Liver transplantation for extra hepatic biliary atresia

Area of Science:

• Pediatric Surgery
• Hepatology
• Transplantation Immunology

Background:

• Kasai portoenterostomy improved prognosis for Extra Hepatic Biliary Atresia (EHBA).
• End-stage liver disease in EHBA patients often necessitates liver transplantation (OLT).
• Previous portoenterostomy was common in children evaluated for OLT.

Purpose of the Study:

• To evaluate the outcomes of liver transplantation (OLT) in children with Extra Hepatic Biliary Atresia (EHBA).
• To assess patient and graft survival rates after OLT in this pediatric population.
• To analyze complications and long-term development following OLT for EHBA.

Main Methods:

• Retrospective analysis of 79 liver transplants performed between 1989 and 1996 in 64 children with EHBA.
• Inclusion of data on prior portoenterostomy, gastrointestinal bleeding, serum bilirubin, and associated syndromes.
• Categorization of grafts (whole, reduced-size cadaveric, living related) and documentation of complications and retransplants.

Main Results:

• A total of 79 transplants were performed in 64 children with EHBA; 85% had prior portoenterostomy.
• Vascular complications occurred in 16 patients, biliary complications in 10, and bowel perforation in 10.
• Five-year actuarial patient survival was 84% and graft survival was 69%, with normal development in most survivors.

Conclusions:

• Liver transplantation (OLT) is a satisfactory treatment for children with end-stage liver disease due to EHBA.
• OLT provides the majority of patients with EHBA long-term survival and positive developmental outcomes.
• Despite complications, OLT significantly improves the prognosis for children with EHBA requiring liver replacement.