ABSTRACT: Laryngotracheo-esophageal cleft (LTEC) is a rare disorder that is still associated with high mortality. Less than 100 cases have been reported in literature till now. It varies in anatomical extent and clinical severity. Here we are presenting a case of LTEC with an extended broncho-esophageal cleft (type 4) in a newborn presented with respiratory distress. Only 4 cases of this extended type of cleft has been reported in literature till now. This type 4 cleft is a challenge to the surgeon. It requires meticulous preoperative, operative and postoperative care. Upper GI contrast study and endoscopy are diagnostic. Surgery was performed in stages but the patient succumbed on the 146th day of life. Review of literature and brief discussion about thisrare anomaly is presented here.

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ABSTRACT: Mullerian inhibiting factor is responsible for regression of the fetal mullerian structures in the male subject; and its absence is responsible for a rare form of male pseudohermaphroditism, the persistent mullerian duct syndrome. We report an otherwise normal male with bilateral undescended testes and a right inguinal hernia in whom on exploration persistent mullerian duct structure was found in association with transverse testicular ectopia (TTE) in the hernial sac.

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ABSTRACT: The association of anomalies of face, eyes and brain with anorectal malformation is not well-known as also a combination of two types of anorectal malformations. A male newborn presented with hypoplasia of left side of face, with a normal ear. He had no anal orifice. He underwent colostomy followed by perineal rectoplasty (Durham Smith's modification of PSARP). He was found to have anal agenesis (intermediate anorectal malformation) in addition to a rectal atresia Type I (high anorectal malformation). He had severe anomalies of both eyes, and bilateral renal anomalies. He is now 7 years old and has not developed speech, has defective vision, is unable to walk without support, is hyperactive, and is constipated. Literature on the association of double anorectal malformations and of hemifacial microsomia and anorectal malformation is reviewed.

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ABSTRACT: The case of a one year old boy who had bilateral grade V vesico-ureteral reflux is being reported. At antireflux surgery, which comprised of bilateral cross trigonal ureteric reimplantation, polyps were seen at both ureteral orifices. It is surmised that the polyps may have had an etiological role in the causation of the reflux by interfering with the natural antireflux mechanism.

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ABSTRACT: Schwannomas are benign nerve sheath tumours commonly found in cranial nerves. A case of presacral schwannoma with intraspinal extension in a 10-year old boy with neurofibromatosis type-2, presenting with left sciatic pain and difficulty in walking,is described here. On examination, he had multiple neurofibromas. On screening by MRI, there was associated unilateral vestibular schwannoma and an intrathoracic schwannoma. The intraspinal portion of the presacral schwannoma was removed first followed by complete excision of the presacral portion after 15 days. The histopathological examination showed features of well differentiated synovial sarcoma which was differentiated by immunohistochemical staining with S-100 and cytokeratin which confirmed the diagnosis of schwannoma. The child is well at one year follow up and can walk without difficulty.

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ABSTRACT: Two boys presented to our unit between March 2003 and August 2003, required pericardectomy. Thoracoscopic window pericardectomy was done for both these children after a full work up. Using the 30 degree telescope the diagnosis of the pyopericardiumwas confirmed. Thick, yellow and non foul smelling pus measuring 300ml was let out and a window pericardectomy was done taking special care to safeguard the phrenic nerves. Both the children made uneventful recovery. The histopathalogical examination of the excised pericardial tissue revealed non specific inflammation in both cases.

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ABSTRACT: The clinical experience of modified Duhamel procedure as a definitive treatment of Hirschsprung's Disease (HD) over 10 years was analyzed. Forty eight patients of HD were treated with modified Duhamel procedure. Forty six patients had short segmentand 2 had long segment disease. Spur remnant was seen in 8 patients. Persistent constipation (n=6), soiling (n=2) and incontinence (n=2) were seen in long term follow up. The procedure is technically easy and gives satisfactory long term results for definitive treatment of HD.

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ABSTRACT: The present series describes the retrospective analysis of 75 pediatric patients diagnosed as germ cell tumors after surgery and histopathology collected over last 20 years. The Male: Female ratio was 23: 52. Age of the patients at presentation ranged from 3 days to 10 years, of which 12 cases presented in neonatal period, 43 cases between 1 month and 3 years, and 13 cases between 4 and 7 years and 7 patients were above 8 years. Extragonadal location was twice commonly involved compared to gonadallocation (50:25). Among extragonadal sites, sacrococcygeal region was the commonest to be involved (41 cases) followed by retroperitoneum (6 cases). Present series recorded only single cases of germ cell tumors each of mediastinum, stomach and vagina. Ovary was the site of involvement in 18 cases and testis in 7 cases. Mature (benign) teratoma was the commonest histopathological diagnosis (58 cases), followed by dysgerminoma (6 cases), yolk sac tumor (5 cases), immature teratoma and embryonal carcinoma (3 cases each). Patients with mature teratoma were treated with surgical excision only and complete removal was curative. Malignant germ cell tumors required radiotherapy and chemotherapy. Follow-up data were recorded.

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ABSTRACT: Aim: To describe the technique and results of Anterior Sagittal Anorectoplasty for the treatment of vestibular fistula and perineal canal in females. Methods: Patients with vestibular fistula and perineal canal admitted to the department of pediatric surgery were taken up for surgery at presentation. The technique involves minimal preoperative preparation and no colostomy is required. The rectum is separated from the vagina and placed in the sphincter muscle, the perineal body is created. Postoperative management is simple and the child is usually discharged by the fourth postoperative day. Results: ASARP was performed on 644 patients (age range 3 days - 44 years) between July 1970 and May 2003. Five hundred thirty six patients were followed up at 3 months. Five hundred twenty nine had a normal looking perineum and normal defecation. Three hundred fifty four children were assessed after the age of 3 years, 329 had no problems, were fully continent and had normal defecation; 25 children were constipated. There were 2 postoperative deaths; 7 patients had postoperative complications. Conclusions: ASARP is a suitable technique for single stage management of vestibular fistula and perineal canal in the female

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ABSTRACT: Fistula in ano is an uncommon disease in the pediatric age group. Treatment of complex and high fistulae is difficult and involves considerable morbidity and requires higher surgical skills. As far back as the 8th century AD, Sushrutha mentioned the use of a cutting ayurvedic seton in the treatment of fistula in ano. Though several workers have subsequently reported the use of Ksharsutra in adults there have been no published reports of its use in children. We report a case of traumatic fistula inano which we treated by this method and discuss its merits in the pediatric population.

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ABSTRACT: Over a period of 15 years, we have treated 22 patients of pseudocyst of the pancreas. Two of our patients developed pseudocyst after a prolonged treatment with Sodium Valproate, a known but rare complication of the drug. We also treated two patients of pancreatic duct calculi with pseudocyst, a rare association. Initially all the patients received conservative management. Two patients had USG guided aspirations. Different drainage procedures like cysto gastrostomy, cysto jejunostomy, pancreatico jejunostomy were performed. Two patients had mild wound infection. Two patients came with occasional abdominal pain. Follow up USG has not shown any abnormality in those patients. Two patients were lost to follow up.

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ABSTRACT: Pseudocyst formation is a rare complication of ventriculoperitoneal shunt. Two cases are being reported who presented with abdominal symptoms following ventriculoperitoneal shunt. The shunt was functioning in one case and partially blocked in the other. The diagnosis was made with the help of ultrasound. Both the cases were operated and the complete cyst was excised in both cases. The distal end of the shunt catheter was repositioned in the suprahepatic space. Both cases are doing well in 6 monthsand 9 months follow up respectively.

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ABSTRACT: Congenital giant hairy nevi (CGHN) need excision for aesthetic purpose and for its predisposition to malignant transformation. Serial excision using a tissue expander gives an excellent full thickness cover and aesthetically a pleasing result.

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ABSTRACT: A 4 month old boy presented with fever, cough and respiratory distress for 3 weeks. An X-ray of the chest showed a large right-sided lung cyst. The history revealed that an antenatal ultrasound (US) performed at 26-27 weeks gestation had shown a cystic lesion in the right lung. Surgical excision of an intralobar bronchogenic cyst (BC) involving the middle lobe of the right lung was curative. A brief review of the literature is presented.

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ABSTRACT: Abdominal cystic lymphangiomas are relatively rare congenital malformations, predominantly found in the pediatric age group. This report describes a case of a large mesenteric lymphatic cyst in a 3-year old child. The differential diagnosis and modalities of treatment are also discussed.

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