Successful Obstetric Outcome in a Woman with Prior Kasai Procedure for Biliary Atresia and Concurrent Alpha-1 Antitrypsin Deficiency: A Case Report

Abstract

We present the case of a 33-year-old primigravida with a history of Kasai portoenterostomy at six weeks of age for congenital biliary atresia and co-existing alpha-1 antitrypsin deficiency. She received antenatal and intrapartum care in our maternity unit, in collaboration with a hepatologist at a tertiary facility. Her management involved a multidisciplinary team comprising maternal medicine and fetal medicine consultants, hepatologists, radiologists, and specialist midwives.

At booking (16 weeks), liver function tests were normal. She underwent four weekly growth scans and liver ultrasounds, with biochemical monitoring throughout her pregnancy. At 38 weeks, she presented with right upper quadrant pain over her surgical scar; clinical assessment and repeat liver tests were normal. Labour occurred spontaneously at 40 weeks and 2 days. She had a spontaneous vaginal delivery of a healthy female infant weighing 3.2 kg, with Apgar scores of 9 and 10 at one and five minutes. Both mother and baby remained well and were reviewed postnatally by the hepatology team.

This case is notable for demonstrating a successful term pregnancy and birth in a woman with rare dual hepatobiliary pathology (post-Kasai biliary atresia and alpha-1 antitrypsin deficiency) without hepatic decompensation. It highlights the importance of multidisciplinary coordination, close biochemical and fetal surveillance, and individualised antenatal planning in optimising maternal and neonatal outcomes. Informed consent for publication was obtained.