Am J Surg 1994 Jul;168(1):41-8

Living related liver transplantation in children

Tanaka K, Uemoto S, Tokunaga Y, Fujita S, Sano K, Yamamoto E, Sugano M, Awane M,
Yamaoka Y, Kumada K, et al

Second Department of Surgery, Faculty of Medicine, Kyoto University, Japan

Abstract

We reviewed 37 living related liver transplantations (LRLT) performed by our
department during the last 27 months on children with end-stage liver disease.
The patients were 15 boys and 22 girls aged 7 months to 15 years with biliary
atresia (27), cryptogenic cirrhosis (3), Budd-Chiari syndrome (2), progressive
intrahepatic cholestasis (2), protoporphyria (1), Wilson's disease (1), and
fulminant hepatitis (1). The donors were 14 fathers and 23 mothers. Grafts were
made from the left lateral segment (19), left lateral segment with partial S4
(11), left lobe (6), and right lobe (1). After graft harvesting all donors
resumed normal liver function and normal life. The recipient underwent total
hepatectomy with preservation of the inferior vena cava. FK506 and low-dose
steroids were used for immunosuppression. The survival rate was 90% (27/30) in
elective cases and 57% (4/7) in emergency cases. Six recipients had functioning
grafts but died of extrahepatic complications. Hepatic vein stenosis occurred in
3 cases at 3 months after LRLT and was successfully treated by balloon
dilatation. Portal vein stenosis occurred in 1 case at 8 months after LRLT and
was also safely dilated. We incurred no hepatic artery thrombosis after
introducing microsurgery techniques. Among 12 viral, 5 bacterial, and 3 fungal
postoperative infections, 1 Candida pneumonia and 1 EBV-associated lymphoma were
lethal. Three patients with ABO-blood group compatible grafts and one with an
incompatible graft developed acute rejection, which was controlled in evey case
by steroid bolus and/or increasing the dose of FK506. There were no definite
episodes of rejection in ABO-identical cases. Children with moderate growth
retardation (> or = -1.5 SD of normal growth) caught up in growth soon after
LRLT, but those with severe retardation (<-1.5 SD) were slow to attain
age-normal height. Appropriate timing, meticulous surgical procedures, and
comprehensive management of complications are crucial for successful outcome
with LRLT. LRLT is a promising option for alleviating the shortage of livers for
pediatric transplantation and may be regarded as an independent modality to
supplement cadaver donation

PMID: 7517649, UI: 94295884