Biliary atresia, splenic malformation, and situs inversus managed with Kasai portoenterostomy: case report

Introduction: Biliary atresia (BA) involves the gradual sclerosis of the bile ducts. It commonly manifests as an isolated abnormality but may also occur in syndromic forms. Case Presentation: We present the case of an 80-day-old male infant with the syndromic variety of BA associated with polysplenia syndrome, dextrocardia, situs inversus totalis, and malrotation of the intestines. From the first week of life, the patient exhibited jaundice, clay-colored stools, and dark yellow urine. Clinical discussion: A physical examination revealed an icteric child with a palpable liver. Laboratory testing showed a total bilirubin level of 16 mg/dL, with direct bilirubin at 9 mg/dL, indicative of obstructive jaundice. The patient also presented with elevated liver enzymes and an alkaline phosphatase level of 935 IU/L. Abdominal ultrasonography failed to visualize the gallbladder but demonstrated situs inversus with polysplenia. Echocardiography confirmed dextrocardia, with no other cardiac anomalies detected. The patient underwent a Kasai portoenterostomy, after which bile was present in the stool, and hyperbilirubinemia was reduced. Conclusion: BA polysplenia syndrome is a rare congenital disorder, and surgeons operating on these patients need to be aware of potential challenges caused by abnormal rotation and mirroring of the digestive system anatomy.