Outcomes of paediatric liver transplant for biliary atresia

• Published in: South African journal of surgery Vol. 57; no. 3; pp. 17 - 23
• Main Authors: Van Heerden, Y., Botha, J., Maher, H., Grieve, A., Loveland, J., Etheredge, H., Fabian, J.
• Format: Journal Article
• Language: English
• Published: South Africa Medpharm Publications 01.09.2019; South African Sports Medicine Association
• Subjects: Analysis; Biliary atresia; Biliary Atresia - surgery; Child; Child, Preschool; Female; Graft Survival; Health aspects; Humans; Infant; Infant, Newborn; Infections - etiology; Infections - mortality; Kaplan-Meier Estimate; Liver transplant; Liver Transplantation - adverse effects; Liver Transplantation - mortality; Liver transplants; Male; Medical centers; Mortality; Organ transplantation; Paediatric; Patient Selection; Pediatrics; Postoperative Complications - etiology; Postoperative Complications - mortality; Retrospective Studies; South Africa; Survival analysis; Survival Rate; Tissue donation; Treatment Outcome; South Africa
• ISSN: 0038-2361; 2078-5151
• DOI: 10.17159/2078-5151/2019/v57n3a3069

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. Sixty-nine 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.Abbreviations: BA, Biliary Atresia; WDGMC, Wits Donald Gordon Medical Centre; KPE, Kasai Portoenterostomy; MUAC, Mid Upper Arm Circumference; LDLT, Living Donor Liver Transplant; SNL, Survival with Native Liver