ANTENATAL DIAGNOSIS OF BILIARY ATRESIA (TYPE-I CYST) AT 19 WEEKS GESTATION - DIFFERENTIAL-DIAGNOSIS AND ETIOLOGIC IMPLICATIONS

At 19 weeks' gestation, two cystic structures were first identified in the abdomen of a fetus. A repeat ultrasonography at 34 weeks confirmed a definite cyst communicating with the liver. The baby was born at 39 weeks, and serum direct bilirubin started to rise to 4.1 mg/dL. An operative cholangiogram at 23 days of life showed a cystically dilated choledochus with distal atresia and a relatively smooth yet hypoplastic intrahepatic biliary tree. Complete obliteration of the cystic duct was also noted. After excision of the cystic common bile duct, hepatico jejunal anastomosis was performed, and the patient did well for 8 months postoperatively. Liver biopsy showed proliferation of the bile ductules, but no interlobular bile ducts were observed in any portal triad. A diagnosis of biliary atresia was established. Including the present case, five cases of antenatally diagnosed biliary atresia have been reported. All of them had type 1 cyst, and antenatal diagnosis was made at 19 to 32 weeks' gestation. Differential diagnosis between biliary atresia of type 1 cyst and choledochal cyst with complete distal obstruction has been a matter of discussion, and recognition of the entity of antenatally diagnosed biliary atresia is of significant importance from an etiological point of view. Copyright (C) 1995 by W.B. Saunders Company