Outcomes of paediatric liver transplant for biliary atresia

Abstract

Background: Despite the widespread use of Kasai Portoenterostomy (KPE) for biliary atresia, more than two thirds of these patients require liver  transplant. Liver transplantation is not widely available in South Africa, and Wits Donald Gordon Medical Centre is one of two centres performing  paediatric liver transplantation in the country, and the only centre performing living related donor transplants.

Methods: A retrospective review was performed at the centre. Demographic data were collected, and tabulated. Survival analysis was performed using  the Kaplan Meier method. Complication rates were categorised into biliary, vascular and enteric, and classified as early and late.

Results: Sixty-seven first time liver transplants were performed for biliary atresia at WDGMC from 2005 to 2017. Sixtynine percent were female patients and thirty-one percent were male patients. Forty-eight percent of patients under the age of 5 years had a z-score of -2 or worse for mid upper arm  circumference (MUAC). One year overall survival of the cohort is 84.5%, and overall graft survival is 82.9%. Overall mortality was 22%, with infection being  the most common cause of death.

Conclusion: Early referral of all patients with biliary atresia to a paediatric liver transplant centre is essential for early assessment of indications, and  medical and nutritional optimisation of patients. Primary liver transplant should be considered for a select group of patients with unique clinical  indications.