Mother-to-daughter occurrence of biliary atresia: a case report

Abstract

We herein report a case of mother-to-daughter occurrence of biliary atresia (BA). The mother underwent a Kasai hepatic portoenterostomy at the age of 64 days with a diagnosis of type III BA. Her postoperative course was uneventful, with a good bile discharge, and she became pregnant at the age of 20 years. At 21 weeks of gestational age, fetal ultrasonographic examination of the baby revealed a cystic mass at the hepatic hilum. The baby was born via vaginal delivery at 41 weeks of gestation with a birth weight of 3269 g. Because of an increasing direct bilirubin level and the absence of bile excretion, a laparotomy was performed at the age of 29 days. Intraoperative cholangiography revealed the diagnosis of BA I-cyst, and a hepaticojejunostomy was performed with a good bile discharge. To our knowledge, these patients are the first occurrence of BA in a transmission pattern of mother to daughter.