LIVING-RELATED LIVER-TRANSPLANTATION IN CHILDREN

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 t o 15 years with biliary atresia (27), cryptogenic cirrhosis (3), Budd- Chiari syndrome (2), progressive intrahepatic cholestasis (2), protopo rphyria (1), Wilson's disease (1), and fulminant hepatitis (1). The do nors were 14 fathers and 23 mothers. Grafts were made from the left la teral segment (19), left lateral segment with partial S4 (11), left lo be (6), and right lobe (1). After graft harvesting all donors resumed normal liver function and normal life. The recipient underwent total h epatectomy with preservation of the inferior vena cava. FM506 and low- dose steroids were used for immunosup pression. The survival rate was 90% (27/30) in elective eases and 57% (4/7) in emergency cases. Six re cipients 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 o ccurred in 1 case at 8 months after LRLT and was also safely dilatated . We incurred no hepatic artery thrombosis after introducing microsurg ery techniques. Among 12 viral, 5 bacterial, and 3 fungal postoperativ e infections, 1 Candida pneumonia and 1 EBV-associated lymphoma were l ethal. Three patients with ABO-blood group compatible grafts and one w ith an incompatible graft developed acute rejection, which was control led in every 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 (greater than or equal to-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 heig ht. Appropriate timing, meticulous surgical procedures, and comprehens ive management of complications are crucial for successful outcome wit h LRLT. LRLT is a promising option for alleviating the shortage of liv ers for pediatric transplantation and may be regarded as an independen t modality to supplement cadaver donation.