Adv Pediatr Surg.  2022 Jun;28(1):17-20. 10.13029/aps.2022.28.1.17.

Biliary Atresia Splenic Malformation With Situs Inversus: A Case Report

• Solanki S
• Kanojia R
• Reddy M
• Ahmad MF
• Lal SB
• Samujh R

• KMID: 2538284
• DOI: http://doi.org/10.13029/aps.2022.28.1.17

Abstract

Biliary atresia is a progressive sclerosing cholangiopathy of bile ducts. Most of the time it is an isolated anomaly but can present with syndromic forms. The biliary atresia splenic malformation (BASM) syndrome is associated with splenic anomalies, vascular anomalies, and visceral asymmetry along with biliary atresia. The surgical anatomy of BASM is distinctive and creates a challenge for the surgeons. We are describing a case of BASM with situs inversus and highlighting the approach, intraoperative anatomy, and surgical intricacies of Kasai portoenterostomy in such a situation with the review of pertinent literature.

Keyword

Biliary atresia; Polysplenia; Situs inversus; Kasai procedure; Jaundice