Hepatic portoenterostomy or Kasai operation has been widely accepted as the standard therapy for biliary atresia. Recently, more female patients have grown up and reached adulthood; therefore, pregnancy in women with biliary atresia is sometimes inevitable. The authors report a 17-year-old woman with biliary atresia post Kasai operation at 3 months of age. After the operation, she became jaundice-free but developed portal hypertension with abnormal liver function. She had several episodes of esophageal variceal bleeding and was treated by beta-blocker and endoscopic sclerotherapy. Since then, she was lost to follow up for nearly 2 years. She came back again with 12 weeks of gestation with no symptoms of gastrointestinal bleeding for antenatal care. At 32 weeks of gestation, she presented with severe hematemesis from variceal bleeding and had thrombocytopenia from hypersplenism. She was treated with somatostatin analogue, fluid and blood component replacement and other supportive treatments. Cesarean section was performed when she was stable at 33 weeks of gestation. After the operation, her clinical status was improved and had no other complications. Her baby experienced complications of prematurity but improved after treatment. Pregnancy may affect the natural course of portal hypertension and worsen the clinical outcome. Pregnancy should be avoided in patients with portal hypertension, however it is not contraindicated. Pregnancy in biliary atresia patients needs intensive prenatal care.

Keywords: Biliary atresia, Hepatic portoenterostomy, Pregnancy, Portal hypertension