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 confirme d a definite cyst communicating with the liver. The baby was born at 3 9 weeks, and serum direct bilirubin started to rise to 4.1 mg/dL. An o perative cholangiogram at 23 days of life showed a cystically dilated choledochus with distal atresia and a relatively smooth yet hypoplasti c intrahepatic biliary tree. Complete obliteration of the cystic duct was also noted. After excision of the cystic common bile duct, hepatic o jejunal anastomosis was performed, and the patient did well for 8 mo nths postoperatively. Liver biopsy showed proliferation of the bile du ctules, but no interlobular bile ducts were observed in any portal tri ad. A diagnosis of biliary atresia was established. Including the pres ent case, five cases of antenatally diagnosed biliary atresia have bee n reported. All of them had type 1 cyst, and antenatal diagnosis was m ade at 19 to 32 weeks' gestation. Differential diagnosis between bilia ry atresia of type 1 cyst and choledochal cyst with complete distal ob struction has been a matter of discussion, and recognition of the enti ty of antenatally diagnosed biliary atresia is of significant importan ce from an etiological point of view. Copyright (C) 1995 by W.B. Saund ers Company