UNOPERATED BILIARY ATRESIA WITH BILIARY CYSTS

E Mas, S Martin, F Alvarez

Sainte-Justine Hospital, Montreal, Quebec

Cystic dilatation of intrahepatic bile ducts (CDIB) associated with cholangitis is described in 20% of patients following hepatico-portoenterostomy for biliary atresia (BA), but is rare at the time of diagnosis.
We report the case of a 4-month-old girl referred for jaundice since birth and fever of recent onset, associated with acholic stools, hepatosplenomegaly, a firm liver consistency and ascites. Investigations revealed severe cholestasis, elevated transaminases and mild coagulopathy. Infections and metabolic etiologies were ruled out. Initial ultrasound (US) showed homogeneous enlargement of the liver, a thickened, atrophic gallbladder and a possibly dilated common bile duct. Percutaneous liver biopsy showed severe portal fibrosis, ductular proliferation, bile plugs and neutrophils around bile ducts. The parenchyme were disorganised with prominent nodule formation compatible with cirrhosis. Initial therapy included intravenous antibiotics, fat-soluble vitamins, ursodeoxycholic acid (UDCA) and diuretics. Ten days later, serpiginous intrahepatic bile duct dilatation was noted on US and confirmed by CT and MR cholangiography. Percutaneous cholangiography filled the cystic dilatation without communication peripherally and without drainage to the intestine. At liver transplantation, biliary atresia was confirmed; the intrahepatic biliary tree consisted of multiple non-communicating saccular dilatations.
Only 5 other patients have been described with CDIB and unoperated BA. The dilatation is often preceded by cholangitis, usually considered incompatible with unoperated BA. Rapid appearance of the cysts may occur following cholangitis, which is thought to develop by hematogenous spread. Antibiotic therapy is often ineffective, possibly due to bile stasis and disrupted vascular flow in presence of biliary cirrhosis. In this case, administration of UDCA may have contributed with the rapid development (10 days) of CDIB.