Congenital biliary tract dilatation in infancy and childhood--74 cases experience.

This report presented a twelve-year experience from 1981 to 1992. Seventy-four cases of congenital biliary tract dilatation were at diagnosed an age of 6 days to 16 years. Twenty-two cases were infants. There were 54 females and 20 males. The ratio of female to male was 2.7:1. The classic triad of abdominal pain, jaundice and a palpable mass was seen in eleven cases (14.9%). Most children suffered from abdominal pain (50/74), vomiting (45/74), anorexia (42/74) and jaundice (34/74). Prolonged jaundice was the main symptom in infancy (15/22). A long common pancreatico-biliary channel was seen in six cases (6/47); the bile amylase level was elevated in five cases (5/20), one patient had a complex union with obstructive jaundice. All these cases were diagnosed by preoperative sonography accurately (100%). According to the Todani's classification, type Ia was the most common (40/74), followed by type IV-A (25/74) and type Ic (8/74). Cholelithiasis (13/74), perforation (9/74), and atresia/stenosis of distal choledochus (8/74) were the most common associated conditions. Cyst excision with biliary tract reconstruction was performed in all cases. Reoperation was needed in ten cases. Two cases died postoperatively due to sepsis and cholangitis induced hepatic failure.