Liver transplantation for children--the Red Cross Children's Hospital experience.

UNLABELLED: Liver transplantation for infants and children has been available in South Africa at a single centre, the only established service in Sub-Saharan Africa, for more than a decade. Current concerns have shifted from an initial target of early post-transplant survival to quality of life in the long-term. MATERIALS AND METHODS: Since 1985, 225 infants and children have been assessed, with 146 accepted for transplantation. Sixty-nine have had 71 orthotopic liver transplants (OLTx). Biliary atresia was the most frequent diagnosis (54%) followed by acute liver failure (ALF) (15%). Waiting list mortality has remained high (23%), particularly for the ALF group (50%). Forty-three were reduced size transplants with donor: recipient weight ratios ranging from 2:1 to 11:1. Twenty-seven were <10 kg. RESULTS: Fifty (74%) survive 1 month-12 years post-transplant. Actuarial survival after 1996 since HBV core antibody positive donor livers were refused and prophylactic IV ganciclovir used has been >82%. Early post-OLTx mortality was low (5%), one primary non-function, one IVC thrombosis, one PV thrombosis, but late morbidity and mortality (20%) was mainly due to viral infection: de novo hepatitis B (five patients, three deaths), EBV-related post-transplantation lymphoproliferative disease (PTLPD) (eight patients, six deaths) and CMV disease (11 patients, five deaths). Tuberculosis prophylaxis, required in six cases, resulted in major morbidity in two and mortality in one. Poor compliance played a significant role in seven deaths. Hypertension requiring medication along with some compromise of renal function has been present in all but two patients. However, all those of school-going age (25) attend school normally and remain in good health and only three of the survivors have abnormal liver function tests. CONCLUSIONS: Successful liver transplantation is possible in a developing country with limited resources. Scarcity of virus-free donors (HBV and HIV) leading to waiting list mortality and infrequent re-transplantation along with long-term consequences of immunosuppression (infection, lymphoma and renal toxicity) remain problems. Intense education of the caregiver and close follow-up, particularly of those living at long distances has partly addressed the compliance problem.

Extended hepatic resection with transplantation back-up for an "unresectable" tumour.

Liver transplantation (LT) for malignancy has had disappointing long-term results due to tumour recurrence. Ex-vivo dissection and auto-transplantation have had poor results when the tumor was obstructing bile ducts. Advances in liver surgery have made extensive liver resection safer, but cases of "unresectable" tumours due to site and size still present. A 10-year-old boy was referred with jaundice due to a 6 x 8-cm central (segment 4) tumour shown on biopsy to be a fibrolamellar hepatocellular carcinoma. Ultrasound (US) and Computed Tomography also showed dilatation of intrahepatic bile ducts in both lobes. Angiography showed a large tumour mass supplied by the left branch of the hepatic artery, a low take-off of a right branch of the hepatic artery, and a very displaced but patent portal vein. The initial surgical consensus was that the tumour was unresectable. The patient was listed for LT with the plan of first attempting resection with a liver graft-in-waiting. An extended left hepatectomy was performed under total vascular exclusion with resection of the tumour, which had extended from segment 4 into surrounding segments 1, 3, 5, and 8. Intraoperative US assisted in planning the resection. The right hepatic vein, artery, and the right branch of the portal vein could be preserved and a Roux loop was anastomosed to a markedly dilated segment 6 and 7 intrahepatic duct for bile drainage. Vascular exclusion time was 30 min. The patient made a good recovery without major complications. Jaundice and bile-duct dilatation resolved. On follow up at 5 years there was no recurrence. The liver graft-in-waiting gave the surgical team confidence to proceed with an extensive resection beyond a "point of no return" and allowed good clearance of the disease and avoidance of LT with all the long-term consequences of immunosuppression. This mandates that extensive hepatic surgery in children should be carried out in centres that have a facility for LT should the need arise.