LIVER-TRANSPLANTATION FOR EXTRA HEPATIC BILIARY ATRESIA

Kasai portoenterostomy has transformed the prognosis for children with Extra Hepatic Biliary Atresia (ERBA). However, for children developin g end stage liver disease following portoenterostomy, liver transplant ation (OLT) is the treatment of choice. Between February 1989 and Marc h 1996, 64 children with EHBA underwent 79 transplants (26 males, 38 f emales; median age 2.2 years, range 5 months-17 years; median weight 1 1.4 kg, range 5-65 kg). Of these, 58 (85%) had undergone previous port oenterostomy. Nineteen patients (30%) had gastrointestinal bleeding pr ior to OLT assessment. Mean serum bilirubin was 229 mu mol/liter (rang e 11-801 mu ml/liter). Four children had associated polysplenia syndro me. Of the 79 transplants, 30 received whole and 41 reduced-size cadav eric grafts and 9 living related grafts. Eleven patients (17%) died, n ine within one month of surgery. Thirteen patients were retransplanted once and one twice. There were 16 vascular complications (10 hepatic artery thrombosis, 3 portal vein thrombosis, 3 venous outflow obstruct ion) and 10 biliary complications (4 anastomotic leaks, 6 strictures). Ten patients (16%) had bowel perforation following the transplant. Th e 5 year actuarial patient and graft survival for this group is 84% an d 69% respectively with normal physical and mental development in the majority. OLT provides satisfactory treatment for children with EHBA w ith end stage liver disease with long term survival in the majority.