Biliary atresia splenic malformation syndrome presenting with hepatic abscesses

Biliary atresia, a destructive inflammatory cholangiopathy, leads to liver cirrhosis and subsequent death by the age of 2 years if left untreated. Biliary atresia splenic malformation (BASM) syndrome makes up 10% of all cases of biliary atresia. Kasai hepatoportoenterostomy (KPE) may establish continuity of bile flow and slow down progression to cirrhosis if the procedure is performed early in infancy. We describe an 8.5-year-old boy with known BASM syndrome (polysplenia, intestinal malrotation, interrupted inferior vena cava, shortened pancreas, centralised liver and left atrial isomerism) who underwent a successful KPE at the age of 3 months. He presented with features suggestive of a late onset ascending cholangitis (AC) complicated by cholangitic liver abscesses. Resolution of the abscesses with prolonged antibiotic therapy avoided the need for percutaneous drainage. Once the abscesses resolved, the child underwent a successful cadaveric liver transplantation.