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congenital pulmonary vein stenosis occured due to failed incorporation of common right and/or left pulmonary vein into the left atrium . main pulmonary artery branch or ppas should be suspected when a continuous murmur is heard or chest x - ray shows post stenotic dilatation or area of decreased vascularity . below is a case report of a 13 year old boy who presented with a rare combination of congenital pulmonary vein stenosis along with congenital stenosis of right pulmonary artery . a 13-year - old male patient presented to cardiology outpatient department with a complaint of easy fatigability for last 2 years . , he had blood pressure of 100/70 mmhg , pulse rate of 78/min ( regular ) , grade iii parasternal heave , and loud and palpable second heart sound in left second intercostal space . a systolic ejection murmur of grade 3/6 intensity was audible in left second intercostal space . echocardiography with color doppler revealed moderate pulmonary artery hypertension ( pulmonary artery systolic pressure of 55 mmhg ( using mahan 's formula ) , turbulent , continuous , and high velocity flow consistent with severe stenosis of both left superior and inferior pulmonary vein and also right inferior pulmonary vein as viewed in modified parasternal short axis view [ figure 1 ] . a pressure gradient of 32 mmhg was documented at the insertion of right inferior pulmonary vein in left atrium in apical four - chamber view [ figure 2 ] . a stenosis of the right pulmonary artery was also detected distal to the bifurcation of main pulmonary artery [ figure 3 ] . a pressure gradient of approximately 100 mmhg was observed at the level of the right pulmonary artery in parasternal short axis view [ figure 4 ] . computed tomography ( ct ) angiogram of thorax confirmed the stenosis of three pulmonary veins as well as branch stenosis of the right pulmonary artery . ct scan revealed dilated main pulmonary artery ( diameter = 3.9 cm ) and dilated left pulmonary artery ( diameter = 2.7 cm ) . right pulmonary artery ( diameter of proximal part = 1.7 cm ) had a focal narrowing in distal part ( diameter = 0.49 cm ) . narrowing was seen at junction of bilateral inferior and left superior pulmonary vein with left atrium . the diameter of the right inferior pulmonary vein was 2.2 mm , and left superior and inferior pulmonary vein was 3.9 mm each . turbulent flow detected at the insertion of left upper pulmonary vein and left lower pulmonary vein seen in modified parasternal short axis view . a pressure gradient detected at the level of insertion of right inferior pulmonary vein in apical four - chamber view . it occurs due to failed incorporation of common right and/or left pulmonary vein into the left atrium . usually , the condition is diagnosed in older infants and children , but it may be manifested as severe persistent pulmonary hypertension of the newborn . the most common cause of acquired pulmonary vein stenosis is post - radiofrequency ablation for treatment of atrial fibrillation . the condition is relentlessly progressive causing 50% death in 5 years even after successful surgery . initial experience with cutting balloon angioplasty was successful as palliative procedure though longest interval before repeat intervention was only 6 months . nowadays , ppas can lead to pulmonary hypertension but is likely misdiagnosed as either idiopathic pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension . therefore , a high index of suspicion during the initial evaluation of pulmonary hypertension is essential for its prompt diagnosis and adequate treatment . a combination of predominantly left - sided pulmonary vein stenosis ( as both the left upper and lower pulmonary veins were involved ) with right pulmonary artery branch stenosis is very rare , and in this particular patient , development of pulmonary artery hypertension was delayed because of restricted blood flow through right pulmonary artery .

congenital pulmonary vein stenosis is a rare entity caused due to failed incorporation of common right and/or left pulmonary vein into the left atrium . below is a case report of a combination of predominantly left - sided pulmonary vein stenosis with right pulmonary artery branch stenosis . the patient was an adolescent boy with mild symptoms . clinical examination revealed features of pulmonary artery hypertension . echocardiography and computed tomography scan were done to confirm the disease .

a 20-year - old refugee from liberia was referred to our hospital because of a loud murmur . , he had been admitted to a hospital in his home country several times because of respiratory tract infections and heart problems. he had a low body weight of 45 kg at a height of 1.63 m. he was hypertensive with a 50 mmhg difference in systolic blood pressure between his upper and lower extremities : 170 vs. 120 mmhg . the electrocardiogram showed sinus rhythm 84 beats / min with signs of left ventricular hypertrophy . 1 ) revealed a dilated , hypertrophied and hyperdynamic left ventricle due to a large pda with a continuous left - to - right shunt . just distal to the pda , narrowing of the aorta was visible , with an increased systolic flow velocity . the absence of a classic sawtooth pattern in the continuous wave signal from the descending aorta as well as the presence of holodiastolic flow reversal in the abdominal aorta suggested non - severe coarctation . the cardiac valves showed no abnormalities except for a mild pulmonary regurgitation with an early - diastolic flow velocity of around 3 m per second , suggestive of elevated pulmonary artery pressure . the descending thoracic aortic wall had an irregular aspect and its narrowing was most severe just distal to the pda . right ventricular and main pulmonary artery pressures were elevated ( systolic right ventricular pressure 55 mmhg , main pulmonary artery pressure 48/31 mmhg at a systemic blood pressure of 167/84 mmhg ) , but peripheral pulmonary artery pressures were normal . a peak - to - peak gradient of 50 mmhg was measured in the descending aorta . 1transthoracic echocardiography in patient a. a apical four - chamber view showing dilated and hypertrophied left ventricle . b parasternal view of the pulmonary trunk and arteries showing turbulent flow due to a pda with left - to - right shunting ; a stenosis of the proximal right pulmonary artery is also visible ( asterisk ) . c suprasternal view showing pda ( asterisk ) as well as coarctation of the aorta . d continuous wave doppler signal from the descending aorta , showing elevated systolic flow velocity of about 4 m per second . 2preoperative and postoperative magnetic resonance angiography . a preoperative magnetic resonance angiography ( mra ) of the aorta ( sagittal plane ) , showing large pda ( black arrow ) and irregular aspect of the descending aorta with coarctation distal to the pda ( white arrow ) . b repeat mra of the aorta after surgical correction of the pda , showing irregular aspect and ( mild ) residual stenosis / coarctation of the descending aorta transthoracic echocardiography in patient a. a apical four - chamber view showing dilated and hypertrophied left ventricle . b parasternal view of the pulmonary trunk and arteries showing turbulent flow due to a pda with left - to - right shunting ; a stenosis of the proximal right pulmonary artery is also visible ( asterisk ) . c suprasternal view showing pda ( asterisk ) as well as coarctation of the aorta . d continuous wave doppler signal from the descending aorta , showing elevated systolic flow velocity of about 4 m per second . a typical sawtooth pattern is lacking preoperative and postoperative magnetic resonance angiography . a preoperative magnetic resonance angiography ( mra ) of the aorta ( sagittal plane ) , showing large pda ( black arrow ) and irregular aspect of the descending aorta with coarctation distal to the pda ( white arrow ) . b repeat mra of the aorta after surgical correction of the pda , showing irregular aspect and ( mild ) residual stenosis / coarctation of the descending aorta the patient was referred for surgery . closure of the ductus arteriosus was performed and the pulmonary artery was reconstructed ( fig . after ligation of the pda , the pressure gradient in the descending aorta had decreased from 50 to 20 mmhg and this ( mild ) residual coarctation was left untreated . whether it will need ( percutaneous ) treatment in the future remains to be seen . he had previously been diagnosed with severe hypertension with a poor response to medical therapy . approximately eight years prior to referral to our hospital , echocardiography had already revealed left ventricular hypertrophy . moreover , it showed a pda with a continuous left - to - right shunt . a murmur had never been heard . at that time when he was referred to our outpatient clinic he did not have any cardiac complaints , although his exercise capacity had gradually deteriorated over the past year . in addition to the pda , echocardiography now showed not only left , but also right ventricular hypertrophy and an estimated right ventricular systolic pressure of 57 mmhg . he was referred for treatment of his pda on the assumption that it had contributed to the development of pulmonary hypertension . it must be noted that the pda was silent and did not appear very large on echocardiographic and mri . moreover , diastolic dysfunction due to left ventricular hypertrophy may have played an important role in the development of pulmonary hypertension as well . however , a contribution of the pda to the development of pulmonary hypertension could not be excluded and the patient was therefore accepted for percutaneous closure of the pda . angiography of the aortic arch and the occluded pda with coil in situ can be seen in fig . in due time , echocardiography will be repeated to document whether his pulmonary artery pressure will decrease . mri visualised the jet of the pda flow entering the pulmonary bifurcation ( black arrow)fig . mri visualised the jet of the pda flow entering the pulmonary bifurcation ( black arrow ) angiography of the aortic arch . pda is usually diagnosed shortly after birth , and treated surgically or percutaneously to prevent the development of pulmonary hypertension and heart failure . the first case represents a more complex form of pda , associated with additional congenital cardiovascular anomalies and with left ventricular volume overload as one of its main characteristics . in the second patient , pulmonary hypertension was the sole reason for closure of the pda , and left ventricular dilation had not developed . a difference in shunt size is probably the main reason for this difference . in addition , it may be possible that patient b s severe left ventricular hypertrophy due to hypertension prevented left ventricular dilatation . closure of a pda is only feasible when irreversible pulmonary hypertension has not yet developed . in adults , a , surgical correction of the pda was performed because of its large size and concomitant anomalous aortic anatomy . pda patients who are beyond surgical or percutaneous closure should be treated according to the guidelines for eisenmenger syndrome .

patent ductus arteriosus ( pda ) is a rare diagnosis in adults , since symptoms and signs usually occur in infancy and most cases are treated shortly after diagnosis . we present two patients who were first diagnosed with pda during adulthood . the first case represents a more severe form of pda , where the need for closure of the pda is obvious . in the second case the sequelae of the pda are less clear . in both patients , closure of the pda ( surgically in one case , percutaneously in the other ) was successful .

plexiform neurofibroma is the term applied to a diffuse neurofibromatosis of nerve trunks , which is often associated with an overgrowth of the skin and subcutaneous tissues . it is a distinct type of neurofibroma that expands a nerve into a large tortuous mass of fibers that has a bag of worms appearance . plexiform neurofibromas are the least common variant and usually are pathognomonic for nf i. the most commonly involved sites are the temple , upper lid , back of the neck , and the tongue . oral manifestations of neurofibromatosis have been reported in only 4 - 7% affected persons , in which the tongue was the most commonly involved site . this systemic process , originally described by von recklinghausen , is an autosomal dominant disorder caused by a defect on chromosome 17 . patients usually present in childhood with numerous cutaneous or subcutaneous neurofibromas , melanotic lesions in the iris called lisch nodules , caf au lait spots , and optic gliomas . plexiform neurofibromas occur in only 17% of cases of nf i. when they occur in the setting of nf i , 91% are solitary lesions , and most of the lesions occur in the trunk , extremities , and head neck region . histologically , neurofibroma consist of endoneural ground substance and schwann cells that extend outside the perineurium . we present a case of an isolated plexiform neurofibroma of the tongue in a 11-year - old girl not associated with neurofibromatosis . an 11-year - old girl was admitted to the department of e.n.t . of our institute , with the complaint of inability to close the mouth because of a large , asymmetrical , protruding tongue . the girl had difficulty in swallowing , breathing , and clear speaking since early months of her life . on palpation , the right side of the tongue was hypertrophied and the movements of the tongue were normal , but she was unable to hold the tongue completely in the mouth because of its enormous size . there was also slight generalized swelling of the right side of the base of the tongue . no neck glands were palpable and functions of v , vii , x , and xii cranial nerves were intact . a magnetic resonance image was obtained to better assess the base of tongue , which revealed a 3 3 2.5 cm heterogeneously enhancing mass with infiltration in the tongue and tongue base , this lesion was hypervascular and well - circumscribed . under general anesthesia , gross examination of the specimen revealed irregular surface which was soft in consistency [ figure 1 ] . histopathological examination revealed well circumscribed multiple nodular lesions composed of elongated spindle wavy cells with muscle fibers in between , these wavy cells were arranged against a myxoid background . an ophthalmologic examination was carried out which showed no eye lesions of neurofibromatosis ( such as lisch nodules in the iris , ephelides on eyelids , or ectopia lentis ) . the skin of the whole body was examined and no caf au lait spots or frecklings of the axillary and inguinal region were observed . on plain radiograph , no bone pathology was found . after the diagnostic work - up , surgical treatment was planned . under general anesthesia , the tumor was approached intraorally , and the biopsy report was consistent with plexiform neurofibroma . the movements of the tongue were preserved , and , in addition , a significant improvement in her speech was observed . the patient has been on follow - up for 10 months and no additional treatment has been necessary . gross picture of neurofibroma h and e - stained section showing spindle wavy - shaped cells seperated by fibromyxoid stroma , 400 h and e - stained section showing discrete well - circumscribed collection of spindle cells with muscle fibers in between , 100 neurofibromas are benign tumors of neural origin , of which roughly 90% appear as solitary lesions . solitary plexiform neurofibromas arising outside the context of nf i , as illustrated in the patient presented here , are quite rare , with only scattered cases reported in the literature till date . in the literature , there are only few reports of macroglossia caused by plexiform neurofibroma , and the cases are almost always associated with neurofibromatosis . our patient had no eye lesion , no bone pathology , and no caf au lait spots or frecklings on the skin . we could not find any other signs or symptoms other than isolated plexiform neurofibroma of the tongue to make a diagnosis of neurofibromatosis , and thus it was concluded to be a case of isolated plexiform neurofibroma of the tongue . when these ill - defined tumors grow in the head and neck , they are commonly symptomatic and disfiguring , causing upper airway obstruction , swallowing or mastication difficulties , or cosmetic distortion of the face . diffuse plexiform neurofibroma , is also known as elephantiasis neurofibromatosa , which shows overgrowth of epidermal and subcutaneous tissue along with a wrinkled and pendulous appearance . neurofibromas of the large nerves , which appear clinically as soft , drooping , and doughy masses , are benign neoplasms composed of neurites , schwann 's cells and fibroblasts within a collagenous or myxoid matrix . in contrast to schwannomas , they are nonencapsulated and engulf the nerve of origin . plexiform neurofibromas , forming tortuous cords along the segments and branches of a nerve with a tendency to grow centripetally , are poorly circumscribed tumors . it needs to be differentiated from schwannoma which is encapsulated , while plexiform neurofibroma is noncapsulated ; moreover , in schwannoma there are antony a and antony b along with prescence of verocay bodies on microscopic examination . since neurofibromas are usually multiple lesions , the whole body must be examined and investigated . in patient with oral neurofibroma , larynx and trachea must also be examined as in such a case lesions in the upper airway may cause respiratory obstruction . plexiform neurofibromas should be monitored frequently because 5% may turn into malignant peripheral nerve sheath tumors . differential diagnosis of such a tongue mass must include neurofibroma , schwannoma ( neurilemoma ) , lymphangioma , hemangioma , hamartoma , teratoma , pyogenic granuloma , nerve sheath myxoma , and cystic lesions such as mucoid cysts and dermoid cysts . the standard treatment for neurofibromas has been surgical excision and the diagnosis can only be confirmed by histological examination . early diagnosis in such a patient is very important and these patients need regular follow - up during their lifetime to detect recurrences . fortunately , there were no signs of recurrence or other manifestations during the follow - up period of our patient till date . we present this case for its rare site along with nonassociation with neurofibromatosis , excellent recovery , and no recurrence till date on follow - up .

neurofibromas ( nf ) are benign tumors of neural origin , of which roughly 90% appear as solitary lesions . they are classified into cutaneous , subcutaneous , and plexiform subtypes . plexiform neurofibromas are the least common variant and usually are pathognomonic for nf i. here , we present a very rare case of isolated plexiform neurofibroma with a painless enlarging mass of the tongue of an 11-year - old girl . this rare benign tumor has the potential for malignant transformation , and the diagnosis was difficult owing to the patient 's age and to the insidious clinical presentation . the present case is a diffuse isolated plexiform neurofibroma of the tongue that was not associated with neurofibromatosis that was treated with intraoral surgery .

ventral hernias are the second most common type of abdominal wall hernias , after inguinal hernias . the majority of ventral hernias are incisional , occurring in 3% to 13% of laparotomy incisions , necessitating approximately 90 000 ventral hernia operations per year . the use of prosthetic material , such as polypropylene mesh , polytetrafluoroethylene , and marlex mesh has decreased rates of recurrence . however , wound complications with the use of mesh in open ventral hernia repair are significant . since 1992 , the laparoscopic technique has been applied to the repair of ventral hernias because of its many advantages including the absence of large subcutaneous flaps , a lower incidence of wound infection , and a reduction in postoperative pain and hospital stay . the lower recurrence rates are most likely due to the tension - free and intraperitoneal placement of the mesh . laparoscopy offers a clear view of the entire fascial defect , which is not always possible with the open technique , especially if a thus , the entire defect is completely circumscribed with the laparoscopic technique . the surgical technique described for laparoscopic ventral hernia repair varies by surgeon . however , all techniques reported use at least 3 port sites , with one or more being 10 mm . using this technique , either the primary surgeon or the first assistant is working in mirror - image . between july 2002 and january of 2003 , 3 patients underwent laparoscopic ventral hernia repair with mesh in which only two 5-mm ports were used for small incisional or ventral hernias . follow - up surveillance for complications and hernia recurrence was performed in the immediate postoperative period , 1 week to 2 weeks after surgery . additional follow - up was obtained by telephone interview 6 months to 12 months postoperatively . after the abdomen was prepped , an iodine impregnated adhesive drape dressing was placed on the patients ' abdomen . access to the abdomen was obtained in an area lateral to the hernia by using a veress needle . after exploration of the abdomen , a second 5-mm trocar was introduced under direct visualization on the opposite side of the fascial defect . adhesiolysis was performed to free the anterior abdominal wall , and the margins of the hernia defect were circumferentially cleared to a distance of at least 4 cm . a dual , expanded polytetrafluoroethylene ( eptfe ) mesh ( dualmesh , wl gore , flagstaff , az ) was used , ranging in size from 8.5 cm to 10 cm . once the mesh was place intracorporeally , the sutures were retrieved through the abdominal wall by using a laparoscopic suture passer . the sutures were tied extracorporeally , and the knots were buried in the subcutaneous tissues . the circumference of the mesh was then affixed , by using a 5-mm spiral tacking device , to the posterior abdominal wall at intervals of approximately 1 cm . after the abdomen was prepped , an iodine impregnated adhesive drape dressing was placed on the patients ' abdomen . access to the abdomen was obtained in an area lateral to the hernia by using a veress needle . after exploration of the abdomen , a second 5-mm trocar was introduced under direct visualization on the opposite side of the fascial defect . adhesiolysis was performed to free the anterior abdominal wall , and the margins of the hernia defect were circumferentially cleared to a distance of at least 4 cm . a dual , expanded polytetrafluoroethylene ( eptfe ) mesh ( dualmesh , wl gore , flagstaff , az ) was used , ranging in size from 8.5 cm to 10 cm . once the mesh was place intracorporeally , the sutures were retrieved through the abdominal wall by using a laparoscopic suture passer . the sutures were tied extracorporeally , and the knots were buried in the subcutaneous tissues . the circumference of the mesh was then affixed , by using a 5-mm spiral tacking device , to the posterior abdominal wall at intervals of approximately 1 cm . the size of the mesh used varied , based on the size of the original defect . additionally , no recurrences were noted after a mean follow - up of 9 months ( range , 6 to 12 ) . laparoscopic ventral hernia repair is a procedure that has gained acceptance among surgeons and patients over the last 5 years to 10 years . the documented advantages of laparoscopic surgery in general include faster recovery and return to normal activity , less postoperative pain , fewer wound - related complications , and a better cosmetic result . in addition , the intraperitoneal repair performed laparoscopically poses a significant mechanical advantage and is associated with less recurrence . we present 3 patients who underwent laparoscopic repair of a ventral hernia in which two 5-mm ports were used . these patients had no surgical complications , and the mean operating room times were acceptable . this technique offers all the advantages inherent to laparoscopic surgery , with the added benefit of the use of only two 5-mm ports . laparoscopic repair of ventral or incisional hernias can be performed using only two 5-mm ports . this technique can be done on an outpatient basis in a safe , timely fashion .

objective : high recurrence rates have been documented after primary repair of incisional hernias . laparoscopic ventral and incisional hernia repairs have been performed with very low rates of recurrence . we have modified the standard technique of laparoscopic repair in patients with small incisional and ventral hernias . the purpose of this study was to document the technique utilizing only two 5-mm ports and demonstrate that it is safe , effective , and feasible.methods:three patients with small incisional or ventral hernias were examined . the standard laparoscopic ventral hernia repair technique was modified as follows : two 5-mm ports were inserted on opposite sides of the defect . the defects ranged from 2.5 cm to 4 cm in size . expanded polytetrafluoroethylene mesh ( dualmesh , wl gore , flagstaff , az ) was used to cover the hernia defect , overlapping the defect margins circumferentially by 3 cm . the mesh diameter ranged from 8.5 cm to 10 cm . the mesh was inserted through a 5-mm skin incision site and affixed into position with transfascial sutures and spiral tacks.results:the operative time ranged from 53 minutes to 57 minutes . all patients were discharged home the day of surgery and reported minimal postoperative pain . follow - up ranged from 6 months to 1 year ; all patients were doing well without recurrence.conclusion:laparoscopic repair of ventral or incisional hernias can be performed using only two 5-mm ports . this technique can be done on an outpatient basis in a safe , timely fashion .

errors in the administration of chemotherapeutic agents have been reported in the literature . the majority of these reports concern the overdose of a single agent during combination chemotherapy . mistakes are usually decimal point errors or occur by confounding drugs with similar names . although combination chemotherapy is the treatment of choice for many forms of cancer , accidental overdose of multiple chemotherapeutic agents has not been reported so far . this 35-year - old man was diagnosed as having non - hodgkin s lymphoma in april 1986 . a cervical lymph node biopsy was compatible with the diagnosis of a low grade non - hodgkin s lymphoma which was diffuse centroblastic centrocytic according to the kiel classification . he was treated with three cycles of the chop regimen followed by daily oral leukeran for 6 months . in may 1987 , progression of the disease in the abdomen trials with daily prednimustine therapy as well as 3 cycles of vp-16 therapy were unsuccessful and the disease was progressing . in november 1988 , he was hospitalized for a more aggressive chemotherapy . a macop - b - type regimen of adriamycin and cyclophosphamide , alternating with vincristine and bleomycin every other week , combined with daily oral prednisone , ketoconazole and bactrim was scheduled . due to a misunderstanding , the following doses were administered : adriamycin 50 mg / m per day and cyclophosphamide 350 mg / m intravenously for 6 days followed by vincristine 1 mg / m and bleomycin 10 mg / m intravenously for 4 days , resulting in a total of 600 mg adriamycin , 4200 mg cyclophosphamide , 80 mg bleomycin and 8 mg vincristine within 10 days . on the 10th day of chemotherapy , the patient developed fever , generalized weakness and malaise , severe oral and anal discomfort , epistaxis and loss of fresh blood per anus . the patient was severely ill , hypotensive with a bp of 110/50 , pr of 114 and febrile with a temperature of 39.2c . the following laboratory data were obtained : hb 8.4 g / l , hct 25% , wbc 11.98010/1 with 97% lymphocytes and 0.8% pmn . the boen marrow aspiration on admission showed an absent normal hemopoiesis but still massive infiltration with b4 , b1 , hla - dr , surface igg , igd and lambda light chain positive lymphocytes compatible with the diagnosed lymphoma . he was treated with broad spectrum antibiotics , initially with kefzol , tobramycin and azlocillin , which was changed to tienamycin , amikacin and metronidazole because of persistent fever . recombinant human granulocyte macrophage colony stimulating factor ( rhgm - csf ) was given 400 ug daily as a continuous infusion for 17 days . an episode of intractable severe oral mucosal bleeding leading to hemorrhagic shock was treated with an intravenous infusion of 8-ornithine - vasopressin . he required a total of 21 units of packed red cells and 14 units of single donor platelet concentrates during hospitalization . a bone marrow examination one week after admission and rhgm - csf therapy showed three lineage hemopoiesis and only a few remaining small lymphocytes . by this time , the spleen was no longer palpable and the signs of ileus had improved . during the whole hospitalization , recovery of the first granulocytes was observed after 10 days of rhgm - csf infusion , corresponding to the 26th day after chemotherapy . his wbc count was 2.210/1 with 60% pmn s and his platelet count was 2110/1 . physical examination was normal with the exception of minimal splenomegaly and the absence of the deep tendon reflexes . the reevaluation of the disease status showed areas of lymphocytic infiltration in the bone marrow aspirates and still significant retroperitoneal , mesenteric and iliac lymphadenopathy on abdominal computed tomography . this patient was treated with 600 mg adriamycin , 4200 mg cyclo - phosphamide , 80 mg bleomycin and 8 mg vincristine within 10 days and suffered mainly from life - threatening pancytopenia , mucositis and ileus . our major concern upon admission was adriamycin toxicity because he had received six time the scheduled dose of adriamycin on 6 subsequent days ; this in combination with the other drugs . the maximum tolerated dose of adriamycin in human beings is not known . in mice , intravenous administration of adriamycin at a dose of 20 to 40 mg / kg is lethal within four days after injection . a single intravenous dose of 10 mg / kg causes a well defined acute cardiac pathology leading to diffuse chronic cardiomyopathy within fifty days . our patient did not show any signs of acute cardiac decompensation nor signs of arrhythmia . the echocardiographic findings of minimal cardiac dilatation after discharge may be the effects of this acute overdose as well as those of the previous adriamycin therapy . activated charcoal hemoperfusion has been suggested in reducing blood levels of adriamycin in patients with hepatic disease or accidental overdosage . since 5 days had already passed since the last dose , we did not perform hemoperfusion . it is possible , although not certain , that rhgm - csf has accelerated granulocyte recovery in this patient . it could have been spontaneous recovery 26 days after chemotherapy and 10 days after initiation of rh - gm - csf infusion . the tumor response of this refractory lymphoma with a spleen enlarged up to the pelvis prior to therapy was impressive but not complete . it remains to be seen whether low grade lymphoma can be treated at an earlier stage with high dose chemotherapy or by allogeneic bone marrow transplantation . in conclusion , this case demonstrates the danger of prescribing anticancer agents by in experienced physicians and underlines the need for specialized institutions for such therapies in order to allow control of dose and concentration and to minimize the possibility of such an accident .

a 35-year - old man with refractory low grade diffuse centroblastic centrocytic non - hodgkin s lymphoma was treated accidentally with an overdose of multiple chemotherapeutic agents . he was given adriamycin 50 mg / m2 and cyclophosphamide 350 mg / m2 for 6 days followed by 4 days of vincristine 1 mg / m2 and bleomycin 10 mg / m2 . he was transferred when he developed pancytopenia , fever , severe mucositis , ileus and peripheral neuropathy . he was treated with broad spectrum antibiotics , red cell and single donor platelet transfusions and strict parenteral nutrition . in addition , he was given a continuous infusion of 400 ug daily human recombinant granulocyte macrophage - colony stimulating factor ( rh gm - csf ) for 17 days . intractable severe bleeding from his oral mucositis necessitated treatment with a continuous infusion of 8-ornithine - vasopressin for 8 days . he recovered and could be discharged home after 36 days of hospitalization with normal blood counts and without severe sequelae .

bilateral cystic nephromas are very rare with five cases described in the english literature . though rhabdomyosarcoma is a very common soft tissue sarcoma that occurs in children , rhabdomyosarcoma affecting the penile urethra is extremely rare . here , we present a case of bilateral cystic nephroma who subsequently developed embryonal rhabdomyosarcoma of the penile urethra . a 4-month - old male child presented in 2009 with the right flank mass that was progressively increasing for 2 months . examination revealed a soft , cystic , mobile , nontender , bimanually palpable and ballotable mass in the right lumbar region . imaging ( ultrasonography and computed tomography ) revealed two well - circumscribed multilocular noncommunicating cystic lesions , the larger one arising from the middle and lower pole of the right kidney , and a smaller one from the upper pole of the left kidney . a diagnosis of bilateral cystic nephroma / bilateral cystic partially differentiated nephroblastoma ( cpdn ) was made on the basis of the imaging . he was well for a period of 2 years when he developed a polypoidal mass protruding from the external urethral meatus . he was categorized as stage 1 rms and complete excision of the penile urethra with eight cycles of the postoperative vincristine , actinomycin - d , and ifosfamide was administered . the child is doing well more than 2 years after excision of the urethral rhabdomyosarcoma . bilateral cystic nephromas are extremely rare benign renal neoplasms with five cases described in the english literature . for the diagnosis of cystic nephroma , the other seven being solitary lesion ; multilocular lesion ; noncommunication of the cyst with the renal pelvis ; noncommunication of the cysts with each other ; loculi lined by epithelium ; intralocular septa devoid of renal parenchyma ; and if residual renal tissue was present , it should be normal . these criteria were modified by boggs and kimmelstiel to include the presence of immature renal tissue in the intervening septa . more recently elimination of unilaterality from the diagnostic criteria has been proposed in consideration of the increasing numbers of bilateral cystic nephroma . cpdn is a lesion with low malignant potential , which is radiologically and grossly indistinguishable from cystic nephroma . differentiation between the two is based on histological confirmation of the presence of blastemal cells within the septa . differentiation from cystic wilms is based on the absence of solid elements other than septa in cystic nephroma . however , an association of bilateral cystic nephroma with pleuropulmonary blastoma has been described with dicer-1 mutation being implicated as the cause . germline mutation in dicer-1 causes a variety of cancers , the most common being pleuropulmonary blastoma , cystic nephroma , and ovarian sertoli - leydig cell tumors . dicer-1 gene expresses a protein that plays an important role in regulating the activity of other genes by aiding the formation of micro rna . since bilateral cystic nephroma and urethral rhabdomyosarcoma are extremely rare lesions , their occurrence in the same child is unlikely to be coincidental . dicer-1 mutation could be the cause of this association , but the genetic analysis could not be done due to financial reasons .

bilateral cystic nephroma is an extremely rare benign renal neoplasm . here we present a case of bilateral cystic nephroma in a four month old boy who subsequently developed embryonal rhabdomyosarcoma of the urethra . both tumors were successfully treated . to our knowledge this is the first reported case of this association which could be related to dicer-1 mutation .

bladder cancer ( bca ) is the fifth most common cancer in the united states . in 2012 , it is projected that 73,510 people will be diagnosed with bca and 14,880 will die from the disease . muscle invasive bc ( nmibc ) , about 30% of patients will either initially present or later progress to muscle invasive bladder cancer ( mibc ) . radical cystectomy ( rc ) with urinary diversion ( ud ) is the ultimate curative treatment . diagnosis of bca in patients aged < 40 years is rare and is extremely rare in patients < 30 years of age . however , cases of bca have been previously reported in pediatric and young adult patients [ 4 , 5 ] . our aim is to report our experience with a young adult patient with mibc and review the literature for the natural history and outcome . a 28year old caucasian male patient with 10 pack year smoking history with no family history of bca presented to the emergency department with gross hematuria and left flank pain . the patient underwent computerized tomography ( ct ) of the abdomen and pelvis without contrast due to an elevated creatinine . ct showed a 6 cm fungating mass at the left posterior wall of the bladder extending to the left ureteral orifice and left hydronephrosis . no abnormal laboratory findings were noted except for a serum creatinine of 1.73 mg / dl . the patient was initially managed by left nephrostomy tube placement and subsequent transurethral resection of bladder tumor ( turbt ) . during cystoscopy , multiple large masses originating from the left side of the trigone extending to the prostatic urethra and the right ureteric orifice were noted ( figure 2 ) . no attempt was made to obtain muscle bites secondary to the large volume of disease . serum creatinine was 1.6 mg / dl following nephrostomy tube placement . the patient was counseled about the possibility for restaging turbt , rc , and urinary diversion by either ileal neobladder or ileal conduit . the patient then elected to undergo rc and ileal conduit urinary diversion as the patient had elevated serum creatinine and was reluctant to perform cic . urothelial carcinoma of the bladder in the first three decades of life is extremely rare . invasive disease and only 1.7% had high grade tumor . since the vast majority of young patients with bca present with non muscle invasive low grade disease , these patients have lower progression and recurrence rates compared to older patients . this further suggested that the nature of bca in the younger population is different compared to elderly patients . on the otherhand , yossepowitch and dalbagni found no difference in grade or stage upon comparing 74 patients < 40 years of age to 75 patients > 65 years of age . however , when focusing on only bca patients diagnosed in the first two decades of life , it is evident that these patients had a relatively indolent behaving bca . nevertheless , aggressive bca has been reported in children a 31 month old and a 14 year old . exposure to polycyclic aromatic hydrocarbons ( pahs ) is responsible for 10% to 15% of cases as a result of bladder carcinogenesis . genetic predisposition to bca has been suggested by reports of multiple case families with bca . however , it is unclear whether it resulted from a genetic predisposition or common environmental exposure among family members . as in elderly patients , radical cystectomy is the curative intervention for patients with mibc , recurrent high grade superficial bca , and high grade t1 disease . prostate and seminal vesicles sparing rc in addition to nerve sparing procedure can be a valuable option for those patients . preservation of urinary continence by performing a nerve sparing procedure and orthotopic neobladder urinary diversion is preferred to attain a proper quality of life and body image . in our patient , ileal neobladder urinary diversion was not performed due to the patient 's unwillingness to perform cic and the fear of chronic renal insufficiency considering the elevated baseline creatinine , ( 1.73 ) which remained elevated ( 1.6 ) following nephrostomy tube placement . this tendency is more pronounced in patients presenting in the first three decades of life and is likely to decrease with age . quality of life and fertility preservation is particularly significant in young patients undergoing radical cystectomy and urinary diversion . our case demonstrates a rare example of a high grade t2 transitional cell carcinoma in a 28 year male .

the peak incidence of bladder cancer ( bc ) is in the sixth decade of life . muscle invasive bladder cancer ( mibc ) in young adults is extremely rare . we report a case of mibc in a 28year old smoking male patient . the patient presented with hematuria and flank pain for which he underwent a computerized tomography ( ct ) scan of the abdomen and pelvis with and without contrast . the ct scan showed a 6 cm mass on the left side of the trigone extending to the left urteric orifice and left hydronephrosis , but no lymphadenopathy was noted . the patient then underwent a left nephrostomy tube placement followed by trans urethral resection of bladder tumor ( turbt ) . the tumor involved both ureteric orifices and extended to the prostatic urethra . complete resection was not feasible . pathology showed high grade t1 urothelial carcinoma . ct scan of the chest showed no distant lung metastasis . the patient then elected to undergo radical cystectomy with ileal conduit urinary diversion . final pathology revealed t2a n0 urothelial carcinoma of the bladder . our aim is to present our experience and review the literature for the natural history and oncological and quality of life outcomes of urothelial carcinoma of the bladder in young patients .

dronedarone is a relatively new antiarrhythmic drug and is held to be less proarrhythmic than comparable compounds , although its proarrhythmia potential in humans has not been sufficiently evaluated . we describe a so far unreported dronedarone effect , namely a significant alteration of both the morphology and the duration of the qrs complex on electrocardiogram ( ecg ) in a 41-year old patient with symptomatic paroxysmal atrial fibrillation . dronedarone is a derivate of the potent antiarrhythmic drug amiodarone that was designed to have less toxic side effects ( 1 ) . although dronedarone was approved following reports of an improved safety profile , more recent studies have cast doubt on its safety ( 1 - 2 - 3 ) . however , dronedarone is still held to be less proarrhythmic than comparable compounds , although its proarrhythmia potential in humans has not been sufficiently evaluated ( 1 ) . we describe a hitherto unreported dronedarone effect , namely a significant alteration of both the morphology and the duration of the qrs complex on ecg . a 41-year old patient was admitted to our emergency unit because of a highly symptomatic attack of paroxysmal atrial fibrillation ( af ) that a few hours later spontaneously converted into sinus rhythm . atrial fibrillation had been first diagnosed in this patent in 2008 and the af episodes had been becoming more frequent in the months immediately preceding hospital admission . on admission , it should be noted that , during sinus rhythm , the ecg was normal showing an unremarkable qrs morphology and a qrs duration of 85 msec ( fig . clinically , the patient was in a good state of health . his risk profile consisted of grade 1 obesity ( body mass index 30.9 kg / m ) and arterial hypertension that was well controlled by a combination therapy of an ace - inhibitor and a beta - blocker . routine blood tests , including kidney , liver and thyroid function analysis , were all within normal values . coronary artery disease had been previously excluded by coronary angiography in 2011 , and a recently performed transthoracic echocardiography revealed a structurally normal heart exhibiting normal systolic and diastolic function . after giving informed consent , the patient was reluctant to undergo an invasive approach and preferred pharmacological rhythm management with dronedarone . according to our standard approach for af patients admitted to our institution , the initiation of antiarrhythmic therapy , regardless of the antiarrhythmic drug , is always performed in the hospital setting . during the hospital stay , the patients are not restricted to bed rest but are allowed to be ambulant while being continuously monitored using ecg telemetry . in addition , 12-lead standard ecg is registered in each patient on a daily basis . in the index patient , after two doses of dronedarone ( 400 mg each ) , a marked prolongation of qrs duration with a left bundle branch block pattern was documented on the 12-lead ecg ( fig . , the qrs complexes again showed a normal morphology and a normal duration of 90 msec ( fig . subsequently , the patient refused further attempts at pharmacological rhythm control , but requested a catheter ablation of atrial fibrillation that had recurred . therefore , circumferential pulmonary vein isolation was successfully performed . return to normal qrs - width after dronedarone withdrawal and self - limiting episode of atrial fibrillation . three months later , at the first follow - up visit in our rhythmology outpatient clinic , the patient was free of af and continued to show a narrow qrs - complex . dronedarone is a multichannel blocker exhibiting all of the 4 vaughan williams classes of action ( 4 ) . in fact , qt - interval prolongation , increased frequency of ventricular premature beats , and episodes of atrial flutter have all been reported as potentially proarrhythmic side effects of dronedarone ( 2 , 3 ) . mechanistically , this phenomenon may be explained by a reduction in the maximum slope of the action potential upstroke due to an increased inhibition of the fast na(+ ) inward current by dronedarone ( 4 , 5 ) . thus , a widened qrs complex may be indicative of slowed impulse conduction in the ventricles and may herald potential proarrhythmia . therefore , careful telemetry monitoring appears to be mandatory , particularly during the period following the first administration of dronedarone .

dronedarone is a relatively new antiarrhythmic drug and is held to be less proarrhythmic than comparable compounds , although its proarrhythmia potential in humans has not been sufficiently evaluated . we describe a so far unreported dronedarone effect , namely a significant alteration of both the morphology and the duration of the qrs complex on the electro cardiogram in a 41-year old patient with symptomatic paroxysmal atrial fibrillation .

peri - renal sepsis in the setting of chronic renal failure requiring an emergency nephrectomy is rare . there is a single case report of overwhelming mrsa urosepsis in a patient with stag horn calculus who required an emergency nephrectomy . pyelonephritis accounts for 34 hospital admissions per 10 000 females , with normal renal function , and the incidence in patients with chronic pyelonephritis is not known . in patients with chronic kidney disease who are anuric , it is difficult to establish the diagnosis of renal or peri - renal sepsis . cross - sectional imaging may remain inconclusive early in the illness and at times take 23 weeks to become apparent . worsening abdominal pain with severe lactic acidosis and without peritonitis usually heralds a major intra - abdominal vascular event . while a similar presentation has been described in the setting of stone disease and mrsa sepsis , to our knowledge , this is an index case where severe peri - renal sepsis presented with life - threatening metabolic acidosis in the absence of any obvious predisposing case and possibly secondary to a fully sensitive s. aureus infection . metastatic sepsis from infective endocarditis is a well - recognized complication , and we postulate that despite adequate antibiotic therapy , this may have been the source of sepsis in this case . the interesting features that this case illustrates are that the absence of common conditions causing an acute abdomen , obscure renal or peri - renal sepsis should be considered as a possible source in anuric patients with chronic kidney disease , and that surgical management with a nephrectomy may prove necessary after failure of conservative management .

severe worsening lactic acidosis in an elderly patient following an episode of atrial fibrillation , who is not haemodynamically compromised , usually indicates an intra - abdominal vascular catastrophe . we describe a unique case of severe peri - renal sepsis in a patient with long - standing dialysis - dependent chronic kidney disease unrelated to urolithiasis that masqueraded as an acute abdominal condition requiring emergency laparotomy and nephrectomy .

early detection of a hearing problem with a diagnostic tool like newborn hearing screening test is important . early detection can bring improvements in hearing ability and language development of children by promoting the use of rehabilitation strategies including hearing aid and cochlear implant . auditory neuropathy is characterized by abnormal auditory brainstem response ( abr ) and normal otoaoustic emission ( oae ) responses . although auditory neuropathy manifests as various patterns in pure tone and speech audiometry , most patients have very poor word discrimination function.1 ) we present a case of auditory neuropathy improved in a child who was diagnosed with moderate hearing loss by abr testing at 16 months of age , but who presented normal pure tone threshold and speech discrimination score 3 years after wearing hearing aids . a six - year - old boy presented to the outpatient clinic with abnormalities of hearing and language development . no congenital anomalies were evident at birth . however , he was not able to speak until 16 months of age and his response to external sound stimuli was slow . he showed no abr - response to 65 db nhl click stimuli at 16 months age . since then he had worn a hearing aid in the right ear ( fig . 1 ) . at 25 months of age , aided hearing threshold was about 30 db hl in sound field pure tone audiometry ( fig . he wore the hearing aid for another 2 years . at 5 years , 10 months of age , he visited our hospital for a hearing evaluation . his parents related that his hearing function seemed to have improved with time , because he had no difficulty communicating with others without a hearing aid . abr test showed no click sound response in both ears at 90 db nhl ( fig . transient evoked otoacoustic emission showed response reproducibility of 88% ( right ear ) and 95% ( left ear ) , and normal distortion product otoacoustic emissions responses were reported ( fig . however , normal pure tone threshold was evident at both ears ; speech reception threshold was 22 db ( right ear ) and 26 db ( left ear ) and speech discrimination score was 96% ( right ear ) and 88% ( left ear ) ( fig . 4 ) . he was recommended to stop wearing the hearing aid . a language development test conducted at 7 years , 4 months of age revealed an age equivalent of 6 years on expressive vocabulary test and 5 years on receptive vocabulary test . the concept of auditory neuropathy was first introduced by starr , et al.1 ) in 1996 to describe 10 patients , including infants and adolescents , showing normal oae results , abnormal abr results and poor speech discrimination score . auditory neuropathy is characterized by the auditory pathway lesion in the inner hair cells , the junction between the inner hair cells and the nerve , and the spiral ganglion or the auditory nerve in the presence of normal outer hair cell function . , auditory neuropathy is described as peripheral neuropathy resulting from the demyelination of auditory nerves . demyelinating lesions decrease the speed of neurotransmission , which in turn impairs nerve stimulation responses of the brain stem and decreases the synchronized activity of the entire auditory nerve activity , leading to unusual waveforms.1,2 ) auditory neuropathy accounts for nearly 2.4 - 15% of all childhood sensorineural hearing loss . auditory neuropathy most often occurs in both ears among infants aged 2 years or younger although it can occur in adults.3 ) more recently , auditory neuropathy has been increasingly referred to as auditory neuropathy spectrum disorder in recognition of its heterogeneity . these risk factors include hypoxia , preterm birth , hyperbillirubinemia , ototoxic medication use and neurological diseases , such as mitochondrial disease . especially , hyperbilirubinemia is a leading cause of auditory neuropathy.4 ) infants with one of these risks need to undergo oae testing , especially when they have a poor abr roche , et al.5 ) reported the relationship of radiologic findings with auditory neuropathy in 118 patients ; radiologic abnormalities were observed in 64% and 23% of patients examined using magnetic resonance imaging ( mri ) group and computed tomography , respectively . the most common abnormality in mri is the absence of the auditory nerve , followed by brain lesion . mri is the most important radiologic tool in deciding on a course of treatment and assessing the prognosis of auditory neuropathy . in this case , the treatment for childhood auditory neuropathy includes hearing aids , language therapy and regular hearing test - based monitoring . also , cochlear implant can be considered in case of poor outcomes.6 ) kim , et al.7 ) compared outcomes of six children with auditory neuropathy and four children with sensorineural hearing loss after cochlear implant , and reported no difference in hearing recovery outcomes between the two groups . fulmer , et al.8 ) also reported no significant difference in hearing improvement following cochlear implant in 10 patients with auditory neuropathy and 10 patients with sensorineural hearing loss . for infants , abr waveforms can be improved with time , so hearing conditions should be tested and examined regularly . dunkley , et al.9 ) introduced a case in which a child showed pure tone threshold average of 35 db hl and normal word discrimination test 5 years after the diagnosis of audiometry neuropathy based on abr waveforms of 50 db nhl with normal oae at 1 week of age . psarommatis , et al.10 ) also chronicled the improvement of auditory neuropathy in 20 children of 25 patients who had abr waveforms of more than 75 db nhl and normal oae . of the 20 children , 12 exhibited abr less than 40 db nhl and one child had 50 db nhl but normal results in other hearing tests . berlin , et al.11 ) studied 260 children with auditory neuropathy and found that 13 ( 5% ) had no problem in language development and ended up needing no treatment , even hearing aids . the patient in the present case also showed typical clinical features of auditory neuropathy including non - response for abr in addition to normal oae results . however , hearing improved to the extent that he had normal hearing for puretone and speech audiometry , and language development was only mildly delayed compared to other children in the same age group . we suggest following observation on a regular basis and reassessment of audiologic status and language development .

newborn hearing screening test is very important in the early diagnosis of childhood hearing loss because it affects language development . auditory neuropathy is a spectrum disorder characterized by abnormal auditory brainstem response but preserved otoacoustic emission and cochlear microphonics . in general , auditory neuropathy patients have poor word discrimination and variable patterns of pure tone audiometry . we report on a patient with auditory neuropathy diagnosed at 16 months of age and started wearing hearing aids , but showed normal pure tone and speech audiometric findings 3 years later . close follow - up for patients with auditory neuropathy is recommended .

autoimmune polyglandular syndrome ( aps ) is a rare genetic disease that causes multiple endocrine disorders . the vague presentation of endocrine dysfunction can cause a delay in both the diagnosis and the treatment of life - threatening conditions . we , herein , present a case of aps in a 21-year - old man whose condition did not respond to initial treatment and in whom a definitive diagnosis was subsequently achieved by workup for hypocortisolism . a 21-year - old man with a history of hashimoto 's thyroiditis with hypothyroidism presented to the emergency department for evaluation of an altered mental status . he was very lethargic , stared blankly , and was unable to respond appropriately to questions . he reported no fever , difficulty swallowing , shortness of breath , muscle or joint pain , headache , vomiting , or excessive thirst or urination . physical examination revealed a temperature ( t ) 34.3c , heart rate ( hr ) 68/min , respiratory rate ( rr ) 16/min , and blood pressure ( bp ) 87/57 mmhg . he was very lethargic , confused , and able to answer only yes - or - no questions . the patient exhibited hyponatremia ( 128 meq / l ) , hypoglycemia ( 29 mg / dl ) , and a high thyroid - stimulating hormone ( tsh ) level ( 14.31 iu / ml ) . test for other metabolic panel , complete blood count , triiodothyronine and thyroxine levels , head computed tomography , and urine toxicology screening results were all normal . he was diagnosed with severe hypothyroidism and received 2 l of fluid for resuscitation , 25 g of dextrose , and a single intravenous ( iv ) dose of 100 mcg levothyroxine at the emergency department . a random serum cortisol level was < 1.0 g / dl . a 250 g adrenocorticotropic hormone ( atch ) stimulation test was < 0.1 g / dl . he was diagnosed with primary adrenal insufficiency and received a treatment with iv hydrocortisone . his mental status , generalized fatigue , and metabolic parameters including glucose and sodium levels substantially improved . aps type 2 ( aps2 ) was suspected based on his history of hypothyroidism and primary adrenal insufficiency . the results of an additional liver function test , anti - gad65 antibody titer , testosterone level , and plasma renin activity were all within normal limits . the patient had returned to his normal level of activity by the time of the outpatient follow - up and reported feeling much better after treatment with hydrocortisone , fludrocortisone , and levothyroxine . the present case illustrates an example of aps2 , the clinical presentation of which may overlap that of primary adrenal insufficiency and hypothyroidism due to autoimmune thyroiditis . the present patient exhibited lethargy , an altered mental status , hypothermia , hypotension , hypoglycemia , an elevated tsh level , and normal thyroxine levels . he was later found to have primary adrenal insufficiency and his clinical symptoms improved after receiving corticosteroid replacement therapy . other autoimmune diseases , especially of the skin , stomach , and gonads , may occur with increased frequency in patients with aps2 . the greatest danger associated with aps2 is treatment of the presenting hypothyroid state without recognition of concomitant hypoadrenalism . hypothyroidism reduces cortisol clearance , and the addition of thyroid hormone replacement increases cortisol clearance . additionally , hypothyroidism reduces the metabolic rate , thereby , reducing the need for cortisol . the increased metabolic rate accompanying thyroxine replacement increases the requirement for cortisol . furthermore , a high serum concentration of tsh in the absence of primary thyroid failure can be a feature of adrenal insufficiency . the development of an adrenal crisis due to underlying adrenal insufficiency has been reported after the administration of thyroxine replacement therapy to patients initially thought to have only hypothyroidism . the management of aps2 is based on individualized , lifelong replacement therapy for the affected endocrine organs plus monitoring for development of insufficiency in other organs or associated conditions . the use of medical alert sensors and other measures should be taken to ensure that adrenal function is considered during illness , surgery , or emergency situations . in summary , we have reported a case involving a patient with aps2 who presented with lethargy , hypotension , hypoglycemia , and an elevated tsh level . the vague presentation of this syndrome can cause missed or delayed treatment for adrenal insufficiency resulting in a life - threatening adrenal crisis .

context : autoimmune polyglandular syndrome is a rare condition that causes a variety of clinical symptoms due to autoimmune processes involving multiple endocrine organs . its vague presentation can cause missed or delayed treatment for adrenal insufficiency , resulting in a life - threatening adrenal crisis.case report : a 21-yr - old man presented with lethargy , hypotension , hyponatremia , hypoglycemia , and an elevated thyroid - stimulating hormone level . he was binge drinking the day before presentation . no significant response to initial treatment with levothyroxine and dextrose occurred . diagnostic workup later revealed primary adrenal insufficiency . all initial symptoms completely resolved following treatment with hydrocortisone , fludrocortisone , and levothyroxine.conclusion:autoimmune polyglandular syndrome causes dysfunction of multiple endocrine organs such as the thyroid gland , adrenal gland , and pancreas . initial diagnosis of aps is crucial and difficult because of its vague , acute presentation , which often involves hypothyroidism and adrenal insufficiency . delayed treatment of adrenal insufficiency can result in a life - threatening adrenal crisis . a diagnostic workup for adrenal insufficiency should be performed in patients who do not respond to hypothyroidism treatment .

crestal incision placed about 1.5 mm palatal to the greatest bony height with an angulation of 45 with no . 15 bard parker blade [ figure 1 ] a vertical releasing incision placed mesially and distally to the crestal incision without disturbing the adjacent teeth papilla and extending about 3 mm labially [ figure 2 ] soft tissue reflected to expose the cover screw [ figure 3 ] palatal extension of the incision followed closer to the adjacent teeth on both sides , and a release incision made around the palatal portion of the exposed cover screw [ figure 4 ] then healing abutment is placed after thoroughly cleaning the soft tissue around internal hex of the implant [ figure 5 ] soft tissue approximated with 40 suture material ( vicryl)sutures removal has to be done after 10 daysafter 2 weeks , second stage prosthetic procedure should be carried out . crestal incision placed about 1.5 mm palatal to the greatest bony height with an angulation of 45 with no . 15 bard parker blade [ figure 1 ] a vertical releasing incision placed mesially and distally to the crestal incision without disturbing the adjacent teeth papilla and extending about 3 mm labially [ figure 2 ] soft tissue reflected to expose the cover screw [ figure 3 ] palatal extension of the incision followed closer to the adjacent teeth on both sides , and a release incision made around the palatal portion of the exposed cover screw [ figure 4 ] then healing abutment is placed after thoroughly cleaning the soft tissue around internal hex of the implant [ figure 5 ] soft tissue approximated with 40 suture material ( vicryl ) sutures removal has to be done after 10 days after 2 weeks , second stage prosthetic procedure should be carried out . incision and flap outlined ( after reflection ) more importantly it plays an important and crucial role in an esthetic component of a natural dentition . hence , it is very vital to maintain the integrity of the papilla during second stage implant surgery . several methods with unique incisions and suturing designs have been proposed to regenerate interdental papilla around implants . palacci suggested that a full thickness flap be raised from the buccal and palatal side of the implant on the ridge and rotated 90 to accommodate the interproximal space of the implant . palatal sliding strip flap to form papilla between implants and natural dentition in the anterior maxilla . nemcovsky et al . suggested an u- shaped incision , with opening toward the buccal aspect of the implant site with divergent incisions . tinti and benfenati reported a ramp mattress suture design , which pulled the buccal flap coronally , to obtain a papilla between two implants . suggested a split finger surgical method in which three interlacing finger like incisions were made and each of the fingers was sutured over the desired interdental papillary position . proposed a method for papilla regeneration wherein the healing abutment is buried beneath the gingival flap to produce a dead space with expectation of the space being filled by the soft tissue . even though these procedures provided papillary height , the results were not predictable . various factors were involved in the success of any papilla regeneration procedures , such as preservation of blood supply , prevention of scar formation , and more importantly ideal placement of the implant into the bone . this new and innovative surgical technique provided significantly excellent results compared to the conventional mid - crestal technique [ figures 6 and 7 ] . in this case report , in order to overcome the limitations of above - mentioned techniques a novel window technique was followed to regenerate papilla around implants during second stage surgery prior to prosthetic restoration . unlike most other procedures mentioned above , this technique avoided the labial papilla on the adjacent teeth and involved wholly the palatal papilla , which is considered to be the key element in the success of this technique . moreover , there is no cross incisions on to the papilla , which may lead to , disturbed wound healing and scar formation . above all avoiding more invasive surgery is considered one of the best methods of soft tissue regeneration , which is being incorporated in this technique . the major advantages of this technique , when compared with earlier ones , are minimal invasive incision , less postoperative discomfort , and excellent esthetic and emergence profile obtained . the aim of any soft tissue procedure should be to obtain a resilient and scar less soft tissue . this can be obtained with atraumatic tissue handling , minimal tension during approximation , adequate vascularity , adequate hydration , and expedient surgical procedures that do not compromise blood supply . this case report shows the excellent results following the above requisites to obtain esthetic soft tissue profile . the patients were followed for 2 years at regular intervals and still the esthetic results obtained during the surgery and after 2 years were the same [ figure 8 ] . with above considerations with respect to follow - up and esthetic component , this technique provided the required and desired results . in order to obtain esthetic papillary height and tissue integrity around implants proper and meticulous implant positioning is needed . being a long term follow - up report this technique can be considered as a simple , minimal invasive procedure , which can be followed during 2 stage surgery .

reconstructing predictable and esthetic papilla is the most complex and challenging aspect of implant dentistry . to obtain an esthetic and predictable gingival architecture and implant restoration , interdental papilla plays an important role . the main objective of the surgeon during the second stage of implant treatment should be the creation of interdental papilla prior to prosthetic restoration . the aim of this case report was to demonstrate a novel window technique for developing predictable and esthetic papilla around dental implants , which was followed for 2 years with excellent esthetic results .

cryptosporidiosis is a parasitic disease that causes diarrhea lasting for about 1 - 2 weeks among the immunocompetent and becoming a more severe life - threatening illness among immunocompromised individuals . it constitutes 24% of human immunodeficiency virus ( hiv ) positive patients with diarrhea compared with 6.1% in the immunocompetent individuals . association of cryptosporidiosis with malignancies is not as robust as acquired immunodeficiency syndrome ( aids ) . here , we report a case of cryptosporidiosis causing severe persistent diarrhea in a patient with multiple myeloma . the case we present here is the patient was a 64-year - old female who had been diagnosed to have multiple myeloma , stage iii b , type igg kappa 17 years ago in 1993 . she was being treated with oral lenalidomide ( 25 mg for 21 days ) and dexamethasone ( 40 mg on days 1 , 8 and 15 ) of 28 days cycle for the current progression of myeloma . the baseline serum m band prior to the start of this regimen was 3.4 g / dl . she presented on day 17 of the 2 cycle with the complaints of profuse watery diarrhea , 10 - 12 times / day for the previous 15 days . the diarrheal product was non - bloody , without any mucus and not foul smelling . this was associated with nausea , mild crampy abdominal pain and a sense of fecal urgency without any fever . there was anemia ( hemoglobin of 9.7 g / dl ) and leucopenia ( white blood cells count 2800/mm ) but the absolute neutrophil count ( 1700/mm ) and platelets were normal . the liver and renal function tests were within normal limits but serum potassium was low ( 2.2 meq / l ) . the routine blood culture was sterile and the polymerase chain reaction test in peripheral blood for cytomegalovirus deoxyribonucleic acid was negative . stool examination showed 2 - 3 polymorphonuclear leucocytes per high power field with mucus but no visible or occult blood . colonoscopy was performed , which suggested colonic mucosal inflammation likely to be infective in origin [ figure 1 ] . at this point , a diagnosis of cryptosporidial infection was suspected and the stool was submitted for examination with the modified kinyoun acid - fast stain . the patient was then started on oral nitazoxanide 500 mg 12 hourly for 10 days . the diarrheal frequency decreased after 5 days and it resolved completely by the 10 day of this drug . the patient was able to resume treatment with lenalidomide and dexamethasone later without any complications . colonic mucosal inflammation on colonoscopy of the patient oocysts of cryptosporidium parvum in modified kinyoun acid - fast stain cryptosporidiosis is a protozoal intestinal infection caused by many species of the genus cryptosporidium that are either obligate human or human and bovine parasites . the first association with human diarrheal disease was reported in 1976 , one case in an otherwise healthy child and one in an immunosuppressed adult . the infection is acquired by ingesting parasites in water or food contaminated by human or animal feces or by having contact with soil , a person , or an item that has been contaminated with the parasite . the diagnosis is established by demonstration of oocysts in the stool by the modified acid - fast kinyoun stain . since the initial reports four clinical syndromes of cryptosporidial infection have been described in hiv positive patients : chronic diarrhea ( affecting 36% of patients ) , cholera - like disease ( 33% ) , transient diarrhea ( 15% ) and relapsing illness ( 15% ) . the severity of illness is related to the cd4 count in these patients . in immunocompetent patients , usually children < 5 years of age the clinical presentation in our patient with severe diarrhea of 2 weeks duration that resulted in dehydration and dyselectrolytemia was more consistent with an underlying immunodeficiency which is expected in a patient with long standing multiply treated myeloma . although uncommon , cryptosporidial diarrhea has been described in patients with malignancy . in one study , the incidence of cryptosporidiosis was 1.3% in the 560 cancer patients presenting with symptoms of diarrhea out of which 71% had hematolymphoid malignancy . in other studies it was found that the risk of severe disease is largely limited to children with acute leukemia and lymphoma . interestingly , bone marrow transplantation does not seem to confer any additional risk over that of the underlying disease . cryptosporidial diarrhea should be included in the differential diagnosis of any patient with persistent severe diarrhea , especially in patients with hematological malignancies . diagnosis can be established by stool examination and administration of nitazoxanide is effective in controlling the infection .

cryptosporidiosis is a protozoal infection that leads to self - limited diarrheal disease in immunocompetent individuals and a more severe illness in immunocompromised patients especially those infected with the human immunodeficiency virus . although patients with hematolymphoid malignancies can develop this infection , it is an uncommon cause of diarrhea in these patients . the patient was a 64-year - old woman , a known case of multiple myeloma for 17 years , who had been treated with multiple lines of chemotherapy earlier . she was being treated with lenalidomide plus dexamethasone for active myeloma at the time of this episode . she presented with profuse watery diarrhea of 15 days duration that was proven to be due to cryptosporidium parvum on stool examination . the diarrheal illness resolved after treatment with nitazoxanide . although uncommon , cryptosporidial infection should be suspected in patients with hematological malignancies who have persistent diarrhea . stool examination with the modified acid - fast kenyoun stain establishes the diagnosis in the majority of cases . antiparasitic treatment is effective in controlling the infection .

histologic confirmation of this lesion is difficult , although it is very important for decision of treatment method.1 various methods , including endoscopic ultrasound - guided fine needle aspiration ( eus - fna),2,3 eus - guided trucut biopsy ( eus - tcb ) , endoscopic submucosal - mucosal resection ( esmr),4 and open surgery have been reported to be used for confirmation of the diagnosis.5 however , the best method for tissue acquisition has not been established yet . here , we report a case with a 12 cm submucosal signet ring cell type adenocarcinoma , diagnosed by eus - tcb and immunochemical studies . a 57-year - old man presented with epigastric pain and a 6 kg weight loss over 3 months . laboratory tests at admission showed white blood cell count of 13,050/mm ( normal range , 4,800 to 10,800 ) , hemoglobin 14.2 g / dl ( 13 to 18 ) , carcinoebryonic antigen 1.5 ng / ml ( 0 to 5 ) , and ca19 - 9 107.3 u / ml ( 0 to 36 ) . the endoscopy showed an intraluminal protruding lesion covered with normal mucosa along the anterior wall of the lower body , antrum , and duodenal bulb ( fig . a computed tomography of the abdomen showed about a 1012 cm exophytic mass at the antrum and lower body of the stomach , with invasion to the liver , pancreas , and transverse colon ( fig . 2 ) . eus with a radial endoscope ( ue 260 ; olympus , tokyo , japan ) was performed subsequently to assess the gastric wall and evaluate the characteristics of the mass . the eus showed a heterogeneous echogenic texture with multiple hyperechoic deposits and anechoic necrotic zones inside the large tumor mass that was thought to have developed in the fourth hypoechoic layer ( muscularis propria ) . however , the mucosal and submucosal layer were intact , and the extraluminal border could not be assessed due to the very large size of the mass ( fig . 3 ) . a non - operable malignant gastrointestinal stromal tumor ( gist ) was first suspected . therefore , eus - tcb with a linear endoscope ( uct240 ; olympus ) was performed for rapid pathology to confirm the clinical suspicion with immunohistochemical staining and to save costs . the signet ring tumor cells were immunoreactive for cytokeratin ( ck , 200 ) ( fig . 4 ) . chemotherapy with ts-1 ( ts - one ; jeil pharmaceutical co. , seoul , korea ) plus iv cisplatin ( cisplatin ; ildong pharmaceutical co. , seoul , korea ) was started . ts-1 was given orally at a dose of 40 mm / m twice daily for 2 weeks followed by a 1-week rest , and cisplatin was given intravenously on day 1 at a dose of 60 mg / m . during the admission , a percutaneous drainage ( pcd ) tube the general condition of the patient deteriorated rapidly after about 2 weeks from admission and the patient died 2 months after the diagnosis . this case report of a patient with gastric primary signet - ring cell carcinoma with features of gist was confirmed by eus - tcb . gastric cancers have various endoscopic findings , and histology is used to confirm the diagnosis.6 however , it is often difficult to confirm the histologic diagnosis despite taking a biopsy specimen , because the tumor surface of gastric cancers mimicking a submucosal tumor ( gcsmt ) is covered by normal mucosa.1 gcsmts are very rare ; they account for 0.1% to 0.63% of all resected gastric cancers.7 moreover , the histological confirmation of a signet ring cell type is uncommon . eus alone can provide useful information on gcsmts;8 however , it is difficult to determine the histological nature of the lesions from the eus image alone.9 the methods used to overcome this problem include eus - fna,2,3 eus - tcb , esmr,4 laparoscopic excision biopsy,10 and open surgery.5 however , the best method for confirmation of the diagnosis has not been established yet . for a definite diagnosis of a submucosal tumor , tissue acquisition , and pathology confirmation recently , mekky et al.11 reported that the results of eus - fna in 141 patients with gastric smts were diagnostic , suspicious , and nondiagnostic in 43.3% , 39% , and 17.7% of cases , respectively , with an overall accuracy of 95.6% and the accuracy of differentiating potentially malignant lesions of 94.2% . sftoiu et al.12 reported that the yield of adequate tissue sampling was similar for eus - fna and eus - tcb ( 96.4% vs. 89.3% , p = not significant ) . however , the accuracy for obtaining a specific diagnosis was significangly lower for the eus - fna compared to the eus - tcb ( 5.3% and 68.4% , p<0.005 ) . cantor et al.13 suggested that the esmr has a significantly higher diagnostic yield than jumbo forceps biopsy with the use of the bite - on - bite technique for the evaluation of subepithelial lesions limited to the submucosa . the diasgnositc yield of the jumbo forceps biopsy was four out of 23 cases ( 17% ) compared to 20 out of 23 cases ( 87% ) of the esmr ( p<0.001 ) . the esmr , however , accompanies major complications such as bleeding ( 0% to 24% ) and perforation ( 0% to 5% ) . therfore , the eus - tcb was performed in this case for rapid pathology in order to confirm the clinical suspicion with immunohistochemical staining , with a relatively lower complication rate . in conclusion , this is the first report of the eus - tcb used as a diagnostic tool in a case of gastric primary signet - ring cell carcinoma with features of gist . eus - tcb has low complication rate and enables immunohistochemical staining unlike other methods such as eus - fna , esmr , or open surgery , which is why it should be considered when results of a mucosal biopsy are not diagnostic . the possibility of signet ring cell carcinoma , which has a poor prognosis , should always be considered even in cases with gcsmt developing in the fourth gastric layer .

a submucosal gastric adenocarcinoma , especially the signet ring cell type , is rare . the histologic evaluation techniques for this lesion has not been established ; however , histologic confirmation is very important for decision of treatment method . here , we report a 57-year - old man with a 12-cm gastric submucosal signet ring cell type adenocarcinoma , diagnosed by an endoscopic ultrasound - guided trucut biopsy and immunochemical studies . this case suggests that the endoscopic ultrasound - guided trucut biopsy might be a useful diagnostic method in cases of gastric adenocarcinoma with features of gastrointestinal stromal tumor .

oncocytes are defined as modified epithelial cells with characteristic finely granular eosinophilic cytoplasm , which are abundant in mitochondria [ 1 , 2 , 3 , 4 ] . tumors composed of oncocytes have been described in the major and minor salivary glands , the thyroid , parathyroid , pituitary , and adrenal glands as well as in the kidneys , testes , pharynx , larynx , trachea , pancreas , respiratory tract , skin , and other organs [ 1 , 5 ] . in the vast majority of cases , oncocytic lesions of the ocular adnexa occur in the caruncle ; other locations rarely include the conjunctiva , plica semilunaris , lacrimal sac , eyelid , and lacrimal gland [ 1 , 4 , 6 ] . oncocytic lesions occur more commonly in elderly patients , usually in their seventh decade of life , with a 2:1 female : male ratio , especially for those occurring in the caruncle [ 1 , 5 , 6 ] . the most common presenting symptom is a mass or lump , although patients are usually asymptomatic [ 4 , 5 ] . oncocytic lesions of the eye are generally benign , and complete surgical excision is the preferred treatment since it has often proved to be curative . a 69-year - old woman was referred to the ocular oncology service for the evaluation of a pigmented mass in the caruncular region of the right eye . the patient was regularly followed for chronic blepharoconjunctivitis in both eyes and dry eye syndrome , treated with lubricant eye drops . the patient took daily medications for hypertension and was suspected to have rosacea . aside from this , her personal medical history and family history were unremarkable . the patient complained of a tan - colored lesion on her right caruncle , but denied symptoms of bleeding , discharge , an increase in size , or a color change of the lesion . on ocular examination , visual acuity was 20/20 and intraocular pressure was 16 mm hg in both eyes . slit lamp biomicroscopy revealed a well - circumscribed , partially pigmented lesion in the caruncle of the right eye . no involvement of the lacrimal system was noted , and the dilated fundus examination of both eyes was unremarkable . the patient was diagnosed with conjunctival nevus of the right caruncle . due to the lesion 's unfavorable location , histopathological examination disclosed a benign , cystic , tubular , well - circumscribed lesion composed of tall , columnar , granular eosinophilic cells ( fig . 1b ) . a thin fibrous tissue capsule penetrating the tumor from the periphery was also present . the pigmented macroscopic appearance of the lesion was thought to be due to concretions within the tumor ( fig . 1b ) . a diagnosis of oncocytoma of the caruncle was made an oncocytoma is a rare benign tumor that usually arises in the glandular epithelia of tissues throughout the body . among cutaneous sites , oncocytomas have also been known as oxyphil adenomas , papillary cystadenomas , adenolymphoma - like tumors , and warthin 's tumor . the lesion is usually composed of large oval cells with eosinophilic granular cytoplasm filled with malformed mitochondria , most likely representing an age - associated metaplastic and neoplastic transformation of the glandular epithelium . clinically , caruncular oncocyto - mas occur more frequently in female elderly patients [ 1 , 4 , 6 ] and usually present as a mass or lump that rarely shows a tendency to grow . they are commonly red - blue or orange tan in color , sometimes with a lobulated , fleshy , or cystic appearance . histopathologically , oncocytomas have been attributed with four main architectural patterns : cystic - micropapillary , confluent - glandular , solid - organized , and solid - disorganized . benign lesions are commonly described as having a cystic - micropapillary pattern , while potentially aggressive growth is associated with solid patterns . reported atypical features of oncocytomas include the presence of pigmentation , feeder vessels , keratinization , a pedunculated growth pattern , and superficial lobulations . furthermore , it has been reported that oncocytomas that are not dark blue in color are more likely to be misdiagnosed preoperatively . surgical treatment outcomes are excellent for benign oncocytic neoplasms , with no recurrence found after surgical excision [ 1 , 4 ] . several entities must be taken into consideration in the differential diagnosis of carun - cular lesions . nevi ( 40.7% ) , squamous papillomas ( 8.5% ) , and oncocytomas ( 5.1% ) are the most common lesions at this body site . more specifically , oncocytomas are clinically often mistaken for hemangiomas , nevi , and cysts . the case we reported showed several atypical characteristics : the lesion was partially pigmented and lobulated and its macroscopic appearance resembled that of a conjunctival nevus . conjunctival nevi tend to remain stationary throughout life , with less than 1% risk for transformation into malignant melanoma . nevertheless , malignant melanoma of the conjunctiva is a potentially life - threatening tumor . tumors found in unfavorable locations such as the palpebral conjunctiva , fornices , plica , caruncle , and lid margins have been associated with a higher rate of metastatic death . given this knowledge , nevertheless , complete surgical excision had already been performed , and no further treatment was required . at follow - up after 12 months , the patient remained asymptomatic , with no signs of recurrence of the oncocytoma . in conclusion , although nevi are the most common lesions present in the caruncle , onco - cytoma should be considered in the differential diagnosis of a pigmented caruncular mass , especially in elderly female patients .

we report an unusual case of a pigmented oncocytoma of the caruncle . the lesion presented the following atypical features : a well - circumscribed , partially pigmented , and lobulated mass that macroscopically resembled a caruncular nevus . however , an excision biopsy revealed an oncocytoma instead . thus , although nevi are the most common lesions found in the caruncle , oncocytoma should also be considered in the differential diagnosis of a pigmented caruncular mass , especially in elderly female patients .

to report a case of bilateral spontaneous anterior lens dislocation associated with retinitis pigmentosa ( rp ) . a 45-year - old male with rp presented with elevated intraocular pressure ( iop ) in the right eye and was treated with laser iridotomy ( li ) . surgical intervention , including anterior vitrectomy , intracapsular cataract extraction ( icce ) , and iol scleral fixation was performed . two years later , the same episode occurred in his left eye and a similar treatment was done . this is the first report of bilateral spontaneous anterior lens dislocation in a rp patient . a 45-year - old korean male presented on march 27 , 2003 with right ocular pain . right and left iop , measured by a goldman applanation tonometer , was 66 and 11 mmhg , respectively . right and left eye best corrected visual acuity ( bcva ) was 20/70 with -4.50 + 1.00165 and 20/100 with -3.50 + 1.00180 , respectively . right eye slit lamp examination showed steamy opaque cornea and a central anterior chamber depth of 2 corneal thickness ( ct ) . , bone - spicule pigmentation sparing macula , and pale optic disc was noted in the right eye ( fig . the patient was given intravenous 15% mannitol ( 300 ml ) , oral acetazolamide ( diamox ) ( 500 mg ) , oral 50% glycerin ( glycerol ) ( 50 cc ) , carbonic anhydrase inhibitor ( cosopt ) , and 0.2% brimonidine ( alphagan ) eye drops . one day after treatment right eye iop decreased to 50 mmhg , the cornea became clear , and the central anterior chamber depth remained 2 ct . six days later , the cornea was clear , the anterior chamber was deep and clear bilaterally , and the right eye li opening was patent . using the humphrey visual field ( model 750 , humphrey instruments , inc , dublin , california ; c24 - 2 program ) 2 ) , and electroretinogram findings were prolonged in a and b amplitudes ( fig . the right eye li opening was patent , and the anterior chamber was deep and clear . six months later , on december 19 , 2003 , the patient presented with right ocular pain . for the surgical procedure , pupillary constriction was achieved using 4% pilocarpine , administered 3 times in 5 minute intervals . a 7 mm wide scleral tunnel was made 1.5 mm posterior to the limbus at the superotemporal side . healon gv was injected into the anterior chamber to create space between the posterior lens capsule and iris . icce was done successfully using capsule forceps . prolapsed vitreous in the anterior chamber was removed by vitrector . a posterior chamber lens ( alcon cz70bd ) was introduced and sutured to the sclera by the ab interno method . three days postoperatively , the iol was well positioned and no abnormal ocular findings were noted . two years later , on june 8 , 2005 , the patient presented with left ocular pain lasting one day . right and left bcva was 20/50 with -1.50 + 2.00165 and 20/160 with -3.50 + 1.0010 , respectively . slit lamp showed anterior lens dislocation with mild inflammatory cells , and there were no abnormal findings in the right eye ( fig . 4 ) . anterior vitrectomy , icce , and iol scleral fixation was done on the left eye . four weeks post - operatively , left bcva and iop was 20/200 with -1.50 + 1.2515 and 10 mmhg , respectively . the causes of ectopia lentis are trauma , pseudoexfoliation syndrome , spontaneous dislocation , and various heredofamilial diseases including marfan syndrome , homocystinuria , and rp.4,5 in rp patients , hayashi et al . has suggested that zonular weakness can cause ectopia lentis6 and namiki et al . showed unilateral zonular dehiscence.7 we report the first case of anterior lens dislocation in an rp patient following high iop . after glaucomatous attack , mid - dilated pupil occurred with a weakened iris and ciliary body ; these structure caused anterior segment lens dislocation . the mechanisms of glaucoma associated with a dislocated lens are pupillary block , lens degenerative changes , and concomitant anterior chamber angle damages.8 allingham et al . these include pupillary block by subluxating the lens posterior to the iris , lens incarceration directly within the pupil , and complete lens dislocation into the anterior chamber.9 in this case , iop elevation was due to posterior pupillary block , which responded dramatically to laser iridotomy . if the lens is displaced into the anterior chamber , it may be possible to relieve the condition by dilating the pupil and allowing the lens to reposition into the posterior chamber . however with a totally dislocated lens in the anterior chamber , it is better to constrict the pupil and surgically remove the lens . choi et al . have treated anteriorly dislocated lens by lensectomy , vitrectomy , phacoemulsification , and ab externo scleral iol fixation.10 for scleral fixation , young et al . used a scleral tunnel to relieve iol fluctuation and to maintain anterior chamber depth.11 in this case , the anteriorly dislocated lenses were removed using a 7 mm wide scleral tunnel , combined with an anterior vitrectomy and ab interno scleral fixation . this is the first case of bilateral spontaneous anterior lens dislocation in an rp patient reported in the literature .

purposeto report a case of bilateral spontaneous anterior lens dislocation associated with retinitis pigmentosa ( rp).methodsa 45-year - old male with rp presented with elevated intraocular pressure ( iop ) in the right eye and was treated with laser iridotomy ( li ) . after li , complete crystalline lens dislocation into the anterior chamber occurred . surgical intervention , including anterior vitrectomy , intracapsular cataract extraction ( icce ) , and iol scleral fixation was performed . two years later , the same episode occurred in his left eye and a similar treatment was done.resultssurgery was successful in both eyes.conclusionsthis is the first report of bilateral spontaneous anterior lens dislocation in a rp patient .

messori and colleagues are to be congratulated on their interesting report on the italian observational study of antithrombin iii ( at iii ) use in intensive care units . audits of this sort are difficult and time consuming to conduct . observational studies are also difficult to analyse and interpret because of the heterogeneity of real - life patient populations , the lack of standardised treatment regimens , the lack of standardised indications for treatment , and the lack of predefined endpoints for assessing survival ( e.g. 28 day survival or in - hospital mortality ) . for these reasons we take issue with messori and colleagues over the very strong conclusion they draw on the basis of their survey . in our opinion , this nonrandomised observational study is not able to provide scientific evidence to support the authors ' statements . as pointed out by pocock and elbourne , observational studies have one crucial deficiency : the design is not an experimental one . experimental design involves randomisation , the use of entry criteria , and the rigorous use of standard definitions of index medical conditions such as disseminated intravascular coagulation and septic shock . none of these are to be found in the survey by messori and colleagues . in the absence of rigorous experimental methodology , it is not possible to be sure that the findings of an observational study are predictive of the results of a randomised , controlled trial ( rct ) . the published finding that observational studies usually agree with rcts comes from studies in indications unrelated to sepsis and septic shock . furthermore , it is a logical fallacy to suggest that agreement in one direction implies prediction in the other direction . observational studies are not scientifically capable of proving or disproving any hypothesis . with this in mind , it is interesting to note that one of the main observations of messori and colleagues ' survey ( n = 56 for sepsis ) is at variance with the results from a very much larger rct reported in the same indication by warren and colleagues ( n = 2341 for sepsis ) . whereas warren and colleagues ' rct found significantly lower mortality in a prespecified subgroup analysis of at iii - treated patients not receiving heparin ( n = 352 ) compared with placebo - treated patients not receiving heparin ( n = 346 ) , messori and colleagues ' survey observed an increase in mortality in patients not receiving heparin . this casts serious doubt over the scientific value of a survey reporting outcomes in a treatment that was not randomised to patients , with only 56 patients with sepsis treated with at iii . such a type of study is not scientifically or statistically capable of proving causality or supporting statements . other crucial weaknesses of messori and colleagues ' survey lie in its observational nature and the lack of any standardisation of definitions or outcomes . controlled studies usually indicate the 28 day mortality whereas observational studies indicate the hospital mortality , which normally is higher than the 28 day mortality . in addition , hospital mortality may depend on local factors affecting clinical practice such as when to discharge patients from hospital , which varies among countries . messori and colleagues also report the results of a meta - analysis of four studies of at iii in sepsis . it is probably unwise , however , to place too much weight on meta - analyses of studies conducted in critical care settings because the spectacular failure of meta - analyses to predict the outcomes of subsequent large - scale rcts is well known . furthemore , if confidence intervals overlap or p > 0.05 , this does not prove that there is no difference ; on the contrary , there is still a possibility that the observation suffers from a type ii error ( failure to detect a true difference due to inadequate sample size ) . this is particularly likely to occur when the number of patients studied is small , as in the phase ii trials on at iii . in this context , it is misleading of messori and colleagues to use figure 1 of their paper to suggest , with reference to the confidence intervals , that there is no difference between the studies or that their observational study has a very similar result to the other studies cited . in the same way , potentially misleading claims are made about the subgroup analysis of the effects of coadministered heparin . in the results section it is suggested , erroneously , that no differences exist between results on the basis of p values alone . surveys and audits are very important ways of documenting routine clinical practice and of confirming the implementation of guidelines based on the results of rcts . surveys and audits are also useful for generating hypotheses , but they are not a substitute for rcts when proving or disproving hypotheses . messori and colleagues have raised interesting concerns but they have not provided answers to those concerns . the author has participated as a clinical investigator in the kybersept trial and served as coordinator in a basic research program of the university of innsbruck on anti - inflammatory mechanisms of antithrombin , which was in part supported by a grant from aventis behring gmbh ( marburg , germany ) .

observational studies usually agree with randomised , controlled trials . it is a logical fallacy , however , to suggest that agreement in one direction implies prediction in the other direction . observational studies are not scientifically capable of proving or disproving hypotheses such as the efficacy and safety of the treatment of severe sepsis with antithrombin . observational studies are difficult to analyse and interpret because of the heterogeneity of real - life patient populations , the lack of standardised treatment regimens , the lack of standardised indications for treatment , and the lack of predefined endpoints .

pulmonary sequestration ( ps ) is a rare developmental disorder characterized by the presence of a nonfunctioning lung parenchyma that lacks normal tracheobronchial tree connection and is supplied by a systemic artery.1 congenital diaphragmatic hernia ( cdh ) is a malformation characterized by a diaphragmatic defect through which the abdominal viscera migrate into the chest during fetal life.2 the association between ps and cdh has been previously described.1 2 3 4 5 6 7 we report the case of a neonate affected by prenatally diagnosed cdh treated by thoracoscopy . during the procedure , we detected an associated extralobar ps , which was preoperatively undiagnosed , and removed it . a female infant was delivered by elective cesarean section at 35 weeks of gestation and was referred to our department for prenatal diagnosis of left diaphragmatic hernia detected at 32 weeks of gestation by ultrasounds and characterized by a lung - to - head ratio ( lhr ) of 1.6 . weight at birth was 3,400 g , and apgar index was 9 at 1 minute and 10 at 5 minutes , respectively . at birth , the newborn underwent nasotracheal intubation and high - frequency oscillatory ventilation , positioning of a nasogastric tube , and chest x - ray that confirmed prenatal diagnosis . during the first day of life , the patient maintained clinical stability , so we decided for surgical correction through thoracoscopic approach on the second day of life . a 5-mm trocar was positioned in the fifth intercostal space over the middle axillary line , and two further operative trocars ( 3 mm , reusable ) were placed in the sixth intercostal space over the anterior and posterior axillary line , respectively . capnothorax was created with a 6mmhg pressure and 0.5l / min flow , and maintained with intermittent insufflation to reduce side effects of co2 absorption . once entered in the pleural cavity , we noted a diaphragmatic hernia with a sac . a basal extralobar sequestration was also detected and removed after ligation of the feeding artery with two endoloops ( fig . the diaphragmatic defect was repaired with an interrupted 3/0 braided polyester suture ( ethibond ) ( figs . 2 and 3 ) . the patient underwent first follow - up chest x - ray after 2 months , and she is currently asymptomatic without radiologic signs of recurrence ( 6-month follow - up ) . ps includes two forms : intralobar ps ( within the normal parenchyma ) , which is the most common form , and extralobar pulmonary sequestration ( eps).1 eps has its own pleural covering and can be located inside or outside the thorax . the associations are more common in the extralobar type and include congenital pulmonary airway malformation , configuring a lesion termed as hybrid lesion , congenital heart disease , and cdh in up to 15 to 40% of cases.7 8 9 10 a possible explanation is the interference related to the presence of a sequestered lung mass with the fusion of diaphragmatic components ( mechanical obstacle).7 11 in recent years , there has been speculation on the prognosis associated with the two simultaneous defects ( cdh and ps).7 ps can be associated with pulmonary hypoplasia , and being a space - occupying lesion , it could constitute a further obstacle to lung development.3 8 indeed , the prognosis of ps combined with cdh remains unclear , and although there is not sufficient evidence regarding the influence of ps on cdh prognosis , some authors hypothesized that ps acts as a protective factor during pregnancy and probably improves the prognosis of patients with cdh.7 two possible explanations for this improved outcome may be given : ( 1 ) ps acts as an anatomical barrier to the rise of the abdominal viscera ; ( 2 ) ps causes a mediastinal shift leading to an underestimation of lhr , thus distorting the prognosis that seems poorer.7 prenatal identification of both cdh and ps is possible , and frequently occurs.7 in our case , prenatal diagnosis of concurrent cdh and ps was not possible probably because herniated organs obscured the presence of a sequestered lung mass . the treatment of choice of a symptomatic eps in addition to a cdh is surgical excision.1 2 however , some authors recommend observation of asymptomatic eps , as these lesions rarely produce complications.12 we believe that resection should be the treatment of choice in all cases since there are hybrid lesions misdiagnosed as pure sequestrations and one can never be sure that the lesion will remain asymptomatic.4 the most frequent argumentation for removal is the prevention of recurrent infections and malignancy . malignancy has been reported to be associated to cystic lesions , in particular to type i congenital cystic adenomatoid malformation in 1% of cases.12 although the risk is quite low , in case of recurrent infections or neoplastic evolution , the previous wait and see approach would be difficult to justify . regarding the surgical approach , minimally invasive alternatives to the traditional surgical technique have been recently proposed , although the best approach to cdh is controversial.2 recently , hypercapnia and acidosis during thoracoscopy in newborns have been reported.13 14 in our case , we used co2 insufflation at the beginning of the procedure to facilitate reduction of herniated organs only for brief periods to improve visibility . the distinction between a diaphragmatic hernia with sac and a congenital localized eventration may be difficult . sac removal seems to be associated with a lower recurrence rate,15 but during thoracoscopy the presence of a sac can facilitate reduction , can lessen visceral trauma , and keeps the two cavities separate.2 according to a recent survey among the international pediatric endosurgery group members,16 22% of surgeons declared to leave the sac in place during the thoracoscopic repair of cdh , so this approach is quite accepted even if in a minority of cases . accurate closure of this vessel is mandatory to avoid postoperative bleeding , which can be life - threatening.17 in case of eps , vessel sealing can be achieved with different devices such as ligasure , clips , and ligation . in this case , since the eps was of limited size , we closed the feeding artery with two endoloops and cut the vessel with cold scissors to avoid thermic damage of the ligatures . we would probably have missed the ps using a different surgical approach ( classical laparotomy ) , as during the laparotomic approach the posterior position of the diaphragmatic defect makes it hard to inspect the entire hypoplastic lung that is atelectatic and displaced in the upper part of the chest . the thoracoscopic approach for cdh offers the advantage to easily diagnose and treat associated lung lesions . the case report describes the usefulness of thoracoscopy as diagnostic and therapeutic tool in case of diaphragmatic hernia associated with undiagnosed lung malformation . thoracoscopy allows to explore thoracic cavity looking for lung lesions that are associated to diaphragmatic hernia in 1540% of cases and to remove them .

we report the case of a neonate affected by prenatally diagnosed congenital diaphragmatic hernia ( cdh ) , treated by thoracoscopy . during the procedure , we detected an associated extralobar pulmonary sequestration ( eps ) , which was preoperatively undiagnosed , and we removed it . eps has been shown to be associated with cdh in up to 15 to 40% of cases . a possible explanation hypothesized is that pulmonary sequestration , which develops at 4 to 5 weeks of gestation , can disturb the fusion of the diaphragm and closure of the pleuroperitoneal canal . the thoracoscopic approach permitted us to identify the thoracic eps that probably would have been otherwise missed .

transversetesticular ectopia ( tte ) is a rare form of testicular ectopia in which one of the testes crosses midline and occupies contralateral side of hemiscrotum . tte with persistent mullerian duct syndrome usually presents as undescended testis with contralateral inguinal hernia . laparoscopy is being increasingly used for diagnostic as well as therapeutic management of undescended testis . a 4-month - old male child was brought to uswith complaint of pain in the left inguinal region for 3 days . ultrasonography showed funiculitis of the left cord structures and 1.5 1 cm testis - like structure present just above the left testes . after recovery of funiculitis , we posted the patient for diagnostic laparoscopy followed by definitive repair . during the procedure , a 5 mm port was inserted at umbilicus forthe camera and two working ports were inserted 5 cm away on either side of the umbilical . right - sided testicular vessels crossed from right to left , entering in left internal ring [ figure 1 ] . we divided the uterus in midline with help of bipolar cautery [ figure 3 ] . after mobilization of right testicular vessels we found adequate length to bring it out through right - sided internal ring . both testes brought out and fixed in the subdartous pouch . left - sided hernial sac closed with intracaropreal suturing . photograph showing right - sided testicular vessels crossing midline to left and entering into internal ring photograph showing uterus in between two testis photograph showing division of uterus in midline we successfully managed transverse testicular ectopia with persistent mullerian duct syndrome with hernia laparoscopicaly . on 6 months of follow - up , transverses testicular ectopia is a rare form of testicular ectopia in which testis is present in contralateral side of hemiscrotum . the first case is reported by lenhossek in 1886 , he found it during an autopsy of adult man . gupta suggested that adherence of developing wolffian duct with mullerian duct causes descend of both testis on the same side . three types of tte are described : ( 1 ) tte with hernia ( 4050% ) , ( 2 ) tte with pmds ( 30% ) , ( 3 ) tte with hypospadias , pseudohermaphrodite , or some complexity ( 20% ) . classified pmds into three groups : group a - the testes are in the position of the normal ovaries ( female type ) ; group b - one testis is found in a hernia sac orscrotum along with the uterus and tubes ( male type ) ; group c - both testes are found in the same hernia sac with associated tubes and the uterus ( male type ) . our patient belongs to group c. most common presentation is the absence of testis on the one side with inguinal hernia on opposite side . ultrasonography and magnetic resonant imaging helps to diagnose this condition but most of them are diagnosed at the time of surgery . various methods to manage this condition have been described , like herniotomy with trans - septal fixation of testis , that is , ombredanne technique , fixation on opposite side through suprapubic subcutaneous tunnel , and staged procedure . dean and shah reported laparoscopically assisted correction of tte . in 2007 , evans reported total laparoscopic correction of tte . uniqueness of our case is that our case was having tte with hernia and pmds , which was totally managed by laparoscopy . division of uterus in midline and mobilization of cord and vessels carried out by laparoscopy .

a 4-month - old male child presented with right undescended testis and left inguinal hernia with funiculitis . ultrasonography showed funiculitis on the left side testis along with presence of 1.5 1 cm testis like structure just above left testis and empty right scrotal sac without any evidence of mullerian structures . on diagnostic laparoscopy , right testicular vessels were crossing from right to left and had uterus with both testes in left hernia sac . mobilization of vessels , division of uterus , and hernia repair was done laparoscopically . on the review of literature , there is only one case report of total laparoscopic repair of transversetesticular ectopia ( tte ) with hernia without persistent mullerian duct ( pmds ) . the uniqueness of our case is that it had tte with hernia and pmds , which were totally managed by laparoscopy . on 6 months of follow - up , both the testes are palpable in scrotum .

alopecia areata ( aa ) is a chronic inflammatory disease of the hair follicles and nails , its etiology is unknown , probably multifactorial with evident autoimmune and genetic components . the first clinical description of aa is attributed to celsus ( 14 - 37 b.c . ) and the designation aa was given by sauvages . the importance of genetic factors in aa is underlined by the high frequency of a positive family history in affected individuals . in most reports , these range from 10% to 20% of cases . a study in monozygotic and dizygotic pairs found a concordance rate of 55% for alopecia amongst monozygotic twins with no concordance among the dizygotic pairs . though it can affect any age group , demographical data on aa is still lacking . two brothers aged 5 and 7 years presented to the outpatient department with complaints of asymptomatic patchy loss of scalp hair for the past 2 and 4 months respectively . there was no similar complaint in any other family member , siblings and no history suggestive of systemic involvement , drug intake , trauma , pus filled lesions or any other skin eruptions . mucocutaneous examination revealed multiple smooth patches of alopecia ranging from 2 cm 2 cm to 4 cm 6 cm over occipital and vertex area of elder child and multiple patches over occipital and temporal region measuring 2 cm 2 cm to 2 cm 4 cm of the younger one [ figure 1a and b ] . ( a ) multiple areas of nonscarring alopecia involving the occipital and vertex area in a 7-year - old child ( b ) multiple areas of nonscarring alopecia involving the occipital and temporal area in sibling 5-year - old surface of the patches was smooth with no apparent changes . histopathological examination of the biopsy sample from the alopecia patch from the scalp confirmed the diagnosis , which showed inflammatory infiltrate in and around the bulbar region of hair follicle . alopecia areata is t cell mediated autoimmune disease with genetic predisposition resulting in partial and total nonscarring alopecia . scalp is the predominant site of involvement with the most common clinical pattern involving multiple areas of patchy hair loss . most patients develop aa before the age 40 years with 11 - 20% of all cases occurring in children . the ratio of male : female in aa in pediatric age group is 1:1 where as in adolescent and adult it is more common in female . human leukocyte antigen ( hla)-dq3 and hla - dqb1fnx0103 alleles appear to be marker for genetic susceptibility to aa with the latter serving as a special genetic marker for more severe variants . the diagnostic pathologic feature is peribulbar lymphocytic inflammation ( swarm of bees ) predominately cd4 + cells along with cd8 + t cells affecting anagen follicles or follicles in early catagen . short , easily extractable broken hairs , known as exclamation mark hairs , are often seen at the margins of the bald patches during active phases of the disease . the scalp is the first affected site in most cases , but any hair - bearing skin can be affected . the term alopecia totalis is applied to total or almost total loss of scalp hair , and alopecia universalis is the loss of all body hair . children with aa demonstrate an increased levels of activated t cells leading to various autoimmune diseases like vitiligo , thyroiditis , connective tissue disorders , lichen planus , type 1 diabetes , and pemphigus foliaceous . geometric and superficial pits are typical of aa whereas deep and irregularly distributed pits are seen in psoriasis and atopic dermatitis . familial aggregation of aa has been studied and it has been found that estimated lifetime risk has been 7.1% in siblings , 7.8% in parents , 5.7% in offspring . aa has been also reported after allogenic bone marrow transplantation from an affected , hla matched sibling . menon and kiran reported a case of concomitant occurrence of aa in the sibling with emphasis on environmental precipitating factors . evidence based management of aa in children is limited due to the lack of well controlled randomized studies . reassurance should be given to patients with limited disease as spontaneous remission occurs in 80% of patients . various treatment options tried in aa are topical , intralesional , and systemic corticosteroids , topical immunomodulators , and topical irritants like dithranol , psoralen plus ultraviolet a therapy , excimer laser therapy .

alopecia areata ( aa ) is a t cell mediated autoimmune disease with multifactorial etiology resulting in partial and total nonscarring alopecia . we hereby report a case of two brothers ( aged 7 and 5 years ) presenting with coincidental aa over scalp which is a rare presentation .

mllerian anomalies are congenital defects of the female reproductive tract resulting from failure in the development of the mllerian ducts and their associated structures symptoms appear principally during adolescence or early adulthood , and affect the reproductive capacity of these women . when clinically suspected , investigations leading to diagnosis include imaging methods such as hysterosalpingography , ultrasonography , magnetic resonance imaging , and cystoscopy . a 25-year - old nulliparous lady , presented to the gynecology opd with complaints of inability to conceive . she was married for 7 years , cohabiting since 5 years . at 16 years of age , she consulted a local doctor in her village for primary amenorrhea and cyclical pain in abdomen , where some operative procedure was done . since then the patient has passage of blood mixed urine periodically every month for 2 to 3 days with suprapubic abdominal pain . external urethral meatus appeared dilated , and there was blindly ending vagina approximately 3 cm in length . on per rectum examination , uterus appeared normal in size and there was no bulge felt below it to suggest any collection . on investigations , the ultrasound showed normal uterus with endometrial thickness 9.5 mm , ovaries were normal , and bilateral kidneys were also normal . mri of abdomen showed normal uterine body and cervix with small amount of fluid in endometrial cavity , endocervical cavity , and in upper part of vagina ; below that there was a transverse vaginal septum of about 3.5 cm in thickness . [ figure 1 ] on cystoscopy , a fistulous opening of approximately 2 mm was seen below the internal sphincter of urethra through which menstrual blood was seen coming out ; the bladder and ureteric orifices were normal . vaginoplasty and repair of fistulous communication between the genital tract and the urethra was planned for the patient . the mri shows uterus with cervix and upper 2 cm of vagina containing some blood products . there is a septum between the upper and lower part of vagina ( the lower part identified by tampon in vaginal canal ) the woman was subjected to operation using abdominoperineal approach . on laparotomy , uterus was normal in size , bilateral tubes were present , and ovaries were normal . the methylene blue dye was injected into the fundus of the uterus ; dye was visualized coming out of urethra by the side of the indwelling catheter . from the perineal approach , a transverse incision was made through the vault of the short vagina , the dissection was done upward in the connective tissue between the bladder above and rectum below and vaginal space was created . after sharp and blunt dissection , cervix was visualized as dye was coming through it . the urethrovaginal fistulous tract was identified and repair was done in two layers . over the raw area of vagina , the mould change was done after a week ; the amnion graft had successfully taken up . the patient was found to be fine at follow - up after a month , with no hematuria and normal menstruation . she was explained about the fertility period and asked to come at 3 monthly follow - up . when a mullerian duct becomes obstructed , the patient may present with an abdominal mass and dysmenorrhea . if the patient is not treated in a timely fashion , the consequences can be severe , extending even to infertility . in this case , there was transverse vaginal septum in the upper part of vagina leading to hematocolpus and amenorrhea . an iatrogenic fistulous tract was probably created which led to cyclical hematuria . magnetic resonance imaging ( mri ) is the mainstay in imaging for the evaluation of mullerian agenesis and is considered a very useful diagnostic tool . in our case , mri determined the precise mullarian anomaly giving us the details regarding the presence of normal cervix with precise length of vaginal septum to help us judge the prognosis and success of the operative procedure , but we got no information about the fistulous communication as it was quite fine and narrow . the site of the opening of fistulous tract in the urethra was apparent on cystoscopy and led to the assumption that the other end tract opened in the vagina . therefore , apart from mri , cystoscopy played a major role in determining the anomaly in this case . upon literature review of cases with genitourinary fistula the fistula in that case , however , was congenital , unlike our case in which it was iatrogenic in all probability . it also presented with cyclical hematuria with no incontinence of urine due to imperforate hymen . congenital vesicouterine , cervicovesical , and vesicovaginal fistula associated with other mullarian anomalies has also been reported in literature.[46 ] iatrogenic fistulous communication between the genital tract and the urinary tract , known to occur as a complication of cesarean section , has been described in literature as youssef 's syndrome . it is characterized by cyclical hematuria , absence of vaginal bleeding , and complete urinary continence . in all these reports in which there was a fistulous communication between the urinary and the genital tract , cystoscopy , hysterosalpingography , and dye test were required for confirmatory diagnosis and only computerized tomography ( ct ) or mri did not give the full clinical picture . this report highlights the diagnostic and therapeutic challenge faced by the clinician in dealing with a fistulous genital and urinary tract communication with obstructing transverse vaginal septum . increasing awareness of this rare entity calls for more meticulous evaluation before any surgical intervention in patients with complex genitourinary anomalies .

we present a case of iatrogenic urethrovaginal fistula with transverse vaginal septum . the patient presented with cyclical hematuria and infertility . the vagina was blind - ending . the magnetic resonance imaging ( mri ) showed normal uterus with transverse vaginal septum . the cystoscopic examination during cyclical hematuria revealed bloody efflux through a small fistula below the internal urethral sphincter . vaginoplasty and repair of the urethrovaginal fistula was done . the vagina was reconstructed using an amniotic mould . the report emphasizes the importance of mri and cystoscopy in diagnosing such rare and complex anomalies .

ureteric injury from herniorrhaphy is unusual . we present a case of ureteric injury complicating an inguinal herniorrhaphy for a huge right inguino - scrotal hernia . case reports are important as they describe rare clinical conditions , highlight uncommon challenges or therapeutic adaptations . they can , however , draw attention to uncommon sequel of standard treatment to serve as caution in future practice . ureteric injury following herniorrhaphy is rare .this is probably the first reported case from nigeria . we present a 66-year - old male farmer with right inguino - scrotal swelling noticed 10 years earlier which became irreducible 2 years prior to presentation . he had dysuria , urgency , urge incontinence , and feeling of incomplete emptying of the urinary bladder . he had a chronic dry cough and smoked two packets of cigarette per day in last 15 years . his respiratory rate , pulse rate and blood pressure were 22 cycle per minute , 100 beats per minute and 200/120 mmhg respectively . a provisional diagnosis of lower urinary tract infection ( uti ) , incarcerated right inguino - scrotal hernia with bladder outlet obstruction due to prostatic enlargement was made . the complete blood count , fasting blood sugar , serum electrolytes , urea , and creatinine , prostate - specific antigen , urine microscopy , culture and sensitivity , chest x - ray , and elecrocardiography done were all normal . ultrasonography revealed a thickened urinary bladder wall , bilateral pelvi - calyceal dilatation of kidneys but the ureters could not be visualized . scrotal ultrasound reported a large cystic mass on the right extending into the groin and surrounded by loops of bowel . intraoperative findings were : a right pantaloon hernia sac ( the indirect sac containing an inflamed appendix about 16 cm in length , while the direct sac component contained grossly normal cecum and the urinary bladder ) . the right ureter was inadvertently transected during dissection which necessitated laparatomy and conversion from spinal anesthesia to general anesthesia . the patient had bassini - type right inguinal herniorrhaphy and reimplantation of the transected right ureter into the urinary bladder over a transvesical ureteric stent ( figure 1 and 2 ) an urologist at the same session . surgery lasted about 3 h. he developed features of systemic inflammatory response syndrome ( sirs ) which progressed to multiple organ failure culminating in death by the fourth day postsurgery . . probable cause of death was septicemia with multiple organ failure . showing the transected ureter held with a clamp . showing the insertion of size 5 feeding tube as a stent postureter - neocystostomy . groin hernia surgery demands sound knowledge of the anatomy of the region as it does the principles of good perioperative care . complications may arise from groin hernia repair ; these commonly include : hemorrhage , surgical site infection , numbness of groin , chronic pain , and abnormal scar . injuries to gut and bladder can occur during repair of complicated hernias ( incarcerated , obstructed or ischemic / gangrenous ) and in hernia - en - glissade . the latter type was noted in our patient . generally ureteric injuries are either caused by external violence , usually penetrating missiles , or more commonly from surgical misadventure . . the most common procedures are hysterectomy , salpingo - oophorectomy , vesico - urethral suspension , ureteroscopy , endo - pyelotomy , and uretero - lithotomy and surgical procedures on the great vessels and colon as well as retroperitoneal tumor excision . lately , laparoscopic procedures have become a common cause of ureteric injuries . it is likely to occur if the ureter forms part of the sac or is contiguous with the spermatic cord as was seen in this patient . ureteric herniation can either have a peritoneal covering ( para - peritoneal herniation , 80% ) , or lacks peritoneal covering ( extraperitoneal , 20% ) . para - peritoneal ureteric hernia is common on the right , in males between fourth and sixth decades of life , and occurring in association with a sliding indirect hernia . the patient also had uti , symptomatic prostatic enlargement , and hypertension ; all of which could promote adverse surgical outcome . ureters within the hernia sac or contiguous to it can be identified as a thickened cord which exhibits peristalsis especially when stimulated , and contain fluid ( urine ) on test aspiration . the lower urinary symptoms ( luts ) noted in the patient could have been from the prostatic enlargement or from the involvement of the bladder in the hernia process or from the uti . the presence of prostatic enlargement with dilatation of proximal urinary tract as seen in our patient was previously reported by kate et al . . preoperative diagnosis of groin hernia containing the ureter or bladder can be made with a micturating cystogram or an intravenous urogram . early detection at time of injury and primary repair is said to confer better prognosis . intraoperative localization of point of ureteric injury can be achieved using intravenous 5 ml of indigo carmine intravenously and observing its extravasations into the periureteric tissues . the diagnosis of a ureteric injury generally is not made until many days after the injury has occurred . most common signs and symptoms seen in these patients are flank pain , fever , anuria ( if bilateral ) , uretero - vaginal fistula , and uretero - cutaneous fistula . in these conditions , an excretory urogram or computerized tomography ( ct ) if the patient is scheduled for exploration or the presence of an injury is in doubt , then a retrograde ureterogram is strongly advised . uretero - neocystostomy can be used to repair distal ureteric injuries that occur so close to the bladder thus precluding need for a psoas hitch or boari procedure . mortality is said to be worse in the elderly , following emergency procedure and in the face of comorbid diseases . the mortality recorded may be due to his high preoperative cardio - pulmonary risk status ( long - term smoking with chronic cough , and severe hypertension ) ; long duration of surgery in an elderly patient who had spillage of potentially infected urine in to the surgical wound . the resultant septicemia from the latter coupled with the metabolic response to surgery in an already challenged cardio - pulmonary system could be enough to tip him into sirs and finally death from multiple organ failure . we can not categorically state the primary cause of death ; an autopsy would have helped elucidate this . complete inguinal hernia may contain the lower urinary tract especially if the patient presents with luts . preoperative radiological evaluation is advised in such cases to avoid injury to the ureter and/or bladder .

key clinical messageherniorrhaphy is a common surgery worldwide . common complications include hemorrhage , infection , chronic pain , and recurrence . ureteric injury from herniorrhaphy is unusual . we present a case of ureteric injury complicating an inguinal herniorrhaphy for a huge right inguino - scrotal hernia . patient had primary uretero - neocystostomy but died from septicemia in the postoperative period .

acute - onset postoperative endophthalmitis is characterized by marked inflammation of intraocular fluids and tissues . gram - negative bacteria are less commonly isolated than gram - positive bacteria in patients with acute - onset postoperative endophthalmitis . gram - negative organisms have been isolated in 26%42% of patients with cataract surgery related to endophthalmitis in developing countries,1,2 as compared with 5.9%11.8% in developed countries.3 the more common gram - negative organisms causing endophthalmitis include species of pseudomonas , haemophilus , proteus , and klebsiella . there have been reports of multidrug - resistant strains of gram - negative bacteria being isolated from patients with endophthalmitis.4 endophthalmitis caused by klebsiella pneumoniae is associated with generally poor visual outcomes , despite treatment with appropriate antibiotics.5 three cases of acute postoperative endophthalmitis due to multidrug - resistant k. pneumoniae are reported . three patients developed acute - onset endophthalmitis within 13 days following cataract surgery ( table 1 ) . all the patients were in good general health and were not known to be suffering from any known systemic illness in the pre and postoperative periods . presenting visual acuity ranged from 20/50 to light perception . hypopyon was observed in all patients and corneal infiltrate was observed in two patients who presented with visual acuity of light perception . one patient underwent vitreous tap with intraocular injection of vancomycin 1 mg/0.1 ml , amikacin 400 g/0.1 ml , and dexamethasone 400 g/0.1 ml . the other two patients underwent pars plana vitrectomy with intravitreal injection of the aforementioned antibiotics and dexamethasone . gram - negative bacilli were isolated and confirmed to be k. pneumoniae by vitek 2 compact ( biomrieux , france ) . the organism that was isolated in case 3 was also found to be sensitive to ceftazidime and colistin . additional procedures in the form of pars plana vitrectomy with intravitreal injection of imipenem 50 g/0.1 ml were carried out in all patients . since there was no improvement 2 days after the second intervention , all patients were reinjected with intravitreal imipenem . at last follow - up , visual acuity was poor in all the patients , there was light perception in two patients , and no light perception in the third patient . multidrug resistance is defined as acquired nonsusceptibility to at least one agent in three or more antimicrobial categories.6 incidences of multidrug - resistant gram - negative bacilli have been increasing in tertiary care hospitals.7 it was reported that 5.2% of microbiological isolates from endophthalmitis patients were multidrug resistant , and 78.6% of those isolates were gram - negative bacteria.4 the various mechanisms contributing to virulence of k. pneumoniae are the presence of capsular k1/k2 serotypes , hypermucoviscosity , and the presence of the maga gene and the blandm-1 gene , which is a carbapenemase -lactamase.79 these bacteria can be resistant to all currently available antimicrobial agents or remain susceptible only to older , potentially more toxic agents such as the polymyxins , leaving limited and suboptimal options for treatment.6 intravitreal imipenem has been studied previously in rabbit eyes and has been reported to be nontoxic to retina.10 it acts by inhibiting cell wall synthesis of various gram - positive and gram - negative bacteria . it is stable to hydrolysis by the common plasmid - mediated -lactamases produced by various bacteria and lacks cross - resistance with penicillins and third - generation cephalosporins.11 one case series reported use of intravitreal imipenem in the treatment of endophthalmitis caused by acinetobacter baumannii . the authors reported that despite the use of multiple intravitreal injections of imipenem , the visual outcomes were poor.12 based on reported antimicrobial sensitivity , 50 g/0.1 ml intravitreal imipenem was utilized in all patients in the current series . k. pneumoniae has been commonly associated with endogenous endophthalmitis . in reported series , most patients have poor visual outcome despite appropriate antibiotic therapy , and the outcomes are usually phthisis and blindness.5,13 this may be attributed to the highly virulent organism as well as to delayed recognition of antibiotic resistance and susceptibility . the outcome of patients in the current case series was either phthisis or blindness , even after the best possible treatment .

the purpose of this paper is to report outcomes of intravitreal imipenem in the treatment of multidrug - resistant klebsiella - related postoperative endophthalmitis . this observational case series consists of three eyes from three patients seen between 2013 and 2014 . multidrug - resistant klebsiella pneumoniae is characterized by a rapid , fulminant course and severe intraocular inflammation . intravitreal imipenem may be used to treat such infection .

to date only two species , mangrovibacter plantisponsor dsm 19579 and mangrovibacter yixingensis kctc 42181 have been described under the genus mangrovibacter ( http://www.bacterio.net/mangrovibacter.html ) . the draft genome of mangrovibacter sp . strain mfb070 , isolated from an aquaculture farm in india has been described recently which is the only available genome sequence within genus mangrovibacter . members of the genus mangrovibacter have been shown to possess plant growth promoting features such as nitrogen fixation and may provide niche - specific advantage to the plant . in context to invasive weeds , this could provide a better ecological fitness in an invaded territory compared to native vegetation . thus , understanding the microbiota and role they may play during plant invasion could lead to more directed and sustainable management of weeds . chilika lagoon ( 1928-1954n ; 8506-8535e ) is a brackish water lagoon located in the odisha state of india . p. karka is a large perennial grass of the family poaceae and occupy most of the northern shoreline of lagoon . in order to understand the microbial basis of the invasive success of this weed , a study was undertaken to investigate the culturable diversity of rhizosphere microbiota associated with p. karka . during this study , a gamma - proteobacterium , facultative anaerobic , endophytic nitrogen fixing mangrovibacter sp . strain mp23 grew at temperatures between 20 c and 40 c with an optimum at 37 c in presence of 1% nacl . the 16s rrna gene phylogenetic analysis showed that strain mp23 was most closely ( 99.71% similarity ) related to m. yixingensis kctc 42181 and m. plantisponsor dsm 19579 indicating that it belong to genus mangrovibacter . here , we described the draft whole genome shotgun sequence of strain mangrovibacter sp . strain mp23 ( dsm 100250 = kctc 42580 ) , which will provide genetic insights into the nitrogen fixation , stress tolerance , plant niche adaptation , and comparative evolution of this species . the genome of mangrovibacter sp . strain mp23 was sequenced by a shotgun sequencing method using the illumina miseq sequencing system with a paired - end module . the ngs qc toolkit v 2.3 was used to filter the data for high - quality ( hq ) vector- and adapter - free reads for genome assembly . a total of 6,895,374 paired end reads were generated out of which 6,702,332 high quality vector - filtered reads were considered , representing approximately 310 fold coverage of the genome . these reads were assembled using masurca v. 3.1.3 and resulted into 50 contigs with a total size of 4,947,475 bp and an n50contig length of 428,946 bp . annotations of protein - coding genes , as well as other functional genome units were carried out through ncbi prokaryotic genome annotation pipeline ( pgap ) . the genome contained a total of 4592 genes and 4392 protein - coding genes with predicted function . the complete genome of strain mp23 was 4,947,475 bp in length with an estimated g + c content of 49.9% and consists of 77 trna genes and 28 rrna ( 8 = 5s , 5 = 16s , 15 = 23s ) genes ( table 1 ) . this whole genome shotgun project has been deposited at ddbj / embl / genbank under the accession number lyrp00000000 . the authors declare that there is no conflict of interests with respect to the work published in this paper .

till date , only one draft genome has been reported within the genus mangrovibacter . here , we report the second draft genome shotgun sequence of a mangrovibacter sp . strain mp23 that was isolated from the roots of phargmites karka ( p. karka ) , an invasive weed growing in the chilika lagoon , odisha , india . strain mp23 is a facultative anaerobic , nitrogen - fixing endophytic bacteria that grows optimally at 37 c , 7.0 ph , and 1% nacl concentration . the draft genome sequence of strain mp23 contains 4,947,475 bp with an estimated g + c content of 49.9% and total 4392 protein coding genes . the genome sequence has provided information on putative genes that code for proteins involved in oxidative stress , uptake of nutrients , and nitrogen fixation that might offer niche specific ecological fitness and explain the invasive success of p. karka in chilika lagoon . the draft genome sequence and annotation have been deposited at ddbj / embl / genbank under the accession number lyrp00000000 .

thymomas are the most common low - grade malignant tumors that often emerge in the anterior mediastinum in adults . while thymectomy by sternotomy is the traditional method of treating thymoma , recently , video - assisted thoracic surgery ( vats ) approaches have been often selected . vats thymectomy is reportedly feasible and safe and is less invasive than transsternal thymectomy [ 2 , 3 ] . however , we experienced a rare complication of port - site recurrence that would not have occurred with transsternal thymectomy . a 60-year - old woman was referred to our hospital with an anterior mediastinal tumor on computed tomography ( ct ) performed for follow - up 6 years after a complete response to chemotherapy for malignant lymphoma . ct revealed a tumor in the right anterior mediastinum with a diameter of 3.5 cm , round shape and demarcation from the neighboring tissue ( fig . nmol / l ( within normal range ) . to confirm the diagnosis and treat the patient , we planned tumor resection via vats . figure 1:initial ct revealed a tumor in the right anterior mediastinum with a diameter of 3.5 cm , round shape , which was clearly demarcated from the neighboring tissue . initial ct revealed a tumor in the right anterior mediastinum with a diameter of 3.5 cm , round shape , which was clearly demarcated from the neighboring tissue . the first 15-mm thoraco - port for the videoscope was placed in the sixth intercostal space on the mid - axillary line . the second 15-mm port was placed on the fifth intercostal space on the anterior axillary line . the third 5-mm port was placed in the fourth intercostal space on the anterior axillary line . the tumor was easily identified under the mediastinal pleura as an elevated mass in the thymic tissue . through the second and third ports , the normal thymus around the tumor was grasped by forceps and cut using an ultrasonic scalpel with marginal tissues . after the resected tumor was placed into a specimen bag , it was removed through the second port . frozen sections during the operation revealed that the tumor was capsulated type a masaoka stage i thymoma with sufficient marginal tissue . follow - up ct performed 36 months after the operation revealed two pleural tumors located near the second and third ports ( fig . initially , the operation was performed only by vats with three ports . after removing the severe adhesion between the lung and chest wall given these findings , we diagnosed these tumors as port - site implantations after the first vats . figure 2:follow - up ct at 36 months after the operation revealed two pleural tumors locating at the second ( a ) and third ( b ) ports used in the vats operation . figure 3:in the operative findings , the tumors were found to have arisen from the intercostal space , and their surfaces were covered with parietal pleura with partial irregularity . follow - up ct at 36 months after the operation revealed two pleural tumors locating at the second ( a ) and third ( b ) ports used in the vats operation . in the operative findings , the tumors were found to have arisen from the intercostal space , and their surfaces were covered with parietal pleura with partial irregularity . with no definitive findings suggesting tumor invasion into the rib we performed mini - thoracotomies just above the tumors and enucleated those two tumors via the mini - thoracotomies using an electric scalpel . to obtain a sufficient surgical margin , we visualized the adequate resected line using a thoracoscope while cutting the intercostal tissue surrounding the tumor . the pathological diagnosis was type a thymoma , which was consistent with port - site metastasis of the initial tumor . after relating these findings and the incomplete resection to the patient , we decided on close follow - up without reoperation . ishibashi et al . reported a case of port - site recurrence after a vats operation for type b2 thymoma , to our knowledge , this is the only such previous case report . the current type a and the reported type b2 thymomas are low - grade malignant tumors . despite their mild biological behavior , implantation at cases of implantation after medical procedures have been reported , not only for malignant tumors , but also for benign tumors . surgeons should thus be aware that port - site implantation can occur regardless of the tumor 's malignant potential . willard et al . reported a case that recurred in the small vats port through which partially resected lung cancer had been extracted without a specimen bag . in our case , the implantation developed only at the second and third ports , through which the forceps and ultrasonic scalpel had passed repeatedly . although we did not detect any tumor exposure during the first operation , we can not help but assume that the implantation occurred due to contamination with tumor cells from either the ultrasonic scalpel or forceps that had grasped tissue near the tumor . of further note , implantation did not develop in other pleura in which tumor tissue must surely have been disseminated by contaminated equipment . taken together , these findings suggest that one of the possible causes of port - site implantation was the rubbing of tumor cells , such as thymoma , which might have a high affinity for pleura , against the vats ports . surgeons should take extreme care to grasp the tissue as far as possible from the tumor and to use a specimen bag even when tumor exposure is not recognized . given the lack of any definitive clinical findings that the tumors had invaded the neighbor ribs , we believed that the tumors could be enucleated completely while preserving the ribs . however , the enucleation of the tumors located mainly in the narrow intercostal spaces with a sufficient margin was very difficult . although no definitive tumor invasion to the skin or subcutaneous tissue was identified , they might nevertheless invade the ribs pathologically . ishibashi et al . successfully performed a curative operation in a case of port - site recurrence via wide chest wall resection and reconstruction . in the process a small - extent resection of the skin and wide resection of the chest wall is therefore recommended when performing surgery for port - site recurrence after vats thymectomy .

a 60-year - old woman was referred to our hospital with an anterior mediastinal tumor measuring 3.5 cm in diameter on computed tomography ( ct ) . we performed tumor resection by video - assisted thoracic surgery ( vats ) with three ports . the final diagnosis was type a masaoka stage i thymoma . on follow - up ct performed 36 months after the operation , two pleural tumors were detected at the port sites through which the forceps and ultrasonic scalpel had passed repeatedly during the operation . we therefore performed a second operation and enucleated the tumors while preserving the ribs . however , other tumor tissue was detected along the surgical marginal line during the pathological diagnosis after the operation . surgeons should thus be aware that port - site recurrence can occur after vats resection of type a thymoma , despite its mild biological behavior . wide resection of the chest wall is therefore recommended for operations of port - site recurrence after vats thymectomy .

premature beats arising from foci other than pulmonary veins have been related to its pathogenesis . a 64-year - old female underwent superior vena cava ( svc ) isolation after triggers were identified originating from the svc following pulmonary vein isolation ; immediately after svc isolation , she developed junctional rhythm with symptomatic hypotension requiring emergent management . apical motion abnormalities were noticed in the echocardiography suggesting stress - induced cardiomyopathy which resolved 48 hours later . although received a dual chamber pacemaker , intact sinus node function returned 2 weeks later . superior vena cava isolation in those with trigger mediated atrial fibrillation following pulmonary vein isolation ( pvi ) is performed to enhance long - term outcomes . we present the first case of time course of recovery of sinus node function , injured during svc isolation . atrial fibrillation ( af ) is the most common sustained arrhythmia ; its incidence increases with aging . this condition could increase morbidity and mortality due to its high potential to cause heart failure , thromboembolic events and the related conditions associated to its treatment such as bleeding . the cornerstones for the management of atrial fibrillation are anticoagulation to prevent embolic events , control symptoms and avoid deterioration of heart function . af arising from the pulmonary veins ( pv 's ) was recognized by haissaguerre et al . , in a study that showed 94% of the time pvs are implicated . however , there is some evidence showing that non - pulmonary veins ( npv 's ) premature beats could trigger af in up to 28% of cases . the most common npv foci include the left atrial posterior wall ( 38% ) and superior vena cava svc (37% ) . other areas acting as npv include crista terminalis , coronary sinus , ligament of marshall and interatrial septum . catheter techniques looking for the electrical isolation of pv antrum and other atrial tissue originating premature beats has emerged as therapeutic option for symptomatic patients with atrial fibrillation , providing symptomatic benefit . we present a clinical case of a patient who developed a transient sinus node block as a complication during pvi and superior vena cava isolation ( svci ) . , although patient did receive a dual chamber pacemaker , two weeks later , sinus node function returned back to baseline , identifying the time course of recovery of sinus node function . a 64 year - old female with drug refractory af and atrial flutter underwent pvi and cavotricuspid isthmus(cti ) ablation . basal measurements and conduction study were considered normal . during the isoproterenol testing the patient developed recurrent episodes of recurrent atrial tachycardia ( at ) , left bundle branch block ( lbb ) and rapid ventricular response af ( cycle length up to 410 msec ) . pvi was carried out with irrigated radiofrequency ( rf ) ablation and lesions were tagged on geometry created using carto 3d sound system ( biosense webster , diamond bar , ca , usa ) ; we did a wide area circumferential ablation on all four pv 's including carinal ablations , with evidence of entrance block . however , triggered af was inducible with isoproterenol , and triggers in vein of marshall was successfully ablated . further testing revealed svc origination of npv triggers and svci was performed ( see fig . 1 , fig . 2 ) . during immediate observation , junctional rhythm at 36 bpm ( fig . 3 ) with hypotension requiring emergency administration of epinephrine and temporary transvenous pacemaker insertion was needed . an echocardiogram showed apical motion abnormalities , and coronary angiogram was negative for coronary artery disease . patient was implanted with a permanent pacemaker after failure of recovery of sinus node function at 48 hours . on weekly observations in clinic , recovery of sinus node function yamaguchi et al . showed that in those patients with npv foci pvi alone is not enough to reach long term responses suggesting that they should always be addressed during the index ablation procedure . controversies still exist about the npv foci management especially with svc triggers and conflicting evidence exists based on randomized control trials ( rct ) . . showed in 2003 that there were no difference between patients randomly assigned to pvi versus pvi + svci regarding af recurrence after ablation . however in a more recent clinical trial evaluating 2 randomly assigned groups to pvi compared to pvi + svci , the authors found that those patients who underwent both procedures were significantly less prone to paroxysmal af after 12 months of follow - up . the most common are svc stenosis , phrenic nerve damage with diaphragmatic paralysis and sinus node injury ( sni ) . described 6 patients who developed sni after svci , in this clinical study 5 patients had transient junctional rhythm that resolved in the first 48 hours postprocedure and just one patient required permanent pacemaker implantation . probably the sni is due to the damage to the atrial muscle sleeve that extends into the svc and contains sinus node pacemaker activity . to the best of our knowledge ours is the first report of sni associated with pvi + svci where time course of recovery of sinus node function was noted . identification of npv triggers and subsequent successful ablation is performed in order to get higher responses in the long term follow up after the index procedure . premature beats arising from svc triggering af is infrequent but when present has to be approached cautiously due to proximity of phrenic nerve and inadvertent sinus node injury .

introductionatrial fibrillation is the most common sustained heart arrhythmia . premature beats arising from foci other than pulmonary veins have been related to its pathogenesis.methods and resultsa 64-year - old female underwent superior vena cava ( svc ) isolation after triggers were identified originating from the svc following pulmonary vein isolation ; immediately after svc isolation , she developed junctional rhythm with symptomatic hypotension requiring emergent management . apical motion abnormalities were noticed in the echocardiography suggesting stress - induced cardiomyopathy which resolved 48 hours later . although received a dual chamber pacemaker , intact sinus node function returned 2 weeks later.conclusionsuperior vena cava isolation in those with trigger mediated atrial fibrillation following pulmonary vein isolation ( pvi ) is performed to enhance long - term outcomes . sinus node injury has been related previously to this procedure . we present the first case of time course of recovery of sinus node function , injured during svc isolation .

we report the case of a patient who underwent a laparoscopic cholecystectomy with gallstone spillage 34 months before presenting to the thoracic surgery service . the patient first complained of streaks of hemoptysis at 6 months from the time of the original procedure . a lower lobe infiltrate was noted and treated successfully with oral antibiotics . over the next 2 years , the patient 's symptoms waxed and waned . due to the chronic infiltrate in his lung , a thoracotomy was performed that revealed erosion of the stone through the right diaphragm with formation of a lung abscess . a high index of suspicion for a gallstone - related problem should be entertained by the practitioner when presented with a patient who has a right lung infiltrate and a history of open or laparoscopic cholecystectomy . gallbladder perforation during laparoscopic cholecystectomy occurs in 10% to 32% of patients , with gallstone spillage in 0.2% to 20% of cases . there have been very few reported thoracic complications from this common problem . before laparoscopic cholecystectomy , cholelithoptysis and intrathoracic complications from gallstones were rare because the operative field was packed off to facilitate the open surgery . introduction of a pneumoperitoneum and intraoperative irrigation facilitates distant migration of gallstones , specifically to the subphrenic space . a review of the literature reveals 11 reported cases of gallstones being found in the thoracic cavity following cholecystectomy . eight cases presented as cholelithoptysis and the latest one as massive hemoptysis from a gallstone associated abscess . we believe these findings reiterate the need for fastidious stone removal at the time of initial cholecystectomy . furthermore , while most patients with this problem present with cholelithoptysis , a high index of suspicion should be entertained by the practitioner when encountering a patient who has a right lung infiltrate and a history of open or laparoscopic cholecystectomy . a 73-year - old man with a past medical history of hyper - tension and chronic obstructive lung disease presented with a 4-month history of biliary colic and mild pancreatitis . after 3 days of conservative management with nothing by mouth and intravenous antibiotics , his pancreatitis resolved , and he underwent laparoscopic cholecystectomy . he did well postoperatively and was asymptomatic for 6 months until he developed low - grade fevers and streaks of hemoptysis . computed tomography of the chest revealed a 57-cm pulmonary infiltrate in the posterior basilar segment of the right lower lobe associated with a 59-mm oval calcification at the level of the right diaphragm ( figure 1 ) . shows eccentric calcification of a soft tissue mass in the lower lobe of the right lung . a repeat ct scan 2 months later demonstrated marked improvement of the pulmonary infiltrate , but the persistence of a calcified nodule on the surface of the diaphragm . over the next 2 years , the patient developed episodic bouts of scant hemoptysis every 2 to 3 months that resolved spontaneously . thirty - four months after his laparoscopic cholecystectomy , after an episode of self - limited hemoptysis , he underwent a surveillance ct scan that revealed a new 53-cm opacity in the posterobasal segment of his right lower lobe . further evaluation with a mediastinoscopy showed no evidence of malignancy . at the time of exploration , the right lower lobe was densely adherent to the diaphragm . to ensure an oncologic resection , the final pathology was returned as chronic pulmonary abscess and foreign body giant cell reaction to a gallstone . transection of the gross lung specimen shows a gallstone encapsulated by surrounding fibrous reactive tissue . gallstone spillage is estimated to occur in 0.2% to 20% of cases with very few causing complications . a case review of 1130 consecutive laparoscopic cholecystectomies performed at 2 different institutions demonstrated a 0.3% complication rate from intraoperative stone spillage at 13-year follow - up . complications from gallstone spillage vary from trocar - site wound infections to overt intraabdominal abscess as well as gynecological and intrathoracic complications . thoracic complications of retained gallstones with pleurobiliary and bronchobiliary fistulas were reported as early as 1955 . the mean time to onset of complications is 6 months , but a case report exists of a gallstone - associated subphrenic abscess presenting 10 years after the cholecystectomy . our case represents a complication of a spilled gallstone at the time of laparoscopic cholecystectomy that eroded through the right diaphragm and caused pneumonitis presenting as intermittent hemoptysis . although our patient never had an overt intraabdominal abscess , clinically and by imaging , he had a prolonged right subdiaphragmatic inflammatory process that allowed the intrathoracic migration of the gallstone . if stone spillage occurs , a serious attempt to retrieve as many stones as possible with a larger forceps should be made followed by copious amounts of irrigation with a large bore suction catheter . this should be performed laparoscopically without conversion to an open procedure , as overall complication rate of stone spillage is 0.3% . before the advent of laparoscopic cholecystectomy , cholelithoptysis and intrathoracic complications from gallstones coughing up stones , was classically due to calcified peribronchial lymph nodes that eroded through the bronchial wall . there are 11 case reports in the literature of gallstones in the thoracic cavity following cholecystectomy ( table 1 ) . nine cases presented as cholelithoptysis , and the latest one as massive hemoptysis from a gallstone - associated abscess . complications of intrathoracic gallstones three possible routes of intrathoracic migration of gallstones exist : the lymphatic channels of ranvier , congenital diaphragmatic defects , and transdiaphragmatic tracts that form from local infection / inflammation . as seen in our case , prolonged chest symptoms , especially with findings in the rll , following a laparoscopic cholecystectomy should raise the suspicion of intrathoracic gallstones . one must search for an intraabdominal collection and consider antibiotic treatment , percutaneous drainage , or laparotomy . thoracotomy is rarely required and only indicated for obstructing pneumonia , refractive hemoptysis , or nodules of uncertain cause . if stone spillage occurs during a laparoscopic cholecystectomy , documentation in the chart would be valuable to clinicians following the patient , allowing them to have a high clinical suspicion when abdominal or thoracic symptoms occur .

background : laparoscopic cholecystectomy is one of the most commonly performed procedures in general surgery . there have been very few reported thoracic complications from this surgical procedure.methods:we report the case of a patient who underwent a laparoscopic cholecystectomy with gallstone spillage 34 months before presenting to the thoracic surgery service . the patient first complained of streaks of hemoptysis at 6 months from the time of the original procedure . a lower lobe infiltrate was noted and treated successfully with oral antibiotics . over the next 2 years , the patient 's symptoms waxed and waned.results:due to the chronic infiltrate in his lung , a thoracotomy was performed that revealed erosion of the stone through the right diaphragm with formation of a lung abscess.conclusion:a high index of suspicion for a gallstone - related problem should be entertained by the practitioner when presented with a patient who has a right lung infiltrate and a history of open or laparoscopic cholecystectomy .

a 75-year - old male farmer presented to a primary care skin cancer practice in west australia with a 6-month history of an enlarging painless lesion on his left cheek with no history of any possible precursor lesion . there was a past history of treatment of two separate squamous cell carcinomas in - situ , one on the forehead and the other on an ear , in the previous two years . other comorbidities included asthma , coronary heart disease , chronic obstructive pulmonary disease and arthritis . there was no history of nevus sebaceous and there was neither history nor clinical signs suggestive of cowden s syndrome . examination showed a single small non - pigmented nodular lesion located on the left cheek in continuation with a small , pigmented macular lesion ( figure 1 ) . dermatoscopy ( figure 2 ) revealed a raised pink lesion , 3.5 mm in diameter , with a radial arrangement of vessels peripherally , with centered blood vessels in the middle of the lesion , these vessels being centered in skin - colored clods . no keratin structures ( keratin scale , white structureless areas or white circles ) were seen . at one edge of this lesion , in direct contact with it , was a heavily pigmented flat lesion , 1 mm in diameter with a pattern of pigmented lines radial , converging ( figure 2 ) . the lesion was subjected to an excision biopsy with a 4 mm peripheral margin and submitted for dermatopathological evaluation . dermatopathology ( figure 3 ) showed both the trichilemmoma and bcc ( basal cell carcinoma ) components . the trichilemmoma ( figure 3 a1 and a2 ) was a well - circumscribed , sharply demarcated lesion with surface papillomatosis , horn cysts and a degree of central desmoplasia . under high power an eosinophilic mantle of thickened basement membrane , mimicking the outer root sheath of the hair follicle infundibulum , was seen . peripheral palisading was evident adjacent to the basement membrane and extending from that there were clear cells which were banal cytologically , lacked mitosis or individual cell necrosis ( apoptosis ) and contained an abundant amount of glycogen within their cytoplasm . the bcc component ( figure 3 b1 and b2 ) showed superficial bcc at the dermo - epidermal junction . in the high power view melanin pigment trichilemmoma is a benign tumor derived from the external sheath cells of pilosebaceous units and desmoplastic trichilemmoma is a rare variant , which can present clinically with features suggesting invasive malignancy . there has been controversy as to whether trichilemmomas are actually aged warts , this belief being proposed by well - known dermatopathologist bernie ackerman but vigorously opposed by others including marty brownstein . basal cell carcinoma is a malignant tumor , of trichoblast differentiation but uncertain origin , although it is probably of follicular derivation , predilection for non - glabrous skin being consistent with this . trichilemmoma has previously been reported in association with bcc in the context of lesions arising in a preexisting nevus sebaceous . in addition , three cases of desmoplastic trichilemmoma arising in association with bcc without a preexisting nevus sebaceous have been reported . the dermatoscopy of four trichilemmomas has been reported with images being presented for three of them . in the first case keratin masses ; perivascular whitish halos . the other three were reported together , with the first two described as having a hyperkeratotic central area with peripheral erythematous radial lines , the third having a peripheral radiated red area . all three were then presented as depicting what the authors called a red iris like structure . examination of the photographs published in fact displayed a non - pigmented lesion in each case with a radial arrangement of vessels peripherally and structureless white centrally with some centred vessels . in the case we report , the clinician interpreted the lesion as a pigmented lesion and assessed it using the chaos and clues decision - algorithm . the lesion was regarded as chaotic ( asymmetrical ) with the clues of an eccentric structureless ( pink ) area as well as lines radial segmental , which lead to a clear decision to do an excision biopsy . the pathologist , as is often the case with esoteric lesions , was able to deliver the answer to which particular type of malignancy this was . the dermatoscopic features of the trichilemmoma presented here are consistent with those published previously , with a radial pattern of vessels peripherally and centred vessels centrally . association with a bcc is a previously reported , unusual variation , consistent with both lesions having hair - follicle derivation . this case demonstrates the utility of using a decision algorithm in esoteric cases with the final diagnosis to be delivered by the gold standard of dermatopathology .

a case of trichilemmoma in continuity with a pigmented basal cell carcinoma is presented with dermatoscopy and dermatopathology . the distinction between the two lesions was evident dermatoscopically and was confirmed dermatopathologically . while trichilemmoma has been reported in association with basal cell carcinoma and dermatoscopy images of four previous cases of trichilemmoma have been published , no previous dermatoscopy image has been published of trichilemmoma associated with basal cell carcinoma .

a young boy , 16 years old , presented with complaints of poor vision in the left eye since childhood . , he had a vision of 20/200 in the right eye ( re ) and 20/20 in the left eye ( le ) . zonules were lacking around the fissure between the two lobes [ fig . 1 ] . 2 ] revealed a bilobed lens in the anterior segment of the eye with normal posterior segment . partial congenital aphakia is a rare condition in which the lens appears to be partially divided by a deep furrow.1 in the case of congenital duplication of lens reported by thakkar et al.2 two lenses were placed obliquely in a straight line with a clear area in between . richardson3 reported a case in which the two lenses were asymmetrical , one being larger than the other . the mechanism for this anomaly is not known yet . whether it is part of a phylentogenic spectra or represents development from two embryonically separate lens remains a question . duke elder has tried to explain the occurrence of the two entities based on separate embryonic developmental sequences.1 he suggested that the metaplastic changes in the surface ectoderm leads the lens plate to invaginate at two place and forms two lens vesicles which results in double lens.1 this suggests that the lens is divided before the lens starts developing . our case has a deep furrow in the center fig . 1 with localized absence of zonules . we , therefore , suggest that once the deep furrow is formed due to absence of zonules , it may go further ( deep ) enough to divide the lens into two distinct parts . therefore , the absence of zonules is significant and these furrows may be deep enough and progress to duplication . it is a possibility that the lens duplication might be occurring when the lens vesicle is single and it is the laxity and abnormal stretching which causes the deep furrow and possibly duplication . it is interesting to note that both the cases previously reported2 - 3 had lens place in a straight axis at opposite ends . this gives further credence to our theory of the fissure dividing the lens into two and the remaining zonules pulling them away from each other . since lens coloboma occurs frequently such deep furrows ( bilobed lens ) are uncommon . we believe that it represents the missing link between the colobomatous lens and duplicated lens . we understand that a single case report may not be suggestive of any mechanism but nevertheless it does indicate a possible mechanism of lens duplication .

congenital anomalies of the lens include a wide range from lens coloboma to primary aphakia and doubling of lens . there have been few case reports of double lens ; the etiology suggested is metaplastic changes in the surface ectoderm that leads to formation of two lens vesicles and hence resulting in double lens . we report a case with bilobed lens , which raises the possibility of explaining the etiology of double lens .

the course is divided into 5 modules , where the leading experts in the field provide online lectures within their area of expertise ( table i ) . during the first module of the course therefore , topics that are covered during the introduction week are the nomenclature for the different subpopulations of evs as well as an introduction to the diversity of organisms releasing evs and the tissues and body fluids where evs can be found . furthermore , one of the pioneers , professor emeritus philip stahl , shares the story about how he and his colleagues discovered exosomes in the early 1980s ( 4 ) . the second module focuses on the biogenesis and release of evs and how this differs between the ev subpopulations : exosomes and microvesicles . additionally , the different uptake mechanisms of evs when they are encountered by a recipient cell are covered in depth ( 10 ) . as evs have been shown to contain functional rnas , proteins and lipids , this module also covers the different types of molecules present in evs as well as a brief overview on what the potential functions of these molecules are . furthermore , the techniques that are commonly used to detect these molecules and to analyse the cargo of evs will be highlighted . in the third module , the focus is on the collection and processing of cell culture media and body fluids prior to isolation of evs . here , considerations and guidelines that are important during the collection of the ev - containing material and when isolating evs from these fluids are discussed ( 11,12 ) . this module will help the students to reflect over the many different choices , such as anticoagulants , collection time points and protein inhibitors , which are important for the outcome when working with a particular body fluid compared with conditioned media or other body fluids . this module also illustrates some examples of studies on evs from body fluids such as blood , urine , breast milk and cerebrospinal fluid and why it is of interest to analyse evs from these bodily fluids . the basic concepts and some guidelines for methods such as differential ultracentrifugation , density gradient centrifugation , size - exclusion chromatography and kit - based precipitation are covered . furthermore , this module covers how the techniques are used in the field of evs as well as their limitations and benefits . the importance of evaluating the heterogeneity , purity and characteristics of the isolated vesicles regardless of isolation method is also highlighted ( 13 ) . the fifth module covers some of the different techniques that can be used to characterise evs . here , the basic concepts for techniques such as electron microscopy , cryo - transmission electron microscopy ( cryo - tem ) , flow cytometry , atomic - force microscopy ( afm ) and nanoparticle - tracking analysis ( nta ) are covered . furthermore , this module covers how the techniques are used in the field of evs as well as their limitations and benefits . this course is recommended for anyone interested in the field of evs including biology and medical students and phd students without previous experience in the field as well as clinicians , cell and molecular biologists and researchers who want to broaden their understanding of the field and deepen their knowledge about particular techniques . the course contains 5 modules , where each module contains 47 recorded lectures ( 635 min / lecture ) . each module contains in total 12.5 h of recorded materials , and all lectures are in english . each of the 5 modules is followed by a quiz in the format of multiple choice questions . after completing the course , the student should be able to : discuss the nomenclature and subgroups of evs , describe the release and uptake mechanisms of evs , describe the rna , protein and lipid content of evs , explain the considerations that are important during the collection and isolation of evs from different body fluids , describe the basic concepts about the most common isolation and characterisation techniques and how these techniques are used in the ev field andstate the benefits and limitations of the most common isolation and characterisation techniques for evs . discuss the nomenclature and subgroups of evs , describe the release and uptake mechanisms of evs , describe the rna , protein and lipid content of evs , explain the considerations that are important during the collection and isolation of evs from different body fluids , describe the basic concepts about the most common isolation and characterisation techniques and how these techniques are used in the ev field and state the benefits and limitations of the most common isolation and characterisation techniques for evs . the initial response to the course has been overall positive with high ratings , and one student commented the course as : this course was really well organized and paced but packed full of a lot of really good information from great sources and leaders in the field . i really did n't know anything about exosomes before i started this course and now i feel like i can even teach the people in my own lab a few tricks . this course was really well organized and paced but packed full of a lot of really good information from great sources and leaders in the field . i really did n't know anything about exosomes before i started this course and now i feel like i can even teach the people in my own lab a few tricks . we are pleased to see this initial feedback to the course and isev will now initiate the work of producing more online courses on other related topics such as the biological functions of evs in health and disease . this course was funded by the international society for extracellular vesicles and supported by grants for pedagogic development from the sahlgrenska academy , university of gothenburg . ysg is the inventor of patents for using evs as therapeutics , diagnostics and vaccines and is the founder of aeon medix and rosetta exosome and own stock in the company . jl is the co - owner of patents for using exosomes as therapeutics and is currently an employee of codiak biosciences , inc . in parallel with his position at university of gothenburg .

the international society for extracellular vesicles ( isev ) has organised its first educational online course for students and beginners in the field of extracellular vesicles ( evs ) . this course , basics of extracellular vesicles , uses recorded lectures from experts in the field and will be open for an unlimited number of participants . the course is divided into 5 modules and can be accessed at www.coursera.org/learn/extracellular-vesicles . the first module is an introduction to the field covering the nomenclature and history of evs . module 2 focuses on the biogenesis and uptake mechanisms of evs , as well as their rna , protein and lipid cargo . module 3 covers the collection and processing of cell culture media and body fluids such as blood , breast milk , cerebrospinal fluid and urine prior to isolation of evs . modules 4 and 5 present different isolation methods and characterisation techniques utilised in the ev field . here , differential ultracentrifugation , size - exclusion chromatography , density gradient centrifugation , kit - based precipitation , electron microscopy , cryo - electron microscopy , flow cytometry , atomic - force microscopy and nanoparticle - tracking analysis are covered . this first massive open online course ( mooc ) on evs was launched on 15 august 2016 at the platform coursera and is free of charge .

antiphospholipid syndrome ( aps ) is an autoimmune disease characterized by the presence of antiphospholipid antibodies , venous and/or arterial thrombosis , and repetitive fetal loss [ 1 , 2 ] . the laboratory criteria to diagnose aps are high serum levels of lupus anticoagulant as well as anticardiolipin and -2 glycoprotein - i antibodies . aps can occur with other systemic diseases such as systemic lupus erythematosus or as a single entity . deep vein thrombosis represents the most frequent aps complication , as it occurs in 2955% of patients ; however , acute or indolent chronic ischemic presentations can involve any organ including the lung , skin , brain , liver , kidneys , adrenal glands , heart , and eyes . ocular involvement can occur in 888% of patients [ 5 , 6 , 7 ] and it can be the first sign at presentation . it is therefore important to make a quick diagnosis in order to consider anticoagulant treatment to prevent further thrombosis , as the risk of this complication is up to 29% in untreated aps patients . visual symptoms at presentation vary from a reduction of visual acuity and amaurosis fugax to transient scotoma and visual field defect , all of which can be unilateral or bilateral . it is important to underline that , when amaurosis fugax involves both eyes simultaneously , it is often a manifestation of central nervous system ischemia . ocular discomfort , conjunctival hyperemia , and pain have often been reported in the literature as common symptoms of aps patients . a 38-year - old female patient presented with reduced vision in the right eye , which had occurred the same day . the patient had a negative ocular and medical history and no previous miscarriage as well as no relevant family history for ophthalmic pathologies . visual acuity was finger count in the right eye ( 1-meter distance ) and 20/20 in the left eye . fundoscopic examination revealed a subhyaloid hemorrhage , engorged and tortuous retinal veins , intraretinal hemorrhages , and cotton wool spots ( fig . 1 ) . fundus fluorescein angiography demonstrated a delayed inferior hemiretinal venous filling ( fig . 3 ) . as choroidal ischemia has been previously reported with aps , indocyanine green angiography was performed , which did not show any significant modification of choroidal perfusion . prothrombotic conditions secondary to other factors such as sepsis , homocystinemia , and genetic defects of coagulation factors ( thrombin mutations , factor v leiden , antithrombin deficiency , etc . ) were ruled out . laboratory investigations showed that the erythrocyte sedimentation rate , igg anticardiolipin , and -2 glycoprotein - i antibody were above normal limits . thromboplastin inhibition test was strongly positive for lupus anticoagulant , while antinuclear antibody and antinative dna antibodies were negative . serum total complement ( c3 , c4 ) , proteins s and c , and antithrombin iii were within normal limits . other studies were normal or negative , including complete blood count , platelet count , renal , liver , and thyroid functions , hemoglobin a1c , ferritin , transferrin , and ldl cholesterol . the patient met the criteria for aps ; however , according to the american college of rheumatology classification criteria , a concomitant diagnosis of systemic lupus erythematosus could not be established . she started treatment with hydroxychloroquine 200 mg / daily and ticlopidine 250 mg / daily . considering the central location of the hemorrhage , we deferred any invasive treatment and opted for watchful waiting . the subhyaloid hemorrhage resolved spontaneously and the patient recovered a visual acuity of 20/20 in her right eye 3 months after the initial episode ( fig . this is the first reported case of simultaneous subhyaloid hemorrhage and branch retinal vein subocclusion as presentations of aps . a prompt diagnosis of this condition is fundamental to consider a systemic treatment to avoid any further thrombosis . this is often difficult , as ocular manifestations of aps can be variable . assessing the patient 's thrombotic risk by investigating the presence of high blood pressure and hypercholesterolemia , the use of tobacco and oral anticontraceptive agents as well as the history of previous of thrombosis , fetal loss , and acute ischemic events is mandatory in the process of deciding whether to start systemic treatment .

we report an atypical presentation of antiphospholipid syndrome ( aps ) with concomitant subhyaloid hemorrhage , engorged and tortuous retinal veins , intraretinal hemorrhages , and cotton wool spots in a 38-year - old female . medical treatment was preferred to any invasive treatment . the subhyaloid hemorrhage resolved spontaneously and the patient recovered a visual acuity of 20/20 in her right eye 3 months after the initial episode . a prompt diagnosis of this condition is fundamental to consider a systemic treatment to avoid any further thrombosis .

bulking agent injection is now a standard treatment for patients with grade 1 - 3 primary vesicoureteral reflux ( vur ) . patients with higher grades and secondary types of vur show an unacceptably high failure rate when treated with injections of a bulking agent . collagen has been used for many years as a bulking agent in ureterovesical orifice and bladder neck to treat vur and incontinence . collagen , however , can get calcified in the tissue , a process that can cause a series of problems for patients . we present a case of a patient in whom calcified injected collagen showed imaging features mimicking ureteral stone . a 15-year - old girl presented with moderate degree of right flank pain for 2 weeks . pain was localized on the right side , altered with change in position , and did not relate to any particular organ . the patient had been treated successfully for bilateral grade 2 vur when she was 3 years old . a kidney ureter bladder ( kub ) radiography was requested and the report indicated presence of bilateral ureteral stone [ figure 1 ] . ultrasonography showed bilateral ureterovesical junction ( uvj ) stone and mild stasis in both kidneys , which was compatible with the findings of the non - enhanced spiral abdomino - pelvic computed tomography ( ct ) scan [ figure 2 ] . however , diethylene triamine pentaacaetic acid ( dtpa ) scan with furosemide injection and radionuclide cystography ( rnc ) scan ruled out obstruction and relapse of vur . calcification of bilateral intravesical collagen injected to treat her vur 12 years earlier was responsible for the positive findings on kub , ultrasonography , and spiral ct . 15-year - old female with bilateral flank pain , which was suspected to be due to stones in bilateral ureterovesical junction , later diagnosed as due to calcification of collagen used 12 years earlier to treat vesicoureteral reflux . 15-year - old female with bilateral flank pain , which was suspected to be due to stones in bilateral ureterovesical junction , later diagnosed as due to calcification of collagen used 12 years earlier to treat vesicoureteral reflux . spiral ct scan of pelvis without intravenous and oral contrast shows bilateral uvj calcifications ( arrows ) . endoscopic therapy for vur has gained popularity because of elimination of invasive surgical procedures in children . in addition , it has an acceptable success rate and a low level of complications . although a transition has occurred in the material of choice from teflon to collagen and now to hyaluronic acid copolymers . however , we do find patients who were treated with collagen now in the second decade of their life . the injected material , being a foreign body , can induce inflammatory response that leads to tissue calcification . this calcification has been reported to be symptomatic with some patients reporting episodes of stone passing , hematuria , and even obstruction . this is the second case report of ureterovesical injection calcification presenting as a ureteral stone during differential diagnosis and the first report of its imaging . unlike the first case report where the patient presented with renal colic and passing of stone , our patient was completely asymptomatic , except for positional non - related flank pain . there is no difference between a ureteral stone and calcification of injected collagen , when viewed by different imaging modalities like kub [ figure 1 ] , ct scanning [ figure 2 ] , and also ultrasound examination . this similarity is because of the place where the bulking agent is injected . in doubtful cases , as in our case , uvj obstruction can be ruled out by dtpa nuclear scan in which glomerular filtration of injected nuclear radioisotope reveals any obstruction in the upper urinary tract . the diagnosis of uvj stone in the presence of uvj calcification , possibility of ureteral stone passing ( in a patient with renal colic ) after uvj injection therapy , and the probability of recurrent vur after ureteral intervention ( in which calcified ureteral orifice will tolerate dilatation to pass the ureteroscope ) remain to be studied . vur can be assessed by rnc in which a radioisotope passing to the bladder via the urethral catheter reveals presence of vur . the standard treatment for grade 1 and 3 primary vur is injection of a bulking agent into the uvj . clinicians should be aware that calcification of bulking agent like collagen can occur and they need to differentiate this from ureterovesical stones on imaging findings .

primary vesicoureteral reflux can be treated by injection of a bulking agent into the wall of the ureterovesical junction . over time , the bulking agent can get calcified . radiological images of the area show findings that mimic those seen in ureterovesical junction calculi . in this report , we present the imaging findings of this phenomenon and discuss its challenging aspects .

vaccine programs are underpinned by a rigorous science determining their efficacy and safety in populations . there needs to be a similar level of commitment to identifying and testing the interventions designed to increase uptake of vaccines among vaccine - hesitant parents . accordingly , governments and research agencies need a greater investment in the strategic direction , capacity building , research and evaluation to meaningfully address vaccine hesitancy . vaccine hesitancy involves developing a deep understanding of the psychological and social dimensions of vaccine acceptance , building good measures that can identify and monitor patterns of vaccine hesitancy in populations and over time , and systematically testing interventions using valid and reliable outcome measures . interventions should consider communication interventions operating at the individual , family , and community level . they should be economically viable and shown to not cause harm prior to their implementation . only good monitoring of vaccine acceptance attitudes will determine true trends in the prevalence of vaccine hesitancy . many commentators consider increasing rates of disease , vaccine exemptions , and alternative vaccine schedules or reductions in coverage as an indication of declining vaccine acceptance . while these epidemiological and behavioral outcomes may be indicative , they are influenced by a range of possible factors . with respect to changes in coverage , parsing acceptance from access is essential . this most basic differentiation is hampered by the lack of national monitoring of both acceptance attitudes and perceptions of financial , physical , and social cultural barriers to access . few countries undertake active monitoring of actual vaccine acceptance over time using valid and reliable measures deployed in populations large enough to enable confident conclusions . these returns were most clearly demonstrated with the uk government 's yearly monitoring of mothers attitudes toward vaccination between 1991 and 2012 enabling program managers to anticipate shifts in public confidence following the mmr - autism scare . two major influences on vaccine acceptance and rejection emerge from the literature to date : social norms and provider interactions . with regard to the former , rejection of vaccination tends to cluster in communities typically characterized by alternative or religious belief systems . to the extent that rejection and hesitancy toward vaccination is a community phenomenon , solutions need to be considered at the community level . community - based interventions are already utilized in low- and middle - income countries , where they may be used to build trust and increase community participation in populations that are difficult to reach with mass media or standard health services . adversarial and/or top - down approaches can polarize communities and alienate parents less favorable toward vaccination . in contrast , communication that engages communities in dialog through local opinion leaders or peer groups has the potential to build community support and advocacy for the benefits of vaccination . a second major influence on whether a hesitant parent accepts or rejects a vaccine is the provider interaction . a number of approaches have been advocated yet , as noted by williams , the evidence base for effectiveness is wanting . confident recommendation combined with respectful engagement , narrative and personalized approaches that address the needs of vaccine hesitant parents appears to be the most constructive way . reviews should also appropriately assess the quality of included studies to ensure that the evidence base is not founded on low - quality studies . the outcomes measured need attention . as williams notes , outcome measurement across studies is inconsistent , making it difficult to build the evidence base through systematic reviews of effects . we need a comprehensive understanding of both process outcomes ( e.g. , knowledge or intent to vaccinate ) as well as endpoints ( e.g. , timely and complete vaccination ) in order to determine how and at what stages specific communication interventions affect vaccination attitudes and behavior . measuring process outcomes allows the identification of components of the package that are effective and indicates whether the end effect is related to implementation issues or to the intervention itself . along with vaccination timeliness and completion , it is prudent to measure outcomes of communication such as informed decision - making , satisfaction , and decisional conflict . these reflect good and ethical process and are essential if we are to ensure sustainable trust at an individual and population level . a committed , confident and competent vaccination workforce is integral to ensuring high vaccine coverage . . there should be sufficient time devoted to vaccination in university curriculum and continuing education . if health professionals have a nuanced understanding of vaccines and vaccine hesitancy , they will be better prepared for a guiding partnership in vaccine decisions with parents . another emerging priority is better engagement of maternity care nurses and complementary and alternative medicine practitioners who have a voice at crucial times of vaccine decision making in key groups . for over two hundred years , industrialized countries have sustained the political will , financial support , purchasing structures , cold chain , program management , workforce capacity , and communications to ensure that most children get most or all the vaccines recommended to them . vaccine hesitancy presents particular challenges because the attitudes and beliefs underlying it may be self - sustaining and not be amenable to centralized and administrative efforts . undoubtedly though , the global vaccination community has the capacity to address vaccine hesitancy . it will be able to do this if there is sufficient political will , professional commitment , and research investment to develop and evaluate new and innovative solutions that make a meaningful difference .

public acceptance of vaccination has never been a given . today there is a set of societal circumstances that may contribute to a growing parental hesitancy about vaccination . these include : increasingly crowded vaccination schedules ; lower prevalence of vaccine - preventable diseases ; greater access to , and more rapid dissemination of , vaccine - critical messages via digital networks ; hyper - vigilance of parents in relation to children and risk ; and an increasingly consumerist orientation to healthcare .

chronic itch , which is defined as itch lasting more than 6 weeks , is a prevalent problem that occurs in ~10% of the population ( mollanazar et al . , 2015 ) . chronic itch conditions negatively affect quality of life , and yet there are no therapies that are both efficacious and selective for itch . the lack of effective treatment is partly attributable to a poor understanding of the mechanisms that underlie it . although antihistamines are frequently prescribed as a treatment for itch , they are typically ineffective because most types of chronic itch are not histamine - mediated ( mollanazar et al . , 2015 ) . unfortunately , while there are numerous mediators that can cause itch , the factors that are responsible in most circumstances of chronic itch are largely unknown . human psychophysical studies have shown that application of serotonin into the skin causes itch ( weisshaar et al . , 2004 ) . in rodents , serotonin is a key component of mast cells , and it is a potent mediator of itch . however , until recently , the mechanisms through which serotonin causes itch have remained uncertain . many pruritogens bind to metabotropic receptors on primary sensory neurons ; however , these receptors must be coupled to ionotropic channels via intracellular signaling pathways in order to allow sufficient current influx to generate action potentials . several groups have shown that the cation channels trpv1 and trpa1 are coupled to different pruritogen receptors and that they are critical for different forms of itch transmission ( ross , 2011 ) . more specifically , trpv1 is required for histaminergic itch , whereas trpa1 is required for several types of non - histaminergic itch , such as that induced by chloroquine , bam8 - 22 , il-31 , endothelin-1 , thymic stromal lymphopoietin , and bile acids . until recently , whether serotonin receptors were likewise coupled to trps remained unknown . understanding serotonin - mediated itch has been complicated by the fact that there are numerous serotonin receptors that are expressed on primary afferents , as well as on immune mediators that could be involved in itch . it was previously hypothesized that the primary pathway through which serotonin causes itch is via stimulation of histamine release from mast cells . however , contrary to this idea , antihistamines failed to reduce serotonin - induced itch sensation in humans ( hosogi et al . , 2006 ) . a recent study has demonstrated that one way in which serotonin induces itch is via direct activation of 5-ht7 ( encoded by htr7 ) , which is expressed on subsets of primary sensory afferents ( morita et al . , 2015 ) . in this study , mice lacking either htr7 or trpa1 showed substantially reduced scratching behavior in response to an intradermal injection of a 5-ht7-selective agonist . furthermore , htr7 and trpa1 knockout mice scratched considerably less in a model of atopic dermatitis . however , it seemed likely that this was only part of the serotonin - itch story , because the 5-ht2-selective agonist , -methyl-5ht , is a potent pruritogen in mice . as reported in this issue of jid , the study by carstens and colleagues ( 2015 ) provides further insight into the molecular players involved in serotonin - evoked itch by defining a trpv4-dependent pathway that is likely to be downstream of 5-ht2-mediated itch . the original goal of this study was to investigate a possible role for trpv4 in itch . trpv4 is upregulated in the skin of individuals with certain itch conditions ( moore et al . , 2013 ; yang et al . , 2015 interestingly , trpv4 knockout mice displayed a significant reduction in scratching behavior in response to serotonin , but not to histamine , chloroquine , or sligrl ( akiyama et al . a trpv4 antagonist also reduced substantially the amount of serotonin - evoked scratching , supporting the idea that trpv4 is critical to serotonin signaling in normal mice . importantly , the authors showed that the change in response to serotonin in the trpv4 knockout mice was specifically a decrease in serotonin - evoked itch behaviors , and not a change in serotonin - evoked pain behaviors . this study demonstrates that trpv4 is a key downstream component of serotonin - evoked itch ( figure 1 ) . in order to link serotonin to trpv4 and the activation of sensory neurons serotonin - mediated activation was dependent on trpv4 , as a trpv4 antagonist reduced significantly the calcium response to the application of serotonin . in support of this finding , the authors demonstrated that the proportion of neurons that responded to serotonin was reduced significantly in trpv4 knockout mice . interestingly , the proportion of neurons responding to other types of pruritogens did not change in mice lacking trpv4 , indicating that trpv4 plays an important and specific role in responses to serotonin in primary sensory neurons . to identify the receptor through which serotonin acts , akiyama et al . the 5-ht2 antagonist , but not the 5-ht1 antagonist , reduced serotonin - evoked scratching . this finding raises the possibility that 5-ht2 , acting via trpv4 , is key mediator of serotonin - evoked itch . thus , there appear to be at least two distinct pathways through which serotonin mediates itch : a trpa1-dependant pathway that mediates 5-ht7-mediated itch , as well as a trpv4-dependent pathway that likely mediates 5-ht2-mediated itch . what remains to be tested is whether these receptors are expressed on distinct or overlapping populations of primary sensory afferents .

previous studies have revealed that trpv1 and trpa1 function downstream of many itch receptors , where they mediate inward current to trigger action potentials in primary afferents . although other trp channels , such as trpv4 , are expressed in primary afferents , whether or not they play an analogous role in itch was previously unknown . now , akiyama et al . provide evidence that trpv4 is a key mediator of serotonin - induced itch . this finding is important because it uncovers an unanticipated role for trpv4 in itch , thereby identifying a novel therapeutic target .

pelvic pain results from many causes such as primary dysmenorrhea , uterine anomalies , menstrual outflow obstruction , endometriosis , myoma and adenomyosis . a 15-year - old nulligravida young woman with a history of severe intermittent pelvic pain presented a 4 - 5 centimeter mass . laparotomy revealed left rudimentary horn , non - communication was confirmed by postoperative hysterosalpingogram ( hsg ) and magnetic resonance imaging ( mri ) . rudimentary horn should be considered in differentiation of pelvic pain and mass in young females . dysmenorrhea is categorized as primary when no pelvic pathology is revealed and secondary when there is a known pathological source . characteristics of secondary dysmenorrhea include beginning of pain 7 - 14 days before menstruation , continuation of pain after menstruation , resistance to non - steroidal anti - inflammatory drugs and contraceptive pills . in case of secondary dysmenorrhea , uterine and vaginal anomalies , menstrual outflow obstruction , endometriosis , adenomyosis and uterine myoma are considered in the differential diagnosis ( 1 ) . some of the congenital abnormalities in the mullerian duct present with dysmenorrhea including imperforated hymen , cervical atresia , transverse septum of the vagina and rudimentary horn besides unicorn unite uterus . in imperforated hymen , complete absence of the cervix might result in pain and obstruction for which hysterectomy is necessary ( 3 ) . the incidence of mullerian duct malformation in the general population is estimated to be 43% while that of unicornuate uterus is about 0.4% ( 4 ) . in another study ultrasonography performed two months ago , revealed a mass of 4.5 centimeters in her left adnexa . another sonography described the mass as a 5 cm solid tumor in favor of uterine myoma on the left side . expert sonography of this center and color doppler velocimetry reported a 5.3 centimeters myoma on the left side with high vascular flow suggesting leiomyosarcoma or degenerated myoma . surgical findings revealed bilateral normal appearing ovaries . on the left side there was a solid mass with the fallopian tube connected superior to this mass and in a lower position in comparison to the right side . a rudimentary horn was considered . thus , a small incision was made on the mass extracting about 2 cm of chocolate fluid compatible with old blood . exploration of the cavity by a narrow metal applicator , showed a small cavity without an outflow pathway . at this stage , blue dye transit from fallopian tubes occurred without entry to the peritoneal cavity . due to no bilateral entrance of dye , while passing from tubes , technical problems was not ruled - out . in the operative field non - communication of the rudimentary horn biopsy of rudimentary mass and cavity was done for pathological review and surgical intervention was not taken . the treatment plan was the use of contraception while hysterosalpingography and mri were performed to achieve definite diagnosis before the next surgery . unfortunately the patient rejected surgery due to post - surgical pain relief resulting from evacuation of chocolate fluid . pathological report of mass biopsy suggested a " vascular myoma " and cavity biopsy reported " inactive endometrium " . hysterosalpingography ( hsg ) report indicated a right unicornuate uterus with normal right fallopian tube and normal peritoneal spillage . the other side of the rudimentary horn and fallopian tube was not shown by the hsg examination . the mri report about 1.5 month after the operation revealed a 4 cm mass on the left side of the uterus with a small central cystic blood - containing locus of about 1.1 cm . the patient accepted a third operation after menarche and resection of rudimentary horn and left fallopian tube was done about 10 months after the second surgery , due to recurrence of severe pain . uterine anomalies are rare and they are seldom considered in differential diagnosis of patients with clinical presentation of pelvic pain ( 6 ) . in the present case , uterine myoma was considered as the first diagnosis and uterine anomalies were not mentioned in differential diagnosis . most rudimentary horns are asymptomatic due to their non - functional and non - communicating pattern ( 3 ) . in the case of endometric development in rudimentary horn , menstrual fluid is produced and implantation of conception product might occur . thus , rudimentary horn pregnancy and pelvic pain occur . rudimentary horn pregnancy mimics the clinical presentation of ectopic pregnancy resulting in surgical emergency ( 7 ) . ultrasound revealed an empty uterus and free fluid in the abdominal cavity . in the laparotomy procedure , rudimentary another case report of ruptured rudimentary horn pregnancy at 19 weeks was by hassan et al . rudimentary horn pregnancy is visually miss - diagnosed until the occurrence of rupture and emergency . there is a case report on an asymptomatic 33-year - old pregnant woman who was diagnosed by laparoscopy - hysteroscopy following the diagnosis of ectopic pregnancy . in the laparoscopy , this case cooperated due to hysteroscopic and pre - rupture diagnosis . in our case , intraoperating dye injection was done , as well , although definite diagnosis was not done . as mentioned earlier , diagnosis of rudimentary horn is not made until the reproductive age when ruptured rudimentary horn pregnancy or pelvic pain occur . pain is due to endometrial response to hormonal activities after menarche . in the present case , her history included several surgical interventions and miss diagnosis with appendicitis and other entities . in the rare case of unicornete non - communicating horn , surgical resection is necessary to relief pain and prevent rudimentary horn pregnancy and resulting emergency situations ( 10 ) . surgery was done more than two years after menarche as the third surgical intervention . in the first surgery diagnosis was appendicitis due to pain . after the second surgical intervention definite diagnosis was made . due to transient pain relief after the second surgery , the patient and her family rejected definite surgery until she experienced severe pain again within 10 months . intraoperative limitation of case management ( in the second surgery ) was inability to show open tube on the right side , thus definite diagnosis and surgery at that stage was not possible . hysteroscopy was acceptable , although it was not done due to the inability to diagnose this case . rudimentary horn should be considered in cyclic pelvic pain starting after menarche besides a mass like lesion , sometimes mistaken by myoma . early diagnosis is needed to relief pain and prevent surgical emergency by rudimentary horn resection .

introduction : pelvic pain results from many causes such as primary dysmenorrhea , uterine anomalies , menstrual outflow obstruction , endometriosis , myoma and adenomyosis . this study reports on a rare case of non - communicating functional rudimentary horn.case presentations : a 15-year - old nulligravida young woman with a history of severe intermittent pelvic pain presented a 4 - 5 centimeter mass . a surgical procedure for appendicitis was previously performed on this patient . per - operative diagnosis was myoma and suspicion of leismus sarcoma . laparotomy revealed left rudimentary horn , non - communication was confirmed by postoperative hysterosalpingogram ( hsg ) and magnetic resonance imaging ( mri ) . resection of mass and left fallopian tube was done during the second surgery.conclusions:rudimentary horn should be considered in differentiation of pelvic pain and mass in young females . early diagnosis and horn resection prevents emergency surgery and reliefs pain .

urea cycle disorders ( ucd ) are among the most common inborn errors of metabolism of the liver . citrullinemia can be classified into three types : neonatal / infantile ( types i and iii ) and the adult form ( type ii ) . more than 20 patients with citrullinemia have undergone liver transplantation , with favorable therapeutic results . however , the number of cases with auxiliary partial orthotopic liver transplantation ( apolt ) is limited . apolt was initially performed in patients with acute liver failure and non - cirrhotic metabolic liver disease to compensate for enzyme deficiencies without complete removal of the native liver and for small - for - size living donor grafts [ 3 - 5 ] . our patient was a 27-year - old man who was diagnosed with adult - onset type ii citrullinemia ( ctln2 ) at age 24 . the patient had been treated with a low protein diet and oral administration of lactulose . recently , the patient has had more frequent alterations of his mental status with irritability and altered consciousness . even though oral administration of l - arginine , kanamycin and intravenous hyperalimentation with branched chain amino acids was started , his plasma levels of ammonia and citrulline were highly elevated , up to 844 g / dl ( normal range , 19 to 87 g / dl ) and 682 mol / l ( normal range , 12 to 55 mol / l ) , respectively . despite the conservative medical treatment including alternative pathway medication , intermittent continuous renal replacement therapy and hemodialysis , his condition failed to improve . therefore , living donor liver transplantation was planned using a right lobe graft from his 25-year - old sister . however , the estimated volume of the donor 's right lobe was 800 ml compared to 180 ml of the left and in addition there was a type iii portal vein anomaly . we eventually decided to perform an apolt using the extended left lobe of the liver from the donor . the actual graft volume was 200 g and the graft - to - recipient weight ratio ( grwr ) was only 0.33% . the patient 's extended left lobe ( including caudate lobe ) was resected and the extended left lobe graft ( including middle and left hepatic vein ) was transplanted orthotopically . the left and middle hepatic vein trunk of the extended left lobe graft was anastomosed to the left and middle hepatic vein trunk of the recipient in end - to - end fashion with interposition venoplasty using the recipient 's greater saphenous vein as a patch . the left portal vein of the graft was anastomosed to that of the recipient in end - to - end fashion . considering that the graft was too small , the recipient 's right portal vein was narrowed by surgical clipping , instead of total diversion , for prevention of the development of portal venous hypertension . the left hepatic artery of the graft was anastomosed to that of the recipients in end - to - end fashion . intraoperative doppler ultrasonography demonstrated that portal and arterial blood flow were well maintained in both the native liver and the graft . on postoperative day 7 , abdominal computed tomography demonstrated orthotopically transplanted small left lobe graft with well - enhanced pattern ( fig . the post - operative course was uneventful and the patient was discharged without any problems . the plasma concentrations of ammonia were at the upper limits of normal , so only a low protein diet ( 50 g / day ) was supplied without any specific medication . on post - operative day 33 , the patient reported a sudden severe visual disturbance and the plasma levels of ammonia were noted to have increased up to 209 g / dl . the estimated computed tomography volume of the graft was decreased to 190 ml and a left portal vein stenosis was suspected . immediate coil embolization of 8th segmental branch of right anterior portal vein and self - expandable stent placement for left portal vein was performed in order to increase the portal flow to the graft ( fig . stent insertion day 7 , the calculated computed tomography volume of the graft was increased to 250 ml ( fig . apolt was initially introduced as a temporary or permanent support for patients with fulminant hepatic failure . apolt can provide an adequate hepatic mass to correct underlying enzyme deficiencies in adult patients with ucd . this procedure can be carried out to overcome the limitations of graft volume in living donor liver transplantation . in korea , therefore , living donor liver transplantation is the only treatment option for patients with metabolic liver disease such as ucd . in this case , the graft weight of 200 g and the grwr of 0.33% are the smallest graft and lowest margin ever reported to meet the minimum requirements for the demands of adequate metabolic function in adults . because the remaining native liver function , except for the metabolism of ammonia , is normal , the differential diagnosis including portal steal phenomenon and rejection as well as other problems associated with the vascular system is difficult . thus the plasma ammonia level is a valuable follow up marker and should be closely monitored in such patients . in addition , careful monitoring of the portal flow to the graft is important for the detection of delayed graft dysfunction . selective portal vein embolization is a useful method for the increase of portal flow to the graft . in conclusion , apolt can be a life saving procedure and an effective treatment for patients with adult - onset type ii citrullinemia , especially when a deceased donor graft is not available or when candidate donors have insufficient graft volume . however , further confirmation is needed with additional patients and long - term follow - up . how much volume of graft can be transplanted successfully is questionable in cases of inborn error of metabolism , such as ucd .

adult - onset type ii citrullinemia ( ctln2 ) is a disorder caused by an inborn error of metabolism affecting the liver . ctln2 is an autosomal recessive disorder characterized by recurrent encephalopathy with hyperammonemia due to highly elevated plasma levels of citrulline and ammonia , caused by a deficiency of argininosuccinate synthetase in the liver . a small number of patients have undergone liver transplantation with favorable results . in korea , the limitations of the deceased donor pool have made living donor liver transplantation a common alternative treatment option . we report the case of a patient with type ii citrullinemia who was treated successfully with auxiliary partial orthotopic liver transplantation ( apolt ) from a living donor . this is the first description of an apolt for a patient with adult onset type ii citrullinemia in korea .

so many authors have studied clinostomum parasites , for example , the few recent studies like ( 14 ) are well documented . however , record on the occurrence of co - infection among three clinostomum species is unknown . the present study is the first record of co infection of three clinostomum species and report on the parasites of opi lake . 29 35 e is a tropical freshwater lake located in the valley of river uhere , northeast of nsukka , nigeria . the lake is about 300 meters from uhere river , the soil is porous and subject to severe erosion ( 5 ) . however , the best knowledge about the lake was information on the systematic and basic biology of the species of organisms other than parasites found there . for instance , the climate , hydrobiology , macro invertebrates , and vegetation of the dominant flora have been described ( 69 ) . therefore , investigation into its parasites and description of co infection by three clinostomum species was necessary . species of tilapia zilii ( 10 ) were collected using multiple fishing gear ; cast nets , hook and line , and seine nets ( 150 mm 200 mm ) monthly for twelve months from nov 2007 oct 2008 . rank - abundance and species diversity of the parasites were determined using quantitative analysis of shannon - wienner index ( 14 ) . three metacercariae of clinostomatids were discovered to be the parasites associated with the t. zilii of opi lake . the parasites include : clinostomum tilapiae ukoli , 1966 , c. complanatum rudolphi , 1819 , and euclinostomum heterostomum rudolphi , 1809 . c. complanatum ( 9.4 % ) , e. heterostomum ( 10.4 % ) , and c. tilapiae ( 4.8 % ) with mean intensity ; c. complanatum ( 4.2 ) , c. tilapiae ( 4.6 ) , and e. heterostomum ( 2.1 ) ( table 13 ) . out of 392 t. zilii examined , 38 were infected with c. complanatum ; c. tilapiae 19 , and 41 hosts had e. heterostomum . c. complanatum and c. tilapiae were equally abundant ( pi 0.26 ) , while e. heterostomum was most abundant ( pi 0.48 ) ( fig . 1 ) . three major microhabitats buccal cavity , eye , and skin were seriously affected . in addition , the infection of the buccal cavity had the highest infection than the other two microhabitats of the eye and skin . implications of their presence caused pronounced inflammation as well as roughening of the skin by the encysting metacercariae . out of 85 e. heterostomum only 15 were not excysted , 57 c. tilapiae and 112 of c. complanatum were encysted respectively . these excysted forms caused serious damages to the infected fish as they burrowed through the organs of the host . damages to the skin , blindness and bumps on the skin could affect the palatability and marketability of the infected fish as well as the acceptance of fish as the primary source of animal protein . for instance , nigeria with population of over 120 million individuals is the largest consumer of fish in africa and fish serve as primary source of animal protein to this huge population . the crowding population of opi around the lake is also prone to the inflammation of the naso - pharynx known as pharyngitis . this is because ingestion of under cooked fish with clinostomum infection results in the attachment of the trematode on the pharynx . spatial adaptation of theses clinostomatids in the various microhabitats of t. zilii indicate selection for relatively better adaptiveness , and host location . consequently , this resulted in a trade - off among excysted individuals , encysted individuals , damages to organs by scavenging large population size of the parasites in the host . adaptation is a heritable trait that either spread because of natural selection or has been maintained by selection to the present or currently spreading relative to alternative traits because of natural selection . in all such cases , the trait in question has conferred and continues to confer or is just beginning to confer higher genetic or reproductive success on c. complanatum with highest population than the other two species of the parasites . even with more encysted forms , adsorption of nutrients such as free proteins , amino acids and transaminases was readily very successful ( 15 ) . fitness is a measure of an individual s reproductive or genetic success , so that fitness benefit refers to the positive effect of a trait on the number of surviving offspring produced by an individual or the number of genes it contributes to the next generation whereas fitness cost refers to the damaging effects of the trait on these measures of individual genetic success ( 16 ) . although , encysted larvae can feed from the host , excysted larvae could be more voracious feeder . these suggestive pressures on natural selection would favor e. heterostomum in t. zilii during the cause of time and possible domination in the fish hosts in this freshwater lake . also , because the parasites do not depend on stored food of the host ; the nutrients they can obtain from their intermediate hosts are sustainable . it might last throughout the lives of the host fish due to difficulty in locating their definitive hosts , piscivorous birds such as egreta egreta ( 17 ) . ethical issues ( including plagiarism , informed consent , misconduct , data fabrication and/or falsification , double publication and/or submission , redundancy , etc ) have been completely observed by the authors .

the present study investigated the effects of first record of co - infection of three clinostomum sp . ; clinostomum complanatum ( rudolphi , 1819 ) , c. tilapiae ( ukoli , 1966 ) , and euclinostomum hetereostomum ( 1809 ) in tilapia zilii . there was differential parasitic effects resulting in selection for relatively better adaptiveness to host s microhabitats , more population size , and frequent host location of these parasites during the one year survey ( nov 2007oct 2008 ) in opi lake , nigeria . prevalence of 9.4 % was recorded in c. complanatum , 10.4 % in e. heterostomum and 4.8% in c. tilapiae . the parasites were recovered from three major microhabitats of buccal cavity , skin and eye .

cases were identified through the hospital - based active surveillance system of the hfrs maintained by the korean army . cases occurring from january 1 , 2002 , to january 1 , 2004 , were enrolled prospectively . for early detection of hfrs , patients with hfrs may have sudden onset of fever ; experience pain in the head , abdomen , and lower back ; and report bloodshot eyes and blurry vision . the patient 's face , chest , abdomen , and back often appear flushed and red , as if sunburned . a confirmed case of hfrs is defined as a positive result on the high - density particle agglutination test . for each case , one control was selected from among the other patients at each hospital where the case - patient had been hospitalized . the control was matched with the case - patient according to unit , age at the time of hospitalization ( 3 years ) , date of hospitalization ( 3 months ) , and date of transfer to the present unit ( 3 months ) . if no suitable control could be found , the intervals around the case - patient 's unit , age , date of hospitalization and date of transference were progressively widened until one or more potential controls were found . for each case - patient , all eligible controls were listed , and one suitably matched control was identified at random . as with the case - patients , the final decisions about each control patient 's eligibility for the study were made on the basis of a detailed review of hospital records . decisions about the eligibility of potential controls were made without knowledge of their vaccination status . vaccine had to be received at least 3 weeks before hospitalization because of the time required for antibodies to develop and because the incubation period is 3 weeks on average ( 5 ) . estimates of the relative odds of hfrs associated with vaccination were estimated by using methods developed by mantel and haenszel ( 6 ) , which are appropriate for matched designs . the protective effectiveness of the vaccine was estimated as 1 minus the relative odds associated with vaccine use , times 100 . ninety - five percent confidence limits for the effectiveness were derived from the 95% confidence interval ( ci ) of the relative odds . chicago , il ) . from january 1 , 2002 , to january 1 , 2004 , a total of 57 hfrs cases were identified among troops of the korean army . of one of three deaths occurred in previously healthy , vaccinated ( first and second doses ) personnel . twelve , 9 , and 2 cases occurred in personnel who were vaccinated with one , two , and three doses , respectively . most cases occurred in october ( 15.7% ) , november ( 35.7% ) , and december ( 17.1% ) , although disease also occurred during the spring and the summer ( figure 2 ) . hemorrhagic fever with renal syndrome cases among republic of korea military personnel , by month of onset , january 2002 to january 2004 . of the 54 persons identified with hfrs from january 2002 to january 2004 , those who were vaccinated within 3 weeks of hospitalization were excluded from analysis . because the effectiveness was calculated by comparing each dose ( exactly one to three ) with no vaccination , case - patients or controls not applicable to that comparison were excluded from each matched set . finally , 41 , 38 , and 31 matched sets were formed for one , two , and three doses of the hantavirus vaccine , respectively ( table ) . estimates of vaccine effectiveness according to the number of doses received rose from 25% ( 95% ci 78% to 68% ) for one dose to 46% ( 95% ci 35% to 78% ) for two doses to 75 % ( 95% ci 18% to 95% ) for three doses . when recipients for whom 1 year had passed since their second dose were excluded , effectiveness of two doses increased markedly to 70% ( 95% ci 9% to 92% ) . the protective effectiveness of the vaccine strongly depends on the number of doses . in particular , effectiveness increased when persons for whom > 1 year had passed since their second dose were excluded , which suggests that the protective effect of the second primary vaccination does not persist beyond the period recommended for having the booster dose . in addition , we do not know whether the recommended immunization schedule was optimal for military personnel and farmers , groups for whom hantavirus vaccination is recommended . the vaccination schedule should be epidemiologically relevant , immunologically effective , operationally feasible , and socially acceptable ( 7 ) . in a field study from the former republic of yugoslavia conducted by korean researchers , including the developers of the hantavirus vaccine ( 8) , no case of hfrs was observed among 1,900 vaccinees , while 20 confirmed cases were observed among 2,000 nonvaccinated controls . considering that our study showed low protective effectiveness for one or two doses , that no case of hfrs occurred in yugoslavian vaccinees before they received the full three doses was surprising . because the case - control studies were not experimental , they may be subject to biases . the most important potential biases that might affect this kind of study are detection and selection bias . if all cases of hfrs were identified , no detection bias would occur . because patients were identified prospectively by active surveillance , we believe that virtually all cases of hfrs diagnosed during the study period were identified . selection bias may occur when controls do not represent the general population . in this study , controls were selected randomly from a list of potentially eligible controls by using a systemic algorithm . confounding influences affect the results of a case - control study if controls differ from case - patients in characteristics related to risk of contracting the disease and likelihood of receiving the vaccine . since the population of this study consisted of military personnel , bias due to sociodemographic differences may be negligible . therefore , other candidate confounding factors determined by considering the military milieu were used as matching variables . therefore , while point estimates show effectiveness , this finding could be due to chance . of course , the range of point estimates in studies with relatively small samples can be wide , and wide confidence intervals that include zero are not uncommon in many studies on vaccine effectiveness ( 9 ) . however , caution is appropriate in interpreting our estimates of vaccine effectiveness . finally , this study represents a short - term ( 7.3 months average ) evaluation of protective effectiveness of three doses of the hantavirus vaccine . to assess the long - term effectiveness , protection

a case - control study in the republic of korea evaluated the protective effectiveness of the hantavirus vaccine . point estimates showed increasing effectiveness with increasing numbers of doses received : 25% for one dose , 46% for two doses , and 75% for three doses . all 95% confidence intervals overlapped zero ; therefore , the findings could be due to chance .

our clinical unit has recently offered mental health care at asylum seekers , rescued from ships from the horrors of wars , as have many other psychiatric facilities in sardinia , sicily and greece . in fact the number of migrants crossing the mediterranean towards europe has dramatically increased in 2015 ; as the number of incidents and deaths [ 2 , 3 ] . this editorial summarizes our activity and the results of our work and highlights some critical aspects that hinder the care to asylum seekers with stress related disorders . screening for mental distress was performed in all migrant joint three camps in sardinia over january september 2015 using k6 , short screening scale for post traumatic stress disorder ( ptsd)(french , english and arabic validated versions ) and with an interview on wellness conducted by psychologists with cultural facilitators . people with psychiatric needs have been treated and evaluated at the start of treatment and three months later by means of cgi - s . in a sample of 860 asylum seekers ( 21.7% female , age : 36.9+/-14.7 ) , we found that 190 ( 22.1% of sample , 22.6% female , age ; 38.5+/-12.9 ) were positive for at least one screener ; seventy - five ( 8.7% , 24% female , age 36.9+/-13.3 ) had a diagnosis of depressive or bipolar dsm-5 disorder ( md ) and 56 ( 7.6% , 25% female ; age 39.4+/-12.6 ) of ptsd or ptsd plus md . syrians showed the higher risk of ptsd against other groups ( or=6.24 , ic95% 1.20 - 28.0 ) . psychiatric disorders were treated with : antidepressants ( 64.1% ) mood stabilizers ( 24.4% ) ; antipsychotics ( 4.5% ) and/or psychological support ( 100% ) . after three months of treatment : 51 treated people ( 26.8% ) had left the camps without giving any explanation ; some were stopped by illegally trying to escape from italy . those who remained in the hosting facilities had psychopathological conditions worse than those that had gone away , cgi - s score 3.86+/-0.8 vs 3.45 + /-0.9 ( df 1,188 , 189 , f= 9.15 , p=0.003 ) ; 70.8% of those remaining declared that they should not be officially registered in italy as destination of arrival because under european union dublin regulation they have to stay in the country of first asylum ; 53.1% had relatives in northern europe that they wanted to reach . in those who stay , cgi - s score change from 3,86+/-0.8 to 3.69+/-0.9 at three months ( df 1,276 , 272 , f= 2 , 77 , p=0.010 ) . only 8 ( 8.3% ) showed a significant clinical improvement , scoring 2 points or less at gci - s . dissatisfaction and feeling of prisoners because they could not join relatives may have had a negative impact on outcome . studies have shown that social support influenced long - term response to trauma and the lack of social contacts in exile predicted the maintenance of ptsd symptoms . thus in severe ptsd , with the experience of torture and seeing family members killed , staying with surviving relatives in stable conditions would be an important part of treatment . from this point of view , the dublin regulation seems not to be in agreement with articles 23 and 25 of the un convention on the rights of persons with disabilities , which the european union has signed , because in these rules reunification with families is taken into consideration but it is impossible to do due to the long burocracy required ( and in fact no migrant asked for reunification ) . this condition is a de facto denial of the right to appropriate treatment for asylum seekers with ptsd with doubt of illegality in face to the un convention .

background : the migrants crossing the mediterranean towards europe have dramatically been increased in 2015 as the number of incidents and deathsobjective : this editorial summarizes the results of our work and highlights some critical aspects that hinder the care to asylum seekers with stress disorders.method:screening for mental disorders was performed in all migrants joint three camps in sardinia ( january - september 2015 ) using k6 , short screening scale for post traumatic stress disorder ( ptsd ) and with an interview . positives were evaluated by psychiatrists and if they needed , have been treated and evaluated at the start of treatment and three months later.results:22.1% of the sample , ( 22.6% female , 38.512.9 years ) were positive for at least one screener ; 8.7% , ( 24% female ) had a diagnosis of depressive or bipolar dsm5 disorders and 7.6% , ( 25% female ) of ptsd . after three months of treatment : 51 treated people ( 26.8% ) had left the camps . 53.1% of those remaining declared had relatives in northern europe that they wanted to reach . only 8.3% showed a significant clinical improvement.conclusion:clinical improvement was dramatically poor in people who stay in the camps . dissatisfaction and feeling they could not join relatives may have had a negative impact . in ptsd , with the experience of torture and seeing family members killed , staying with surviving relatives in stable conditions would be an important part of treatment . from this point of view the ue dublin regulation seems not to be in agreement with the un convention on the rights of persons with disabilities

papillary thyroid carcinoma ( ptc ) is the most frequent type of thyroid malignancy , and the usual metastasis sites include the locoregional lymph nodes . distant metastasis of ptc is rare and usually involves the lungs , liver , bones and brain . the skin metastasis of ptc is a very rare condition , and the scalp is the most frequent cutaneous area which is involved . other cutaneous areas that are involved with less frequency are the cheeks , shoulders , arms , abdomen and thighs . a 64 year - old female with past medical history of metastatic papillary thyroid carcinoma ( ptc ) with both liver and lung metastasis presented with a 2.5 3 cm erythematous tender nodule in right parietal scalp of two months duration . she had a past medical history of total thyroidectomy and also had received repeated doses of radioactive iodine ( rai ) therapy in the past two years . her last dose of rai131 ( 200 mci ) was three weeks prior to the time of presentation . she also had a history of subglottic mass as a result of thyroidal carcinoma involvement with severe tracheal stenosis , and had undergone tumoral resection and tracheal anastomosis and partial hemilaryngectomy two years ago because of subglottic involvement by thyroidal carcinoma . high resolution computed tomography ( hrct ) demonstrated multiple metastatic nodules in both lungs and loculated left side pleural effusion consistent with advanced metastatic involvement . a differential diagnosis of pillar cyst and metastasis was given by dermatologist for scalp lesion . histopathologic examination of the lesion revealed skin tissue with tumoral involvement of the dermis composed of numerous follicles ( likely thyroid follicles ) , lined by relatively large cuboidal epithelial cells with round nuclei , fine chromatin pattern , little granular cytoplasm and eosinophilic colloidal material within some of follicles , and mitosis , which was consistent with metastatic thyroidal carcinoma [ figures 1 and 2 ] . she was discharged with suppressive therapy with levothyroxine and referred to oncology clinic for continuation of the treatment plan . scalp lesion biopsy showing skin tissue with tumoral infiltration of dermis by numerous follicles ( likely thyroid follicle ) , consistent with metastatic thyroidal carcinoma ( h and e , 40 ) scalp metastasis of thyroidal carcinoma composed of numerous follicles lined by relatively large cuboidal epithelial cells with round nuclei , fine chromatin , little granular cytoplasm and eosinophilic colloid material within some of follicles with mitotic division ( h and e , 400 ) the skin metastasis of ptc is mostly associated with aggressive and disseminated disease and shows a poor outcome . some authors suggested that the average patient survival time after discovering of skin metastasis is 19 months . here , we have reported a case of scalp metastasis of ptc in a patient with metastatic pulmonary and liver involvement . metastasis of thyroid cancer should be considered in the differential diagnosis of scalp tumors in any patient with a history of thyroidal carcinoma ; and , excisional skin biopsy is necessary for confirming the diagnosis .

the skin metastasis of papillary thyroid carcinoma ( ptc ) is a rare condition and the lesions should be differentiated from primary skin tumors . the scalp is the most frequent site which is involved in skin metastasis of ptc . it shows the poor outcome and aggressive nature of disease . in this report , we aim to present a case report of a 64 year - old female with scalp metastasis of ptc in the context of disseminated pulmonary and liver metastasis .

necrobiosis lipoidica ( nl ) is a rare chronic granulomatous dermatitis first described in 1929 by oppenheim that usually appears in the lower extremities that affects about 0.31.2% of diabetic patients , the majority of whom have type 1 diabetes . we report a case of a 16 y old girl who developed lesions on the lower extremities and in the interscapular area , which is an unusual location . the patient , a caucasian 16-y - old girl , was born at term by non - consanguineous , healthy parents . type 1 diabetes ( t1 dm ) had been diagnosed at one year of age . our patient s medical history was otherwise unremarkable . until the age of 12 she maintained an adequate glucose control with hba1c < 34 mmol / mol . thereafter her glucose control progressively worsened with a hba1c of 86 mmol / mol . at the age of 16 , during a routine diabetes follow - up visit , the patient presented with erythematous subcutaneous nodular lesions which had appeared 4 mo earlier , initially in the pretibial area and subsequently in the interscapular area . her hba1c was 64 mmol / mol and daily insulin requirements were 0.95 u / kg / day . in the suspect of necrobiosis lipoidica , a skin biopsy was performed ( lower extremities and interscapular area ) ( figs . 1 and 2 ) the smaller lesions in the interscapular area showed signs of perivascular dermatitis which could be consistent with early stages of necrobiosis lipoidica ( fig . it may initially be represented by erythematous circular papules which may gradually progress to yellowish - brown , telangectatic plaques with central atrophy and raised violaceous border . the etiology of nl is still currently elusive and few data exist regarding the pediatric population . however , due to the significant relationship with diabetes ( 7590% of patients with nl have or will develop t1 dm ) , many studies have proposed microangiopathy as leading etiological suspect . immune - complex vasculitis and collagen abnormalities may also represent potential underlying causes . previous studies have shown that glucose control does not appear to be correlated with nl onset and severity . however , a study by cohen concluded on the contrary , that nl is usually associated with poor glucose control and that a tighter glucose control , as currently practiced , might improve or prevent this disorder . diagnosis of nl is mainly clinical , but a bioptic evaluation may be useful especially in the early stages of disease . differential diagnosis include erythema nodosum , lupus panniculitis , granuloma annulare , sarcoidosis and amyloidosis . microscopic evaluation usually shows degeneration of collagen , granulomatous inflammation of subcutaneous tissues and of blood vessels , capillary basement membrane thickening and obliteration of vessel lumen . other therapeutic options are antiplatelet agents , cyclosporine , thalidomide , clofazimine , anti - tnf agents , fumaric acid esters , puva , photodynamic therapy , hydroxychloroquine , and tacrolimus . in patients with t1 dm , diagnosis of nl of the lower legs is usually unequivocal . however , diagnosis may be more challenging in the presence of lesions with recent onset and/or atypical clinical presentation and unusual site . in these cases , nl must always be taken in consideration in order to avoid misdiagnosis , wrong / late treatment decisions and progression to ulceration . written informed consent was obtained from the patient and parents for publication of this manuscript and any accompanying images . a copy of the written consent is available for review by the editor - in - chief of this journal .

introduction : necrobiosis lipoidica ( nl ) is a rare chronic granulomatous dermatitis that usually appears in the lower extremities . it affects about 0.31.2% of diabetic patients , the majority of whom have type 1 diabetes . the etiology and pathogenesis of this disorder are still unclear . nl is characterized by skin rash that usually affects the shins . the average onset is 30 years , with females being affected more commonly . there are very few reported cases of necrobiosis lipoidica in children . case report : we report a case of a 16 year old girl affected by type 1 diabetes mellitus ( 15 years disease duration ) who developed an erythematous nodular rash on the lower extremities and interscapular area . in the suspect of necrobiosis lipoidica , a skin biopsy was performed ( lower extremities and interscapular area ) . the microscopic evaluation of the pretibial lesions was suggestive of necrobiosis lipoidica . the smaller lesions in the interscapular area showed signs of perivascular dermatitis which could be consistent with early stages of necrobiosis lipoidica . local treatment with tacrolimus determined a progressive improvement of the lesions . conclusion : in patients with t1 dm , diagnosis of nl of the lower legs is usually unequivocal . however , diagnosis may be more challenging in the presence of lesions with recent onset and/or atypical clinical presentation and unusual site . in these cases , nl must always be taken in consideration in order to avoid misdiagnosis , wrong / late treatment decisions and progression to ulceration .

flurodeoxyglucose ( f - fdg ) positron emission tomography / computed tomography ( pet / ct ) has become the imaging modality of choice for initial staging , follow - up and treatment response assessment in patients with hodgkin 's lymphoma and has proved superior to contrast enhanced ct ( cect ) in these settings . f - fdg pet / ct has accuracy of almost 100% in diagnosing primary splenic involvement during initial staging of lymphoma . however , in the post - therapy setting its role for evaluation of secondary splenic involvement is limited . one of the pattern of splenic activity that help to detect splenic involvement on pet / ct is diffusely increased f - fdg uptake greater than that in the liver and bone marrow with or without corresponding ct lesions . in this context , we report a case of a patient with hodgkin 's lymphoma in remission presenting with f - fdg avid splenomegaly . the present case report is about a 22-year - old male patient who presented with enlarged right cervical lymph node . f - fdg pet / cect performed for staging revealed metabolically active lymph nodes on either side of the diaphragm [ figure 1a , broken arrows ] , enlarged spleen with multiple hypodense hypermetabolic , lesions [ figure 1a , arrow ] ( suvmax= 9.4 ; spleen suvmax / liver suvmax ratio = 3.76 ) and bone lesion . he was then given 6 cycles of chemotherapy and f - fdg pet / cect was done for response evaluation . pet / ct showed complete metabolic response , with normal spleen uptake [ figure 1b , arrow ] ( spleen suvmax= 2.5 , liver suvmax= 2.6 s / l ratio = 0.96 ) . at 1-year later routine follow - up the patient complained of mild fever , lethargy and listlessness . in view of previous history of hodgkin 's lymphoma , pet / ct revealed enlarged spleen with diffusely increased fdg uptake [ figure 2a c , arrow ] ( suvmax= 5.3 ; liver suvmax= 2.3 s / l ratio = 2.30 ) . the first differential in the given clinical scenario was splenic relapse of lymphoma , however , a second differential diagnosis of some infective / inflammatory process was considered . on further evaluation , peripheral smear showed evidence of malaria parasite infection ( plasmodium vivax ) . follow - up pet / ct after 7 months revealed normalization of size and f - fdg uptake of spleen [ figure 2d , arrow ] ( suvmax= 3.0 ; liver suvmax= 2.7 s / l ratio = 1.1 ) . hence , malaria and other relevant ( endemic ) infective possibilities ( kala - azar etc . ) should be considered and further investigation , if warranted , should be advised . f - flurodeoxyglucose ( f - fdg ) positron emission tomography/ computed tomography ( pet / ct ) done for staging and response evaluation . ( a ) staging maximum intensity projection ( mip ) pet image showing multiple metabolically active lymph nodes in thorax , abdomen and pelvis ( broken arrows ) with splenic ( arrow ) and bone lesion ( left femur ) . ( b ) mip pet image done for response evaluation demonstrates complete metabolic response with normal splenic fdg uptake ( arrow ) . physiological gastric fdg uptake noted ( bent arrow ) f - flurodeoxyglucose ( f - fdg ) positron emission tomography / computed tomography ( pet / ct ) done for recurrence detection and follow - up . ( a - c ) follow - up pet / ct performed 1 year after chemotherapy showing enlarged spleen with diffusely increased fdg uptake ( arrow ) . ( d ) maximum intensity projection pet image performed after anti - malarial therapy , showing normalization of size and fdg uptake of spleen ( arrow ) . ( a and d ) focal fdg uptake noted in the pelvis is due to urinary fdg activity in the left ureter ( curved arrows ) pet / ct is a useful modality for staging and restaging of hodgkin 's lymphoma with high sensitivity and specificity . furthermore , it is a sensitive modality for early detection of relapse in asymptomatic patients making surveillance f - fdg pet / ct clinically important . splenic uptake on f - fdg pet can be due to a wide variety of causes such as lymphoma , anemia , granulocyte colony stimulating factor treatment , beta - thalassemia , inflammation and infections . splenic uptake , greater than hepatic uptake , is a relatively reliable indicator of lymphomatous involvement of the spleen , in the absence of recent cytokine administration . in early stage hiv infection , diffusely increased splenic uptake is noted due to reactive stimulation of b - cells in the spleen . post - therapeutic reactive splenic uptake is also noted after administration of granulocyte colony - stimulating factor for myelosuppression or high - dose interferon - alpha-2b adjuvant therapy for melanoma . until date , only a single report by liu et al . have demonstrated f - fdg uptake in spleen in case of malaria .

18flurodeoxyglucose ( 18f - fdg ) positron emission tomography / computed tomography ( pet / ct ) is nowadays routinely used in management of lymphoma patients . we here present a case of hodgkin 's lymphoma which showed 18f - fdg avid splenomegaly on pet / ct done for clinically suspected relapse . further evaluation by peripheral smear examination revealed malaria . the patient was then started on anti - malarial medications and follow - up pet / ct revealed resolution of hypermetabolic splenomegaly . this report highlights that in endemic regions malaria can cause 18f - fdg avid splenomegaly and might mimic relapse of lymphoma .

superficial leiomyosarcomas are rare malignant tumors that account for only 510% of all soft tissue sarcomas . they can be subdivided into two types according to its primary site of origin : deep subcutaneous and superficial cutaneous types . primary cutaneous leiomyosarcoma ( pcl ) is a rare subtype with few isolated case reports and occasional case series described in the literature . we report the clinical , histopathological and immunohistochemical findings in a 70-year - old male patient with pcl arising in the right leg . a 70-year - old male patient presented with a painful nodule in the right leg , which had been progressively increasing in size for 4 months . on palpation , an irregular , firm , tender exophytic swelling , measuring 5 cm 5 cm , with overlying skin showing ulceration was noted on the lateral aspect of the lower one third of the right leg [ figure 1a ] . his past history was significant of a trauma and subsequent non - healing ulcer formation at the same site in the right leg . ( a ) clinical photograph showing an irregular , exophytic swelling , measuring 5 cm 5 cm , with overlying skin showing ulceration on the lateral aspect of the lower one - third of the right leg . ( c ) gross photograph showing greyish white , solid , homogenous and fleshy cut surface of the irregular tumor mass measuring 5 cm 5 cm 2.5 cm systemic examination and investigations , including complete blood count , fasting blood sugar level , liver function test and renal function test , were within normal limits . hepatitis b virus surface antigen and human immunodeficiency virus enzyme - linked immunosorbent assay were non - reactive . a clinical diagnosis of squamous cell carcinoma was considered and fine needle aspiration cytology of the swelling was requested . fine needle aspiration cytology of the same was reported as a spindle cell neoplasm with suspicion of malignancy . the swelling was excised with a 5 cm resection margin and submitted for histopathological examination [ figure 1b ] . gross inspection revealed a grey brown , irregular , nodular soft tissue mass , measuring 5 cm 5 cm 2.5 cm and partly covered with skin . the cut surface of the mass was greyish white , solid , homogenous and fleshy [ figure 1c ] . the neoplasm was composed of bundles of elongated spindle cells arranged in interlacing fascicles , with intensely pink , fibrillary cytoplasm and pleomorphic nuclei with coarse irregularly dispersed chromatin . mitosis , including atypical , amounting to 22 mitotic figures per 10 high power fields and multinucleate tumor giant cells , was evident . ( a ) light microscopy revealed a dermal neoplasm with extension into the subcutaneous tissue ( h & e , 40 ) . ( b ) neoplasm composed of interlacing fascicles of elongated spindle cells ( h & e , 100 ) . ( c ) tumor cells are spindle shaped with intensely pink , fibrillary cytoplasm and pleomorphic hyperchromatic nuclei frequently exhibiting atypical mitosis ( black arrow ) ( h & e , 400 ) . ( d ) tumor cells with markedly pleomorphic nuclei and multinucleate tumor giant cell ( inset ) ( h & e , 400 ) a provisional diagnosis of spindle cell malignant neoplasm of the skin , which typically includes a host of diagnostic possibility , namely fibrosarcoma , leiomyosarcoma , malignant peripheral nerve sheath tumor , monophasic synovial sarcoma , malignant fibrous histiocytoma and spindle cell variant of squamous cell carcinoma , was considered and immunohistochemistry was advised for confirmation . on immunohistochemistry , tumor cells showed strong immunopositivity for vimentin , muscle - specific actin ( msa ) , smooth muscle actin ( sma ) , caldesmon and desmin and negative for pancytokeratin , epithelial membrane antigen ( ema ) , myogenin , cd34 and s-100 protein [ figure 3 ] . immunohistochemical staining : neoplastic cells showing positive immunostaining for ( a ) vimentin ( vimentin , 400 ) , ( b ) caldesmon ( caldesmon , 400 ) , ( c ) muscle - specific actin ( msa , 400 ) and ( d ) smooth muscle actin ( sma , 400 ) based on the immunophenotypic features , a final diagnosis of pcl was rendered . the patient is currently on periodic follow - up since 1 year and no recurrence or metastasis has been identified so far . although pcl most commonly arises in the extremities , particularly hair bearing surfaces , it may occur at any anatomic site on the body . most case series described in the literature report a predilection for the middle aged to elderly male . in a large case series reported by fields and helwig , 95% of the patients with pcl presented with a solitary nodule with a median size of 1.8 cm at presentation . pain was reported by 24% of their patients at presentation , whereas pain could be elicited on pressure in an additional 27% of the patients . histologically , pcl is characterized by a poorly circumscribed proliferation of interwoven fascicles of spindle shaped atypical myomatous cells that merge with a collagenous stroma . mitosis , equivalent to one or more per 10 high - power fields , high cellularity and bizarre myomatous cells are the generally accepted criteria for malignancy . described two growth patterns : a nodular pattern characterized by high cellularity , prominent nuclear atypia , conspicuous mitosis and a diffuse pattern that is less cellular , well differentiated and inconspicuous mitosis . morphologic differential diagnosis includes a galaxy of other malignant spindle cell neoplasms , namely desmoplastic malignant melanoma , spindle cell synovial sarcoma , spindle cell angiosarcoma , fibrosarcoma , malignant fibrous histiocytoma and malignant peripheral nerve sheath tumor . cutaneous leiomyosarcoma often exhibit immunophenotypic polymorphism , thereby mandating the use of a large panel of antibodies . however , none of these markers is absolutely specific for smooth muscle , and positivity for two of these markers is more supportive of leiomyosarcoma . jensen et al . identified several poor prognostic factors , namely tumor size 5 cm , acral location , deep localization with fascia involvement and high histological grade . cutaneous leiomyosarcomas have fewer incidences of local recurrence ( 30 - 50% ) and negligible potential for distant metastases ( 010% ) . in contrast , subcutaneous leiomyosarcomas may develop local recurrence in about 4060% and distant metastasis in 2060% of patients . a wide local excision with a 35 cm margin is usually an effective treatment for pcl . , pcl is a rare entity that must be borne in mind when encountered with a malignant spindle cell neoplasm of the skin and usually requires a panel of immunohistochemical markers to distinguish it from other cutaneous malignancies with spindle cell morphology .

primary cutaneous leiomyosarcoma ( pcl ) is an exceedingly uncommon malignant superficial soft tissue sarcoma with a predilection for middle - aged to elderly male . morphologic differential diagnosis includes a host of other malignant spindle cell neoplasms , thereby necessitating the use of a panel of immunohistochemical markers to arrive at a definitive diagnosis . we report a case of pcl arising in the right leg of a 70-year - old male .

situs inversus totalis ( sit ) is a rare congenital anomaly with an incidence of 1 in 10,00050,000 persons . sit refers to complete right - left transformation or a mirror image of the normal arrangement of body organs . the anatomic arrangement in a patient with sit is complete transposition of all viscera in the thoracic and abdominal cavities . although this condition does not affect normal health or longevity , its recognition is very important for treating many diseases , particularly those requiring surgical intervention . herein , we report a patient with gastric cancer and sit who was treated with radical subtotal gastrectomy . a 50-year - old man was admitted to our hospital for treatment of early gastric cancer ( egc ) , which was diagnosed during a screening esophagogastroduodenoscopy ( egd ) at a local clinic . his height was 171 cm , weight was 64.8 kg , and his body mass index was 22.2 . he had no anemia ( red blood cell count , 4.25 10/l ; hemoglobin , 13.4 g / dl ; hematocrit , 43.6% ) . the serum carcinoembryonic antigen concentration was not elevated ( 3.16 ng / ml ; reference range , 04.3 ) . a chest radiograph showed dextrocardia and a right subphrenic gas pattern in the stomach ( fig . 2 ) . abdominopelvic computed tomography ( ct ) showed complete transposition of the abdominal viscera , confirming sit ( fig . 3 ) . an f - fluoro2-deoxyglucose - d - glucose - positron emission tomography ( fdg - pet ) scan revealed no abnormal uptake and also noted a complete mirror image of the intrathoracic and abdominal viscera ( fig . 4 ) . we decided to perform a laparotomy and found complete transposition of the viscera ; the stomach and spleen were located on the right side of the abdomen , and the gall bladder , liver , cecum , and appendix were located on the left side . the macroscopic biopsy report revealed a 2.0 1-cm egc type iic located at the gastric angle with 2 and 6.8 cm proximal and distal safety margins , respectively . pathological examination showed tubular adenocarcinoma , moderately differentiated with depth of invasion into the muscularis mucosa . we followed the patient for 78 months in the outpatient department without recurrence of cancer . sit is transmitted as an autosomal recessive trait , but the exact genetic cause remains unknown . suggested that immobility of nodal cilia inhibits the flow of extraembryonic fluid during embryogenesis , leading to the development of sit . sit has various manifestations ; dextrocardia with complete reversal of the heart chambers can be seen . the stomach and spleen are located in the right side and the liver and gallbladder are located in the left side of the midline . cardiovascular abnormalities ( septal defects , pulmonary arterial stenosis , tetralogy of fallot , or transposition of great arteries ) and problems with the alimentary tract ( atresia or stenosis of the duodenum ) can also occur . no significant cardiac or respiratory abnormalities were detected in our patient at the preoperative screening . about 60% of patients with sit have other congenital anomalies of the gastrointestinal tract , including rotational anomalies , biliary atresia , splenic agenesis , small bowel atresia , duplication , and colon aganglionosis . the initial diagnosis and surgical intervention for diseases , such as cholelithiasis , reversed side liver dullness , an apical beat in the right fifth intercostal space , a chest radiograph revealing dextrocardia , and abdominal x - ray film demonstrating stomach gas on the right side can be diagnostic clues of sit . an fdg - pet scan was obtained as a whole body image in a single picture for the stomach cancer stage workup . many different cancers have been reported in patients with sit , including pancreatic , hepatocellular , colorectal , and gastric cancers . fabricus reported the first case of mirror image transposition in a man in 1600 [ 8 , 9 ] . allen first reported a gastrectomy on a 30-year - old patient with gastric cancer and sit who died 3 weeks after surgery in 1936 . the symptoms of kartagener 's syndrome , including bronchiectasis , chronic sinusitis , and male infertility is a disease that is associated with sit . surgery in a patient with sit and gastric cancer could be difficult due to the anatomic anomalies , including perigastric major vessels . although the incidence of intra - abdominal malignancies in a patient with sit is very rare , the surgeon must anticipate the complexity of the surgical procedure in patients with sit and cancer . advanced surgical skill is required to perform a precise lymphadenectomy in a patient with sit by visualizing the exact mirror image of the anatomy during the operation . visualizing the mirror image of the anatomy therefore , preoperative recognition of the anatomic variations might be needed when operating on a patient with sit . it might be helpful to perform a three - dimensional ct angiography reconstruction prior to the operation . in conclusion , surgery in a patient with gastric cancer and sit can be safely performed by paying attention to the inverted anatomic structures during the operation . written informed consent was obtained from the patient for publication of this case report and any accompanying images .

situs inversus totalis ( sit ) is a rare congenital anomaly that refers to a completely reversed location of the abdominal and thoracic organs . we report the case of 50-year - old man with gastric cancer and sit who was diagnosed during a screening esophagogastroduodenoscopy . a chest x - ray , abdominopelvic computed tomography , and 18f - fluoro2-deoxyglucose - d - glucose - positron emission tomography scans revealed sit . we performed a radical subtotal gastrectomy with d2 lymph node dissection . advanced surgical skill is required to perform a precise lymphadenectomy in a patient with sit by visualizing the exact mirror image of the anatomy during the operation . the patient had an uneventful intra- and postoperative course and was followed up at the outpatient department without any evidence of recurrence . in conclusion , surgery in a patient with gastric cancer and sit can be safely performed by paying attention to the inverted anatomic structures during the operation .

laceration of the lumbar artery during renal biopsy has been reported only in four instances . a 58-year - old lady presented with swelling of face that progressed to involve abdomen and feet , along with oliguria , cola colored urine and breathlessness on minimal exertion . on examination , she had puffiness of eyelids and swelling of feet . cardiovascular examination revealed left ventricular third sound and respiratory system examination revealed bilateral crackles in infrascapular areas . investigations showed hemoglobin of 12.1 g / dl , platelet count 2.57 lakhs / cumm , random blood glucose 82 mg / dl , blood urea 88 mg / dl , serum creatinine 5.63 mg / dl , total serum proteins 7.8 g / dl , and albumin 4.0 g / dl . urinalysis revealed albumin : + , red blood cells : 1012/hpf , and red blood cell casts . the 24 h urine protein was 1.1 g , total cholesterol 258 mg / dl , antistreptolysin o > 400 u / l , complement 3 435 mg / l ( reference range : 10321495 mg / l ) , and complement 4 150 mg / l ( reference range : 167385 mg / l ) . patient was subjected to a renal biopsy with a 16 gauge automated spring - loaded biopsy gun with length for sample notch being 1.6 cm and a possible penetration depth of 22 mm ( bard biopty systems , usa ) . after second pass , the patient complained of pain from the biopsy site to the left flank and anterior abdomen . the radiologist noticed the appearance of an echogenic band around left kidney and in the muscular planes . a computerized tomography scan of abdomen revealed left perinephric collection of 1200 ml and heterogenous collection in left lumbar region [ supplementary figure 1 ] . angiogram revealed a focal active contrast extravasation from arcuate or interlobular artery in lower pole of left kidney and from lumbar artery at third lumbar vertebra [ supplementary figure 2 ] . her blood pressure fell to 80/60 mmhg from the pre - biopsy reading of 140/100 mmhg , and the hemoglobin dropped to 8.1 g / dl . right femoral artery was cannulated with a cordis 6.0f femoral sheath , into which a judkin 's diagnostic catheter 5.0f was passed . into this catheter , floppy percutaneous transluminal coronary angioplasty guidewire , 0.014 inch in diameter and 300 cm in length the bleeding site was approximately 10.5 cm away from the vertebral column [ figure 1 and supplementary figure 3 ] . over this guidewire , a micro catheter , 2.25f and 130 cm in length ( asahiintecc ) gelfoam was injected so as to occlude the bleeding site [ supplementary figure 5 ] . computed tomography scan abdomen : bleeding from lumbar artery ct showing angiogram : bleeding from lumbar artery angiogram : bleeding from lumbar artery renal angiogram : no bleeding in the kidney 2.25f micro catheter introduced into lumbar artery lumbar artery embolized with gelfoam usually , four on each side [ figure 2 ] , they arise posterolaterally from the aorta opposite the lumbar vertebrae . a fifth , smaller pair occasionally from the median sacral artery but lumbar branches of the iliolumbar arteries usually takes their place . the lumbar arteries posterolaterally on the four upper lumbar vertebral bodies , behind the sympathetic trunks , to intervals between the lumbar transverse processes and continue into the abdominal wall . the right arteries pass posterior to the inferior vena cava ; the upper two right and first left are also posterior to the corresponding crus . arteries of both sides pass under tendinous arches ( which span the lateral cavities of the vertebral bodies ) for attachment of psoas major , proceeding posterior to the muscle and the lumbar plexus . they then cross the quadratus lumborum , the upper three posterior , the last usually anterior to it . at its lateral border , they pierce the posterior aponeurosis of the transversus abdominis , advancing between it and the internal oblique . they anastomose with one another and the lower posterior intercostal , subcostal , iliolumbar , deep circumflex iliac and inferior epigastric arteries . as the upper three pairs of lumbar arteries pass behind the quadratus lumborum , they are at risk injury from the renal biopsy needle . anatomy of lumbar arteries ( 14 ) in the two previous reports [ table 1 ] , the presence of bleeding sites and arteriovenous fistula in kidney has misled and the laceration of the lumbar artery was missed . there was a delay of 20 h and 48 h in these two patients respectively before the laceration of the lumbar artery was recognized . previous reports of hemorrhage from lumbar artery following percutaneous renal biopsy embolization of lumbar artery is fraught with the risk of infarction of the spinal cord . the artery of adamkiewicz ( or great anterior radiculomedullary artery or arteria radicularis anterior magna ) is the dominant thoracolumbar segmental artery that supplies the spinal cord . this artery has a variable origin ; but most commonly arises on the left ( ~80% ) at the level of 912 intercostal artery ( ~70% ) . it arises from the radiculomedullary branch of the posterior branch of the intercostal or lumbar artery , which arises from the thoracic or abdominal aorta respectively . the variations of its origin include from lumbar arteries at the level of l1 or l2 ( 10% ) , intercostal arteries at the level of t5 to t8 ( 15% ) and arises on the right ( 20% ) . the injection of gelfoam and placement of coils should be done only after considering all these variations in the origin of this artery . the use of liquid embolization materials is also contraindicated due to higher risk of spinal cord injury . the lumbar artery embolization has been reported to have caused infarction of paraspinal muscles in two patients . bleeding from lumbar artery is a rare complication but can be successfully managed with prompt recognition and intervention .

we present a 58-year - old lady who underwent ultrasound - guided renal biopsy for suspected acute glomerulonephritis . within minutes , the radiologist noticed an echogenic band around left kidney and in the muscular planes . computerized tomography revealed focal active contrast extravasation from arcuate or interlobular artery in lower pole of left kidney and lumbar artery at third lumbar vertebra . the bleeding vessel was occluded with gelfoam .

he presented with a 10-year history of voiding difficulty . before this presentation , a digital rectal examination revealed bilateral enlargement of the prostatic lobes . under a clinical diagnosis of bph , he was administered tamsulosin 0.2 mg once with the insertion of a urethral foley catheter . transrectal ultrasonography revealed that the volume of the prostate was approximately 136 ml ( fig . his prostate - specific antigen level was 2.12 ng / ml . two weeks later , a pressure flow study ( pfs ) was performed to evaluate his voiding symptoms and showed an obstructive pattern and normal detrusor function according to the international continence society and schffer nomogram ( fig . 1c ) . his maximal urine flow rate ( qmax ) was 9 ml / sec on the pfs . simple x - ray and computed tomography ( ct ) scan suggested the presence of several bladder stones . we decided to perform concomitant lessv and step under a diagnosis of bladder stones and bph . informed consent was obtained after a complete description of the less with the use of a homemade single - port device . with the patient under general anesthesia , a midline incision of approximately 3 cm was made at the level of three fingerbreadths above the symphysis pubis , followed by a dissection to reach the bladder . the anterior wall of the bladder was then incised at approximately 2 cm to minimize the bladder wall and port - device . pneumovesicum was formed while maintaining 12 cm h2o pressure . using a 10-mm rigid laparoscope ( conmed linvatec , largo , fl , usa ) , the prostatic adenoma , urethral foley catheter , and bilateral ureteral orifices urinary drainage was halted to prevent gas leakage , and the urethral catheter tip was then moved to the bladder neck . using rigid instruments , the bladder stones were grasped and extracted through a hole in another finger of the glove . after removal of the bladder stones , a dissection was made starting from the right prostatovesical junction using a rigid laparoscopic dissector and an enseal trio tissue sealing device ( ethicon endo - surgery inc . , air ventilation was continuously maintained to minimize vesical neuromuscular trauma due to visual field securing and pneumovesicum . after sufficient dissection of the huge adenoma , the port - device was removed . using ellis forceps , the huge adenoma was pulled outward and the index finger was inserted into the dissection plane as described in a previous report . the suprapubic catheter was used to make up for the obstruction of the urethral catheter . the bladder was sutured with 3 layers , and the skin was approximated by using a skin stapler ( 3 m precise skin stapler , 3 m co. , preston , mn , usa ) . the suprapubic and urethral catheter were removed on the 7th and 8th postoperative day , respectively . first reported step for large bph using an r - port ( advanced surgical concepts , wicklow , ireland ) between 2008 and 2009 . the two groups of desai and sotelo jointly reported 34 cases of step including a case of r - less in 2010 . in their study , the authors conducted the enucleation by using electrocautery and ultrasonic shears under laparoscopic video assistance . interestingly , oh and park reported 32 cases of f - step in the treatment of a huge prostate following desai et al . in their study , they reported a new enucleation method using fingers and excellent outcomes . after the prostatic capsule was dissected with the use of articulating instruments , traction suturing was performed on the prostatic adenoma . the adenoma was enucleated by using the index finger after removing the homemade single - port device . in our case , we used the same method to remove the prostatic adenoma , because the prostatic adenoma was too large to remove with the use of laparoscopic instruments . in fact , use of less for the primary treatment of urological cancers might be limited because of issues such as the extraction method of the specimen and maintaining oncologic principles . the ideal indications for less may be pediatric urologic diseases and benign , non - life - threatening diseases . recently , kaouk et al . reported a worldwide multi - institutional analysis of 1,076 cases of less in urology . in their study , malignant disease including renal tumor , adrenal tumor , prostate cancer , and bladder cancer made up approximately 46.6% of the sample . more than half of the cases were benign disease , for which less was performed . in benign urologic disease , there were 42 cases ( 3.9% ) of less for bph including step . to the best of our knowledge , concomitant lessv and f - step have not been reported in previous studies . step was estimated to be a very difficult procedure according to autorino et al . . however , it may be more advantageous for surgeons with experience in less or conventional laparoscopy , but may show a steep learning curve as shown for holmium laser enucleation of the prostate ( holep ) or an even steeper learning curve in surgeons without experience in less or conventional laparoscopy . although step has better cosmesis than open surgery , it may require a suprapubic catheter and may have an external wound compared to turp or holep . however , step or f - step might be a viable treatment option in patients with huge prostate of more than 80 ml . in our case , the concomitant lessv and f - step has some advantages , as follows : urethra and bladder neck injuries may occur due to the long - time use of a rigid resectoscope , but they can be prevented in concomitant lessv and f - step . meanwhile , no suturing with laparoscopic instruments is required in f - step as compared with pyelolithotomy or ureterolithotomy . for a large bladder stone in addition , the single incision in the concomitant lessv and f - step without the need for a position change is an important advantage . our case suggests that concomitant lessv and f - step can be used as an alternative technique in the treatment of huge bph with huge prostate and bladder stones , for which open surgery is usually performed .

transurethral resection of the prostate is the most common surgery for benign prostatic hyperplasia . however , it does n't work best for men with very large prostate and bladder stones . herein we report our initial experience with concomitant laparoendoscopic single - site surgery and finger - assisted single - port transvesical enucleation of the prostate for the treatment of the condition .

it arises from the superior margin of the sphenopalatine foramen and accounts for 0.05% of all head and neck neoplasms . the typical histopathologic appearance of jna is numerous wide , irregular vessels with a single layer of endothelial cells embedded in fibrous stroma . the abundant vascular component is responsible for the excessive bleeding during surgery or following biopsies.1 extranasopharyngeal angiofibromas ( enas ) have been reported sporadically in the literature . they most commonly originate from the maxillary sinus , and women are affected . compared with nasopharyngeal fibromas , the lesion is diagnosed earlier , is less vascularized , and occurs in older patients.2 similar to jnas , the treatment of choice for enas is surgery . the surgical approach is tailored to the location and size of tumor.1 in our case , the tumor was small enough for total excision with an endonasal endoscopic approach . this is the first case report in the english literature of angiofibroma originating in the inferior turbinate in a young woman . enas are rarely seen , and the inferior turbinate is an extremely rare location for them . there are reports in the english literature of inferior turbinate angiofibroma in male patients.3 4 5 6 in female patients , there is only one case report in the english literature of inferior turbinate angiofibroma in a 52-year - old woman.7 recently , a report was published of a 9-year - old girl with angiofibroma obstructing the nasal cavity and originating from the inferior turbinate.8 differential diagnosis includes fibrosed antrochoanal and ethmoidal polyp and other fibrovascular tumors , such as capillary hemangioma , hemangiopericytoma , and solitary fibrous tumor.3 a 26-year - old woman was referred to our clinic with intermittent epistaxis from the right nasal passage for the previous 2 months . maxillofacial magnetic resonance imaging showed a lobular , contoured mass originating from the right inferior turbinate and hanging to the right of the nasal cavity , with dense contrast enhancement denoting hypervascularity ( figs . 1 , 2 , and 3 ) . we decided to embolize the vascular feeding of the mass to make surgery more feasible . the patient consulted with the invasive vascular radiology department and decided on the embolization process under intravenous sedation . vascular feeding of the mass was seen from the right internal maxillary artery , and this branch was embolized . when looking at the posterior wall of the sinus and pterygopalatine fossa , no masslike structure was evident . the approximately 3-cm - diameter mass originated from the posterior edge of the right inferior turbinate ( fig . a partial turbinectomy was performed , and the posterior edge of the remaining turbinate was cauterized . a mean age of 20 to 30 years in enas was found in the literature.1 2 9 our case was also in this group . enas mostly originate from the maxillary sinus.2 there are also some reports of tumors located in the ethmoid sinus , nasal cavity , nasal septum , larynx , sphenoid sinus , cheek , conjunctiva , oropharynx , retromolar area , and middle turbinate.3 10 11 12 13 14 15 16 the clinical presentation of enas depends on tumor localization . in our case , but because of the limited space in the nasal cavity , diagnosis was made at an early tumor stage . the tumor 's location indicated that the origin may be from ectopic tissues located further from its usual place.17 there is also a reported case of an angiofibroma arising from the inferior turbinate after co2 laser turbinoplasty.18 in our case , there was no history of turbinate surgery . selective angiography is a useful diagnostic method to demonstrate tumor vascular composition and to confirm the diagnosis . endoscopic and radiologic examination is important , but definitive diagnosis is made by histopathologic analysis . we should consider these neoplasms possibly in female patients , at every age , with extranasopharyngeal localization , and we should not perform biopsy because of massive bleeding .

introduction juvenile nasopharyngeal angiofibroma is a rare benign neoplasm in the nasopharynx . the tumor tends to be locally aggressive and is typically seen in adolescent boys . extranasopharyngeal angiofibromas have been reported sporadically in the literature . they most commonly originate from the maxillary sinus . objectives a 26-year - old woman was referred to our clinic with intermittent epistaxis from the right nasal passage for the previous 2 months . maxillofacial magnetic resonance imaging showed a lobular , contoured mass originating from the right inferior turbinate and hanging in the right nasal cavity , with dense contrast enhancement denoting hypervascularity . resumed report vascular feeding of the mass was seen from the right internal maxillary artery with angiography , and this branch was embolized . on the following day , the patient underwent transnasal endoscopic excision of the mass . an approximately 3-cm - diameter mass was excised by partial turbinectomy , and the posterior edge of the remaining turbinate was cauterized . conclusion extranasopharyngeal angiofibromas are rarely seen , and the inferior turbinate is an extremely rare location for them . this young woman is the first case reported in the english literature of angiofibroma originating from the inferior turbinate . we should consider these neoplasms can be found in female , nonadolescent patients with extranasopharyngeal localization , and we should not perform biopsy because of its massive bleeding .

hepatocellular carcinoma ( hcc ) is the sixth most common cancer worldwide , and it is the third most common cause of death from cancer . recent studies demonstrated clinical features of multiple primary malignancies involving hcc [ 2 , 3 ] . however , hcc patients bearing other malignancies with distal metastasis are not only uncommon , but also difficult to diagnose . therefore , the therapeutic approach for and the clinical course of these patients remains unclear . herein , we describe a case of metachronous multiple primary malignancies consisting of hcc and gastric cancer with intracranial metastasis . a 76-year - old man visited our hospital because of visual disturbance , weakness of the left upper and lower limbs , and gait disturbance . the patient had been treated for cirrhosis caused by chronic hepatitis c virus ( hcv ) infection . because hcc was detected , approximately 60 mm in diameter , in the right lobe , he had undergone transarterial chemoembolization 3 and 10 years earlier . when radiofrequency ablation was performed for the recurrent hcc 2 years ago , the patient had neither a history of smoking nor an elevation of tumor markers for primary lung cancer ( such as squamous cell carcinoma antigen and neuron - specific enolase ) . since the resected gastric cancer histologically lacked an involvement of lymph nodes , we considered the possibility that lung tumors were of hcc origin . subsequently , sorafenib was administered . because of adverse events such as a hand - foot skin reaction , the dose of sorafenib was reduced from 800 to 200 mg / day . on admission , serum levels of hepatobiliary enzymes and renal function markers such as blood urea nitrogen and creatinine were normal . although blood count tests showed slight anemia ( hemoglobin : 11.5 g / dl ) and thrombocytopenia ( 8.5 10/l ) , his white blood cell count was normal ( 4,000/l ) . however , the levels of alpha - fetoprotein ( afp ) , des - gamma - carboxy prothrombin ( dcp ) , and carbohydrate antigen 199 ( ca199 ) were within the normal range ( 5.6 ng / ml , 21 u / ml , and 10.2 u / ml , respectively ) . although the patient 's consciousness level was normal [ glasgow coma scale was a full score ( e4v5m6 ) ] , he exhibited left - sided hemifield visual disturbance and left - sided hemiparesis . enhanced mri revealed an intra - axial tumor in the right occipital cerebral lobe ( fig . 2 ) . taking into consideration the markedly elevated level of carcinoembryonic antigen , but not afp or dcp , we made a preoperative diagnosis of intracranial metastasis of gastric cancer , and a craniotomy of the tumor was performed . macroscopic examination of the resected specimen revealed that the tumor was yellowish - white , solid , and accompanied by necrosis ( fig . pathological examination revealed that the tumor was consistent with metastatic adenocarcinoma of gastric cancer origin ( fig . the patient 's visual disturbance , hemiparesis , and gait disturbance markedly recovered to normal after surgery . follow - up mri conducted 1 week after the craniotomy revealed the successful removal of the tumor . because a small lesion was detected in the left precentral gyrus , the patient received additional gamma - knife treatment ( 20 gy ) . sorafenib administration was discontinued and s-1 ( tegafur / gimeracil / oteracil potassium , 50 mg / day ) was administered postoperatively . he was alive for 5 months after the gamma - knife treatment , without any recurrence . advances in diagnostic modalities and therapeutic approaches have improved the prognosis of patients with multiple primary malignancies . in the diagnosis of multiple primary malignancies , according to the criteria , each of the tumors must be distinct and present a definite picture of malignancy . it has been reported that the prevalence of multiple primary malignancies varies from 0.7 to 11.7% [ 5 , 6 ] . in western countries , prostate , colon , and in contrast , gastric cancer predominates in asian countries [ 8 , 9 ] . in our case , although multiple lung metastases were observed concurrently , it was difficult to determine whether the tumor was derived from hcc or gastric cancer . we considered the possibility of hcc origin because of an increase in the serum level of afp and pivka - ii despite the curative treatment of recurrent hcc and histological findings of resected gastric cancer not involving the lymph nodes . the frequency of intracranial metastasis in patients with gastric cancer ranges from 0.16 to 2.0% [ 10 , 11 ] . it has been documented that aggressive treatments such as resection and/or whole brain radiation therapy could improve the quality of life and prolong the survival [ 12 , 13 ] . in our case , although another intracranial lesion was detected after craniotomy , the tumor was successfully treated with a gamma - knife . given that the multiple lung tumors gradually enlarged during the administration of sorafenib , it was undeniable that the lung tumors were derived from gastric cancer . together , these findings indicate the possibility that sufficiently aggressive treatment for intracranial metastasis of gastric cancer enables further anti - cancer treatment and improves the prognosis , even of patients with multiple primary malignancies . when original tumors are radically treated in patients with multiple primary malignancies , further treatment including craniotomy and gamma - knife for the metastatic intracranial tumors might contribute to prolonged survival . considering that intracranial metastasis of gastric cancers is frequently accompanied by bone , liver , and lung metastases [ 10 , 14 ] , it is of importance to determine whether or not aggressive treatment , including surgical resection , improves both the performance status and the prognosis of the patients .

a 76-year - old man was referred to our hospital with visual disturbance , weakness of the left upper and lower limbs , and gait disturbance . he had previously received transarterial chemoembolization for hepatocellular carcinoma ( hcc ) 3 and 10 years ago . when he had received radiofrequency ablation for hcc recurrence 2 years ago , total gastrectomy was also performed for his gastric cancer . subsequently , sorafenib had been administrated for concomitant lung metastatic tumors . on admission , mri revealed an intra - axial tumor with perifocal edema . the level of carcinoembryonic antigen , but not alpha - fetoprotein , markedly increased . the tumor was successfully removed by craniotomy and pathological examination revealed that it was composed of adenocarcinoma , which was consistent with the primary gastric cancer . after surgery , his neurological disturbances rapidly resolved . additional gamma - knife treatment was also performed for another small brain metastasis detected after craniotomy . subsequently , sorafenib administration was discontinued and s-1 was administered postoperatively . successful treatment of intracranial metastasis of gastric cancer is important and meaningful , even in patients with multiple primary malignancies .

an incisional biopsy from mandibular region of a 45-year - old female patient was submitted to our department of oral pathology and microbiology with the history of pain and swelling in the right lower back teeth region for past 2 months . the clinical details given to us were , swelling in the right lower cheek region extraorally . intraorally the swelling was extending from right canine to retromolar area and 45 and 46 were mobile . the hematoxylin and eosin stained histopathological stain showed numerous follicular structures within the connective tissue . the follicles were lined by tall columnar ameloblast like cells with reverse polarity and surrounding stellate reticulum like cells . both peripheral tall columnar cells and central stellate reticulum like cells showed dense granularity . hematoxylin and eosin stained section showing ameloblastic follicles with granular cells ( h and e , 10 ) following the histopathological diagnosis hemimandibulectomy was done . the hemimandibulectomy specimen showed thinned out buccal cortical plate and perforation of the lingual article plate at multiple locations . anterior and posterior surgical margins were sectioned , and histopathological examination was done after decalcification . all the sections from the excised specimen showed granular cell ameloblastoma while the anterior and posterior surgical margin did not show any residual tumor . patient is currently on regular follow - up for the past 22 months and no sign of recurrence . ameloblastoma sometimes exhibits granular transformation of cytoplasm occurring in central stellate reticulum like cells , and this change often extends to peripheral columnar or cuboidal cells . hematoxylin and eosin staining and periodic acid schiff staining showed granular neoplastic cells residing within the tumor follicles . light microscopic study of granular cell ameloblastomas shows numerous neoplastic epithelial islands in a scant , mature fibrous stroma . the most striking features of this tumor were that the center of the island had large eosinophilic granular cells , surrounded by tall columnar cells resembling the inner enamel epithelium of the enamel organ . their cytoplasmic granules ranged from fine to coarse and in some cells pink , homogenous rounded masses which resembled inclusion bodies larger than coarse granules . the peripheral cells lining the islands were tall columnar or cuboidal with nuclei displaced opposite to the base of the cell and many peripheral cells were granular . the granular cells are a transitional or matured phase in the life cycle of ameloblasts , starting with normal stellate reticulum like cells leading to the production of granules and finally resulting in degeneration and the formation of cystic areas . ultrastructurally , in the granular cell ameloblastoma the peripheral cells of the tumor islands were separated from surrounding stroma by continuous basement membrane . the most striking cytoplasmic feature is the presence of numerous granules ( pleomorphic osmiophilic granules ) . the granules are round or oval , composed of homogenous amorphous osmiophilic material of different density . the granules were known to be lysosomes , which might play a role of autophagy and of remodeling the cytoplasm , as opposed to the aging or degenerating function . the cytoplasmic granularity in granular cell ameloblastomas might be caused by increased apoptotic cell death of neoplastic cells and associated phagocytosis by neighboring neoplastic cells . lysosomal aggregation within cytoplasm is caused by dysfunction of either a lysosomal enzyme or lysosome associated protein involved in activation or targeting of the enzyme or lysosomal biogenesis . the granular cell ameloblastoma is a locally aggressive tumor with a high frequency of recurrence and potential to metastasize . it shows a slightly higher rate of recurrence of about 33.3% when compared with other common variants . however , very high recurrence rates were reported by hartman et al . ( 73% ) . the presence of tumor cells within the bone beyond the clinical and radiological margins are thought to be responsible for the recurrence when the tumors are treated with enucleation or curettage . the malignant transformation is rare , but the cases with metastasizing granular cell ameloblastoma lymph nodes , lungs and cervical vertebrae have been reported . however , further studies with long - term follow - up are necessary for the better understanding of the tumor 's diagnosis , prognosis and treatment .

granular cell ameloblastoma is a slow growing odontogenic ectodermal tumor . the tumor shows typical ameloblastoma with the cells showing eosinophilic granularity . this variant of ameloblastoma is aggressive with high recurrence rates . we report a case of aggressive ameloblastoma of granular cell variant

voluntary euthanasia ( ve ) is the act of requesting deadly medical practices in order to terminate one s life ( 1 ) . the process of decision making ( like that for ve ) is composed of two interwoven brain based components ( 2 ) . one is the conscious power of analyzing the condition and utilizing algorithmic like strategies , which is known as cognition ( 2 , 3 ) . this part is important since it affects the person s ability of seeking in the condition ( 4 ) , estimating the parameters of a situation ( 5 ) and finally deciding ( 2 ) . chronic pain patients ( cpps ) develop disappointing emotional backgrounds , which affect their mental power of decision - making ( 6 ) . this article argues about the mental incompetence relating decision - making that accompanies chronic pain in such patients . this can be considered as equal to the historically known conditions of torturing a person and wanting him / her to accept or confess something in return for relieving pain ( 7 ) . in case of ve , the agent is the internal mental burden of a disease and the thing wanted in return for reliving the pain is dying acceptance . therefore , in this condition the stimulator of accepting death is the emotion of escaping from pain in return for anything . this is the exaggerated form of the normal emotion of fearing and escaping from painful situations ( 8) . in fact , a cpp fears from experiencing excess pain . according to this outlook and having in the mind that emotions - in the way it is described in the literature ( 9 ) - are the most important determinants of the alternative to be chosen ( 10 ) , here it is proposed that cpps are emotionally and hence mentally incompetent for making critical decisions . studies using mri technology have shown brain deficits in cpps ( 1114 ) . more specifically in the state of chronic pain it is stated that the orbitofrontal part of the brain cortex is preferentially involved . this part functions as a link between multiple brain regions with distinct roles in the emotional assessment . these regions include lateral prefrontal areas which handle memory , effector components of the brainstem which are responsible for producing emotional responses and those parts that are considered as the brain substrates of feelings like insula and amygdale ( 6 ) . according to these findings a cpp does not have a biologically healthy brain for providing the accuracy of the act of choice - making especially when he / she wants to do so for emotion - laden alternatives like that of whether to escape or cope with the pain . this can be considered as equal to the historically known conditions of torturing a person and wanting him / her to accept or confess something in return for relieving pain ( 7 ) . in case of ve , the agent is the internal mental burden of a disease and the thing wanted in return for reliving the pain is dying acceptance . therefore , in this condition the stimulator of accepting death is the emotion of escaping from pain in return for anything . this is the exaggerated form of the normal emotion of fearing and escaping from painful situations ( 8) . in fact , a cpp fears from experiencing excess pain . according to this outlook and having in the mind that emotions - in the way it is described in the literature ( 9 ) - are the most important determinants of the alternative to be chosen ( 10 ) , here it is proposed that cpps are emotionally and hence mentally incompetent for making critical decisions . studies using mri technology have shown brain deficits in cpps ( 1114 ) . more specifically in the state of chronic pain it is stated that the orbitofrontal part of the brain cortex is preferentially involved . this part functions as a link between multiple brain regions with distinct roles in the emotional assessment . these regions include lateral prefrontal areas which handle memory , effector components of the brainstem which are responsible for producing emotional responses and those parts that are considered as the brain substrates of feelings like insula and amygdale ( 6 ) . according to these findings a cpp does not have a biologically healthy brain for providing the accuracy of the act of choice - making especially when he / she wants to do so for emotion - laden alternatives like that of whether to escape or cope with the pain . the central concept of this article is that a person who is under the mental pressures is not capable of making critical decisions . the same concept is already accepted for the other categories of people who can not think well such as drunken individuals ( 15 ) . the fact that mentally under pressure patients can not think well is also indirectly accepted by all societies that refuse to obey a mentally ill patient s will in the light of labeling the patient unqualified for making a will according to the judgment of court . with the same bases , this article s concept can be accepted by the court and become a law in such societies . therefore , the patient s will of terminating his / her life must not be accepted like that of a drunken person . deciding to undergo euthanasia is the most critical decision of a person ( not only a patient ) since this decision leads to the end of making other decisions . in this respect , deciding to die is different from deciding to buy a car because the latter is reversible or can be compensated in the future and does not violate the right of making other decisions , something that the former intrudes in . therefore the ability of making the decision of undergoing euthanasia must be considered as something different from just a right . in this mental incompetence is the common quality in both conditions of chronic pain and will rejection in the court . therefore the accepted rules for both of these two conditions must be the same . this means that any nation that rejects the will of a mentally incompetent individual must do the same about the request of a cpp for ve .

emotional status and its brain bases change in a chronic pain patient and this change affects his / her decision making ability . moreover , it is accepted that a mentally disturbed individual is not competent to make critical decisions . according to these bases , this article demonstrates that such patients are not entitled to request voluntary euthanasia .

benign acute childhood myositis ( bacm ) is a self - limiting process characterized by sudden onset of muscle pain , more often calf pain , manifested by walking difficulty . since many clinicians are not familiar with bacm , it is often misdiagnosed and interpreted as a more severe and complex disease . we report the case of a patient suffering from bacm in absence of any previous documented viral infections . we underline how medical history and clinical examination are sufficient for a correct diagnosis of bacm , as well as to avoid unnecessary tests . based on the previous reports , we updated etiology and clinical data of bacm , and we propose differential diagnosis of muscle pain in children . a 7-year - old boy with acute calf pain and progressive walking difficulty was referred to our pediatric center to rule out muscle degenerative diseases . two days earlier the child had suffered from fever , cough , malaise and rhinorrhea , which promptly resolved . the onset of calf pain was early in the morning , and it was so sharp that he had to walk on his heels mimicking a spastic gait . the child had undergone all compulsory vaccinations , and his previous clinical history was not suggestive of any remarkable diseases . no travels , trauma , vigorous exercise or similar episodes of limb pain were reported before onset of symptoms . on admission , reflexes and sensitivity of the upper and lower limbs were also normal . at rest , the patient kept his feet in slight plantar flexion . gastrocnemius - soleus muscles on both sides were soft on palpation , and there were no inflammatory changes . passive dorsiflexion of the ankles caused sharp pain as well as passive stretching and gentle palpation of his calves . despite normal neurological examination , the patient was hospitalized in order to allow medical observation and exclude severe diseases . on admission laboratory tests showed a significant increase in blood levels of creatinine phosphokinase ( 2161 u / l , normal range < 227 u / l ) , alanine transaminase ( 69 u / l , normal range : 0 - 45 ) and aspartate transaminase ( 92 u / l , normal range : 17 - 59 ) . mg / l ) and white blood cell count ( 7400/l ) were within normal limits . complete blood count , electrolytes , blood urea nitrogen , creatinine , prothrombin time , partial thromboplastin time and other routine blood parameters were also within normal limits . furthermore , serological tests ruled out recent viral infections by adenovirus , respiratory syncytial virus , cytomegalovirus , herpes virus and epstein barr virus , as well as serological tests for mycoplasma pneumonia . since the patient refused to eat and drink , he received intravenous hydration for 24 h. during the 2 day of hospitalization pain spontaneously resolved and a normal gait was regained . considering that daily urine output was normal , the boy was discharged , and no long - term follow - up was required . benign acute childhood myositis is a self - limiting process which frequently affects the gastrocnemius and soleus muscles . in a review of 2004 other agents such as enterovirus , mycoplasma pneumonia and dengue virus have been involved in the process . furthermore , five cases of bacm following h1n1 influenza a virus infection and human parainfluenza virus type 1 infection have been reported . in most cases , the disease is self - limiting and when the myositis develops the patient is already in the early convalescent phase of the viral illness . nevertheless , it is reported that 10/316 children ( 3% ) developed severe rhabdomyolysis , and temporary dialysis was necessary in six cases . during winter 20072008 , influenza b virus was responsible for a large number of cases of bacm in germany , especially among male children aged from 6 to 9 years . median time between the onset of fever and the beginning of bacm symptoms was 3 days . despite the clinical course of bacm being well described , mall et al . furthermore , the exact current incidence , prevalence and pathogenesis of bacm are not known . this could simply be explained by the increased tropism of viruses for immature muscle cells . furthermore , each virus could act as a trigger in genetically predisposed children and in few patients with undiagnosed metabolic diseases . nevertheless , the onset may be mistaken for very severe neurological illness such as guillain barr syndrome or chronic autoimmune diseases . as a consequence unnecessary invasive tests , such as radiography , echocardiography , daily medical examination and urine dipstick ( with measurement of myoglobin ) are sufficient to promptly detect complications and rule out more severe illnesses and creatine kinase ( ck ) should be measured only at diagnosis in order to early exclude degenerative diseases . viral tests for bacm - related viruses should not be routinely performed because of the time interval between infection and seroconversion . moreover , not always etiological agents can be found , and antiviral drugs are not usually recommended . nevertheless , influenza test may be useful since influenza viruses can affect the severity of bacm . however , parents should be encouraged to monitor the child 's urine output and the appearance of coca - cola colored urine and swollen legs . hospitalization should be scheduled only when the patient 's parents do not seem able to monitor the condition of their child at home . we report signs and symptoms which can help clinicians to make a correct differential diagnosis when a child suffers from acute muscle pain in table 1 . . a family history of neuromuscular disorders , myoglobinuria , trauma , chronic progression , rash , edema , muscle weakness or neurological diseases are not typically associated to bacm ; in these cases further investigation is required .

acute muscle pain and walking difficulty are symptoms compatible with both benign and severe degenerative diseases . as a consequence , in some cases invasive tests and hospitalizations are improperly scheduled . we report the case of a 7-year - old child suffering from acute calf pain and abnormal gait following flu - like symptoms . a review of the literature will be helpful to better define differential diagnosis in cases of muscle pain in children . daily physical examination and urine dipstick are sufficient to confirm the diagnosis of benign acute childhood myositis ( bacm ) during the acute phase , to promptly detect severe complications and to rule out degenerative diseases . children with bacm do not require hospitalization , medical interventions or long - term follow - up .

teratomas are congenital tumors that are usually found in the gonads , and are only rarely detected in extragonadal areas , such as , the mediastinal , retroperitoneal and sacrococcygeal regions . to the best our knowledge , an extragonadal teratoma of the long bone has not been previously reported in the literature . we describe clinical , radiologic and pathologic findings of an exceedingly rare case of malignant teratoma of the proximal humerus . a 14-yr - old female was admitted with painful limited motion of the left shoulder joint . she was transferred to our institute for further study and proper management under impression of malignant bone tumor . on physical examination , however , a radiograph of the humerus showed ill defined moth eaten to permeative destructive lesion associated with cortical perforation of proximal humerus ( fig . 1a ) . magnetic resonance imaging ( mri ) well demonstrated the entire extent of the tumor , which showed inhomogeneous enhancement after contrast agent administration . both the intraossoeus and extraosseous tumor components were evident on transaxial gradient - echo mr images ( fig . 99 m tc - mdp scintigraphy and an f - fdg whole body positron emission tomography ( pet ) scan showed uneven increased uptake at the left proximal humerus , but no other abnormality was detected . initial diagnostic considerations included ; leukemic infiltration , lymphoma , osteomylelitis , and a malignant bone tumor , such as , an osteosarcoma . a subsequent surgical biopsy was performed and the pathological report revealed an immature bony teratoma . on gross inspection , the cut surface of the resected proximal humerus showed a homogenous grayish white flesh - like tissue that had replaced the whole epimetaphysis and a part of the diaphysis ( fig . malignant teratoma were graded using a method described by robboy and scully ( 1 ) ; degree of immaturity , presence and quantity of a neuroepithelial component . grade 1 tumors with rare foci of immature neuroepithelial tissue occupy less than one low power field ( 40x ) , grade 2 tumors occupy 1 to 3 , and grade 3 tumors occupy more than 3 low power fields . the latter looked immature in appearance , which is not found in normal adult tissue . immunohistochemical staining demonstrated the expressions of glial fibrillarary acidic protein ( gfap ) and cd56 expression in neuronal tissue ( fig . based on the above - mentioned grading system , the tumor of the present case was classified as a grade 1 immature teratoma bearing immature endodermal glandular structures . there was no evidence of local recurrence or distant metastasis at 30 months postoperatively . according to the musculoskeletal tumor society scoring system ( 2 ) , teratomas are germ cell neoplasms that include tissues arising from the three germ cell layers ( 3 ) . they usually originate from pluripotent cells , which can differentiate to a wide variety of tissues . the majority of extragonadal teratomas develop from germinal elements , and they usually occur along the migration pathway of the germ cells ( 4 ) . resultantly , the sacrococcygeal region is the most frequent extragonadal location ; other sites include the mediastinum and retroperitoneum ( 5 ) . exceptionally rarely intraosseous teratoma has reported but there has been no reported case involving a long bone in the literature . in 1992 , chinoy et al . ( 6 ) reported a case of an extragonadal malignant teratoma of the foot and in 2000 , vazquez et al . ( 7 ) described an intraosseous teratoma of the iliac bone . in these previously published reports , initial diagnostic considerations included fibrous dysplasia , langerhans ' cell histiocytosis , aneurysmal bone cyst and a malignant bone tumor , such as , an osteosarcoma . most skeletal germ cell tumors are metastatic ( 8) but the pathogenesis of teratoma in the appendicular skeleton is still enigmatic . another possible pathogenetic theory concerns the heterotopic location of extragonadal germ cells . however , the humerus is not in the migration path of germ cells , and in fact , no teratoma has been identified previously at this site . thus , we are uncertain about the pathogenesis of the bony teratoma in this case . nevertheless , since the tumor was confirmed as a grade 1 immature teratoma , a wide surgical excision only was undertaken for comprehensive treatment . she is doing well without evidence of local recurrence or distant metastasis at final follow - up examination .

a extragonadal malignant teratoma of the extremity is a rare pheonemenon . we describe a extremely rare case of malignant teratoma of the left proximal humerus in a 14-yr - old female . radiologic evaluations , including magnetic resonance imaging , suggested a malignant bone tumor , but a pathological examination revealed an immature bony teratoma . bone scintigraphy and positron emission tomography computed tomography scan showed increased uptake of proximal humerus but no other abnormal lesion .

the facial nerve trunk exits through the stylomastoid foramen , and enters the parotid gland where it divides into the cervicofacial and temporofacial divisions . then divides into five peripheral branches to supply the muscles of facial expression ( 1 ) . the branches arising from the rami form to parotid plexus in parotid gland . the superior buccal nerve arising from temporofacial ramus and the inferior buccal nerve arising from the cervicofacial ramus form to the buccal plexus without parotid gland ( 1 ) . the zygomatic ( 2 ) , the marginal mandibular ( 3 - 5 ) , the buccal ( 6 ) , and the temporal branches ( 7 ) of the facial nerve were investigated by many researchers . different surgical approaches and landmarks to the trunk of the facial nerve have been reported ( 8 - 12 ) . many landmarks such as the mastoid process ( 8 - 10 ) , the transverse process of the atlas ( 8) and axis ( 11 ) , the temporomandibular joint , the angle of the mandible ( 9 , 11 ) , insertion of the sternocleidomastoid muscle , pointer cartilage of the ear ( 10 ) , tragal pointer ( 11 ) and the marginal mandibular branch of the facial nerve ( 12 ) can be used to identify the trunk of the facial nerve . but identification of this trunk may be difficult because it is encompassed by dense connective tissue ( 13 ) . in this paper the double facial nerve trunk emerged from the stylomastoid foramen and petrotympanic fissure was found in a 65-yr - old caucasian male cadaver during a routine dissection course . firstly , the skin and superficial fascia between the mastoid process and ramus of the mandible were reflected in all subjects . the anterior border of the parotid gland was carefully elevated and the rami of the nerves followed proximally up to the stylomastoid foramen under a stereomicroscope ( stemi 2000 ; carl zeiss , jena , germany ) . finally , magnetic resonance ( mr ) imaging was performed by using 1.5 t scanner to this cadaver . to be able to demonstrate the stylomastoid foramen and the petrotympanic fissure in same section , we obtained t1 and t2 weighted oblique sagittal scans . in the present cadaver , difference with the exit point of the facial nerve was observed . in this specimen , in this side , although cervicofacial ramus of the facial nerve exited from the stylomastoid foramen , temporofacial ramus of the facial nerve exited from petrotympanic fissure ( fig . the buccal branch dividing from temporal branch of temporofacial ramus and first buccal branch dividing from cervicofacial ramus formed to first buccal plexus . the buccal branch dividing from zygomatic branch of temporofacial ramus and second buccal branch dividing from cervicofacial ramus formed to second buccal plexus ( fig . 2 ) . these two buccal plexuses and other branches formed to structures like to polygon ( fig . the temporofacial ramus exiting from petrotympanic fissure was seen on magnetic resonance image ( fig . several studies relating to the trunk of the facial nerve have been reported in the literature ( 14 - 19 ) . katz and catalano ( 14 ) reported three cases ( 3% ) presenting two main trunks , known as the major and minor trunks , with the latter joining the larger temporofacial division , the origin of the main buccal branch . the minor trunk of the facial nerve was noted in eight of 30 cases ( 26.7% ) and , in all of them , the minor trunk entered the lower division of the facial nerve ( 15 ) . botman and jongkees ( 16 ) reported that the facial nerve within the mastoid segment of the temporal bone can split into two or three branches , and each branch exits through a separate osseous foramen . in this study , a trunk of the facial nerve exiting from the petrotympanic fissure is present one case . in addition , baker and conley ( 17 ) reported the possibilities of trifurcation , quadrifurcation , or even a plexiform branching pattern of the trunk of the facial nerve . salame et al . ( 18 ) identified one case of trifurcation out of 46 cases . but we did not identify any case of trifurcation , quadrifurcation or a plexiform branching pattern of the trunk . the facial nerve can easily be injured by sharp or penetrating trauma to the cheek . there are number of studies concerning protection extracranial branches of the facial nerve during plastic surgery procedures and operations intended for the parotid gland , but relatively little care has been given exit point of the facial nerve . knowledge of the trunk of the facial nerve is essential for preserving the nerve during surgical procedures of the mastoid process , parotid gland , the cranial base and the facial nerve ( 13 , 17 ) . in this study , we exposed exit point of the trunk of the facial nerve through the cranium . surgeons should be aware of the possible anatomical variations of the trunk of the facial nerve , because a trunk of the facial nerve exiting from the petrotympanic fissure may also be present .

there are several studies concerning branches of the facial nerve , but we encountered less information about the trunk of the facial nerve in the literature . during the routine dissection of a 65-yr - old caucasian male cadaver , double facial nerve trunk emerged from the stylomastoid foramen and petrotympanic fissure were encountered . because of an extremely rare variation , we presented this case report . in addition this cadaver had two buccal plexuses . these plexuses and other branches were formed to structures like to polygon . these anatomic peculiarities were described , photographed and illustrated . finally , magnetic resonance imaging was performed by using 1.5 t scanner to this cadaver . the facial nerve trunk can be damaged during surgical procedures of the parotid gland tumours and submandibular region . surgeons who are willing to operate on this area should be aware of the possible anatomical variations of the facial nerve trunk .

extralobar pulmonary sequestration is defined as a mass of pulmonary parenchyma anatomically separate from the normal lung . in extralobar pulmonary sequestration , the tissue has a distinct pleural covering and may be supplied by an aberrant artery from the aorta or its branch , in rare instances , extralobar pulmonary sequestration communicates with the esophagus or stomach . the pulmonary sequestration is found lying mostly at the left lung base but can be found in the right lung and in the mediastinum at any level from the neck to below the diaphragm . extralobar pulmonary sequestration is often discovered at the time of routine roentgenogram of the chest or at the time of repair of associated congenital anomalies such as diaphragmatic hernia and fistulous communication with the gastrointestinal tract because extralobar sequestration rarely presents like the intralobar form with recurrent pulmonary infection . a 27-year - old man was admitted to yong dong severance hospital , yonsei university medical center , for evaluation and management of mediastinal mass . three years prior to admission , he found mediastinal mass on computerized tomographic ( ct ) scan performed in another institution . at admission , he was noted to be with generalized weakness and right chest pain , but he denied cough , sputum and dyspnea . the temperature was 38.6c , the pulse rate 72/min and the blood pressure 110/70 mmhg . on examination , he appeared chronically ill , the skin was warm and dry , the head and neck were normal and cervical lymphadenopathy was not found , the lungs were clear and the heart was normal . the urine was normal and the hemoglobin was 13.5gm / dl ; hematocrit 41.9% ; white cell count 5,500/mm with 64 percent neutrophils , 35 percent lymphocytes and 1 percent eosinophils , the serum electrolytes and liver function tests were normal . t3 : 150 ng / dl , t4 : 7.1 ug / dl , ft4 : 1.6ng / dl and tsh was 1.5 uu / ml . the electrocardiogram showed right axis deviation and tall r wave in right precordial leads , and the sputum exanination did not suggest malignancy or tuberculosis . chest ct scan revealed large multi - cystic mass lesion on the right paratracheal area which was attached to the lower pole of right thyroid gland , displaced the superior vena cava anteriorly and extended to the level of the azygous arch vein ( fig . the lung perfusion scan and whole body bone scan were normal and the thyroid scan revealed cold area in the lower pole of right thyroid gland . the pulmonary function studies showed mild restriction with fev1:2.85 l / sec and fvc : 3.49 l / sec . 88 cm sized mass composed of multi - cystic and solid part was located in the pericardium and excised easily from the surrounding structures and the feeding vessel maybe originated from the subclavian artery . grossly , the mass was composed of several cystic lesions and yellow solid lesion , resembling unexpanded lung tissues ( fig . mlicroscopically , the cyst wall showed a bronchial tissue which was composed of ciliated columanar epithelium , submucosal gland and cartilage ( fig . 5 ) and solid portion of the mass showed alveolar space filled with inflammatory cells such as macrophages or lymphocytes ( fig . pulmonary sequestration is an uncommon congenital malformation characterized by the presence of nonfunctioning lung tissue which usually has no communication with the normal bronchial tree and which receives its blood supply from an anomalous systemic artery instead of a pulmonary arterial branch . in intralobar sequestration , the abnormal pulmonary tissue is incorporated within the normal lung and shares a common covering of visceral pleura , and in extralobar sequestration , the abnormal pulmonary tissue is separate from the normal lung and has its own pleural covering . is generally accepted and their spectrum of bronchopulmonary foregut malformation includes intralobar and extralobar sequestration , esophageal diverticuli , foregut duplication cysts ( esophageal cysts ) and maybe expanded to include bronchogenic cysts . the incidence rate was 1.1 to 1.8 percent of all lobectomied patients by carter and 0.15 to 6.4 percent of all congenital pulmonary malformation by savic . savic found 133 cases of extralobar sequestration in a review of 547 cases of pulmonary sequestration . the male predominance of patients with extralobar pulmonary sequestration ( 3:1 ) was described by carter and savic described that extralobar sequstration was located between the diaphragm and lower lobe in 77.4% and on left lung in 78.9% of the cases . most of the published cases of extralobar pulmonary sequestration were located outside the pericardium , only four reported cases were intrapericardial . since an extralobar sequestered segment is enveloped in its own pleural sac , the chance of becoming infected are very small , unless there is communication with the gastrointestinal tract . the extralobar sequestration ( 42% ) is more often associated with other anomalies than intralobar type ( 14% ) such as diaphragmatic hernia and fistulous communication with the gastrointestinal tract . consequently , extralobar sequestration is usually an incidental finding on routine roentgenograms of the chest or during management of some other congenital anomaly and infrequently , extralobar sequestration presents with symptoms similar to those seen with the intralobar form and with a picture of congestive heart failure or pulmonary overcirculation . in all cases of intrapericardial extralobar pulmonary sequestration , a large quantity of pericardial fluid was found , but no sign of congestive heart failure or cardiac tamponade was noticed . a correct preoperative diagnosis is made in 39 percent of intralobar lung sequestration and only in 9 percent of extralobar lung sequstration by savic and correct preoperative diagnosis is more difficult in the cases of unusual location . preoperative angiography is important to confirm the diagnosis and to afford a safe operative approach . the sequestratered segment is typically made up of multiple cysts or noncystic mass containing branching bronchi which run in the direction of the aberrant artery . histologically , the sequestration consists of normal lung elements in an abnormal and disorderly arrangement with a variable presence of cartilage , bronchial glands and alveolar parenchyma . extralobar pulmonary sequestration with symptoms or discovered at operation for another problem should be excised and it is feasible to observe asymptomatic and definite extralobar pulmonary sequestration without operation .

extralobar pulmonary sequestration , known as accessory lung , is a rare congenital anomaly and intrapericardial lung sequestration is extremely rare.to the best of our knowledge , only four reported cases were intrapericardial . we report a case with intrapericardial extralobar pulmonary sequestration confirmed by operation and morphologic basis .

the stubbed toe is a common innocuous injury , usually sustained barefoot , which can result in a wide range of injuries including nail bed lacerations , fractures of the distal phalanx and , uncommonly , bony or soft tissue mallet injuries . the mainstay of management of simple fractures and nail bed injuries is usually non - operative , with good long - term function and little or no morbidity . mallet fingers are common injuries with well - established treatments depending on the presence or absence of a fracture or joint subluxation . however , similar injuries to the hallux are rare with only a handful of single case report in the literature [ 37 ] . the rarity of these injuries likely relates to the relative sizes of the digits and tendons and the protection offered by footwear . owing to their relative rarity , there is no consensus as to the optimum treatment which ranged from non - operative treatment in a rigid soled shoe , closed reduction and stabilization with kirschner wires or open repair and reattachment of the tendon with suture anchors . failure to recognize and treat these injuries can lead to extensor lag of the hallux , which carries some morbidity . a 13-year - old girl injured her left great toe while running downstairs barefoot , incurring a forced plantar flexion injury . there was pain , swelling and bruising to the hallux , but she was able to weight bear . she noted a progressive drooping of the hallux over the following 3 weeks and presented to the trauma clinic via her gp . on examination , she had a swelling overlying the interphalangeal joint ( ipj ) of the hallux , which was held in 20 of plantar flexion , and she was unable to actively dorsiflex at the ipj . plain radiographs showed no bony injuries , but the ipj was subluxed in a plantar - flexed position ( fig . 1 ) . an urgent ultrasound scan confirmed a full thickness rupture of the extensor hallucis longus ( ehl ) tendon at its insertion into the distal phalanx and evidence of a healing tear to the dorsal joint capsule ( fig . 2 ) . plain radiograph showing plantar - flexed hallux ipj . ultrasound image showing ruptured ipj capsule and avulsed ehl tendon . under general anaesthesia , an l - shaped incision was made to avoid the dorsomedial sensory nerve and avoid injury to the germinal matrix of the nail . the ehl tendon was found to be completely ruptured from the distal phalanx and had retracted back towards the mtpj . the tendon end was debrided back to healthy tissue and was secured back to the distal phalanx using a mitek anchor and a non - absorbable suture in a modified krakow technique . the ipj was stabilized with a 1.2-mm kirschner wire ( figs 35 ) . figure 4:operative photograph showing transfixion of the ipj with a k - wire and insertion of a mitek anchor into the distal phalanx . operative photograph showing transfixion of the ipj with a k - wire and insertion of a mitek anchor into the distal phalanx . the k - wire was removed 4 weeks postoperatively and she was placed into a thermoplastic extension splint for a further 4 weeks , allowed to weight bear normally in shoes and undertake a passive range of movement exercises with physiotherapy . at this stage , she had good power of dorsiflexion , but limited plantar flexion due to stiffness . at the point of discharge , 6 months following surgery , she had regained a full range of movement compared with the contralateral side , full power of plantar flexion and dorsiflexion , and had returned to normal function . the earliest reports of mallet hallux by rapoff in 1999 and saxby et al in 2001 advocated non - operative treatment with a rigid soled shoe and an extension splint , respectively [ 3 , 7 ] . these cases were both middle - aged patients who had sustained bony mallet injuries and following treatment had stiff ipjs , but were asymptomatic with good function . more recently , three of these reports describe temporary stabilization with k - wires ( either to reduce and stabilize fracture fragments directly or to act as blocking wires ) [ 5 , 6 , 8 ] . in all of these cases , all report bony union within 58 weeks and full function at final follow - up . however , only one of these patients ( who was 16 years old ) regained a full range of movement . mallet hallux using bio - absorbable suture anchors and non - absorbable sutures , in combination with early mobilization of the joint . they were concerned that popular k - wire transfixion was associated with a high degree of stiffness and the possibility of infection and wire breakage . they showed that with this combination of treatment , both of their patients were able to get back to full function relatively sooner with no stiffness of the joint . hallux mallet as the ehl tendon is larger than the extensor tendons involved in soft tissue mallet injuries of the fingers , meaning that the repair is of better quality . in our reported case , due to the chronicity of the rupture ( nearly 4 weeks at the time of surgery ) and degree of retraction of the ehl tendon , it was felt that the patient would benefit from the combined treatment with a suture anchor and k - wire transfixion . following this combination of treatment , the patient has made a full functional recovery and achieved a full range of movement . we feel that this reflects the strength of the reconstruction and relative protection that the k - wire affords in addition to a suture anchor , with relatively early ( 4 weeks ) passive movement . additionally , the only other patient to achieve a full range of movement following treatment was also a teenager ( despite having ipj transfixion ) , suggesting that , as with most injuries , the virtues of youth enable better recovery . unstable bony and soft tissue mallet injuries of the hallux are uncommon . in this more chronic case in a younger patient with significant retraction of the tendon , we advocate open reconstruction with combined suture anchor repair and k - wire transfixion , with a return to normal functional outcomes and a range of movement .

we describe a case of a delayed presentation of a traumatic soft tissue mallet injury to the distal phalanx of the hallux in a teenager . reports of this rare injury are sparse and there is no consensus in their treatment . this injury was treated with open repair of the tendon using a mitek suture anchor , and stabilization with a 1.2-mm k - wire and extension splinting for 8 weeks . following this treatment , she regained full power and a range of movement compared with the contralateral side , and achieved full function . we advocate surgical reconstruction of these injuries with suture anchors and stabilization of the interphalangeal joint .

to report the effective treatment of radiation macular edema following ruthenium-106 plaque brachytherapy for a choroidal melanoma with a dexamethasone 0.7-mg ( ozurdex ) intravitreal implant . a 65-year - old caucasian woman was suffering from radiation macular edema following ruthenium-106 plaque brachytherapy for a choroidal melanoma on her left eye . she had undergone one intravitreal injection of 0.5 mg bevacizumab ( avastin , genentech / roche ) in the following months without functional or anatomical improvement . seven months after the development of radiation macular edema , she received a single intravitreal injection of dexamethasone 0.7 mg ( ozurdex ) . four weeks following the injection , her best - corrected visual acuity improved from 0.3 to 0.5 . radiation macular edema resolved with a reduction of central retinal thickness from 498 m before ozurdex injection to 224 m after ozurdex injection , as measured by oct scan . dexamethasone 0.7 mg ( ozurdex ) has proven to be an effective treatment option in retinal vein occlusion and noninfectious uveitis . it can also be considered as off - label treatment in radiation macular edema following ruthenium-106 plaque brachytherapy for a choroidal melanoma . radiation retinopathy is a predictable complication after radioactive scleral plaque application for treatment of intraocular tumors , most commonly choroidal melanoma . macular edema is a common manifestation of radiation retinopathy that may lead to severe visual loss . according to guyer et al . , it was the most frequent and early finding after proton beam therapy for paramacular choroidal tumors , with 87% of 218 patients who developed macular edema . other studies suggest rates of radiation maculopathy from plaque radiotherapy of 18% , 23% , and 42.8% . these include laser photocoagulation , photodynamic therapy , pentoxifylline and intravitreal bevacizumab [ 9 , 10 ] . corticosteroids are potent anti - inflammatory agents that can counteract many of the pathological processes thought to play a role in the development of macular edema . recently , a sustained - release dexamethasone implant ( ozurdex ) proved to be effective for the treatment of macular edema secondary to a variety of underlying diseases with a potentially lower rate of adverse events . there are no cases of radiation macular edema after ruthenium ( ru)-106 plaque brachytherapy for choroidal melanoma resolved by an intravitreal dexamethasone 0.7-mg implant described so far in the literature . we report a case of radiation macular edema after ru-106 brachytherapy for a choroidal melanoma , refractory to a previous treatment with intravitreal bevacizumab , and resolved with significant improvement of visual function following an intravitreal injection of dexamethasone 0.7 mg . a 65-year - old caucasian woman developed radiation macular edema 24 months following ru-106 plaque brachytherapy for a choroidal melanoma on her left eye ( fig . she underwent one intravitreal injection of 0.5 mg bevacizumab ( avastin , genentech / roche ) in the following months without functional or anatomical improvement . seven months after the development of radiation macular edema , she received a single intravitreal injection of dexamethasone 0.7 mg ( ozurdex ) as off - label treatment . four weeks following the injection , her best - corrected visual acuity ( bcva ) improved from 0.3 to 0.5 . 1b ) with a reduction of central retinal thickness from 498 m before ozurdex injection ( fig . her condition has been stable for at least 5 months now , as seen on the last examination , with bcva 0.6 and resolved macular edema in the treated eye . shields et al . reported the use of intravitreal triamcinolone ( 4 mg / ml ) in a prospective , nonrandomized , single - center case series of 31 patients with visually symptomatic radiation - induced macular edema after plaque radiotherapy . they reported that visual acuity was stable or improved in 91% of patients by one month and 45% by six months ; they also found a decrease in the mean central subfield foveal thickness measured by oct . despite the potential benefits , intravitreal injection of triamcinolone acetate is associated with side effects , including glaucoma , cataracts , retinal detachment , and endophthalmitis [ 13 , 14 ] . also intravitreal bevacizumab has been evaluated as a treatment option for radiation maculopathy secondary to plaque radiotherapy [ 9 , 10 ] . our patient was initially treated with an intravitreal injection of bevacizumab which has not proved effective . gupta et al . stated that intravitreal injection of bevacizumab for treatment of radiation maculopathy caused by plaque radiotherapy was not useful for long - standing macular edema or when direct irradiation was received by the fovea . one of the main mechanisms of the chronic macular edema is the alteration of muller cells functionality ; it has been experimentally shown that steroids , by reducing the osmotic swelling of the muller 's cells , improve their functionality and reduce the macular edema . this could indicate that dexamethasone implant ( ozurdex ) might be an effective treatment option not only in retinal vein occlusion and noninfectious uveitis , but can also be considered as off - label treatment in radiation macular edema after ru-106 plaque brachytherapy for choroidal melanoma .

purposeto report the effective treatment of radiation macular edema following ruthenium-106 plaque brachytherapy for a choroidal melanoma with a dexamethasone 0.7-mg ( ozurdex ) intravitreal implant.methodsan interventional case report with optical coherence tomography ( oct ) scans.resultsa 65-year - old caucasian woman was suffering from radiation macular edema following ruthenium-106 plaque brachytherapy for a choroidal melanoma on her left eye . she had undergone one intravitreal injection of 0.5 mg bevacizumab ( avastin , genentech / roche ) in the following months without functional or anatomical improvement . seven months after the development of radiation macular edema , she received a single intravitreal injection of dexamethasone 0.7 mg ( ozurdex ) . four weeks following the injection , her best - corrected visual acuity improved from 0.3 to 0.5 . radiation macular edema resolved with a reduction of central retinal thickness from 498 m before ozurdex injection to 224 m after ozurdex injection , as measured by oct scan.conclusiondexamethasone 0.7 mg ( ozurdex ) has proven to be an effective treatment option in retinal vein occlusion and noninfectious uveitis . it can also be considered as off - label treatment in radiation macular edema following ruthenium-106 plaque brachytherapy for a choroidal melanoma .

peripheral arterial aneurysms are rare , and among these , deep femoral arterial ( dfa ) aneurysms are extremely rare [ 13 ] . dfa aneurysms are not associated with any characteristic symptoms , and their early diagnosis is difficult . these aneurysms have a high rate of rupture , and surgery plays an important role in their treatment . however , standard methods have not yet been established because of the rarity of dfa aneurysms [ 36 ] . we describe a case of dfa aneurysm in a patient who was successfully treated with aneurysmectomy and revascularization with an artificial blood vessel graft . a 58-year - old man presented with paralysis and pain in the left leg , and a mass was found in his thigh . because of the growth of the mass and the worsening of his symptoms , the patient visited a neighboring hospital . physical examination revealed a pulsatile mass with a diameter of 8 cm in the left thigh . multidetector computed tomography performed in our hospital revealed a large dfa aneurysm with an intraluminal thrombus ( fig . 1 ) . surgical intervention was planned because of the large size of the aneurysm , the high risk of perforation and the worsening symptoms . figure 1:multidetector computed tomography revealed a large dfa aneurysm ( arrows show aneurysm ) . the common femoral artery , superficial femoral artery , superficial femoral vein and deep femoral vein were recognized and preserved ( fig . 2a ) . after clamping the dfa proximal and distal to the aneurysm , we opened the sac and found a massive thrombus . aneurysmectomy and revascularization of the distal dfa with an artificial blood vessel graft were performed ( fig . pathological examination of the specimen revealed atherosclerosis in the vascular wall and confirmed that it was a true aneurysm . figure 2:(a ) the common femoral artery , superficial femoral artery , superficial femoral vein and deep femoral vein were recognized and preserved . ( b ) aneurysmectomy and revascularization of the distal dfa with an artificial blood vessel graft were performed . ( a ) the common femoral artery , superficial femoral artery , superficial femoral vein and deep femoral vein were recognized and preserved . ( b ) aneurysmectomy and revascularization of the distal dfa with an artificial blood vessel graft were performed . following surgery , a follow - up multidetector computed tomography scan showed good blood flow in the graft , superficial femoral artery and popliteal artery ( fig . figure 3:a follow - up multidetector computed tomography scan showed good blood flow in the graft , superficial femoral artery and popliteal artery . a follow - up multidetector computed tomography scan showed good blood flow in the graft , superficial femoral artery and popliteal artery . although many dfa aneurysms have been associated with other peripheral aneurysms or peripheral occlusive diseases , there was no evidence of aneurysms or occlusive lesions in the other arteries of our patient [ 4 , 7 , 8 ] . therefore , we consider our case of an isolated dfa aneurysm to be extremely rare . there are no characteristic symptoms of dfa aneurysms in the early stage , and their diagnosis is difficult . they are often found accidentally or after their enlargement , when symptoms include pulsating groin swelling , paralysis or pain in the leg , which were the symptoms that our patient experienced . enlarged dfa aneurysms must be treated because of the high risk of rupture , which may cause severe complications , including hemorrhagic shock , ischemia of the foot , thigh embolization , thrombophlebitis and sciatic or femoral neuropraxia [ 1 , 5 , 9 , 10 ] . several treatment options , including simple ligation , revascularization of the distal dfa and bypass grafting to the popliteal artery , exist for dfa aneurysms ; however , standard methods have not been established because of the rarity of dfa aneurysms . in our case , we performed an aneurysmectomy and revascularization to the distal dfa with an artificial blood vessel as the graft . revascularization was performed to avoid thigh ischemia and preserve the collateral circulation if ipsilateral peripheral occlusive disease occurred . we believe that revascularization to the distal dfa should be done whenever it is technically feasible . in previous reports , if the patient must undergo operations such as coronary artery bypass grafting or other peripheral arterial bypass grafting , the saphenous veins will be required for those surgeries . therefore , we did not use the saphenous vein as the graft . in this case , we consider the surgery a success because the patient recovered well from the surgery without complications , his symptoms improved and the blood flow in the leg was preserved . in conclusion , dfa aneurysms are extremely rare . surgery plays an important part in the treatment of this disease , but standard methods have not been established because of its rarity . in our patient , we successfully performed an aneurysmectomy and revascularization to the distal dfa with an artificial blood vessel graft .

a 58-year - old man presented with paralysis and pain in the left leg , and a mass was found in his thigh . because of the growth of the mass and the worsening of his symptoms , the patient visited our hospital . multidetector computed tomography revealed a large deep femoral arterial ( dfa ) aneurysm . surgical intervention was planned because of the large size of the aneurysm , the high risk of perforation and the worsening symptoms . aneurysmectomy and revascularization of the distal dfa with an artificial blood vessel graft were performed . dfa aneurysms are extremely rare . these aneurysms have a high rate of rupture , and surgery plays an important role in their treatment . however , standard methods have not yet been established because of their rarity of dfa aneurysm . we describe a case of dfa aneurysm in a patient who was successfully treated with aneurysmectomy and revascularization with an artificial blood vessel graft .

additional reports were also extracted from promed mail ( 8) and healthmap ( 9 ) . we performed the analysis by using 3 dates : 1 ) date of case registration in the surveillance system of the brazilian ministry of health ( model 1 ) ; 2 ) earliest of either date of symptom onset ( if available ) or registration date ( model 2 ) ; and 3 ) earliest of either case registration date , date of symptom onset , or date of case report by other sources ( model 3 ) . surface trend analysis was used to interpolated a continuous estimate of disease spread speed in magnitude and direction ( 10 ) by using available spatial and temporal information . time of dispersal was calculated from the start of the epidemic for each model ( technical appendix ) . data provided by the brazilian ministry of health on may 31 , 2016 , indicated that zika had been confirmed in 316 of 5,564 municipalities in 26 states ; 6 additional municipalities were identified from other reporting sources . contour maps of interpolated temporal trends ( figure 1 ) indicate a trend of spread into southern and western brazil , and initial outbreak reports originated from municipalities along the northeastern coast . on the basis of confirmed cases , the earliest location of spread was the northeastern coastal area between the states of paraba , cear , baha , alagoas , and rio grande do norte . there were also earlier dates of self - reported symptom onset in the northwestern state of amazonas ( january 1 , 2015 ) , the west - central state of matto grosso ( january 4 , 2015 ) , and the southeastern coastal state of rio de janeiro ( january 1 , 2015 ) . contour surface trends and directional vectors for reconstructing zika introduction in brazil . a ) date of case registration ( model 1 ) ; b ) earliest date between date of symptom onset ( if available ) and date of registration ( model 2 ) ; c ) earliest date between date of case registration , date of symptom onset , and date of case reporting by other sources ( model 3 ) . each contour line represents a 1-day period , and contour lines farther apart show that the disease spread rapidly through an area , whereas lines close together show slower progression in an area . . magnitude of speed and direction should be interpreted cautiously near the edges of the study area . estimates of speed are subject to edge effects , which indicates that estimates are less stable because they are based on fewer data ( not as many neighboring values ) . contour maps ( figure 1 ) indicate slight differences in patterns of dispersion between the models . model 1 indicates the strongest trend of a southward spread from the northeastern coast toward the populous southeastern coastal states of rio de janeiro , esprito santo , and so paulo ; the estimated time of dispersal was 22 weeks ( figure 1 , panel a ) . in addition to west to east spread of zika in southern brazil , there was a pattern of movement west toward bolivia . the dispersal trend for model 2 was more varied but also indicated spread to the southeastern coastal states of rio de janeiro , esprito santo , and so paulo ( figure 1 , panel b ) . this model also suggests an initial spread north from the earliest reports in the northeastern region and a spread west toward bolivia . model 3 suggests a strong southward spread originating from the northeastern coast toward the southeastern coastal states ( approximate dispersal time of 29 weeks ) and toward the western border and northwestern state of amazonas ( figure 1 , panel c ) . overall , the average speed of diffusion was 42.1 km / day or 15,367 km / year . the minimum speed across all 3 models was 6.9 km / day , and the maximum speed was 634.1 km / day ( figure 2 ) . municipalities in northeastern and northern regions had the slowest speeds , and municipalities in the west - central and southeastern regions had the highest speeds . more cases occurred closer in time and over larger areas in southern , southeastern , and west - central regions , which resulted in faster rates of case introduction . speed or log speed ( km / d ) of zika introduction into municipalities in brazil . a ) june 2015may 2016 ; b ) january 2015may 2016 ; c ) january 2015may 2016 . gray circles indicate central western region , green circles indicate northern region , light blue circles indicate northeastern region , black circles indicate southern region , and dark blue circles indicate southeastern region . all models were consistent in agreement that zika dispersal in brazil followed a general pattern of southward spread toward the populous coastal states ( average speed of introduction of 42 km / day ) , which could be explained by multiple introductory cases into different areas probably caused by movement of viremic persons . we estimate that it took 56 months for zika to spread from the northeastern coast to the southeastern coast and western border of brazil . these findings are supported by the first report of local transmission of zika virus in paraguay in late november 2015 ( 11 ) and in bolivia in january 2016 ( 12 ) , 7 months after the first registered case in brazil . limitations of this analysis include quality and timeliness of surveillance data that provided the basis for this study . symptom onset date is subject to error because it is based on self - report , and earlier introductions of zika in some municipalities might not have been captured by the ministry of health surveillance system and supplementary data sources , given the mild and generic nature of zika symptoms and the high proportion of asymptomatic persons ( 3 ) . the northern region of brazil had a major dengue outbreak in early 2015 , and given symptom similarities between dengue and zika , it is probable that some suspected dengue cases were in fact early cases of zika . sporadic geographically disparate cases were recorded in various parts of brazil , which increased the uncertainty associated with speed analysis . these cases , such as those in northwestern brazil , increased uncertainty in direction and speed estimates , which are also related to edge effects . edge effects occurred along the boundary of the study area , which in this study were constructed by using fewer data points and are therefore less stable . this effect is shown with directional arrows pointing toward earlier areas of spread versus toward later areas of spread ( figure 1 , panels b , c ) . the arrival and rapid spread of zika virus in the americas resembles that of chikungunya virus , which was introduced into saint martin in the caribbean in 2013 ( 13,14 ) . increased knowledge of the speed of spread and direction of zika spread can help in understanding its possible future directions and pace at which it travels , which would be essential for targeted mosquito control interventions , public health messages , and travel advisories . future work will investigate underlying causes for the southward and westward spread in brazil by incorporating mobility data and seasonal events , such as movement of persons between northeastern and southeastern regions for vacations , which could have driven the spatial transmission pattern . furthermore , multicountry analysis is needed to understand continental spatial and temporal patterns of dispersion of zika virus and co - circulating viruses , such as chikungunya virus .

we estimated the speed of zika virus introduction in brazil by using confirmed cases at the municipal level . our models indicate a southward pattern of introduction starting from the northeastern coast and a pattern of movement toward the western border with an average speed of spread of 42 km / day or 15,367 km / year .

about 96% are symptomatic at presentation with a history of stricture disease in more than half and sexually transmitted disease in one fourth of all patients . rarely , it can manifest as a periurethral abscess , fistula , or fungating perineal tumor . while urethral carcinoma is known to aggressively infiltrate periurethral tissue ; concomitant involvement of suprapubic catheter tract has not been reported in the literature . in this paper , we reiterate the importance of high clinical suspicion in making this rare diagnosis , the consequences of delayed diagnosis and the role of biopsy of a suprapubic tract with clinical features of malignancy . a 65-year - old diabetic male with a long history of urethral stricture developed rapid worsening of his lower urinary tract symptoms and noticed a painful swelling in his upper scrotum for 2 weeks . he was diagnosed with a periurethral abscess and underwent incision and drainage at another center . his perineal wound did not heal and over the next 3 months it developed into a fungating ulcer . he consulted his surgeon , who performed a biopsy revealing squamous cell carcinoma ( scc ) . at presentation to us , he had a 10 6 cm , fungating , malodourous , ulceroproliferative scrotal growth fixed to the region of the bulbar urethra . the abdominal entry site of the suprapubic catheter was ulcerated with rolled out edges and induration [ figure 1a ] . ct scan showed an enhancing ill - defined soft tissue density in the region of bulbar urethra infiltrating corpora cavernosa , adjacent skin and subcutaneous tissue [ figure 1b ] . transverse section [ figure 1c ] at the level of bladder and suprapubic cystostomy ( spc ) tract showed induration surrounding the tract . he underwent a total penile amputation , wide local excision scrotum , radical urethrocystoprostatectomy , ileal conduit diversion , and pelvic lymphadenectomy [ figure 2a ] . we included wide local excision of the spc site , en bloc with the cystoprostatectomy specimen . histopathological examination showed moderately differentiated scc of the bulbar urethra [ figure 2b ] with 2 right external iliac lymph nodes positive for metastasis . postoperative staging was t3n1 ; he was given a course of adjuvant chemotherapy ( cisplatin and gemcitabine ) and has completed follow - up of 6 months . ( a ) ulceroproliferative growth at the penoscrotal junction and , suprapubic cystostomy ( spc ) site with ulcerated , everted mucosal edges ; ( b ) computed tomography scan showing an ill - defined , enhancing mass in the region of posterior urethra infiltrating cavernosae ( arrow ) ; ( c ) transverse section showing bladder and spc tract ( arrow head ) ( a ) postoperative picture following radical urethrocystoprostatectomy with en - bloc excision of suprapubic cystostomy ( spc ) tract and wide local excision of scrotum ; ( b ) h and e , 10 showing primary squamous cell carcinoma ( scc ) of urethra with keratin pearls ; ( c ) h and e , 20 showing foci of scc in the spc tract our patient had a history of long - standing urethral stricture complicated by a periurethral abscess . others are chronic irritation after intermittent catheterization , following urethroplasty , external - beam radiation therapy , radioactive seed implantation , and chronic urethral inflammation or urethritis following sexually transmitted diseases . male urethral carcinoma most commonly occurs at the bulbomembranous urethra ( 60% ) , while the penile and the prostatic urethra are afflicted in 30% and 10% of cases respectively . histologically , scc accounts for 80% of urethral malignancy , 15% are transitional cell carcinoma and less than 5% are adenocarcinoma . symptoms of this malignancy are neither pathognomonic nor diagnostic . the often insidious onset of the disease and non - diagnosis at initial presentation might result in substantial delay between first symptoms and treatment . common presentations include bleeding per urethra , discharge , urinary obstruction , penile or perineal pain , hematospermia , and a palpable mass . in rare cases such as this , a periurethral abscess or urethral fistula in the background of stricture disease may be the sequel of advanced infiltrative malignancy . the early obstructive symptoms can mimic stricture disease and indeed , progressively difficult dilatation of urethral stricture may presage the diagnosis in the presence of adequate clinical suspicion . similarly , urethral bleeding in the absence of prior trauma or disease may reveal urethral malignancy on evaluation . regional lymph nodes are to be examined carefully in all cases ; palpable lymphadenopathy present in 20% of cases most often represents metastatic disease rather than inflammatory process as seen in penile cancer . local spread occurs by direct infiltration of adjacent tissue through the spongiosum and periurethral tissue into the urogenital diaphragm , perineum and scrotal skin . spread via lymphatic embolization into regional lymph node basins generally occurs late in the process . lymphatics from the anterior urethra generally drain into the superficial and deep inguinal nodes and less often directly into the external iliac group . faced with advanced clinical stage of disease , we undertook radical resection of the lower urinary tract with urinary diversion . in this case , malignancy at the suprapubic site was unlikely to have been caused by chronic irritation from the catheter given the relatively short duration of the diversion . previous reports of scc at the suprapubic tract involved period of catheterization from 5 to 35 years . this is the first report of suprapubic tract scc in the presence of primary urinary tract malignancy . we presume that the seeding of the tract occurred either from extension of tumor via transdermal lymphatic spread or exposure to malignant cells in urine . urine cytology in this case was however negative and the bladder was also free of tumor . perhaps the distal obstruction in combination with the breach of tissue planes by both tumor and intervention allowed viable malignant cells in urine access to the suprapubic site around the time of its creation . our anecdotal experience leads us to recommend biopsy of the suprapubic tract whenever examination arouses suspicion , especially when urinary tract malignancy is present . locally advanced urethral scc must be excised with a wide margin along with flap reconstruction of the defect when indicated , to allow optimal local oncologic clearance .

a 65-year - old man with stricture urethra underwent drainage of periurethral abscess and suprapubic cystostomy ( spc ) placement . he presented to us 3 months later with a fungating ulcer at the site of perineal incision , the biopsy of which revealed squamous cell carcinoma ( scc ) . he underwent a total penile amputation , wide local excision scrotum , radical urethrocystoprostatectomy , ileal conduit with the en - bloc excision of the spc tract . histopathological examination of the suprapubic tract also revealed scc . this is the first documented case of scc of a suprapubic tract in the presence of primary urethral scc .

all available dna sequences for coi ( 108 head and 58 clothing lice ) , 18s rrna ( 10 head and 12 clothing lice ) , ef-1 ( 25 head and 9 clothing lice ) , and rpii ( 25 head and 10 clothing lice ) for p. humanus and the outgroup p. schaeffi ( chimpanzee louse ) were downloaded from genbank ( available as supplementary table s1 , supplementary material online ) . all sequences were aligned by hand using se - al v2.01a11 ( http://tree.bio.ed.ac.uk/software/seal/ ) , with the 18s rdna aligned to secondary structure ( gillespie 2004 ; gillespie et al . substitution rates ( table 2 ) for the four genes were estimated in beast v.1.5.3 ( drummond and rambaut 2007 ) . rates were calibrated by placing an exponential prior distribution ( lower bound = 5 ma , mean = 5.5 ma ) on the divergence of p. humanus ( human ) and p. schaeffi ( chimpanzee ) lice that reflects conservatively recent estimates for the divergence of their hosts ( kumar and hedges 1998 ) . each gene was analyzed using a range of substitution and clock models , as well as tree priors , with posterior estimates made from the model that best fit the data as determined by marginal likelihoods estimated in the program tracer v1.5 ( http://tree.bio.ed.ac.uk/software/tracer ) . markov chains were run for at least 100 million generations , sampled every 10,000 generations , and the first 10% of samples were discarded as burn - in . models of substitution , likelihood scores , and mean substitution rates per gene calculated in beast . between site rate variation partitioned by codon position ( cp ) . multilocus bayesian i m coalescent analysis was performed on the p. humanus sequences using the program i m ( hey 2005 ) . all analyses used the hky substitution model , whereas priors on model parameters were broad uniform distributions conservatively estimated from preliminary runs . markov chains were run for > 200 million generations and replicated 8 times to ensure convergence . a louse generation time of 21 days ( 18 generations per year ) and the substitution rates in table 2 were used to convert parameter estimates from mutational to demographic units .

clothing use is an important modern behavior that contributed to the successful expansion of humans into higher latitudes and cold climates . previous research suggests that clothing use originated anywhere between 40,000 and 3 ma , though there is little direct archaeological , fossil , or genetic evidence to support more specific estimates . since clothing lice evolved from head louse ancestors once humans adopted clothing , dating the emergence of clothing lice may provide more specific estimates of the origin of clothing use . here , we use a bayesian coalescent modeling approach to estimate that clothing lice diverged from head louse ancestors at least by 83,000 and possibly as early as 170,000 years ago . our analysis suggests that the use of clothing likely originated with anatomically modern humans in africa and reinforces a broad trend of modern human developments in africa during the middle to late pleistocene .

the dominant motoric view of cerebellum has now changed to include its role in all cognitive functions and behavior as for the cerebral cortex . lesions of the cerebral cortex producing secondary mania are commonly described , but cerebellar lesion producing mania is relatively uncommon . this was a case report of a 28-year - old unmarried man from a rural low middle - income group , farmer by occupation presented to our out - patient department ( opd ) on 05.05.2008 with acute excitement and inability to walk . although working in the fields the previous day , he had a feeling of giddiness and had projectile vomiting . he could not sleep on the day of onset of his complaints . throughout the night he was disturbing others and demanding things . he was talking spontaneously and excessively to everyone as if they were familiar to him . he was expressing grandiose ideas that he has a lot of power and can even beat 500 men . he lacked insight to his mental illness , but accepted his walking difficulty and said he wants medical attention . there was mild slurring of speech , but the classical speech of cerebellar disorder was absent . there was no history of fever , convulsions , incontinence , swallowing or visual difficulty . urine examination , blood examination , including electrolytes , x - ray chest and skull were within the normal limits . bender - gestalt test showed signs of organicity [ figure 1 ] . in young mania rating scale , he scored 32 . bender - gestalt test drawing of the patient showing evidence of organicity like poor form level & poor relationship to one another and to the whole spatial background he was seen by neurologist twice and was diagnosed of having left cerebellar stroke . computed tomography ( ct ) brain was done twice , which showed a hypodense lesion in left cerebellar hemisphere suggestive of vascular infarct [ figure 2 ] . magnetic resonance imaging ( mri ) he fulfilled the criteria to diagnose organic manic disorder f06.30 as per icd 10 . computed tomography of brain showing hypo dense lesion in left cerebellar hemisphere suggesting vascular infarct patient was followed - up in the next 1 month . he was given tablet sodium valproate 200 mg 2 bd and tablet olanzapine 5 mg 2 bd and tablet diazepam 5 mg 2 h. neuroimaging studies confirm that the cerebellum is activated milliseconds before cerebral activation in all aspects of cognitive function like memory and thinking as it is before motor movements . cerebellum has rich to and fro connections not only to pyramidal areas but also to prefrontal lobe , temporal lobe , limbic area , cingulate gyrus , hypothalamus and thalamus . the role of the cerebellum in cognition and behavior is gaining more and more importance now . movement abnormalities in catatonia are again thought to be due to cerebellar and basal ganglia involvement . they beautifully illustrated a patient aa who presented with cerebellar signs as well as symptoms of schizophrenia . andreason suggests the presence of cognitive dysmetria due to cerebellar involvement in schizophrenia which is analogous to motor dysmetria so far described in cerebellar patients . lauterbach analyzing 45 post - stroke patients noted mania in three patients who had lesions in left cerebellum or left cerebellar tracts to right caudate and thalamus . unlike previous reports in literature , our patient is interesting in the sense that the cerebellar lesion has produced cerebellar and manic features simultaneously . schmahman and sherman in his editorial in brain documents the evidence for the neuropsychological and behavioral involvement in cerebellar lesions . he suggests that cerebellar lesions produce not only motor dysfunction but it produces a cerebellar cognitive affective syndrome . its defining features are disturbances in executive function , spatial recognition , language and emotional regulation of behavior . posterior lobe lesions produce core cerebellar syndrome , whereas vermian lesions produce pronounced affective disturbance . now we recognize subcortical dementia and obsessive - compulsive disorder is due to basal ganglia lesions . similarly , by analogy we should give more attention to the cerebellum for cognition and behavior . contribution of the cerebellum to cognition and emotion can no longer be ignored . from a clinical perspective , cerebellum has to be more closely looked for in ct and mri pictures of our patients . we have to learn more about cerebellum and we should not confine it to only a minor motor role . frick mentions that the cerebellum may form a major neurological component of the ego , particularly sub serving the autonomous ego functions . before concluding i shall quote what dow says about cerebellum in his book , just as cerebellum maintains motor balance it can as well balance other functions of brain of particular relevance to psychiatry .

secondary mania resulting from cerebral cortex are described commonly . but secondary mania produced by cerebellar lesions are relatively uncommon . this case report describes a patient who developed cerebellar stoke and manic features simultaneously . 28 years old male developed giddiness and projectile vomiting . then he would lie down for about an hour only to find that he could not walk . he became quarrelsome . his psycho motor activities and speech were increased . he was euphoric and was expressing grandiose ideas . bender gestalt test showed signs of organicity . score in young mania relating scale was 32 ; productivity was low in rorschach . neurological examination revealed left cerebellar signs like ataxia and slurring of speech . computed tomography of brain showed left cerebellar infarct . relationship between psychiatric manifestations and cerebellar lesion are discussed .

in february 2012 , a previously healthy 40-year - old man visited the academic medical center outpatient department , reporting fever , headache , sweating , and nausea . the signs and symptoms had started 1 day earlier , on the day of his return from a 1-month holiday in borneo . he reported frequent insect bites and exposure to fresh water . he had taken malaria chemoprophylaxis as recommended , and his vaccinations were up to date . physical examination indicated that he was afebrile , was hemodynamically stable , and had a discrete macular rash on the trunk but no eschar . laboratory results showed a hemoglobin concentration ( 16.8 g / dl ) within reference range , a leukocyte count of 4,700 cells / mm with lymphopenia ( 1,090 cells / mm ) , and thrombocytopenia ( 116,000 cells / mm ) . c - reactive protein ( 42 mg / l ) and serum creatinine ( 1.32 mg / dl ) concentrations were moderately elevated . a thick smear showed no plasmodia , and a dengue antigen test result was negative . by the next day , the patient s condition had deteriorated ; he was experiencing chills , his temperature was 39c , and the rash had become more pronounced . he was admitted to the hospital and given doxycycline ( 200 mg twice a day ) for suspected rickettsiosis or leptospirosis . after admission , his condition deteriorated further ; increasing dyspnea progressed to respiratory failure , necessitating intubation and admission to the intensive care unit on the second day after admission . chest radiograph of 40-year - old man with acute respiratory distress syndrome as a complication of murine typhus . on the fourth day of intubation , cultured blood , urine , and bronchial fluid remained sterile , and test results for leptospira , legionella , influenza virus , and hiv were negative . all antimicrobial drugs except doxycycline were discontinued ; doxycycline was continued for a total of 14 days . serum collected 1 day after admission showed weakly positive igg against r. typhi ; after 7 days , the immunofluorescent antibody titer had increased 4-fold ( from 1:64 to > 1:256 ) . the patient recovered completely and was doing well at his last follow - up visit . to determine prevalence of such cases , we conducted a search of published studies mentioning pulmonary manifestations of murine typhus ( details in the technical appendix ) . from 779 records , we selected 22 cohort studies and 18 case studies that , according to title and abstract , were relevant to our research question . we differentiated between studies with individual patient data ( case reports and case series ) and studies without individual patient data ( cohort studies ) . for each study , we recorded year of publication , study design , and country of infection . we also recorded the presence of pulmonary involvement , defined as cough and any mention of an abnormal finding on chest radiograph , without further distinction . an overview of study characteristics detailing prevalence of cough and chest radiograph abnormalities is provided in the technical appendix table 1 . two studies were prospective population - based studies of the causative agent of fever of unknown origin . specific ; in 17 of these studies , patients had been recruited retrospectively from hospital databases or chart reviews . the 22 study reports that contained data on the presence or absence of cough together accounted for 1,060 patients with murine typhus . mean prevalence ( all patients from all studies combined ) of cough was 30.1% ( 95% ci 23.336.9 ) . data on presence or absence of radiographic abnormalities were mentioned in 9 study reports ( 412 ) . taken together , these studies evaluated 621 patients and 104 chest radiographs showing abnormalities , leading to a prevalence rate of chest radiograph abnormalities of 16.7% ( 95% ci 8.2125.5 ) . pulmonary manifestations were also documented by the case studies . among these studies , 2 patients had ards , 7 had bilateral pulmonary infiltrates , 5 had unilateral pulmonary infiltrates , 1 had pulmonary embolism , and 1 had respiratory failure ( no chest radiograph was reported ) ( technical appendix table 2 ) . we also found that cough occurred more commonly among patients in studies conducted in asia ( 99 [ 38.2% ] of 259 patients ) than among those in studies conducted in the mediterranean region ( 118 [ 25.8% ] of 457 ) and north america ( 56 [ 23.3% ] of 240 ) . in the literature , we found 7 reported cases of respiratory distress associated with murine typhus ( including the case reported here ) , 2 of which described respiratory distress not classified as ards ( 2,13 ) . of these 7 case - patients with ards / respiratory distress , 5 ( 71% ) patients were from asia ( 2,3,11,13 ) , 1 was from the mediterranean region ( 4 ) , and 1 was from the united states ( 14 ) . cough and chest radiograph abnormalities were frequent manifestations of murine typhus . for cough associated with murine typhus , we found a prevalence rate of 30% . the prevalence of chest radiograph abnormalities was more difficult to ascertain because this result was less often reported and more influenced by bias . the pulmonary aspects of r. typhi infection are probably the result of damaged pulmonary microcirculation , leading to pulmonary edema . the case reported here is unusual in that the symptoms progressed rapidly and the response to doxycycline was relatively slow . it is worth noting that we found no more than 2 reported cases of fatal murine typhus associated with pulmonary system disease ; both were the result of severe disease complicated by ards . most cases of severe murine typhus with pulmonary manifestations originated in asia , and cough was more frequently a symptom among patients in asia . the primary strength of our study is the extensive literature search , which covered studies from different parts of the world and included cohort studies and case series . the main limitation of this study is the retrospective nature of the data collection for most studies , which is prone to bias and renders meaningful statistical analysis of results impossible . therefore , prospective studies evaluating pulmonary manifestations of murine typhus and possible geographic variation are needed . although murine typhus usually follows a benign course , severe disease with pulmonary manifestations , including ards , can occur , as described for the patient reported here . we suggest that murine typhus should be included in the differential diagnosis for any patient who has a fever and respiratory signs and who has been in a typhus - endemic area within the incubation period . flowchart of study selection for review , table of cohort studies of murine typhus , and table of case studies detailing chest radiograph abnormalities .

we encountered a case of severe murine typhus complicated by acute respiratory distress syndrome . to determine worldwide prevalence of such cases , we reviewed the literature and found that respiratory symptoms occur in 30% of murine typhus patients . in disease - endemic areas , murine typhus should be considered for patients with respiratory symptoms and fever .

dowling degos disease ( ddd ) is a rare autosomal dominant condition characterized by multiple , small , round pigmented macules usually arranged in reticular pattern . the lesions are chiefly distributed in axillae and groins , but other areas may be involved , including the intergluteal and inframammary folds , neck , scalp , trunk , and arms . other features include scattered comedo - like lesions and pitted acneiform scars near the angles of the mouth . dyschromatosis universalis hereditaria ( duh ) is an autosomal dominant condition characterized by hyperpigmented macules admixed with hypopigmented lesions involving the trunk , extremities , and the face . herein we report three cases of ddd in a family with features overlapping with duh . a 65-year - old male ( index case ) presented with lesions over his face , trunk , and axillae since the age of 25 years . the lesions initially appeared over the face and later progressed to involve the axillae and trunk . he was accompanied by his two sons , 40 years ( case 2 ) and 28 years ( case 3 ) of age , who had similar lesions since 23 years of age . on examination , all the three cases had reticulate hyperpigmented macules over the neck , both axillae [ figure 1 ] and flexural aspect of both forearms . mottled hypopigmented macules were seen over the chest and abdomen [ figure 2 ] . multiple pitted scars and open comedones with few cysts were present over the back [ figure 4 ] , chest and abdomen . hyperpigmented macules over the axilla in case 1 hypopigmented macules over the chest and abdomen in case 1 pitted scars over the perinasal and perioral area in case 1 comedones over the back in case 1 a skin biopsy performed from the index case showed elongated branched pigmented rete ridges [ figure 5 ] . a diagnosis of ddd was arrived at based on the clinical and histopathologic findings , the atypical feature in these three cases being the presence of hypopigmented macules over the chest and abdomen . histopathologic examination of the skin biopsy performed from the index case showing elongated branched pigmented rete ridges . ddd is characterized by a triad of reticular pigmentation in the flexures , comedo - like lesions and pitted acneiform scars . the histology is diagnostic , with a distinct form of acanthosis , characterized by an irregular elongation of thin branching rete ridges , with concentration of melanin at the tips ( antler like appearance ) . follicular infundibulum may be involved and in some cases there is follicular plugging . in the present study , the association of ddd with reticulate acropigmentation of kitamura ( rapk ) has been earlier described in the literature . vasudevan et al . reported a patient having overlapping features of ddd , rapk , and reticulate acropigmentation of dohi ( rapd ) . there patient had acral pigmentation and palmar pits resembling rapk with flexural involvement resembling ddd . they proposed that all these entities may be a part of the single pigmentary disorder . in the present study , all three cases had all features of ddd . absence of palmar pits and acral pigmentation excluded coexistence of rapk ; however , the presence of hypopigmented macules admixed with hyperpigmented macules on the trunk suggested the coexistence of duh . wu et al . described a family with autosomal dominant inheritance of a skin disorder with clinical features of both ddd and duh and they coined the term generalized ddd . they believed that number of previously reported cases of ddd - rapk syndrome or ddd - duh overlap may be the cases of generalized ddd . most patients in this group have the characteristic histologic findings of epidermal atropy , elongated rete ridges , horn cyst formation , and hyperpigmented tips . the distribution may be reticulate in flexural areas ( classic ddd ) or generalized with hypopigmented papules ( generalized ddd ) . histopathology of hypopigmented area shows elongation of rete ridges , basal hypopigmentation with pigment in the tip of rete ridges . patients may or may not have atrophic brown macules on the back of hands and feet , palmar pits , and broken epidermal ridges , the entity known as rapk . clinically , both have hyperpigmented and hypopigmented lesions ; and histopathology of hyperpigmented lesions shows basal hyperpigmentation and pigment incontinence , whereas hypopigmented lesions shows basal hypopigmentation . although duh and dsh have similar histopathologic features , they differ from ddd group . in the present study , cases 1 , 2 , and 3 had lesions similar to the case described by wu et al . galli galli disease ( ggd ) is the name given to a rare form of acantholytic ddd . described a case of ggd in an indian family where 25 persons had ddd and they have been able to document a heterozygous nonsense mutation c.c10 t in exon 1 of the krt5 gene in four members of the family . all the cases described by them showed a mottled pigmentation comprising hypo- and hyperpigmented asymptomatic macules similar to our case . they pointed out that the hypopigmented lesions in the ddd spectrum has been underreported in india . a similar mutation has been described in chinese family with ddd with hypopigmented macules adjacent to the classic lesions showing histologic features of ddd . 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 .

dowling degos disease ( ddd ) is a rare autosomal dominant condition characterized by multiple , small , round pigmented macules usually arranged in reticular pattern , chiefly distributed in axillae and groins . here we are reporting three atypical cases of ddd in a family . they had hypopigmented macules with typical features of ddd indicating generalized ddd . histopathology confirmed the diagnosis . we present these three cases to stress the existence of generalized ddd phenotype in the indian population .

abdominal compartment syndrome ( acs ) is defined as a sustained intra - abdominal pressure ( iap ) associated with new organ dysfunction / failure . acs or intra - abdominal hypertension ( iah ) may occur after intra - abdominal events such as surgery , trauma and peritonitis . acs progression can be life threatening with multiple organ dysfunction in the respiratory , cardiac , renal and gastrointestinal systems . emergency laparotomy for abdominal decompression is often considered in patients with acs , , , , , , . we report the first known case of acs owing to simple elongated sigmoid colon , without volvulus or peritonitis . an 86-year - old man presented at our hospital for chronic constipation with elongated sigmoid colon . he did nt take any medicine that could slow up the bowel movements , as anti - depressive drugs or another . moreover , the frequency of his admission to hospital had increased up to once a month . at each admission , he was treated with colonoscopy to remove sigmoid colon gas , subsequently becoming symptom - free . the patient has developed several symptoms of dementia along with his aging , which have led to poor performance status . recently , on admission , he suffered from severe abdominal distension with hypotension , tachycardia and tachypnea twice a week . iap was measured using the trans - bladder method and showed elevated iap of 21 mmhg . abdominal x - ray showed a large quantity of sigmoid colon gas and elevated bilateral diaphragm ( fig . however , laboratory investigations were unremarkable for blood cell count , biochemical factors and blood gas . contrast - enhanced computerized tomography ( ct ) scan demonstrated that the sigmoid colon was only elongated at the extremities and dilated with the large quantity of gas , and apparent volvulus was not observed . meanwhile , the patient s inferior vena cava had collapsed because of the dilated colon ( fig . , we decided to decompress colon gas by colonoscopy , instead of performing emergency laparotomy . however , we decided to perform elective surgery the day after the interventional decompression by colonoscopy . on laparotomy , his sigmoid colon was approximately 100 cm in length , but his rectum was normal . the dilated sigmoid colon was successfully resected , and the subsequent reconstruction was done by functional end - to - end anastomosis ( fig . histological examination showed normal sigmoid ganglion cells , and the patient was diagnosed with simple elongated sigmoid colon without volvulus and idiopathic megacolon . an 86-year - old man presented at our hospital for chronic constipation with elongated sigmoid colon . he did nt take any medicine that could slow up the bowel movements , as anti - depressive drugs or another . moreover , the frequency of his admission to hospital had increased up to once a month . at each admission , he was treated with colonoscopy to remove sigmoid colon gas , subsequently becoming symptom - free . the patient has developed several symptoms of dementia along with his aging , which have led to poor performance status . recently , on admission , he suffered from severe abdominal distension with hypotension , tachycardia and tachypnea twice a week . iap was measured using the trans - bladder method and showed elevated iap of 21 mmhg . abdominal x - ray showed a large quantity of sigmoid colon gas and elevated bilateral diaphragm ( fig . however , laboratory investigations were unremarkable for blood cell count , biochemical factors and blood gas . contrast - enhanced computerized tomography ( ct ) scan demonstrated that the sigmoid colon was only elongated at the extremities and dilated with the large quantity of gas , and apparent volvulus was not observed . meanwhile , the patient s inferior vena cava had collapsed because of the dilated colon ( fig . , we decided to decompress colon gas by colonoscopy , instead of performing emergency laparotomy . however , we decided to perform elective surgery the day after the interventional decompression by colonoscopy . on laparotomy , his sigmoid colon was approximately 100 cm in length , but his rectum was normal . the dilated sigmoid colon was successfully resected , and the subsequent reconstruction was done by functional end - to - end anastomosis ( fig . histological examination showed normal sigmoid ganglion cells , and the patient was diagnosed with simple elongated sigmoid colon without volvulus and idiopathic megacolon . acs was first reported by kron et al . in 1984 , who described its pathophysiology resulting from iah secondary to aortic aneurysm surgery . acs is defined as a sustained iap > 20 mmhg that is associated with new organ dysfunction / failure . elevated iap possibly induces adverse effects on pulmonary , cardiovascular , renal , splanchnic , musculoskeletal and central nervous system physiology , , , , , , . for example , iap causes a direct reduction in cardiac output resulting from the decreased cardiac venous return from the inferior vena cava and portal vein . the elevated diaphragm causes reductions in total lung capacity , functional residual capacity and residual volume . the disordered physiology following iah may result in a clinical syndrome with significant morbidity and mortality . the correct diagnosis of acs onset and timely appropriate intervention are required for optimal outcome . although the exact incidence of acs has not been established , studies have increasingly examined acs in certain groups . abdominal surgery , major trauma , volvulus , acute pancreatitis , liver dysfunction , sepsis , shock , obesity and age have all been reported as risk factors for acs , . in the present case , we diagnosed acs originating from a simple elongated sigmoid colon , because we observed abdominal distension with elevated iap , hypotension , tachycardia and tachypnea . the mental and physical functions of this patient were gradually declining owing to his progressive dementia . iah or acs may become more common with a growing aging population , particularly in japan . several previous reports have demonstrated iah and acs triggered by sigmoid volvulus or peritonitis . however , ours is the first case of acs based on simple elongated sigmoid colon . this report may serve as a warning case for surgeons that acs may occur even in simple elongated colon without volvulus or peritonitis . we described for the first time a case of acs originating from an elongated sigmoid colon in an elderly man . considering the growing aging population and higher likelihood of acs , surgeons will need to precisely diagnose acs and determine the optimal time for surgery . written informed consent was obtained from the patient for publication of this case report and accompanying images . a copy of the written consent is available for review by the editor - in - chief of this journal on request . yk has been involved in drafting of the manuscript and revised it critically for important intellectual content .

highlightsabdominal compartment syndrome may result in a clinical syndrome with significant morbidity and mortality.intra-abdominal events , obesity and age have been reported as risk factors.we report a first case of abdominal compartment syndrome in an elderly patient without other risk factors.abdominal compartment syndrome may become more common with a growing aging population.surgeons will need to precisely diagnose and determine the optimal time for surgery .

peripheral vascular disease of upper extremity seldom leads to tissue loss because of the relatively low metabolic demand and extensive collaterals of upper limb . patients with end - stage renal failure ( esrf ) are more prone to finger gangrene , especially those with multilevel stenosis or calcification involving the forearm vessels , as shown in clinical and autopsy studies.1 this is also particularly important in patients with elbow - based arteriovenous fistula for hemodialysis , as there is a danger of arterial steal . other differential diagnoses included rheumatological diseases , raynaud s disease , frostbites , or distal emboli . many patients with finger gangrene presented to orthopedic surgeons for primary amputation , but there are more reports29 now of revascularization of forearm arteries to be a useful adjunct for finger salvage . we report a case of successful angioplasty of forearm arteries in a patient with esrf , and thereby saving her finger , with good clinical and functional outcome . a 51-year - old right - handed lady with esrf presented with a 2 months history of progressively worsening infected gangrene involving her right ring finger which developed after a nail clipping injury . past medical history was significant for 20 years of esrf due to unknown etiology , currently on hemodialysis via a right neck permcath catheter , hypertension , and percutaneous coronary angioplasty via a right femoral access . on presentation , her right hand was pale and there was infected gangrene of her right ring finger , which was edematous and inflammed and spreading to the proximal pharynx . her right radial and ulnar pulses were absent at the wrist , but her brachial pulse was palpable . duplex ultrasound ( philips iu22 ; philips healthcare solutions , bothell , wa , usa ) showed severe multilevel stenosis of the forearm radial artery and occlusion of the forearm ulnar artery . the ultrasound criteria of moderate stenosis ( 50% ) was at - stenosis and pre - stenosis velocity ratio of more than or equal to 2.0 , or a peak systolic velocity of more than or equal to 200 cm / s . criteria of severe stenosis ( 75% ) was velocity ratio of more than or equal to 4.0 , or peak systolic velocity of more than or equal to 400 cm / s . in view of the duplex findings , urgent revascularization of forearm arteries brachial access was established at the antecubital fossa , with antegrade insertion of a 4-french sheath ( cordis corporation , fremont , ca , usa ) . on - table angiogram showed multilevel mid to distal ulnar artery occlusion and severe mid to distal radial artery stenoses . all the lesions were passed intraluminally with 0.018/0.014 terumo guidewire ( terumo medical corporration , somerset , nj , usa ) and angioplastized with 2 mm 25 mm medtronic sprinter legend balloon ( medtronic inc , fridley , mn , usa ) . completion angiogram showed satisfactory radiological result with some ulnar artery spasm which improved with 50 g of intra - arterial glyceryl trinitrate ( ucb pharma limited , berkshire , uk ) ( figure 1 ) . the wound swab grew citrobacter , and her finger infection was controlled by giving unasyn ( ampicillin and sulbactam ) for 2 weeks . clopidogrel and aspirin were given for 3 months and then was advised to continue clopidogrel lifelong . apart from clinical and duplex ultrasound outcomes , digital pressure was also a good indicator of revascularization , although this was not measured in our patient . angioplasty of forearm arteries is a standard treatment in our territory and this paper does not require the institutional review board of the university of hong kong / hospital authority hong kong west cluster to review . digital gangrene due to chronic atherosclerotic disease is rare due to low metabolic demand and extensive collateral circulation,10 except in patients with esrf , rheumatological diseases , raynaud s disease , frostbites , or distal emboli . patients with renal failure are more prone to develop occlusive disease of the upper limb . acceleration of atherosclerosis , upregulation of markers of inflammation and oxidative stress , and more severe calcification of plaques and the arterial media among esrf patients were possible mechanisms.1 steal from ipsilateral concomitant arteriovenous fistulae also contributed to some cases of hand ischemia.11 there is a relative paucity of literature on endovascular treatment of upper extremity arterial disease compared to lower limb , with medical therapy involving antiplatelet agents and lifestyle and risk factor modifications being the mainstay of treatment.12 many patients with septic digital gangrene had primary amputation by the orthopedic services , without preoperative careful vascular assessment or even consideration for revascularization . chang et al13 reported 18 forearm bypass procedures for digital ulceration or gangrene with patency rate of 88.9% at mean follow - up of 18 months . systematic review of 16 studies reporting outcomes of distal upper extremity bypass surgery showed an overall patency rate of 87% at an average follow - up of 34 months among 152 bypass grafts performed.14 angioplasty is an alternative option given its minimal invasive nature , but there are only a handful of cases described in contemporary literature . search keywords comprised angioplasty , percutaneous angioplasty , revascularization and finger gangrene , ischemia , tissue loss . all studies using angioplasty as a treatment for finger gangrene due to various etiologies are included and tabulated in table 1 . report on long - term efficacy of more than 1 year , however , was lacking . the aim of the treatment in our patient was infection control and the gangrenous fingertip was then allowed to autoamputate . with improvements in endovascular technology and wide availability of low - profile small balloons , angioplasty of the forearm arteries should be considered for finger salvage in patients with esrf . this report emphasized the importance of specialist vascular care with a combination of expert care and angioplasty of forearm arteries , even in delayed setting , with successful salvage of the patient s finger .

due to the relatively low metabolic demand and extensive collaterals of the upper limb , peripheral arterial disease seldom leads to tissue loss , except in patients with end - stage renal failure ( esrf ) , rheumatologic diseases , raynaud s disease , frostbites , or distal emboli . we report a case of a 51-year - old lady with esrf who presented to our tertiary referral vascular center with infected gangrene of her right ring finger . duplex ultrasound showed that her forearm arteries were severely diseased . digital subtraction angiogram showed severe multilevel stenoses / occlusions in her forearm radial and ulnar arteries . these lesions were successfully angioplastized with 2 mm 25 mm angioplasty balloon . completion angiogram showed good radiological results with some post - dilatation spasm which improved with intra - arterial glyceryl trinitrate . the sepsis improved after revascularization , and the distal phalanx was allowed to self - demarcate with dressings and autoamputate with good clinical results . our case illustrated that even in delayed setting , patients could still benefit from specialist vascular care with a combination of expert care and angioplasty of forearm arteries , with successful salvage of her finger .

phakomatosis pigmentovascularis ( ppv ) is a rare condition first described by ota et al . , in 1947 . there is no sex predilection , but the japanese are found to be affected more . though four main types have been described , a fifth type with cutis marmorata and aberrant mongolian blue spot have also been added to the list . a 13-year - old female presented with port - wine stain of left side of the body including face along the distribution of 5 cranial nerve , upper limb , trunk , and lower limb [ figure 1 ] . additionally , this lesion was present on right side of the back as well . there was associated hypertrophy of the left lower limb with a visible vessel along the lateral aspect of the leg [ figure 2 ] . ophthalmological examination showed raised intraocular tension in left eye but the right eye was normal . the color doppler study of left lower limb showed absence of great and short saphenous vein . an abnormal dilated vein on lateral aspect of leg and thigh was present which was seen to drain into superficial circumflex iliac vein [ figure 4 ] . non - contrast computed tomography ( ct ) scan of brain showed focal parenchymal atrophy in the left parietal lobe region with foci of gyral calcification . widespread port - wine stain involving left side of face and chest engorged vein over lateral aspect of left leg along with hypertrophy of lower limb bluish discoloration of sclera of left eye color doppler picture showing anomalous draining vein on lateral aspect of left leg phakomatosis is a developmental abnormality simultaneously involving eye , central nervous system , and skin . happle and steijen proposed the genetic concept of twin spotting phenomenon to explain its etiology . ppv were classified into four types namely ; type i : nevus flammeus and epidermal nevus , type ii : nevus flammeus , mongolian spots , nevus anemicus , type iii : nevus flammeus , nevus spilus , nevus anemicus , type iv : nevus flammeus , mongolian spots , nevus spilus , nevus anemicus . a , cutaneous involvement only ; and subtype b , both cutaneous and systemic involvement . ppv type i , iii , and iv have very rarely been reported in literature . systemic syndromes which may be associated with it are sturge - weber syndrome , nevus of ota , and klippel - trenaunay syndrome . our patient had port - wine stain and nevus of ota and also had systemic involvement which tilted the diagnosis in favor of ppv subtype sturge - weber syndrome is defined as a facial port - wine stain with associated ipsilateral vascular malformation of leptomeninges and eye . brain changes show increased vascularity of leptomeninges which is usually unilateral and long - term pathological changes show calcification and atrophy . here our patient 's ct scan changes showed long - term changes like atrophy and calcification of parietal lobe of brain . eye changes in the form of glaucoma and buphthalmos may occur . though definition of klippel - trenaunay syndrome include cutaneous capillary malformation of a limb and soft tissue swelling with or without bone hypertrophy , original description indicate that varicosities of vein on affected limb is the usual finding and lateral venous anomaly is the most common abnormality . clinical picture showing widespread port - wine stain and hypertrophy of lower limb along with abnormal color doppler finding , suggests the diagnosis of klippel - trenaunay syndrome . thus , our patient fulfilled all the criteria for the diagnosis of ppv along with sturge - weber syndrome and klippel - trenaunay syndrome . few cases of ppv along with sturge - weber syndrome have been reported in literature , but ppv associated with both sturge - weber syndrome and klippel - trenaunay syndrome has been reported very rarely . in our case report we are presenting phakomatosis pigmentovascularis with sturge - weber syndrome and klippel - trenaunay syndrome which is so far very rare .

phakomatosis pigmentovascularis ( ppv ) is a rare cutaneous disorder characterized by combination of capillary malformation and other pigmented naevi . four types and two subtypes have been described where subtype a present only with cutaneous form and subtype b also with systemic association like in sturge - weber syndrome or klippel - trenaunay syndrome . hereby , we report a case where our patient presented with port - wine stain , nevus of ota , sturge - weber syndrome , and klippel - trenaunay syndrome ; which has made it a rare combination .

pseudosarcomatous fibromyxoid tumor of the prostate ( pfmt ) first reported by hafiz et al . in 1984 , is a benign fibroproliferative process that histologically resembles sarcoma . although these benign fibroproliferative processes of the prostate are unusual , it is important to recognize these lesions in order to avoid unnecessary radical procedures . also the pathologist should use special immunohistochemical stains to verify that it is a benign fibroproliferative process . a 70-year - old man has nearly a 2-year history of lower urinary tract symptoms ( luts ) such as nocturia , urgency and difficulty in voiding . due to a high level of prostate specific antigen ( psa ) , prostate biopsy was performed in another urology department . the psa level was 5.02 ng / ml . the measurement of the prostate volume with pelvic ultrasound was nearly 200 gr . cystoscopy revealed a normal anterior urethra with a large protruding prostatic mass and normal bladder . based on these findings , open adenomectomy ( suprapubic prostatectomy ) was performed nearly 2 years ago . the patient has been followed - up for two years and has no urinary symptoms in this period . small gland proliferations was observed in the limited periphery place and in this place the reaction with hmw ck and p63 antibodies was seen in the basal cells ( fig . 3 ) . staining in the spindle cells was negative for vimentin and cd 34 . the overall clinical and pathological features are consistent with pseudosarcomatous fibromyxoid tumor of the prostate . staining for smooth muscle actin ( original magnification x10 ) . staining for p63 in the basal cell ( original magnification x10 ) . the pseudosarcomatous fibromyxoid tumor of the prostate ( pfmt ) is a rare lesion , which , despite its resemblance to sarcomas , follows a benign course without the need to perform radical retropubic prostatectomy . another lesion that has a somewhat similar histological appearance to pfmt is the postoperative spindle cell nodule ( poscn ) , which was first described by proppe et al . . differentiation between poscn and pfmt is based on the few mitoses in the latter and the history of operative trauma in the former . pfmt is usually characterized by scattered stellate and spindle cells in an edematous , myxoid stroma with large number of capillaries and inflammatory cells . the nuclei of the stellate and spindle cells are hyperchromatic and pleomorphic ; few , if any mitoses are seen and none are atypical . despite the rarity of pfmt , it is important for urologists and pathologists to recognize and be aware of its benign course in order to avoid unnecessary radical procedures .

we report a rare case of inflammatory pseudotumor of the prostate revealed on suprapubic prostatectomy . a 70-year - old man presented with nocturia , urgency and difficulty in voiding . the histopathological examination of the suprapubic adenomectomy specimen reported fibromyxoid pseudosarcomatous tumor ( inflammatory prostatic pseudotumor ) . it is important to take this benign lesion under consideration to avoid unnecessary aggressive radical complementary treatments .

megalourethra is a rare congenital mesenchymal anomaly of the male anterior urethra characterized by a nonobstructive dilatation of the penile urethra . nesbitt in 1955 defined it as a congenital dilatation of penile urethra without distal obstruction . dorairajan classified congenital megalourethra into two types based on findings of urethrography . the more common scaphoid type with a deficiency of the corpus spongiosum is seen as bulging of ventral urethra . the fusifrom type with deficiency of both corpus spongiosum and cavernosum is seen as circumferential expansion of urethra . a 6-month - old male child presented with dribbling of urine with a noticeable swelling of undersurface of penis which increased during the act of micturition . on examination a scaphoid swelling of size 4 3 cm was seen on the ventral aspect of shaft of penis which ballooned out during micturition . ultrasound of abdomen and pelvis showed normal kidneys and bladder with no post void residue . retrograde urethrogram showed scaphoid dilatation of ventral aspect of distal and mid penile urethra confirming the diagnosis of congenital megalourethra [ figure 1b ] . cystourethroscopy showed wide saccular dilatation of penile urethra about 1 cm from external urethral meatus . retrograde urethrogram showing ventral saccular dilatation of urethra a reduction urethroplasty was done through circumcoronal penile incision . after degloving the penis scaphoid dilatation of ventral aspect of penile urethra was noted [ figure 2 ] . the dilated urethra was incised in ventral midline and the redundant urethra with deficient corpus spongiosum was excised and the urethra was reconstructed over 6 fr infant feeding tube using 5 - 0 pds suture . postoperative recovery was uneventful . on removal of the catheter after 2 weeks child voided normally without any penile swelling . regular follow - up at 1 year showed no recurrence of penile swelling with normal voiding pattern and sonologically normal bladder and upper tracts . the most commonly held theories propose a defect in the migration , differentiation , or development of the mesenchymal tissues of the phallus . another assumption is that delayed or deficient canalization of the glandular urethra may be associated with maldevelopment of the corpus spongiosum and corpora cavernosae . due to the poor development of erectile tissue which normally provides support two types of megalourethra are described depending upon the extent of associated embryonic maldevelopment of erectile tissue . jones et al . , in a review identified associated anomalies in 66 of 78 reported cases of megalourethra . in this review , associated congenital anomalies are seen in 80% of scaphoid type and 100% of fusiform type . the commonly described genitourinary anomalies include renal dysplasia - hypoplasia , hydronephrosis , hydroureter , vesicoureteric reflux , prune - belly syndrome , urethral duplication , megacystis , hypospadias , posterior urethral valves , and undescended testes . other system anomalies including vater ( vertebral , anal atresia , trachea - esophageal fistula , and renal anomalies ) and vacteryl ( vertebral , anal atresia , cardiac , trachea - esophageal fistula , renal , and limb deformities ) are described . in view of associated anomalies , the workup of megalourethra should include renal function tests and imaging of upper and lower urinary tracts urethral anomalies mimicking megalourethra like urethral atresia , a web , duplication , and a diverticulum are considered in the differential diagnosis . prenatally diagnosed megalourethra has to be thoroughly evaluated for associated congenital anomalies and followed up serially by ultrasound for amniotic fluid index , urinary bladder volume , and upper tract dilatation . termination of pregnancy may be advised in cases with other severe congenital anomalies and renal impairment . in some milder forms of megalourethra spontaneous resolution has been observed in fetuses at 19 - 34 weeks gestation . the treatment of megalourethra may be one stage or two stage urethroplasty depending on the age of presentation and general condition of the patient . for scaphoid type , nesbitt described a longitudinal reduction urethroplasty . heaton and colleagues described a technique of urethral plication for some cases of scaphoid megalourethra . the management of fusiform type is complicated ranging from sex reassignment to major phallic reconstruction . long - term follow - up is required in these patients to see for the erectile function and fertility potential . management has to be individualized depending on type and the presence of associated congenital anomalies . in isolated scaphoid type of megalourethra reduction

megalourethra is a rare congenital disorder of anterior urethra and erectile tissue of penis . it mainly appears in two types a milder scaphoid type and severe fusiform type . fusiform type is commonly associated with congenital anomalies of various systems of the body . isolated megalourethra without other congenital anomalies is extremely rare . we report one such case which was detected postnatally and successfully treated by reduction urethroplasty .

reactive arthritis or fiessinger - leroy disease is a genetically determined disease characterized by the triad of urethritis or cervicitis , conjunctivitis , and arthritis , which closely follows lower urogenital or enteric infection . mucocutaneous findings such as circinate balanitis ( 3040% ) , keratoderma blenorrhagicum ( 15% ) , oral ulcers ( 17% ) and dystrophy of nails ( 2030% ) are also associated with reactive arthritis . our case presented with circinate balanitis as the only initial clinical finding , which otherwise is the most common finding associated with reactive arthritis , but is rarely seen as a preceding finding . a 22-year - old married male presented with asymptomatic superficial lesions present over the glans and under surface of prepuce since 1-year . he was treated elsewhere with oral antibiotics and topical antifungal preparations assuming it to be an infection , but without any relief . patient also had mild lower back pain with morning stiffness and left knee joint pain since 6 months . examination revealed multiple well - defined superficial erosions of size 0.52.5 cm , round to oval in shape with irregular margins , which coalesced at places to form circinate pattern over the glans and under surface of prepuce [ figures 1 and 2 ] . from the above findings , circinate lesions present over glans before treatment lesions present on under surface of prepuce before treatment laboratory investigations showed mildly raised total leukocyte count 11,700/mm ( normal 400011000 ) . viral markers such as hepatitis b surface antigen , antihepatitis c antibodies , human immune deficiency virus ( hiv ) antigen , and venereal disease research laboratory tests were nonreactive . c - reactive protein ( crp ) ( 7.31 mg / dl , normal < 0.6 mg / dl ) and human leukocyte antigen ( hla ) b-27 were positive . radiologically magnetic resonance imaging lumbosacral spine and sacroiliac joints showed straightening of the lumbar spine and right sided sacroiliitis . histopathology of lesions from the glans showed parakaratosis containing neutrophils forming intracorneal and superficial epidermal pustules resembling spongiform pustules . various psoriasiform changes seen on histopathological examination based on the above clinical and histopathological findings , diagnosis of reactive arthritis was made . patient was treated for circinate balanitis with pimecrolimus 1% cream twice daily for 3 weeks . lesions cleared completely , and no recurrence was seen subsequently [ figures 4 and 5 ] . patient was referred to rheumatologist for management of arthritis . post - treatment complete resolution of lesions from glans post - treatment complete resolution of lesions from under surface of prepuce reactive arthritis was first described by hans conrad julius reiter , a german physician during thefirst world war in 1916 . in the same year , fiessinger and leroy published similar finding as oculo - urethro - synovial syndrome . although it is a disease of young males with hla - b27 associated with 80% of cases , other hla types that are linked with hla - b27 and other age groups are not excluded . caucasians are the most commonly affected , probably because of higher prevalence of hla - b27 in this population group . it occurs in response to enteric infections such as salmonella , shigella , yersinia , campylobacter or urethral infection from chlamydia trachomatis . it has been proposed that damaged exogenous pathogen - associated molecular patterns , derived from microbes , can disseminate upwards via the pelvic and spinal lymphatic pathways and activate toll - like receptors ( tlrs ) . their activation triggers signaling pathways that result in the expression of immune response genes and cytokine production . however , recent human data suggest that tlr-2 , not tlr-4 , is important in determining reactive arthritis susceptibility after salmonella infection other rare factors , which can induce the disease are immunotherapy with bacillus calmette - gurin and interferon , or following hepatitis b vaccination . one of the various treatment modalities tried for mucosal lesions include use of topical steroids like hydrocortisone or triamcinolone . a combination of keratolytic agents likes 10% salicylic acid ointment with hydrocortisone 2.5% cream , and oral aspirin has also been reported to clear circinate balanitis . topical 0.1% tacrolimus or pimecrolimus 1% cream has been used in refractory case with variable success [ table 1 ] . various studies showing topical calcineurin inhibitors as treatment modalities for balanitis the diagnosis in our patient rested on a constellation of signs and symptoms suggestive of reactive arthritis which included diarrhea , arthritis and circinate balanitis . however , the above mentioned clinical findings along with hla - b27 and crp positivity favored diagnosis of reactive arthritis . the interesting finding in our patient was the presence of circinate balanitis as the preceding feature , which has been rarely reported to the best of our knowledge . this case is being reported for its rarity in which circinate balanitis is the only presenting feature of reiter 's disease , which was successfully treated with topical 1% pimecrolimus cream . however , further clinical trials and research is needed to study underlying mechanism of action of topical pimecrolimus in its management .

circinate balanitis , although a common manifestation of reactive arthritis , is usually an associated finding present along with the triad of arthritis , conjunctivitis , and urethritis . it is rarely seen as the only preceding manifestation of reactive arthritis . we hereby report a case of circinate balanitis as alone preceding presentation of reactive arthritis that was successfully treated with topical pimecrolimus 1% cream .

a 55-year - old male patient developed sudden loss of vision in the right eye following sirsasana with wall support ( 2 minutes ) without supervision . the patient had suffered from pulmonary thromboembolism 5 years back and was receiving warfarin prophylaxis . patient was practicing yoga since past 2 years for 45 minutes , 34 times / week . on examination , best - corrected visual acuity ( bcva ) was finger counting 1 m in the right and 20/20 in the left eye . fundus examination showed disc edema , scattered superficial hemorrhages , and superficial whitening in macular area in the right eye [ fig . 1 ] , whereas the left eye fundus was unremarkable . 2 ] confirmed findings of ischemic crvo with cilioretinal artery occlusion ( red free photos showed retinal whitening along the course of cilioretinal artery in the maculopapillary region and non - filling of the cilioretinal artery in the early phase of angiogram ) all systemic investigations ( hemoglobin , erythrocyte sedimentation rate , lipid profile , blood sugar , serum homocysteine levels ) were normal , and international normalization ratio ( inr ) was 2.0 . over 6 months follow - up , the patient developed neovascularization of the iris and received pan - retinal laser photocoagulation with no improvement in vision . red - free photograph showing disc edema , scattered superficial hemorrhages and superficial whitening in the macular area in the right eye fundus fluorescein angiography photo : venous phase shows altered vascular caliber , blocked fluorescence at the disc due to hemorrhages , and macular ischemia this is an advanced pose and should be attempted after practicing forward and backward bends ( asanas ) , under the supervision of a qualified yoga instructor . sirsasana is known to cause elevation of iop , which reverts to normal after cessation of the same.[2 ] a sudden variation in iop has decompression effect on the eye , as after antiglaucoma surgery , causing decompression retinopathy . the practice of sirsasana is reported to increase the flow of blood to the brain , improving memory and other intellectual functions . although , increased sympathetic vasomotor and sudomotor tone is known to occur after sirsasana , its effect on ocular hemodynamics has not been studied . instructors suggest avoiding sirsasana in patients with hypertension , congestive heart failure , berry aneurysms , and those above 50 years of age . however , there is no existing study to support the above . inr was established by the world health organization ( who ) and the international committee on thrombosis and hemostasis for reporting the results of blood coagulation ( clotting ) tests . a person taking warfarin might optimally maintain a prothrombin time of 2 - 3 inr . this international standardization permits the patient receiving warfarin to travel and still obtain comparable test results . it is known that patients receiving warfarin prophylaxis may not to be protected against a crvo , as in our patient ( inr-2 ) . hence , both the above , in a case of pulmonary embolism , would have predisposed our patient to crvo . we conclude that predisposed subjects such as those having a previous thromboembolic phenomenon and age above 50 years ( as in our patient ) can be warned against doing sirsasana or head down postures in yoga . because of the increased popularity of yoga , a long - term study to know its impact on ocular hemodynamics is needed .

we report a case of central retinal vein occlusion ( crvo ) following sirsasana , a head - down postural yoga . a 55-year - old male patient presented to us , with sudden - onset loss of vision following sirsasana , in the right eye . the patient had suffered from pulmonary thromboembolism 5 years earlier and was receiving warfarin prophylaxis . over 6 months of follow - up , the patient developed neovascularization of the iris and was subjected to panretinal laser with no improvement in visual acuity . sirsasana could be an important risk factor for crvo especially in predisposed patients .

a 91-year - old gentleman presented with incidental finding of left neck of femur fracture on a routine radiograph . a quick review of the patient notes revealed a fall , with a lengthy hospital admission , approximately 1 year previously . despite his initial inability to weight - bear and protracted slow progress with physiotherapy no further imaging of the hip was obtained beyond an initial , negative pelvic radiograph . doctors must be bold in questioning a radiograph that does not fit with the clinical picture . clinical suspicion of neck of femur fracture in the face of a negative radiograph necessitates further imaging to obtain a definitive answer . the more experienced doctor knows that some cases are not clear - cut , as between 2% and 10% are occult on conventional radiography [ 1 - 4 ] . in cases , where there is clinical suspicion of a neck of femur fracture , but imaging has been negative or equivocal , further imaging ( e.g. magnetic resonance imaging / computed tomography ) is required . hip fractures , like everything else , can be missed and this can have serious implications for patients . a delay of over 48 h from admission to surgery doubles the risk of death within 1 year postoperatively . following a routine radiograph performed by his general practitioner a 91-year - old gentleman was referred to accident and emergency with an incidental finding of fractured left neck of femur ( fig . 1 ) . his general practitioner had requested a pelvic radiograph to further investigate the cause of this gentleman s left knee pain . despite no falls within the previous year the radiograph demonstrated the well - corticated edges of an old fracture , and the patient was able to mobilize using his frame . routine pelvic radiograph of a 91-year - old man requested by his general practitioner to further investigate the cause for his left knee pain old , left sided , displaced , intracapsular had as neck of femur fracture with well - corticated edges . review of past incidents to find a cause for this neck of femur fracture quickly illuminated a possible event . approximately 1 year prior , the patient had been admitted to hospital following a mechanical fall at home , after which he was unable to weight - bear . clinical examination had demonstrated none of the salient features of a neck of femur fracture or had initial radiographs of the pelvis exposed a fracture ( fig . radiographs of the knee demonstrated no abnormality , yet the gentleman was unable to walk and was admitted . lateral radiograph of the left hip from the same 91-year - old gentleman taken 1 year previously , following a fall . anteroposterior radiograph of the left hip from the same 91-year - old gentleman taken 1 year previously , following a fall . degenerative changes of the left hip joint are demonstrated although no obvious fracture is evident . magnetic resonance imaging of his left knee was requested to exclude the possibility of soft tissue injury , which demonstrated a tear of the medial meniscus . felt to be the cause of his delayed progress , further physiotherapy was encouraged and eventually this gentleman managed to mobilize independently with a frame . hip fractures are often simple to diagnose clinically and radiologically , with initial imaging sensitivity estimated as 90 - 98% [ 1 - 4 ] . approximately 75,000 femoral necks are fractured in the uk per annum and this is projected to rise further . if initial radiographs alone are used to exclude a neck of femur fracture then this poses the alarming possibility that up to 7,500 ( of the known ) neck of femur fractures could have been missed . delays in diagnosis increase the risk of avascular necrosis , arthroplasty , nonunion , thromboembolic events , and mortality [ 9 , 10 ] . this case and figures above demonstrates the need for clinicians to have a high index of suspicion when evaluating elderly patients following a fall . if there is clinical suspicion of a neck of femur fracture , then a negative radiograph is not enough and further imaging is required ; magnetic resonance imaging - or computed tomography , if unavailable or contraindicated . the sheer prevalence of neck of femur fractures , combined with the knowledge that up to 10% are occult on radiograph [ 1 , 2 , 3 , 4 ] necessitates clinicians to have a high index of suspicion of occult neck of femur fractures in elderly patients following a fall . although occult fractures are well documented in the literature , hilton s law , as well as referred pain from the joint above or below , are reasons for the threshold for further imaging modalities to be lowered . the aim of this case report is to raise awareness , to encourage doctors to be bold in questioning a radiograph that does not fit with the clinical picture and to increase requests for further imaging to obtain a definitive answer ; the numbers and guidelines are on your side . to encourage clinicians to question a radiograph that does not fit with the clinical findings of a neck of femur fracture and consider further imaging to obtain a more definitive picture

introduction : on rare occasions , hip fractures can be missed . approximately 75,000 neck of femur fractures occur in the united kingdom per annum . up to 10% of hip fractures are occult on plain radiograph . this case demonstrates a usual presentation of an incidental neck of femur fracture , which had been missed 1 year prior.case report : a 91-year - old gentleman presented with incidental finding of left neck of femur fracture on a routine radiograph . a quick review of the patient notes revealed a fall , with a lengthy hospital admission , approximately 1 year previously . despite his initial inability to weight - bear and protracted slow progress with physiotherapy no further imaging of the hip was obtained beyond an initial , negative pelvic radiograph.conclusion:doctors must be bold in questioning a radiograph that does not fit with the clinical picture . clinical suspicion of neck of femur fracture in the face of a negative radiograph necessitates further imaging to obtain a definitive answer .

the name granulosa cell tumor ( gct ) was proposed by von werdt in 1914 . to have primarily originated retroperitoneal gct is even rarer . in medical literature in english from 1938 till today , only 12 cases have been reported . although , the histology of ovarian adult - type granulosa cell tumors ( agcts ) is well - documented , it is rarely encountered in cytological specimens and the identification of tumor cells is very difficult . here . per abdominal examination revealed a well - defined mass palpable in the left hypochondriac and lumbar regions . computerized tomography ( ct ) of the abdomen revealed a solid heterogeneous mass lesion measuring 11.2 cm 8 cm 12 cm , consistent with retroperitoneal hematoma [ figure 1a ] . ultrasonography ( usg)-guided aspiration of the mass was performed and the smears were hypercellular with small , overlapping cell clusters and cells in a microfollicular pattern [ figure 1b ] . on enquiry , she gave a history of hysterectomy with bilateral salpingo - oophorectomy 22 years ago for uterine leiomyoma . with these cytological features and the clinical history , a diagnosis of extraovarian agct was proposed . we received multiple dark brown and necrotic tissue fragments together measuring 10 cm 8 cm 3 cm . the cut section of all fragments showed gray white , yellowish , and hemorrhagic areas . ( a ) ct showing a solid heterogeneous mass in the left anterior pararenal space compressing left kidney ( b ) cytology smear showing cells in microfollicular pattern ( papanicolaou , 400 ) ( c ) cells in sheets and microfollicular pattern ( h and e , 400 ) ( d ) tumor cells showing strong positivity for inhibin ( inhibin , 400 ) histologically the neoplasm was composed of small round to oval neoplastic cells with predominantly microfollicular [ figure 1c ] , diffuse , and watered - silk patterns . immunohistochemistry ( ihc ) for inhibin and epithelial membrane antigen ( ema ) was done . the tumor was positive for inhibin [ figure 1d ] and negative for ema . with the typical histopathological features and ihc findings , a diagnosis of primary agct of retroperitoneum was confirmed . there are two subtypes : adult and juvenile , based on different clinical and histological features . agct of the ovary is oftentimes a hormonally active stromal cell neoplasm that is distinguished by its ability to express aromatase and to secrete sex steroids such as estrogen . the histogenetic origin of extraovarian agct is thought to be from the ectopic gonadal stromal tissue from the mesonephros . the cytological smears of an agct are usually highly cellular of both single cells , syncytial aggregates , and follicular pattern . the cells are monomorphic with scanty to moderate amount of pale cytoplasm and monomorphic round to oval nuclei having longitudinal grooves and granular chromatin . histologically , the tumor cells resemble normal granulosa cells with uniform , round or oval nuclei having finely granular chromatin and longitudinal nuclear grooves or folds . they show microfollicular , macrofollicular , trabecular , watered - silk , or diffuse patterns . extraovarian gct should be differentiated from other tumors such as small - cell carcinoma , undifferentiated carcinoma , carcinoids , and lymphoma . gct is positive for inhibin and calretinin and negative for ema whereas other tumors do not show positivity for inhibin and calretinin . the case of primary retroperitoneal gct is reported for its rarity after excluding previous ovarian origins and to describe its relevance to the histologic origin . due to their rarity , agcts present a diagnostic challenge for cytologic preparations . we believe that sensitive cytologic evaluation , histopathologic correlation , taking clinical history , and a positive immunohistochemical reaction with inhibin could help us in practice . surgery is the primary treatment for these tumors , however , long - term follow - up with history , clinical examination , and tumor markers are crucial for gcts , as later relapse is a common behavior for these unique tumors .

the most frequently occurring retroperitoneal tumors are those of the kidneys , adrenal glands , and the pancreas . a primary retroperitoneal tumor composed of granulosa cells and developing far away from the normal location of the ovary is less frequently observed . a 69-year - old female patient presented with abdominal discomfort . computerized tomography ( ct ) of the abdomen revealed a solid heterogeneous mass lesion measuring 11.2 cm 8 cm 12 cm consistent with retroperitoneal hematoma . ultrasonography ( usg)-guided aspiration smears revealed cytological features suggestive of adult - type granulosa cell tumor ( agct ) . as the patient had a history of hysterectomy with bilateral salpingo - oophorectomy 22 years ago for leiomyoma , a diagnosis of extraovarian agct was made . intraoperatively , the tumor was removed in piecemeal that showed yellowish areas with extensive necrosis and hemorrhage . histopathological examination of the excised mass and inhibin positivity confirmed the diagnosis . primary retroperitoneal extraovarian gct is a rare tumor with only 12 cases reported in medical literature in english .

a 71-year old caucasian male with dilative cardiomiopathy and irrelevant family history was admitted to our hospital for the evaluation of a suspected liver disease . laboratory findings revealed a slight anemia ( hemoglobin level of 12,5 g / dl , hematocrit of 38% , red blood cells count of 3,9 mil / mm ) . iu / l ) and elevated gammagluta - myltranspeptidase ( ggt=230 iu / l ) . ultrasound showed multiple small hypo- and hyperechoic lesion foci , some cystic lesions with comet - tail echoes , the biggest cyst measured 8 mm in the vii segment ( fig . ct scan revealed multiple small cystic lesions ; the largest hypodense nodule was in the vii hepatic segment with no peripheral or central enhancement ( fig . multiple small cystic lesions were detected with t1 hyposignal and t2 hypersignal , the largest being in segment vii ( fig . corroborating data from these imaging techniques with 6 month follow up , the final diagnosis was biliary hamartoma ( complex von meyenburg ) . biliary hamartoma is a benign congenital malformation of the biliary duct which was described for the first time in 1918 by von mayenburg , therefore it is also called von mayenburg complex ( vmcs ) . although jaundice and portal hypertension may arise as a result of mass effect , patients are usually asymptomatic . the biliary hamartomas may be single or multiple , with size ranging between 1 and 15 mm . due to the small size of the lesions , histologically , it consists of disorganized and dilated bile ducts and ductules surrounded by fibrous stroma . the multiple comet - tail sign is thought to be the specific us finding of vmcs ( 6 ) . it has been suggested that lesional echogenicity may depends on the number and size of the dilated bile ducts and on the degree of fibrosis . on contrast enhanced ct , biliary hamartomas are usualy of low attenuation and may have irregular margins . the majority of cases reported suggest that vmc does not show contrast enhancement . on mri , vmcs are described as hypointense on t1 and hyperintense on t2 in comparison with surrounding liver parenchyma . although biliary hamartoma is a benign condition , there are some isolated reports of hepatic malignancies on a background of vmc , including hepatocellular carcinoma and cholangiocarcinoma . biliary hamartomas usually presents as multiple small nodules and despite the fact that they are rare , they may be confused with liver metastatic disease , microabscesses , diffuse primary hepatocellular carcinoma , biliary cysts or caroli s disease . in conclusion , associating different imaging modalities with the follow - up are very useful in the diagnosis of biliary hamartoma . a correct diagnosis is established when typical imaging findings are present , otherwise histological confirmation might be needed .

biliary hamartoma or von mayenburg complex ( vmcs ) is a rare benign congenital malformation of the biliary duct . patients are usually asymptomatic . hepatic lesions are incidentally discovered on ultrasonography ( us ) and subsequent radiological methods are necessary for confirmation . a correct diagnosis is established when typical imaging findings are present , otherwise histological confirmation might be needed .

, individuals can live for 80100 years , much longer than in past generations . according to the who , the proportion of people over 60 years of age is increasing quickly and is expected to exceed 2 billion people worldwide by the year 2050 . immune senescence comprises a set of changes occurring to the innate and adaptive immune responses that accompany human aging . age - associated immune senescence is a catch - all phrase that has been used to describe a plethora of changes to the immune system over the lifespan . immune aging is a complex process that comprises many reconstructions and regular changes rather than being a simple one - way reduction in all immune functions ; thus , all parts of the immune system in immune senescence are not affected equally . for instance , it has been observed that natural immunologic structures that are common to all living things are less affected than are acquired immune system structures . active participation of inflammation , which forms the most basic defense mechanism in the aging process , is also an indicator of this . the decline in immune function with age is unanimously recognized and supported by epidemiologic and clinical studies [ 46 ] . many studies have demonstrated that immune functions and cells in the immune system are affected by aging . some studies reported that differences in immune system due to aging vary between males and females . sex - related differences in immune system susceptibility have also been observed in several mouse models and may be related to differences in the expression patterns of immune response genes . understanding the basis of sex and age differences in immune response genes is important for developing new approaches to prevention , diagnosis , and treatment of diseases . our aim in this study was to investigate how lymphocyte subgroups in peripheral blood are affected by aging among males and females . study participants were 70 healthy individuals from 3 different age groups , observed from january 2010 to january 2012 . participants were divided into 3 different groups according age : group 1 ( n=20 ) was 2545 years old ( 10 males , 10 females ) , group 2 ( n=25 ) was 4565 years old ( 12 males , 13 females ) , and group 3 ( n=25 ) was older than 65 years old ( 13 males , 12 females ) . we determined the average levels of cd3 + , cd4 + , cd8 + , cd19 + , cd16+/cd56 + , cd3+/cd69 + , and cd19+/cd69 + by age group and sex . individuals who were smokers or who had a chronic disease were excluded from the study . collected data were statistically analyzed by kruskal - wallis - anova , with p<0.05 considered to be statistically significant . we found a significant reduction in the rate of cd3+t cells related with age , but no significant change in cd19 + b cell rates ( p<0.005 ) . another noteworthy result was that the level of cd8+t cells was lower in males compared to females and varied by age group ( p<0.005 ) . level of activated t and b cells did not differ by age group , but levels of activated b cells ( cd19+/cd69 + ) decreased with age in males ( p<0.005 ) ( figure 1 ) . projections indicate that by 2025 the world population over age 65 will be increasing 3.5 times as rapidly as the total population and the proportion of individuals age 60 years and older , which accounted for 10% of the world population in 2000 , will increase to approximately 22% of the world population by 2050 . aging is a highly complex and continuous process that affects almost all organ systems , causing molecular and physiological changes , both qualitatively and quantitatively . the aging of the immune system is a dynamic process that may at least partly reflect adaptation of the response to the evolving pathogen surroundings [ 16 ] . aging is increasingly recognized as being associated with a pro - inflammatory state that plays an important role in the development of chronic diseases . immune senescence comprises a set of changes occurring to the innate and adaptive immune responses that accompany human aging . these result in complex manifestations of still poorly defined deficiencies in the elderly population . in the evaluation of t and b cell activations , no change due to aging was observed in level of activated t cells . on the other hand , our results show that levels of activated t and b cells did not differ by age group . we found that that aging reduces activated b cell ( cd19+/cd69 + ) levels of males . males have shorter life expectancy than females due to the effects of aging making them more sensitive . the immune system in males , we observed the lower cd8+t cell rates of males compared to those of females , especially when compared by age . therefore , we especially wanted to observe the changes in peripheral blood lymphocytes of males and females who belong to different age groups . recent studies have revealed that changes in the number of lymphocytes and a decrease in cell activation and proliferation appear with aging . however , the difference between males and females in immune senescence is still a hotly debated topic because many different factors may be involved . differences between males and females in susceptibility to infection and differences in prevalence of autoimmune diseases by sex may help understand these differences in aging and health [ 25,12 ] . in general , it has been demonstrated in human and mouse studies that females produce stronger humoral and cellular immune responses against varied antigens than males do . this difference also plays a role in female allograph rejection , which has been demonstrated in mouse studies . for instance , it is reported that female estrogen stimulation , which leads telomerase induction by c - myc , has an anti - aging effect . another theory that has been put forward about this topic in recent years suggests that depending on the development needs mitochondria in female cells have a better adaptation than male cells have [ 1113 ] . the immune response to infections , immunizations , and tumors in the elderly is quite different from that in young people . our study shows that there may be differences between males and females in terms of immune senescence .

backgroundage - associated immune senescence is a catch - all phrase that has been used to describe a plethora of changes to the immune system across the lifespan . aging is associated with a decline in immune function . our aim in this study was to investigate how lymphocyte subgroups in peripheral blood are affected by aging among males and females.material/methodsstudy participants were 70 healthy individuals from 3 different age groups , observed from january 2010 to january 2012 . the average levels of cd3 + , cd4 + , cd8 + , cd19 + , cd16+/cd56 + , cd3+/cd69 + , and cd19+/cd69 + were determined for each group and compared in terms of age and sex.resultswe found significant reduction in the level of cd3+t cells related with age , but no significant changes in cd19 + b cell levels ( p<0.005 ) . aging significantly reduces activated b cell ( cd19+/cd69 + ) levels in males ( p<0.005).conclusionsour results show that there may be differences between males and females in terms of immune senescence .

1a ) could not tolerate rgp lens - wearing and did not want to have a corneal transplant or other corneal surgeries . the ucva was 0.04 and the bscva was 0.7 with -3.75 -3.00 155a . in the left eye , the ucva was 0.02 , and the bscva was 0.4 with -12.00 -3.50 30a . the corneal refractive power was 58.40 / 48.70 diopters 117a in the left eye ( fig . he was told that the maximal possible visual correction would be 0.4 or less because irregular astigmatism could not be corrected with toric icl implantation . with informed consent , toric icl implantation after surgery , the manifest refraction became -0.75 180a with a ucva of 0.5 and bscva of 0.7 at 1 month , which then shifted to + 2.25 -0.50 180a with a ucva of 0.4 and bscva of 0.7 at 9 months , and to -1.75 180a with a ucva of 0.2 and bscva of 0.5 at 20 months . the anterior chamber depth , from the endothelium to the icl , decreased from 2.21 mm at 1 month to 2.13 mm at 9 months follow - up , corresponding with mild corneal flattening and transient hyperopic changes ( fig . 1d ) , without any vault changes ( fig . 1e and 1f ) . at the last follow - up , 20 months after surgery , the icl vault was decreased , resulting in mild deepening of the acd from 2.13 mm to 2.17 mm , and the manifest refraction results finally stabilized at mild myopic astigmatism ( fig . the efficacy and safety index were 1.0 and 1.75 at 9 months and 0.5 and 1.25 at 20 months ( table 1 ) . toric icl has been used as an alternative for the correction of high myopic astigmatism in eyes with stable keratoconus , though irregular astigmatism is a concern due to its ability to interfere with visual rehabilitation . the two - step procedure of combining intrastromal corneal rings and phakic intraocular lens implantation may be another alternative treatment option for keratoconus with high myopic astigmatism . toric icl may have advantages in terms of expense , time , and being a one - step procedure . toric icl implantation can improve clinically useful vision even if perfect vision can not be achieved in patients with advanced stable keratoconus . in our case , the patient was satisfied with partial visual rehabilitation because his daily activities could be comfortably performed with his spectacles - corrected vision with his icl implants . there are still issues regarding toric icl target diopter , which may not be precisely calculated exactly in keratoconus and postoperative refractive changes due to keratoconus progression . we used the astigmatism decomposition method recommended by the manufacturer ( staar surgical ) to perform the toric icl power calculation . exact calculation of the target diopter , however , is more difficult in keratoconus than in other high myopic astigmatism conditions due to the difficulty of achieving a precise keratometric reading and manifest refraction . moshirfar et al . reported that phakic icl selection for emmetropia in keratoconus eyes with high myopic astigmatism may lead to hyperopia . our patient had a transient hyperopic shift that appeared to be associated with a suspicious progression of scarring that resulted in flattening of the cornea and subsequent shallowing of the anterior chamber depth . this hyperopic change resolved with decreasing the icl vault and the refraction stabilized by 20 months follow - up . despite transient refractive changes during the follow - up , the visual improvement with correction was maintained , and the patient was satisfied with the postoperative vision and the lack of postoperative complications . it is important that the patient is well informed before the surgery of possible visual fluctuations and only a partial restoration of vision . because toric icl aims to correct spherical and cylindrical errors , it can not correct a large amount of high order aberrations caused by irregular shapes of the cornea . after a toric icl implantation , our patient still had a similar amount of high order aberrations despite correcting the high myopic astigmatism . examples include patients with rgp intolerance , stable keratoconus , or fear of corneal surgery . to select suitable candidates , correction of high myopia and astigmatism with spectacles before the surgery should be tried in the clinic . recently , to reduce both irregular astigmatism and high myopia , cross - linking , or corneal ring implantation combined with toric icl , has been newly adapted for treating keratoconus . nevertheless , our case report suggests that toric icl implantation may be a possible alternative surgical option for the partial visual rehabilitation of high myopic astigmatism in keratoconus patients with rgp contact lens intolerance who do not want to have a corneal transplant or other corneal surgeries .

we report the case of a 37-year - old man with a high myopic keratoconus eye that was treated with a posterior chamber toric implantable collamer lens ( icl ) in korea . the patient had a history of contact lens intolerance and did not want to have a corneal transplantation . his uncorrected visual acuity ( ucva ) and best spectacle corrected visual acuity were 0.02 and 0.4 in the left eye , respectively . preoperatively , the manifest refraction was -12.0 -3.5 30a . postoperatively , the manifest refraction was -1.75 180a . ucva improved markedly after implantation . no intraoperative or postoperative complications were observed during 20 months of follow - up . toric icl implantation may be a possible alternative surgical option for the visual rehabilitation of high myopic astigmatism in keratoconus patients with rigid gas permeable contact lens intolerance and in patients who do not want to get a corneal transplant .

the increase in bacterial antibiotic resistance is a serious problem , increasing both in severity and importance . therefore , it is necessary to find means of limiting or even preventing the transmission of pathogens within medical facilities . surfaces in patients surroundings , including the door knob or handle , are a relevant transmission route for viruses and microorganisms , , , . hospital staff , visitors , cleaning services and others are a potential source of contamination for door handles . in particular , the nursing staff with a hand - disinfection rate of under 50% , can spread pathogens , as can other employees , 4.6% of whom have been found to be colonized with mrsa . one alternative to avoid this route of transmission is to equip doors with a foot - operated door opener rather than with a door handle . a device for opening the door with the foot was fitted flush into the door leaf . a pedal ( figure 1 ( fig . 1 ) ) is integrated into the device , which is built into the bottom of the door leaf . to open the door , this pedal is pulled out with the foot from the inside of the device , while the foot constantly remains on the surface of the pedal . after completely pulling the pedal out , the door can be pushed open or pulled open while keeping light pressure on the pedal with the foot . the surface design of the pedal provides the necessary grip of the shoe on the pedal during the movement to be executed . subsequently , the door will briefly remain in the open position before the door closer starts to pull the door back into its frame . using the foot pedal alone , this device makes it possible to open the door without manual operation . after opening of the door , there is ample time to pass through the open door . the foot - operated door opener is optionally equipped with a soft - closing mechanism . as a result , while opening the door with the foot door opener , the door latch ( figure 1 ( fig . 1 ) ) will be kept inside the door lock by a mechanism integrated into the foot door opener . only after complete closing of the door ( i.e. , the door is back in its frame ) is the door latch released by the mechanism : the door latch slowly and quietly returns to its normal position in the counterpart within the door frame . the function of the door handle and the door lock itself is not affected . in both cases , opening with the door handle or foot - operated door opener , the door latch is triggered , which means a change in the mechanics of the normal door handle with its lock is not necessary . only if the door lock itself is locked is it impossible to open the door with either the handle or the foot door opener . equipped with the soft closing mechanism , the noise generated by the door latch hitting the door frame is completely prevented , and the noise generated by the door latch quickly snapping back into its counterpart in the door frame is almost imperceptible . the foot door opener makes the door handle superfluous in most cases . in addition , the transport of items that must be carried with both hands , e.g. , trays , is facilitated . another means of opening doors without using the hands is provided by door handles designed to be operated with the forearm . however , this type of handle can still be operated by hand and is thus in danger of becoming contaminated . nevertheless , when used correctly , the forearm - operated door opener reduces the risk of cross - infection . where door handles are coated with oligodynamic effective metals such as silver and copper in nanocrystalline form , the antimicrobial action takes effect only many hours later , and a false sense of security is produced . based on unproven efficacy within a relevant exposure time , inactivation through protein loading , and the absence of evaluations on possible toxic risks , coating door handles with such compounds is not a reasonable alternative to the foot - operated door opener . if used correctly , the mechanical foot - operated door opener can completely avoid the transfer of pathogens . additional advantages include : no electrical energy is necessary for operationno follow - up costs arise , e.g. , from consumablesthe functionality of the existing door lock is not compromised , andnothing protrudes from the plane of the door leaf . no electrical energy is necessary for operation no follow - up costs arise , e.g. , from consumables the functionality of the existing door lock is not compromised , and nothing protrudes from the plane of the door leaf . with the foot - operated door opener described here instead of the traditional manually operated door handle , it is possible to open doors with a foot pedal . contamination of door handles with pathogens is thus avoided . stefan preiss is responsible for sales / production at sass - systeme gmbh axel kramer declares that he has no competing interests . stefan preiss is responsible for sales / production at sass - systeme gmbh axel kramer declares that he has no competing interests .

aim : as door handles represent a transmission route for viruses and micro - organisms , a door opening and closing mechanism should be developed without manual operation.method : to solve the problem , a device for opening the door with the help of a foot pedal was built into the door leaf.results : the design enables mechanical opening of the door with a foot pedal without manual operation . subsequently , the door closes with the help of a mechanical locking mechanism.discussion : the foot - operated door opener constitutes an additional option to the door handle . together with the equipment of a soft closing mechanism , it is possible to prevent noise emanating from the door latch impinging on the door frame . using this construction , the door handle as a transmission vector is eliminated . in addition , the transport of goods held with two hands simultaneously is facilitated.conclusion : with a foot - operated door opener instead of the traditional manual door handle , it is possible to open doors with a foot pedal . this prevents contamination of door handles with pathogens .

as the impact of neural signaling on cancer becomes clearer , it is imperative that stress - sensitive steps in cancer progression are identified for the development of targeted interventions . the findings presented here suggest that targeting either neural or inflammatory signaling activated by stress may limit metastatic dissemination and cancer - related mortality ( fig . 1 ) . this is in line with recent retrospective epidemiologic studies linking -blocker use to improved cancer outcomes . we now demonstrate that -blocker use in cancer patients is linked to reduced tumor cell dissemination to lymph nodes . these findings provide a mechanistic rationale for ongoing clinical trials using -blockers as a possible treatment option for patients with cancer . activation of the sympathetic nervous system increases levels of catecholamines , norepinephrine ( ne ) , and/or epinephrine ( e ) . tumor - associated macrophages respond to ne / e by secreting inflammatory molecules such as prostaglandin e2 ( pge2 ) , which drive the production of vascular endothelial growth factor c ( vegfc ) in tumor cells . various points in this signaling cascade can be targeted using drugs such as -blockers ( bbs ) or non - steroidal anti - inflammatory drugs ( nsaids ) . activation of the sympathetic nervous system increases levels of catecholamines , norepinephrine ( ne ) , and/or epinephrine ( e ) . tumor - associated macrophages respond to ne / e by secreting inflammatory molecules such as prostaglandin e2 ( pge2 ) , which drive the production of vascular endothelial growth factor c ( vegfc ) in tumor cells . various points in this signaling cascade can be targeted using drugs such as -blockers ( bbs ) or non - steroidal anti - inflammatory drugs ( nsaids ) . the research described here was funded by the australian national health and medical research council ( 1008865 and 1053535 ) , the australian research council ( le110100125 ) , the national cancer institute ( ca160890 ) , the australian and new zealand college of anaesthetists ( n13/002 ) , the co - operative research center for cancer therapeutics and the national breast cancer foundation .

abstractchronic stress drives cancer progression , but the routes of metastasis are unclear . we recently demonstrated that chronic stress activates a neural - inflammatory signaling axis to remodel lymphatic vasculature and increase lymph flow . this unanticipated crosstalk between stress and the lymphatic system provides pathways of tumor cell dissemination and accelerates metastasis .

spontaneous perforation of rectum is a rare event ; however evisceration of the small bowel through the perforated site without predisposing factors is extremely rare , complex and worth reporting . the operative findings revealed it to be a case of spontaneous perforation of rectum with evisceration of the small bowel through the perforation . sudden increase in the intra - abdominal pressure leads to the perforation in the chronically deranged rectal wall and pushes the small bowel loops into the pelvis and through the perforated rectum to appear transanally . evisceration of small bowel through a spontaneous perforation in rectum is an extremely rare event . although 55 cases of evisceration of small bowel through anus due to perforation of rectum have been documented in world literature since the first report in 1827 by benjamin brodie , the number of cases without any significant predisposing factor is very less . the article reports a case of spontaneous perforation of rectum with evisceration of small bowel in a normal bowel without an apparent cause which is an extremely rare presentation . a 14 years old male was admitted with the chief complaint of acute lower abdominal pain which started suddenly after he passed stools . the abdomen was tender on deep palpation in the left iliac fossa and bowel sounds were sluggish . however , on per rectal examination gut loops were felt in the anal canal and finger was stained with blood thereby making intussusception the most probable diagnosis . a lump was felt in the left iliac fossa once the patient was anaesthetized for surgery . peroperative findings revealed a distended and a tubular sigmoid colon ( intussusception like appearance ) and twisting of ileal loops around the sigmoid colon simulating ileo - sigmoid knotting . on further mobilization and gentle traction on the ileal loops gangrenous segment of ileum became evident till it finally emerged out from a longitudinal perforation ( approximately15 mm ) in the anterior wall of rectum just above the peritoneal reflection ( fig . rectal tear was closed primarily in single layer after taking the biopsy from the whole edge which revealed non specific inflammation . on repeated questioning in postoperative period patient denied any history suggestive of constipation , rectal prolapse , trauma , weight lifting , rectal instrumentation or homosexual activity ; however intermittent straining at stools was present . figure showing rectal perforation and the gangrenous segment of ileum which had eviscerated through the perforated rectum . urinary bladder ( small square ) , the peritoneal reflection ( thin arrow ) and the rectal perforation ( thick arrow ) are clearly visible . spontaneous rectal perforation usually occurs due to excessive straining on the anterior rectal wall with a pre - existing pathology like , diverticulosis , colitis , ulceration , malignancy , adhesions , irradiation , rectal and uterine prolapse and as a consequence of iatrogenic injuries and blunt trauma abdomen . . chronic strain because of the underlying pathology causes progressive deepening of rectovesical and rectouterine pouches and the rectal wall becomes thin and weak as it is unsupported . contraction of the abdominal muscles increases the intra - abdominal pressure and spontaneous perforation occurs through this thinned out area , mostly at the ant mesenteric border where the blood supply is poorest . similarly , in a constipated patient the changed defecation pattern in some individuals per se causes a preliminary lesion in the intestinal wall which becomes friable due to chronic inflammation of its layers and may perforate . nevertheless intestinal evisceration through the perforated gut without any contamination of the peritoneal cavity is enigmatic and little difficult to explain . in a recent study presumed that two factors predisposed patients to this unusual complication : one was the sudden increase in intra - abdominal pressure and the other was the presence of rectal prolapse . whatever the mechanics involved the sudden increase in the intra - abdominal pressure seems to be the main contributing factor . it pushes the ileal loops into the rectovesical / rectouterine pouch which violently presses upon the anterior rectal wall . as the week rectal wall gives way the ileal loops sneak in . this seems to be the only plausible explanation for the spontaneous act particularly when there are no signs of peritonitis as in our case . in a perforated simple ulcer of rectum there is an area of induration around edges while a spontaneous perforation appears slit like , runs longitudinally ( rarely transversally ) with minimal signs of inflammation . it can occur in all age groups , the youngest one reported was six years old and oldest being ninety six years old . the treatment follows basic surgical principles . if reduced bowel is not viable it should be resected and followed by anastomosis or ileostomy depending upon the condition of the patient and contamination involved . in the follow up period patients should be investigated to detect exact etiology and cause specific management will prevent recurrence and complications . therefore , one must remember that per rectal palpation of bowel loops may be more than just rectal prolapse or intussusception .

context : spontaneous perforation of rectum is a rare event ; however evisceration of the small bowel through the perforated site without predisposing factors is extremely rare , complex and worth reporting.case report : a 14 years old presented to us apparently as a case of intussception . the operative findings revealed it to be a case of spontaneous perforation of rectum with evisceration of the small bowel through the perforation.conclusion:sudden increase in the intra - abdominal pressure leads to the perforation in the chronically deranged rectal wall and pushes the small bowel loops into the pelvis and through the perforated rectum to appear transanally .

epidermoid cysts are benign developmental malformations arising from abnormal epithelial constituents of ectodermal tissue formed during the fetal period . these lesions can be seen anywhere in the body , with the occurrence of approximately 7% in the head and neck region and their incidence in the oral cavity makes up for 1.6% of the total occurrences and they constitute < 0.01% of all the cystic lesions of the oral cavity . epidermoid cysts of the oral cavity , in adults , are mostly seen on the floor of the mouth and in other locations including the labial , palatine tonsil and in the soft palate . in infants incidence is highest in the floor of the mouth and lowest in the soft palate . only four cases have been reported in literature of epidermoid cysts in infants involving the soft palate . we present here a report of a 9-month - old female patient who underwent surgery for cleft palate and growth on the uvula of soft palate clinically diagnosed as a benign fibrous tumor which was confirmed by histology as epidermoid inclusion cyst . a 9-month - old indian infant was brought to the cleft care center for a surgery of cleft palate and associated growth in the soft palate . family history was not significant and her mother had an uncomplicated delivery . on physical examination , the patient was alert and intraoral examination revealed a cleft palate involving the soft palate and a whitish , oval shaped , solitary solid mass with pale overlying mucosa located behind the uvula and right side of the soft palate measuring approximately about 1 cm 1 cm [ figure 1 ] . no other external facial or neck cysts , sinuses or lesions were noted and the rest of the intraoral space was unremarkable . a provisional diagnosis of benign fibrous tumor was considered and differentials included salivary gland neoplasms and benign tumors of muscular origin . the patient was admitted to the hospital for the surgery of cleft palate as the medical history was irrelevant . surgery was performed under general anesthesia , surgery was uneventful , and the patient recovery was good . histopathological examination revealed a parakeratinized stratified squamous epithelium with flattened rete ridges and a cystic space with keratin flecks . the patient is under follow - up and there is no evidence of recurrence even after 1.5 years . clinical image revealing cleft palate with a whitish , oval shaped , solitary solid mass with pale overlying mucosa located behind the uvula and right side of the soft palate ( a ) gross specimen creamish white in color and soft in consistency ; ( b ) cut section of the gross specimen showing cystic space ; ( c ) photomicrograph demonstrating the cystic cavity containing keratin ( h&e stain , 40 ) ( d ) photomicrograph demonstrating parakeratinized stratified squamous epithelium with flattened rete ridges and the cystic space with keratin flecks ( h&e stain , 100 histopathological image demonstrating epithelium and keratin ( h&e stain , x40 ) high power view of the epithelium and keratin in the lumen ( h&e stain , x100 ) photomicrograph showing keratinizing epithelium and keratin flecks in the lumen ( h&e stain , 100 ) histopathological figure at 10 photomicrgraph showing keratin flecks ( h&e stain , x200 ) a thorough search using the keywords such as soft palate , pediatric / congenital , and epidermoid cyst / dermoid cyst/ in various combinations was made in pubmed . a keyword search using pidermoid cyst , palate , cleft in the pubmed literature revealed 10 cases , but none of them were associated with a cleft soft palate table 1 . reported cases of epidermoid cysts of soft palate and uvula dermoid cysts were classified by new and erich in 1937 as acquired implantation , congenital teratoma and congenital inclusion dermoid cysts and by meyer in 1955 as true dermoid cysts , epidermoid cysts and teratoid cysts . a true dermoid cyst is lined with keratinized epithelium and has skin appendages like hair follicles or sebaceous glands . an epidermoid cyst is lined with simple squamous epithelium and does not contain skin appendages . a teratoid cyst in addition to skin appendages contains other tissues such as muscle , bone and cartilage . according to new and erich , who conducted a study on 1495 cases , the common location was anal region ( 44.5% ) followed by ovarian ( 42.1% ) region . the overall incidence of pediatric head and neck cysts was 7% , with the periorbital region being the most common site . this is equally consistent with the study by pryor et al . which concentrated on pediatric dermoid cysts of the head and neck , in which 61% of head and neck dermoid cysts were periorbital in location . in neither of these series , were cysts of the soft palate mentioned . dermoid cysts and epidermoid cysts can be acquired or congenital , but in infants , they are usually congenital . during the 6 week of gestation , the secondary palate begins to develop . it starts initially as two outgrowths from the maxillary prominences which are oriented in a vertical direction and layout on either side of the tongue . during 8 week , these palatine shelves orient themselves in a horizontal direction and the tongue descends downward as mandible elongates and the fetal head tilts upward . the palatal shelves continue to grow toward each other and contact at the medial edge epithelia . a transient midline epithelium or midline seam is formed at this time and as the growth of head increases the seam thins into a single layer of cells and undergoes disintegration completely resulting in the merging of the mesenchymal portion of two palatal shelves by the process of fusion . this process of shelf elevation and fusion takes place about a week later in girls than in boys which explain why girls are more prone than boys for cleft palate formation . in contrast , after the fusion of palatal shelves the soft palate uvula forms through migration and proliferation of two confluenced sub epithelial mesenchymal growth centers at the posterior edge of the newly formed palate with a groove between them filled by merging . failure of the merging process during soft palate and uvula development can result in complete or partial clefts of the soft palate and uvula . and hence , in distinction to the previous epidermoid cysts that were reported on the soft palate , in the present case cleft of secondary palate might have developed due to lack of fusion and merging ; and the epidermoid cyst would have developed due to aberrant ectodermal entrapment in the uvular area followed by reactivation of these cleaved cells . identification of epidermoid cyst is essential in neonates as they may cause difficulty and obstruction in breathing and swallowing which might turn out to be fatal .

epidermoid cysts are benign malformations that can be encountered anywhere in the body and are rarely observed in the oral cavity accounting for < 0.01% of all cysts of the oral cavity . they can be classified as either congenital or acquired without any clinical or histologic differences . our literature search did not find any report of a congenital epidermoid cyst located in the soft palate associated with a complete palatal cleft in an infant . this is a case report of a 9-month - old female patient who had a cleft palate with an associated soft tissue mass at the junction of soft palate and uvula .

to foster greater coordination on surveillance , research , education , and prevention of tickborne diseases , cdc established ticknet during 2007 . ticknet is a public health network that includes partners from state health departments and academic institutions collaborating through the emerging infections program ( eip ) , staff of state and local health departments collaborating through the epidemiology and laboratory capacity ( elc ) cooperative agreement , and cdc staff in the division of vector - borne diseases and the division of parasitic diseases and malaria . ticknet provides funding to state and local health departments through the elc cooperative agreement to help sustain and enhance routine surveillance for tickborne diseases . approximately 18 state and local health departments are funded annually for lyme disease surveillance , with priority given to states with a reported incidence of lyme disease greater than the national average and to bordering states where the disease may be spreading . during 2014 , an additional 7 state and local health departments received elc funding to support surveillance for other tickborne diseases . together with elc funding for program support , funding through eip has allowed ticknet partners in maryland , minnesota , and new york to undertake special studies to quantify underreported tickborne diseases . these studies include a review of patient charts and codes from the international classification of diseases , ninth edition , and provide insights into the use of electronic medical records for public health surveillance . other studies in massachusetts , minnesota , and new york are examining ways to streamline the evaluation of positive laboratory reports by using random sampling methods . , ticknet partners at eip sites in connecticut , maryland , minnesota , and new york conducted a survey of commercial , clinical , and state laboratories to evaluate practices and volume of testing for 5 leading tickborne diseases . collectively , 7 large commercial laboratories reported testing 2.4 million patient specimens for evidence of b. burgdorferi infection during 2008 , at an estimated cost of $ 492 million . after correcting for test sensitivity , specificity , and stage of illness , the overall frequency of infection among patients for whom samples were tested was estimated at 12% . applied to the total number of specimens , this percentage yielded an estimated 288,000 true b. burgdorferi infections ( range 240,000444,000 ) among source patients during 2008 ( 18 ) . results of this study will be compared with results of other ongoing cdc studies to estimate the overall frequency of lyme disease and other tickborne infections in the united states . to better quantify the public health burden of tickborne diseases , ticknet eip partners in connecticut , maryland , minnesota , and new york have undertaken a study to quantify current costs associated with individual cases of lyme disease . begun during 2014 , the cost of lyme disease study uses a prospective survey design to capture individual and societal costs of lyme disease , including out - of - pocket medical costs , nonmedical costs , and productivity losses , as well as total direct medical costs to society by using billing codes from enrolled patients providers . this estimate will be used to guide impact assessments of current and future prevention methods . as an adjunct to personal protective measures such as use of insect repellents , several yard - based interventions have been proposed to reduce tick abundance in the home environment . to assess the efficacy of such interventions in preventing human illness , ticknet sites have instituted a series of studies to evaluate the efficacy of novel and commercially available prevention strategies . one study , a randomized , blinded , placebo - controlled , multistate trial assessing the effectiveness of acaricide barrier sprays , will cover 2,700 households in 3 states , with outcomes measures including tick density on acaricide - treated properties , the number of tick human encounters , and the number of tickborne diseases in humans . a second study , begun in connecticut during 2012 , uses a similar design to evaluate the effectiveness of bait boxes that apply fipronil to rodents that are the reservoirs of b. burgdorferi . used by veterinarians to prevent flea and tick infestations on dogs , fipronil kills ticks on the rodents for several weeks and may potentially interrupt the local transmission cycle of b. burgdorferi . recent experience indicates that additional tickborne pathogens are waiting to be discovered . in collaboration with the tennessee and minnesota health departments , the mayo clinic , and vanderbilt university , ticknet has recently initiated a study to identify novel agents of tickborne disease . over the next 3 years , > 30,000 clinical specimens from us patients with suspected tickborne diseases will be screened by using high - throughput molecular methods designed to detect bacteria , followed by use of genomic sequencing to characterize detected pathogens . the ultimate goal is to better describe the epidemiologic and laboratory features associated with recognized and novel tickborne pathogens and to guide the development of new diagnostic methods . although sometimes overlooked , tickborne diseases pose an increasing threat to public health . factors driving the emergence of tickborne diseases are poorly defined , but current prevention methods are clearly inadequate . addressing this problem requires a multidisciplinary approach with input of entomologists , epidemiologists , educators , and infectious disease and communications specialists . built on the pillars of the eip and the elc cooperative agreements , ticknet provides a collaborative network that brings together these resources at the federal and state levels to enhance surveillance , improve prevention , and identify new tickborne diseases .

ticknet , a public health network , was created in 2007 to foster greater collaboration between state health departments , academic centers , and the centers for disease control and prevention on surveillance and prevention of tickborne diseases . research activities are conducted through the emerging infections program and include laboratory surveys , high - quality prevention trials , and pathogen discovery .

on april 21 , 2008 , three whooper swans ( 2 adults and 1 juvenile ) were found dead at lake towada , akita prefecture , japan ( figure 1 ) . it was taken to the wildlife protection center in akita but had to be euthanized that day in moribund status . homogenates from the tracheas , cloacas , and internal organs of 3 swans were pooled and inoculated into embryonated chicken eggs for virus isolation . map of japan and nearby countries , with enlargement of the northern part of the country ( inset ) showing location of lake towada . agents were confirmed to be type a influenza viruses by a commercial rapid antigen assay kit and were excluded from being newcastle disease virus by the hemagglutination inhibition test with newcastle - specific antiserum . after those tests conducted at the animal hygiene service center , viruses were brought to the national institute of animal health , tsukuba , japan , for further analysis . the viruses were subtyped as h5n1 with a panel of antiserum , and 1 yielded from cloaca homogenates was designated as a / whooper swan / akita/1/2008 ( wsak08 ) and was further analyzed . wsak08 was shown to be highly pathogenic to chickens by an intravenous administration of 10-fold diluted infectious allantoic fluid . this result coincides with the sequence analysis of the hemagglutinin ( ha ) gene , showing that the ha protein possesses a series of basic amino acids ( pqrerrrkr ) at the cleavage site . phylogenetic analysis of the ha1 region of the ha gene ( figure 2 ) showed that wsak08 belongs to clade 2.3.2 and is clearly distinguishable from the hpaivs previously isolated in japan in 2004 , a / chicken / yamaguchi/7/2004 ( clade 2.5 ) , and in 2007 , a / chicken / miyazaki / k11/2007 ( clade 2.2 ) . although sequence data were not found in genbank , a / common magpie / hong kong/5052/2007 reportedly resides in the same clade ( 9 ) . antigenic analysis of wsaki08 with a panel of antiserum and monoclonal antibodies showed low reactivity against antibodies in the panel ( appendix table ) . a > 32-fold reduction from homologous titers of all hyperimmune serum used postinfection duck serum against a / chicken / yamaguchi/7/2004 and a / chicken / miyazaki / k11/2007 did not react with wsak08 . none of the monoclonal antibodies against ha protein of a / chicken / yamaguchi/7/2004 reacted with wsak08 . thus , wsak08 is genetically and antigenically distinguishable from the hpaivs that caused previous outbreaks in japan . phylogenetic tree constructed based on the hemagglutinin ( ha ) 1 region ( 966 bp ) of the ha gene of the highly pathogenic avian influenza viruses ( h5n1 ) . clade designation follows the criteria proposed by the world health organization / world organisation for animal health / food and agriculture organization h5n1 evolution working group ( 8) . representative strains of the previous highly pathogenic avian influenza outbreaks in japan are in boldface . ab436731ab436738 ) showed that it does not contain amino acid substitutions conferring resistance to adamantane or neuraminidase inhibitors . unlike many isolates related to qinghai lake strains that have spread worldwide the neuraminidase protein has a 20-aa deletion at aa 49 to 68 in the stalk region . nonstructural protein 1 has a 5-aa deletion at aa 80 to 84 , commonly observed in currently circulating hpaivs ( h5n1 ) in southeastern asia . whooper swans breed in northern eurasia and winter in europe and eastern asia i.e. , china , the korean peninsula , and japan . in japan , 35,00038,000 whooper swans spend every winter primarily in hokkaido , tohoku , and the hokuriku area ( 10 ) . in the lake towada area , 300 whooper swans arrive beginning in late october ; they leave the area between late march and late april ( 11 ) . in late march , summer birds begin to arrive . according to results of satellite tracking of 8 swans ( 12 ) , as well as the results of banding studies since 1961 ( 13 ) , whooper swans that winter in japan migrate from the northern end of honshu island to eastern hokkaido , by means of sakhalin , and reach eastern siberia , where they breed . to our knowledge , there have been no reports of whooper swans that winter on the eurasian continent migrating north through japan . in light of the migratory route mentioned above , the whooper swans found dead at lake towada were most likely recently infected with hpaiv ( h5n1 ) in japan . it is unlikely that the swans were infected before they flew to japan in autumn , maintained the virus within the flock , and then suddenly developed the disease after no apparent infections for several months . although the susceptibility of a certain species of birds to a subtype h5n1 virus may be different depending on the virus strain ( 14 ) , whooper swans as well as mute swans have been considered to be susceptible species to hpaiv ( h5n1 ) , as they showed a fulminant course of disease at the outbreak in germany in 2006 ( 7 ) . the possibility that the swans were infected by domestic fowl is low because there has been no report of hpai among domestic fowl in japan since the beginning of 2008 . one possible explanation is that other wild birds brought the virus from outside the country . although it is not known whether any birds wintering on the continent migrate north through japan , passage visitor birds such as wader birds migrate from south to north through japan in spring ; summer birds , e.g. , egrets , swallows , songbirds , and some raptors , come to japan from the south in spring for breeding . also , the possibility of anthropogenic introduction of virus , such as by inappropriate importation of birds , meats , or materials , can not be excluded . in conclusion , genetic analysis demonstrates that the virus that killed the 4 swans in japan in 2008 is genetically distinguishable from the strains that caused previous poultry outbreaks in japan , ruling out a possibility of resurgence of previously introduced hpaiv in japan . after the incident we describe , 2 other whooper swan cases of hpaiv ( h5n1 ) infection were confirmed in eastern hokkaido in early may . possible involvement of wild birds in the introduction of the virus to japan requires further scrutiny . antigenic analysis of whooper swan / akita/1/2008 highly pathogenic avian influenza virus ( h5n1 ) versus related isolates *

on april 21 , 2008 , four whooper swans were found dead at lake towada , akita prefecture , japan . highly pathogenic avian influenza virus of the h5n1 subtype was isolated from specimens of the affected birds . the hemagglutinin ( ha ) gene of the isolate belongs to clade 2.3.2 in the ha phylogenetic tree .

a 73-year - old female was admitted for surgical removal of a left renal pelvic mass . she has taken antihypertensive agents for 30 years and received percutaneous coronary intervention due to stable angina 3 years ago . during routine follow - up , a renal mass was incidentally detected 2 months ago . laboratory values revealed a marginal elevation of serum creatinine ( 1.21 mg / dl ) . the ultrasonography showed an echogenic mass within the renal pelvis and mild dilation of several calyces . the calcified lesion was not found on plain radiograph of the abdomen ( kidneys , ureters , and bladder ) . an abdominopelvic computed tomography revealed a noncalcified soft tissue mass in the left renal pelvis on precontrast enhancement scan . the cut surface of the resected specimen revealed a 532 cm - sized , well - defined ovoid , cystic mass which was compactly filled with white and reddish tan - colored amorphous materials in the pelvis . histologically , the cystic lumen contained multiple keratinous materials and much of the cystic lining epithelia were denuded . , santa cruz , ca , usa ) and cytokeratin 7 ( ck7 ) ( 1:100 ; dako , glostrup , denmark ) was performed to identify epithelium - specificdifferentiation . in contrast , most of the cells in the lining of squamous epithelia were negative for these urothelial specific antibodies . however , some portion of the squamous epithelia interestingly contained a few uroplakin - positive cells ( fig . even though the epidermoid cyst of the kidney is a very rare to be characterized , the typical features of the lesion can be summarized as follows . the clinical symptoms are flank pain , gross hematuria , or lower urinary tract symptoms . the calcified inhomogeneous lesion , combined renal stone , and previous renal surgery are other important clinical clues for the renal epidermoid cyst ( table 1 ) . to date , two cases of epidermoid cyst in the renal pelvis have been reported . they also showed a typical calcified lesion combined with renal stone like other renal epidermoid cysts . with these reasons , it seemed hard to think of epidermoid cyst as a presumptive diagnosis before surgery in this case . to the best of our knowledge , this case of a noncalcified epidermoid cyst in the renal pelvis is the first report in the english literature . the histology of a typical epidermal cyst that was lined by stratified squamous cells with laminated keratins . although epidermoid cyst of the skin is a common lesion resulted from implantation of epidermis into the dermis , histogenesis of epidermoid cyst in the kidney remains uncertain . as a possible histogenetic mechanism , either epidermal embryonic remnants of wolffian ducts or traumatic implantation of transformed epithelial cells during renal operation have been proposed . the extension of squamous metaplastic changes from the upper ureter to the pelvocalyceal system in prolonged irritative condition induced mostly by stones is considered as the other plausible explanation . however , the cellular basis of squamous metaplasia is not fully understood in this rare condition . the apical surface of urothelium is highly specialized as it is covered almost by urothelial plaques which are composed of uroplakins . four uroplakins ( ia , ib , ii , and iii ) have been isolated and are considered to be biochemical markers of terminal urothelial differentiation . ck7 is an intermediate filament proteins expressed in a wide variety of simple epithelia but not in the squamous epithelia . it is well known that vitamin a deficiency can induce keratinizing squamous metaplasia in a variety of epithelia including urothelium . the urothelial keratinization in the vitamin a - deficient mice bladder occurred heterogeneously adjacent to areas lined with normal appearing urothelium . the sharp boundary between the keratinized epithelium and the seemingly normal urothelium was maintained with no intermediated cells . according to the our histologic examination , nearly almost of the urothelial cells in the cystic lining were positively stained against both uroplakin ii and ck7 , but most of the squamous epithelial cells were negative for these antibodies . the two epithelia were sharply delineated and more distinctly distinguishable in the immunohistochemical stained sections . these features were similar with heterogenous metaplasia of the animal urothelium in the previous study . however , some squamous epithelia contained a few uroplakin - positive cells and ck7-positive cells to some extent . even though the significance of few uroplakin expressions in squmous epithelium should be evaluated by other studies , is this an indirect evidence of the direct transformation of umbrella cells to squamous metaplasia or dedifferenctiation of metaplastic squamous epithelium to urothelium ?

since the renal epidermoid cyst is too rare , the mechanisms of squamous morphogenesis have not well characterized . a 73-year - old female was referred with an incidentally detected renal pelvis mass . abdominopelvic computed tomography scan revealed a noncalcified soft tissue mass in the renal pelvis . total nephroureterectomy was performed under the impression of a renal pelvis malignancy . the patient was discharged without postoperative complication . the outer surface of mass lesion was lined with urothelia and squamous epithelia , containing keratinous materials . the urothelia were positively stained against uroplakin ii and cytokeratin 7 , whereas almost of the squamous epithelia were negative with uroplakin ii . the two different epithelia were generally sharply demarcated . interestingly , some part of squamous epithelia contained uroplakin - positive and many more cytokeratin 7-positive cells . the atypical clinical features in our case can reconsider the diagnostic clues of renal epidermoid cysts that have been reported before , and the unique immunohistochemical results may understand the histogenetic implications of the lesion .

zonisamide ( zns ) is an antiepileptic drug that has recently been approved in many countries . it is also effective for the management of migraine , neuropathic pain , essential tremor , anxiety , and other conditions . recently , zns has been approved as a new adjunctive therapy for motor complications of parkinson 's disease ( pd ) in japan . more recently , zns was reported to be effective for the management of impulse control behavior in pd , suggesting potential effects on non - motor pd symptoms . dream enactment associated with aggressive , violent behavior can carry a serious risk of injury to patients , as well as to spouses or caretakers . we describe a patient with pd who had vivid nightmares and dream - enacting behavior that resolved after treatment with zns . in 2009 , a 71-year - old man with a history of pulmonary surgery noticed tremor of the left hand and akinesia . in march 2010 , he showed features of moderate parkinsonism , including masked face , stooped posture , bradykinesia , left - side - dominant rigidity , and resting tremor . bradykinesia , rigidity , and resting tremor responded to treatment with pramipexole ( 0.5 mg / day ) . since july 2010 , his wife was awakened nearly every night because of the patient 's aggressive or violent behavior during the middle of the night . for example , he suddenly flew up or walked , collided with furniture or walls , cried out , or exercised his limbs noisily . he was often injured . on the nights he presented with this behavior , he remembered having vivid nightmares , such as being chased by a cat , bear , or his wife or of fighting with a thief or grandchild . interviews with the patient 's wife indicated that this aggressive , violent behavior during the night occurred sometimes in 2009 . in april 2011 , stooped posture and lumbar pain developed , which were attributed to pramipexole . we switched from pramipexole ( 1.0 mg / day , once before sleep ) to zns ( 25 mg / day , once before sleep ) . before switching to zns , motor and non - motor symptoms were as follows : the scores on parts i , ii , iii , and iv of the unified parkinson 's disease rating scale ( updrs ) were 3 , 5 , 17 , and 0 , respectively . the scores on the mini - mental state examination ( 29/30 ) and frontal assessment battery ( 18/18 ) were normal . the heart - mediastinum 123i - metaiodobenzylguanidine uptake ratio was significantly decreased . because he noticed reduced olfactory sensitivity , we assessed olfactory recognition ability by using the odor stick identification test for japanese ( osit - j ) , as described previously . briefly , the osit - j tests for 13 kinds of odors familiar to japanese people ( curry , cooking gas , perfume , japanese cypress , india ink , menthol , nattou [ fermented soybeans]/sweaty socks , rose , putrid smell , wood , roasted garlic , condensed milk , japanese orange ) . we asked the patient to choose an answer among four possible odor names , one of which was correct , plus detectable but not recognizable and odorless. he could identify four odors ( perfume , curry , cooking gas , and condensed milk ) . the identification rate was 33% , consistent with that of patients with olfactory disturbances ( 36 34% ) . the score on the pittsburgh sleep quality index ( ranging from 14 for normal to 56 for unsleeping ) was 16 , and the 2-point increase above normal was ascribed to difficulty in sleeping caused by nightmares . his wife was no longer awakened since the patient 's aggressive and violent behavior had resolved . we obtained informed consent from the patient and tested whether pramipexole elicited nightmares or violent behavior . first , he was given pramipexole ( 0.5 mg / day ) in the context of stable doses of zns ( 25 mg / day ) for 2 weeks . our patient showed aggressive and violent behavior associated with vivid nightmares , which probably preceded such behavior , during the middle of the night . this disorder strongly suggested a rapid - eye - movement sleep behavior disorder ( rbd ) . in our patient , who had rbd dopamine agonists can lead to vivid dreams , nightmares , and parasomnia - like motor activity , but rbd in our patient was not induced by rechallenge with pramipexole . in patients with pd , pramipexole was reported to be effective for rbd , but to worsen rem sleep electromyographic abnormalities on video - polysomnography . one study showed that pramipexole did not improve rbd in pd , and the authors mentioned that rbd in pd may be either 2 different stages of the same condition or 2 completely different conditions . this can explain our observations during treatment with pramipexole , but we believe that pramipexole did not alter rbd in our patient since the wife 's interviews indicated that rbd was evident before starting pramipexole . this notion is supported by the results of a previous study showing that the frequency and severity of rbd were unaffected by pramipexole therapy . however , our finding raises an open question whether zns is useful for treating rbd in patients with early pd . dream generators are suppressed by inhibition of brainstem locomotor pattern generators , which can modify dream content in rbd . dopaminergic dysfunction may play a role in the pathophysiology of rbd and dopamine modulates the expression of locomotion and other rhythmic motor patterns in neural circuits known as central pattern generators . a zns - induced effect on the dopaminergic system might have modulated or inhibited brainstem locomotor pattern generators , consequently suppressing vivid nightmares , leading to the resolution of dream - enacting behavior . our experience suggests that zns potentially might be effective for the management of vivid nightmares or dream - enacting behavior in patients with early pd . the authors report no conflicts of interest . there was no financial disclosure related with this work .

recently , zonisamide ( zns ) has been approved as a new adjunctive therapy for motor complications of parkinson 's disease ( pd ) . more recently , zns was reported to be effective for the management of impulse control behavior in pd , suggesting potential effects on non - motor pd symptoms . dream enactment associated with aggressive , violent behavior can carry a serious risk of injury to patients , as well as to spouses or caretakers . this report describes a patient with pd who had vivid nightmares and dream - enacting behavior that resolved after treatment with zns . the present case raises the question whether zns might potentially be effective for the management of vivid nightmares or dream - enacting behavior .

pneumopericardium is defined as the presence of air - fluid level in the pericardial sac and has been reported to result from a spontaneous or iatrogenic cause of underlying disease.1 ) it is a rare condition but is important in the differential diagnosis of chest pain . posteroanterior chest radiographs typically reveal air - fluid level and a radiolucency of air surrounding the cardiac boarder is outlined by a fine line representing the pericardial sac . although severe complications occur in some patients , the iatrogenic pneumopericardium is self - limiting and requires no specific therapy.2 ) we discuss a case of iatrogenic pneumopericardium in a young man who underwent pericardiocentesis due to tuberculous pericardial effusion . a 20-year - old man was referred to the hospital with extensive pericardial effusion on a computed tomography . the patient had a history of pulmonary tuberculosis for 2 years and had taken anti - tuberculosis medication for the past 8 months . physical examination showed stable vital signs : blood pressure , 131/65 mmhg ; pulse rate , 92 bpm ; respiratory rate , 26 per minute ; body temperature , 36.5. posteroanterior chest radiographs showed cardiomegaly without any lung lesion ( fig . 1 ) . an emergent echocardiogram showed a large circumferential pericardial effusion and diastolic right atrium collapse without respiratory variation of the mitral inflow . the left ventricular ejection fraction was estimated to be 60% . a 7fr arrowgard blue catheter ( arrow international inc , bernville , pa , usa ) was used for subxiphoid pericardiocentesis . over 1,000 ml of serous fluid was drained from the pericardial sac over the following 12 hours . the pericardial fluid was a lymphocyte dominant exudate , containing protein 6.5 g / dl , albumin 3.6 g / dl , lactate dehydrogenase 466 u / l and white blood cell 7,200 cells/l ( lymphocyte 84% ) . polymerase chain reaction for mycobacterium tuberculosis deoxyribonucleic acid was negative with pericardial fluid and adenosine deaminase in pericardial effusion was 96 iu / l ( normal , 5 to 23 iu / l).3 ) on day 5 after the pericardiocentesis the patient developed a pleuritic chest pain . his blood pressure was 110/70 mmhg , heart rate was 72 bpm , and respiratory rate was 20 per minute . follow up chest radiographs showed a new lucent outline around the heart with a clear lung , representing the existence of air - fluid level in the pericardial space ( fig . 3 ) . echocardiography revealed scanty pericardial effusion , a bright echogenic spot swirling in the pericardial cavity and an absent tamponade ( fig . the diagnosis of pneumopericardium can be made by conventional chest radiographs , ct , or echocardiography.5 ) in posteroanterior chest radiographs , a continuous thin radiolucent rim of air and air - fluid level follows the cardiac silhouette and is outlined by a fine line representing the pericardial sac.2 ) pericardiocentesis with extended catheter drainage is a safe treatment for managing clinically significant pericardial effusions and can be performed effectively under local anesthesia.6 ) the subxiphoid window should be the standard initial procedure in most patients requiring drainage for effusive pericardial disease . it is less morbid than the transthoracic approach and allows a shorter hospital stay.7 ) for our patient we hypothesized that pneumopericardium was induced by chance due to forced coughing during drainage after pericardiocentesis . in tension pneumopericardium , rapid fluid resuscitation and emergent echo - guided pericardiocentesis , followed by pericardial drainage , should be performed . if the hemodynamic condition is stable , the underlying condition should be treated and the patient should be monitored closely.2 ) the patient was in a tolerable state , so we treated him conservatively and removed catheter drainage . the current case showed that pneumopericardium is a rare complication of pericardiocentesis , which occurs as a result of a leaky drainage system or direct pleura - pericardial communication .

pneumopericardium is a rare complication of pericardiocentesis , occurring either as a result of direct pleuro - pericardial communication or a leaky drainage system . air - fluid level surrounding the heart shadow within the pericardium on a chest x - ray is an early observation at diagnosis . this clinical measurement and process is variable , depending on the hemodynamic status of the patient . the development of a cardiac tamponade is a serious complication , necessitating prompt recognition and treatment . we recently observed a case of pneumopericardium after a therapeutic pericardiocentesis in a 20-year - old man with tuberculous pericardial effusion .

the assessment of intravascular volume and the adequacy of volume resuscitation are among the most difficult clinical challenges . systolic blood pressure , heart rate and urine output change minimally in early hemorrhagic shock . hypotension , tachycardia , cold extremities , decreased urine output and poor capillary refill are only present in patients who have lost in excess of 30% of their blood volume ( classiii hemorrhage ) . furthermore , both the central venous pressure and the changes in the central venous pressure in response to volume loading are poor indicators of intravascular volume and recruitable cardiac index . while flow to the brain and the myocardium is preserved in patients with ' compensated shock ' , splanchnic and renal perfusion may be seriously compromised . splanchnic hypoperfusion leads to both functional and structural changes in the gut mucosa , with increased permeability and translocation of bacteria and bacterial products . increased mucosal permeability has been strongly associated with the development of the multiorgan dysfunction syndrome . the expedient detection and correction of tissue hypoperfusion associated with ' compensated shock ' may limit organ dysfunction , may reduce complications and may improve patient outcome . it is probable that the earlier tissue hypoperfusion is detected and corrected , the greater the likelihood that outcome will be improved . indeed , rivers and colleagues reported a 32% relative reduction in the 28 day , all cause mortality of patients with severe sepsis who received early aggressive volume resuscitation in the emergency department . used the central venous oxygen saturation as the endpoint of resuscitation in the intervention group , while treatment in the control group was guided by standard clinical endpoints including the central venous pressure . while their study clearly demonstrates the value of early aggressive volume resuscitation , the use of central venous oxygen saturation to guide early resuscitation is not practical and has important limitations . the base excess ( be ) has become the standard endpoint of resuscitation in trauma patients . remarkably , while the be has been demonstrated to be of prognostic value , it has never been assessed prospectively in trauma patients . the use of the be is based on the principle that tissue hypoxia associated with poor perfusion will result in the generation of hydrogen ions and a metabolic acidosis . however , it is probable that tissue hypoperfusion may occur in the absence of a significant change in the be . furthermore , as significant time is required for the liver and kidney to regenerate bicarbonate , it can be expected that there will be a long lag phase between the correction of intravascular volume and normalization of the be . both of these assumptions are elegantly demonstrated in the study by totapally and colleagues reported in the present issue of critical care . in a rat hemorrhage model these authors demonstrated that the be responded slowly to changes in intravascular volume and that there was a significant increase in the be only when the mean arterial blood pressure fell by greater than 50% . however , totapally etal . demonstrated that changes in the esophageal carbon dioxide gap closely mirrored changes in the intravascular volume . similar findings have been reported by other investigators . in patients with penetrating trauma , baron and colleagues demonstrated that sublingual carbon dioxide measurements correlated well with the degree of blood loss . both ivatury and colleagues and kirton and coworkers have demonstrated that gastric intramucosal ph correlates well with the degree of injury and that optimizing the gastric intramucosal ph in the first 24 hours following trauma is associated with a reduction in the incidence of organ failure and death . the study by totapally and colleagues suggests that the be is an insensitive indicator of the degree of the intravascular volume deficit following hemorrhage and that it responds slowly to volume resuscitation . esophageal and sublingual capnometry , however , appear to provide near instantaneous information regarding the degree of the volume deficit and the adequacy of volume resuscitation . this technology is simple and noninvasive , and is ideally suited for use in the emergency room and the trauma bay . the esophageal or sublingual pco2 gap may prove to be a useful endpoint for the resuscitation of trauma victims . the author has received a research grant from optical sensors inc , minneapolis , mn , usa , the manufacturer of the nellcor n-80 capnoprobe sl device .

although it has never been prospectively validated , the base excess ( be ) is regarded as the standard end - point of resuscitation in trauma patients . in a rat hemorrhage model , in this edition of critical care , totapally and colleagues demonstrate that the be is an insensitive and slowly responsive indicator of changes in intravascular volume . this contrasts with changes in the esophageal - arterial carbon dioxide gap which more closely followed changes in blood volume . esophageal or sublingual capnometry may prove to be a useful tool for monitoring the adequacy of resuscitation in trauma victims .

during july and august 2012 , investigators from cdc s viral special pathogens branch reviewed shipping records from facility b and subsequent distributors and notified health departments in states that had received potentially infected mice during january 1may 7 , 2012 ; frozen mice were considered a low public health risk and were not traced . health departments were provided with a list of facilities that had purchased these mice , educational resources about lcmv , and an algorithm to determine whether potentially infected mice remained at these purchasing facilities resulting from the presence , comingling , or breeding of these mice , which would maintain lcmv among the mouse population ( figure 1 ) . as a result of varying state statutes concerning regulation and licensing of pet stores and animal breeders or distributors , the government agencies that had jurisdiction to perform these investigations included local and state departments of public health , environmental health , food safety , and agriculture . algorithm used to determine whether mice were potentially infected with lymphocytic choriomeningitis virus ( lcmv ) during a multistate investigation , united states , 2012 . this algorithm was used to determine whether 1 ) potentially infected mice remained at the facilities being assessed , 2 ) mice from the original shipment remained , 3 ) offspring from these mice remained , or 4 ) shipments of mice had been comingled or had shared equipment with mice from the original shipment . lcmv is easily maintained in a mouse colony , and a clear break among the population ( i.e. , a time when no remaining mice are maintained and equipment is disinfected ) is necessary to ensure that no ongoing infection continues . state investigators interviewed purchasing facility managers by telephone , mail , email , or in person to determine whether potentially infected mice remained on the premises and to encourage euthanization of these mice . interviews also assessed whether pregnant , ill , or immunocompromised employees might have been exposed to lcmv by directly handling potentially infected mice or bedding or equipment used for the mice . because of risk for severe disease , facility managers were asked to offer serologic testing to these employees for lcmv igm and igg , which was performed by cdc by using elisa as described ( 10 ) . no additional case - finding activities were conducted . because of resource limitations , diagnostic testing of live mice at purchasing facilities was not conducted . reviews of shipping records indicated that 304,000 live mice distributed by facility b were shipped to 561 purchasing facilities : 543 pet stores , 11 breeders or distributors , and 7 zoos or aquariums in 21 states , potentially exposing thousands of employees and pet store mouse purchasers to lcmv . facility b had shipped mice to 4 subsequent distributors ; the largest was located in georgia , and it had shipped > 183,000 mice to 420 purchasing facilities in 16 states ( figure 2 ) . interviews of facility managers at purchasing facilities revealed that 48% still had potentially infected mice ; > 10,000 mice were subsequently euthanized . the most common reason for still having potentially infected mice was comingling of rodent shipments , followed by breeding or still having mice from the original shipments . the distribution of mice potentially infected with lymphocytic choriomeningitis virus originating from facility a to 500 pet stores and other animal facilities in 21 states , united states , 2012 . serologic testing was performed on blood samples from 34 pet store or zoo employees from 6 states who self - identified as pregnant or ill , were potentially exposed to lcmv , and agreed to serologic testing . fourteen were pregnant ; 1 had aseptic meningitis ; and 23 reported nonspecific symptoms including fever , headache , body aches , cough , and vomiting . these captive feeder mice had a wide and complex distribution chain , potentially exposing thousands of persons to lcmv . no additional human cases were identified after distribution of these mice ; none of the pet store or zoo employees tested had serologic evidence of infection . although no additional human cases were identified , euthanasia of all potentially infected rodents was recommended to mitigate potential risk . wild mice that access captive breeding populations are often the source of infection of captive rodent populations ( 8,11 ) . after being introduced , lcmv transmission is easily maintained among mouse colonies and is difficult to recognize because mice do not appear ill . because persistently infected mice pass infection to their offspring , the number of infected mice in a breeding colony can quickly multiply . mice can be persistently infected without having serologic evidence of infection ( 12 ) ; thus , lcmv can be missed by serologic screening alone . therefore , preventing introduction of the virus into breeding colonies , depopulation of infected rodents , and correct use of personal protective equipment are the most efficient ways to mitigate human exposure . we recommend preventive measures at each point in the distribution process , both domestically and abroad ( table ) ( 1315 ) . more research is needed to develop methods for detecting lcmv in rodents at distributors and pet stores . employees tested were a fraction of those who had had contact with potentially infected mice . also , pet store mouse purchasers and purchasing facility employees were difficult to contact , and no pet store customers were tested . frozen feeder mice are considered pet food and can be regulated by the food and drug administration ( fda ) , but neither fda nor the us department of agriculture has the authority to regulate live mice and rats because they are not regulated under the animal welfare act ( 7 cfr 2132 , may 13 , 2002 , www.aphis.usda.gov/animal_welfare/downloads/awa/awa.pdf ) and the food , drug and cosmetics act ( 21 cfr 500 , april 1 , 2012 , www.accessdata.fda.gov/scripts/cdrh/cfdocs/cfcfr/cfrsearch.cfm?cfrpart=500&showfr=1 ) . therefore , regulatory authority falls to the states , which have an array of regulations governing the handling , breeding , and distribution of rodents , including the licensing of pet breeders and distributors ( 15 ; thomas edling , pers . because of the lack of consistent regulation , we recommend that state and federal partners and rodent industry advisory groups work with breeders , distributors , and pet stores to increase awareness of lcmv infection and implement recommended best practices ( table ) to prevent introduction of lcmv into captive rodent populations , prevent subsequent dissemination of potentially infected rodents , and reduce the potential for human exposure and disease among employees and consumers of pet stores and rodent breeding facilities . multistate lcmv outbreak , united states , july 2012 : recommendations for trace - back and safe disposal of potentially lcmv - infected mice .

during follow - up of a 2012 us outbreak of lymphocytic choriomeningitis virus ( lcmv ) , we conducted a trace - forward investigation . lcmv - infected feeder mice originating from a us rodent breeding facility had been distributed to > 500 locations in 21 states . all mice from the facility were euthanized , and no additional persons tested positive for lcmv infection .

kawasaki disease , first described by tomisaku kawasaki in japan in 1967 , is an acute systemic vasculitis of unknown etiology involving the small and medium - sized vessels with predilection for coronary artery involvement . the disease affects infants and children under the age of 5 years with no racial predilection and a sex ratio of 1.5:1 in favor of male children . typically , the disease is a multisystem febrile vasculitis with predominant cardiovascular manifestations . if untreated , it can lead to development of coronary artery aneurysm in nearly 25% of patients and systemic vascular aneurysms in less than 2% cases . a 6-month - old male infant was brought to the emergency department with complaints of fever for 1 month , passage of dark stools for 1 week , and discoloration of upper extremity for 2 days . the child was referred for doppler imaging ( philips hd 11 xe ultrasound machine , philips usa ) of the upper extremity and sonography of the abdomen on emergent basis . doppler usg showed partially thrombosed fusiform aneurysm of right subclavian artery , axillary artery , brachial artery , and non - thrombosed aneurysm of the right subclavian artery [ figure 1a and b ] . color doppler image shows non - thrombosed aneurysm of left subclavian artery ( a ) with partially thrombosed fusiform aneurysm of right subclavian artery ( b ) subsequently , ultrafast low - dose ct angiography ( philips brilliance 40 slice ct scanner ; philips usa ) was done in emergency settings which confirmed the color doppler findings and additionally showed aneurysms of bilateral common carotid , extracranial internal and external carotid , and bilateral vertebral arteries [ figure 2 ] . there were aneurysms involving multiple coronary arteries , with giant fusiform aneurysm of left anterior descending artery measuring 13 mm [ figures 3 and 4 ] . ct angiography of thoraco - abdominal aorta revealed small saccular aneurysm at the origin of celiac artery measuring 29 25 mm , with fusiform dilatation of superior mesenteric artery [ figure 5a and b ] . there were non - thrombosed fusiform aneurysms of bilateral common iliac artery and common femoral artery without the involvement of the main aortic trunk [ figure 6 ] . ct angiography ( coronal mip image ) shows dilated bilateral subclavian and axillary arteries with hypodense non - enhancing thrombus within the right subclavian also , fusiform aneurysms of bilateral extracranial carotid and vertebral arteries are noted ( short arrows ) axial ct angiography image at the level of aortic sinus shows giant aneurysm of left anterior descending artery ( short arrow ) with aneurysm of right coronary artery ( long arrow ) volume - rendered image ( left anterior oblique view ) shows giant fusiform aneurysm of left anterior descending artery aneurysms of branches of abdominal aorta . ct angiography sagittal mip image ( a ) shows aneurysm of superior mesenteric artery ( arrow ) . volume - rendered image ( b ) shows saccular aneurysm measuring 29 25 mm at the origin of celiac artery ( arrow ) ct angiography coronal mip image shows fusiform aneurysms of bilateral common iliac and femoral arteries based on the imaging features , a possibility of infantile panvasculitis was considered . review of the clinical parameters revealed normochromic - normocytic anemia , leukocytosis , and elevated erythrocyte sedimentation rate ( esr ; 43 mm in first hour ) and c - reactive protein ( 34 mg / l ) . the blood cultures and peripheral smears were negative for microbes and had no malarial parasites [ table 1 ] . based on the clinical history of conjunctivitis , fleeting rash , swelling of extremities , and multiple aneurysms of medium to small - sized vessels , a diagnosis of infantile atypical kawasaki disease was made . apart from the supportive management , the infant was started on intravenous immunoglobulin , but succumbed to death on the third day of admission . kawasaki disease or mucocutaneous lymph node syndrome is a multisystem idiopathic vasculitis affecting medium - sized vessels . the diagnosis is made based on the amalgam of clinical criteria which occur in a sequential and fleeting progression , thus proving a diagnostic challenge . the defining criteria for the diagnosis of kawasaki disease include fever for at least 5 days and four of the following five principal features : conjunctivitis , mucositis , cervical lymphadenopathy , truncal rash , and edema of the extremities . atypical forms of the disease are not rare and are defined as the disease with three principal features or coronary artery involvement with less than three principle diagnostic criteria . the higher incidence of the atypical form of the disease in infantile age group and the unusual presentations lead to a delay in diagnosis and management , consequently increasing the complication rates . coronary artery aneurysms , the most serious complication of the disease , occur in up to 25% , whereas other systemic aneurysms occur in less than 2% cases . majority of the mortality in kawasaki disease is attributed to cardiovascular events secondary to these aneurysms . aneurysm formation is a major cause of morbidity and mortality in children with kawasaki disease with increased risk in patients with delayed diagnosis , age less than 6 months or more than 9 years , male gender , prolonged ( > 14 days ) or persistent fever despite treatment , leukocytosis , elevated esr , low albumin , hyponatremia ( < 135 thus , the diagnosis of kawasaki disease is suspected on the basis of clinical features and an exclusion of other possibilities . the possible differential diagnoses in the given case of infantile vasculitis consisted of takayasu disease , fibromuscular dysplasia , infective vasculitis , and kawasaki disease . takayasu 's arteritis is a panarteritis of young adults with involvement of aorta and its main branches with the possible involvement of coronary or pulmonary vasculature . aneurysms and dilatation in aorto - arteritis commonly involve the ascending aorta and aortic arch . fibromuscular dysplasia is a congenital non - inflammatory angiogenic dysplasia with predominant involvement of the renal vasculature in young females and classically shows string of beads appearance of angiography . to conclude , this report emphasizes the role of a radiologist in emergency settings in suspecting the diagnosis and the role of imaging in diagnosis of clinically silent aneurysms in infantile atypical kawasaki disease .

kawasaki disease is a systemic medium vessel vasculitis of unknown etiology affecting children under 5 years of age . there are no specific diagnostic tests , and thus , the diagnosis of the disease is primarily made on the basis of clinical criteria . unusual presentations of kawasaki disease have been variably reported from different parts of the world . however , presentation of the disease in the form of peripheral thromboembolism and florid non - coronary aneurysms has rarely been described this report describes the imaging findings in infantile atypical kawasaki disease with aneurysms of multiple medium - sized arteries , including coronary arteries , emphasizing the detection of clinically silent aneurysms in the disease .

thymoglobulin , rabbit anti - human thymocyte immunoglobulin ( atg ) , is an immunosuppressive drug used as an anti - rejection therapy in solid organ transplantation and in hematological diseases . an association between atg and acute lung injury was first described in an experimental model in 1975 . since then , few cases have been reported from which it is believed that in rare cases , atg is responsible for a spectrum of lung injuries varying from transient infiltrate to full - blown acute respiratory distress syndrome ( ards ) . we report a case of atg - induced non - cardiogenic pulmonary edema ( ncpe ) , its diagnosis and management under general anesthesia . a 28-year - old , 50 kg man with asa risk iii was posted for laparoscopic renal transplantation . he was diagnosed to have hypertension since 2 years and chronic interstitial nephritis leading to chronic renal failure ( crf ) since 6 months and was on maintenance hemodialysis twice a week . he had undergone laparoscopic cholecystectomy and bilateral nephrectomy 2 months back without any complications . in preoperative examination , his blood pressure was controlled with nifedipine 20 mg , metoprolol 50 mg , and clonidine 0.1 mg . preoperative electrocardiogram ( ecg ) was normal nd 2-dimensionl echocardiography showed 60% ejection fraction with no wall motion abnormality . induction was done with thiopentone sodium 350 mg , and suxamethonium 75 mg was given to facilitate endotracheal intubation . right - sided internal jugular vein was cannulated by seldinger 's technique for central venous pressure ( cvp ) monitoring . continuous monitoring of ecg , pulse oxymetry , capnography , invasive blood pressure , cvp , airway pressure and temperature was done . for surgical procedure , airway pressure was 21 cm h2o after induction of anesthesia , which increased to 25 cm h2o after pneumoperitonium and stabilized at 28 cm h2o in head down position . after confirming negative reaction to test dose of atg and prophylactic administration of 100 mg iv hydrocortisone and 45.5 mg of iv chlorpheniramine maleate , 75 mg of atg diluted in 100 ml normal saline was started in central line which was to be given over a period of 4 hours . methyl prednisolone 500 mg in 500 ml of normal saline was also started as an anti - rejection therapy . intraoperatively , 3 l of normal saline and 100 ml albumin 20% was given to keep the cvp between 15 and 20 mm hg . ureteric reimplant was done . at the end of surgery , which lasted for 4.5 hours , the urine output was around 2 l. throughout the procedure , all parameters remained normal , but at the time of port closure , suddenly airway pressures were elevated up to 3540 cm of h2o . on manual ventilation , resistance was felt . gradually , oxygen saturation started falling to less than 90% even with 8 l / min of oxygen , with persistent high airway pressures . cvp line was changed to pa catheter and pulmonary capillary wedge pressure ( pcwp ) of 15 mm hg was noted . diagnosis of ncpe was confirmed . when surgery was completed , the patient was given inj . diazepam 5 mg , furosemide 100 mg iv and shifted to icu . in icu , the patient was kept on ventilator on synchronized intermittent mandatory ventilation ( pressure control)+ pressure support mode with 10 cm of positive end - expiratory pressure and 100% fio2 ) chest x - ray was done which showed diffuse infiltrates consistent with pulmonary edema . gradually , as the patient 's oxygenation and x - ray chest improved , he was weaned off the ventilator and extubated by next day morning . pulmonary edema can be cardiogenic due to increase in the net hydrostatic pressure across the pulmonary capillaries or fluid overload or non - cardiogenic due to increase in the permeability of alveolar capillary membrane . ncpe is characterized by simultaneous presence of severe hypoxemia , bilateral alveolar infiltrates on chest radiograph and no evidence of left atrial hypertension / congestive heart failure / fluid overload . the common causes are gastric aspiration , sepsis , trauma , and respiratory obstruction leading to negative pressure pulmonary edema . less appreciated is the fact that various drugs , either taken as standard therapy or as an overdose , may precipitate ncpe . the first extensive review on agents causing pulmonary insult was published in 1972 which included 20 medications . since that time bauman et al . published a paper on drug - induced lung diseases in which atg is mentioned as a causal agent for drug - induced ncpe . dean et al . and murdock reported atg - induced ards . like the other case reports , it was impossible for us to definitely prove that pulmonary edema was secondary to atg . cardiogenic pulmonary edema and fluid overload were ruled out by the absence of preexisting heart disease , good left ventricular systolic function and normal pcwp . the other causes of ncpe are less likely as it was an elective surgery , there was no respiratory obstruction and pulmonary edema developed at the end of surgery with endotracheal tube in situ . pulmonary edema developed at the time of completion of atg infusion probably due to administration of antihistaminics and steroids during surgery . although rechallenging can definitely establish the causal link , we did not believe it safe for purely diagnostic purposes . diagnosing drug - induced ncpe is actually an exercise of exclusion as there is no diagnostic test available . it is related to the time proximity of administration of drugs , and pathogenesis involves direct cytotoxic insults to the lung epithelial cells and induction of cytokine triggered inflammatory responses . various postulates are as follows : ( 1 ) atg contains antibodies of animal origin which are active against human t lymphocyte antigen , with cellular activation , degranulation and respiratory burst response damaging pulmonary endothelium . pulmonary capillary endothelial permeability increases in response to tumor necrosis factor and alpha interleukin 1 and 8 released from damaged or activated lymphocytes similar to the pathogenesis of ards in sepsis . our purpose of reporting this case is to alert anesthesiologists involved in transplantation program of this acute complication related to atg infusion so that they are prepared to treat it promptly .

non - cardiogenic pulmonary edema ( ncpe ) is a clinical syndrome characterized by simultaneous presence of severe hypoxemia , bilateral alveolar infiltrates on chest radiograph , without evidence of left atrial hypertension / congestive heart failure / fluid overload . the diagnosis of drugrelated ncpe relies upon documented exclusion of other causes of ncpe like gastric aspiration , sepsis , trauma , negative pressure pulmonary edema . we describe a 28year - old , 50 kg male with asa risk iii posted for laparoscopic renal transplantation , who developed ncpe after 4 hours of administration of rabbit anti - human thymocyte immunoglobulin ( atg ) . he was successfully treated with mechanical ventilatory support and adjuvant therapy . this report emphasizes that this fatal complication may occur with use of atg .

during august 2010december 2012 , we recruited all patients who had clinically suspected m. ulcerans infection and had attended a clinic in the buruli ulcer endemic asante akim north district in ghana . age- and sex - matched household contacts of patients were also asked to participate ; all study participants were > 5 years of age . the study protocol was approved by the ethics review committee of the school of medical sciences , kwame nkrumah university of science and technology ( chrpe/91/10 ) . whole blood samples were taken at baseline , at week 6 , and at week 12 from 66 patients in whom the diagnosis of buruli ulcer disease had been confirmed by pcr for the is2404 repeat sequence specific for m. ulcerans ( 8) ; samples were also obtained from 20 household contacts at the same intervals . the samples were heparinized , and peripheral blood mononuclear cells were separated from 10-ml samples . filarial infection was confirmed on a blood film stained with giemsa and delafield hematoxylin and examined for microfilariae at 10 and 40 magnification ( the knott technique ; 10 ) . m. perstans nematodes were distinguished from l. loa and w. bancrofti nematodes by their small size and the absence of a sheath ( figure 1 ) . m. perstans nematodes can be distinguished from loa loa and wuchereria bancrofti nematodes by relative small size , detection in blood samples obtained during the day , and lack of a sheath . patients in whom m. ulcerans infection was found were treated with 10 mg / kg oral rifampin and 15 mg / kg intramuscular streptomycin , administered daily at village health posts under direct observation for 8 weeks ( rs8 treatment ) . the patients were followed up every 2 weeks in the clinic and monitored for complete healing or recurrence of skin lesions . we compared the proportion of household controls versus the proportion of buruli ulcer patients infected with m. perstans nematodes and the time to complete healing of m. ulcerans lesions in co - infected versus monoinfected patients . categorical variables such as sex , clinical form of m. ulcerans lesion , and category of m. ulcerans lesion were compared by using the fisher exact test , and cumulative healing was compared by using the log - rank test . we found all forms of m. ulcerans disease among the group of patients ; proportions of each type and category are shown in the table . of 66 patients with m. ulcerans disease , 15 ( 22.7% ) were co - infected with m. perstans nematodes , whereas 4 ( 13% ) of 30 household controls had m. perstans infection ( p = 0.4 by fisher exact test ) . three patients in the co - infected group and none in the m. ulcerans monoinfected group reported pruritus . no other clinical signs of m. perstans infection were found . * na , not applicable . comparison of combined m. ulcerans monoinfected and m. ulcerans co - infected with m. perstans versus household contacts , determined by 2-tailed fisher exact test . comparison of m. ulcerans co - infected with m. perstans versus m. ulcerans monoinfected group , determined by 2-tailed fisher exact test . all 66 patients completed rs8 treatment , but 9 were lost to follow - up during the 12-month follow - up period . buruli ulcer lesions healed completely in 14 co - infected patients by 58 weeks ( median 20 weeks , 95% ci 14.630.2 ) and in 43 monoinfected patients by 50 weeks ( median 21 weeks , 95% ci 16.725.5 ) . we found no difference in cumulative time to healing for co - infected versus monoinfected patients ( p>0.05 by log - rank test ) ( figure 2 ) . buruli ulcer patients who had m. perstans nematodes co - infection were treated with doxycycline ( 200 mg ) and ivermectin ( 150 g / kg ) daily for 6 weeks , starting during the second to fourth week of rs8 treatment . viable microfilariae were still visible in peripheral blood mononuclear cell cultures from all co - infected patients after ivermectin and doxycycline treatment , but pruritus subsided in the 3 patients who had reported it . survival analysis curve of cumulative healing for patients with mycobacterium ulcerans infection who were co - infected with mansonella perstans nematodes compared with those who had m. ulcerans monoinfection , ghana , august 2010december 2012 . no difference in cumulative healing was found between the 2 groups ( p = 0.93 by log - rank test ) . we found co - infection with m. perstans in 23% of buruli ulcer patients in a disease - endemic district in ghana , but this prevalence was not significantly difference from prevalence among household contacts who served as controls ( 13% ) . as with buruli ulcer , m. perstans filariasis is predominantly found in rural populations and infection begins in childhood ; the highest infection rates are found in children 1014 years of age ( 11 ) , similar to those for children at highest risk for m. ulcerans infection . m. perstans infection occurs in ghana and was seen in the volta region of ghana around hohoe during the 1990s , but its prevalence is unknown ( 12 ) , and no information is available about the average number of worms per infection . in uganda , prevalence of m. perstans infection has been found to range from 0.4% to 50% ( 13 ) . m. perstans nematodes are transmitted by the bites of culicoides midges , but it is not known whether m. perstans infected midges can be co - infected with m. ulcerans . in a guinea pig model , skin penetration was shown to be a requirement for establishment of m. ulcerans disease ( 14 ) , and it has been postulated that mosquito bites cause m. ulcerans disease in australia ( 6 ) . these organisms might share a common route of transmission , but our findings in this small study do not support this concept . our findings suggest that m. perstans nematodes are common in rural ghana and coincidentally infect patients with m. ulcerans disease , necessitating the consideration of these organisms in the management plan of buruli ulcer patients . although often asymptomatic , m. perstans infection may cause eosinophilia , subcutaneous swellings , aches , pains , and skin rashes in a considerable proportion of patients ( 9 ) . because filarial nematodes are known to polarize the host immune responses from t - helper type 1 cells needed for protection against mycobacterial infections , toward humoral and t helper type-2 mediated immunity , we plan to undertake a study to investigate this interaction .

during august 2010december 2012 , we conducted a study of patients in ghana who had buruli ulcer , caused by mycobacterium ulcerans , and found that 23% were co - infected with mansonella perstans nematodes ; 13% of controls also had m. perstans infection . m. perstans co - infection should be considered in the diagnosis and treatment of buruli ulcer .

angiomyxomas are benign , locally infiltrative mesenchymal neoplasms with a predilection for the female pelvis and perineum in their reproductive age group . these tumours often reach too large dimensions before becoming clinically symptomatic and they usually tend to recur after excision . accurate preoperative diagnosis should alert the surgeon to the need for wide excision , which is essential for prevention of local recurrence . this is easily done with robotic assistance due to its advantages like three dimentional vision and advanced degree of freedom within the pelvic cavity . we report here a similar case presenting as a retrovesical tumor in a male patient , which was excised with robotic assistance . a 62-years - old male presented to us with mild obstructive lower urinary tract symptoms ( luts ) since last 1 year , with a history of acute retention of urine 3 month back with failed catheter free trial . he underwent holmium laser enucleation of prostate ( holep ) elsewhere with histopathology reporting as benign prostatic hyperplasia . patient failed to void after catheter removal and on further evaluation he was found to have pelvic mass . digital rectal examination revealed a cystic mass simulating benign prostatic hyperplasia ( grade 3 ) . magnetic resonance imaging ( mri ) pelvis revealed a well - marginated lesion within the prostate gland resulting into significant prostatomegaly with no extra prostatic extension [ figure 1 ] . transrectal biopsy was done , which was inconclusive with no identifiable prostatic tissue . in view of discrepancy between mri report and biopsy report we performed cystoscopy which showed a hump in the prostatic urethra extending into the trigone , with prostatic lobes not visible ( because of previous holep ) . tumor was ~6 cm 4 cm 2.5 cm in size [ figure 2 ] . we were not able to separate the mass from bladder due to difficulty in entering into proper plane . hence we opened bladder deliberately and resected from within the bladder , closed cystostomy and drained bladder . histo - pathological examination [ figure 3 ] revealed moderately cellular tumor composed of sheets of spindle to stellate shaped cells with vascular channels in between . on immunohistochemistry cells were positive for desmin , focal positivity with smooth muscle antibody ( sma ) , s100 and ki-67 . magnetic resonance imaging showing a well - marginated lesion within the prostate gland resulting into significant prostatomegaly , with no extra prostatic extension . also gross specimen of the tumor with gelatinous appearance and size of ~6 cm 4 cm 2.5 cm . histopathology revealed moderately cellular tumor composed of sheets of spindle to stellate shaped cells with vascular channels in between . on immunohistochemistry cells were positive for desmin , focal positivity with smooth muscle antibody , s100 and ki-67 . aggressive angiomyxoma was first described in 1983 by steeper and rosai , and fewer than 150 cases have been reported in the world medical literature . the recurrence rate is high , and often extensive resections are performed with considerable morbidity . in men , the tumour involves analogous sites including the scrotum and inguinal area and usually appears at an older age . there might be a relation with hormonal status that might explain a female to male ratio of slightly more than 6:1 . these lesions are characterized as soft , non - encapsulated tumours with finger - like projections infiltrating the surrounding soft - tissues . the tumour grows slowly and is benign as suggested by the histology and by the fact that it shows no tendency to metastasize . the low attenuation at unenhanced computed tomography ( ct ) and high signal intensity at t2-weighted mri are consistent with a myxoid matrix , high water content within the mass , or both . the enhancement of t1-weighted mri after administration of gadolinium is related to the vascularity of aggressive angiomyxoma . there is no evidence of gross fat within the mass , which distinguishes this tumor from other fat - containing tumors that also occur in the pelvis of women . surgery is usually the first line of treatment ( open , laparoscopic or robotic assisted ) , radical surgery with wide margins and long - term follow - up is advised . hormonal suppression seems to be a plausible treatment option because these tumours occur predominantly in premenopausal women of reproductive age , may grow rapidly during pregnancy and have been shown to express immunohistochemical positivity for oestrogen and progesterone receptors . their surgery is challenging because of the infiltration and the difficult dissection . in the past , most authors advocated wide excision along with genito - urinary and digestive tract resections if necessary . most recently robotic assisted laparoscopic excision has been promising , as seen in our case . male angiomyxoma , athough a rare benign tumour , should be considered in the diferential diagnosis of pelvic mass in a male patient presenting with severe obstructive luts , and robotic assisted excision should be utilised wherever available .

angiomyxoma is a rare tumour found predominantly in pelvis of young females . less than 150 cases have been reported , more than 90% in females and only few cases in males . its surgical excision is a big challenge and usually leads to recurrence due to incomplete excision . we report a case of retrovesical angiomyxoma in an elderly male . the aim of this report is to highlight the rarity of this disease , especially in males , and robotic assisted excision as an evolving option of treatment .

the first report of vaginal evisceration was described by hyernaux in 1864 , as disruption of the anterior wall of the proximal vagina , resulting in prolapse of the abdominal contents ( 1 ) . since then , there have been just over 120 reports in the literature , although in reality some cases may go unreported ( 1 ) . vaginal evisceration is a rare postoperative complication of hysterectomy ( regardless of the surgical approach ) in women of this age group that carries a high risk of morbidity and mortality and requires rapid surgical intervention . there are different aetiologies depending on the age group pre - menopausal vs postmenopausal ( 2 ) . in post - menopausal women interestingly , in pre - menopausal women there have been reports of vaginal evisceration following transvaginal ultrasonography ( 3 ) . the use of each approach depends on the viability of the bowel , whether the defect in the vaginal wall is sufficiently large to replace the bowel back into the peritoneal cavity and on any evidence of foreign bodies in the abdominal cavity . a 66 year old woman presented to a&e with per vagina bleeding , abdominal pain and a mass protruding from the vagina . prior to this presentation , the patient had suffered for the past decade with cystocoele and retrocoeles . three decades ago the patient had received an abdominal hysterectomy in order to relieve her symptoms of menorrhagia . other than arthritis , the patient does not suffer from any other medical condition and was in good health . on examination the patient seemed well , but her abdomen was distended and tender in the suprapubic , left and right iliac fossa regions . there was evidence of vaginal prolapse with a 20 - 30 cm evisceration of small bowel via a vaginal vault defect . prior to the emergency operation which aimed to salvage the small bowel , the patient was given antibiotics . the aim of the surgery was to reduce the prolapsed small bowel and re - suture the vaginal wall defect . on inspection by the consultant gynaecologist , the small bowel appeared slightly dusky and an opinion was sought from a consultant general surgeon ( who deemed the bowel healthy ) . the small bowel was replaced through the vaginal wall defect and vaginal wall biopsies taken . the patient recovered well in the first few days after the operation , though she did have some trouble maintaining the catheter in situ , due to the still uncorrected vaginal prolapse . the catheter was removed on day 5 post - operatively , but the patient had developed a urinary tract infection while catheterised and had to be on i.v . the patient was discharged on day 8 , but was to come back 6 - 8 weeks later for an elective sacrocolpopexy to correct the vaginal wall prolapse . initially exploratory laparoscopy was performed , the pelvis appeared normal and the consultant decided to perform an abdominal sacrocolpopexy . a pfannenstial incision was made , small bowel adhesions to right pelvic side wall were noted and divided . the peritoneum between sacrum and posterior vaginal wall was exposed , allowing prolene mesh to be attached to the vaginal wall with prolene and vinyl sutures . the rectus sheath was sutured using vinyl and the skin closed with staples ( with a drain in situ ) . cystoscopy was used to verify the function of the ureters , which revealed both were working well . the patient recovered quickly , there were no post - operative complications and was discharged on day 2 post - operatively . this patient had had a hysterctomy many years ago and it is unlikely her vaginal evisceration was a consequence of this surgery directly , but hysterectomy can lead , for example to changes in the vaginal axis . the vagina may come to lie in a more descended position , as it has lost part of its more proximal attachments . this new position combined with factors such as cystocoeles and retrocoeles and their management ( for example the insertion of pessaries ) may predispose the vagina walls to more wear and tear damage . these are risk factors that are likely to have contributed to this lady s transvaginal evisceration , together with her increasing age susceptibility . vaginal evisceration if not recognised quickly can lead to peritonitis and bowel ischaemia . in this lady however , it is important to make emergency doctors aware of these complications and encourage their proper management . when the patient is received by an emergency department and the problem is determined , the emergency doctors must not delay the administration of broad spectrum antibiotics in preparation for taking the patient to theatre and ensure the prolapsed bowel is covered in saline - soaked gauze , as this improves the bowel chances of viability ( 4 ) . this case illustrates the need for rapid recognition of vaginal evisceration and its appropriate management in order to avoid morbidity and mortality to the patient .

a 66 year old woman presented to a&e with per vagina bleeding and a mass protruding from the vagina . the patient was examined under anaesthesia , which revealed vaginal prolapse with evisceration of approximately 20 - 30 cm of bowel . the patient had received an abdominal hysterectomy 30 years ago for menorrhagia . in the last decade , the patient had experienced other recurrent episodes of prolapse ( cystocoele and retrocoele ) . vaginal vault evisceration is a recognised rare complication of hysterectomy and is a gynaecological emergency . this patient s condition was rapidly recognised and surgically managed . the repair was achieved in two surgeries . initially , the small bowel was re - inserted into the peritoneal cavity through the vaginal wall defect and the vaginal defect repaired . after sufficient time for healing , a sacrocolpopexy was performed to repair the prolapse .

there is an awareness for researchers of the need to share experimental data using the internet . proteomics , which is the study of the entire protein complement expressed by a genome in a cell , holds a key position in the present biology ( 1 ) . for the separation of such complex protein mixtures , 2-dimensional electrophoresis ( 2-de ) the proteins are detected by chromophoric staining and appear as colored spots on the 2-d gels that can be scanned for computerized spot detection . afterwards , to determine protein content , spots are analyzed with mass spectrometry either directly or after in - gel enzymatic cleavage for peptide mass fingerprinting . mass spectrometry results are compared with theoretical data from protein databanks allowing content identification of protein spots . thus , researchers desirous of sharing their experimental 2-de data need to display : 2-de experimental conditions , gel pictures with stained and numbered spots and , for a given spot , the protein(s ) content with appropriate links to the identification pages in databanks . when considering the high and always increasing amount of results obtained from a single 2-de gel ( commonly up to 1,000 spots ) , the more convenient way for storing , organizing , and displaying them lies undoubtedly in internet databases . php , a recursive acronym that stands for php : hypertext preprocessor , is a widely used general - purpose html - embedded scripting language that especially suits for web development . the code is executed on the server side and allows to dynamically generate pages , in particular , using information accessed from databases . now , most of the web hosting companies offer the opportunity to use mysql databases and dynamic languages such as php . thus , php could facilitate 2-de presentations on web sites because information can be stored in a database and assembled using php commands into web pages on the fly when requested . it also allows to create user - friendly interfaces to store easily content into the database tables . we have developed a php - written module , namely phproteomicdb , that can be used in the purpose to share 2-de data without knowledge for php or database manipulations . it can display smartly online 2-de gel data , including gel characteristics , gel pictures , and numbered spots with their related identifications pointing to their reference pages in protein databanks ( swiss - prot or ncbi ) . using phproteomicdb implies that your web site hosting company must have php - mysql enabled server , which is usually the case . no technical or php knowledge is required except a few basics about web site management . download the last compressed version of the module from http://www.huvec.com/index.php3?rub=download and unzip the downloaded file on your local drive to obtain : ( 1 ) a folder ( phproteomicdb ) , ( 2 ) a file for data table creation ( proteomicdb.sql ) , and ( 3 ) a readme.pdf file for ultimate detailed instructions . 2 . upload the phproteomicdb folder to your web site root directory using any ftp software , after having updated the mysql database configuration file with your own parameters ( login , password , ) . 4 . execute the proteomicdb.sql file using the phpmyadmin interface of your mysql database to create the data tables to store your 2-de gel data . fill these tables with data of your gels , spots , and proteins in the user - friendly administration area using forms and drop - down menus upload your pictures and documents ( gel pictures in jpg format , pdf documents , ) in the appropriate folders of the module . when achieved , click the link installed on your web site at step 3 to display the module index page with pictures and characteristics of the 2-de gels . when clicking one of these pictures , the list of the spots and the link with the attached pdf document related to this gel are displayed . finally , clicking a spot number allows to access a new page that displays the identified proteins in this spot ( figure 1 ) with hypertext links to their reference pages in the protein databanks ( swiss - prot or ncbi ) . if necessary , specific pages in the administration area allow canceling or updating data . for every page generated with the module , the retrieved data are merged with page templates that provide minimal components controlling the style of presentation . thus , to respect his / her web site graphic chart , the webmaster is free to edit the php pages displaying his / her data ( index.php3 , 2dpatterngel.php3 , and identification.php3 ) to add html codes for style or decoration . as an example what distinguishes our work from others is that it allows researchers that are newcomers in web site management to benefit from the power and the flexibility of the php dynamic language and the efficient use of databases . for people who are reluctant to use complex tools such as the make 2d - db ii package ( 2 ) , which is a federated database for 2-de users , the development of the module is being pursued with future updates with the latest version available on our web site at http://www.huvec.com/index.php3?rub=download .

phproteomicdb is a php - written module to help researchers in proteomics to share two - dimensional gel electrophoresis data using personal web sites . no technical or php knowledge is necessary except a few basics about web site management . phproteomicdb has a user - friendly administration interface to enter and update data . it creates web pages on the fly displaying gel characteristics , gel pictures , and numbered gel spots with their related identifications pointing to their reference pages in protein databanks . the module is freely available at http://www.huvec.com/index.php3?rub=download .

a 22-year - old - male patient presented with a painless swelling over the left eye along with mechanical ptosis of the upper lid . the mass was located anterior to the orbital rim in the superolateral part of the orbit ; with the posterior edge not palpable as it was within the orbit . anterior segment evaluation , intraocular pressure , and fundus examination of both eyes were normal . a computed tomography showed an iso - to - hypodense mass lesion in the superotemporal aspect of the left orbit , arising possibly from the lacrimal gland since the gland could not be clearly delineated from the mass . no obvious changed to the bony orbit could be noted on the scans [ fig . fine needle aspiration cytology smears drawn showed no malignant cells ; however , a conclusive diagnosis could not be made . a working diagnosis of a pleomorphic adenoma of the lacrimal gland was made , and an excision biopsy was performed . intraoperatively , the mass was pink , vascular , and well circumscribed but not encapsulated . the lacrimal gland was seen in close relation to the mass , compressed between the mass and the superolateral orbital bony rim . ( a ) external clinical photograph shows the mass located in the superolateral quadrant of the orbit . ( b ) axial slice of the computed tomography scan showing the mass in the vicinity of the lacrimal gland ( yellow arrow ) the cut surface was homogeneous and did not have any distended vascular channels , lacrimal tissue , or cystic dilatations . on microscopic examination , spindle cells in compact sheaves closely opposed to thin walled vessels were seen in a fibrocollagenous background [ fig . the nuclei were open , oval , and contained small , uniform nucleoli [ fig . . no necrosis was noted , and a few engorged vessels were seen in the outlying area . immunohistochemical studies showed that the tumor cells stained positive for cd 34 and cd 31 , which are markers for endothelium [ fig . furthermore , the tumor stained positive for smooth muscle actin and negative for cd68 [ fig . immunostaining was negative , and the ki-67 proliferation index was low with < 5% of the cells staining positive [ fig . thus , the tumor had no conclusive features of a malignancy and the final diagnosis based on its microscopic appearance , and immunohistochemical characteristics was that of a solid variant of angioleiomyoma of the orbit . as it had been excised completely , no further treatment was warranted . at 1-year follow - up , no recurrence was observed . ( a ) low power view showing spindle cells in a background of fibrocollagenous tissue ( h and e , 10 ) . ( b ) high power view demonstrating uniform oval nuclei ( h and e , 40 ) ( a and b ) immunohistochemical studies showed that the tumor cells stained positive for cd 34 ( endothelium ) and cd 31 ( 10 ) ( a and b ) immunohistochemical studies positivity for smooth muscle actin and immunonegativity for cd68 ( 10 ) . ( c ) the ki-67 proliferation index was low with < 5% of the cells staining positive ( 10 ) the differentials for such an orbital tumor could be cavernous hemangioma , angiomyofibroma , or complex orbital angiomyoma . the tissue of origin of angioleiomyoma is considered to be smooth muscle ; so the possible native tissues from which the tumor could have arisen include blood vessels , pericytes , mller 's muscle or the capsulopalpebral muscle of hessar . angioleiomyoma is well recognized within the spectrum of vascular lesions of the soft tissues and has been subdivided into three types solid , venous , and cavernous . since there were no dilated cavernous spaces within our tumor , we consider our case to be a solid variant . morimoto , who classified angioleiomyomas , observed that solid variants are often painful and seen in the extremities . in contrast to his comments , our case was a solid variant which was painless . described an angiomyofibroma of the orbit as a hybrid tumor exhibiting characteristics of a vascular leiomyoma and cavernous hemangioma . the lack of the cavernous venous channels , the unusual location , and absence of the classical purplish hue rule out cavernous hemangioma and angiofibroma in our case . jakobiec has also described in detail the characteristics of a complex orbital angiomyoma , which had features of a lymphangiohemangioma . three of them were located in the muscle cone , two of them were located in the superotemporal orbit , and one was located in the inferior orbit . two other eyelid angioleiomyomas were also described in the same series . of these eight cases , based on their histological classification , however , only one case was the same type as our case : the solid variant ; five cases were of the cavernous type and two cases were venous type . alam et al . , have also reported a similar tumor from the anterior orbit , which was found to be of the cavernous subtype . preoperative magnetic resonance imaging may provide some clues : magnetic resonance findings of peripheral angioleiomyomas were relatively nonspecific , but t2-weighted images show a mass with mixed areas that are both hyper- and iso - intense relative to the skeletal muscle and a hypointense rim . angioleiomyomas of the orbit are rare tumors with good prognosis , and the treatment of choice remains complete surgical excision .

we describe the clinicopathological features of a solid variant of orbital angioleiomyoma . a review of clinical records , diagnostic , and radiographic studies combined with histopathological evaluation with standard histochemical staining and immunohistochemistry was conducted . a 22-year - old male patient presented with a mass in the region of the left lacrimal gland that was gradually increasing over the past 2 years . radiological and clinical examinations showed no signs suspicious of a malignancy and fine needle aspiration cytology was inconclusive . therefore , an excision biopsy was performed . on histopathological examination , the picture was consistent with a benign spindle cell tumor . immunohistochemistry showed positivity for cd 34 and cd 31 ( markers for vascular endothelium ) . the tumor also showed positivity for smooth muscle actin and ki-67 proliferative index was low . angioleiomyomas are rarely encountered in the orbit and has features seen in leiomyoma as well as some vascular tumor elements . in most cases , surgical excision is usually curative .

the bacterial isolates were collected throughout finland during 19982007 as described ( 10 ) . of bionumerics version 5.1 software ( applied maths , kortrijk , belgium ) was used for sequence assembly . allele numbers , sequence types ( sts ) , and clonal complexes ( ccs ) were assigned by using the pubmlst database ( 5 ) . a serum sensitivity assay was conducted with 73 c. jejuni isolates according to a described protocol ( 8) . the same pool of serum samples from 10 healthy blood donors was used in all experiments . c. jejuni all statistical analyses were performed by using graphpad prism version 4.03 ( graphpad software , san diego , ca , usa ) and pasw statistics version 18 ( spss inc . , chicago , il , usa ) . a total of 72 c. jejuni isolates from blood were successfully typed by mlst ; 1 isolate had a mixed mlst pattern . five isolates were in unassigned sts , and the rest were distributed among 11 ccs ( table ) . genetic relatedness of these isolates was further confirmed by using pulsed - field gel electrophoresis . * mlst , multilocus sequence typing ; st , sequence type ; nd , none detected . however , bacteremia episodes caused by st-677 cc isolates were exclusively diagnosed during the seasonal peak during may august ( figure 1 ) . of c. jejuni blood culture isolates detected during may august , most ( 64% ) were st-677 cc . furthermore , st-677 cc was the most prevalent complex in 4 geographic regions of finland . annual and seasonal distribution of 72 camplyobacter jejuni blood culture isolates belonging either to the st-677 clonal complex ( cc ) or to the other multilocus sequence typing ( mlst ) ccs . one isolate with a mixed multilocus sequence type was not included . c. jejuni bacteremia was diagnosed during may august ( m a ) or during any other month of the year ( o ) . susceptibility to human serum varied between c. jejuni isolates from different ccs ( figure 2 ) . st-677 cc isolates were significantly less susceptible to human serum than all other isolates ( p<0.0001 ) . st-45 cc isolates were significantly more susceptible to human serum than all other isolates ( p<0.0001 ) . percentage of surviving bacteria in human serum for 73 blood culture isolates of campylobacter jejuni ( cj ) , grouped according to major multilocus sequence typing clonal complexes ( ccs ) , and for controls c. jejuni cj11168 and c. fetus . we characterized a unique collection of 73 c. jejuni isolates from blood obtained during a nationwide study in finland over a 10-year period . despite the high population diversity of c. jejuni , nearly half of the isolates from blood showed clustering within the st-677 cc , a rare cc in other countries ( 12,13 ) . thus , invasiveness of blood culture isolates could not be solely explained by their serum resistance , although the predominant isolates of st-677 cc were more serum resistant than other isolates . c. jejuni has high st diversity . as of may 2 , 2013 , a total of 6,564 sts were registered ( 5 ) . in this study , we detected clustering of c. jejuni isolates from blood in an uncommon st-677 cc . further studies are needed to clarify whether bacterial characteristics might explain this finding . in our previous study , which included human fecal c. jejuni isolates obtained in finland from the mid-1990s through 2007 , which is nearly the same period as in the current nationwide study , 11.7% of the isolates belonged to st-677 cc ( 11 ) . the 2 most prevalent ccs in that study , st-45 cc ( 43.6% of fecal isolates ) and st-21 cc ( 19.4% of fecal isolates ) , were detected only among 12 ( 16% ) and 10 ( 14% ) of blood culture isolates , respectively , in the present study . st-45 cc and st-21 cc have been shown to be prevalent in several countries ( 4,13 ) . however , our results suggest that these 2 ccs are not common among c. jejuni isolates from blood in finland , which cluster more in the st-677 cc . on the basis of the present results , we speculate that st-677 cc might have a special invasive capability or has adapted to the environment in finland . in general , complement - mediated killing of serum - susceptible isolates plays a major role in restricting access of pathogens to the bloodstream . however , available information about possible serum sensitivity of c. jejuni isolates from blood is scarce ( 8,9 ) . in our study of nonselected c. jejuni isolates from blood , susceptibility to human serum varied according to mlst cc . in conclusion , in this nationwide study during a 10-year period in finland , we found by mlst analysis that half of the bacteremia isolates of c. jejuni clustered within an otherwise uncommon st-677 cc . whether this finding indicates special adaptation of st-677 cc to finland or to the human bloodstream our findings emphasize the role of using well - defined clinical materials in studies on bacterial pathogenicity and severity of human disease .

campylobacter jejuni bacteria are highly diverse enteropathogens . seventy - three c. jejuni isolates from blood collected in finland were analyzed by multilocus sequence typing and serum resistance . approximately half of the isolates belonged to the otherwise uncommon sequence type 677 clonal complex . isolates of this clonal complex were more resistant than other isolates to human serum .

gastrointestinal stromal tumours ( gists ) are rare tumours of the gi tract , making up 0.21% of gastrointestinal malignancies . first identified by mazur and clark in 1983 , these mesenchymal tumours can occur anywhere along the gastrointestinal tract [ 1 , 3 ] . relative rarity combined with non - specific presentation results in delayed diagnosis [ 4 , 5 ] . we present the first case of gist leading to intussusception at the jejuno - ileal junction in an otherwise well patient prior to presentation . a 47-year - old female presented as an emergency with severe abdominal pain and profuse vomiting . she had been experiencing symptoms of intermittent vague abdominal pain associated with weight loss over 6 months , but no change in bowel habit . pyrexial and abdominal examination revealed distended abdomen with localized tenderness in the left iliac fossa . blood tests revealed an elevated white cell count ( 18.8 ) , c - reactive protein ( 25 ) , microcytic anaemia ( hb 108 ) and a mildly raised lactate ( 2.37 ) . she was commenced on intravenous fluids . an abdominal plain film showed prominent bowel loops and ultrasound of the abdomen revealed numerous loops of a distended , fluid filled , non - peristalsing bowel . there was some evidence of bowel wall thickening , but no clear source of obstruction was identified . a ct scan was performed showing mesenteric vascular gas and a target sign , virtually pathognomonic of intussuception [ 5 , 6 ] ( figs 1 and 2 ) . the patient underwent a laparotomy , which revealed small bowel obstruction with evidence of impending perforation . a pedunculated polypoid lesion at the jejuno - ileal junction was identified as the lead point for intussusception . the intussuscepted segment of small bowel was resected and end - to - end primary anastomosis formed . histological findings were in keeping with a 75-mm gist , jejuno - ileal in origin , with a mitotic count of < 5/mm and a moderate ( 24% ) risk of recurrence . the tumour was positive for cd117 and carried a c - kit mutation with a deletion in exon 11 . intussusception is the telescoping of a proximal loop of bowel and its mesentery ( intussusceptum ) into the lumen of bowel distal to it ( intussuscipiens ) . the phenomenon is rare in adults and presents with non - specific symptoms , such as recurrent abdominal pain , nausea and vomiting . up to 90% of adult cases have a defined lesion acting as the lead point , which is malignant in overhalf of cases [ 1 , 6 ] . while correct diagnosis is based on operative findings , suspicion may be raised following imaging techniques . ct with both oral and intravenous contrast is now widely recognized as the most accurate diagnostic tool ( 58100% accuracy in a recent case series ) [ 1 , 4 ] . alternating hyper- and hypodense layers of the bowel wall of the intussuscepted segment give rise to a classical gists are rare tumours demonstrating a broad spectrum of invasiveness [ 5 , 7 ] and unpredictable behaviour , with recent suggestion that these tumours should no longer be classified as benign or otherwise . classically gists grow exophytically , into the peritoneal cavity or adjacent organs , further decreasing their likelihood of causing an intussusception . rarely , however , gists can grow as a pedunculated polyp , as in our case , which may go on to act as a lead point for an intussusception . their growth pattern leads to a range of non - specific symptoms ( much like an intussusception ) , meaning gists often remain unnoticed until advanced enough to cause complications such as ulceration , obstruction or gi bleed . gists originate from a malignant transformation of the interstitial cells of cajal [ 1 , 3 ] . 95% of cases show a mutation in the kit proto - oncogene , setting them apart from leiomyomas , leiomyosarcomas and schwannomas of the small intestine . furthermore , near universal expression of cd117a product of the kit proto - oncogene which makes up part of the kit transmembrane receptor tyrosine kinase is found . again following the relatively recent discovery of this immunohistochemical profile , management of gists has been transformed . imatinib , a targeted tyrosine kinase inhibitor , has revolutionized treatment since its introduction in 2001 . universal guidelines for its use are not currently available ; however , it has a clear role in both adjuvant therapy and neoadjuvant therapy for unresectable or borderline resectable tumours [ 1 , 3 , 9 , 10 ] . prior to the introduction of imatinib , surgery was the only treatment available and to this day offers the only chance of cure . surgery is offered for all tumours over 2 cm in diameter , along with a role in debulking or symptomatic relief . below 2 cm the natural history of these rare tumours is relatively unknown ; therefore , treatment planning is difficult . given the rarity of spread to lymph nodes , regional lymphadenectomy has not been found to be useful . intussusception is a rare finding in adults , even more so when occurring secondary to a gastrointestinal stromal tumour . we present the first case of a jejuno - ileal intussusception secondary to a gist in an otherwise well patient . diagnosis can be difficult given the non - specific nature of the symptoms , and while different imaging modalities may be useful in evaluating these patients , a firm diagnosis can not be made until histological analysis . curative treatment can only be offered by surgery ; however , imatinib has a role in both adjuvant and neoadjuvant treatment .

gastrointestinal stromal tumours ( gists ) are rare tumours , making up 0.21% of gastrointestinal malignancies [ zakaria and daradkeh ( jejunojejunal intussusception induced by a gastrointestinal stromal tumour . case rep surg 2012;2022:173680 ) ] . their relative rarity combined with non - specific presentation results in tumours often remaining undiagnosed until surgery or histological examination [ martis et al . ( a rare case of jejunojejunal intussusception in an adult . indian j surg 2013;75(suppl 1):1820 ) ] . presentation as a lead point for intussusception is particularly rare . we present the first case of gist leading to intussusception at the jejuno - ileal junction in an otherwise well patient prior to presentation . provisional diagnosis was made during emergency laparotomy , and confirmed through histological analysis . a typical immunohistochemical profile was identified , after which the patient was commenced on adjuvant imatinib therapy . we discuss classical presentation of intussusception and gist . further considerations of the investigation and treatment options of gists are also presented .

it is a common practice in south east asia to consume alternative medicine which may include toxic metals or animal products for various health reasons . herein we report a case of anuric acute renal failure with dyspnea and hemoptysis following volitional consumption of rohu fish ( labeo rohita ) gall bladder . forty two year old male chronic alcoholic , smoker and marijuana addict presented with history of diffuse abdominal pain with profuse vomiting followed by oliguria . he was treated with intravenous fluids and diuretics in a primary health centre near nagpur . as he developed edema feet , puffiness of face , breathlessness and hemoptysis he was transferred to our tertiary care hospital . there was no history of fever , joint pains , rash , jaundice or bleeding from other sites . on admission , examination revealed medium frame , poorly nourished patient with pallor and no icterus . investigations revealed hemoglobin-11.4 gm / dl with total white blood cell count-9600/cumm and platelet count-80000/cumm . urine routine showed protein 2 + with 20 - 25 red blood cells and 70 - 80 white blood cells per high power field . renal chemistry revealed blood urea nitrogen-160 mg / dl , serum creatinine-16.8 mg / dl , serum sodium-113 meq / l , serum potassium-3.3 meq / l , total proteins-6.6 g / dl with albumin 3.4 g / dl , ast / alt-56/42 iu / l , serum bilirubin-1.1 mg / dl , serum uric acid-11.6 mg , serum calcium-8 mg / dl and serum phosphate-5.5 mg / dl . ultrasonography of the abdomen showed normal size kidney with no dilatation of pelvi - calyceal system . as patient had hemoptysis and acute kidney injury there was a strong suspicion of pulmonary renal syndrome . however his igg anti - glomerular basement membrane ( gbm ) antibody was 1.8 u / ml ( negative < 12 u / ml ) . a plain computerized tomography ( ct ) of the chest showed patchy perihilar ground glass opacities with peribronchial cuffing , segmental atelectasis of bilateral lower lobes with bilateral pleural effusion . persistent enquiry as regards a possible toxic etiology for his acute onset complete anuria revealed he had consumed the entire bile from a raw fish gall bladder ( rohu or labeo rohita ) a day prior to the onset of symptoms as a cure for his general debility . in view of his pulmonary edema and deteriorating renal function he was dialysed once on admission after which there was a gradual increase in urine output over 6 days . he improved symptomatically with a decrease in serum creatinine from 16.8 mg / dl to 10 mg / dl on day 5 to 4.8 mg / dl on day 15 . he was eventually discharged after 18 days of in - hospital treatment with serum creatinine of 2.5 mg / dl . subsequent follow - up after 15 days showed serum creatinine was 1.08 mg / dl . fish gall bladder is considered a traditional medicine to treat rheumatism , decreased visual acuity , and urticaria and to increase sexual vitality . however it can lead to acute renal failure and acute liver injury as reported in cases of fish poisoning from india , japan and hong kong . the ichthyotoxic effects of fish gall bladder is attributed to the presence of a toxin , sodium cyprinol sulfate which is a c27 bile acid . the toxin is heat stable and insoluble in alcohol as cases are reported even after consumption of cooked bile . the family includes grass carp ( c idellus ) , common carp ( cyprinuscarpio ) , silver carp ( hypophthalmichthysmolitrix ) , mylopharyngodonpiceus , labeorohita and aristichthys nobilis . amongst these , fish of the grass carp variety has been commonly reported for its toxicity but xuan et al . also reported toxicity secondary to ingestion of shark ( minnow ) fish ( m chrysophekadion ) and the bony - lipped barb fish ( o melanopi ) . even though fish gall bladder can cause severe systemic complications , toxicity does not occur in all cases . in rural vietnam , gall bladder from smaller fish are consumed regularly without evident toxicity . thus it can be said that toxicity is directly proportional to the size and quantity of gall bladder or bile consumed . following ingestion almost all patients develop gastrointestinal symptoms within a few hours as reported by xuan et al . in his series of 17 cases from vietnam . has also reported extra - renal manifestations of the toxin with deleterious effects on the heart and liver with eventual multi - organ dysfunction syndrome . however patients usually seek therapy for renal failure which has an incidence of 55 - 100% . renal failure is mostly secondary to acute tubular necrosis which may be a direct effect of the toxin . kidney biopsy has shown the presence of proximal tubular cell damage with focal destruction of epithelial cells on light microscopy . the toxin is believed to damage lysosomes and inhibit cytochrome oxidase thus blocking cellular metabolism , leading to cell necrosis . this in addition to loss of fluid can lead to decreased effective circulating blood volume and eventually renal failure . however the exact mechanism of systemic injury especially renal failure is not known and needs further study . our patient had reported hemoptysis prior to admission and had an abnormal ct scan of the chest . the cause for the same in such a setting could include fluid overload - pulmonary edema ( anuria ) or pulmonary renal syndrome . toxic bile is also reported to cause pulmonary renal syndrome however the patient 's primary workup including ana , c3 , c4 and igg anti gbm was normal . toxin induced pulmonary renal syndrome remains a possibility however a workup for the same including kidney biopsy and bronchoalveolar lavave was not done in view of steady improvement in his condition . most of the reported cases till date have undergone hemodialysis in view of renal failure pandey et al . in their case good awareness programs can aid diagnosis and prevent death from such a dangerous preventable cause of acute kidney injury which is the aim of international society of nephrology 0 by 25 initiative .

a forty two year old male was admitted with history of anuria and breathlessness following consumption of raw rohu fish gall bladder . he had azotemia and required hemodialysis . his renal failure improved over a period of about four weeks . incidences have been reported from south east asian countries associating consumption of raw rohu fish gall bladder with acute renal failure .

uterine fibroids are the most common uterine neoplasm of uterus and the female pelvis and the most common indication for hysterectomy as well . when the tumor fills the whole pelvis or is pedunculated , there is always an uncertainty regarding the uterine or ovarian origin of the tumor . a cervical fibroid especially with degenerative changes mimics an ovarian tumor and causes a clinical dilemma . a 40-year - old woman , p 2 living 2 , presented in the department of gynaecology with the complaint of progressively increasing distension in abdomen for last 1 year . surprisingly , there were no complaints regarding change of menstrual pattern , urinary or bowel habits , anorexia or fever . on examination , a huge abdominal mass occupying whole of the hypogastrium , both iliac fossa , umbilicus , and left hypochondrium , the mass was 10 8 inches in size , smooth surfaced with regular margin , soft to firm in consistency , nontender , side to side mobile , lower limit not reachable , and engaged in the pelvis more toward the left side . the same mass was felt obliterating all the fornices ; uterus was easily palpable of normal size but dextro deviated . ultrasonography revealed a huge complex mass arising from the pelvis and extending into the abdomen occupying almost whole of the abdomen with well - defined margins ; complex internal echotexture , predominantly echogenic with multiple poorly hypoechoic areas along with two cystic areas , each measuring around 4 cm 5 cm in size . a normal size uterus was seen pushed to the right side with normal central endometrium . clinically provisional diagnosis of the benign ovarian tumor was made based on doppler and biomarker ca 125 levels . after preanesthetic work up , the patient was taken up for laparotomy . per operatively soft mass of size 12 10 inches with intact external capsule was seen , growing into the leaves of left broad ligament and extending above the umbilicus . both ovaries , right tube and uterus was normal but left fallopian tube was stretched over the mass . on opening the broad ligament leaf , it was found to be arising from posterior surface of upper part of cervix . mass was shelled out followed by total hysterectomy with left - sided salpingo - oophorectomy [ figure 1 ] . cut section showed few solid areas along with multiple cystic areas , one containing black tarry material suggestive of red degeneration . surgical specimen showing normal uterus with huge cervical fibroid histopathology of the mass revealed benign spindle cell tumor ( leiomyoma uteri ) with red , cystic , myxoid and hyaline degenerations and dystrophic calcification [ figure 2 ] . histology : spindle cells tumor ( leiomyoma ) with cystic , fatty , myxoid , and red degenerations fibroids extending into broad ligament are rare and may mimic an ovarian tumor . it is known that degenerative changes result in unusual appearance that adds to diagnostic confusion . pedunculated uterine , cervical , and true broad ligament fibroid , especially with degeneration , may be mistaken for lesion of ovarian origin and therefore must be kept in the differential diagnosis . cervical fibroids pose enormous surgical difficulty by virtue of their relative inaccessibility and proximity to bladder and uterus and distorting the normal anatomical relationships . as in this case it bulges outward between the layers of broad ligament , displacing uterine artery outward and upward and ureter outward toward the pelvic side wall .

uterine fibroids are the most common uterine neoplasm of uterus and the female pelvis and the most common indication for hysterectomy worldwide . when the fibroid is large and fills the whole pelvis or is pedunculated , it becomes difficult to differentiate from ovarian mass clinically . cervical fibroids pose enormous surgical difficulty by virtue of their relative inaccessibility and proximity to the anterior bladder , posterior rectum , and distorting the normal anatomical relationship of pelvic structures . a cervical fibroid especially with degenerative changes mimics an ovarian tumor and poses a clinical dilemma . this case is also exemplified for its rarity and diagnostic difficulty .

the influence of culture on the development of eating disorders such as anorexia nervosa ( an ) and bulimia nervosa ( bn ) has been long appreciated . these syndromes are more prevalent in industrialized , and often western cultures and are far more common among females than males , mirroring cross - cultural differences in the importance of thinness for women . these patterns indict current cultural beauty ideals in the etiology and maintenance of eating disorders . a secular trend of increased prevalence of bn is observed in the west during the current century , and the recent point prevalence of bn is around 1% of young western women . with another 3 - 5% suffering from similar eating disorders , known as eating disorder not otherwise specified ( ednos ) in the diagnostic and statistical manual - iv ( dsm - iv ) . only few classical cases has been reported so far from the asian countries , particularly having more western influence , such as japan , hongkong . atypical case of bn has recently been reported from india . to our best knowledge , a 22-year - old , unmarried female medical undergraduate , belonging to an urban hindu extended nuclear family of the upper socio - economic status from a metropolitan city , with predominantly narcissistic and a histrionic traits and family history of recurrent depressive disorder in paternal grandmother , presented with the poor eating habits of insidious onset for 9 years . during her 8 class , she developed liking for a boy in her class who rejected her calling fat . though , she managed to move on ; however , developed dissatisfaction for her body image , and would consider herself fat on the mirror and started looking for means to reduce weight . with gradually increasing concern over growing fat , she started skipping two meals and would take only one meal and salads in class 10 . over next 6 - 7 month period , she lost up to 12 kg and looked thin , although she would consider it inadequate and would find herself flabby , in front of the mirror , although at other times , she could appreciate that her clothes had become loose . however , she never had symptoms of micronutrient deficiency or menstrual irregularity . at the same time , she also developed intense liking for the high calorie foods . she would binge on them 5 - 6 times a month and would regret afterwards . she started exercising for 1 - 1 h in order to compensate weight gain out of binging . this pattern continued for next 1 - 1 when she gave up working out unwillingly , to focus more on studies , and she gained about 4 - 5 kg . she restarted dieting ; however , within few months she again started having increased craving for the high calorie foods and binging , which would be more when she would deny food in parties . though she knew that her body mass index ( bmi ) was well within normal range , she started taking one tablet of orlistat daily secretly along with skipping meals and rejoining gymnasium in order to reduce her weight to below 50 kg , which was below normal for her height . she could undergo a single session after which it came to the knowledge of a family member , who refrained her . during last 5 year , she would compare herself with every female she met or read about in novels , would feel better on seeing obese females , and feel let down if they were slim . , she would avoid parties , going out with friends , standing for photos , and would spend hours in the gymnasium . at the time of consultation in the psychiatry out - patient department , her bmi was 23 , which is within normal range . her laboratory investigations including , complete hemogram , liver , and renal function tests , serum electrolytes , plasma blood glucose levels were normal . she is under regular opd follow - up with sustained improvement since last 18 weeks . this case is a typical case of bn with obvious presence of a morbid dread of fatness , body image dissatisfaction and setting a sharply defined weight threshold and binging associated with compensatory behavior . rapid and sustained improvement with the low - dose fluoxetine and cognitive behavioral therapy as observed in this case is usually not seen . despite ongoing adoption of western values world - wide , body dissatisfaction is remarkably lower in non - western countries . study on indian medical students by the srinivasan et al . found 15% of the 210 students had a form of distress and disorder in attitude towards eating habits and body weight , which are milder or subtle than an or bn . the author termed this as eating distress syndrome . pary - jones , referring to the historical evolution of eating disorder have mentioned about its archaic form , a less sever and benign form of an or bn . the authors stated that the current severe form of eating disorder such as an , bn might have emerged form of this archaic form . this historical evolution of major eating disorder from older form had been observed in studies carried out across different culture and region over different periods of time . hence , it is possible that major eating disorder might be in evolution phase in countries like india , and largely present here in its archaic form . however , this case may be taken as an indicator of emergence of bn in the context of rapidly increasing western influence in india .

a classic case of the bulimia nervosa in a young indian female is reported . this is in the context of the impression that due to increasing western influence , and change in cultural concepts of beauty and thinness among women , illnesses previously considered rare in indian subcontinent might be becoming more prevalent . many of the established pre - disposing factors such as female gender , metropolitan domicile , family history of depressive disorder have conglomerated in this case . rapid and sustained improvement with the low - dose fluoxetine and the cognitive behavioral therapy is also worth paid attention .

the altered postoperative physiology after pneumonectomy interventions can cause severe implications , especially in patients receiving adjuvant therapy for advanced lung cancer . a 60 year - old man with malignant pleural mesothelioma underwent a thoracotomy through double incision ( 5 and 7 intercostal spaces ) for left pleuro - pneumonectomy and diaphragmatic resection after chemotherapy . the pre operative chest computed tomography scan showed a pattern of centrolobular emphysema . a thoracic epidural catheter was placed before anaesthesia induction and a mixture of ropivacaine 0.375% and fentanyl was used during the intervention and postoperative period . parietal pleural dissection produced a significant blood loss and red blood cells and fresh frozen plasma transfused . the patient did not meet the extubation criteria and was transferred to the intensive care unit . on intensive care unit admission the patient was hypotensive ( blood pressure= 90/60 mmhg ) and treated with fluids . chest x - ray was normal ( figure 1 ) and the patient was extubated 40 minutes thereafter . this figure shows the first chest x - ray ( immediately after the admission in the intensive care unit ) . an hypotensive episode ( blood pressure 60/40 mmhg ) and tachypnea ( respiratory rate = 32 breaths per minute ) were treated with fluids , dopamine ( 5 mcg / kg / min)and interruption of the epidural infusion . a chest x - ray with contrast administration trough nasogastric tube was performed . respiratory arrest and asytolia the chest x - ray showed gastric and spleen herniation through a diaphragmatic breach ( figure 2 ) . this figure shows the diaphragmatic herniation ( the chest xray was performed immediately before cardiorespiratory arrest ) . dopamine infusion was interrupted during the operation , the patient was extubated in the intensive care unit six hours later discharged from the hospital on the 6th post operative day without further complications . the mortality rate after pneumonectomy is 6% , the major causes of death being pneumonia , pulmonary edema , pulmonary embolism , myocardial infarction , empyema and bronchopleural fistula . gastric hernia is a rare postoperative complication usually diagnosed at the first postoperative chest xray . in our case the diagnosis was challenging because the hernia became evident only after intensive care unit admission and because the epidural analgesia confused the clinical scenario : this technique was considered the cause of persistent hypotension . the left pneumonectomy likely created a vacant space into which the stomach acutely herniated compressing the heart and creating a cardiac tamponade . in patients with hypotension after left pneumonectomy a high index of suspicion should be observed for diaphragmatic hernia during the whole postoperative period .

patients undergoing pneumonectomy can suffer by cardiovascular and respiratory postoperative complications that can affect patient s outcome by increasing morbidity and mortality . we describe a diaphragmatic hernia occurring after pneumonectomy . with late presentation and with epidural analgesia confusing the scenario suggesting that anesthesiologists should remain aware on this complication even in the late post operative period .

a 19-year - old , single , unemployed , caucasian female ( skin type iii ) presented with multiple facial lesions . she had been suffering from multiple pigmented annular papules and plaques on her face for 7 years . she had been a normal , healthy , full - term baby and had normal physical milestones of development as a child . she appeared to be immunocompetent with no history of recurrent infections or any other skin lesions . her parents and siblings ( 3 brothers and 2 sisters ) were all normal and healthy . her medical history was unremarkable with no other obvious complaints , and she was not overexposed to sunlight as she spent most of her time indoors . upon examination , the central part of the face , i.e. the malar area and nose including the ala nasi , showed scattered annular papules and plaques of different sizes ranging from 0.3 to 2 cm . the lesions were asymptomatic and symmetrically distributed on both sides of the face ( figs . 1 , 2 ) . the diagnosis was confirmed as dsap , with a classic and very illustrative pathological picture ( figs . all physical modalities were excluded to avoid the possibility of side effects like scarring and disfigurement , as the lesions were located in the central part of the face and involved the skin overlying the nasal cartilage . we first prescribed a topical retinoic acid 0.1% cream that has keratolytic and antineoplastic effects , and it was assumed that this might rectify the faulty clonal epidermopoiesis . six months later , the patient presented again and the picture was almost unchanged except for a few small new lesions in the malar area and on the nose . we decided to give her imiquimod 5% cream to be used once a day ( 3 times per week ) for 24 weeks . side effects like erythema , crustations and pruritus were experienced and were controlled by topical emollient cream ; we avoided corticosteroids and calcineurin inhibitors because they can counter the effects of imiquimod . on completion of the therapy , central scarring and the scars across the nose improved in their color , thickness and texture , constituting an added benefit of imiquimod in this indication . porokeratosis is inherited as an autosomal dominant trait ; however , sporadic cases are also known to occur . chernosky and freeman first described dsap in 1967 in a texas population . dsap is the most common of the clinical variants and may account for almost half of all cases in the usa , while in singapore it is second to the mibelli type in frequency ( 18 and 56% , respectively ) . the incidence in females is double that of males , unlike the classic mibelli type . dsap is characterized by multiple lesions that are superficial , relatively smaller than the mibelli type , slightly pigmented , annular , keratotic and with a central atrophic area . it mainly affects areas exposed to sunlight ; however , paradoxically , the limbs are more affected than the face . other regions such as the extensor surfaces of the extremities constitute the majority of reported cases , with cases of lesions appearing exclusively on the face being probably less frequent . , dsap does not appear to have more risk of malignant change than other types . it can sometimes coexist with other forms of porokeratosis ( mibelli type , linear porokeratosis , porokeratosis palmaris et plantaris disseminata and punctate porokeratosis ) . there is good evidence that ultraviolet light can precipitate the development of new lesions or exacerbate pre - existing lesions . our patient was minimally exposed to the sun and she spent most of her time indoors . the exact pathogenesis of porokeratosis is not clear ; however , it has been assumed that a focal clonal expansion of abnormal cells gives rise to the shape of the coronoid lamella . the coexistence of different variants in one patient or in several members of an affected family indicates different phenotypic expressions of a common genetic entity which could possibly be explained by a simultaneous expression of closely linked genes . genetic studies have mapped the loci responsible for dsap to chromosomes 12q , 15q and 18q . several mutations have been identified in the ssh1 gene on chromosome 12 , which encodes a phosphatase that plays a pivotal role in actin dynamics [ 10 , 11 ] . in a genome - wide scanning and linkage analysis performed on six generations of a chinese family with dsap , two missense mutations in ssh1 and arpc3 were found , which are involved in the actin cytoskeleton pathway and interact with adherent junctions in the epidermal cells . this finding suggested that cytoskeleton disorganization in epidermal cells was likely associated with the pathogenesis of dsap . in a recent study on four chinese families , in 33% of the patients , three mutations were found in the mevalonate kinase ( mvk ) gene which plays a role in lipid metabolism . psoriasis and phototherapy ( uva , bb - uvb and nb - uvb ) have been associated with porokeratosis . dsap has also been associated with immune suppression , hiv infection , diabetes mellitus , liver cirrhosis , acute pancreatitis , crohn 's disease , solid malignancy , the administration of immunomodulating drugs used to treat autoimmune diseases and following the transplantation of organs ( particularly in kidney transplant patients ) [ 14 , 15 ] . therapies such as cryotherapy , photodynamic therapy , erbium yag and co2 lasers or the application of topical 3% diclofenac gel or 5-fluorouracil cream are usually partially successful , but with inconsistent results . this therapy treated the plaques effectively , there were no more keratotic elevations or furrows , and even the central scars showed an improvement in color and texture . biologically , imiquimod is also antineoplastic and is used to treat nonmelanoma skin cancers ; this is a major added advantage because dsap may , in rare instances , be complicated by squamous cell carcinoma . further studies are required to confirm whether imiquimod should have a place in the management of dsap . the mechanism of action of imiquimod in the treatment of dsap is not yet clear : it may suppress or switch off the abnormal mutant genes through its immunological effects on both adaptive and innate immunity . in conclusion , we presented a case of facial sporadic dsap , which was treated by application of imiquimod 5% cream in conjunction with regular sunscreens . follow - up of this case is important to rule out the possibility of malignant transformation .

porokeratoses are a group of hereditary or acquired disorders characterized by annular lesions with an atrophic center and a prominent peripheral ridge . pathologically , porokeratosis is characterized by the presence of abnormal clones of keratinocytes that form a column of parakeratotic cells , called the cornoid lamella . fifteen percent of patients of disseminated superficial actinic porokeratosis ( dsap ) have facial lesions ; other regions like the extensor surface of extremities constitute the majority of reported cases . exclusively facial lesions are probably less frequent . rarely , actinic porokeratosis is confined to the nose only . of reported porokeratosis cases , 7.5% have revealed a malignancy arising within the lesion . we present a case of facial sporadic dsap that was treated with imiquimod 5% cream in conjunction with a regular sunscreen . follow - up of this case is important to rule out the possibility of malignant transformation of the lesions .