Successful pregnancy outcome in a patient with congenital biliary atresia

Congenital biliary atresia is one of the most common congenital anomalies of the biliary tree. Without surgical correction, two-thirds of all cases of extrahepatic biliary atresia are fatal within 18 months. Historically, the preferred surgical procedure to correct this anomaly has been the Kasai procedure. Surgical treatment of congenital biliary atresia is associated with substantial perioperative morbidity and mortality, as well as long-term sequelae. Portal hypertension and hypersplenism can complicate the course of up to 50% of patients who survive for more than 2 years. Not surprisingly, few of these patients reach adulthood. We report the case of a woman with congenital biliary atresia surgically corrected with the Kasai operation who subsequently reached adulthood and became pregnant. Despite a prenatal course complicated by portal hypertension, bleeding esophageal varices, and hypersplenism, the woman delivered a healthy infant at 36.5 weeks' gestation.