Vesicoureteric reflux: Evaluation by bladder volume graded direct radionuclide cystogram.

AIM: Evaluation of vesicoureteric reflux (VUR) in children by bladder volume graded direct radionuclide cystogram (BVG DRC). This technique allows detection of VUR at different bladder volume grades. MATERIALS AND METHODS: In this prospective study, 33 patients (66 renal units) with suspected vesicoureteric reflux were subjected to a voiding cystourethrogram (VCUG) and BVG DRC. The patients were assessed further with radioisotope renal scans for renal cortical scars. RESULTS: Twenty-two patients and 36 renal units were found to have VUR in either of the reflux studies. A VCUG was able to detect 20 units (55.50%) and a BVG DRC was able to detect 35 units (97.2%). A VCUG had a test accuracy of 77.8% and a BVG DRC had a test accuracy of 98.6%. There was a positive correlation between bladder volume grades and scarring on a DMSA scan. CONCLUSIONS: Like a conventional DRC, BVG DRC is a sensitive and an accurate test. It gives additional information on the reflux phenomenon with respect to bladder filling. The bladder volume graded technique is better than conventional DRC for grading of VUR.

Ureteric valve: Case report with an insight into anatomy, embryology, presentation and management.

Congenital ureteric obstruction caused by a ureteric valve is an exceedingly rare entity. Our patient, a nine-year-old male, had undergone evaluation for recurrent pain in the abdomen and was diagnosed as a case of left hydronephrosis on ultrasound abdomen. Intravenous urography and magnetic resonance urography showed incomplete duplex system on the right side along with left hydronephrosis and hydroureter. Cystoscopy with left ascending gram followed by excision of lower third of ureter along with valve and Cohen's ureteroneocystostomy was done. Histopathology revealed Type II ureteric valve. A high index of suspicion is required to make a correct preoperative diagnosis.

Anovestibular fistula with normal anal opening: Is it always congenital?

AIM: To review 12 cases of anovestibular fistula with normal anal opening. METHODS: Retrospective analysis of 12 children with anovestibular fistula and normal anal opening were treated between the years 2000 and 2007. Of these, 11 patients were diagnosed as having acquired anovestibular fistula with normal anal opening and were managed by conservative management. RESULTS: Most of them presented with diarrhea and labial redness. One patient was considered to have fistula of congenital origin and was managed surgically. Eleven patients presented between the ages of 1.5-11 months and were considered as cases of acquired anovestibular fistula and only two of them required surgical management in the form of colostomy and fistula excision. Others were successfully managed by conservative treatment; the fistulous output and labial redness decreased gradually within a period of 5-19 (average 11.5) days. CONCLUSIONS: Not all presentations of anovestibular fistula with normal anal opening can be considered as congenital. Presence of inflammation, paramedian fistula, and a favourable response to conservative management/colostomy suggest acquired etiology. Trial of conservative management should be given in the acquired variety.

Posterior urethral polyps and review of literature.

Urethral polyp is a rare finding in young children. Fibroepithelial polyps of the urethra are usually diagnosed during the first decade of life. They present with obstruction, voiding dysfunction and hematuria. They can be associated with other congenital urinary tract anomalies. They are usually benign fibroepithelial lesions with no tendency to recur and are treated by surgical ablation, fulguration or laser therapy.

Thoracoscopic excision of mediastinal cysts in children.

AIM: Thoracoscopy offers great advantages when compared with open surgery in terms of postoperative pain and pulmonary complications. Considering the benign nature of most of the mediastinal cysts, thoracoscopy is safe and feasible with minimal morbidity. The purpose of this article is to review our experience with four cases of mediastinal cysts resected successfully within a period of one year by thoracoscopy. MATERIALS AND METHODS: The cases of mediastinal cysts operated by thoracoscopic excision in K.E.M. Hospital, Mumbai from November 2005 to December 2006 were reviewed. The age varied from six months to 10 years. The patients presented with respiratory distress or recurrent lower respiratory tract infection. All patients underwent Chest X-ray and CT scan thorax to delineate the location of the cyst and its relationship with adjacent vital structures. Two patients had anterior and two had posterior mediastinal cyst. The ports were placed depending on the location of the cyst on the CT scan, following the principles of triangularization. The cysts were excised mainly by blunt dissection. RESULTS: All the patients were successfully managed by thoracoscopic surgery. None of them had intraoperative complications. Dissection in patient with history of recurrent respiratory tract infection was difficult because of adhesions. Intercostal drain was removed within 48hrs and the patients were discharged on the fourth postoperative day. CONCLUSIONS: Thoracoscopy in mediastinal cysts is a safe and effective procedure with low morbidity and a shorter hospital stay.

Congenital diaphragmatic hernia with gastric volvulus.

Gastric volvulus is a surgical emergency presenting in various forms. Association with diaphragmatic defect is well known. Here we describe three cases of gastric volvulus associated with diaphragmatic defect having varied presentations and their management. A rare case of gastric volvulus with complete gangrene of the stomach is also reported. Three types of gastric volvulus have been described depending on the rotation axis: organoaxial, mesentericoaxial and combination of both types. Operative treatment includes reduction, correction of underlying cause and gastropexy.

Unusual aero-digestive foreign bodies: tribulations and tragedies.

Aero-digestive foreign bodies are a common occurrence in infants and children. The manifestations, hazards and consequences depend upon the location, nature of the foreign body and the time lapse. This communication reports a series of four cases with unusual course and consequences of aero-digestive foreign bodies. These were generalized tetanus, hypernatremia and metabolic acidosis with intra-cranial hemorrhage and severe pneumonia and empyema. Awareness of these consequences would help treating doctors to take appropriate steps in devising preventive and therapeutic strategies.

Extrarenal Wilm's tumour - a rare entity.

A rare case of extrarenal Wilms' tumour (ERWT) that presented as an asymptomatic subcutaneous lumbar mass is reported. The diagnosis could only be established postoperatively.

Glanular diphallus with urethral stricture.

An unusual case of a 5-year-old boy with duplication of the glans, a rare variety of diphallia, is reported. The two glandes were arranged one above the other, the dorsal glans had only a blind pit while the ventral glans had a patent urethra with glanular hypospadias. The child also had right renal agenesis and a posterior urethral stricture.

Rapunzel syndrome with a long tail.

A rare form of gastric trichobezoar extending into small bowel with varying gastrointestinal symptoms is known as Rapunzel syndrome. A case history of 6 years old boy is described. This is the twelfth patient with Rapunzel syndrome in the literature.

Gastric teratoma: a rare cause of gastrointestinal bleeding.

Gastric teratoma is a rare tumor of infancy, almost exclusively found in males. We report a 6-month-old male child with gastric teratoma who presented with gastrointestinal bleeding; he was treated successfully with local excision of the tumor.

Spontaneous perforation of the extrahepatic bile duct in an infant.

A 1.2-year-old male presented with gradual-onset biliary ascites, mild icterus, and failure to thrive due to spontaneous bile-duct perforation (SPBD) confirmed by technetium 99Tc HIDA scan and abdominal paracentesis. Peritoneal tube drainage for 2 weeks helped the perforation to seal spontaneously, as there was no distal obstruction. No surgery was needed. Only less than 100 cases of SPBD have been reported in the English literature. The pathogenesis and treatment options are reviewed and discussed.

Wilms' tumor arising in a horseshoe kidney.

The incidence of horseshoe kidney (HK) is estimated at 1 in 400 cases. The occurrence of Wilms' tumor (WT) in a HK is an uncommon event, estimated at 0.4%-0.9% of all WTs. We report a case of WT arising from the isthmus of a HK and review the literature on the subject.

Infantile hemangioendothelioma.

Primary hepatic tumors are uncommon in children and account for only three per cent of the tumors in children. Infantile hemangioendothelioma is a rare benign hepatic tumor arising from mesenchymal tissue. Most of the cases present before six months. An unusual presentation and progression of infantile hemangioendothelioma is reported in a 19-month-old female child. The diagnosis was arrived at by radiological and histopathological examination. The patient underwent excision surgery, following which made an uneventful recovery. On follow-up at six months, patient was asymptomatic with no evidence of recurrence.

Complete spontaneous regression of a functioning adrenocortical carcinoma in an infant.

A 9-month-old female presented with Cushing's syndrome. Investigations suggested the diagnosis and revealed a functioning left adrenal carcinoma without metastasis. However, when the abdomen was explored 30 days later after controlling the hypertension only normal adrenals were encountered, suggesting complete spontaneous regression. This is the fourth case of its kind. The case along with a review of this rare phenomenon is presented.

Morgagni hernia with Down syndrome: a rare association -- case report and review of literature.

Morgagni hernia is a rare diaphragmatic hernia accounting for only 2% of the congenital diaphragmatic defects. A case of Morgagni hernia was diagnosed radiologically in a 12-months-old male with Down syndrome, with recurrent respiratory distress and chest deformity. The 2-dimensional echocardiography was normal. The diagnosis of Morgagni hernia was confirmed by barium studies. The patient underwent a corrective surgery at 18 months of age following which his symptoms subsided. Literature review revealed only 18 cases of Morgagni hernia with Down syndrome reported till date, with age of presentation varying from neonatal age group to 12 years of age. The mode of presentations varied from asymptomatic detection to severe respiratory distress. The possible mechanism of association and its clinical implication has been discussed. The case emphasises a need for diaphragmatic hernia to be looked for as a possible cause of respiratory distress in Down syndrome.

Factors responsible for successful primary closure in bladder exstrophy.

To assess the important factors for successful primary closure in staged reconstruction of bladder exstrophy, 25 patients (18 males, 7 females) underwent primary bladder closure during the years 1993-1997. Twenty-one were more than 72 h old; all of these underwent bilateral posterior iliac osteotomies followed by primary bladder closure during the same anesthetic. Bladder closure was done in a double layer. The ureteric catheters were removed after 2 weeks and the bladder catheter after 3.5-4 weeks. Only 1 patient had a bladder dehiscence on the 10th postoperative day due to infection; 3 had partial wound dehiscences but no bladder dehiscence. One had a partial bladder prolapse. The osteotomies needed no drainage, and no complications occurred. One patient needed a urinary diversion 3 years after surgery as the bladder capacity did not increase. Eleven important factors play a pivotal role for successful primary bladder closure: (1) Proper patient selection; (2) A staged approach; (3) Anterior approximation of the pubic bones with placement of the bladder and urethra in the true pelvis; (4) Posterior bilateral iliac osteotomies in all indicated cases; (5) Double-layered closure of the bladder; (6) Two weeks' proper ureteric catheter drainage; (7) Prevention of infection; (8) Prolonged and proper postoperative immobilization; (9) Prompt treatment of bladder prolapse; (10) Prevention of abdominal distension postoperatively; and (11) Ruling out bladder-outlet obstruction before removing the bladder catheter.

Bladder agenesis with urometrocolpos.

A 20-day-old female presented in acute renal failure with an absent bladder and vaginal atresia. The right ureter opened into the uterus, resulting in a urometrocolpos, and the left ureter opened through a stenotic opening into the introitus. The literature is reviewed for reported cases and the embryo-pathology of bladder agenesis is discussed.

Anterior tibial artery pseudoaneurysm.

Traumatic pseudoaneurysms are rare in civilian trauma, more so in childhood. A case of anterior tibial artery pseudoaneurysm is described which was successfully treated non surgically by percutaneous coil embolisation, thus questioning the need for surgery in peripheral pseudoaneurysms.