BILIARY OBSTRUCTION IN INFANTS WITH CYSTIC-FIBROSIS REQUIRING KASAI PORTOENTEROSTOMY

Cystic fibrosis is associated with an inspissated bile syndrome produc ing cholestasis secondary to plugging of macroscopically normal bile d ucts. In extreme neonatal forms, with early profound intrahepatic chol estasis, the process can be associated with a marked decrease in ducta l diameter, varying from hypoplasia to atresia. From 1990 to 1995 thre e infants were identified with cystic fibrosis, persistent jaundice, a nd complete absence of biliary excretion despite expectant and conserv ative treatment including choleretics and surgical biliary irrigation. Abdominal ultrasounds showed contracted gallbladders and no evidence of dilated ducts. Liver biopsy results in two infants showed portal fi brosis, paucity of bile ducts, and minimal inflammation. The third inf ant had moderate inflammation, bile duct replication, and plugging. Tw o infants had undergone intestinal resection followed by hyperalimenat ion for complications of meconium ileus in the newborn period. Surgica l exploration was undertaken at 7 to 12 weeks of age. Gross findings w ere typical of biliary atresia with microgallbladders and nonpatency o f the cystic duct. Cholangiograms failed to document ductal patency in two patients who were then treated with a Kasai portoenterostomy. The third infant had patent hypoplastic ducts and underwent only biliary irrigation. Although bile flow was transiently achieved, jaundice recu rred, and at reexploration at 16 weeks of age a Kasai poroenterostomy was performed. Histological review of the biliary specimens showed mic roscopically patent ducts in two patients and proximal patency and dis tal atresia in the third. All the ducts had mural fibrosis with cystic changes. Bile drainage was achieved in each instance, although in one patient with hypoplastic ducts scant output of highly concentrated bi le proved insufficient to arrest progressive liver failure. The subseq uent two patients responded with resolution of hyperbilirubinemia and normalization of liver function. They remain free of biliary complicat ions at 30 and 40 months postoperatively. This manifestation of cystic fibrosis in infants is suggested by prolonged jaundice unresponsive t o choleretics, nondilated bile ducts and gallbladder on ultrasound, ab sent biliary excretion on nuclear scan, and characteristic liver biops y. Exploration is warranted, and discovery of atrophic bile ducts may be best managed with reconstruction. Copyright (C) 1997 by W.B. Saunde rs Company.