A Life-Saving Liver Transplant in Salvaging a Case of Complicated Biliary Atresia - A Case Report

Abstract

Background: Biliary atresia involves the progressive obliteration of the hepatic bile duct. It is a common cause of surgical jaundice in neonates, and is one of the commonest indications for liver transplant in children. The treatment is multidisciplinary, involving the surgical re-establishment of bile drainage into the intestine through a portoenterostomy. In certain patients with hepatic complications, liver transplantation is the definitive modality of treatment, however this is not readily available in Nigeria. We aim to share our experience and the surgical outcome in the management of a patient with biliary atresia who had a successful liver transplant.

Case Summary: A 2-year-old female child, who presented at 7 months of age on account of persistent yellowness of the eyes since birth, with associated passage of pale stool. Initial care was at different peripheral facilities, where she had been on medical therapy, with no improvement in symptoms, accounting for a delayed presentation to our teaching Hospital.

She had an exploratory laparotomy, with intraoperative findings of a hard craggy cirrhotic liver and an atretic gall bladder. Liver biopsy done showed cholestatic liver with cirrhosis.

She was subsequently referred to a facility in India where she had a successful liver transplantation done. Procedure was well tolerated with significant improvement in clinical and biochemical parameters, from a pre-operative conjugated bilirubin level of 11.4mg/dl (0-0.4mg/dl) to a current post-operative level of 0.2mg/dl

Conclusion and Recommendations: Liver transplant remains an important modality in the treatment of biliary atresia, with good surgical outcome. The management of biliary atresia however pose a challenge in Nigeria due to delayed presentation, and lack of facility for liver transplant. Despite these challenges, through an international collaborative effort, we were able to save the life of the patient and re-unite her with her family.