Biliary atresia and situs inversus in infant: a rare case report

Background. Biliary atresia is the leading cause of liver transplantation in children. It is associated with other congenital anomalies. The presentation of biliary atresia with situs inversus is a rare case. Case presentation. A 2-month-old female presented with jaundice since 15 days of life, acolic stools, and tea-colored urine. On further evaluation, she was found to have dextrocardia and situs inversus. A liver biopsy was performed and showed hepatic fibrosis due to extrahepatic cholestasis. Abdominal ultrasound and MRCP confirmed a liver in the left hypochondrium and suggested biliary atresia. Conclusion. Although rare, every patient with biliary atresia should be screened for congenital situs inversus, which has a poor prognosis.