Heterotaxy syndrome with biliary atresia: a case report

TL;DR

A rare case of a baby with heterotaxy syndrome and biliary atresia is reported, showing successful treatment with a Kasai procedure.

Contribution

This case report documents the rare co-occurrence of biliary atresia and heterotaxy syndrome with detailed clinical management.

Findings

The patient had multiple severe congenital heart defects and biliary atresia associated with heterotaxy syndrome.

A laparoscopic Kasai procedure led to significant clinical improvement in the patient.

Early diagnosis and multidisciplinary care are critical for managing such complex conditions.

Abstract

We report the case of a late preterm female neonate, born at 36 weeks' gestation, with heterotaxy syndrome, severe congenital heart defects (dextrocardia, situs inversus, left atrial isomerism, complete atrioventricular septal defect (AVSD), and double outlet right ventricle), and biliary atresia. The association of biliary atresia with heterotaxy syndrome is exceptionally rare and adds complexity to the patient´s management. The patient underwent a laparoscopic Kasai procedure, which resulted in significant clinical improvement. This case highlights the importance of early diagnosis, a multidisciplinary approach, and timely surgical intervention in managing rare and complex conditions.

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