , who had a medical history of panic disorder , visited another hospital due to dyspnea on mild exertion . a coronary angiography showed a single coronary artery originating from the right coronary ostium . a single coronary artery bifurcated into the right coronary artery and left main coronary artery . the left main coronary artery coursed between the main pulmonary artery and aorta before bifurcating into the left descending artery and circumflex artery . we thought that panic symptoms or dyspnea on exertion might be a sign of myocardial ischemia due to compression of the left main coronary artery by the pulmonary artery and aorta . we decided that surgical treatment was the best option due to the high risk of sudden death associated with a coronary anomaly . under general anesthesia we dissected the left main coronary artery between the aorta and the main pulmonary artery on the beating heart . the proximal left main coronary artery was bifurcated from a single coronary artery that originated from the right coronary sinus . under cardiopulmonary bypass , only one coronary ostium was observed in the right coronary sinus , and a single coronary artery originated from the ostium . a 5-mm arteriotomy was made to the left main coronary artery at the site in which the left coronary ostium should have been located . neo - ostium formation was performed with a 5-mm puncher in the left coronary sinus . anastomosis between the neo - ostium and the left main coronary arteriotomy site was performed using a 7 - 0 prolene continuous running suture ( fig . the aortic cross clamping time was 88 minutes , and total cardiopulmonary bypass time was 117 minutes . follow - up computed tomographic angiography before discharge showed good patency of the neo - ostium in the left coronary sinus without stenosis at the anastomosis site ( fig . the patient remained asymptomatic without any complications or events for 15 months after the surgery . angelini reviewed 1,950 coronary angiographies and reported that the incidence of right coronary artery originating from the left coronary sinus was 0.92% and vice versa was 0.15% , with a total incidence of 1.07% . patients are usually asymptomatic . however , it may cause angina , syncope , and even life - threatening complications such as myocardial infarction or ventricular fibrillation . thus , we must consider surgical treatment or intervention if signs of myocardial ischemia are present . several surgical techniques can be utilized to treat coronary anomalies , such as coronary reimplantation to the original sinus , coronary artery bypass graft ( cabg ) , pulmonary artery translocation , unroofing , and neo - ostium formation . in this case , the left main coronary artery was bifurcated from a single coronary artery originating in the right coronary sinus . furthermore , the left main coronary artery passed between the pulmonary artery and aorta to reach the left heart . cabg , coronary reimplantation , unroofing or neo - ostium formation could therefore have been considered as viable surgical options . coronary reimplantation is one of the most physiologically beneficial repairs , but is technically difficult , and stenosis may occur at the site of anastomosis . cabg is technically feasible , but the arterial conduit has a competitive flow problem if no stenotic lesions are present on the natural coronary artery . also , a vein conduit may be problematic if the patient is young because of long - term patency . however , extended unroofing may cause valve insufficiency if the anomalous coronary artery is located under the valve commissure . unroofing was not proper for our case because a separated left main coronary artery originated from a single coronary artery , not the right coronary sinus . neo - ostium formation in the left coronary sinus without unroofing was considered to be a proper surgical treatment in this case . successful surgical treatment of anomalous of coronary anomaly depends on expertise in anatomic and hemodynamic pathophysiology , in addition to the selection of the appropriate surgical treatment option . we report that this case was successfully treated with neo - ostium formation in anomalous origin of the left coronary artery from the right coronary system .

anomalous origin of a coronary aortic artery is a rare cardiac anomaly . although it can cause angina , syncope , and palpitations , most patients are asymptomatic . this anomaly requires surgical treatment or intervention because it is associated with sudden death . several surgical techniques , such as coronary reimplantation , coronary artery bypass grafting ( cabg ) , unroofing , and neo - ostium formation , have been proposed as treatments . we report a case surgically treated with neo - ostium formation in anomalous origin of the left coronary artery from the right coronary sinus .

incidental vessel injury caused by some of these variations may require more extensive lung resection than would be necessary without repair during pulmonary resection . we herein describe a patient with a common trunk of the left pulmonary vein that was incidentally transected with a mechanical stapler during a left upper lobectomy . we also describe the reconstruction procedures that were successfully used for the concomitantly transected inferior pulmonary vein . a 62-year - old man complained of cough and bloody sputum and consulted our hospital . chest computed tomography ( ct ) showed a nodule with a diameter of 15 mm in the left upper lobe . positron emission tomography and brain magnetic resonance imaging revealed a clinical stage of t1n0m0 as categorized by the 7th edition of the union for international cancer control ( uicc ) classification . the common trunk of the left pulmonary vein was misidentified as a superior pulmonary vein until incidental transection with an endostapler during port access surgery . the left upper bronchus and pulmonary arteries to the left upper lobe were transected with endostaplers , and the common trunk of the pulmonary vein was left intact . the left main pulmonary artery was then clamped under intravenous heparin injection to achieve an activated coagulation time of 200 s. annuloplasty of the inferior pulmonary vein was completed during intermittent declamping of the left pulmonary artery . the orifice of the inferior pulmonary vein was augmented by a cuff technique using an orifice of the superior pulmonary vein , as shown in fig . 1 . the staples on the stump of the left common pulmonary vein on the atrial side were removed to adjust the augmented orifice of the left inferior pulmonary vein under partial clamping of the left atrium following pericardiectomy . end - to - end anastomosis was completed using running sutures with a 4 - 0 polypropylene thread . the postoperative course was uneventful , and the patient was discharged on postoperative day 14 . a pathologic study showed stage t1n2m0 small - cell carcinoma as categorized by the 7th edition of the uicc classification . postoperative enhanced ct demonstrated successful reconstruction of the left common trunk ( fig . 2 ) . 1.orifice of an inferior pulmonary vein was augmented with a cuff technique using the orifice of a superior pulmonary vein . the augmented orifice was anastomosed to the common pulmonary vein on the left atrial side after removal of staples as long as necessary under a partial clamp of the left atrium following pericardiotomy . fig . 2.postoperative enhanced ct showing ( frontal view ) successful reconstruction of an inferior pulmonary vein ( arrow ) . orifice of an inferior pulmonary vein was augmented with a cuff technique using the orifice of a superior pulmonary vein . the augmented orifice was anastomosed to the common pulmonary vein on the left atrial side after removal of staples as long as necessary under a partial clamp of the left atrium following pericardiotomy . postoperative enhanced ct showing ( frontal view ) successful reconstruction of an inferior pulmonary vein ( arrow ) . the branching pattern of the pulmonary vessels is variable . pulmonary vein variations such as upper lobe venous drainage posterior to the intermediate bronchus and a common trunk of the left pulmonary vein may cause lethal complications during and after anatomical pulmonary resection . based on ct findings , a common trunk of the left pulmonary vein reportedly occurred with a frequency of 14% among 201 cases . although three - dimensional ct and multidetector ct have been developed to detect pulmonary vessel variations preoperatively , incidental transection of the common trunk may be unavoidable when the pulmonary vein is anteriorly transected with no identification of the inferior vein during left upper lobectomy . furthermore , the use of video - assisted surgery and endoscopic devices has become more widespread , and skin incisions and exposure of anatomical structures have become more limited . a previous paper reported that the orifice of the inferior pulmonary vein was augmented with the use of a pericardial patch followed by anastomosis when the common trunk was incidentally transected . in this report , annuloplasty of the inferior pulmonary vein with a cuff technique using an orifice of the superior pulmonary vein allowed for much easier performance of end - to - end anastomosis , leading to successful reconstruction without a patch or prosthesis . this augmented technique may also be applicable to patients with lung cancer in the right upper lobe when the tumor is invading the right common superior trunk branching pulmonary veins to the upper and middle lobes .

a common trunk of the left pulmonary vein is an anatomical variation in the pulmonary vessels and may be incidentally transected during left upper lobectomy . difficulty in reconstruction of the left inferior vein often requires completion pneumonectomy . we herein describe a patient with lung cancer in the left upper lobe of the lung . his common trunk of the left pulmonary vein was incidentally transected with a mechanical stapler during a thoracoscopic left upper lobectomy . the concomitantly transected inferior pulmonary vein was augmented with a cuff technique using an orifice of the superior vein followed by end - to - end anastomosis . the postoperative course was uneventful . this technique should also be considered in patients with lung cancer when a right upper lobe tumor is invading the right superior trunk branching pulmonary veins to the upper and middle lobes .

massive hemoptysis is a life - threatening medical emergency with mortality rates ranging as high as 75% . transcatheter embolization of bronchial arteries has been regarded as an effective and safe method for the management of massive hemoptysis . chronic pulmonary thromboembolism ( pte ) is one of these causes , though its exact incidence remains unknown . compensatory hypertrophy of bronchial arteries is known to occur in chronic pte and pulmonary hypertension . however , the exact mechanism of hemoptysis in these patients is unclear . bleeding due to the rupture of smaller vessels or bleeding from hypertrophied bronchial arteries though bronchial artery embolization has been proposed to be effective in the management of hemoptysis , the ideal treatment for hemoptysis in patients with chronic pte remains uncertain . we report one case of hemoptysis in chronic pte with pulmonary arterial hypertension ( pah ) treated successfully with bronchial artery embolization . a 30-year - old male patient presented to the emergency department of our institute with recurrent bouts of hemoptysis for 1 week , along with shortness of breath . , he was found to have tachycardia ( pulse rate : 100 bpm ) and tachypnea ( respiratory rate : 30 breaths / min ) with decreased oxygen saturation ( 85% in room air ) . the patient underwent computed tomography ( ct ) and bronchial angiography that showed two bronchial arteries on the right side , which were hypertrophied and tortuous ( measuring ~2.6 mm and 2.4 mm , respectively , in maximum caliber ) [ figure 1a ] . in addition , there was a thrombus in the right pulmonary artery , extending from its origin till the subsegmental branches [ figure 1b ] . the right pulmonary artery was reduced in caliber with the dilated main and left pulmonary arteries . two - dimensional echocardiogram ( 2d echo ) was done for the patient that showed severe pah with dilated right atrium and ventricle . on bronchoscopy , active bleeding with clots was seen in the segmental bronchus of the right lower lobe . ( a , b ) coronal maximum intensity projection ( mip ) images of ct bronchial angiography showing hypertrophied right bronchial arteries ( arrows in a ) . one of the bronchial arteries was arising from the intercostobronchial trunk from the descending aorta ( not shown here ) . origin of the second right bronchial artery was , however , not clear on ct angiography . a thrombus was seen in the right pulmonary artery ( arrow in b ) extending till the subsegmental branches the patient was taken up for bronchial angiography . the second bronchial artery was being reconstituted by the internal mammary artery and was hypertrophied [ figure 3 ] . both the bronchial arteries were embolized with polyvinyl alcohol ( pva ) ( 500 - 700 m ) and gelfoam slurry . no other significant systemic collateral was identified on diagnostic runs of the bilateral subclavian arteries . digital subtraction angiogram of the right intercostobronchial trunk ( anteroposterior projection ) showing hypertrophied and tortuous bronchial artery . postembolization angiogram showed no opacification of the distal bronchial artery ( image on the right side ) digital subtraction angiogram of the right internal mammary artery showed hypertrophied second right bronchial artery arising from the internal mammary artery . chronic pte is associated with compensatory hypertrophy of the bronchial arteries and bronchopulmonary collaterals . increased incidence of bronchial artery hypertrophy on ct angiography has been reported in chronic pte and pah . though hemoptysis can occur due to hypertrophied bronchial arteries secondary to any cause , its exact incidence in chronic pte is unknown . one systemic review of the literature reported chronic pte as a cause of moderate - to - massive hemoptysis in 0.1% of cases . we report one case in which bronchial artery embolization was successful in managing hemoptysis in a patient with chronic pte with pah . one patient was successfully treated while the other patient continued to have active hemoptysis due to collaterals from the subclavian artery . reembolization was carried out for this patient but the patient died due to right ventricular failure . it was suggested that due to the requirement of anticoagulation for pte , even mild hemoptysis in these patients should be offered immediate intervention . however , another study reported the failure of bronchial artery embolization to prevent hemoptysis in a patient with chronic thromboembolic pah . in chronic pte , there is a propensity for the formation of thromboemboli . at the same time even mild hemoptysis in these patients should be given prompt treatment due to the underlying risk of progression to massive hemoptysis due to the ongoing anticoagulation therapy . however , very limited literature is available on the management of hemoptysis in chronic pte . no study has been carried out to evaluate the long - term effect of bronchial artery embolization in preventing hemoptysis . also , the precise mechanism of hemoptysis in chronic pte is still not clear . thus , further studies are required to establish the role of bronchial artery embolization in chronic pte .

bronchial artery embolization is the treatment of choice for the management of life - threatening massive hemoptysis . chronic pulmonary thromboembolism ( pte ) is one of the rare causes of hemoptysis . management of hemoptysis in chronic pte is a point of debate . in this article , we have reported one case of hemoptysis in chronic pte managed successfully with bronchial artery embolization .

patients with obsessive compulsive disorder ( ocd ) have been traditionally described as having a good insight into their symptoms ; they perceive their obsessive - compulsive ( oc ) symptoms as excessive , unreasonable , and distressing . the dsm iv field trial demonstrated that about a quarter of the patients were uncertain about whether their symptoms were unreasonable or excessive , indicating that a broad range of insight exists among patients with ocd . it is now well - recognized that patients with ocd may present with varying degrees of insight , including poor and complete lack of insight into their oc symptoms.[24 ] pathological beliefs appear to be placed along an symptom component properties , they determine obsessions at one end , where beliefs are recognized as irrational . overvalued ideas lie somewhere in the middle , and delusions where the beliefs are considered rational , lie at the other pole . it can be a phenomenological challenge when the thin line of separation between these becomes difficult to discern . ms . s , a 35-year - old , married lady presented with a one - year history of marked social withdrawal , muttering to herself , and suspiciousness . on clarification with her husband regarding her suspiciousness it was found that she kept enquiring from him whether their acquaintances had visited her or not , subsequently she would report about her belief that she felt her acquaintances were not visiting her often , and hence , they could possibly be cheating her , but would not elaborate further . she firmly held on to these beliefs in spite of her husband telling her otherwise . for the last two weeks there had been a worsening of illness , characterized by crying spells , along with two suicidal attempts of high intentionality and lethality . after hospitalization , she was found to be tearful and withdrawn , reported of being fearful and expressed death wishes . an initial impression of psychosis , with a phenomenological inference of delusion of persecution , was made as per the longitudinal course of the illness from the available information , and she was started on tablet risperidone 2 mg . she continued to express fearfulness and did not show much improvement with risperidone . during the course of hospitalization , she was noticed to be repeatedly asking others to forgive her for a mistake she had committed . on further clarification , she elaborated that she had been getting repeated thoughts that she had done something wrong , which could be the reason for her feeling that her acquaintances were not visiting her often . suspicious behavior. she would get these repetitive , anxiety provoking thoughts so often that she started wondering whether she had actually done something wrong . the level of conviction regarding this thought was quite high and she did not feel that this thought was in anyway irrational . hence , she had started feeling , she would constantly ask reassurance from others , would check with her husband as to why a particular acquaintance had not visited her , and would keep repeatedly muttering to herself about this issue . the thoughts were very repetitive , stereotyped , and distressing , while they remained uncontrollable . as the frequency of these thoughts increased , she started feeling sad throughout the day , along with ideas of hopelessness , guilt , and suicidal ideas . subsequently , her diagnosis was revised to obsessive compulsive disorder ( ocd ) , with obsessions of intrusive thoughts / images and compulsion of reassurance seeking as denoted in the yale brown obsessive compulsive scale ( ybocs ) symptom checklist . the ybocs obsession score was 16 and compulsion score was 10 ( ybocs total score of 26 ) . she was started on fluoxetine 20 mg / day , which was increased to 40 mg per day in a week . after two months follow - up , she had significant improvement in her symptoms with the ybocs total score decreasing to 10 . this case demonstrates the importance of eliciting psychopathology in greater detail for specific diagnosis and treatment decisions , especially in the absence of a clear history . the presentation of the case with suspiciousness as the major symptom raised the possibility of psychosis . as there was no morbid illogical reasoning for the belief / thought , delusion was ruled out . additionally , the repetitive nature of the thought , which was stereotyped , causing severe distress , pointed toward a possibility of obsessions . this was supplemented by the compulsive nature of reassurance seeking , which provided some temporary relief to the anxiety caused by the thought . however , the difficulty here was the lack of insight of the patient into this thought phenomenon . even though traditionally viewed as a condition with , egodystonic thoughts recognized as illogical by patients , ocd could present with lack of insight . furthermore , depressive ruminations were usually associated with negative emotions regarding some past event , while in this case the event appeared like an intrusive meaningless thought when it originated . insight in ocd had therapeutic implications such as poorer response to medications and prognostic implications , wherein , schizophrenic spectrum symptoms could lead to worse prognosis . the continuity model between obsessions , overvalued ideas , and delusions seems to be more satisfactory , although it calls for a careful analysis of the phenomenon in clinical settings , for prudent treatment choices .

obsessive compulsive disorder ( ocd ) is commonly regarded as a disorder with good insight . however , it has now been recognized that insight varies in these patients . pathological beliefs seem to lie on a continuum of insight , with full insight at one end and delusion at the other . this can indeed pose a considerable challenge , especially in a scenario where the phenomenon is difficult to discern . we report a case of ocd , which was initially diagnosed as psychosis .

fear of hypoglycaemia and gain in body weight are barriers for initiation of insulin therapy . modern insulin analogues are a convenient new approach or tool to glycaemic control , associated with low number of hypoglycaemia and favourable weight change . a1chieve , a multinational , 24-week , non - interventional study , assessed the safety and effectiveness of insulin analogues in people with t2 dm ( n = 66,726 ) in routine clinical care . this short communication presents the results for patients enrolled in biphasic insulin aspart sub group from libya . please refer to editorial titled : the a1chieve study : mapping the ibn battuta trail . the study was started with an aim to collect data on all the insulin analogue viz insulin detemir , biphasic insulin aspart and insulin aspart . however , due to ongoing revolution , the complete data could not be collected . here the majority of patients ( 189 ) were started on or were switched to biphasic insulin aspart . other groups were insulin detemir ( n = 58 ) , basal + insulin aspart ( n = 44 ) and other insulin combinations ( n = 25 ) . being the biggest treatment group , this communication describes the results for patients treated with insulin therapy before and then switched to biphasic insulin aspart . overall demographic data 179 patients started on biphasic insulin aspart ogld , and all the patients were on insulin therapy prior to the study . after 24 weeks of switching to biphasic insulin aspart , hypoglycaemic events reduced from 7.2 events / patient - year to 3.7 events / patient - year [ tables 2 and 3 ] . all parameters of glycaemic control improved from baseline to study end [ table 4 ] . overall efficacy data 179 patients started on biphasic insulin aspart ogld , and all the patients were on insulin therapy prior to the study . after 24 weeks of switching to biphasic insulin aspart , hypoglycaemic events reduced from 7.2 events / patient - year to 3.7 events / patient - year [ tables 2 and 3 ] . all parameters of glycaemic control improved from baseline to study end [ table 4 ] . our study reports improved glycaemic control following 24 weeks of treatment with biphasic insulin aspart with or without ogld . hypoglycemic events decreased from baseline and sadrs did not occur in any of the study patients . though the findings are limited by number of patients , still the trend indicates that biphasic insulin aspart can be considered effective and possess a safe profile for treating type 2 diabetes in libya .

background : the a1chieve , a multicentric ( 28 countries ) , 24-week , non - interventional study evaluated the safety and effectiveness of insulin detemir , biphasic insulin aspart and insulin aspart in people with t2 dm ( n = 66,726 ) in routine clinical care across four continents.materials and methods : data was collected at baseline , at 12 weeks and at 24 weeks . this short communication presents the results for patients enrolled in biphasic insulin aspart sub group from libya.results:a total of 179 patients were enrolled in the biphasic insulin aspart subgroup . all the patients were prior insulin users . at baseline glycaemic control was poor ( mean hba1c : 9.3% ) . after 24 weeks of treatment there was an improvement in hba1c ( 0.9% ) . hypoglycaemic events reduced from 7.2 events / patient - year to 3.7 events / patient - year in 24 weeks . sadrs did not occur in any of the study patients.conclusion:starting or switching to biphasic insulin aspart was associated with improvement in glycaemic control with a low rate of hypoglycaemia .

a 28-year - old woman , with primary infertility of three years was referred for laparoscopic myomectomy with a history of severe dysmenorrhea and a diagnosis of posterolateral wall myoma . on pelvic examination , a palpable myoma was noted on the posterior uterine surface , and the uterus was felt to be approximately 10 gestational weeks in size . a well - circumscribed mass measuring 5.16 6.0 4.25 cms was seen in the posterolateral fundus and was found to be impinging on the endometrial stripe . the patient was given a choice between conventional laparoscopic myomectomy and the single incision approach . a 2 cm vertical incision was made at the base of the umbilicus and peritoneal access was gained . , norwalk , ct ) , [ figure 1 ] was inserted through the incision . the device , made from an elastic polymer , was slightly hourglass shaped and could be deployed through 2 cm fascial incision . it contained four openings : one for insufflation via a right - angled tube and three that could accommodate trocars 5 to 12 mm in size . the compressibility of the elastic polymer allowed for the access ports to expand and form fit the space in which they resided , and the ports also passed through the working channels . the sils tm port has the capacity of up to three laparoscopic instruments of 5 to 12 mm after careful survey of the abdomen and pelvis , dilute vasopressin , 20 u , in 100 ml of saline solution , was injected subserosally over the posterior myoma . once the correct plane was entered , the myoma was dissected out of the uterus using a 5 mm myoma cork screw and blunt scissors . using mostly blunt dissection , the enucleation of the myoma was done [ figure 2 and 3 ] . hemostasis was ensured and a piece of adhesion barrier ( gynecare interceed ; ethicon inc . , west somerville , new jersey ) was then cut in half , introduced into the abdomen through one of the 5 mm trocars , and placed over the hysterotomy incision . the myoma was then grasped with a 12 mm claw forceps and electric morcellation was done using rotocut g1 morcellator morcellation ( rotacut ) the advantages obtained by electronic morcellation over manual morcellation from umbilical incision site are : reduction in operative timedecreased risk of hernia formation due to absence of tearing or stretching of fascialower risk of injury to the surrounding tissues . reduction in operative time decreased risk of hernia formation due to absence of tearing or stretching of fascia lower risk of injury to the surrounding tissues . as laparoscopic myomectomies are performed routinely in our center , rotocut is preferred over gynecare morcellex for its cost effectiveness and speed . it is reusable and can be used indefinitely with periodic replacement of the cutting blade . the fascia was closed with a running 0 polyglactin 910 ( vicryl , somerville , nj ) suture . the skin was then approximated with a series of interrupted 3/0 monocryl sutures ( ethicon inc . ) . ten milliliters of 0.5% bupivacaine hydrochloride ( sensorcaine ) were injected into the incision site , and dermabond adhesive ( ethicon inc . ) was applied . the total procedure time ( time from first incision to end of procedure , d and c ) single - incision surgery has been reported to offer patients improved cosmetic outcomes as compared to multiport laparoscopic surgery , and possibly less postoperative pain , although these potential benefits have yet to be demonstrated in a well - designed prospective trial . a number of advantages have been proposed including cosmesis , less incisional pain , less blood loss , and the ability to convert to standard multiport laparoscopic surgery . an additional morcellation port is avoided as specimen retrieval / morcellation can be done through the umbilical incision . the primary limitations of sils are the restricted degrees of freedom of movement , lack of triangulation , the number of ports that that can be used , and the proximity of the instruments to each other during the operation all of which increase the complexity and technical challenges of the operation . many of these difficulties may be related to the technique of port placement and utilization during single incision laparoscopic surgery . a number of methods have been described for port access to perform sils , including multiple fascial punctures through one skin incision and use of novel port access devices . to further overcome the technical challenges of sils , different instruments that provide angulations and small profile trocars , endostitch , are being developed . the barbed suture greatly facilitates myometrial closure because there is no need to tie knots and there is no backsliding of the suture , which enables continuous wound closure with even distribution of tensile strength throughout the repair . these benefits of barbed suture are especially valuable in single - incision surgery , because intracorporeal knot tying can be more challenging here than in the multiport approach . currently , careful case selection is paramount , so that these procedures can be explored safely , with a low threshold to convert to standard laparoscopy , as indicated , for safety and quality of care . the use of novel port access devices , articulated instruments , and endostitch self - retaining sutures , makes the procedure easier , with a potential for saving time .

single port laparoscopic surgery ( spls ) , also called sils is the natural extension of multi - incisional laparoscopic surgery , in the quest for reduction of traumatic insult and residual scarring to the patient . today with the evolution of newer instruments , bidirectional self - retaining sutures , and surgical experience we are able to perform many surgeries in gynecology .

several cell lines were isolated during independent experiments from dissected annulus fibrosus ( af ) tissue of mature bovine intervertebral discs ( ivd ) via a reproducible non - enzyme driven protocol . the cell lines were frozen at low passage number and they recovered well after freeze - thawing ( see fig . preliminary characterization of the af cells was carried out with bovine specific rna probes derived from bovine genomic dna using plate rna in situ hybridisation ( pish ) for col1a1 and col2a1 expression , two structural proteins found in the mature ivd . less type - ii collagen fibers were described for the outer af in rabbit correlating with a common notion that type ii collagen is higher in the np than the af , . the dissected outer af of mature bovine ivds was the source for our af cell lines and we did not detect col2a1 expression by either rna in situ hybridization ( sish ) , , , on sections of the outer af tissue or by pish on the cells derived from the outer af , while col2a1 expression was very prominent in cells of the np as shown by sish on the same section ( fig . the discrepancy between our findings and that of increased col2a1 expression in the bovine af over the np reported by minoque et al . might reflect differences in defining the af . we see col1a1 expression in af and np cells by sish in vivo and by pish in vitro ( fig . skinned bovine tails were collected fresh from local abattoirs , remained chilled and were processed within 2 h. tail pieces were immersed in 10% povidone - iodine solution , rinsed with tap water , followed by immersion in 70% etoh prior to removing all fat and muscle tissue . ivds were dissected away from adjacent vertebrae endplates , briefly dipped in 70% etoh and rinsed with 1 pbs/10% gentamicin prior to separating the outer af from the remaining ivd tissue . outer af tissue was cut into smaller pieces using sterile procedures and placed in uncoated as well as 0.1% gelatin coated 35 mm culture dishes ( falcon ) . sterile filtered fbs - hi with 10% gentamicin and 5 g / ml amphotericin b ( all gibco ) was added prior to the incubation at 37c , 5% co2 and atmospheric o2 . after 24 h the fbs mix was diluted 1:1 with standard dmem based growth medium containing 1 dmem with 4.5 g / l glucose , 1 pyruvate , 1 glutamax , 1 nonessential amino acids , 10% v / v hi - fbs , 0.48% v / v gentamicin ( all gibco ) , 0.12 mm beta - mercapthoethanol ( sigma ) and additional 5 g / ml amphotericin b and the tissue . following 48 h of incubation cells had attached to the bottom of the wells and were expanded in fresh standard dmem based growth medium ( see above ) . cell lines derived in such manner from af tissue could be passaged with 0.05% trypsin / edta ( gibco ) at 1:10 dilutions for more than 10 passages without slowing down in population growth or dramatic changes in morphology ( fig . early and late passages were subjected to plate rna in situ hybridization ( pish ) for preliminary gene expression analysis ( fig . during embryogenesis , the af part of the ivd is believed to be of sclerotomal origin , . cultured cells derived from the outer af of mature bovine caudal ivds with our procedure were assayed for the expression of two major collagen genes col1a1 and col2a1 . the observed in vitro expression of these two genes mirrored the in vivo expression in cells of the mature the af : presence of col1a1 expression and absence of col2a1 expression in cells of the outer af ( fig .

the adult bovine ( bos taurus ) intervertebral disc is primarily comprised of two major tissue types : the outer annulus fibrosus ( af ) and the central nucleus pulposus ( np ) . we isolated several primary cell lineages of passage ( p ) 0 cells from the af tissue omitting typically used enzymatic tissue digestion protocols . the cells grow past p10 without signs of senescence in dmem + 10% fcs on 0.1% gelatin coated / uncoated surfaces of standard cell culture plates and survive freeze - thawing . preliminary analysis of the af derived cells for expression of the two structural genes col1a1 and col2a1 was performed by pish recapitulating the expression observed in vivo .

a 52-year - old thai woman presented with asymptomatic annular erythematous plaques on the forehead and both cheeks that persisted for 2 years . dermatologic examination showed few discrete annular erythematous plaques on her forehead and both cheeks , 15 cm in diameter . there were solar lentigines and telangiectasias on the malar area , nose , and forehead as shown in fig . 1 and fig the routine histopathology demonstrated nodular and interstitial inflammatory cell infiltrate of histiocytes intermingled with some lymphocytes in the dermis as shown in fig . elastic stain showed elastotic material phagoticized by multinucleated cells and marked decrease of elastic tissue in some foci of the affected dermis ( fig . 4 ) . according to the clinical and histopathological findings , the dermatologic diagnosis was actinic granuloma ( ag ) . complete blood count , liver enzyme , fasting blood glucose , and glycated hemoglobin were within normal range . the patient 's dermatologic condition was treated with prednisolone 15 mg / day for 6 weeks with a good response currently , she has been treated with hydroxychloroquine ( 200 mg / day ) , topical 0.1% mometasone furoate cream , broad spectrum sunscreen , and sun avoidance with partial improvement . it was also termed annular elastolytic giant cell granuloma , atypical necrobiosis lipoidica of the face and scalp , miescher 's granuloma of the face , and granuloma multiforme [ 1 , 2 , 3 , 4 ] . ultraviolet ( uv ) radiation , especially uva , and heat are recognized as causal factors , by changing the antigenicity of elastic fibers . the immune response mediated by helper t cells to degenerated elastic tissue the typical cutaneous lesion of ag is an initially smooth , elevated , nonscaly , erythematous papule which centrifugally extends to an annular plaque with central clearing . they are usually distributed on chronically sun - exposed areas such as the face , neck , upper back , forearms , and dorsum of the hands . apart from the skin , conjunctival involvement has been reported in a few cases [ 6 , 7 ] . there are some reports on the association between ag and internal diseases such as hematologic and solid malignancy , monoclonal gammopathy , temporal arteritis , erythema nodosum , and x - linked dominant protoporphyria [ 8 , 9 , 10 , 11 ] . diabetes mellitus has been found in about 3740% of patient with ag , and may be caused by injury of elastic fiber from hyperglycemic state . as for the renal condition , focal segmental glomerulosclerosis has been described in association with various granulomatous diseases ( e.g. , sarcoidosis , wegener 's granulomatosis , churg - strauss syndrome , and kimura 's disease ) [ 13 , 14 , 15 , 16 ] . these include granuloma annulare , erythema annulare centrifugum , annular lichen planus , secondary syphilis , necrobiosis lipoidica , tinea corporis , and tuberculoid leprosy . the best method to obtain a precise histopathology is an elliptical biopsy across the annular rim and stained with elastic van gieson to demonstrate the three zones of elastic tissue change . in the first zone , solar elastosis is identified in the surrounding unaffected skin . in the second zone , a granulomatous reaction consisting of histiocytes and foreign - body type multinucleated cells is seen , with engulfment of elastotic fibers , representing the annular rim . in the third zone , an absence of elastic tissue in the superficial dermis is found in the center of the plaque . due to aesthetic concern in our case topical corticosteroids , intralesional corticosteroids , systemic corticosteroids , topical calcineurin inhibitors , phototherapy and photochemotherapy ( narrowband uvb , puva , re - puva ) have been used with some benefit [ 18 , 19 , 20 ] . cyclosporine a , dapsone , pentoxifylline , isotretinoin , and acitretin have been reported to be effective in some cases [ 21 , 22 , 23 ] . there are a few case reports with positive results from antimalarial therapy ( chloroquine and hydroxychloroquine ) . to prevent the development of new lesions , patients should be instructed to avoid sun exposure and regularly use sunscreen . our patient had good response to a short course of low - dose prednisolone . however , rapid relapse occurred after the treatment was discontinued . currently , she has been treated with hydroxychloroquine ( 200 mg / day ) , topical 0.1% mometasone furoate cream , broad spectrum sunscreen , and sun avoidance with partial improvement . to the best of our knowledge , the correlation between ag and focal segmental glomerulosclerosis is still unidentified , further investigation is needed to establish the relationship between these two conditions . the authors declare no conflicts of interest . there was no funding for this work .

actinic granuloma is an uncommon granulomatous disease , characterized by annular erythematous plaque with central clearing predominately located on sun - damaged skin . the pathogenesis is not well understood , ultraviolet radiation is recognized as precipitating factor . we report a case of a 52-year - old woman who presented with asymptomatic annular erythematous plaques on the forehead and both cheeks persisting for 2 years . the clinical presentation and histopathologic findings support the diagnosis of actinic granuloma . during that period of time , she also developed focal segmental glomerulosclerosis . the association between actinic granuloma and focal segmental glomerulosclerosis needs to be clarified by further studies .

a 59-year - old asymptomatic diabetic male was referred to our retinal service with presumed diagnosis of asteroid hyalosis . the patient had undergone laser treatment and intravitreal preserved triamcinolone acetonide injection for clinically significant diabetic macular edema in the right eye 4 years previously . the right eye showed focal laser scars in the macula and several shiny refractile crystalline deposits [ fig . 1 ] . optical coherence tomography localized these deposits to the posterior hyaloid [ fig . 2 ] . these deposits were isofluorescent and not associated with specific angiographic abnormalities [ fig . 3 ] . the patient has been followed for two additional years without any change in the visual acuity or status of the macula . although a posterior vitreous separation has not developed , the crystals have been observed to migrate slowly in distribution . a fundus photograph of the right eye demonstrating small pre - retinal crystals concentrated in the macula , although peripheral crystals are noted ( arrow ) para - foveal optical coherence tomography scan of the right eye demonstrating triamcinolone crystal deposits on the posterior hyaloid surface ( arrow ) fluorescein angiogram of the right eye revealing several micro - aneurysms and focal laser scars , but no crystals , which are isofluorescent triamcinolone has been shown to aggregate into crystalline structures that may resist the washout process and accumulate over the macula . a recent study has identified intra - vitreal triamcinolone acetonide injections , both preserved and preservative - free , as a cause of crystalline retinal deposits . our case report also identifies triamcinolone crystal deposition , all on the posterior hyaloidal surface . with follow - up now extending greater than 6 years , no functional toxic effects have been noted in our patient . whether the crystals retain any biological activity is unclear . in our case and the previous series no adverse visual effect or we suggest the term drug - induced benign hyaloidopathy and recommend this to be included as a differential diagnosis of crystalline maculopathy and asteroid hyalosis .

we report a case of unusually long persistence of triamcinolone crystals after intra - vitreal injection . crystals were noted on fundus examination predominantly confined to the posterior pole . optical coherence tomography localized the crystals to the posterior hyaloidal surface . over 6 years of follow - up the patient has retained good visual acuity and no observable changes in the retina . as the condition clinically resembles both crystalline maculopathy and asteroid hyalosis , we suggest the term drug - induced benign crystalline hyaloidopathy.

the 2010 human influenza a ( h1n1 ) virus pandemic seriously affected many countries , including kuwait . in children , respiratory involvement usually occurs with h1n1 ; extra pulmonary problems are not common.1 liver involvement is rare and needs early identification and treatment . a 9-year - old child was admitted with intermittent low - grade fever , cough , vomiting , and abdominal pain lasting for one week . he received oral antibiotics ; the fever subsided initially but reappeared after a few days , along with jaundice . he was previously healthy , with no past history of liver disease . on examination , he was alert and oriented , his temperature was 39c , he was icteric , appeared toxic , was sweating , had a respiratory rate of 30 breaths / minute , and had congested tonsils ; a respiratory system exam showed prolonged expiration with expiratory rhonchi . there were no signs of meningeal irritation , and the rest of his physical examination was unremarkable . investigations revealed a hemoglobin ( hb ) count of 14.5 g / dl , a total leukocyte count of 3.37 10 , neutrophils 19% , lymphocytes 68% , a platelet count of 255 10 . urinalysis showed mild urobilinogen and ketones , but a urine culture was sterile after 48 hours of incubation . mmol / l , alanine amino - transferase was 1763 u / l , aspartate amino - transferase was 1871 units / l , alkaline phosphatase was 246 units / l , and gamma glutamyl transferase was 107 units / l . g / l , serum ammonia count 74 mmol / l , and serum lactate 2.06 mmol / l . the patient had normal serum amylase and lipase levels , a negative cold agglutinin test , a normal ultrasound of the abdomen , and a negative chest x - ray . nasal and throat swabs for h1n1 were positive by a reverse transcription polymerase chain reaction ( pcr ) test . a hepatitis a , b , and c serological screen was negative , and his serum acetaminophen level was normal . furthermore , an additional work - up to rule out other causes of fulminant liver failure was performed , including negative blood tests for herpes simplex virus pcr , adenovirus pcr , epstein his immunoglobulins ( igg , igm , and iga ) were within normal limits ; he also had a negative antinuclear antibody ( ana ) < 1:40 titer and a negative anti - smooth muscle antibody and anti - liver kidney microsomal antibody ( anti - lkm ) , ruling out the possibility of autoimmune hepatitis . finally , his serum amino acid and urine organic acids were unremarkable . he was treated according to centers for disease control and prevention ( cdc ) guidelines2,3 with tamiflu ( oseltamivir ; genentech , san francisco , ca ) for five days and other supportive measures , including fresh frozen plasma , iv - administered vitamin k , lactulose , and prophylactic intravenous antibiotics . severe infection is characterized by pneumonia , sepsis , septic shock , and multi - organ failure . extra - pulmonary involvement is rare in uncomplicated human infections.1 studies of mouse models suggest multiple organ localization , including the lungs , heart , thymus , liver , and spleen.4 snchez - torrent et al reported h1n1 encephalitis in a 3-month - old infant from spain.5 hepatic involvement is not frequent and accounts for less than 3% of all cases.1 carrillo - esper et al , in 2010 , reported two adult h1n1 patients with hepatic involvement.1 el - shabrawi et al , in 2011 , reported a 10-month - old child with acute myocarditis and fulminant hepatic failure associated with h1n1.6 the subject of the current case report had acute hepatic failure that presented as jaundice , elevated liver enzymes , and coagulopathy . most of the other causes of liver failure had been ruled out by relevant investigations . he responded well to antiviral and other supportive treatment , and showed full clinical and laboratory recovery . no viral replication is needed to produce hepatic damage , as there is evidence of hepatic oxidative stress and a decrease in antioxidant defenses even when the virus is isolated only from the lungs . this might be explained by the production of pro - inflammatory cytokines in the respiratory airway that leads to changes in hepatic metabolism and enzymatic activities.7,8 even though hepatic complications are rare in pediatric h1n1 cases , in reporting this case we would like to draw the attention of pediatric health care professionals to the importance of early recognition , focused investigations , diagnosis , and treatment of complicated human h1n1 infection .

liver involvement in pediatric influenza a ( h1n1 ) infection is rare . focused clinical evaluation and laboratory tests can rule out or identify hepatic complications early on . here we report on a 9-year - old boy treated by the gastroenterology , hepatology , and nutrition unit of al - adan hospital s pediatric department . the patient , who was infected with h1n1 during the 2010 pandemic , showed symptoms of associated acute hepatic failure , was managed conservatively , and recovered completely following treatment . the author would like to draw the attention of pediatricians to the hepatic aspect of human h1n1 infection in order for them to recognize it early and treat it in a timely manner .

it is characterized by acute and mostly intense abdominal pain accompanied by peritonism and potentially meteorism , nausea , vomiting and in severe and advanced cases shock . in most cases abdominal pathologies cause this acute condition including but not limited to appendicitis , cholecystitis , pancreatitis , mesenteric ischemia , ileus and perforations of hollow organs , . however , also extra - abdominal diseases can present with symptoms of an acute abdomen such as testicular torsion , myocardial infarction or diabetic ketoacidosis . due to the fact that an acute abdomen is always potentially life - threatening , prompt action is required to obtain the diagnosis and to immediately initiate adequate therapy . diagnostically , in addition to clinical examination and analysis of serum parameters , imaging such as sonography , computed tomography ( ct ) and magnetic resonance imaging ( mri ) are foremost in leading to the correct diagnosis . however , as mentioned above , the causes for acute abdominal symptoms are versatile and at times are found in medical areas other than gastroenterology itself . in this study we report on an interesting and unique case in which a psychiatric condition was the underlying disease leading to an acute abdominal picture which required immediate surgical intervention . a 28-year - old woman presented in our surgical emergency unit with a sudden onset of progressive abdominal discomfort and pain . routine laboratory analysis did not show any pathologies , including normal hemoglobin , leucocytes , c - reactive protein ( crp ) as well as serum electrolytes . because of the acute and dramatic clinical presentation we immediately performed a computed tomography of the abdomen . this revealed a massively distended stomach with a cranio - caudal extension of 35 cm reaching the lesser pelvis , however , without evidence for perforation ( figure 1 ( fig . a stomach tube was placed , which , however , did not drain any significant amount of stomach content . we performed a gastrotomy of the gastric antrum and recovered ( over the period of one hour ) a large amount of cementitiously clotted and undigested food scraps from the stomach . the stomach tube as well as the intrabdominal drainage could be removed within the first days after surgery . a gradual reintroduction of liquids was well tolerated . on the sixth postoperative day we performed a radiological imaging of the stomach using contrast medium which revealed a re - tonised stomach of a normal size without evidence for any stenosis ( figure 2 ( fig . subsequently , the patient was allowed to normal food intake , which was well tolerated as well . the postoperatively initiated evaluation of the patient by a psychiatrist revealed that she had been suffering from an eating disorder since her childhood . temporarily , her body mass index ( bmi ) had dropped to 11 kg / m . at consultation in our clinic however , the patient reported on daily binge eatings caused by conflicts at her workplace . this had also occurred on the day of hospital admission , however on this day , the routinely self - induced vomiting after the binge attack failed . in a good status of health here , we report on an unusual case in which a binge attack in a young woman suffering from an eating disorder caused a massive dilatation of her stomach . this dilation was not reversible either by self - induced vomiting or by drainage using a stomach tube . some other authors have reported on cases in which eating disorders such as anorexia nervosa or bulimia nervosa lead to acute gastric dilatation , , , . in line with the higher incidence of eating disorder in young females , mostly women in the age between 14 and 30 years were affected . in some cases , the so - called superior mesenteric artery syndrome has been suggested to cause or at least promote gastric dilatation in patients with eating disorders , . for this syndrome it has been proposed that malnutrition leads to the shrinkage of a fad pad localized between the aorta and outlet of the superior mesenteric artery . this , in turn , may cause compression of the duodenum thereby promoting gastric dilatation , in particular in cases when eating binges occur . in the reported case of this study , immediate imaging revealed the diagnosis and emergency explorative laparotomy and gastrotomy allowed for full recovery of the patient without complications or any residuals . this prompt and direct action including emergency laparotomy appears to be justified and essential , given the fact that some authors have reported severe complications such as gastric perforation and/or necrosis in patients with similar conditions , . furthermore , in some cases extended surgical approaches such as partial gastrectomy or even gastric resection were required . the fact that some patients did not recover and , unfortunately , passed away , confirms the severity of this condition as well as the importance of adequate diagnosis and immediate ( surgical ) therapy .

the clinical picture of an acute abdomen is frequently encountered in emergency medicine . in most cases abdominal pathologies underlie this condition , however , also extra - abdominal diseases may present or cause an acute abdomen . the fact that this condition is potentially life - threatening highlights the importance of instant action . here , we report on the case of a young woman that presented with an acute abdomen in our clinic . imaging revealed a massively distended stomach reaching the lesser pelvis . initially , the etiology for the gastric dilatation remained unsolved . on the same day we performed an explorative laparotomy in which massive amounts of clotted , undigested food was recovered via a gastrotomy . postoperatively , upon psychiatric consultation , an eating disorder with daily eating binges could be revealed as being the cause for the acute and dramatic gastric dilatation . the patient fully recovered from surgery and psychiatric co - treatment was initiated . this unique case report demonstrates how a psychiatric condition may lead to an acute abdomen , however , it also emphasizes the importance of prompt diagnosis and adequate therapy to avoid complications and allowing for full recovery .

peritonitis is one of the most common factors responsible for morbidity and mortality in patients on continuous ambulatory peritoneal dialysis ( capd ) . approximately 48% of peritonitis episodes have a fungal etiology , candida species accounting for 75% . the reported incidence of mortality in bacterial peritonitis is approximately 0.63% and that in fungal peritonitis is 1244% . so , it is imperative that fungal peritonitis is diagnosed rapidly and appropriate treatment given so that the survival rates are improved . paecilomyces species are saprophytic fungi and are uncommon pathogens that can produce serious infections in immunocompromised patients and occasionally in immunocompetent hosts . reddy et al . reported a case of fungal peritonitis caused by paecilomyces puntonii from india . we report a case of fungal peritonitis caused by paecilomyces varioti , which is not reported from india till date to the best of our knowledge . a 51-year - old man was on capd had 2 episodes of culture negatibe peritonitis over the period of 2 years . first episode 2 months after the initiation of capd was treated with empirical antibiotics ( vancomycin and amikacin ) by intraperitoneal route ; second episode 6 months prior to current admission and was treated with the same antibiotics . he again presented with complaints of diffuse abdominal pain and high grade fever with cloudy pd effluent . his pd effluent cell count was 3000 cells / mm with 80% polymorphs , gram stain and afb stain were negative , aerobic cultures did not grow any organism . fungal cultures by sabouraud dextrose agar medium had grown filamentous fungi after 48 hours and microscopically it showed chains of single celled phialoconidia produced in basipetal succession from a phialide . phialides are swollen at their bases , gradually tapering towards their apices and formed a brush - like penicillus confirming the diagnosis of p. varioti [ figure 1 ] . accordingly capd catheter was removed , he was initiated on hemodialysis mode amphotericin b was started at 1 mg / kg / day . he was treated for a period of 4 weeks with a cumulative dose of 1.5 g of amphotericin . capd catheter reinsertion was done successfully , he is currently on pd and is doing well . microscopic morphology of paecilomyces varioti showing chains of single celled phialoconidia produced in basipetal succession from a phialide . phialides are swollen at their bases , gradually tapering towards their apices and may form a brush - like penicillus in recent years , unusual and nonpathogenic fungi like paecilomyces have been increasingly reported as etiologic agents of fungal peritonitis . paecilomyces are common saprophytic fungi found in soil , silage , and water . they are not usually associated with human infection . however , some species such as p. varioti , p. marquandii and p. lilacinus are emerging as causative agents of hyalohyphomycosis in the immunocompromised host . p. varioti have been reported to cause pyelonephritis , endophthalmitis , hairy - cell leukemia , cerebrospinal shunt infection , and prosthetic valve endocarditis . the most important risk factors for fungal peritonitis are prolonged use of antibiotics and previous bacterial peritonitis episodes . a study by goldie et al . reported that 65% of fungal peritonitis patients had received broad - spectrum antibiotics within the preceding month , 74% within 3 months , and 87% within 6 months . our patient presented with fungal peritonitis 6 months after empirical antibiotic therapy for suspected bacterial peritonitis . several case series reported increased dialysate eosinophils . in other reported cases as well as in our patient , no eosinophilia was found in the dialysate . the conventional antifungal regimens include fluconazole , amphotericin b , and flucytosine alone or in combination , based on fungal sensitivities . p. varioti is usually very sensitive to amphotericin b. we used intravenous amphotericin b for our patient who responded dramatically with cumulative dose of 1.5 g. it can be easily treated with common antifungal agents like amphotericin b which can facilitate catheter reinsertion .

peritonitis is one of the most common and important complications in patients on continuous ambulatory peritoneal dialysis ( capd ) . fungal peritonitis isreported in 48% of peritonitis episodes . fungal peritonitis due to paecilomyces species is not common . we report a case of capd peritonitis due to p. varioti . we immediately removed the capd catheter and iv amphotericin was administered for 4 weeks along with temporary hemodialytic support followed by successful catheter reinsertion .

intravenous thrombolytic therapy when given within 4.5 h from symptom onset remains the mainstay of treatment for ischemic stroke patients . while intravenous tissue plasminogen activator ( iv tpa ) has been shown to be effective in improving outcome , the most feared complication is symptomatic intracerebral hemorrhage ( sich ) , which occurs in up to 7 percent of patients and significantly increases mortality and morbidity . standard management of post thrombolysis intracerebral hemorrhage includes replacement of coagulation factors with cryoprecipitate and platelets , as suggested by the american heart association , which is based on small case series and expert opinion and the efficacy of such treatment is unknown . thus there exists heterogeneity in clinical practice with respect to the strategies implemented in the management of sich . the lack of consensus on how to manage sich as well as continued poor outcomes despite treatment should create a driving force in the stroke community to investigate effectiveness and rapidity of other potential treatment options . we report a 68-year old right handed hispanic woman with a history of diabetes mellitus type ii , hypertension , hyperlipidemia , and two prior strokes with residual right hemiparesis , who presented with sudden onset vertigo , slurred speech , and blurred vision . the first stroke was a left putamen intracerebral hemorrhage with minimal residual right hemiparesis that occurred over 20 years prior to admission . the second stroke was a cryptogenic stroke that occurred around 5 years prior to admission for which she was outside the time window of thrombolytic therapy and was maintained on aspirin 81 mg daily . her initial exam showed dysarthria , left sided sensory loss , skew deviation with upgaze vertical nystagmus , mild right hemiparesis , and an nihss score of 4 . initial head computed tomography ( ct ) ( figure 1a ) showed evidence of prior strokes and no acute hemorrhage . since she had a new fixed and potentially disabling neurological deficit and she was within the 4.5 h window , decision was made to administer thrombolytic therapy . an hour after the iv tpa infusion was complete , she complained of an acute severe headache without a change in neurological exam and repeat ct head showed right temporal ich with a subdural component ( figure 1b ) . two hours after the completion of this treatment , she became somnolent and had new left hemiparesis . ct head was repeated that showed ich expansion and new intraventricular hemorrhage ( figure 1c ) . in the next 3 h head ct at that point showed further expansion of ich and intraventricular hemorrhage ( figure 1d ) . the decision was made to administer recombinant factor viia ( 50 mcg / kg ) and she was subsequently taken to the operating room with successful clot evacuation . postoperatively , her exam slowly improved and she was back to her baseline neurological exam on post - operative day 3 ( figure 1e ) . alteplase converts plasminogen to plasmin , which in turn converts fibrin into the fibrin split products causing thrombolysis . it also causes a reduction in fibrinogen the degree of which is associated with sich . using cryoprecipitate ( factor viii and fibrinogen ) , in post thrombolysis sich increases fibrinogen levels , enhances fibrin formation and may potentially stabilize the clot thus reducing the risk of further hemorrhage . however , a study from get with the guidelines data showed that around 40 percent of patients with post thrombolysis sich have continued bleeding despite cryoprecipitate treatment . the lack of efficacy may be due to delays in diagnosis and treatment , insufficient dosing of cryoprecipitate to replenish fibrinogen , or lack of augmentation with rapid acting factors that help convert fibrinogen into fibrin . to our knowledge , this is the first case reporting the use of recombinant factor viia along with cryoprecipitate and platelets in the treatment of symptomatic ich following intravenous thrombolysis . activated factor viia has been studied in spontaneous intracerebral hemorrhage and has been shown to reduce hematoma growth with no effect on mortality and morbidity however . activated factor viia , which has a relatively quick time of onset and is an extrinsic and intrinsic pathway activator , may further enhance clot stability and potentially reduce the risk of hematoma expansion , which is likely what happened with our patient . although recombinant factor viia carries a risk of thrombosis and is relatively expensive , its use in this patient population may potentially alter the course of the disease and improve outcome . randomized studies may be considered to compare the outcome of patients with post thrombolysis sich when rapid reversal agents such as recombinant factor viia or prothrombin complex concentrate are added to the standard treatment or given in isolation .

symptomatic intracerebral hemorrhage ( sich ) occurs in up to 7% of stroke patients treated with thrombolytic therapy . there are limited data on the effectiveness of the reversal agents used for intravenous tissue plasminogen activator related intracranial bleeds . we report a patient with sich following intravenous thrombolysis whose intracerebral hemorrhage continued to expand despite treatment with platelets and cryoprecipitate , needing recombinant factor viia use for stabilization before surgical evacuation . factor viia along with routine reversal agents following intravenous thrombolysis related sich may further enhance clot stability and reduce the risk of hematoma expansion . it could be a bridge to definitive surgical management in those patients .

the interpretation of a raised amylase in the acute surgical patient is crucial yet complicated as it is raised in a large number of conditions but with a low specificity . this report argues that a transient rise in serum amylase not caused by sepsis or pancreatitis can be caused by a splenic infarction . therefore any cause of a non - diagnostic amylase rise must not be over- looked as it may be of diagnostic value in a life threatening surgical condition . a 49-year - old previously well male , presented with a 24 hour history of vomiting , abdominal pain and fever . his abdominal pain was of rapid onset and localised to the epigastrium and left upper quadrant . there was no history of trauma but he did admit to a recent history of alcohol excess . clinical examination revealed diffuse upper abdominal tenderness , a mild tachycardia and a low grade pyrexia . blood work up revealed an amylase of 270 u / l and crp of 125mg / l . ct showing evidence of splenic infarction secondary to infective endocarditis a provisional diagnosis of alcohol - induced pancreatitis was made . he received standard management as mild pancreatitis ( glasgow score 0 ) with ward level care . an ultrasound showed no abnormality . due to diagnostic uncertainty and failure of symptoms and signs to rapidly resolve , subsequent echocardiogram for a source of emboli showed multiple valvular vegetations , aortic regurgitation and a 16 mm pericardial effusion . treatment with intravenous flucloxacillin and gentamicin was undertaken and transfer to the regional cardio - thoracic unit was arranged . there he underwent urgent aortic valve replacement with a tissue valve following which he made an uncomplicated recovery . these can be broadly grouped into 3 subdivisions : pancreatic ( eg . pancreatitis , pancreatic tumour or trauma)extra - pancreatic intra - abdominal disease ( eg . cholecystitis , perforated duodenal ulcer , bowel ischaemia)extra - abdominal processes ( eg . macro - amylasaemia ) pancreatic ( eg . pancreatitis , pancreatic tumour or trauma ) extra - pancreatic intra - abdominal disease ( eg . cholecystitis , perforated duodenal ulcer , bowel ischaemia ) extra - abdominal processes ( eg . macro - amylasaemia ) interpretation of a raised amylase in the surgical patient is crucial , potentially avoiding dangerous misdiagnosis . many conditions cause hyperamylasaemia making the specificity of elevated serum amylase level less than 70% . its low specificity drops further after the first 24 hours of hospital admission ( 1,2 ) . elevated serum amylase activity in pancreatitis is attributed to pancreatic auto - digestion , but its pathophysiology is unknown in many other causes . endocarditis is a well known cause of splenic thrombosis and consequent infarction and first described in germany in 1869 ( 3 ) . there have been reports of raised amylase in non - pancreatic sepsis , some showing that this could be used as a prognostic marker in the septic patient ( 4 ) . however , in this patient the transient amylase rise dropped despite worsening sepsis , suggesting an alternative cause for the disturbance . recent studies by antopolsky et al examined clinical presentations in 49 episodes of acute splenic infarction . the most common symptom was abdominal or left flank pain ( 80% of episodes ) with the most common sign being upper left quadrant tenderness ( 35% of episodes ) ( 5 ) . clinicians should be aware of splenic infarction as a clinical entity , and consider it in cases of upper abdominal pain , particularly if there is a non - diagnostic amylase rise .

we present what maybe the only case of splenic infarction causing hyperamylasaemia in a patient with bacterial endocarditis . a 49-year - old gentleman presented a 24 hour history of vomiting , abdominal pain and fever . clinical examination showed diffuse upper abdominal tenderness , a mild tachycardia and a low grade pyrexia . blood investigations showed a hyperamylasaemia . his failure to improve on treatment for a provisional diagnosis of alcohol induced pancreatitis lead to a ct abdomen , which showed a splenic infarct and an echo showing aortic valve vegetation 's as a source of emboli . he underwent urgent aortic valve replacement with a tissue valve following which he made an uncomplicated recovery .

the utah lions eye bank has harvested 159 eyes from strangulations or hangings since 2003 . of these , 18 eyes ( 11% ) were not suitable for surgery , with 13 eyes being excluded during the screening process . the reasons for exclusion included hepatitis b positivity , human t lymphotrophic virus positivity , high risk of creutzfeldt - jakob disease , low endothelial cell count , or time spent incarcerated . the final two eyes belonged to a 25-year - old male who was found hanging and unresponsive . his past medical history was significant for depression , previous suicide attempts , peritonitis , and an appendectomy , with an unremarkable past ocular history . following dissection of the corneoscleral buttons by the utah lions eye bank , total detachment of descemet s membrane was seen in both corneas ( figure 1 ) . the corneas were deemed unsuitable for transplant surgery , and the tissue was sent to another eye bank for use in keratolimbal allografts . the donor s corneoscleral buttons were retrieved by a skilled tissue recovery specialist with many years of experience . iatrogenic trauma during tissue retrieval can not be ruled out since corneas of intact globes are not routinely examined prior to removal . however , this is unlikely to cause such dramatic findings of complete descemet s membrane detachments bilaterally . to our knowledge , this is the first reported case of extensive descemet s membrane detachment following a hanging . dyer observed the hanging of a prisoner and reported a post mortem fracture through the crystalline lens and anterior lens capsule in the prisoner s right eye . he reproduced these findings by hanging three dogs , with two of six eyes showing similar damage following death.1 in 1869 he saw five more hangings , and found crystalline lens and anterior capsule fractures in three of 10 eyes.2 we speculate that a dramatic rise in intraocular pressure during hanging may stretch the cornea and cause rupture of descemet s membrane . tears in descemet s membrane may occur in congenital glaucoma when the intraocular pressure increases , causing an elongation in axial length and subsequent stretching of the cornea.3 recent research has examined how forces applied to the head influence intraocular pressure . bakaran et al measured intraocular pressure in yoga practitioners during sirsasana ( the headstand posture ) and found mean intraocular pressure increased two - fold in patients immediately after assuming this position.4 aykan et al found that the intraocular pressure increases significantly during valsalva movements.5 hanging may cause increased intraocular pressure secondary to increased venous pressure in the brain . he et al examined brain lesions caused by hanging via magnetic resonance imaging and found a venous distribution of brain damage.6 we suggest that further research on the effect of increased venous pressure and iop on corneal tissue is needed . furthermore , we would be interested in learning if other eye banks have observed similar corneal tissue changes following strangulation or hanging .

the eyes of a 25-year - old male were collected by the utah lions eye bank after his suicide by hanging . following dissection of the corneoscleral buttons from intact globes , bilateral detached descemet s membranes with subsequent scrolling in the periphery were observed . we believe these findings were caused by a large increase in intraocular pressure secondary to the hanging . lens and anterior capsule fractures after hanging have been reported , but corneal damage has never been discussed . we invite transplant surgeons and eye bank recovery specialists to share their experience of similar corneal changes in donated eyes following strangulation or hanging .

coronary artery spontaneous dissection is a rare and uncommon cause of sudden cardiac death and acute coronary syndrome . we report a case of a 31-year - old male admitted to our hospital with an acute coronary syndrome . a dissection of the left main coronary artery was diagnosed with a multidetector computed tomography ( ct ) scan and it was decided to perform an emergency coronary artery bypass grafting . a 31-year - old male , airline pilot , with no clinical history or cardiovascular risk factors , was admitted to the emergency room with 12 h history of retrosternal chest pain radiating to the jaw and upper extremities , during a trans - atlantic flight , which did not improve with analgesia . because of persistent pain , he came to our hospital . upon arrival to the emergency room , his vital signs were stable and he had a normal physical examination but complained of precordial pain . the cardiac enzymes were elevated and the ekg detected s - t changes consistent with mi ( fig . 1 ) . a multidetector ct coronary angiography was performed showing left main trunk dissection with 50% stenosis ( fig . figure 2:multidetector coronary angio - tomography reveals dissection at the ostium of the left main coronary artery . multidetector coronary angio - tomography reveals dissection at the ostium of the left main coronary artery . the patient underwent emergent coronary revascularization with saphenous vein grafts to the first obtuse marginal and the left anterior descending artery ( lad ) . control ct coronary angiography was performed 2 months later revealing patent aorto - coronary grafts ( fig . figure 3:ct coronary angiography reconstruction showing patent grafts to the lad and obtuse marginal arteries . in 1931 harold pretty described the first case of spontaneous dissection of a right coronary artery during the autopsy of a woman who presented with precordial chest pain . this condition is a rare cause of ischemic heart disease and affects mainly young healthy women . in 1987 , 85 cases were reported in the literature , currently more than 300 cases have been published [ 2 , 3 ] . before the coronariography era , these cases were reported during autopsies of patients having sudden cardiac deaths and its incidence may have been under - estimated . with the advent of these studies , the incidence has been reported to range between 0.07 and 1.1% . dissection of the lad artery is most common in women , whereas in men it is the right coronary artery [ 2 , 4 ] . the left main coronary artery involvement , like in our case , is rare , occurring in up to 12% of the cases , but is the most severe injury and presents as mi . hemodynamic and hormonal factors determine morphological changes in the arterial wall , and these may contribute to spontaneous dissection of the coronary arteries , with a peak incidence during the second week after birth [ 3 , 4 ] . to date , the pathophysiology is unclear [ 24 ] . in a subgroup of patients it is not possible to identify a specific condition causing spontaneous coronary dissection and is therefore classified as idiopathic . in patients with connective tissue disorders such as marfan s and ehlers danlos syndromes , predisposing to the dissection arises from medial degeneration of the coronary arteries . certain vasculitis , including systemic lupus erythematosus and polyarteritis nodosa , has been associated with the occurrence of coronary artery dissection . other factors that can cause vascular spasm and coronary dissection include intense exercise , sneezing and prolonged cocaine abuse [ 2 , 3 ] . it should show the presence of a double radiopaque lumen separated by a radiolucent intimal flap or a slow clearance of contrast from the false lumen . an intimal tear is present in only a minority of cases and a medial hematoma may not be recognized on coronary angiography as the medial hemorrhage may cause luminal narrowing or occlusion by pushing the inner media against the opposing wall . several cases in the literature have shown that ct coronary angiography provides additional information over invasive coronary angiography , with an accurate demonstration of the intimal flap and extent of the intramural hematoma . this study constitutes an emerging noninvasive alternative for diagnosis and also the follow - up of coronary artery spontaneous dissection . there are no specific guidelines for the management of spontaneous dissections of the coronary arteries [ 3 , 5 ] . coronary artery bypass surgery is indicated in patients with multivessel dissection , failed angioplasty and dissection of the trunk of the left main coronary artery [ 35 ] . although spontaneous dissection of a coronary artery is an uncommon condition , treatment should be based on the clinical status and imaging studies , including conservative and surgical means depending on the clinical presentation , location and characteristics of the dissection , we elected to proceed with surgery based on the ct findings .

spontaneous dissection of the coronary arteries is a rare disease with a wide range of clinical presentations ranging from angina to myocardial infarction ( mi ) ; its pathophysiology has not yet been fully established . in this paper , we present the case of a 31-year - old male with an acute coronary syndrome . the initial results of the electrocardiogram and cardiac enzymes were consistent with mi . however , a coronary angio - tomography revealed a dissection of the left main coronary artery and the patient underwent emergent surgery with coronary artery bypass grafting . the treatment of spontaneous dissection of the coronary arteries depends on the anatomical location and the patient 's clinical presentation . coronary revascularization is associated with good results .

subtilisin solutions were prepared by mixing and vortexing of 1.25 , 5 , 8 , 12.5 , 15 , 17.5 , 20 , and 25 l of subtilisin stock solution ( sigma aldrich , catalogue number p4860 ; lot 056k1213 ) per ml of 100 mm phosphate buffer ( ph = 8 , filtered with a 0.25 m anotop filter ) , which yielded enzyme solutions at concentrations of 0.05 , 0.2 , 0.3 , 0.5 , 0.6 , 0.7 , 0.8 , and 1 mg ml . these concentrations correspond to 1.5 , 6 , 9.6 , 15 , 18 , 24 , and 30 u ml , expressed in activity units used in our previous report . saxs measurements were carried out at beamline 7t - mpw - saxs at bessy ii synchrotron source in berlin , germany , using an x - ray energy of 9650 ev . enzyme solutions at various concentrations as well as pure buffer ( as background ) were injected in glass capillaries and placed in a capillary holder . the capillary holder was then mounted on the sample cell chamber , evacuated , and heated to 25 or 55 c . the individual samples were then exposed to the x - ray beam to record scattering intensity patterns by using a gas detector placed in the vacuum chamber . the integration and processing of the scattering data was carried out by using the software provided at the beamline . static and dynamic light scattering measurements were carried out using the 3ddls instrument ( ls instruments , fribourg , switzerland ) using vertically polarized he ne laser light ( 25 mw with a wavelength of 632.8 nm ) with an avalanche photodiode detector at angles between 15 and 135 at 55 c . the background scattering intensities ( from pure buffer ) were subtracted from the scattering intensities of the enzyme solutions . at each scattering angle , dynamic light scattering measurements were also performed by recording the intensity autocorrelation function g2( ) as a function of lag time . the autocorrelation functions were analyzed by means of the cumulant method in order to determine the initial decay rate and the corresponding average apparent diffusion coefficient dapp . the decay of the normalized autocorrelation function was modeled as g1( ) = ( g2( ) 1 ) = exp( ) , where = dappq is the initial decay rate , q = ( 4n/ ) sin(/2 ) is the scattering vector magnitude , n is the refractive index of the solvent , and is the wavelength of the laser . einstein equation , rh = kbt/6dapp , where kb is the boltzmann constant , t is the absolute temperature , and is the solvent viscosity at the given temperature . the scattering intensity patterns from static light and x - ray scattering experiments can be described as i(q ) kp(q)s(q ) , where k is an instrument- and sample - dependent constant , p(q ) is the form factor , which depends on the size and shape of the primary particles , and s(q ) is the structure factor giving information about the spatial arrangement of the primary particles at length scales larger than that of the primary particles ( radius of rp ) . please note that q = ( 4/ ) sin(/2 ) for saxs measurements . in the limit of qrg < 1 , the mean radius of gyration rg of randomly distributed ( e.g. , freely diffusing ) primary particles or clusters can be determined from the measured scattered intensity i(q ) by using the guinier analysis . in the limit of 1/rg q 1/rp , where rg is the mean radius of gyration of a sufficiently large cluster composed of primary particles with radius rp , the structure factor for fractal clusters with fractal dimension df scales with q through a power law relation as i(q ) s(q ) q.

the structural characterization of subtilisin mesoscale clusters , which were previously shown to induce supramolecular order in biocatalytic self - assembly of fmoc dipeptides , was carried out by synchrotron small - angle x - ray , dynamic , and static light scattering measurements . subtilisin molecules self - assemble to form supramolecular structures in phosphate buffer solutions . structural arrangement of subtilisin clusters at 55 c was found to vary systematically with increasing enzyme concentration . static light scattering measurements showed the cluster structure to be consistent with a fractal - like arrangement , with fractal dimension varying from 1.8 to 2.6 with increasing concentration for low to moderate enzyme concentrations . this was followed by a structural transition around the enzyme concentration of 0.5 mg ml1 to more compact structures with significantly slower relaxation dynamics , as evidenced by dynamic light scattering measurements . these concentration - dependent supramolecular enzyme clusters provide tunable templates for biocatalytic self - assembly .

mucus extravasation cysts or mucoceles are an extremely common lesion of the minor salivary glands . we report upon an unusual case of a submandibular gland mucocele presenting as a lateral neck swelling . the term plunging or cervical ranula refers to mucoceles that extend below the mylohyoid muscle , beyond the sublingual space and invariably are associated with the sublingual salivary gland . differentiation from submandibular gland mucoceles is potentially difficult , though plunging ranula is usually characterised by a so called tail sign on imaging . in our case a 54 year old woman presented with a large asymptomatic swelling of the right lateral neck . the swelling had been present for three months but had attained large dimension and was still felt to be increasing in size . she denied any history of trauma , surgery , infection , dysphagia , voice change or weight loss . palpation of the neck mass revealed a soft , fluctuant , non - tender swelling approximately 6 cm in diameter arising superficially within the right submandibular region . a magnetic resonance imaging ( mri ) scan revealed a large cystic structure in the right lateral neck involving the submandibular space ( fig 1 ) . the sample proved inadequate for cytological assessment , however was found to have a raised amylase and protein content suggesting salivary origin . mri scan showing cystic lesion within lateral neck under general anaesthesia the cystic mass was meticulously excised intact via a low submandibular incision . it was found to be in continuity with the ipsilateral submandibular gland ( fig 2 ) . histolopathology of the specimen confirmed a fibrous - walled retention cyst associated with a submandibular gland showing chronic obstructive changes . the patient made an uneventful post - operative recovery with no evidence of recurrence at six months . the presentation of a submandibular gland mucocele is uncommon with only a few reported cases in the literature ( 1 - 3 ) . it has been postulated that this rarity might be due to the anatomical location of the gland and the protective nature of the mandible1 . furthermore the physiology of the gland is such that unlike the sublingual gland , which exhibits continuous secretion , the submandibular gland secretes saliva only following stimulation ( 4 ) . nevertheless , despite the true aetiology being unknown it is thought likely that trauma , obstruction and congenital anomalies are most likely causes ( 5 ) . the differential diagnosis of a submandibular gland mucocele should include the numerous inflammatory , infective , developmental and neoplastic processes affecting the lateral neck . in particular , a solitary cystic mass should be thoroughly investigated to exclude the possibility of cystic degeneration of a lymph node secondary to metastatic squamous cell carcinoma . fine needle aspiration biopsy for cytological and biochemical assessment can confirm the nature of the cyst and is usually found to have a high amylase and protein content ( 6 ) . the management of a submandibular gland mucocele is potentially controversial and includes a number of treatment modalities that have been advocated in the management of the cervical or plunging ranula . injection of scelorosing agents have been used to induce inflammation and fibrosis from within the lumen of the cyst thus sealing further extravasation . aspiration is a simple technique but yields a high recurrence rate and as such its use has been largely restricted to the conservative management in the paediatric population . marsupialisation has gained popularity not least because of its relative simplicity , but also because it involves only limited dissection and therefore a reduced risk of damage to adjacent structures . recurrence however is unpredictable leading some authors to advocate a modified technique incorporating packing the cyst cavity ( 2 ) . contemporary opinion suggests that because mucoceles are pseudocysts there is no imperative to remove the cyst lining . appropriate management should focus on identification and elimination of the salivary gland responsible for the source of mucus secretion ( 1 ) . removal of the stimulus for secretion should cause involution of the cystic cavity , which seals itself , naturally ( 7 ) . we would advocate that in cases of a submandibular gland mucocele the lesion should be excised via a cervical approach in conjunction with the submandibular gland . if any doubt exists as to the close involvement of the sublingual gland then it would be advisable to remove this gland as well .

mucus extravasation cysts or mucoceles are an extremely rare occurrence in the major salivary glands . we report upon an unusual case of a submandibular gland mucocele presenting as a neck lump . it should therefore be considered in the differential diagnosis of swellings in the lateral neck . diagnosis and management are complicated by their similarity to the plunging or cervical ranula and differentiation may be potentially difficult . detailed imaging often reveals the plunging ranula as being characterised by a so called tail sign . in our case this sign was absent and subsequent excision confirmed origin from the submandibular gland . we discuss potential treatment modalities and propose a rationale for definitive management .

' mesotherapy ' is a non - surgical , minimally invasive method of drug delivery that consists of multiple intradermal or subcutaneous injections of a mixture of compounds ' melange ' in minute doses . it has gained disrepute because of inadequate scientific evidence to prove its efficacy . in spite of this , many new substances such as platelet - rich plasma ( prp ) , peptides , growth factors and collagen are finding their way to enter the field of mesotherapy . since small amounts of material have to be injected by multiple pricks rapidly over a large area , mesogun is used . mesogun is a motor - driven equipment that can be used to rapidly deliver the chemicals into the skin and subcutis . however , most mesoguns are expensive and tend to waste material due to dripping from the needle postulated to be due to rapid withdrawal [ figure 1 ] . while this may not be significant in financial terms when inexpensive substances are injected , products such as prp or meso solutions for injection which are either expensive or too precious should not be wasted . the cause for dripping is possibly due to lack of minimal recoil , following the injection by the motor - driven plunger . on searching the web , we found a syringe with a spring attached to the piston which would provide the recoil needed to prevent dripping . since these syringes are not freely available , we modified the available disposable syringe to rapidly deliver small quantity of material into the skin , similar to a mesogun . a 5 ml syringe or an insulin syringe is taken and the piston is removed from the body and a spring which is sterilised using ethylene oxide is inserted onto the piston which is inserted back into the body of the syringe [ figure 2 ] . the material to be injected is drawn into the syringe and the regular needle is replaced with the meso needle . the specifics of the spring provided for the purpose of duplication and standardisation used are as follows : wastage of injectable material from mesogun spring - loaded 5 cc syringes length 37 mm , thickness 1.21 mm , pitch 4 mm , outer diameter 15 mm , inner diameter 12.6 mm , spring constant ( k ) = 1300 n / m . length 71.30 mm , thickness 0.75 mm , spring outer diameter 5.7 mm , spring inner diameter 4.58 mm , spring constant ( k ) = 890 n / m . spring constant ( k ) is calculated as follows : the spring constant of a spring represents the force required to deform it by a certain amount . as most springs obey hooke 's law , the spring constant k is treated as a constant characteristic operational parameter for the spring . in the case of helical coiled springs , it represents the force required to extend ( or compress ) the spring by a certain length . experimentally , it is measured by fixing it at one end and applying a force ' f ' on the other end , along its axis , causing it to extend by a length x. the spring constant is then calculated by the formula : f is expressed in newton and x is in metres . small quantities of material can be injected by first piercing the needle into the tissue , pressing the piston using the thumb and removing the thumb which results in the recoil of the piston ; the syringe is withdrawn from the skin , following which the next site is injected [ video 1 ] . before disposal of the syringe , the spring can be removed and retained for reuse after sterilisation . the approximate cost of the spring - loaded syringe would be 30 inr for the 5 cc syringe and 26 inr for the 1 cc syringe . thus the modified spring loaded syringe can be an efficient , cost effective method of drug delivery for mesotherapy and intralesional injections . length 37 mm , thickness 1.21 mm , pitch 4 mm , outer diameter 15 mm , inner diameter 12.6 mm , spring constant ( k ) = 1300 n / m . length 71.30 mm , thickness 0.75 mm , spring outer diameter 5.7 mm , spring inner diameter 4.58 mm , spring constant ( k ) = 890 n / m . spring constant ( k ) is calculated as follows : the spring constant of a spring represents the force required to deform it by a certain amount . as most springs obey hooke 's law , the spring constant k is treated as a constant characteristic operational parameter for the spring . in the case of helical coiled springs , it represents the force required to extend ( or compress ) the spring by a certain length . experimentally , it is measured by fixing it at one end and applying a force ' f ' on the other end , along its axis , causing it to extend by a length x. the spring constant is then calculated by the formula : f is expressed in newton and x is in metres . small quantities of material can be injected by first piercing the needle into the tissue , pressing the piston using the thumb and removing the thumb which results in the recoil of the piston ; the syringe is withdrawn from the skin , following which the next site is injected [ video 1 ] . before disposal of the syringe , the spring can be removed and retained for reuse after sterilisation . the approximate cost of the spring - loaded syringe would be 30 inr for the 5 cc syringe and 26 inr for the 1 cc syringe . thus the modified spring loaded syringe can be an efficient , cost effective method of drug delivery for mesotherapy and intralesional injections .

mesotherapy refers to multiple injections of small quantity of the drug over a large area . the mesoguns available are expensive and the motor - driven models tends to waste the expensive material to be injected since the plunger stops after injecting without recoil . we searched for a less expensive device which would inject like the mesogun and still not waste the solution . on searching the web , we identified a spring - loaded syringe . we describe the assembly and use of this inexpensive syringe for delivering multiple injections with minimal wastage .

heidelberger and his postdoctoral fellow forrest kendall who had been rendered one handed ( but , by all accounts , no less dextrous ) by a farm threshing machine took advantage of purified bacterial polysaccharides ( isolated during their earlier studies ) for their quantitative experiments . they incubated varying proportions of purified antigen and antibody and determined the nitrogen content ( i.e. , antibody content ) of the resulting precipitate . the data derived from these assays showed that the precipitin reaction could be expressed based on simple equations derived from the laws of mass action and that antibodies and antigens were multivalent . when they repeated the experiment with whole serum , their calculations held up , thus assuring them that their equations were not exclusively applicable to purified solutions ( 1,2 ) . this was all very well for antibodies to polysaccharides , noted heidelberger in a 1979 article , but what about those elicited by the vast numbers of protein antigens ? ( 3 ) . as n had not yet been discovered , heidelberger and kendall instead used a colorful trick to distinguish antigen - derived nitrogen from antibody - derived nitrogen . hen egg albumin against which they raised specific antibodies in rabbits ( 4 ) . the amount of antigen - derived nitrogen in the redissolved precipitate could then be determined by comparing its color ( by eye , as colorimeters did not yet exist ) with solutions containing known concentrations of the dye ; the remainder of the total nitrogen content was attributed to the antibody ( 5 ) . heidelberger later took on his first graduate student , elvin kabat . together , they helped settle a long - standing debate concerning whether serum precipitins and agglutinins ( antibodies that agglutinate bacteria ) were the same or different . at the time , many people thought that these distinct functional properties of antibacterial antiserum could be ascribed to separate entities . returning to the pneumococcal bacteria , the duo showed that precipitins and agglutinins were present in identical amounts in antipneumococcal serum . reduction of one activity by adsorbing the serum with a bacterial or polysaccharide solution resulted in an equivalent reduction in the other activity , suggesting that the two functions were properties of the same antibody molecules ( 6 ) . kabat went on to show that antibodies in serum came in two sizes large and small ( now known as igm and igg)based on their mass and sedimentation rate ( 7 ) . heidelberger 's work , said former colleague herman eisner ( massachusetts institute of technology ) in a 2001 article , changed the concept of the antibody from an essentially ill - defined set of serum activities to a protein molecule , measurable in conventional chemical units whose recognition of antigens could be analyzed in molecular terms heidelberger , who was also a talented musician and linguist , worked in the lab until his death at age 103 . at his 100th birthday party , he was reportedly asked how many papers he had published in his lifetime . three hundred and four , he answered , adding slyly , so far

having defined the protein nature of antibodies under the tutelage of oswald avery , michael heidelberger was the first to apply mathematics to the reaction of antibodies and their antigens ( the precipitin reaction ) . heidelberger 's calculations launched decades of research that helped reveal the specificity , function , and origin of antibodies .

it is the 10th anniversary of the mailing of the anthrax spores , a tragic event that resulted in five deaths , seventeen other known infections , and untoward fear among the population . the cleanup is estimated to have cost $ 1 billion . what have we learned and accomplished in the interim of course , one can not consider the anthrax mailings in a vacuum , coming on the heels of the september 11 attacks on the world trade center , the pentagon , and united airlines flight 93 . the reaction from congress was swift : the united states patriot act was signed into law in october 2001 , and the public health security and bioterrorism preparedness response act followed in june 2002 . together , these pieces of legislation put restrictions and limits on who could access and possess select agents , those pathogens deemed by the cdc and usda to be the most dangerous . the government solicited studies from the national academies on various topics relating to science and security , other federal advisory panels were formed , and the government itself has addressed bioterrorism at various levels . numerous nongovernmental organizations throughout the world , including the american association for the advancement of science ( aaas ) , have also engaged scientists , security experts , ethicists , and others in an ongoing , fruitful discussion . while the specific charge to each of these groups differed , they all have addressed a common question : how do we ensure a vibrant research enterprise without compromising national security ? this question is of central importance given that the 21st century promises to be the biological century , which will likely spawn many new life science - based technologies and industries that will improve our lives . the answer to this question is not straightforward , mainly because of the nature of life science research . the risks that present themselves as a result of the acquisition of new knowledge are difficult to predict or quantify , while the potential benefits are more concrete . therefore , determining whether certain types of research should be prohibited or regulated is difficult . the consensus reached by both the scientific and national security communities is that research should proceed , with all involved being vigilant for the potential of misuse . the national science advisory board for biosecurity , which has been charged since 2005 with providing recommendations to the u.s . government on the oversight and conduct of dual use research , has developed a series of thoughtful reports that suggest a balanced approach to the topic ( see http://www.biosecurityboard.gov ) . perhaps the most important contribution of this body was to acknowledge that most if not all biological research had dual use capabilities while creating a special category known as dual use research of concern ( durc ) and the tools to identify it . this was important because it had the effect of walling off the small part of the scientific effort that was most relevant to the threat of bioterrorism and thus leaving the vast majority of biological research undisturbed and unregulated . another valid concern is whether certain individuals should not be allowed to access select agents . while some groups have now been excluded categorically by the above - mentioned statutes , vigorous discussions concerning personnel reliability in a broader sense insider threat was amplified by the identification of bruce ivins , a scientist working in a u.s . the emerging consensus is that ongoing attention to lab workers trustworthiness , behavior , and attitude is the best means to reduce the risk that someone might deliberately cause harm . what has the overall effect of these activities been on the life science research enterprise ? the infusion of extra research dollars has been welcome , especially at a time when federal funding of biology research overall has suffered . increased attention to the possible risk of misuse however , a small but significant number of investigators have chosen to discontinue working in the field due to the added regulatory burden . it is difficult to assess how many others are not entering into this area , but anecdotal evidence suggests this is occurring more than one would like . perhaps of greater concern is that important research , such as the development of new vaccines against anthrax , has been slowed by the need to work within the new regulatory and statutory framework . compliance with select agent rules has significantly increased the cost of research on certain pathogens ( 1 ) . the enactment of the select agent rules led to the destruction of several microbial collections ( 2 ) and is almost certainly interfering with the establishment of new collections or saving of new clinical isolates , given the enormous work involved in having such isolates transferred to secure facilities . furthermore , the focus on containing microbial threats by generating lists of organisms for special consideration may have created a false sense of security while greatly increasing the regulatory burden ( 3 ) . we are very fortunate that 10 years have passed with no additional bioterrorism events . this is a testament to the effort of scientists behaving responsibly and working together with the national security community to minimize the risks . while ideally , one would like to reduce the risk to zero , living in the real world , we know that zero risk would mean little progress and that an impaired research enterprise will leave us more vulnerable . our nation was built by , and has thrived on the efforts of , risk takers . the current oversight system for life science research is functional and robust , ensuring that the pace to discovery is limited only by intellectual and fiscal resources .

abstractin the fall of 2001 , bacillus anthracis spores were spread through letters mailed in the united states . twenty - two people are known to have been infected , and five of these individuals died . together with the september 11 attacks , this resulted in a reevaluation of the risks and benefits of life science research with the potential for misuse . in this editorial , we review some of the results of these discussions and their implications for the future .

tetanus is a disease characterized by hypertonia and muscular spasms due to the toxin tetanospasmin formed by clostridium tetani . tetanus continues to be widespread , especially in the developing world though incidences have come down due to immunization . we present a case of tetanus in a young girl which initially mimicked a striatal toe though the potential cause of inoculation was trauma to nose . a 16-year - old girl presented with a history of sudden onset of pain in left leg with difficulty in walking for 2 days . an upturned big toe without fanning of other toes was seen on the left [ figure 1 ] . a clinical diagnosis of a striatal toe was made and the patient was admitted for further evaluation [ figures 1 and 2 ] . contrast - enhanced magnetic resonance imaging of the brain revealed no abnormality of the basal ganglia . liver function test was normal and slit lamp examination did not show a kf ring . corrected serum calcium was 9.8 mg / dl . on the 3 day after admission , she developed stiffness of bilateral lower limbs followed by episodic spasms of the back and neck . there were no signs of autonomic hyperactivity and she did not have any seizures . on further probing , patient 's mother revealed that she had suffered a fall a day before onset of her symptoms and hurt her nose . her mother said that she had received her primary series of tt in infancy and received her last booster 6 years back at around 10 years of age . she was given tetanus immunoglobulin ( tig ) 5000 units intramuscularly stat and also started on intravenous metronidazole and diazepam infusion . on the 5 day after admission , she developed mild trismus and opisthotonus completing the clinical picture of generalized tetanus . she started showing signs of recovery by day 10 and made a complete recovery in 3 weeks from onset of symptoms . tetanus is caused by the toxin tetanospasmin released by the germinating spores of the anaerobic gram - positive bacilli c. tetani . as spores of the bacilli occur in the soil , tetanus frequently occurs following a contaminated wound , but cases have also been reported after dental procedures , surgeries , burns , intravenous drug abuse , and intramuscular injections . our patient presented with a localized form of tetanus affecting initially the left leg that mimicked a striatal toe even though there was no local injury in the leg . it is possible that our patient had another insignificant trauma to her leg that had gone unnoticed . a striatal toe is a finding in clinical neurology that reliably localized the lesion to the caudate nucleus and putamen . the absence of fanning of the rest of the toes differentiates the upturned big toe from the babinski extensor plantar response . tetanus presenting initially with the classic clinical picture of striatal toe has not been reported before . thus , it is important to keep localized tetanus in the differential diagnosis of extrapyramidal disorders presenting with such spasticity , especially in the absence of definite history of trauma . dystonias can be ruled out in such cases by checking for resolution after a dose of benztropine . severity of tetanus can be graded according to the ablett classification into four grades . according to the ablett classification , the world health organization recommends a 5 dose schedule for tetanus vaccination while in india additional doses are recommended in the universal immunization program ( uip ) . our patient had received three doses as dtp in infancy and another dose between 12 and 24 months of age . she also had received two booster doses of dtp and td at 5 years and 10 years according to her mother as per the uip . the advisory committee on immunization practices of the centers for disease control does not recommend either td vaccination or tig for patients who have received more than three doses of tt with the last dose within 5 years . td is recommended in clean wounds with last booster > 10 years back and other wounds with last booster > 5 years back . tig should be given in unclean wounds when vaccination status is unknown or patient has received <3 doses . tetanus prone wounds are > 6 h old , > 1 cm deep , stellate , ischemic , denervated , contaminated or infected . our patient had received tt immunization at the time of primary wound care as per the recommendations . she did not receive tig as she had received more than 3 doses of immunization in the past . identification of vulnerable patients by testing for protective tetanus antibodies is not viable in our setting . this may be due to a defect in the patients humoral immunity due to which she could nt mount an antibody response . rapid bedside tests to screen for tetanus immunization status have been found to be more effective than medical interview and wound assessment in some studies , but cost effectiveness remains an issue . wound care , antimicrobial therapy with metronidazole and human tig are cornerstones of therapy of established tetanus . a full course of active immunization with tt should be started early as neither clinical infection nor tig provide long - term immunity . cases of tetanus continue to come to medical attention in spite of widespread immunization in this age and time . we must be alert to the possibility and institute prompt treatment after a clinical diagnosis as early passive immunization reduces morbidity and mortality .

we report the case of a 15-year - old girl who was initially diagnosed to have a striatal toe . her condition progressed and she later developed clinical features consistent with tetanus . history of blunt trauma to nose was elicited retrospectively . antimicrobial therapy with metronidazole and both active and passive immunization was started immediately . the patient went on to make a complete recovery .

the presence of oral synechia along with cleft palate is a rare syndrome . we encountered a case of cleft palate accompanied by congenital lateral oral synechia the infant was delivered normally at full term with a low birth weight of 1,750 g. there were no particularly notable points in the family history , medical history , or reproductive history . on examination , the patient had a restricted mouth opening resulting from congenital oral synechia due to membranous adhesion between the free margin of the cleft palate and the floor of the mouth , lateral to the tongue on the left side . the congenital oral synechia appeared as a thin membrane with a broad attachment at the floor of the mouth measuring approximately 2 cm in anteroposterior width . at the palatal margin the attachment narrowed to about 0.5 cm [ figure 1 ] . congenital lateral synechia the infant faced feeding problems due to the restricted mouth opening , and therefore an immediate surgical excision of the synechia was decided on . feeding was done using an infant feeding tube till anesthetic clearance was obtained for the procedure . at 1 week after birth , the congenital synechia was excised under sedation uneventfully restoring adequate mouth opening and allowing normal feeding [ figure 2 ] . the first report of oral synechia was by illera in 1875 , but the first documented case of a lateral synechia between the floor of the mouth and free margin of cleft palate was by hayward and every in 1957 . over the years , almost 60 cases of oral synechia have been reported out of which 52 are lateral synechia and 8 were of the median variety . these can be classified into five types : synechia by cord - like adhesion of the alveolar mucosa on one or both sides of the upper and lower jaw ( alveolar synechia ) ; synechia by a membranous adhesion on the hard palate and floor of the mouth , excluding the rear of the tongue ( lateral synechia ) ; synechia in which the hard palate and tongue are partially involved ; synechia in which the soft palate and tongue are widely involved , such that continuity is interrupted between the oral cavity and the pharynx ; and synechia by a membranous adhesion between the hard palate and lower lip . , reported that five family members had cleft palates and synechia , one having a cleft palate without synechia , and one transmitted the gene but did not express it . the etiology of intra - oral bands or synechia of epithelial tissue has been debated , but many theories have been proposed . during the 7 to 8 week of embryological development , the alveolar ridges , tongue , and palatal shelves are in contact with each other . the ensuing palatal closure depends on downward contraction of the tongue . when the tongue protrudes from the mouth as a result of medial movements of the oral cavity walls , it prevents the alveolar ridges from fusing . genetic , teratogenic , or mechanical insults during this critical stage may lead to periods of close , quiescent contact between oral structures , and this predisposes to abnormal fusion . longacre asserts that oral synechia is due to the persistence of the buccopharyngeal membrane , and is for that reason associated with micrognathia and cleft palate . kruger speculates that the mechanical effect of the tongue may contribute to cases in which the periphery of the cleft palate adheres to mucous membranes on the floor of the mouth , and that adhesion in cases of cleft palate may occur as a result of obstruction by the tongue . according to mathis , when adhesion of the palatal shelf occurs during developmental stages , adhering epithelial rudiments , for some reason , lead to synechia . the general consensus on the treatment of cleft palate lateral synechia syndrome is excision of the synechia and palatal closure . dalal et al . , have reported a case of intra alveolar synechia in two siblings , one of whom had a spontaneous resolution of the adhesion . have documented that the synechia provided additional tissue for surgical closure with less tension on the palatal flaps . we believe that such a use of the synechia has been possible , because the membranous adhesion in this case had fibromuscular bands in it unlike our situation where the synechia consisted of thin membranous tissue of unequal width . the conventional oro - tracheal intubation or the use of a laryngeal mask may not be possible due to the presence of the synechia . fiberoptic nasotracheal intubation may not be feasible due to technical difficulties of finding a bronchoscope small enough for a week old infant . performing a procedure on the highly vascular floor of the mouth under local anesthesia in an infant where immediate excision of the synechia is deemed necessary either due to breathing or feeding problems , sedation can be used under constant anesthetic monitoring for the surgical procedure as was done in our case .

cleft lip and palate are the most common congenital craniofacial anomaly in humans . the presence of oral synechia along with cleft palate is a rare syndrome . we encountered one case that had a cleft palate accompanied by congenital oral synechia due to a membranous adhesion between the floor of the mouth and the free margin of the cleft palate .

the mayer - rokitansky - kster - hauser ( mrkh ) syndrome or mullerian duct agenesis is characterized by congenital aplasia of the uterus and the upper part ( 2/3 ) of the vagina in women showing normal development of secondary sexual characteristics and a normal 46 , xx karyotype . till date the presence of alopecia with mrkh syndrome is a mere coincidence or an associated finding is a subject of research and may require further evidence . a 17-year - old girl presented with hair loss from the scalp for 1 year . the loss of hair begins at the occipital area of the scalp , and it progressed along the lateral margins of the scalp in a band like fashion clinically suggestive of ophiasis type of alopecia areata . detailed gynecological examination revealed normal secondary sexual characters with well - developed external genitalia and hypoplastic vagina [ figure 1b ] . the histopathology of the scalp revealed lymphocytic infiltration around the lower third of the hair follicle , a finding suggestive of alopecia areata . to rule out the cause of amenorrhea , ultrasonograhy of abdomen and pelvis was done which revealed the absence of uterus and right kidney with normal ovaries [ figure 2a and b ] . hormone levels ( follicle - stimulating hormone , luteinizing hormone , estradiol , testosterone , and thyroid function test ) were within normal limits . clinical findings shows ( a ) ophiasis type of alopecia areata , ( b ) well - developed external genitalia and hypoplastic vagina ultrasonography of abdomen and pelvis shows ( a ) absence of uterus , ( b ) absent right kidney the detailed review of four case reports of mrkh syndrome with alopecia in world 's literature has been given in table 1 . first , three reports were from the same geographical region of middle east ( jordan , lebanon , and turkey , respectively ) . hypothesized that the founder mutation in middle east population might be responsible for the condition to be restricted to that geographical region . however , the fourth case report from south asia ( pakistan ) questioned the hypothesis of founder mutation leading to mrkh syndrome restricted to that geographical region . we are reporting the fifth case report which is the second case report from south asia and first among indian population . our case report is another evidence to suggest that mrkh syndrome with alopecia is not restricted to middle east population . review of literature of mrkh syndrome with alopecia in all previously reported four case reports , there was a history of parental consanguinity and siblings were also affected with mrkh syndrome and alopecia . in our case , there was no history of parental consanguinity and siblings were normal . there are many case reports and syndromes of hypogonadism with alopecia . in all the previous case reports of mrkh syndrome with alopecia , hypergonadotropic hypogonadism was noted . this case is the first report of mrkh syndrome with alopecia with normal gonadal function . mrkh syndrome may have associated abnormalities such as renal agenesis , skeletal abnormalities , hearing loss , or cardiac defects . in our case , the right renal agenesis was the associated finding of mrkh syndrome with alopecia which is not observed in all the previous four case reports under review . in a female patient of alopecia areata , history of primary this syndrome is caused by embryologic growth failure of the mullerian duct with resultant agenesis or underdevelopment of the vagina , uterus or the both . all the reported cases of mrkh with alopecia including our case had alopecia areata which is an autoimmune disease . there seems to be no direct correlation between these entities as one is due to a genetic defect , and other is an autoimmune disease . hence , the presence of alopecia in the case of mrkh syndrome is a mere coincidence , or an associated finding of the syndrome is a matter of further study . 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 .

a 17-year - old girl presented with alopecia involving lateral margins of the scalp with primary amenorrhea . there was no history of parental consanguinity , and no other siblings were having similar complaints . her secondary sexual characters were well developed with hypoplastic vagina . histopathological findings from scalp biopsy showed features of alopecia areata . ultrasonography of abdomen and pelvis revealed the absence of uterus and the right kidney . follicle - stimulating hormone , luteinizing hormone , estradiol , testosterone , and thyroid function test was within normal limits . the patient had normal 46 , xx karyotype . till date , only four case reports of mayer - rokitansky - kster - hauser ( mrkh ) syndrome with alopecia has been reported . we are reporting the first case of mrkh syndrome with alopecia with normal gonadal function in world 's literature .

coronary artery fistula ( caf ) is an unusual coronary artery abnormality , where connection exists between coronary artery and cardiac chamber or another blood vessel . caf originating from the left main trunk and left circumflex artery is very unusual and rarely reported in the literature . we describe a case of giant left main trunk and left circumflex artery fistula to the right ventricle . a 24-year - old male patient presented with complaints of dyspnea and palpitation during exercise . on physical examination , his blood pressure was 120/80 mmhg and pulse rate was 75 bpm . the heart was rhythmic and chest auscultation revealed a continuous murmur of grade 3/6 at the upper left sternal border . transthoracic echocardiography ( tte ) showed enlargement of the left main trunk and left circumflex coronary artery , with a 25-mm inner diameter , diffusely tortuous dilated and drained into the posterior wall of the right ventricle [ figure 1 ] . left anterior descending artery , right coronary artery , and major side branches were normal . the left ventricular systolic function was normal with an ejection fraction of 65% and there were no abnormalities in the regional wall motion and cardiac valves . laboratory investigations were normal . in order to further evaluate the fistula in more detail , a 320-slice dynamic volume ct ( aquilion one , software release v. 4.3 , toshiba medical systems , japan ) angiogram was performed , using 3-dimensional volume - rendered ( vr ) and curved - plain reconstruction ( cpr ) . it demonstrated a large fistula arising from the left main trunk and left circumflex artery emptying to the right ventricle [ figure 2 ] . color - doppler echocardiography revealed enlarged and tortuous of lm and lcx ( white arrows ) origin from ao , abnormal flow from ao passing lm and lcx to rv through a fistula ( red arrow ) . ( ao : aorta , lm : left main trunk , lcx : left circumflex artery , lv : left ventricle , rv : right ventricle , ra : righe atrium , la : left atrium ) contrast - enhanced ct coronary angiogram . 3-dimensional volume - rendered ( vr ) and curved - plain reconstruction ( cpr ) shows origin and course of the fistula . the vessel runs from the left main trunk and left circumflex coronary artery and drained into the posterior wall of the right ventricle through a fistula ( red arrow ) . ( ao : aorta , lm : left main trunk , lcx : left circumflex artery , lv : left ventricle , rv : right ventricle ) congenital caf is caused by a cardiovascular abnormality in embryo period , which accounts for 0.27 - 0.40% of all congenital cardiac defects . acquired caf has been described after surgical procedures , endomyocardial biopsy , trauma , inflammation , atherosclerosis , and collagen vascular disease . caf usually drains into the right chamber , pulmonary artery , pulmonary veins , superior vena cava , inferior vena cava , and coronary sinus . most caf patients are usually asymptomatic and are found incidentally during angiographic evaluation for other cardiac diseases . clinical symptoms of patients with caf are dependent on the size of fistula and the pressure of its terminal chamber . in our patient , the giant caf increases the volume load of the right heart chambers and the pulmonary artery . if the shunt of caf is significant , steal blood phenomenon may occur and cause myocardial ischemia or infarction , which increases risk of infective endocarditis , accelerates atherosclerosis , and even heart failure . generally , tte is an important primary non - invasive tool for diagnosis of coronary artery abnormality and confirmed by coronary angiography . with the cardiac ct technological improvement in recent years , particularly in temporal / spatial resolution and reduced radiation dose , coronary computed tomography angiography ( ccta ) has been a relatively new imaging modality and non - invasive method for assessment of coronary artery disease . ccta not only precisely demonstrate the origin , course , and drainage site of the fistula , but also provides surgical plan for treatment . it is suggested that caf should be treated as soon as possible to avoid the potential risk of complications , no matter whether clinical symptom exists or not . in our case , he experienced no serious cardiac events over half a year of follow - up . in conclusion , the giant left main trunk and left circumflex artery fistula to the right ventricle are rarely seen .

coronary artery fistula including the left trunk and left circumflex is uncommon . we present a 24-year - old male patient with a giant left main trunk and left circumflex artery to right ventricle fistula , which is diagnosed by transthoracic echocardiography and coronary computed tomography angiography . in this paper , the case report is to provide a better understanding of clinical characteristics for this disease .

a 60-year - old man was presented to an orthopedician in the operating theatre as a case of difficult urinary catheterization . the patient was going for a lengthy orthopedic operation that required a drained urinary bladder beforehand . the urologist was called and he found the patient anesthetized , having circumcised penis and a pin - hole external urethral meatus ( eum ) at the corona penis level . the tight meatus was negotiated first with a lubricated 4-french ( fr ) bougie urethral dilator followed by subsequent sized dilators up to 14- fr caliber when a proper foley catheter was introduced easily into the bladder . the patient was carefully interviewed to reveal that circumcision was performed neontally as a religious rite , and he has no history of urologic diseases or trouble voiding , as this was the first time for him to meet a urologist . the patient , who is unaware of his condition , is a father of 12 children and never underwent urinary catheterization . hypospadias is a highly prevalent congenital anomaly of the male genitalia . in comprehensive review , sorensen ( 1953 ) credited rennes in 1831 with reporting a prevalence of hypospadias of 1 in 300 recruits and also reported the same figure for live male births in denmark . there is a plethora of hypospadias cases of different degrees of severity repaired in childhood that resulted in different psychological , social , and sexual outcomes . psychosexual effects of hypospadias repair are endured in adulthood although affected men maintain satisfaction in their sexual life . it is unusual in the sense that the elderly man should have had his hypospadias deformity repaired decades ago in order to pass urine freely and to impregnate his spouse with a wider , and properly located meatus . the tight meatus in this case was severe , hardly admitting a lubricated 4-fr bougie dilator . litvak et al . , reported that the meatus in children younger than one year accepts a lubricated 5-fr feeding tube . they also reported that , in children aging 1 - 6 years , an 8-fr feeding tube could pass without difficulty . the social and sexual life of adults operated for hypospadias during childhood has been studied by a few authors . a few publications reported patient and partner dissatisfaction with the appearance of genitalia , as sexual dissatisfaction is often attributed to penile size and curvature . noted that self - reported strength of libido was slightly better for controls compared to patients with hypospadias , but without a statistically significant difference . noted that only about 10% of both patients and controls reported that their libido was low . problems reported include weak or dribbling ejaculation , having to milk out ejaculate after orgasm , quantity of semen passing after intercourse , anejaculation with or without orgasm . liu et al . observed that the rates of ejaculation problems in the distal and proximal groups were 19.5% ( 8/41 ) and 48.6% ( 17/30 ) , respectively [ 5 , 6 ] . the erectile problems in repaired hypospadias may be attributed to surgically correctable and non - correctable causes . more commonly encountered correctable causes include persistent chordee , penile torsion , fistula formation , acquired meatal stenosis , and inadequate cosmetic outcome . similarly , in unrepaired cases , as the eum is distal enough to deposit the seminal fluid high in the posterior vagina with no difficulty , fertility is supposed to be preserved . furthermore , aho et al . found that men who had hypospadias during childhood were less likely to live with a partner , and that they had fewer children ( 0.8 vs. 1.1 ) . figure 1 extremely tight external meatus in a fairly asymptomatic 60 yrs , old man with distal hypospadias . the literature is scant about the urinary , social , sexual , and fertility aspects of unrepaired hypospadias cases . to our knowledge , no other case has been reported for a circumcised uncorrected hypospadias with extremely tight eum in a the finding in this treatise supports the previously published data surrounding the unaffected sexual and fertility aspects of patients having distal hypospadias , and limits repair to cosmetic reasons .

hypospadias is a highly prevalent congenital anomaly . english articles , indexed in pubmed , published the long - term sexual and reproductive outcome following hypospadias repair . almost all repairable cases of hypospadias are operated in childhood . although distal hypospadias does not interfere with fertility , it is worthy reporting a case of an elderly man , who fathers 12 children and has no urologic complaint , presented with unrepaired coronal hypospadias and severely tight external urethral meatus .

gram - positive infections account for up to 50% of cases of severe sepsis in the modern intensive care unit . the complex processes by which gram - positive organisms cause sepsis are poorly understood by comparison with gram - negative sepsis . much interest is currently focused on the role of certain protein exotoxins synthesized by staphylococcus aureus and streptococcus pyogenes , which share the immunological property of being super - antigens . superantigens are characterized by the ability to bypass normal major histocompatibility complex ( mhc)-restricted , intracellular , antigen processing and presentation . through direct binding to the mhc class ii molecule and the t cell receptor , at sites away from those involved in conventional antigen binding , superantigens activate up to 50% of the whole t cell repertoire rather than the 1% fraction stimulated by conventional antigens . such toxins are , however , believed to have a role in causation of two classic superantigen - mediated diseases , staphylococcal and streptococcal toxic shock syndromes , that kill 5000 americans per annum . they may also contribute to the pathogenesis of other forms of gram - positive shock . in addition to intensive care support and antimicrobial therapy , various adjunctive treatments for toxic shock syndrome have been evaluated both in vitro and in clinical trials ( table 1 ) . nevertheless , the treatment of toxic shock syndrome , like that of conventional forms of sepsis , remains suboptimal and the disease is still associated with mortality in the region of 50% and associated with considerable morbidity . they identified a dodecapeptide that is highly conserved among different bacterial superantigens and lies in a region of the super - antigen molecule away from sites involved with either mhc class ii or t cell receptor interaction . several modified forms of this peptide acted as antagonists to a range of bacterial superantigens against which they were tested in vitro . one dodecapeptide , which was a particularly effective antagonist , was administered to mice challenged with bolus doses of bacterial superantigen . arad et al speculate that the mechanism of inhibition may involve co - stimulatory pathways of t cell activation . interestingly , protection against subsequent challenges , at 3-weekly intervals , improved with each challenge . this improved protection correlated with antibody production against the whole challenge superantigen , while antibody against the dodecapeptide was not detected . this finding is in keeping with the previously observed correlation between lack of antibody against streptococcal pyrogenic exotoxin a and development of invasive s. pyogenes infection . one consequence of the cytokine storm induced by superantigens may be to disrupt the development of antibody - mediated immunity . by switching off superantigenicity , the dodecapeptide may be allowing normal antibody production to occur . although the prospect of drugs to switch off superantigenicity is exciting , there have been many false dawns in the field of sepsis research . all laboratory animals are intrinsically resistant to the effects of bacterial superantigens . the mouse model , while being one of the best established systems for studying toxic shock , requires far higher doses of superantigen than are needed to induce shock in humans , and prior ' sensitization ' of the animal with the hepatotoxin d - galactosamine . we have recently demonstrated , in a mouse model of invasive streptococcal infection , that other properties of these toxins may be more important than their superantigenicity and , paradoxically , such effects may in fact be advantageous to the host . furthermore , administration of bolus doses of superantigen probably does not reflect the pattern of toxin production in clinical cases . certain findings of the report by arad et al are at odds with our current understanding of bacterial superantigens . the study found that animals protected from one super - antigen in an initial challenge were cross - protected against different superantigens in subsequent challenges . this effect was observed for toxins as dissimilar as staphylococcal exotoxin b and toxic shock syndrome toxin 1 , which have only 6% sequence homology . this is hard to understand in terms of neutralizing antibody since no cross - reactivity between toxic shock syndrome toxin 1 and other superantigens has been demonstrated in serological or neutralization assays . the paper by arad et al is the first published report of superantigen antagonist peptides . encouragingly , at least one other group is making progress in the same area , and have demonstrated a protective effect not only against bolus doses of superantigen , but also in a model of co - challenge with endotoxin . further studies to address the mode of action of these peptides , particularly in super - antigen - sensitive animal models ( sriskandan et al , manuscript submitted ) , are necessary before speculation about clinical trials is warranted . specific approaches to treatment of toxic shock syndromes spea , streptococcal pyrogenic exotoxin a ; spec , streptococcal pyrogenic exotoxin c.

the production of superantigenic exotoxins by gram positive bacteria underlies the pathology of toxic shock syndrome . future treatment strategies for superantigen - mediated diseases are likely to be directed at blocking the three - way interaction between superantigen , t cell receptor and major histocompatibility class ii molecule , which inititates an excessive and disordered inflammatory response . in this article , we review the first published data to address one such strategy in the context of other recognised and experimental treatments .

endometriosis is a common gynaecological condition characterised by the presence of endometrial glands and stroma at extrauterine sites . ectopic endometrial implants have been reported in nearly every tissue or organ , including the skin . of the reported cases of cutaneous endometriosis , over 70% the case of primary cutaneous endometriosis described here remains a rare entity with a reported incidence of 0.51% of all extragenital endometrial ectopia . in december 2012 , a 31-year - old caucasian woman presented to the dermatology outpatient clinic with a 1-month history of a persistent , raised lesion in the umbilicus . the patient had noted swelling and one episode of spontaneous frank bleeding from the lesion . there was no associated cyclical pain at the lesion site . at the time of examination , a single firm 0.5-cm black - coloured papule was seen within the umbilicus at the 3 o'clock position . no discharge or underlying umbilical hernia was noted . the patient had a history of 2 previous melanomas : the first in 2003 on the left abdomen and the second on the right buttock in 2006 . given the anatomical location , a skin punch biopsy of the umbilical lesion was performed . during dermatology review , 1 month later , it was noted that 3 distinct skin - coloured papules had appeared next to the initial lesion ( fig . histological examination of the punch biopsy revealed a lesion in the superficial dermis comprising a single dilated glandular structure , surrounded by cellular endometrial - type stroma ( fig . the gland was lined by a single layer of columnar cells , a few of which had apical cilia . subsequent gynaecological evaluation revealed a 3-year history of dysmenorrhoea . a provisional diagnosis of coexistent pelvic endometriosis was made . a diagnostic and therapeutic laparoscopic examination was advised if the patient remained unable to conceive over the following 6 months . less than 30% of cutaneous endometriosis appears in the absence of a prior surgical history and is termed spontaneous ( primary ) cutaneous endometriosis . of these cases , umbilical endometriosis characteristically occurs in women of reproductive age , who present with an umbilical nodule at the site of a prior surgical incision associated with cyclical pain at the lesion site . however , the inconsistent appearance and rarity of this phenomenon means it is often clinically misdiagnosed . endometriomas under the skin have been described as red , blue , black or flesh - coloured . classic associated symptoms include cyclical pain , discharge , bleeding or swelling of the lesion correlated with the menstrual cycle . the most widely accepted pathogenesis of secondary endometriosis is the iatrogenic implantation of endometrial cells as a result of surgery , commonly laparoscopic procedures . however , the pathogenesis of primary endometriosis remains uncertain . theories suggested include the implantation of cells through sanguineous or lymphatic spread , or differentiation from coelomic pluripotent cells in the skin . the heterogeneity in the clinical presentation of cutaneous umbilical endometriosis reflects the breadth of differential diagnoses that may mimic the condition ( table 1 ) . malignancies , in particular melanoma and umbilical metastasis of visceral carcinoma ( sister mary joseph nodule ) , must be considered . distinctive dermatoscopic , ultrasonographic and magnetic resonance imaging findings have been described ; however , histopathological examination remains the gold standard . hormonal therapy in the form of gonadotropin - releasing hormone agonists , oral contraceptives and danazol may be given preoperatively to reduce the size of lesions and ameliorate symptoms consistent with pelvic endometriosis . subsequent gynaecological evaluation for pelvic endometriosis is recommended for all patients . around 15% of patients diagnosis of the umbilical skin lesion facilitated the provisional diagnosis and management of underlying symptomatic pelvic endometriosis . of note , the patient in this case study has a strong history of melanoma . recent data have reported a statistically significant association between endometriosis and the occurrence of melanoma . further studies are required to examine the possible association of cutaneous endometriosis and the development of melanoma . in summary , nevertheless , it should be considered in the differential diagnosis when examining any umbilical lesion , even in absence of pathognomonic cyclical symptoms .

cutaneous endometriosis that arises de novo , without a prior history of surgery , is a rare phenomenon . the clinical diagnosis of cutaneous endometriosis remains challenging due to the variable clinical appearance and symptoms of the condition , and therefore must be considered in the differential diagnosis of any umbilical lesion . we report a 31-year - old woman who presented with spontaneous cutaneous endometriosis of the umbilicus .

immune checkpoint inhibition with anti - ctla-4 blockade ( e.g. , ipilimumab ) and anti - pd-1 antibodies ( e.g. , nivolumab ) has improved the poor prognosis of unresectable malignant melanoma [ 1 , 2 ] . these newly approved medications induce many types of immune - related adverse events ( iraes ) , including pneumonitis , colitis , hepatitis , and endocrinopathies such as hypophysitis and thyroiditis . although iraes induced by ipilimumab have occasionally been reviewed in the literature , studies on nivolumab are also gradually being described . a 52-year - old male with lung and liver metastasis of malignant melanoma was put on nivolumab therapy ( 3 mg / kg every 3 weeks ) . after approximately 34 weeks of nivolumab administration ( fig . 1 ) , laboratory tests revealed a sudden elevation of liver enzymes ( ast / alt ) to 1,225/824 u / l ( grade 4 ) . computed tomography detected the exacerbation of liver metastasis , identifying a lesion that was slightly larger than the nondiffuse , localized lesion that had been observed 3 months earlier ( fig . 2 ) . therefore , we suspected hepatitis associated with nivolumab because this drug has a low probability of causing serious liver damage to localized lesions , even when they are large . the patient stopped taking nivolumab and was treated with 70 mg / day ( 1.0 mg / kg / day ) of systemic corticosteroids . his ast / alt level promptly improved to 112/389 u / l within 3 days . the median onset of ipilimumab - related hepatitis is approximately 812 weeks after initial treatment . therefore , the interval from nivolumab initiation to the occurrence of hepatitis was much longer than that reported in previous cases . hepatitis induced by nivolumab was also observed at 712 weeks in a pooled analysis of a nivolumab phase iii trial . however , the incidence of grade 23 nivolumab - induced hepatitis was approximately 1% in a phase iii clinical study . in that study , the liver function test improved to grade 1 within 415 days of initiation of corticosteroid treatment . the liver dysfunction caused by long - term nivolumab therapy in this case is noteworthy . although many diseases induce liver dysfunction , in this case , we definitively excluded other causes of hepatitis such as viral infection ( e.g. , hbv , hcv , hsv , cmv , and ebv ) , medication other than nivolumab , and other forms of autoimmune hepatitis . to manage hepatitis associated with nivolumab , these possibilities must be excluded as causes of the disease . the recommended initial treatment of hepatitis in the risk evaluation and mitigation strategy ( rems ) is to administer systemic corticosteroids ( 12 mg / kg / day of prednisone ) . if the symptoms continue after 35 days , alternative immunosuppressive therapy , such as 500 mg of oral mycophenolate mofetil every 12 h , must be considered . in this case , because the laboratory test showed an ast / alt level > 20 the upper limit of normal , we considered systemic corticosteroids to be the most appropriate treatment . fortunately , the ast / alt level rapidly improved to grade 12 in 3 days , and modification was not necessary . it is very important to note that severe hepatitis can occur despite long - term nivolumab therapy . in this case , it was possible to quickly reach a correct diagnosis because we were aware of the possibility of drug - induced hepatitis , even though the period of occurrence of nivolumab - induced hepatitis had passed . therefore , we believe that this report may be very informative when treating hepatitis as an irae of nivolumab . in addition , to our knowledge , this patient exhibited the longest time between nivolumab administration and the onset of hepatitis that has been reported to date .

immune checkpoint inhibitors have drastically changed in the treatment of many kinds of malignancies , especially malignant melanoma . the focus of the recent experiments has not only been on their efficacy but also immune - related adverse events ( iraes ) . we report a case of fulminant hepatitis due to nivolumab . in this case , the patient had undergone long - term nivolumab therapy . he did not complain of any symptoms but his liver enzyme levels were extremely elevated ( grade 4 ) . we promptly decided to start oral corticosteroids in the patient . his liver function rapidly improved . the dose of corticosteroids was gradually reduced . our case demonstrates that sudden onset fulminant hepatitis can occur despite the safe use of long - term nivolumab therapy . the irae can improve rapidly with proper corticosteroid treatment . this report will be useful for the physicians who always use immune checkpoint inhibitors .

the management of critically ill patients with a suspected invasive fungal infection based on predefined clinical and microbiological criteria or the punctuation of a score may be a valid approach when the definitive diagnosis is feasible only in a small proportion of patients . this is what vandewoude and colleagues propose with their retrospective analysis of all patients who had aspergillus spp . fungal infections have increased in intensive care units ( icus ) over the past decades . although less common than candidiasis , aspergillosis is more likely to result in a life - threatening infection . thus , neutropenic patients , and those who receive long - lasting corticosteroid treatments , are at high risk for invasive aspergillosis . this organism grows on a wide variety of organic material and the conidia are easily aerosolised . although exposure is universal , invasive infection occurs almost entirely in immuno - suppressed individuals . outbreaks have been described in bone marrow transplantation , solid organ transplant recipients and leukaemia patients in association with hospital construction and/or ventilation system contamination with aspergillus . indeed , multiple organ failure and prolonged stays in the icu are associated with a complex decrease in immune functions , deactivation of macrophages and altered cellular response . this is usually not feasible given the special circumstances of critically ill ventilated patients . likewise , screening the blood for galactomannan may be very valuable in neutropenic patients but its usefulness in icu patients is limited . before new antifungal agents were available , mortality of critically ill patients with invasive aspergillosis was nearly 100% . currently , the therapeutic armoury has significantly improved with the introduction of new azoles ( i.e. , voriconazole ) and the echinocandins ( i.e. , caspofungin ) , a new class of drugs with a novel target . many problems contribute to the lack of confident and timely diagnosis of invasive aspergillosis in critically ill patients . on one hand , the early administration of antifungal agents may be life - saving , but clinicians must also bear in mind the problems and costs associated with needless treatments derived from the overinterpretation of the potential clinical significance of isolates of aspergillus spp . in respiratory samples . propose a clinical algorithm based on the criteria defined by an international conference on the diagnosis of aspergillosis in immunocompromised patients . with this approach , approximately 50% of the patients were diagnosed with invasive aspergillosis and in the other 50% the isolation was considered colonization . unfortunately , histology was available only in a small proportion of patients ( one - fourth of patients with presumed diagnosis of infection and one - tenth of the patients with the diagnosis of colonization ) . at first glance , these results seem very hopeful , although positive and negative predictive values can not be calculated with these figures . the diagnostic accuracy of this algorithm can be improved . many authors have documented that invasive aspergillosis can occur in certain types of ' non - immunocompromised ' critically ill patients . three high risk groups stand out for invasive aspergillosis : chronic obstructive pulmonary disease , prolonged multiple dysfunction syndrome in the situation of immunoparalysis , and severe hepatic failure . these underlying conditions are not included in the proposed criteria and they should be added to the list . this may avoid the misclassification of these high risk patients if semiquantitative culture of bronchoalveolar lavage was not positive , a criterion not universally accepted . moreover , a high resolution ct scan is nowadays mandatory and a normal portable chest x - ray may lead to an erroneous classification . the significance of a positive respiratory culture for aspergillus spp . in a non - immunodepressed patient causes the clinician great uncertainty and doubt . nowadays , the isolation of aspergillus spp . in a critically ill patient is not an exceptional curiosity . definitions proposed by the european organisation for the research and treatment of cancer were not designed to guide clinical practice . critical care physicians need a helpful instrument to decide in which circumstances antifungal therapy should be initiated early , given the high mortality of this infection but the availability of new and active agents . obviously , this and other strategies need to be validated in large cohorts of critically ill patients before they can be recommended . this is an urgent task because we do not expect to have at our disposal a precise microbiological test in the near future .

the clinical relevance of recovering aspergillus species in intensive care unit patients is unknown . diagnosis of invasive pulmonary aspergillosis is extremely difficult because there are no specific tests sensitive enough to detect it . the rapidly fatal prognosis of this infection without treatment justifies early antifungal therapy . a clinical algorithm may aid clinicians to manage critically ill patients from whose respiratory specimens aspergillus spp . have been isolated . this new tool needs to be validated in a large cohort of patients before it can be recommended .

by using a previously described dna restriction analysis procedure ( 7 ) , we studied 76 archived adenovirus isolates collected among influenzalike - illness surveillance sites across iowa from 1992 to 2002 . among the 76 isolates , 40 ( 53% ) were ad7d2 , and 6 ( 8% ) were ad7h ( figure ) . the first ad7d2 specimen was isolated in march 1994 from a child living in south - central iowa . the first ad7h specimen was isolated in november 1993 from a child living in north - central iowa . number of adenovirus ( ad ) isolates collected in iowa during influenzalike - illness surveillance by genome type and year . ad7d2 caused illness among patients in iowa ranging in age from 3 months to 49 years . although the clinical details are sparse , a number of patients were thought to have influenza or were diagnosed with respiratory distress syndrome . at least 4 children from an ad7d2 october 2000 epidemic at a long - term care facility in des moines , iowa , died . ad7d2 became increasingly more prevalent across iowa , displacing ad7b , the predominant genome type circulating in the united states since the early 1970s ( 8) . in 2002 , data suggest that ad7d2 supplanted all other ad7 genome types ( 9 of 9 ad7 isolates were a7d2 ) ( figure ) . ad7d2 was first detected in israel in 1992 ; beginning in 1995 , it was associated with epidemics of unusually severe respiratory disease with high fevers among children in japan ( 9,10 ) . ad7h was first detected in south america in 1986 ; since then it has supplanted the previous most prevalent genome type , ad7c , in chile , uruguay , argentina , and possibly other countries ( 11 ) . ad7h has caused pediatric respiratory epidemics , and infected children had longer hospitalizations , had higher temperatures , and required more supplemental oxygen ( 12 ) . in at least 1 study , up to 94% of adenovirus deaths were attributed to ad7h ( 11 ) . whether these strains are truly more virulent or whether they better evade the host s immune system is a matter for future study . what does seem to be clear is that a simple mutation ( ad7d2 ) ( 9 ) or recombination ( ad7h ) ( 13 ) may generate new adenovirus strains that could result in more epidemics and higher death rates . the number of immunocompromised patients in the united states is increasing , and they , in addition to young children , may be at increased risk for severe disease from emergent adenovirus strains . developing molecular typing strategies for emerging ad strains seems prudent , as does improving local and national surveillance for adenovirus illness . considering adenovirus to be a potential nosocomial pathogen seems wise , and researchers should seek to identify effective antiviral therapy for outbreak interventions . these actions will help public health officials better understand the changing epidemiology of adenovirus infections . because of increased adenovirus morbidity ( 14,15 ) , the u.s . department of defense recently contracted to again produce ad4 and ad7 vaccines for military trainees . if civilian populations were identified to be at high risk for serious ad4 or ad7 disease , they might also benefit from these vaccines .

we evaluated 76 adenovirus type 7 ( ad7 ) isolates collected in iowa from 1992 to 2002 and found that genome type ad7d2 became increasingly prevalent . by 2002 , it had supplanted all other ad7 genome types . the association of ad7d2 with severe illness and death calls for heightened public health concern .

funding for travel or speaker honoraria : ( 1 ) istanbul ms days , novartis pharmaceuticals , speaker honoraria payment to mayo clinic . no personal compensation . ( 2 ) invited professor program , biogen - boston , speaker honoraria payment to mayo clinic . no personal compensation . other activities : grant review for the national multiple sclerosis society . research support , commercial entities : dr . orhun h. kantarci receives research support from the european regional development fund ( fnusaicrc cz.1.05/1.1.00/02.0123 ) , the national multiple sclerosis society , and has given a scientific presentation at a meeting supported by teva pharmaceuticals but has received no personal fees or personal compensation for this activity ( all compensation for consulting activities paid directly to mayo clinic ) nor has spoken about the specific medications involving this company . kejal kantarci ( spouse ) serves on the data safety monitoring board of takeda global research and development center , inc . , and the data monitoring boards of pfizer inc . and janssen alzheimer immunotherapy and is funded by the nih ( r01 ag040042 [ pi ] , r21 ns066147 [ pi ] , p50 ag44170/project 2 [ pi ] , p50 ag16574/project 1 [ pi ] , r01 ag11378 [ co - i ] , u19 ag10483 [ co - i ] u01 ag042791 [ co - i ] ) and minnesota partnership for biotechnology and medical genomics ( po03590201 [ pi ] ) . kejal kantarci serves on the data safety monitoring board of takeda global research and development center , inc . , and the data monitoring boards of pfizer inc . and janssen alzheimer immunotherapy and is funded by the nih ( r01 ag040042 [ pi ] , r21 ns066147 [ pi ] , p50 ag44170/project 2 [ pi ] , p50 ag16574/project 1 [ pi ] , r01 ag11378 [ co - i ] , u19 ag10483 [ co - i ] u01 ag042791 [ co - i ] ) and minnesota partnership for biotechnology and medical genomics ( po03590201 [ pi ] ) kejal kantarci serves on the data safety monitoring board of takeda global research and development center , inc . janssen alzheimer immunotherapy and is funded by the nih ( r01 ag040042 [ pi ] , r21 ns066147 [ pi ] , p50 ag44170/project 2 [ pi ] , p50 ag16574/project 1 [ pi ] , r01 ag11378 [ co - i ] , u19 ag10483 [ co - i ] u01 ag042791 [ co - i ] ) and minnesota partnership for biotechnology and medical genomics ( po03590201 [ pi ] ) . research support , foundations and societies : 20062009 multiple sclerosis society for support in multiple sclerosis research unrelated to this publication ; 20082009 mayo foundation cr20 award for support in multiple sclerosis research unrelated to this publication ; 20092011 hilton foundation support in multiple sclerosis research unrelated to this publication .

before the genomics technology revolution allowed us to do genome - wide science , genetics research relied on our limited knowledge about a subject to generate hypothesis and candidate genes to study . despite the level of naivet , several associations with susceptibility to a complex disease such as multiple sclerosis ( ms ) were discovered . of these , hla - drb1 and il7r1 stand out as being confirmed and refined early by the genome - wide association studies ( gwas ) that followed.2 despite the expense and gargantuan efforts , these gwas have successfully led to the discovery of more than 100 additional genes , albeit with smaller effect sizes , that contribute to ms susceptibility.3 this list keeps growing , but it comes with no surprise that most of these genes identified the immune system as one large candidate for ms susceptibility .

there is an increasing concern amongst the women and girls about the appearance of their external genitalia . many women and adolescent girls present with labia hypertrophy . a young girl presenting with labia hypertrophy is quite unusual . a child with severe hypertrophy of bilateral labia minora patient a , youngest of six female siblings , was born at term in a hospital through the lower segment caesarean section . at 8 years of age , she noticed progressive increasing size of labia , which caused her pain while prolonged sitting , walking and daily activities . the increased size of labia affected her psychologically as she considered herself abnormal among her siblings . she disclosed the enlargement to her elder sister almost after 6 months of noticing it , and the patient consulted the plastic surgery clinic at the age of 8 years . on clinical examination , her labia minora was found to be thickened and enlarged more than 5.5 cm from the free edge of the labia majora with associated hypertrophy of the clitoral hood [ figures 1 and 2 ] ( type 6 : franco 's classification ; severe hypertrophy : ricci and pardo classificatio ) [ tables 1 and 2 ] . pre - operative view of labia minora hypertrophy size of labia minora measuring 5.5 cm in its width franco classification of labia minora hypertrophy ricci and pardo classification of labia minora hypertrophy for surgical correction on work - up , her hormonal assay revealed no abnormality . her thyroid profile , serum luteinizing hormone , serum follicle - stimulating hormone , serum testosterone and serum estradiol levels were within normal limits . skin and subcutaneous tissues in the labia minora were found to be hyperplastic . extended linear excision was performed which included excision of hypertrophied clitoral hood [ figures 3 and 4 ] . after 24 h , the patient was advised to clean the genitalia with application of antiseptic ointment . linear excision of labia minora and clitoral hood reduction immediate post - operative result ( a and b ) post - operative result after two weeks with good aesthetic appearance histological examination showed acanthosis and chronic non - specific inflammation of underlying dermis [ figure 6a and b ] . ( a and b ) histological picture illustrating mild acanthosis with mild chronic non - specific inflammation with congestion ( 40 , h and e ) hodgkinson and hait first described labia minora hypertrophy in 1984 ; though description regarding circumcision of labia minora and clitoris has been mentioned vaguely in many old scriptures . juvenile labia minora hypertrophy [ jlmh ] is a less common clinical condition and becomes rarer in a developing country like india where social taboos and customs are stringently enforced in the common lives . in such milieu , jlmh cases are detected only when symptoms significantly affect a patient 's functional and psychological well - being . hence , whenever a surgeon in a developing country like india diagnoses a jlhm patient , it should be addressed properly , and relevant procedure should be carried out to relieve the patient from distressing symptoms . although no standard consensus has been established yet to classify labia minora hypertrophy ; many do exist depending on the size and severity of the hypertrophy [ tables 1 and 2 ] . arbitrary landmarks taken in these measurements make it difficult to diagnose hypertrophy on a standard scale . some have measured size from midline horizontally , and some have measured from free edge . . surgical decision should largely be dependant on labia size as well as symptoms addressed by the patient . a number of studies have been published in context to labia minora hypertrophy and their management , but only a few have addressed the occurrence of hypertrophy in adolescent age and most of their patients fall in 2040 age group . although age is not a criterion for labiaplasty , most of the surgeons refrain from doing it at an early age . our patient was an 8-year - old when she noticed labial hypertrophy , which is rare . all the relevant investigations , including hormonal assays and usg were normal suggestive of an idiopathic , isolated and abnormal morphology of the labia . the size of the labia minora as well as presenting symptoms should be taken into account , while planning labiaplasty in any age group , especially in adolescents . a better classification system for labia minora hypertrophy and standardization regarding functional and cosmetic labiaplasty need to be established in this era of ever increasing vulvovaginal surgeries .

labia minora hypertrophy is a relatively uncommon surgical entity being popularised in the realm of vulvovaginal plastic surgeries . apart from the unaesthetic appearance of the hypertrophied minora , these cases are also associated with itching , hygiene problem , pain while sitting down , sports activities , difficulty in wearing tight clothing , bleeding and discomfort while or after sexual intercourse , social embarrassment , insecurity and psychological diminution of confidence and self - esteem . in a country like india , due to sociocultural reasons , patients hesitate to consult a doctor for such deformities . most of the patients suffer in silence for years . although common in the west , very few surgeons in the country perform this simple and rewarding surgery . here , we are presenting a case of premenarchal juvenile labia minora hypertrophy ( jlmh ) in an 8-year - old child . labial hypertrophy in this age group is uncommon . we were unable to find hypertrophy of labia minora in the eight - year - old child on english literature search .

both interstitial granulomatous dermatitis ( igd ) and palisaded neutrophilic granulomatous dermatitis ( pngd ) are rare disorders typically associated with systemic autoimmune conditions . they probably represent different aspects of a disease spectrum encompassing the concept of autoimmunity - related granulomatous dermatitis ( argd ) . the present case shows that the clinicopathological correlation in argd does not always clearly fit with the classical presentations of igd or pngd . a number of autoimmune conditions are associated with cutaneous symptoms . interstitial granulomatous dermatitis ( igd ) the typical clinical presentation corresponds to a linear rope present on the trunk and axillae . on histopathological examination , igd is characterized by a sparse palisaded histiocytic infiltrate with little neutrophilic debris and eosinophils associated with a necrobiotic aspect of the collagen matrix . in the literature , there is some overlap between igd and palisaded neutrophilic granulomatous dermatitis ( pngd ) . lesions of pngd typically correspond to papules occasionally admixed with small crusts on the elbows . other related disorders include rheumatoid papules , churg - strauss granuloma , superficial ulcerating rheumatoid necrobiosis , necrobiotic granuloma , palisading granuloma , cutaneous extravascular necrotizing granuloma , and rheumatoid neutrophilic dermatitis . here , a 61-year - old woman presented with skin lesions over the elbows and the left thenar region . they corresponded to erythematous and discrete hyperkeratotic papules which had been present for a few weeks ( fig . immunohistochemistry revealed the presence of cd3 + t lymphocytes predominating in the perivascular area ( fig . they were associated with cd68 + and mac387 + histiocytes as well as factor xiiia+ dermal dendrocytes ( dd1 ) . the pattern of dd1 distribution was similar to that of cd68 + histiocytes ( fig . it was characterized by the elevation of anti - smooth muscle antibodies ( 1:160 ) and antinuclear antibodies ( 1:1,280 ) . there were no detectable antibodies directed against nuclear antigens , keyhole limpet hemocyanin and mitochondria . hepatitis b and c serologies were negative . at the time of presentation , the patient 's liver tests were in the normal range . however , they recurred a few months later , and some other lesions appeared on the thighs . in some cases , there is a mismatch between the clinical presentation and the histopathological patterns of igd and pngd . indeed , these two conditions possibly represent a continuum or progression of a single disease process corresponding to autoimmunity - related granulomatous dermatitis ( argd ) [ 6 , 8 ] . the clinical associations between argd , autoimmune disease and lymphoproliferative disorders have been intermixed between both igd and pngd diagnoses in the literature [ 6 , 9 , 10 , 11 , 12 , 13 , 14 ] . it is acknowledged that the inflammatory cell infiltrate is sparse to moderate in igd , while pngd has a dense neutrophilic and interstitial histiocytic infiltrate . only a single case of igd with autoimmune hepatitis association has been reported so far . some other autoimmune comorbidities including arthritis , antiphospholipid syndrome [ 18 , 19 ] and hematologic malignancy have been reported in association with igd . in addition , interstitial granulomatous drug reactions have been linked to tnf inhibitors , ace inhibitors , diuretics [ 21 , 22 , 23 , 24 , 25 , 26 , 27 , 28 ] and soy products in food .

aimboth interstitial granulomatous dermatitis ( igd ) and palisaded neutrophilic granulomatous dermatitis ( pngd ) are rare disorders typically associated with systemic autoimmune conditions . they probably represent different aspects of a disease spectrum encompassing the concept of autoimmunity - related granulomatous dermatitis ( argd).case reporta 61-year - old woman presented with argd and autoimmune hepatitis . the clinical presentation suggested pngd , while histopathology was consistent with igd.discussionthe association of argd with autoimmune hepatitis is apparently a rare event . the present case shows that the clinicopathological correlation in argd does not always clearly fit with the classical presentations of igd or pngd .

abnormal variation of the posterior corneal curvature may occur in two forms : the generalized posterior keratoconus , characterized by an regular increase of the curvature of the entire posterior corneal surface has , and the circumscribed posterior keratoconus , in which a localized paracentral or central posterior corneal indentation is seen . in the generalized form , the corneal stroma typically remains clear . in contrast , the circumscribed posterior keratoconus shows stromal opacities overlying the localized anterior ectasia of the posterior surface , which may occupy the full stromal thickness . circumscribed posterior keratoconus is usually bilateral and sporadic , but familial cases have been also documented . despite the anterior protrusion in some cases , the 60-year - old white male of mediterranean origin presented for a cataract extraction on his left eye . visual acuity was 20/25 in the right eye and light perception in the left eye due to cataract formation . the patient denied history of injury , reporting only a bilateral ocular infection in childhood was reported . slitlamp examination revealed a bilateral paracentrally localized depression of the posterior curvature measuring 3 mm in diameter . there was scarring in the overlying corneal stroma ( figures 1 , 2 , and 3 ) . the posterior depression was clearly detectable using ultrasound biomicroscopy ( humphrey , zeiss , oberkochen ) ( figure 5 ) and slit - scanning topography analysis ( orbscan , bausch and lomb ) ( figure 6 ) . corneal thickness measured 450 m within the lesion and 540 m in the adjacent healthy cornea using the orbscan system . the refractive power of both the posterior and anterior corneal curvature was 50 to 56 diopters within the paracentral area . following phacoemulsification and posterior chamber lens implantation visual acuity increased to 20/50 in the left eye . the clinical and topographic findings in this patient are consistent with the paracentral keratoconus posterior circumscriptus . this is the first report on ultrasound biomicroscopy to visualise the local anterior bulging of the posterior corneal surface with concomitant thinning of the stroma . light microscopy of this abnormality has shown focal disorganization of basal epithelium and basement membrane , a replacement of bowman 's layer by fibrous tissue , a thinned stroma with an irregular arrangement of the central collagen lamellae , and a variable appearance of descemet 's membrane with posterior excrescences indentating the vacuolated endothelium correspond to the corneal guttae seen in specular reflection . iron deposits are present in the basal and suprabasal epithelium , corresponding to the brownish epithelial line observed clinically , indicating an irregularity of the anterior corneal surface . visualisation of the posterior keratoconus using corneal topography analysis has been reported so far in a few cases [ 7 , 8 ] . the light microscopy findings suggest an early pathogenic mechanism probably originated in the fifth or sixth month of gestation . it is classified as one of the anterior chamber cleavage anomalies ( mesenchymal dysgenesis ) , as there are other anterior segment and systemic developmental abnormalities , as well as melanin depositions surrounding the posterior depression and iridocorneal adhesions . however , not all cases share this phenomenon . acquired cases occur and are usually associated with trauma [ 9 , 10 ] . the mechanism in such cases involves an oblique penetrating injury with splitting of the inner corneal layers . differential diagnosis also includes congenital disorders as peter 's anomaly and congenital hereditary endothelial dystrophy but they are usually found in new borns . inflammation process as perforated corneal ulcer may also be taken into consideration , but it is usually unilateral . in most of the cases of posterior keratoconus the vision is not affected , rarely it may be associated with other ocular abnormalities as polar cataract , lenticonus , and ectopia lentis .

this paper documents a rare nonprogressive developmental disorder bilateral circumscribed posterior keratoconus in a 60-year - old man referred for a cataract surgery . for the first time ultrasound biomicroscopy was used to visualise the local anterior bulging of the posterior corneal surface with concomitant thinning of the stroma . the amount of localized posterior depression , corneal thickness and the refractive power of both the posterior and anterior corneal curvature were measured using slit - scanning topography analysis ( orbscan ) .

we report the case of a 58-year - old woman with intracranial hemorrhage associated with stress - induced cardiomyopathy . left ventricular dysfunction was confined to midventricular segments , and manifested with transient anterolateral wall aneurysm . although wall motion was severely impaired in the mid - segments , an almost preserved global midventricular radial strain forecasted rapid improvement of ventricular function . this case highlights how deformation imaging can help in clinical practice to interpret the subtle signs of recovery from left ventricular dysfunction . a 58-year - old woman was admitted one year ago to the neurointensive care unit of our hospital for sudden onset of intracerebral and subarachnoid hemorrhage of the right frontal lobe . she had a history of tobacco use and moderate alcohol abuse , hypertension and chronic obstructive pulmonary disease . her surgical history included left lumpectomy for breast cancer , amputation of the third finger on the right hand and jaw surgery a few years ago . at admission her vital signs were within the normal range : blood pressure 140/87 mmhg , heart rate 71 beats per minute ( bpm ) , respiratory rate 20 breaths per minute and oxygen saturation on ventilator 100% . the electrocardiogram ( ecg ) at admission showed normal sinus rhythm ( 60 bpm ) and possible septal necrosis . right craniotomy for evacuation of the hemorrhage and clipping of right middle cerebral artery aneurysm were performed . troponin i levels showed a slight increase from 0.33 to 3.55 ng / ml during the first two days , and then decreased to 1.96 ng / ml on the third day postsurgery , and similar variations were seen for the levels of creatine kinase - mb fraction ( from 4.0 to 11.5 to 4.3 ecg on the second day showed deep negative t waves in almost all leads with prolonged qtc interval [ figure 1 ] . due to abnormal myocardial necrosis markers and ecg , a two - dimensional ( 2d ) transthoracic echocardiogram was ordered , and revealed a normal left ventricular cavity size with hyperdynamic basal motion , akinesis of midventricular segments and nearly normal apical motion [ figure 2 ] . left ventricular global systolic function was moderately decreased with left ventricular ejection fraction at 35% ( normal values > 55% ) . electrocardiogram on second day post - right craniotomy for evacuation of hemorrhage and clipping of right middle cerebral artery aneurysm showing deep negative t waves in almost all leads with prolonged qtc interval ( 580 ms ) two - dimensional transthoracic echocardiography in the apical four - chamber view showing the left ventricle a ) at end diastole and b ) mid - systole . basal and apical segments contract well in contrast to midventricular segments the next day , the patient underwent coronary angiography , which ruled out any coronary artery disease . however , the ventriculogram surprisingly revealed an aneurysmatic anterior wall [ figure 3 ] , which then was characterized by another transthoracic echocardiogram as an anteroseptal wall aneurysm with near - normal motion of the other segments [ figure 4 ] . global radial strain was calculated and , despite seriously compromised midventricular anteroseptal motion , midventricular - averaged radial strain was at the lower end of normal range ( i.e. 16% ) . left ventriculogram showing systolic anterior wall aneurysm two - dimensional transthoracic echocardiography in the apical long - axis view showing anteroseptal wall aneurysm with nearly normal motion of the other segments in a ) end diastole and b ) mid - systole as predicted by the global radial strain in the previous examination , another transthoracic echocardiogram performed three weeks later documented the complete resolution of wall motion abnormalities [ figure 5a and b ] , with completely normal radial strain values [ figure 5c ] . two - dimensional ( 2d ) transthoracic echocardiogram in the apical long - axis view showing complete resolution of wall motion abnormalities at a ) end diastole and b ) mid - systole . on the left , a short - axis view of the mid - left ventricle is shown , and a 2d speckle tracking technique is used to obtain the curves representing radial deformation of each wall segment ( right ) . the averaged global strain is 57% ( represented by white curve ) isolated left ventricular midventricular dyskinesia is a rare variant of stress - induced cardiomyopathy , although it has been described in association with cerebral injuries . left ventricular mechanics in stress - induced cardiomyopathy have not been well defined , although some data about the longitudinal and radial strain are available . our case highlights the importance of global radial strain as a predictor of myocardial wall motion improvement , even when 2d transthoracic echocardiogram did not forecast the improvement .

introduction : we report the case of a 58-year - old woman with intracranial hemorrhage associated with stress - induced cardiomyopathy.results:left ventricular dysfunction was confined to midventricular segments , and manifested with transient anterolateral wall aneurysm . although wall motion was severely impaired in the mid - segments , an almost preserved global midventricular radial strain forecasted rapid improvement of ventricular function.conclusions:this case highlights how deformation imaging can help in clinical practice to interpret the subtle signs of recovery from left ventricular dysfunction .

a 67-year - old male patient had undergone esophagectomy and intrathoracic esophagogastrostomy for carcinoma of the lower thoracic esophagus 27 months earlier . the original pathologic condition was determined to be moderately differentiated squamous cell carcinoma invading the lamina propria without lymph node metastasis ( clinical t1bn0m0 ) . he had undergone follow - up chest computed tomograph and esophagogastroduodenoscopy once or twice a year and a gastric carcinoma at the intra - abdominal part of the gastric conduit was found . the tumor was a 2a+2c type lesion and located in the posterior wall of the antrum . because of its morphology ( flat and depressed type with ulceration ) , size ( more than 10 mm ) and poor localization , endoscopic mucosal resection or partial gastric resection was not suitable ( fig . 1 ) , so we decided to perform a total resection of the gastric conduit . the whole procedures were performed in 3 stages : first thoracic , second abdominal , and third thoracic and abdominal approaches . because of previous intrathoracic esophagogastrostomy , it was necessary to check the intrathoracic condition and operability . the esophago - gastric anastomosis was located at the upper part of the paratracheal area ( figs . 2 , 3 ) . after dissecting the adhesions between the pleura , lung , and gastric conduit with blunt and sharp procedures , we resected the gastric conduit at the level of the previous anastomosis . the intrathoracic portion of the esophagus was about 4 cm segment long , so we saved it as long as possible and inserted the 28 mm anvil into it . the antral portion of the stomach was adhered to the diaphragm , liver , and surrounding structures . the length and vasculatures of the transverse and descending colon were considered to be suitable for an esophageal conduit . the left side of the colon with a pedicle of the left colic artery was prepared for esophageal reconstruction . the proximal sigmoid colon was connected ( end to side ) to the proximal part of the transverse colon with a 28 mm end - to - end anastomosis ( eea ) stapler . after the left - sided colon conduit into the thoracic cavity in the isoperistaltic manner , esophago - colonostomy was done with a 28 mm eea stapler . the distal part of the colon conduit was anastomosed at the side of the proximal jejunum with a 25 mm stapler in the manner of roux - en - y anastomosis . on the 10th postoperative day , an esophagogram showed no leakage or stenosis of the passage ( fig . the patient has received follow - up care for 7 months after surgery without any problems . after warren and gates reported on it in 1932 , the concept of multiple primary malignancies has been accepted widely and is supported by hypothesis of field cancerization . synchronous or metachronous multiple primary malignancies in esophageal cancer have since been reported with a frequency of about 10% . gastric conduit cancer is not only cancer arising in the remnant gastric conduit but also the second primary cancer following esophageal cancer . the incidence of gastric conduit cancer has been reported to be 0.2%-3.5% [ 2 - 4 ] . although the etiology of the secondary carcinoma in the gastric conduit is not well known , gastric conduit cancer has been reported increasingly because of survival prolongation of esophageal cancer patients after surgery . in our hospital , we have annually operated on about 30 esophageal cancer patients with gastric conduit and this surgical management of gastric conduit cancer was our first experience . because thoracotomy with adhesiolysis is necessary for performing a reoperation , total resection of the gastric conduit reconstructed via the posterior mediastinal route is very difficult . in addition , it is more invasive because it requires reconstruction with another organ such as the colon , jejunum , or skin , for the conduit . therefore , it is important to detect the lesion at an early stage so as to treat it with minimally invasive surgery such as endoscopic mucosal resection or partial resection . therefore , careful , periodic endoscopic evaluation after operation in the patient with esophageal cancer is needed . we had performed annual esophagogastoduodenoscopy evaluation and other radiologic studies twice in a year . in conclusion , a careful , periodic , and endoscopic evaluation after surgery for esophageal cancer is needed for early detection of other primary malignancies . in addition , surgical treatment of gastric conduit cancer after esophageal cancer surgery was found to be possible and safe in our experience .

we report a very rare case of surgery on gastric conduit cancer . a 67-year - old male patient underwent esophagectomy and intrathoracic esophagogastrostomy for squamous cell carcinoma of the lower thoracic esophagus 27 months ago . upon follow - up , a gastric carcinoma at the intra - abdominal part of the gastric conduit was found on an esophagogastroduodenoscopy . we performed total gastrectomy and esophagocolonojejunostomy in the manner of roux - en - y anastomosis . the postoperative course was not eventful and an esophagogram on the 10th postoperative day showed no leakage or stenosis of the passage . the patient was discharged on the 17th day with no complications .

a 25-year - old female patient who earlier had implant placement in relation to 46 and 47 reported to our department for second stage surgery . 1994 , in which the attached mucosa at the top of the ridge is moved in a buccal direction at second stage surgery to obtain a papilla like formation and increased bulk of tissue around implants . the following steps are involved in this technique.([figure 1]-schematic representation ) technique ( hand drawn representation ) identify the location of the cover screws through the covering mucosa [ figure 2 ] make an incision at the palatal / lingual aspect of the cover screws , followed by vertical releasing incisions in the buccal direction . it is important to preserve the gingival cuff at neighboring teeth [ figure 3 ] elevate a full thickness flap in the buccal directionremove the cover screwsselect proper abutments and connect them to the fixtures [ figure 4 ] make semilunar bevel incisions in the buccal flap toward each abutment . start at the distal aspect of the most mesially located implant [ figure 5 ] disengage the pedicle , and then rotate it 90 in the palatal direction to fill in the inter - implant space [ figure 5 ] suture the tissues , allowing no tension within the pedicles [ figure 6 ] . identify the location of the cover screws through the covering mucosa [ figure 2 ] make an incision at the palatal / lingual aspect of the cover screws , followed by vertical releasing incisions in the buccal direction . it is important to preserve the gingival cuff at neighboring teeth [ figure 3 ] elevate a full thickness flap in the buccal direction remove the cover screws select proper abutments and connect them to the fixtures [ figure 4 ] make semilunar bevel incisions in the buccal flap toward each abutment . start at the distal aspect of the most mesially located implant [ figure 5 ] disengage the pedicle , and then rotate it 90 in the palatal direction to fill in the inter - implant space [ figure 5 ] suture the tissues , allowing no tension within the pedicles [ figure 6 ] . the clinical significance of attached gingiva around implants include prevent spread of inflammationprevents recession of marginal tissueprovides tight collar around implantsenable patients to maintain good oral hygiene . prevent spread of inflammation prevents recession of marginal tissue provides tight collar around implants enable patients to maintain good oral hygiene . this technique answered the above requirements , and yet it is very simple and provided excellent papilla like formation around implants . the healing abutment should be in place for 2 weeks for the tissues to heal like papilla [ figure 7 ] . healing after 2 weeks with healing abutments the patient was observed every 6 months , till 2 years . after 8 weeks , the final abutments were connected and noted that excellent soft tissue manipulation around implants created a perimucosal soft tissue contour matching the gingival architecture [ figures 8 and 9 ] . this thickness of tissue / seal around implants was present even after 1-year follow - up and 2 years follow - up periods [ figure 10 ] . postoperative restoration postoperative after 3 months two years postoperative mainly because of the thickness of mucosa obtained during the procedure , the patient was able to maintain the soft tissue around the implant with lesser plaque formation . moreover , a tight tissue barrier was created like natural dentition , which correlated well with the tissue barrier concept postulated by goldman and cohen . zone of gingiva would facilitate subgingival plaque formation because of improper pocket closure resulting from the movability of the marginal tissue . the absence of keratinized mucosa increases the susceptibility of peri - implant lesions and plaque - induced destruction . mehdi adibrad et al . reported that there is a significant influence of the width of keratinized mucosa on the health of the peri - implant tissues . the absence of adequate keratinized mucosa around implants supporting over dentures was associated with higher plaque accumulation , gingival inflammation , bleeding on probing , and mucosal recession . the thickness obtained with this technique resulting in less plaque accumulation and better maintenance correlated well with the studies reported above . in order to have better papilla like formation the following should be considered : careful handling of tissues in order to minimize trauma and maximize vascularizationbevel incisions in the mobile flaps should be delicate and should vary according to the needsrotated pedicles should be tension freesuturing technique should provide a tight and firm connection of pedicles to bone and abutments . careful handling of tissues in order to minimize trauma and maximize vascularization bevel incisions in the mobile flaps should be delicate and should vary according to the needs rotated pedicles should be tension free suturing technique should provide a tight and firm connection of pedicles to bone and abutments . it is imperative and proved by this case report that the operating surgeon should know the importance of papilla and attached mucosa around the implant . this case report was followed for 2 years and excellent prognosis obtained is very much evident [ figure 11 ] . this technique is simple and effortless in providing esthetic papilla formation and protective attached mucosa around implants .

peri - implant plastic surgery aims at improving the esthetic aspects of smile and masticatory function . over the years , several techniques such as tissue punch technique , full thickness flaps , and scalloping adjustment of flaps around implants have been employed ; it was very difficult to achieve a papilla like formation around implants . these added time and expense of the final results and led to undesirable complications . in order to overcome these difficulties , this case report describes a surgical technique where in papilla like formation and increase in width of attached gingiva around implants can be achieved with a single surgical procedure .

it is incidentally found in autopsy specimens and surgical specimens with cryptorchidism and testicular germ cell tumor . this non - neoplastic lesion is usually seated in the mediastinum and septal part of the rete testis . it may present as a very small lesion detected in microscopic examination or a solid - cystic mass lesion which is macroscopically evident . in microscopic evaluation cryptorchidism was unilateral and the other testis was normal . there were no clinical or endocrine abnormalities . the testis measured 4 2.5 2.2 cm in size and there was no tumoral lesion in gross examination . on the cut section close to the testis parenchyma , there was a gland like tubular structures . some of these were in back - to - back position with little intervening stroma . the testicular parenchyma was comprised of seminiferous tubules that had sertoli cells only and no spermatozoa ( fig . ema ( novocastra ; 1/150 , clone gp 1.4 , newcastle , united kingdom ) and pankeratin ( novocastra ; 1/150 , clone ae1/ae3 , newcastle , united kingdom ) immunohistochemical stains were performed for diagnosis . adenomatous hyperplasia of the rete testis is a benign lesion which can appear at any time during life and was first described by nistal et al . . it is important to recognize tumor - like lesions of the rete testis to make an accurate differential diagnosis whether the lesion is benign or malign . ahrt etiology is not well understood yet but there seem to be associated conditions such as cryptorchidism , some kidney diseases , and germ cell tumors . our case was cryptorchid and seminiferous tubules near the lesion were lined by only sertoli cells and had no spermatozoa . the authors reported an adult case with ahrt in unilateral testis with no luminal spermatozoa . there are different cases such as prostate adenocarcinoma or embryonal carcinoma together with ahrt in the literature [ 6 , 7 ] . the true epithelial proliferation with gland - like or papillary proliferations replacing normal epithelium , continuous architecture of normal rete testis with adenomatous hyperplasia and lack of signs of malignancy are the proper diagnostic criteria for ahrt . in our case the differential diagnosis should include adenoma , papillary adenoma , primary and metastatic adenocarcinoma and rete testis cystic transformations with epithelial metaplasia . clinical history , localization , histologic features and immunohistochemistry are criteria for differentiating these lesions . complete surgical resection is the proposed therapy for ahrt cases and no report for recurrence has been identified . in conclusion , adenomatous hyperplasia of the rete testis is a rare lesion that can be confused with malignancy and should be remembered in the differential diagnosis of rete testis lesions .

there are several tumor - like lesions and miscellaneous neoplasms of the rete testis . we present a case with adenomatous hyperplasia of the rete testis ( ahrt ) . the patient was 24 years old with undescended testis and was referred to our hospital . there were no clinical or endocrine abnormalities . cryptorchidism was unilateral and the other testis was normal . right orchiectomy was performed and sent to the pathology laboratory for examination . morphologic and immunohistochemistry findings confirmed the diagnosis of ahrt in this case . the patient 's postoperative course continues uneventfully . ahrt is a rare lesion and can be confused with malignancy . it is incidentally detected in microscopic investigation . it may present as a very small lesion detected in microscopic examination or a solid - cystic mass lesion which is macroscopically evident . clinical history , localization , histologic features and immunohistochemistry are criteria for differentiating these lesions.we present this rare case for both surgeons and pathologists due to its importance as it can be confused with malignancy .

emphysematous cholecystitis ( ec ) is an uncommon variant of acute cholecystitis , which is caused by secondary infection of the gallbladder wall with gas - forming organisms [ 15 ] . the mortality rate of ec is as high as 25% because of its high complication rate [ 2 , 5 ] due to gangrene , gallbladder perforation , pericholecystic abscess and bile peritonitis . emergency surgical intervention is necessary for ec treatment for which open cholecystectomy has traditionally been performed [ 1 , 35 ] . here a 78-year - old male was admitted to our hospital for an investigation of vertigo . after admission , he developed fever and abdominal distention . a chest x - ray in the up - right position and abdominal computed tomography ( ct ) the chest x - ray showed the presence of gas within the gallbladder and in its wall ( fig . 1 ) . the ct scan revealed emphysematous changes in the gallbladder wall and air within the gallbladder ( fig . the patient was referred to the department of surgery and emergent surgery was performed . figure 1:up - right chest x - ray demonstrating air in the gallbladder ( arrow ) and in its wall ( arrowheads ) . figure 2:(a and b ) abdominal computed tomographic scans of axial and coronal views revealing emphysematous changes in the gallbladder wall and air in the gallbladder . up - right chest x - ray demonstrating air in the gallbladder ( arrow ) and in its wall ( arrowheads ) . ( a and b ) abdominal computed tomographic scans of axial and coronal views revealing emphysematous changes in the gallbladder wall and air in the gallbladder . based on the hemodynamically stable status of the patient , we selected a laparoscopic approach . exploration of the abdominal cavity revealed gangrene and necrosis of the gallbladder . to facilitate the resection , we initially punctured the gallbladder using a percutaneous needle to extract bile . although there was significant adhesion , we were able to safely dissect the calot 's triangle and detected a cystic duct and artery . his postoperative course was uneventful . a bile culture obtained during surgery revealed clostridium perfringens , enterobacter cloacae and enterococcus faecalis as the causative organisms . ec is an uncommon variant of acute cholecystitis characterized by potentially high mortality and morbidity because of a high incidence of gangrene or perforation . garcia et al . reviewed 20 cases of ec and reported that the mortality rate was as high as 25% and the morbidity rate as high as 50% . emergent surgical intervention for ec is indicated with cholecystectomy as the definitive treatment , although percutaneous cholecystostomy for critically ill patients presents another option [ 1 , 4 , 5 ] . bouras et al . reported that the laparoscopic approach for ec can be considered a safe procedure . owing to the advances of laparoscopic surgery , it has allowed to perform emergency cholecystectomy . our patient was hemodynamically stable ; therefore , we selected the laparoscopic approach , which was successfully and safely completed . the causative organisms of ec include c. perfringens , escherichia coli and species of the genera staphylococcus , streptococcus , pseudomonas and klebsiella [ 15 ] . ec is associated with diabetes mellitus and primarily affects males , as in the present case . the symptoms of ec are almost the same as those of acute cholecystitis , including right upper quadrant pain , nausea , vomiting and low - grade fever [ 2 , 4 ] . a prompt diagnosis of ec is essential and plain abdominal x - rays or ultrasound can be diagnostically useful with a sensitivity of up to 95% . however , contrast - enhanced abdominal ct scans may offer more information . in our patient , a chest x - ray in the up - right position detected air in the gallbladder wall and an abdominal ct revealed typical presentation of ec . emergency intervention is indicated and the laparoscopic approach can be considered a safe and effective method in hemodynamically stable patients .

emphysematous cholecystitis ( ec ) is an uncommon variant of acute cholecystitis , which is caused by secondary infection of the gallbladder wall with gas - forming organisms . the mortality rate of ec is still as high as 25% . emergency surgical intervention is indicated . open cholecystectomy has been traditionally accepted as a standard treatment for ec . we present a case of ec successfully treated by laparoscopic surgery . laparoscopic cholecystectomy for ec is considered to be safe and effective when indicated .

the causes of pulmonary embolism can be venous thromboembolism , and nonthrombotic embolism like septic , fat , air , amniotic fluid and tumour embolism . tumour embolism to lungs can arise from cancers of breast , stomach , liver , kidney and rarely from choriocarcinoma . choriocarcinoma is a malignant , trophoblastic cancer , belonging to the malignant end of the spectrum in gestational trophoblastic disease , which can occur following molar pregnancy , ectopic pregnancy , abortion and even normal pregnancy [ 2 , 3 ] . choriocarcinomas spread via blood and lymphatics with early haematogenous spread to lungs , resulting in pulmonary embolism , pulmonary oedema , pulmonary hypertension or acute respiratory distress syndrome . in a young female presenting with persistent shortness of breath , cough and chest pain , the possibility of metastatic pulmonary embolism should be considered as surgery and chemotherapy can cure the choriocarcinoma metastasizing to the lungs . a 25-year - old lady was admitted in the pulmonology ward with the diagnosis of pneumonia . the patient had presented with a history of progressive shortness of breath , chest pain and persistent cough with occasional haemoptysis . she had a history of being treated with antibiotics and anti - tb drugs in outpatient basis . since her symptoms were persistent and her general condition was deteriorating , she was admitted to the ward . on examination , she had crepitations in bilateral chest and an oxygen saturation of only 80% . cect chest was done , which revealed pulmonary embolus occluding the main pulmonary artery , and right and left pulmonary arteries ( fig . 1 ) . serum beta - human chorionic gonadotrophin ( hcg ) level was found to be significantly high . she was managed further with chemotherapy ( emaco regimen ) with excellent response to the treatment . figure 3:histopathological image showing the trophoblastic elements . a ct scan image showing thrombus in the pulmonary artery . women in the reproductive age group with lung metastasis present with dyspnoea , chest pain , cough and haemoptysis . as the diagnosis can always be misleading and patient might be treated in the line of pneumonia or tuberculosis , it is very essential to have a high index of suspicion . surgery is indicated in patients with haemodynamic instability and those with massive tumour burden occluding main pulmonary and branch pulmonary arteries . so , chemotherapy should be initiated as soon as the diagnosis is strongly suspected or confirmed . pulmonary embolism due to choriocarcinoma should always be suspected in a reproductive age woman presenting with intractable shortness of breath .

pulmonary embolism carries a significant morbidity and mortality . metastatic choriocarcinoma presenting as pulmonary embolism is a rare event . here , we report a case of a 25-year - lady with a history of worsening shortness of breath for 4 months who was treated as a case of pneumonia and tuberculosis . owing to the worsening condition , she had a contrast enhanced computed tomography ( cect ) chest done and was diagnosed to have pulmonary embolism . she underwent pulmonary embolectomy . the histopathological examination of the embolus revealed it to be metastatic choriocarcinoma . she showed a good response to chemotherapy . metastatic choriocarcinoma should be considered as a differential diagnosis in females presenting with pulmonary embolism .

for the self - assembling process , a solution containing 10 nm scaffold strand , 50 nm of each staple strand ( reverse - phase cartridge purified , bioneer inc . ) , 5 mm tris + 1 mm edta ( ph 7.9 at 20 c ) , 16 mm mgcl2 was heated to 80 c for 4 min , cooled down to 60c over the course of 80 min , and cooled further down to 24 c over the course of 72 h. the folded objects were electrophoresed on a 1.5% agarose gel containing 45 mm tris borate + 1 mm edta and 11 mm mgcl2 at 70 v for 3 h. to prevent the origami structures from denaturation during electrophoresis , the gel - box was cooled in an ice - water bath . the gel - band containing the structures was physically extracted from the gel and run through spin columns ( freezensqueeze spin columns , biorad ) at 5000 rcf . the dna tensegrity objects were then imaged with tem after negative staining with uranyl formate on a fei tecnai t12 biotwin at 80 kv . a 12-helix bundle kite with four springs of equal length ( 300 nt ) was designed such that the restriction site for ecori is located in one of the 300-nt spring regions . the assembled kites with a double - stranded region at the ecorri restriction site ( gaattc + 10 bases or more overhang on both sides of the sequence ) were incubated for one hour with the enzymes ( 20 l of 10 nm scaffold , 80 nm each staple , 1xneb - buffer 2 , 20 units of enzyme , new england biolabs , inc . ) . uncut and cut tensegrity kites were electrophoresed on a 1.5% agarose gel . the gel - bands were physically extracted and imaged with tem after negative staining with uranyl formate . for the self - assembling process , a solution containing 10 nm scaffold strand , 50 nm of each staple strand ( reverse - phase cartridge purified , bioneer inc . ) , 5 mm tris + 1 mm edta ( ph 7.9 at 20 c ) , 16 mm mgcl2 was heated to 80 c for 4 min , cooled down to 60c over the course of 80 min , and cooled further down to 24 c over the course of 72 h. the folded objects were electrophoresed on a 1.5% agarose gel containing 45 mm tris borate + 1 mm edta and 11 mm mgcl2 at 70 v for 3 h. to prevent the origami structures from denaturation during electrophoresis , the gel - box was cooled in an ice - water bath . the gel - band containing the structures was physically extracted from the gel and run through spin columns ( freezensqueeze spin columns , biorad ) at 5000 rcf . the dna tensegrity objects were then imaged with tem after negative staining with uranyl formate on a fei tecnai t12 biotwin at 80 kv . a 12-helix bundle kite with four springs of equal length ( 300 nt ) was designed such that the restriction site for ecori is located in one of the 300-nt spring regions . the assembled kites with a double - stranded region at the ecorri restriction site ( gaattc + 10 bases or more overhang on both sides of the sequence ) were incubated for one hour with the enzymes ( 20 l of 10 nm scaffold , 80 nm each staple , 1xneb - buffer 2 , 20 units of enzyme , new england biolabs , inc . ) . the gel - bands were physically extracted and imaged with tem after negative staining with uranyl formate .

tensegrity or tensional integrity is a property of a structure that relies on a balance between components that are either in pure compression or in pure tension for its stability [ 1,2 ] . tensegrity structures exhibit extremely high strength - to - weight ratios and great resilience , and are therefore widely used in engineering , robotics and architecture [ 3,4 ] . here we report nanoscale , prestressed , three - dimensional tensegrity structures in which rigid bundles of dna double helices resist compressive forces exerted by segments of single - stranded dna that act as tension - bearing cables . our dna tensegrity structures can self - assemble against forces up to 14 pn , which is twice the stall force of powerful molecular motors such as kinesin or myosin [ 5,6 ] . the forces generated by this molecular prestressing mechanism can be employed to bend the dna bundles or to actuate the entire structure through enzymatic cleavage at specific sites . in addition to being building blocks for nanostructures , tensile structural elements made of single - stranded dna could be used to study molecular forces , cellular mechanotransduction , and other fundamental biological processes .

hybridization is a controversial topic in coral reef ecology 1 , 2 . while small numbers of hybrid embryos can be produced in a few species in vitro , the evidence for hybrids in the field is often equivocal because the genetic techniques used for corals can not distinguish between hybridization and incomplete lineage sorting . in fact , only one of the over 1300 species in the order is generally accepted to be unequivocally of hybrid origin : acropora prolifera 1 , 5 . nonetheless , hybridization is often invoked as a source of evolutionary novelty in the order scleractinia 6 , 7 . here , we report an incidental observation on the potential for hybridization between two closely related scleractinian corals species in the family fungiidae , ctenactis echinata and ctenactis crassa . these species are sympatric , often dominating large multi - specific assemblages of fungiid corals throughout the central indo - pacific . colonies of these species are superficially very similar ( figure 1a and b ) but can readily be distinguished by the shape of the costal dentitions . both species are gonochoric , that is each colony is either male or female , and reproduce by broadcast spawning , releasing gametes into the water column for fertilization ( figure 1c and d ) . at our study site on sesoko island ( 2638'13.00"n ; 12751'56.24"e ) , okinawa , japan , spawning occurs following the full moons from july to august . furthermore , both species release gametes at the same time and consequently there is the potential for hybridization . in the days before the predicted date of spawning in july 2013 , we collected four colonies of c. echinata and six colonies of c. crassa , to produce larvae for other experiments . coral species in the family fungiidae , such as these colonies of fungia repanda , are gonochoric broadcast spawners : each individual releases either eggs ( c ) or sperm ( d ) into the water column where fertilization takes place ( arrows indicate gametes ) . while the species are relatively easy to identify , determining the sex of each individual prior to spawning is impossible without destructive sampling to expose the gametes . consequently , we placed each individual in a separate 20 l bucket containing sea water in the open air at approximately 20:00 h in order to sex each individual once gametes had been released . on the night of 27 july between 22:30 and 23:30 h three c. echinata and five c. crassa spawned revealing that the three spawning c. echinata were female , while four c. crassa were females and one was a male . the size of the eggs of each species at the time of release was distinct with a range in maximum diameter of 244266 m in c. echinata and 133155 m in c. crassa . in contrast to earlier work on c. echinata , we saw no symbiotic algae in the eggs of either species . we collected approximately 5000 eggs from the three c. echinata females and mixed them with sperm from the c. crassa male . as a positive control we mixed eggs from the four c. crassa females with the c. crassa sperm . approximately 100 eggs were observed under a stereo - dissecting microscope for cleavage , indicating fertilization , every 2 to 6 h over the next 24 h. at no point did we observe cleavage in the cross between species indicating that no hybrid embryos were produced . in contrast , over 90% of c. crassa eggs in the positive control were fertilized within 2 h. we conclude that despite synchrony in the time of gamete release between these two closely related sympatric species there appears to be strong pre - zygotic mechanism to avoid hybridization . while our observations are preliminary and in only one direction ( i.e. we did not cross c. echinata males with c. crassa females ) we predict that hybridization between these species is unlikely . this observation adds to a growing body of evidence indicating strong pre - zygotic barriers to hybridization in many scleractinian corals 11 13 .

hybridization is often cited as a potential source of evolutionary novelty in the order scleractinia . while hybrid embryos can be produced in vitro , it has been difficult to identify adult hybrids in the wild . here , we tested the potential for hybridization between two closely related species in the family fungiidae . we mixed approximately 5000 eggs of ctenactis echinata with sperm from ctenactis crass . no hybrid embryos were produced . this observation adds to a growing body of evidence for pre - zygotic barriers to hybridization in corals and challenges the claim that hybridization is a major source of evolutionary novelty in the order .

a 44-year - old woman presented to the emergency room with shortness of breath and thoracic pain radiating to the left arm and shoulder . her vital signs showed a blood pressure of 158/102 mmhg , a pulse rate of 135 beats / min , a normal temperature and oxygen saturation of 100% on room air and no signs of heart failure . laboratory research revealed an elevated troponin t of 0.18 g / l , a creatinine kinase of 170 u / l and ck - mb of 17.9 non - significant coronary abnormalities were found by coronary angiogram . left ventriculogram showed akinetic wall motion abnormality in posterolateral and diaphragmatic wall segments , and a hypokinetic apex ( fig . 1 ) . medical treatment with furosemide , anticoagulants and an ace inhibitor was started . on the ecg , negative t waves evolved combined with prolonged qt duration ( fig . 2 ) . 1left ventriculogram with wall motion abnormalities in the distal segments and normal wall motion in basal segments comparative to stress - induced cardiomyopathy . a systolic phase , b diastolic phasefig . 2ecg at admission ( a ) with slight st elevation in v2 and 16 h after admission ( b ) with negative t waves in the right precordial leads , i and avl with prolonged qt duration left ventriculogram with wall motion abnormalities in the distal segments and normal wall motion in basal segments comparative to stress - induced cardiomyopathy . a systolic phase , b diastolic phase ecg at admission ( a ) with slight st elevation in v2 and 16 h after admission ( b ) with negative t waves in the right precordial leads , i and avl with prolonged qt duration the patient appeared to have had a previous incidence of thyrotoxicosis ( graves disease ) in 2002 . her current thyroid function confirmed a toxic state with a thyroid - stimulating hormone ( tsh ) of < 0.05 mu / l and free thyroxin ( t4 ) of > 100 pmol / l and tri - iodothyronine ( t3 ) of 26 pmol / l . six days after treatment with propylthiouracil and propanolol , her heart rate reduced to 80 beats / min and the sequential echocardiogram showed an improvement of her systolic left ventricular function ( fig . 3 ) . magnetic resonance imaging measured a left ventricular ejection fraction of 52% . 3transthoracic echocardiogram with return of normal systolic function after initiating medical treatment transthoracic echocardiogram with return of normal systolic function after initiating medical treatment hyperthyroid patients can present with angina symptoms and abnormal ecgs in the absence of coronary artery disease . a myocardial infarction can be induced due to increased demand . a hypertrophic heart due to an increase in cardiac protein synthesis is also seen . forfar et al . discovered that hyperthyroid patients have an elevated ejection fraction at rest but experience significant declines during exercise , also known as hyperthyroid cardiomyopathy . we speculate that a surge of catecholamine release during physical stress in combination with a reduced ejection fraction caused by the thyrotoxicosis state resulted in severe left ventricular dysfunction . after initialising medical treatment the symptoms were reduced and the left ventricular function returned to normal with borderline left ventricular hypertrophy remaining . in this case report we demonstrate a combination of reversible cardiomyopathy related to stress ( tako tsubo ) and thyrotoxicosis that resolved completely with medical treatment .

we report on a 44-year - old woman presenting with chest pain and dyspnoea without previous stress - related events . by means of echocardiography severe left ventricular dysfunction and wall motion abnormalities resembling stress - induced cardiomyopathy ( tako tsubo ) were seen . laboratory investigation revealed thyrotoxicosis and elevated cardiac markers . six days after starting medical treatment , complete restoration of the left ventricular function was observed . the transient left ventricular dysfunction was induced by thyrotoxicosis resembling stress - induced cardiomyopathy that resolved completely after medical treatment .

accidents with a pneumatic nail gun , which has been used in the construction industry , have increased.2 ) however , reports on management of a patient with a nail gun injury to the cranium are rare . the authors report on a case of a patient treated with simple retraction after small skin and prophylactic antibiotic administration . a 56-year - old man had five nail gun - shots due to attempted suicide and was transferred to the emergency room . on his physical examination , his mental status was drowsy and there was no neurological deficit except for nails on his cranium . in his past history , there was no psychological background , however , he used to drink often and became depressed . on plain skull radiography , five nails were found ( figure 1a ) . among the five nails , two penetrated glabella . two nails were located in the right parietal bone and another was located in the left parietal bone . due to the head of the nail , the direction was confined to the outer table of the skull . in addition , a subarachnoid hemorrhage was observed on brain computed tomography ( ct)(figure 1b ) . all procedures were performed in the operating room under general anesthesia in order to treat the emergent condition as rapidly as possible . the nail head was located beneath the scalp and tightly attached to the skull , it was not visible from the outside . a small incision to advance a forceps and a rongeur was necessary , and , all nails were removed successfully ( figure 1c ) . during the period of conservative management antibiotics including vancomycin , ceftriaxone , and metronidazole were administered for two weeks and carbamazepine was administered for a week . conventional angiography was performed , and there was no vascular invasion or newly formed fistula ( figure 1d ) . head injury by pneumatic nail gun injury is rare , and there have been some case reports on management , treatment . according to lee and park,4 ) the speed of the launched nail and presence of the nail head are important determinants of the extent of nail gun related head injuries . if major artery or sinuses are not involved , extraction from the nail head could be the main treatment . ct is typically the first - line radiologic examination in the emergency room for head - injured patients . in general , ct shows excellent details in bone and average value in soft tissue . failure to recognize cerebral damage following penetrating brain injuries , especially when there is retained foreign body , can lead to serious life - threatening complications.1 ) in addition , because involvement of the large vessels is critical to determining the operative strategy , vascular integrity should be evaluated with ct angiography or ct enhanced image in the initial evaluation in the emergency room . in this case , nails were removed by a small scalp incision due to the fact that major artery or sinuses were not involved . if this is not the case , management would be direct ligation or vascular bypass surgery . despite many controversies regarding use of prophylactic antibiotics , the authors believe that broad spectrum antibiotics are appropriate for patients with penetrating brain injury since it is also recommended that antibiotics should be started as soon as possible.3 ) post - traumatic epilepsy is a major cause of disability and inadequate social integration . although there is no prospective study to indicate the efficacy of prophylactic anti - epileptic medication following a traumatic brain injury , it has been recommended that patients be covered for about the first week after injury with a medication such as phenytoin or carbamazepine.5 ) in the patient with head injury by pneumatic nail gun , the first evaluation should focus on the shape of the nail head and vascular integrity from simple x - ray and ct . if the nail head is confined to the depth of skull penetration , cautious extraction of the nail head can be performed without postoperative complication and early start of prophylactic antibiotics can be the next treatment .

a 56-year - old man had five nail gun - shots on his skull due to attempted suicide and was transferred to the emergency room . because the nail head played a role as a brake , the launched nail made a hole in the skull but did not entirely pass through it . if major artery or sinuses are not involved , cautious retrieval after a small scalp incision can be performed and prophylactic antibiotics be administered for treatment .

implantable cardioverter defibrillator ( icd ) implantation is performed at many centers in korea,1)2 ) however , icd lead extraction has not yet been reported . we report our experience of icd lead extraction using locking stylet and polypropylene dilator sheath in a patient with recurrent inappropriate shocks due to lead fracture . a 46-year - old man presented to our institution for evaluation of repeated , inappropriate , icd shocks . icd ( single chamber , dual coils , vitruso dr d164awg , medtronic inc . , minneapolis , mn , usa ) was implanted for sustained monomorphic ventricular tachycardia associated with unstable hemodynamics and underlying systolic left ventricular dysfunction . the ejection fraction of 30% was recorded two years ago at another hospital . during icd implantation , ventricular fibrillation was induced by t - shock and successfully terminated by biphasic shock at 10 j. icd interrogation revealed 33 episodes of shock delivery due to noise sensing . however , a definite break point of icd lead was not detected on chest x - ray . icd therapy was switched off based on a clinical diagnosis of icd lead fracture , and the patient was transferred to our hospital . the icd lead ( sprint quattro 6944 , medtronic inc . ) was tined , and the diameter of lead tip and shaft ( comprising shock coil ) was 2.7 mm . icd lead extraction was performed with support from the cardiac surgery team . following routine preparation for generator removal , the disconnected lead was tested again to exclude connection problem between icd lead and the generator , which showed high impedance over 2,500 ohms . lead extraction was performed after confirming icd lead fracture . a locking stylet ( liberator locking stylet 016 - 032 , cook vascular inc . , vandergrift , pn , usa ) was inserted into the central core of the icd lead to prevent lead disruption . a 12 fr ( 4 mm ) polypropylene dilator sheath ( byrd dilator sheath sets , cook vascular inc . ) mild traction force was applied to the locking stylet to straighten the alignment of the icd lead and the dilating sheath . the dilator sheath was advanced with bidirectional ( clockwise and counter - clockwise ) rotation to dissect adhesive fibrous bands formed around the icd lead . when the dilator sheath was placed 1 - 2 cm below the tip of right ventricular ( rv)-lead , the locking stylet was pulled gently with gradually increasing traction forces . the icd lead and dilator sheath were successfully removed without evidence of lead fragments remaining in the right ventricle ( fig . there was no evidence of major complications , such as hemopericardium , hemothorax , and myocardial inversion . after confirming successful icd lead extraction , a new icd lead was inserted into right ventricle followed by skin closure . pacemaker or icd rv - lead extraction is a high risk procedure with a morbidity of 1.4 - 2.5%.3)4 ) fibrous tissues encapsulate implanted leads and cause adhesion to major veins , the right atrial or the ventricular wall.5 ) major complications such as hemopericardium , hemothorax and death occur during fibrous tissues dissection around the implanted leads.6 ) incomplete lead removal or procedure failure is another clinical problem that occurs in 7 - 13.2% of cases.3)4 ) if an infected lead is retained , the risk of treatment failure with antibiotics will be very high.7 ) even non - infected lead fragment may cause embolic complications.8)9 ) therefore , various extraction techniques have been tried to improve the success rate of complete lead removal . they include direct traction with rotational forces,10 ) traction with locking stylet,11 ) counter - traction with dilating sheath,3)4 ) femoral workstation,12 ) laser sheath,13 ) and electrosurgical sheath.14 ) traction and counter - traction using locking stylet and dilating sheath is a conventional technique for pacemaker and icd lead extraction . it has been proven to be effective and safe in clinical trials.3)4 ) although newly developed techniques such as electrosurgical or laser sheath are in clinical use , they have not been introduced in korea , and the conventional technique using locking stylet and dilator sheath is still useful if performed by experienced operators.14 ) the authors tried 25 pacemaker and one icd lead extractions associated with lead malfunction or infection since 2004 . of the 26 cases , 24 cases were successfully managed with the conventional traction and counter - traction technique using locking stylet and dilator sheath . in the two failed attempts , the operators could not advance the dilator sheath to the icd lead tip due to severe fibrous adhesion at the subclavian vein and in the superior vena cava . of the 24 successful cases , open heart surgery was performed in one case due to cardiac tamponade following complete pacemaker ventricular lead extraction . icd lead extraction is known to have higher complication rate than pacemaker lead extraction.6 ) however , it can be removed successfully with the same techniques . to our knowledge , this is the first icd lead extraction case reported in korea .

a 46-year - old man presented to our institution with inappropriate implantable cardioverter - defibrillator ( icd ) shock delivery . the icd ( single chamber , dual shock coils ) was implanted for sustained monomorphic ventricular tachycardia with unstable hemodynamics and underlying systolic left ventricular dysfunction . icd interrogation revealed recurrent episodes of icd shock due to noise sensing and increased impedance of right ventricular - lead . with the impression of lead fracture , icd lead extraction was performed . the fractured icd lead was completely removed by traction of locking stylet and counter - traction of polypropylene dilator sheath . a new lead was inserted and the patient was discharged without complications after 2 days . to our knowledge , this is the first report on icd lead extraction by conventional traction and counter - traction technique in korea .

patients with collagen vascular disease ( cvd ) such as rheumatoid arthritis , systemic lupus erythematosus , scleroderma , polymyositis , and dermatomyositis , are known to be sensitive to ionizing radiation 19 . irradiation to the lung in patients with interstitial pneumonia ( ip ) is also considered a relative contraindication because ip is a risk for severe radiation - induced pneumonitis 10 . meanwhile , in general , irradiation of extrapulmonary sites in ip patients is not contraindicated . here , we report a patient with bladder cancer and mild ip who received pelvic irradiation and showed unexpectedly severe adverse effects that followed by the onset of polymyositis and fatal exacerbation of ip . a 70-year - old japanese man with bladder cancer ( ct1n0m0 ) presented to the radiation oncology department in march 2014 . he had ip , which had been controlled stable with prednisolone ( 10 mg / day ) since 2010 . a chest computed tomography ( ct ) revealed ground - glass opacity in the subpleural region ( figs.1 and 2 ) . spirometry test results showed a restrictive pattern ( vital capacity , 55% ; forced expiratory volume 1.0 , 76% ) . although he was diagnosed as being tolerable for surgery under general anesthesia , he refused surgery and selected radiotherapy for his bladder cancer . he had a previous history of spinal canal stenosis and cataract in the left eye , however , he did not have a medical history indicative of a risk for severe radiation toxicity including diabetes mellitus . radiotherapy targeting the bladder and pelvic lymph node regions was initiated , with a daily fraction of 2 gy . however , he developed urinary tract pain and erythema in the radiation field much earlier than expected ( i.e. , within the first week ) . alpha - blockers , loxoprofen , intravesical infusion of lidocaine , and betamethasone valerate ointment were ineffective . in the third week , he showed grade 3 urinary tract pain , grade 3 radiation dermatitis ( assessed using the common terminology criteria for adverse events , version 4.0 ) , and needed to urinate every 5 to 10 min . thus , radiotherapy was terminated at 34 gy , much less than the target bladder dose of 60 gy . , he was admitted to our hospital with proximal muscle weakness , which had worsened over the previous 2 weeks . iu / l ) and c - reactive protein ( 3.66 mg / dl ) levels , myogenic changes in the left deltoid and biceps brachii on electromyography , a high signal in the proximal muscles on magnetic resonance imaging ( short - ti inversion recovery ) , and the presence of autoantibodies against aminoacyl - trna synthetase ( anti - ars antibodies ) led to a diagnosis of polymyositis . meanwhile , ct ( chest through pelvis ) showed no signs of infection or progression of cancer . an increased dose of prednisolone ( 60 mg / day ) over 1 month improved the muscle weakness and ck levels ( 639 chest ct showed extensive areas of ground - glass opacity and consolidation , suggesting an acute exacerbation of ip ( fig.3 ) . despite maximum medical therapy , including prednisolone pulse ( 1 g / day ) and cyclophosphamide , he died 1 week later . we conducted radiotherapy to this patient because he had not presented with any symptoms suggestive of cvd . although we could not identify the specific reason of the severe adverse effects during radiotherapy , the following onset of polymyositis suggested that the patients had vulnerability to ionizing radiation . to the best of our knowledge , this is the first case reported in the literature of unexpectedly severe adverse reactions to radiotherapy followed by the onset of cvd . the current case suggests that unexpectedly severe adverse effects after radiotherapy can be an alarm of cvd onset , which enables the prediction and early intervention of the cvd . notably , cvd - associated ip can precede the appearance of systemic cvd symptoms by many years ; sometimes by more than 5 years 12,13 . these findings are consistence with our case which had a 4-year history of ip before the onset of polymyositis . in such cases , it is impossible to distinguish cvd - associated ip from other types of ip ( e.g. , drug - induced ip or idiopathic ip ) because the clinicopathological findings of cvd - associated ip show wide variation 1113 . at the time of radiotherapy however , there may be a considerable number of patients with ip - preceding cvd who are at risk of unexpectedly severe toxicity after radiotherapy . anti - ars antibody - positive polymyositis and dermatomyositis are strongly associated with ip 15 . of note , 2950% of anti - ars antibody - positive ip - associated cases polymyositis and dermatomyositis are ip - preceding type 15 . taken together , these findings suggest that measurement of anti - ars antibody levels in ip patients prior to radiotherapy may help predict severe radiation toxicity associated with the late onset of polymyositis or dermatomyositis . cvd markers such as antinuclear antibody were not evaluated in the stable ip period . in summary , careful observation for the alarm adverse effects of radiotherapy , especially in ip patients , may lead to the prediction and early intervention of the cvd . written informed consent was obtained from the patient s next of kin for publication of this case report and accompanying images .

key clinical messageinterstitial pneumonia ( ip ) sometimes precedes collagen vascular disease ( cvd ) onset . a patient with bladder cancer and mild ip received pelvic irradiation and experienced unexpectedly severe urinary toxicity followed by polymyositis onset and fatal ip exacerbation . careful observation for alarm adverse effects of radiotherapy in ip patients may help predicting cvd onset .

( 1 ) patients may report dysuria , intermittency , haematuria , frequency , hesitancy , nocturia or sudden termination of voiding . a 62 year old lady presented to the hospital with a swelling in her right thigh . she had a past history of myelomeningocele spina bifida which had been closed at birth . she suffered from urinary incontinence and had been managed with long term suprapubic catheters for 20 years . she had required a vesicolithotomy via a midline incision and a vaginal hysterectomy 13 years previously . initial examination revealed a swollen right thigh and a calcified mass protruding through the skin in the right groin . a strong smell of urine was noted although it was thought that this was secondary to a leakage from around the site of her supra pubic catheter . laboratory data demonstrated normal renal function , a raised c - reactive protein and normal white cell count . due to her swollen right leg a deep vein thrombosis was excluded with an ultrasound scan and negative d dimer and plain radiographs of her right hip and pelvis were requested . a urology opinion was sought , initially , with regard to management of the suprapubic catheter . on closer examination , urine was noted to be leaking not from the suprapubic site but around the calcified mass in the right groin and a diagnosis of vesicocutaneous fistula was made . her radiographs demonstrated two urinary calculi ( fig 1 ) and she went on to have an abdominal computed topography scan ( fig 2 ) which confirmed the diagnosis of a vesicocutaneous fistula . plain radiographs demonstrating two bladder calculi ct demonstrating the two bladder calculi pointing towards the right groin vesicolithotomy was performed removing the calculi by enlarging the fistulous opening . ( fig 3 ) two calculi measuring 40 mm 43 mm 30 mm and 43 mm 50 mm 33 mm were extracted . 4 ) the suprapubic catheter was changed and the fistula was left open and urine leakage continued to decrease in quantity until the fistula tract closed . intraoperative view of the bladder calculi emerging through the vesicocutaneous fistula into the right groin the two bladder calculi following extraction urological complications are often a major source of morbidity for these patients . ( 5 ) abnormal bladder sensation and function combined with sphincter dysnergia predispose the spina bifida patient to urinary stasis , recurrent urinary tract infections , stone formation and renal function impairment . ( 5 ) management of the problem can be difficult and may include the use of anti - cholinergics , intermittent self catheterisation ( isc ) , supra pubic catheterisation or surgical intervention to allow urinary diversion . ( 6 ) increased fluid intake , weekly changes of indwelling catheters if isc is not possible and regular ultrasonic screening , to pick up stone formation at an early stage , may help reduce the burden of these problems . ( 2 - 4 ) predisposing factors to bladder stone formation include urinary stasis , bladder outflow obstruction urinary stasis , recurrent urinary tract infections and foreign bodies within the bladder ( 1 ) , all of which can be seen in spina bifida patients . in our case the added sensory abnormalities secondary to spina bifida left the bladder calculus undiagnosed until it had formed the vesicocutaneous fistula . good control of the predisposing factors can dramatically reduce the risk of developing bladder calculi , but especially in cases where this is difficult , a high index of suspicion , regular urological review and timely uss can prevent the development of both the bladder calculus and its complications .

we present a rare case of a vesicocutaneous fistula secondary to two large bladder calculi in a patient with spina bifida . a 62 year old lady presented with swelling of her right thigh and a calcified mass protruding through the subcutaneous tissues of her right groin . urine was seen to be draining from around the area and plain radiographs and a computed topography ( ct ) scan confirmed the presence of two bladder calculi with a vesicocutaneous fistula . vesicolithotomy was performed and the fistula tract left to close spontaneously . worldwide this is only the fourth case of a bladder calculus causing a vesicocutaneous fistula .

pityriasis amiantacea ( pa ) is a scalp disorder presenting with thick , silvery / yellowish , asbestos - like scales wrapping around and binding down tufts of hair . it is typically considered to be a reactive condition to several inflammatory diseases , which may affect the scalp , mainly including psoriasis , atopic dermatitis and seborrheic dermatitis . however , although rarely , pa may be the presenting clinical pattern of dermatophyte infection ( pa - like tinea capitis ) , thus emphasizing the importance of ruling out / confirming the possibility of tinea capitis when dealing with a case of pa we here describe for the first time the usefulness of dermoscopy as a supportive diagnostic tool in an instance of pa - like tinea capitis . a 9-year - old girl presented with a five - week history of a progressively worsening , asymptomatic , scaling patch on the scalp . her past medical history was unremarkable and there was no personal or family history of atopic diathesis and skin diseases . physical examination showed thick , adherent , whitish , asbestos - like scales on the right parietal region of the scalp , surrounding and binding the hair ( figure 1a ) ; no other significant skin , nail or mucosal finding was evident . dermoscopic examination ( performed with dermlite dl3 x10 ; 3gen , san juan capistrano , ca , usa ) revealed diffuse white scales and compact white keratotic material adhering to tufts of hair ( asbestos - like scaling ) without erythema ( figure 1b ) ; interestingly , several hairs displayed a question mark or ( hair shaft bent at more than one point ) appearance ( figure 1b ) . as such dermoscopic findings may be found in tinea capitis [ 37 ] , we decided to carry out a direct microscopic examination of 10% koh preparation of the scales scraped from the scalp , which showed septate branching hyphae . specimens were also cultured on conventional sabouraud s dextrose agar medium , with evidence of microsporum canis growth after three weeks , thus confirming the diagnosis of tinea capitis . the patient was treated with oral griseofulvin ( 15 mg / kg once daily ) with complete resolution of the clinical picture after eight weeks of therapy . clinical distinction between pa due to inflammatory noninfectious diseases and pa - like tinea capitis is a challenging task , with a significant likelihood of diagnostic errors / delays and prescription of inappropriate therapies . over the last few years , dermoscopy has been showed to be a useful auxiliary instrument for the recognition of several hair disorders [ 89 ] , particularly dermatophyte infections [ 37 ] . indeed , beside nonspecific findings ( broken and dystrophic hairs , black dots , scaling , erythema , etc . ) , tinea capitis may display peculiar dermoscopic features such as comma hairs , corkscrew hairs , zigzag hairs , interrupted ( morse code - like ) hairs , elbow - shaped hairs and question mark hairs [ 37 ] . regarding the present instance , even though we observed dermoscopic findings that may be commonly seen in pa , i.e. , diffuse white scaling and the characteristic compact white keratotic material adhering to a tuft of hair ( asbestos - like scales ) , dermoscopy turned out to be very helpful in suspecting tinea infection by showing the aforementioned zigzag and question mark hairs . in fact , although such features ( or similar findings ) may be rarely found in other hair disorders ( e.g. , zigzag hairs in trichorrhexis nodosa , alopecia areata and monilethrix , and question mark hairs in alopecia areata ) [ 37 ] , they are typically not visible in classic pa . in conclusion , even though the final diagnosis relies on mycological testing , dermoscopy might come in very handy for raising the suspicion of tinea capitis presenting with a pa - like appearance by showing peculiar dermoscopic findings ( e.g. , as shown in this case , zigzag and question mark hairs ) , which are typically not detectable in instances of pa due to inflammatory diseases . obviously , further studies on larger groups of patients are needed to confirm our observations .

clinical distinction between pityriasis amiantacea - like tinea capitis and pityriasis amiantacea due to noninfectious inflammatory diseases is a troublesome task , with a significant likelihood of diagnostic errors / delays and prescription of inappropriate therapies . we report a case of pityriasis amiantacea - like tinea capitis with its dermoscopic findings in order to highlight the usefulness of dermoscopy in improving the recognition of such a condition .

wild type cd1 embryos were harvested , staged and stained by wholemount in situ hybridization using established methods , . measurement of palates before dissection and after staining confirmed that no significant shrinkage occurred ( data not shown ) . palatal explants were cultured ( 37c , 5% co2 ) using the trowell technique in dmem ( sigma ) , 20 u / ml pen - strep ( gibcobrl ) , 10% fbs ( gibcobrl ) , 50 mm transferrin ( sigma ) and 150 g / ml ascorbic acid ( sigma ) . for cutting and inhibitor experiments , serum - free advanced d - mem / f12 ( gibcobrl ) supplemented as above , was used . su5402 ( calbiochem ) was diluted in medium from 10 mm in dmso stock ; cyclopamine ( sigma ) from 20 mg / ml - ethanol stock . explants placed in a minimum volume of pbs in wells cut into 1% agarose were digitally imaged under a stereo dissecting microscope and measurements made using the ruler in imagej ( from the nih imagej website ) calibrated with a micrometer slide . dimensions of fixed material were within 8% of those of fresh , unfixed material ( data not shown ) . simulations of reaction - diffusion patterning were performed using the javascript in ref with the parameters du=0.03 , d u=0.02 , a u=0.1 , b u=0.06 , c u=0 , fmax=0.2 , dv= 0.06 , d v=0.5 , a v=0.1 , b v=+0 , c v=0.2 , gmax=0.5 . wild type cd1 embryos were harvested , staged and stained by wholemount in situ hybridization using established methods , . measurement of palates before dissection and after staining confirmed that no significant shrinkage occurred ( data not shown ) . palatal explants were cultured ( 37c , 5% co2 ) using the trowell technique in dmem ( sigma ) , 20 u / ml pen - strep ( gibcobrl ) , 10% fbs ( gibcobrl ) , 50 mm transferrin ( sigma ) and 150 g / ml ascorbic acid ( sigma ) . for cutting and inhibitor experiments , serum - free advanced d - mem / f12 ( gibcobrl ) supplemented as above , su5402 ( calbiochem ) was diluted in medium from 10 mm in dmso stock ; cyclopamine ( sigma ) from 20 mg / ml - ethanol stock . explants placed in a minimum volume of pbs in wells cut into 1% agarose were digitally imaged under a stereo dissecting microscope and measurements made using the ruler in imagej ( from the nih imagej website ) calibrated with a micrometer slide . dimensions of fixed material were within 8% of those of fresh , unfixed material ( data not shown ) . simulations of reaction - diffusion patterning were performed using the javascript in ref with the parameters du=0.03 , d u=0.02 , a u=0.1 , b u=0.06 , c u=0 , fmax=0.2 , dv= 0.06 , d v=0.5 , a v=0.1 , b v=+0 , c v=0.2 , gmax=0.5 .

we present direct evidence of an activator - inhibitor system in the generation of the regularly spaced transverse ridges of the palate . we show that new ridges , or rugae , marked by stripes of sonic hedgehog ( shh ) expression , appear at two growth zones where the space between previously laid - down rugae increases . however , inter - rugal growth is not absolutely required : new stripes still appear when growth is inhibited . furthermore , when a ruga is excised new shh expression appears , not at the cut edge but as bifurcating stripes branching from the neighbouring shh stripe , diagnostic of a turing - type reaction - diffusion mechanism . genetic and inhibitor experiments identify fibroblast growth factor ( fgf ) and shh as an activator - inhibitor pair in this system . these findings demonstrate a reaction - diffusion mechanism likely to be widely relevant in vertebrate development .

acute neurogenic pulmonary edema ( npe ) is an underdiagnosed yet a common clinical entity . presence of preoperative npe presents a dilemma to the neuroanesthetist due to the divergent goals of management of raised intracranial pressure and pulmonary edema and also the possible adverse interaction of the two conditions when they co - exist . we report a patient with acute obstructive hydrocephalus due to cerebellar metastatic lesion who presented with npe that resolved on placement of the ventriculoperitonial ( vp ) shunt . a woman , about 50year old , presented with progressive headache , holocranial and continuous type , associated with multiple episodes of vomiting and swaying gait of two months duration . her magnetic resonance imaging ( mri ) brain revealed fourth ventricular obstruction with obstructive hydrocephalus . as the patient was drowsy though responding to verbal commands and oriented , an emergency vp shunt was planned . she had an unremarkable past history with no previous history of tuberculosis or respiratory illness . she had a pulse rate of 65 per minute and her blood pressure was normal . chest x - ray showed slight haziness in the right lung suggestive of pulmonary edema- [ figure 1 ] . the peripheral oxygen saturation ( spo2 ) was around 84% and the arterial blood gases showed a pao2 53 mmhg . the spo2 increased to 92% on administration of 100% oxygen through the face mask of the anesthetic circuit . as there was no other cause for the respiratory dysfunction such as infection , aspiration , or previous respiratory illness , a diagnosis of npe was considered . anesthesia was induced with propofol 2 mg / kg and oral endotracheal intubation was facilitated with vecuronium 0.1 mg / kg body weight . anesthesia was maintained with air and oxygen mixture , adjusting the fio2 to maintain an arterial saturation of > 90% , and 1% isoflurane along with atracurium and fentanyl infusions . patient remained hemodynamically stable and mean arterial blood pressure ( map ) was maintained at 80 mmhg . after the cerebrospinal fluid ( csf ) drainage spo2 gradually increased to 100% and the fio2 could be reduced to 0.5.the neuromuscular blockade was reversed at the conclusion of surgery . the patient was awake , responding to verbal commands with normal motor power and tone . postoperatively , the pao2 was 253 mmhg on oxygen supplementation with a face mask delivering a fio2 of 0.4 . preoperative chest radiograph of the patient showing pulmonary infiltrates suggestive of pulmonary edema immediate postoperative chest radiograph of the patient showing resolution of pulmonary infiltrates npe may be a consequence of a number of diverse central nervous system insults , including head trauma , brain stem lesions , rupture of intracranial aneurysm , excessive irrigation during endoscopic ventriculostomy , during angioplasty for vasospasm , and postictal period . diagnosis requires a high index of suspicion , especially in the case of respiratory decompensation in neurosurgical patients . the pathogenesis of npe probably involves overactivation of the sympathetic autonomic system with pulmonary hypertension , endothelial dysfunction , and increased vessel permeability . there are two theories on how it occurs : the blast theory and the permeability defect theory , with evidence in favor of both of them . the treatment is mainly supportive using mechanical ventilation and alpha - adrenergic blocking agents for managing increased pulmonary arterial pressure . it has been hypothesized that deep levels of anesthesia might protect against the development of npe due to a more pronounced inhibition of the hypothalamic , brainstem , and spinal vasoactive sympathetic centers . an insufficient anesthesia level may not be able to inhibit the sympathetic nervous system during an injury of the central nervous system and thus predispose to development of npe . therefore , maintenance of adequate depth of anesthesia and attenuation of neuroendocrine response to intubation are important . the use of peep in neurosurgical patients is limited by conflicting reports on its effect on intracranial pressure . the presence of raised intracranial pressure and the need to provide good brain relaxation for surgery may limit the application of peep . a high fio2 was sufficient to maintain optimal blood gases without the necessity for peep in this patient . the reduced lung compliance and high intrathoracic pressure during mechanical ventilation in the presence of pulmonary edema may also pose a problem for providing brain relaxation . the cerebrogenic autonomic and neurohumoral dysregulation due to intracranial hypertension may cause intraoperative hemodynamic dysfunction . a thorough understanding of the patho - physiological mechanisms behind the development of npe helps in the management of these patients , thus preventing further complications . npe after aneurismal sub - arachnoid haemorrage was shown to resolve after endovascular coiling . and npe in this patient resolved after ventriculoperitonial shunt . in conclusion , in patients with central nervous system pathology in respiratory distress , the possibility of diagnosis of npe must be considered and the inciting pathology should be deliberated . anesthetic management must be carefully titrated considering the divergent goals of npe and intracranial hypertension .

neurogenic pulmonary edema may be a less - recognized consequence of obstructive hydrocephalus . the authors report a patient with acute obstructive hydrocephalus due to cerebellar metastatic lesion , who presented with neurogenic pulmonary edema . the edema resolved on placement of the ventriculoperitonial shunt . this report addresses the importance of recognition of neurogenic pulmonary edema as a possible perioperative complication resulting from an increase in intracranial pressure and the issues involved with anesthetic management of co - existing neurogenic pulmonary edema and intracranial hypertension .

we report a 50-year - old female patient with a stage 2 idiopathic macular hole that closed spontaneously . the stage 2 idiopathic macular hole closed spontaneously in 6 weeks with a lamellar defect in the outer retina due to the formation of the bridging retinal tissue , but without any evidence of the common mechanisms of spontaneous closure such as posterior vitreous detachment or epiretinal membrane formation . an idiopathic macular hole was identified as a unique clinical entity more than 100 years ago . most macular holes occur as an age - related primary idiopathic condition , unrelated to other ocular problems or antecedent events . the hallmark inciting event of an idiopathic macular hole formation gass [ 3 , 4 ] classified macular holes into 4 stages . stage 1 , also known as impending macular holes, stages 24 include full - thickness macular holes , which are further divided into smaller holes ( < 400 m in diameter ( stage 2 ) ) , holes larger than 400 m in diameter ( stage 3 ) and with a complete posterior vitreous detachment ( stage 4 ) . it has been clinically established that stage 1 , impending macular holes , have a 50% chance for spontaneous closure with the resolution of symptoms . however , spontaneous resolution with hole closure and a restoration of the normal foveal contour is very rare in full - thickness macular holes ( stages 24 ) . it occurs in 24% of the eyes [ 6 , 7 ] ; therefore , these cases are usually treated surgically by pars plana vitrectomy , with or without internal limiting of the membrane peeling . we report a 50-year - old female patient with a stage 2 idiopathic macular hole that closed spontaneously . she also had a lamellar defect in the outer retina due to the formation of the bridging retinal tissue , but without any evidence of the common mechanisms of spontaneous closure such as posterior vitreous detachment or epiretinal membrane formation . the patient presented to us in may 2013 with a right eye vision diminution , which started 1 month ago . on examination , the best - corrected visual acuity was 20/125 in the right eye and 20/30 in the left eye . slit lamp examination revealed a clear cornea and a pupil that was normal in size and reacting to light ; early cataract in both eyes was also detected . the posterior segment showed the presence of a full thickness centric macular hole ( stage 2 ) in the right eye . further examination with the amsler grid test demonstrated central metamorphopsia in the right eye . in the other eye , the fundus , the disc and the retinal vasculature appeared healthy ; the macula was within normal limits . optical coherence tomography ( oct ) was performed for the right eye , which revealed a full - thickness macular hole ( stage 2 ) with cystic spaces suggesting an accumulation of secondary vitreous fluid and no posterior vitreous detachment ( fig . 1 ) . various treatment options , including conservative management , were discussed with the patient . the best corrected visual acuity was checked and was 20/40 on the snellens chart for the right eye and 20/30 for the left eye . fundus examination of the right eye revealed no evidence of a macular hole , and the watzke - allen test was negative . oct revealed the closure of the macular hole with the resolution of the cystic spaces and bridging of the inner retinal layers with lamellar defects without any posterior vitreous detachment or glial tissue proliferation ( fig . the follow - up after 12 weeks showed persistent outer lamellar defects on the oct ; visual acuity improved marginally to 20/30 ( fig . in our patient , macular hole closure was confirmed by oct examination , showing a sensory retina bridging over the former area of the macular hole with small outer layer defects . the bridging of the retinal tissue allowed the resolution of the cystoid spaces by preventing the influx of vitreous fluid into intraretinal spaces and therefore leading to a spontaneous closure of the macular hole . four explanations have been proposed for the spontaneous resolution of a macular hole : ( 1 ) complete detachment of the posterior hyaloid from the foveal area leading to a release of traction ; ( 2 ) cell proliferation at the base of the hole ; ( 3 ) formation of a contractile epiretinal membrane resulting in shrinkage and closure of the hole , and ( 4 ) bridging of the retinal tissue across the hole . out of the 4 mechanisms mentioned above , posterior vitreous detachment and epiretinal membrane formation may or may not always be evident in patients with spontaneous closure of a macular hole , but the bridging of the sensory retina and the smaller size of the macular hole appear to be the most consistently reported findings for the spontaneous closure of macular holes [ 12 , 13 ] . as to the origin of the bridging of the retinal tissue , proliferation of the retinal postmitotic neurosensory cells has been proposed , but could not be identified ; proliferation of glial or retinal pigment epithelial cells has also been suggested . since the concept of cell proliferation as a mechanism of macular hole closure is still speculative , the exact mechanism of how the spontaneous macular hole closure with maintained normal retinal structure occurs is still unclear . none of the authors have financial or proprietary interests in any material or method mentioned .

objectivewe report a 50-year - old female patient with a stage 2 idiopathic macular hole that closed spontaneously.methodthe case is presented on the basis of an observational case report.resultsthe stage 2 idiopathic macular hole closed spontaneously in 6 weeks with a lamellar defect in the outer retina due to the formation of the bridging retinal tissue , but without any evidence of the common mechanisms of spontaneous closure such as posterior vitreous detachment or epiretinal membrane formation .

arteriovenous malformations ( avms ) present with hemorrhage , seizures , headache or focal neurological deficits . cysts associated with avms are uncommon , and are usually a sequelae of stereotactic radiosurgery or after a hemorrhage . untreated and unruptured avms with large cysts are uncommon , with only five cases reported in literature , and one in the pediatric population . we describe a case of an untreated and unruptured avm in the right parietal lobe , which was associated with a large cyst . an 8-year - old boy presented with history of generalized tonic clonic seizures for the last 2 years . there was no history of sudden onset headache or neurological deficits , suggestive of bleed . on examination , there were no focal neurological deficits . an mri was performed , which showed a nidus , seen as flow voids , in the medial parietal lobe on the right side with a large cystic area abutting it posteriorly . this was hyperintense on t2-weighted images and inverted completely on flair images [ figure 1a and b ] . a digital subtraction angiogram was subsequently performed , which showed the nidus was receiving arterial feeders from the pericallosal branches of the right anterior cerebral artery and draining into the superior sagittal sinus via cortical veins [ figure 1c and d ] . ( a and b ) sagittal t2-weighted ( a ) and axial flair ( b ) mr images showing the avm nidus anteriorly and medially with the large cystic component posteriorly located in the right medial parietal lobe . ( c and d ) digital subtraction angiogram images in ap and lateral planes showing the nidus receiving arterial feeders from the pericallosal branches of the right anterior cerebral artery . ( e and f ) post - operative angiograms in ap and lateral planes showing complete excision with non - visualization of the nidus on right ica injection various treatment options were discussed , and the patient was offered surgical excision of the avm as the first choice . the avm nidus and the draining vein was seen on the surface , and below that , there was a large cyst containing about 30 ml of clear fluid . histological examination of the nidus showed abnormal arterial and venous channels , and the cyst wall had numerous thin - walled vascular channels containing hemosiderin in the fibrous tissue . a post - operative angiogram was done on post - operative day two , which showed complete excision of the nidus [ figure 1e and f ] . his post - operative stay in the hospital was uneventful , and was discharged on the third post - operative day . at 6-month follow - up , patient is neurologically normal , and is seizure - free on a single anti - epileptic drug . avms associated with cyst without undergoing radiosurgery are extremely rare . in previously reported literature of 5 cases , there were 2 females and 3 males , with age between 14 and 38 years . our patient is the youngest case of this type of association , and is only the second patient in pediatric age group reported in the literature [ table 1 ] . three of the patients presented with seizures , 1 with headache and 1 with a focal neurological deficit in the form of a superior quadrantanopia . in 4 of the 5 patients , the avms were located in the temporal lobe and 1 was located in the parietal lobe . cysts accompanying avms have been attributed to cavity formation secondary to liquefaction of the hematoma , which forms due to the bleeding in the avm . the cases reported in literature had no history to suggest any previous episode of hemorrhage , and neither had received stereotactic radio surgery . in our case as well , there was no history suggestive of bleed or previous radiation treatment . multiple hemorrhages in a long period and exudation of fluid from part of the avm have been suggested as the etiology for cyst formation . reported the histology of the cyst wall and showed numerous thin - walled vascular channels and deposits in fibrous tissue , which are also seen in the membrane of the giant cysts associated with cavernous angiomas . hence , they suggest that a capsule forms around the minor hemorrhage from the avm resulting in the cyst and repeated subclinical hemorrhages from the neovascular channels of the cyst wall result in the growth of the cyst . itakura et al . , have propounded the theory of gradual exudation of fluid into the surrounding parenchyma resulting in cyst formation . in the present patient , the fluid in the cyst was clear , without any xanthochromia or hemorrhage , and the characteristic of the fluid was similar to csf on imaging . the histology too showed no evidence of hemorrhage or slit like cavities in the cyst wall . hence , we are in concurrence with this theory of gradual exudation of fluid causing the cyst associated with the avm . these can occur secondary to microhemorrhages or exudation of fluid from the part of the avm .

cysts associated with arteriovenous malformations ( avms ) are either secondary to hemorrhage or after radiosurgery . untreated and unruptured avms with large cysts are rare . we here describe a child with medial parietal avm associated with cyst , without any history of hemorrhage or radiosurgery . surgical excision led to cure for the patient .

in december 2011 , a 51-year - old woman was admitted to our hospital due to massive hemoptysis . she had suffered from recurrent hemoptysis for five years and had undergone bronchial artery embolization many times . the patient s vital signs were stable . on chest examination , decreased breathing sound and crackle were audible in the right lower lung field . a chest x - ray showed patchy consolidation in the right lower lung ( fig . 1a ) . a computed tomography scan of the chest showed a small nodule , ground glass appearance , and consolidation around the nodule in the lateral basal segment of the right lower lobe ( fig we performed an operation for the differential diagnosis between the possibility of a malignancy or life - threatening , massive hemoptysis . under general anesthesia with a double lumen endotracheal tube , we resected the right lower lobe through a posterolateral thoracotomy at the sixth intercostal space . in the operation field , we could palpate a round , hard mass 2.03.0 cm in the lateral basal segment of the right lower lobe . the resected specimen consisted of the right lower lobe , weighed 177.5 g , and was sized 11.511.23.0 cm . on multiple serial sections , a cavity lesion measuring approximately 2.02.5 cm was identified ( fig . histopathological examination confirmed it to be a vegetable foreign body , and clumps of actinomyces , indicating actinomycosis , were present within the abscess cavity ( fig . actinomycosis is a chronic , suppurative pulmonary infection usually caused by actinomyces israelii , which is present in the oropharynx of humans . pulmonary infection with species of actinomyces is uncommon , and usually results from aspiration of oropharyngeal secretions in those with chronic dental infections , extension from a cervicofacial infection , or hematogenous spread from a distant source . bronchial involvement , a rare form of thoracic actinomycosis , has been reported to be associated with foreign bodies . the first report of endobronchial pulmonary actinomycosis induced by a foreign body was the case of a spanish patient in 1991 , when a chicken bone was aspirated . according to chouabe et al . , eleven cases of pulmonary actinomycosis secondary to endobronchial foreign body aspiration have been described . most cases have occurred in middle - aged males with known risk factors such as a chronic debilitated state and poor dentition . the main symptoms include cough , fever , expectoration of yellow pus , chest pain , and weight loss . chest computed tomography shows a thickened bronchial wall , dense pulmonary alveolar opacity , atelectasis , pleural effusion , bronchiectasis , lymphadenopathy , or a radiopaque foreign body . obstructive endoluminal masses have been found on bronchoscopy , but the actual foreign bodies are not easily detected in some cases . in the present case , usually the foreign body is likely to have become a host for subsequent actinomyces contamination once the patient had developed poor dentition . mucosal breaches secondary to foreign body impaction must occur for actinomyces species to colonize and cause a marked granulomatous inflammatory reaction . we could find only one case of pulmonary actinomycosis induced by a vegetable foreign body . our case represents a very rare pathogenesis of pulmonary actinomycosis , which is an important disease in the differential diagnosis of a pulmonary mass .

a 51-year - old woman visited our hospital with massive hemoptysis . she had suffered from recurrent hemoptysis for five years and had undergone bronchial artery embolization many times . the patient had a history of pulmonary tuberculosis and bronchiectasis . chest radiography showed consolidation around the nodule in the lateral basal segment of the right lower lobe . we successfully performed a right lower lobectomy . the histological study of the resected specimen showed a vegetable foreign body and clumps of actinomyces , indicating actinomycosis , which was suggested to be the cause of the hemoptysis . this was a very rare case of hemoptysis caused by a vegetable foreign body and actinomycosis .

urban taxi drivers differ from other professional drivers with respect to their risk profiles for work - related low back disorders1 . biomechanical studies show that the driving activities within automobiles can impose postural strains on lumbar spines1 . reported that back pain is an important health problem for taxi drivers , as well as an urgent occupational safety and health management issue2 . back pain is significantly related to the suitability of the driver s seat pan , job stress , and time2 . the current study developed unilateral exercises for urban drivers and investigated the effect of these exercises on low back pain ( lbp ) . a 40-year - old male , who complained of lbp on the left side at l35 levels , participated in the study . the objective and methods of the study were explained to the subject prior to his participation . informed consent was also obtained according to the ethical principles of the declaration of helsinki . he had been driving a taxi for 4 h / day in an urban area . an examination revealed that his pelvis was tilted posteriorly . when he performed a forward flexion in the standing position with his knees fully extended , he experienced pain and stiffness in his left lower back . the visual analog scale ( vas ) score of his back pain was 7 . an examiner measured the pelvic inclination with a palpation meter ( palm ; performance attainment associates , st . the rotation of the innominate bones in the sagittal plane was also measured with the caliper tips of the palm in contact with the ipsilateral anterior superior iliac spine and posterior superior iliac spine3 . in the initial assessment , the anterior pelvic tilt angles were 2 and 8 on the right and left sides ( normal range , 11 4 ) , respectively . a dual inclinometer ( acumar , lafayette instrument co. , lafayette , usa ) was used to measure the trunk angles . the initial lumbar flexion angle was 60 , and the initial extension angle was 45. an examination revealed that his lumbar was rotated to the left side . this study conducted two 10-day sessions and measured the lbp , pelvic tilt angle , and trunk angle after each session . session 1 involved lumbar extension and flexion exercises , as well as lumbar stability exercises using a ball . session 2 comprised unilateral resistance exercises , which include pushing and holding for 10 s with the left foot on a 45-degree inclined springboard while sitting on a chair with the knee flexed . the unilateral stretching exercise for the quadratus lumborum ( ql ) muscles was performed by crossing the right leg over the left leg and slowly lowering both legs to the right ; this position was held for 30 s with the arms extended out to the sides of the body . after the first session , the anterior pelvic tilt angles were 4 and 8 on the right and left sides , respectively . the lumbar flexion angle was 68 , and the extension angle was 45. lumbar rotation movement was observed during lumbar flexion . after the second session , the anterior pelvic tilt angles were 9 and 10 on the right and left sides , respectively . the lumbar flexion angle was 68 , and extension angle was 50. lumbar rotation movement was not observed during flexion . lumbar extension and flexion and lumbar stability exercises that are generally recommended for people suffering from lumbago are also helpful for normal pelvic alignment , as well as in reducing lbp among urban drivers . however , the effects of these exercises are limited . with the driver s seat on the left side in korean cars , the accelerator is located near the right foot , and the brake pedal is located between the right and left feet . urban drivers spend a considerable amount of time on operating the brake pedal while driving around the busy roads of cities1 , 2 . extending and pushing on the right leg to operate the brake pedal between the right and left feet increase the length of the right leg ; this operation may have caused the right lateral tilt in the right side of the pelvis and one - sided backward tilt of the right pelvis4 , 5 . therefore , resistance exercises with the left foot pushing a decline board , which was performed by the subject in the current study , were considered to reduce greatly the differences in the angles of the right and left sides of the pelvis as well as the pain therein . moreover , while the right foot moves repeatedly back and forth between the accelerator and the brake pedal , the muscles around the left side of the pelvis stiffen4 . in particular , the ql muscles of the waist move in a compensatory manner to maintain the pelvic posture in the sitting position5 . the unilateral stretching exercise for the ql muscles performed in the current study is considered to reduce lumbago by improving the right lateral tilt in the right side of the pelvis and reducing the tension of the ql muscles . in conclusion , analyzing car features and performing individual approaches are necessary in providing treatment for urban drivers with lbp .

[ purpose ] this study aimed to develop unilateral exercises for urban drivers and investigate the effect of these exercises on low back pain ( lbp ) . [ subject and methods ] a 40-year - old male driver , who complained of lbp on the left side at l35 levels , participated in this study . a two - session program was conducted , and lbp , pelvic tilt angle , and trunk range of motion were measured after each session . [ results ] after the unilateral exercises , the anterior pelvic tilt angle was improved and the visual analog scale score of back pain decreased . [ conclusion ] analyzing car features and performing individual approaches are necessary in providing treatment for urban drivers with lbp .

lichen planus ( lp ) is a common inflammatory disease affecting the skin , the mucous membranes , the genitalia , the nails and the scalp . prevalence of lichen planus in the general population ranges from 0.1 to 4 % and it is more common in females , especially in the perimenopausal period . pathophysiology of lp involves an immune - mediated reaction , in which an antigen is processed to t - lymphocytes and they , subsequently , attack basal keratinocytes , leading to apoptosis of the cells . several factors have been suggested as possible antigens , including viruses , bacterials and drugs . the typical clinical manifestations of lp are purple to violaceous polygonal papules with sharp borders , usually pruritic , most commonly developing on the extremities and the trunk . less frequently the disease affects the genital area , mucous membranes , palms and soles and nails . mucosal lesions are typified by the presence of reticular white lines , known as wickham striae . the disorder has several clinical variations : annular , hypertrophic , atrophic , ulcerative , bullous , erythrodermic , inverse , linear , follicular , pemphigoides , pigmentosus , follicularis decalvans and actinic lp . the diagnosis of lp is usually established clinically based on the characteristic morphology of the lesions and the coexisting intense pruritus . however , atypical presentations requiring histopathologic confirmation of the diagnosis do exist . dermoscopy allows the visualization of structures located in the epidermis , dermo - epidermal junction and papillary dermis that can not be seen with the naked eye . , cumulative evidence suggests that dermoscopy is also meaningful for the evaluation of inflammatory and infectious skin disorders . in the field of papulosquamous dermatoses , dermoscopy has been shown to enhance the differential diagnosis among psoriasis , dermatitis , lp and pityriasis rosea . particularly for lp , dermoscopy brought to light that white crossing lines do not characterize only mucosal lesions , but cover virtually every cutaneous papule of active lp . in this report we present a characteristic example of a patient with misleading clinical manifestations of lp resembling psoriasis . a 61-year - old woman visited our department for evaluation of a three - month , mildly pruritic eruption on the soles and the dorsal surfaces of the feet and hands . clinical examination revealed hyperkeratotic plaques on the dorsal surface of the feet and hands and erythematous hyperkeratotic , partially erosive plaques on the soles . . surprisingly , application of dermoscopy did not reveal the expected psoriatic pattern of regularly distributed dotted vessels and white scales ( figure 2 ) . instead , white crossing lines ( the so - called wickham striae ) were dermoscopically evident , along with dotted and short linear vessels and yellow scales . since the dermoscopic presence of wickham striae is considered highly specific of lp , the dermoscopic findings prompted us to perform a biopsy for histopathologic assessment . histopathology , as shown in figure 3 , revealed hyperkeratosis , dense hypergranulosis , vacuolar degeneration of basal cell keratinocytes , band - like lymphocytic infiltration in the upper dermis , as well as presence of colloid bodies , justifying the diagnosis of lp . in the current case , clinical manifestations on the dorsal hands and plantar surfaces were highly suggestive of psoriasis , with eczema and lp included in the differential diagnosis . this was because the lesion deviated from the standard dermoscopic pattern of psoriatic lesions , which are composed of regularly distributed dotted vessels and white - colored scales . although the presence of irregularly arranged dotted vessels and yellow scales were compatible with eczema , the prevailing dermoscopic features were the white crossing lines , corresponding to the so - called wickham striae , which is known as a highly specific criterion of lp . clinical examination is undoubtedly the cornerstone of diagnosis in everyday dermatology practice , and in the majority of our patients , the macroscopic morphology is already enough to establish an accurate diagnosis . this is especially true for widespread inflammatory diseases , where the combination of clinical history , morphology and distribution often points towards a specific diagnosis . however , equivocal clinical manifestations do exist in everyday practice , posing diagnostic doubts and often prompting clinicians to perform diagnostic biopsies . it has been demonstrated that coupling clinical examination with dermoscopy significantly improves the diagnostic performance of clinicians . however , in order to maximize the benefit from dermoscopy in differentiation of inflammatory dermatoses , clinicians have to virtually use their dermatoscope on every lesion . in daily routine furthermore , as shown in the current case , it may change our diagnostic thoughts , saving us from misdiagnosis and potential inappropriate management .

lichen planus ( lp ) is an inflammatory disease that affects the skin mainly the extremities and the trunk the mucous membranes , the genitalia , the nails and the scalp . the diagnosis of lp is usually established clinically based on the typical morphology and distribution of the lesions in conjunction with the associated itch . we report a patient with lp manifesting highly psoriasiform lesions , that could only be correctly assessed after the application of dermoscopy , which revealed lp - specific findings .

it is more common in female with female - to - male ratio of 3:1 . not all the cases of necrobiosis are associated with diabetes ; hence the terminology necrobiosis lipoidica diabeticorum has been abandoned . the ulcers in nl are quite painful leading to impaired quality of life of these patients . we present a case of ulcerative nl , which failed to respond to traditional treatment modalities but responded quickly to thalidomide . a 58-year - old woman with 15 years history of insulin - dependent diabetes presented with nonhealing ulcers on her shin since 5 years . four months later , she noticed a pinhead sized lesion over the red area , which enlarged and ulcerated . three similar ulcers [ figure 1a and b ] appeared on the shin over the next one month . the ulcers were non healing and extremely painful but not associated with any constitutional symptoms . local wound care , topical corticosteroids , topical calcineurin inhibitors were tried along with oral antibiotics , dapsone , pentoxyphylline , and analgesics , without much relief . she had multiple discrete tender ulcers over a waxy , erythematous , atrophic plaque on the anterior aspect of her left leg , variying in size from 3 2 cm to 1 1 cm . the borders of ulcer were undermined , base was indurated , and floor was covered with necrotic debris . her hemogram , renal and liver function tests , and venous doppler test results were normal . pus culture grew staphylococcus aureus sensitive to linezolid , amoxycillin and clavulanic acid , and tazobactam piperacillin . skin biopsy from the edge of the ulcer revealed a hyperplastic , sclerotic epidermis and a dense superficial and deep dermal infiltrate of lymphocyte and plasma cells concentrated around the blood vessels and sweat glands [ figure 2a - c ] . we started the patient on oral linezolid 600 mg twice daily for 7 days and chloroquine 250 mg once daily for 4 weeks along with local dressings . topical human recombinant epidermal growth factor , oral clopidrogel and aspirin were added to the above regime and continued for another 2 months , with no improvement . pain reduced dramatically within 2 weeks and the ulcers healed completely after 4 weeks [ figures 3a and b ] . ( a and b ) multiple non healing ulcers over the shin ( a - c ) hyperplastic , sclerotic epidermis and a dense superficial and deep dermal infiltrate of lymphocyte and plasma cells concentrated around the blood vessels and sweat glands with several granulomas arranged in a horizontal manner in mid and lower dermis . ( h and e , 10 , 40 , 40 ) ( a and b ) complete healing and softening of skin after treatment with thalidomide there is no consistently effective therapy , and lack of uniform guidelines make treatment more challenging . many theories were put forward to explain the pathogenesis of nl : diabetic microangiopathy due to deposition of glycoprotein in the blood vessel wall could lead to impaired blood supply to the skin ; greater cross - linking of the collagen fibres in nl could lead to thickening of the basement membrane zone ; immune complex deposition in the dermal blood vessel walls could lead to vasculitis ; recently a role of disturbance in glucose transport by fibroblasts has been postulated . glut-1 is the human erythrocyte glucose transporter , which mediates facilitative transport of glucose across epithelial and endothelial barrier tissues . this protein was expressed in the sclerotic collagen of nl patients , indicating insulin resistance in these tissues . these include cutaneous blood flow enhancers , such as dypyridamol , clopidrogel , aspirin , pentoxyphylline ; topical and intralesional steroids , and topical calcineurin inhibitors ; wound healing enhancers such as epidermal growth factors , platelet - derived growth factors , collagen gel , hyperbaric oxygen ; surgery and pulse dye laser ; and immune modulators such as antimalarials , cyclosporine , and biologics . our patient was treated with various drugs but failed to respond . in several case studies , tnf - alpha inhibitors such as etanercept , adalimumab , and infliximab were shown to improve ulcerated nld . tnf - alpha has been found in high concentrations in the sera and skin of patients with nl thalidomide acts as an anti - inflammatory agent by suppressing tnf - alpha via degradation of its messenger rna and by decreasing the ratio of helper t cells to suppressor t cells . the ulcer healed rapidly and thalidomide was withdrawn over 12 weeks , with no relapse till date .

ulcerative necrobiosis lipoidica ( nl ) in diabetic patients is a rare , painful condition . it is a difficult - to - treat condition , impairing quality of life of patients . although various drugs have been tried , none of them is consistently effective . biologics in the form of tnf - alpha inhibitors show promising results in the treatment . but because of their high cost we chose thalidomide , which also has tnf - alpha inhibiting properties to successfully treat a long - standing case of ulcerative nl , which was refractory to various treatment modalities .

however , variations in the facial nerve have been reported and they increase the risk of facial nerve injury during parotid surgery . herein , we report a unique case of a parotid tumor in conjunction with a facial nerve anomaly . key pointswe report a new variation in the facial nerve pattern , and an unusual relationship with the retromandibular vein during parotid surgery.clinicians should recognize this facial anomaly and the unusual relationship with the retromandibular vein to avoid injuring the facial nerve during parotid surgery . we report a new variation in the facial nerve pattern , and an unusual relationship with the retromandibular vein during parotid surgery.clinicians should recognize this facial anomaly and the unusual relationship with the retromandibular vein to avoid injuring the facial nerve during parotid surgery . we report a new variation in the facial nerve pattern , and an unusual relationship with the retromandibular vein during parotid surgery . clinicians should recognize this facial anomaly and the unusual relationship with the retromandibular vein to avoid injuring the facial nerve during parotid surgery . we report a new variation in the facial nerve pattern , and an unusual relationship with the retromandibular vein during parotid surgery.clinicians should recognize this facial anomaly and the unusual relationship with the retromandibular vein to avoid injuring the facial nerve during parotid surgery . we report a new variation in the facial nerve pattern , and an unusual relationship with the retromandibular vein during parotid surgery . clinicians should recognize this facial anomaly and the unusual relationship with the retromandibular vein to avoid injuring the facial nerve during parotid surgery . a 22-year - old female presented to our department with left infra - auricular swelling present for > 2 years . a physical examination revealed a solitary , firm , nontender , mobile , and 3-cm sized mass in the left parotid gland . a computed tomography scan of the neck demonstrated a 3.2 2.3 cm well - defined , heterogeneously enhancing mass in the left parotid gland ( figure 1 ) . neck computed tomography scan demonstrates a 3.2 2.3 cm well - defined , heterogeneously enhancing mass in the left parotid gland . based on these observations , the preoperative diagnosis was a left - side superficial parotid tumor . a modified face lift incision was performed and a skin flap was elevated anteriorly , exposing the parotid gland . we found the main trunk of the facial nerve in the anticipated location and the nerve was dissected anteriorly and superiorly . we observed 2 unusual nerves ascending to the preauricular region and connecting to the facial nerve . after superficial parotidectomy preserving the facial nerve including the 2 unusual nerves , the 2 unusual nerves were found to connect to the temporofacial division from the main trunk of the facial nerve and were confirmed to be the facial nerve using the neurophysiological intraoperative monitoring ( nim ) 2.0 system ( medtronic ; minneapolis , mn ) ( figure 2 ) . in addition , an anomalous relationship was detected between the retromandibular vein and the facial nerve . the retromandibular vein was lateral to the cervicofacial division and medial to the temporofacial division ( figure 2 ) . two unusual nerves ( arrows ) connected to the temporofacial division from the facial nerve main trunk are detected after superficial parotidectomy , and the retromandibular vein ( arrowheads ) is lateral to the cervicofacial division but medial to the temporofacial division . this study was approved by the institutional review board of the chonnam national university hwansun hospital . therefore , understanding surgical landmarks for the facial nerve is essential for safe and effective parotid surgery . the most commonly used surgical landmarks are the stylomastoid foramen , the tympanomastoid suture , the posterior belly of the digastric muscle , the tragal pointer , and the retromandibular vein . among these landmarks , several reports have found that the retromandibular vein is medial to the facial nerve in up to 90% of cases . however , in the present case , retromandibular vein was lateral to the cervicofacial division and medial to the temporofacial division . however , we observed 2 unusual nerves connecting the temporofacial division in the present case . a limitation of this report is that we never found the origin of these unusual nerves . in conclusion , we report a new variation in the facial nerve pattern , and an unusual relationship with the retromandibular vein during parotid surgery . clinicians should recognize this facial anomaly and the unusual relationship with retromandibular vein to avoid injuring the facial nerve during parotid surgery . intraoperative nerve monitoring and careful dissection are important to avoid intraoperative facial nerve injury , particularly in patients with a facial nerve anomaly .

abstractthe branching pattern of the facial nerve varies among individuals . these variations increase the risk of facial nerve injury during parotid surgery . we report a new variation of the facial nerve and an unusual relationship with the retromandibular vein during parotid surgery.clinicians should recognize this facial anomaly and the unusual relationship with the retromandibular vein to avoid injuring the facial nerve during parotid surgery .

porokeratosis is a genetic disease transmitted as an autosomal dominant trait featuring abnormal epidermal keratinization , which is histologically characterized by the presence of cornoid lamella . since then many variants of porokeratosis have been described , each with differing morphology , distribution , and clinical course . familial adenomatous polyposis ( fap ) is characterized by numerous adenomatous colorectal polyps that have an intrinsic tendency to progress to adenocarcinoma . fap with extra - intestinal lesions such as epidermoid cysts , osteomas , desmoid tumors , dental anomalies is called gardner 's syndrome . the association of porokeratosis of mibelli with gardner 's syndrome is extremely rare without any recorded cases in the literature , prompted us to report this case . an 18-year - old female of low socio - economic status presented to skin opd with complaints of multiple skin lesions all over the body and swellings on the face , scalp , and extremities of five years duration . lesions started to develop on the scalp insidiously , and later on left mandible , face , and extremities . clinical examination of the patient revealed multiple skin plaques present on the face , forearms , back and both lower extremities with elevated margin with a prominent deep furrow and central part showed normal skin [ figure 1 ] . fluctuant skin swellings were observed on the scalp and back of trunk , with punctum [ figure 2 ] . over the body of left ramus of mandible a clinical diagnosis of porokeratosis of mibelli , epidermoid cysts , and osteoma of mandible was made . family history of the index case revealed father had dysphagia , pain abdomen with episodes of bleeding per rectum , and ultimately died of us suspected gastrointestinal ( git ) origin malignancy . biopsy of the skin plaques , along with the excision biopsy of cystic swelling over scalp and bony hard swelling of left mandible was done . because of the family history and clinical presentation ( sebaceous cysts and osteoma ) , a colonoscopy was done which revealed a solitary rectal polyp of 1 1.5 cm in size situated 7.0 cm from anal verge . porokeratosis of mibelli histopathological examination of the plaque lesions of skin revealed a keratin filled invagination of the epidermis , the center of which showed parakeratotic column ( interpreted as cornoid lamella ) . bone lesion showed the histopathology of a benign osteoma showing mature osteocytes [ figure 5 ] . histopathological examination of rectal polyp showed hyperplastic polyp of rectal mucosa with focal adenomatous change , mild nonspecific inflammation , cystic change , congested vessels with no evidence of malignancy [ figure 6 ] . parakertotic column ( cornoid lamella ) in porokeratosis mibelli , ( h and e , 100 ) osteoma with mature osteocytes ( h and e , 200 ) hyperplastic polyp of rectal mucosa with focal adenomatous change ( h and e , 100 ) the clinical picture , family history and multiple lesions diagnosed histologically in the index patient , we contemplated the possibility of porokeratosis of mibelli with an evolving gardner 's syndrome . gardner 's syndrome start with a few polyps in the 10 - 20 year age group and develop into its full blown clinical picture by the fourth decade of the patient , with an intrinsic tendency to progress to adenocarcinoma . it is caused by a germline mutation in the adenomatous polyposis coli ( apc ) gene located on the long arm of chromosome 5 . the mandible is the most common location ; however , osteomas may occur in the skull and the long bone . osteomas precede the clinical and radiographic evidence of colonic polyposis , therefore , they may be sensitive markers for the disease . porokeratosis is a heterogeneous group of disorders characterized by distinct clinical findings of a keratotic ridge with a central groove that corresponds to the cornoid lamellae on histology . etiopathogenesis of porokeratosis is unknown but genetic inheritance as well as acquired form has been reported . heredity , immunosuppression , infection , and ultraviolet rays have been found to play a role in the causation of porokeratosis . no specific candidate gene has been found . however , genetic loci of disseminated superficial actinic porokeratosis have been detected in the region 12q23.2 - 24.1 ( dsap 1 ) and 15q25.1 - 26.1 ( dsap 2 ) . recently , another loci has been found within an 8.2 cm or 11.9 mb region between markers d1s438 and d1s464 ( dsap 3 ) . larger lesions have higher malignant potentiality , particularly , the giant variety that is most frequently reported to show malignant transformation . malignancies reported in porokeratosis are squamous cell carcinoma , bowen 's disease , basal cell carcinoma , diffuse large b - cell lymphoma , etc . porokeratosis in association with lichen planus , diabetes mellitus , cap syndrome ( craniosynostosis , anal anomalies , and porokeratosis ) , bloom 's syndrome , cystic fibrosis etc , have been reported.[711 ] to the best of our knowledge this is the first case reported of porokeratosis of mibelli with gardener 's syndrome .

porokeratosis represents a heterogeneous group of disorders characterized clinically by a distinctive ridge - like border and histologically by cornoid lamellae . gardner 's syndrome , a variant of familial adenomatous polyposis ( fap ) , is an autosomal dominant disease characterized by colorectal polyps , osteomas , epidermoid cysts and soft tissue tumors . here we report a case of 18 yr old female who presented with porokeratosis of mibelli with osteoma , multiple epidermoid cytsts , and solitary rectal polyp .