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.

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.

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.

Tar syndrome with unilateral absent radius and associated esophageal atresia: a variant?

We report a male child with Thrombocytopenia and Absent Radii (TAR) syndrome but with single absent radius and associated tracheoesophageal fistula which has never been reported till date.

Roundworm obstruction of the hepatic limb of a Roux-en-Y anastomosis.

A 7-year-old female with a type I choledochal cyst underwent an exploratory laparotomy. A Roux-en-Y choledochojejunostomy was done following cyst excision. The patient was re-explored 8 days later for an anastomotic bile leak and high fever. A round-worm was found obstructing the jejunal lumen at the porta hepatis, resulting in a biliary leak. Various aspects of biliary ascariasis are discussed along with the case report.

Covered exstrophy: a rare variant.

A case of covered exstrophy without sequestration of a bowel segment is reported. A 4-year-old female presented with dribbling of urine. Treatment to date has been simple excision of the covered membrane with functional closure of the bladder and bilateral posterior iliac osteotomies, with reconstruction of the bladder neck and genitalia to be performed at a later date. The embryogenesis of this rare variant, a review of the reported cases, and management options are discussed. Keyword Covered exstrophy. Exstrophy. Bladder variants

Duplication cyst of the pylorus--a rare cause of gastric outlet obstruction in the newborn.

Duplication cysts of the pylorus are the rarest of alimentary tract duplications with very few reported cases. We present such a cyst in a neonate presenting with gastric outlet obstruction. We have also reviewed the literature and outlined the theories of origin, modes of presentation, diagnosis and the surgical procedures. Even though several modes of surgery have been described, it is best to individualize the surgical option in each case. The results depend on the nature and site of duplication, complications and the associated anomalies.

Sirenomelia: the mermaid syndrome.

Sirenomelia, or mermaid syndrome, is the extreme form of caudal regression syndrome. We present another case of this fascinating anomaly with fused lower limbs, absent external genitalia, and absent genitourinary system. The patient could not be salvaged because of bilateral renal agenesis. Detailed autopsy findings and a review of the literature are presented.

Extrarenal Wilms' tumour--a case report and review of literature.

Extra renal Wilm's tumour (EWT) is an exceedingly rare entity and needs to fulfill certain criteria. Of the 48 cases reported, only about 36 have been found to be convincingly proved. We present one more well proven case of EWT and have extensively reviewed the literature. The diagnostic, staging and management guidelines are discussed. Amongst the reported cases, the behaviour of the tumour appears to parallel that of similarly staged "intra renal" Wilm's tumour. Hence the staging and the management protocols of "intra renal" Wilm's tumour can well be applied to tumour of extra renal location albeit with some modifications.