# Children With Biliary Atresia Have Substantial Morbidity in Early Childhood and a High Risk of Liver Transplantation

## TL;DR

Children with biliary atresia face high early mortality and a significant risk of needing a liver transplant by age five.

## Contribution

This study provides population-based data on biliary atresia outcomes across Europe, emphasizing the need for prevention research.

## Key findings

- Infant mortality rate was 12.3%, and 18.5% died before age five.
- 37% of children underwent liver transplantation by age five.
- The Kasai procedure was performed at a median age of 57 days.

## Abstract

Biliary atresia is a rare but severe congenital anomaly associated with substantial morbidity and mortality in early childhood. Population‐based estimates of survival, surgical management, and liver transplantation across Europe remain limited. This study aimed to describe mortality and morbidity among children born with biliary atresia using multinational population‐based data.

We investigated children diagnosed with biliary atresia across nine registries from five countries within the European surveillance of congenital anomalies network (EUROCAT), covering births from 1995 to 2014. The data were linked to hospital databases and adjusted for regional differences and follow‐up length.

Our cohort included 171 children, with an infant mortality rate of 12.3% (95% CI: 7.8–17.6) and a mortality rate before age five of 18.5% (95% CI: 10.7–27.7). Among these children, 151 had undergone surgery, including 133 who received the Kasai procedure by the age of 1 year at a median age of 57 days (95% CI: 51–62 days). By age five, 37% (adjusted percentage, 95% CI: 30–44) had undergone liver transplantation, with the median age at transplantation being 318 days (95% CI: 244–391 days). Median age at death in the first year was over 6 months and was not immediately after surgery.

The high mortality and the substantial need for liver transplantation within the first year of life underline the severity of biliary atresia. This highlights the urgent need for further research into pregnancy exposures that may contribute to this rare but severe congenital anomaly to develop primary prevention strategies.