Biliary Atresia Splenic Malformation: A Case Study

Biliary atresia (BA), a rare cholangitis, is considered a progressive inflammatory disease of the biliary tree that if left untreated can lead to cholestatic jaundice, cirrhosis, and liver failure. It remains the most common cause of chronic liver disease and the most common indication for liver transplant in children. Biliary atresia can develop concurrently with laterality defects, namely polysplenia and asplenia. This presentation is termed biliary atresia splenic malformation (BASM) and accounts for merely 10% of all cases of BA. The purpose of this article is to present a case report of a neonate with BASM. Following the case report, we present a discussion of risk factors, clinical manifestations, diagnostic modalities, and management strategies, including the Kasai portoenterostomy procedure. Long-term implications of BASM and care strategies to optimize outcomes for the family unit are also included for the benefit of neonatal providers.