Choledochal cyst - a different disease in newborns and infants

We report experience of managing Choledochal Cyst [CC] in different paediatric ages. Eleven neonates and infants [aged 0-8 months] and 24 paediatric cases [aged 2.5 - 18 years] were managed over 24 years [1988 to 2012]. Neonates and infants presented with jaundice, acholic stools and abdominal mass whereas most of the paediatric cases presented with intermittent non-specific abdominal pain. Morphology of CC was mostly cystic in neonates whereas it was fusiform in majority [62%] of paediatric cases. Biliary amylase was high and correlated with the presence of abnormal pancreaticobiliary junction [PBJ] in 20 /24 paediatric patients. Obstruction at the lower end of bile duct, liver fibrosis and cirrhosis were common in neonates. In conclusion, CC in newborns and infants is different and mimic correctable Biliary Atresia [BA]. Early excision of CC and biliary reconstruction is promising in neonates, infants and children and it can be performed with minimal morbidity

Solitary infantile myofibromatosis of the skull

This is the report of a 6 months old boy presenting with a firm, solitary mass on the temporal region, associated with lysis of local bone. Investigations lead to a diagnosis of infantile myofibromatosis [IM]. Wide local excision was performed. At one year follow-up, no recurrence was noted

Maternal and fetal complications of antiphospholipid syndrome: a case report with long-term follow-up

We report a case of a 26- year old woman having antiphospholipid syndrome in pregnancy with long-term follow up. She presented with recurrent miscarriages, venous thrombosis, avascular necrosis of femoral head, mid-cerebral artery infarction and skin ulcers. Antiphospholipid syndrome is a recognized disorder of pregnancy. Diagnosis requires a high index of suspicion when evaluating women with recurrent pregnancy losses and vascular thrombosis. A low dose aspirin combined with heparin can reduce morbidity and improves the pregnancy outcome

Penile constrictive band injury

Penile strangulation caused by a thread or human hair is an uncommon cause of urethral injury described in paediatric practice. Five children presented with this entity to the Aga Khan University Hospital, Karachi between August, 1991 and August, 1992. Three children had uneventful recoveries after removal of the contricting agent. Two patients developed urethrocutaneous fistulae and partial amputation of the penis because of late presentation. Early recognition and removal of the constricting agent is necessary to prevent serious complications

Corrosive pyloric obstruction: a case report and review of literature

Alkali ingestion usually injures the esophagus, while ingested acid often spares the esophagus and damages the gastric outlet. A case of gastric outlet obstruction without esophageal involvement secondary to acid ingestion is presented to illustrate the difficulties encountered in managing such an injury. The literature on this subject is briefly reviewed

persistent mullerian duct syndrome - a management problem

Two patients with internal male pseudoherma-phroditism, characterized by the persistence of mullerian derivatives in phenotypic male are reported, and current literature reviewed

Retroperitoneal fibrosis

Two patients with Retroperitoneal fibrosis [RPF], involving the ureters and colon are presented with a review of literature. Management includes biopsy of the retroperitoneum, surgical procedures to relieve ureteric and colonic obstruction, and steroids. Long term follow up is recommended in survivors, as there is significant incidence of associated malignancy