Cystic dilatation of the intrahepatic biliary system in biliary atresia after hepatic portoenterostomy.

Five cases of intrahepatic biliary cyst or cystic dilatation and one suspicious case were found (6.4%) among 93 survivors of biliary atresia. The age at manifestation of such cysts ranged from 6 months to 12 years. These cysts or cystic dilatations were treated surgically in two cases, by percutaneous transhepatic cholangiodrainage (PTCD) in two, and not treated in one. Four patients are alive; one died of biliary atresia. Twenty-nine well-documented cases of intrahepatic biliary cyst were found in the literature, including five treated by the authors. The modes of dilatation were as follows: type A, noncommunicating cyst; type B, cyst with tiny communication with the intestinal loop; and type C, cystic dilatation. Clinical symptoms were fever, jaundice, acholic stool, and "cholangitis." Nineteen patients (66%) had symptoms within 4 years after the Kasai operation (first group); the modes of dilatation were mostly types A and B. However, in five of six patients with type C dilatation, symptoms developed after 10 years of age (second group). The prognosis for the second group appears poor because the condition of the liver worsens once cystic dilatation develops.