Liver transplantation in children: The initial Toronto experience

The Hospital for Sick Children's initial 2-year experience with pediatric liver transplantation is reviewed. Patients are divided into high- and low-risk groups according to certain criteria. The high-risk group includes patients under 10 kg in weight, those with extrahepatic biliary atresia (EHBA), those with portal vein atresia or thrombosis, and those in hepatic coma. All others were considered low risk. Twenty-nine patients were assessed for transplantation: 18 were transplanted and 6 (21% of total referred) died while on the waiting list. Eighteen patients received 23 transplants. Of the 18 recipients, nine had EHBA, four had fulminant hepatic failure, two had tyrosinemia, one had glycogen storage disease, one had Indian childhood cirrhosis, and one had idiopathic cirrhosis. Seven of the 13 patients in the high-risk group survived (55% survival) with 1 to 23 month follow-up. Survival was significantly higher (80%) in the low-risk group (P<0.05). Four patients were retransplanted and two survived. Early deaths occurred from prolonged warm ischemia, recurrent portal vein thrombosis, and brain death in a patient who had been transplanted in hepatic coma. Late deaths occurred from cytomegalovirus (CMV) disease (2 patients), acute rejection (1 patient), and myocardial infarction (1 patient). The incidence of primary nonfunction was 4.3% (1 of 23) and of arterial thrombosis was 13% (3 of 23). Survival in patients transplanted for EHBA (67%) was slightly higher than it was for the rest of the group, although not as good as it was in the low-risk group. Complicating factors frequently seen in patients with EHBA at transplantation were small size (mean weight, 9.7 kg) and high incidence of portal vein atresia or hypoplasia (6 of 9). Survival in patients with fulminant hepatic failure was 50%. Four of the 18 patients developed primary CMV infection, and three died. Survival in patients with primary CMV infection was significantly lower than it was in other patients. Our early experience in pediatric liver transplantation confirms that survival in children with certain risk factors may be decreased. Earlier referral for transplantation, prevention of primary CMV disease through matching for CMV status, or better treatment of established CMV infection should considerably improve survival in small pediatric liver recipients.