Aplasia cubs congenmta associated with meningomyelocoel.

Aplasia cutis congenita is a rare congenital anomaly where skin is missing over part of the body. Most of the cases are present on the scalp. We present a case of ACC where skin was absent over the lumbosacral area. It was associated with meningomyelocoel.

Pseudoexstrophy in a female child.

A six-year-old female patient presenting with a swelling in the infraumbilical part of the abdomen, bulging out on straining, was diagnosed to have pseudoexstrophy bladder. The urinary tract was normal. The patient had bifid clitoris. There was no other associated malformation. Surgical repair of abdominal wall defect was done successfully. A new classification of exstrophy variants is proposed.

Intrauterine intussusception--a cause for ileal atresia.

A case of ileal atresia consequent to intrauterine intussusception is reported. The baby presented with features of neonatal intestinal obstruction but signs of peritonitis were absent. The intussusception was discovered on gross examination of distal atretic ileal segment. The case was managed successfully by resection and end to back anastomosis. This case is reported to highlight intrauterine intussusception as one of the causes of ileal atresia.

Choledochal cyst: a 10-year experience.

A retrospective study of 11 cases of choledochal cyst over a period of 10 years is presented. There were 8 females and 3 males in a ratio of 2.67:1. The classical triad of jaundice, mass and abdominal pain was seen in none of our patients. Ultrasonography was diagnostic in 9 out of 11 (80%) patients. Surgical procedures performed were, complete excision of the cyst with Roux en Y hepaticojejunostomy (nine cases), internal drainage of the cyst (one case) and excision of the extrahepatic cyst with Roux en Y hepaticojejunostomy in one case of Type IV choledochal cyst. One patient died in the immediate postoperative period. Others have remained well upto 10 years follow-up. We recommend total cyst excision with hepaticojejunostomy as the treatment of choice.

Cystic lymphangioma with special reference to rare sites.

A 10 year retrospective study of 45 cases of cystic lymphangioma (CL) in children is presented. There were 25 females and 20 males. Age ranged from 6 months to 8 years. Common sites were involved in 38 and rare sites in 7 patients. Rare sites were--gluteal region (1), pelvis (1), retroperitoneum (1), mesentery (2), inguinal region (1) and inguinoscrotal region (1). The clinical presentation included sudden increase in size (25), lump abdomen (3), gluteal abscess (1), abdominal distension (1) and inguinal swelling (2). Diagnosis was established preoperatively in 38 cases, and after surgery and histopathology in 7 cases. Near total or subtotal excision was carried out in all cases. Facial nerve palsy (1) and recurrence (2) were the complications of surgery. The study is presented to highlight the occurrence of the cystic lymphangioma at rare sites to avoid diagnostic errors and unnecessary mutilating surgery.

Post traumatic bile ascites.

A 4 year old girl with progressive distension of abdomen and previous history of blunt abdominal trauma was found to have bile ascites due to left hepatic duct (LDH) injury. The management with a brief review of literature is discussed below.

Esophageal atresia with blocked distal tracheo-esophageal fistula.

A new born male child diagnosed for pure esophageal atresia was subsequently found to have blocked tracheo-esophageal fistula. The case is reported in view of its rarity and difficulty in management.

Abdomino-scrotal lymphangioma.

Retroperitoneum, pelvis and scrotum are unusual sites of lymphangiomas. Retroperitoneal lymphangiomas presenting as inguino-scrotal masses are extremely rare. An unusual case of retroperitoneal lymphangioma extending into scrotum, presenting as acute scrotum is reported due to its unusual location. Complete surgical excision with careful attention to avoid damaging contiguous structures is the treatment of choice.

Morgagni hernia in a neonate with ventricular septal defect.

A neonate with Morgagni hernia and ventricular septal defect is reported. Presentation of Morgagni hernia in neonates is rare, but when it does, it is associated with other congenital anomalies.

Huge omental cyst mimicking ascites.

An unusual case of omental cyst is described. A 4-year-old child presenting as abdominal distension of short duration, clinically diagnosed as ascites, was subsequently proved to be giant omental cyst. The case is reported because of its unique presentation.

Pyloric membrane and congenital hydronephrosis--a rare association.

A rare association of pyloric membrane, Meckel's diverticulum and pelvi-ureteric junction obstruction in a 3-year-old boy is reported.

Duodenal obstruction with isolated levocardia.

We report a patient with isolated levocardia without cardiac or splenic defects associated with duodenal membrane.

Childhood appendicitis. A clinical profile.

A study to assess the reliability of clinical symptoms and signs in 50 patients with a presumptive diagnosis of acute appendicitis is presented. The male to female ratio was 3: 2, with age ranging from 2 to 15 years. Abdominal pain was present in 42; tenderness was localized in 35, generalized in 11 and diffuse in 4 patients. Total leucocyte count was above 11,000/cu mm in 31, below 11,000/cu mm in 17 and above 18,000/cu mm in 2. Of the 48 operated patients, 8 had normal appendices and the diagnosis in them was Meckel's diverticulitis 3, ruptured ovarian follicle 2, mesenteric adenitis 2, and salpingo-oophoritis 1. Abdominal pain and right iliac fossa tenderness with contributory investigations are the most reliable indicators of acute appendicitis with a false positive rate of 16.66% only.