Tohoku J. Exp. Med., 1997, 181 (1)

Liver Transplantation for Extra Hepatic Biliary Atresia

SANJAY NAGRAL1, PAOLO MUIESAN1, HECTOR VILCA-MELENDEZ1, GIORGINA MIELI-VERGANI2, ALISTAIR BAKER2, JOHN KARANI3, EDWARD HOWARD4, MOHAMED RELA and NIGEL HEATON

1Liver Transplant Surgical Service, 2Department of Child Health, 3Department of Radiology, 4Department of Paediatric Surgery, King 's College Hospital, Denmark Hill, London, SE5 9RS UK

  • Kasai portoenterostomy has transformed the prognosis for children with Extra Hepatic Biliary Atresia (EHBA). However, for children developing end stage liver disease following portoenterostomy, liver transplantation (OLT) is the treatment of choice. Between February 1989 and March 1996, 64 children with EHBA underwent 79 transplants ( 26 males, 38 females; median age 2.2 years, range 5 months-17 years; median weight ll.4 kg, range 5-65 kg). Of these, 58 (85%) had undergone previous portoenterostomy. Nineteen patients ( 30%) had gastrointestinal bleeding prior to OLT assessment. Mean serum bilirubin was 229 micro mol/liter (range 11-801 micro ml/liter). Four children had associated polysplenia syndrome. Of the 79 transplants, 30 received whole and 41 reduced-size cadaveric grafts and 9 living related grafts. Eleven patients (17%) died, nine 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 obstruction) and 10 biliary complications (4 anastomotic leaks, 6 strictures). Ten patients (16%) had bowel perforation following the transplant. The 5 year actuarial patient and graft survival for this group is 84% and 69% respectively with normal physical and mental development in the majority. OLT provides satisfactory treatment for children with EHBA with end stage liver disease with long term survival in the majority.
    Key word(s)--- biliary atresia; liver transplantation; portoenterostomy

    Tohoku J. Exp. Med., 1997, 181, 117-127
    Address for reprints: Sanjay Nagral, M.D., Liver Transplant Surgical Service, Department of Child Health, King's College Hospital, Denmark Hill, London SE5 9RS, UK.

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