Objective: To review the clinical characteristics, outcomes, and risk factors for survival among 57 pediatric patients undergoing orthotopic liver transplantation for fulminant hepatic failure at the University of California, Los Angeles, Center for the Health Sciences.
Design: The medical records of 57 consecutive pediatric patients undergoing orthotopic liver transplantation for fulminant hepatic failure from July 1, 1984, to June 25, 1997, were reviewed and survival data were analyzed via univariate and multivariate statistical methods. The type and incidence of posttransplant complications were determined as was the quality of long-term graft function. Median follow-up period was 3.38 years (range, 0-10.02 years).
Results: The 1-, 3-, and 5-year actuarial patient survival rates were 77%, 77%, and 77%, respectively, while graft survivals were 73%, 65%, and 65%. Stepwise Cox regression analysis revealed that recipient age and ventilator dependency at the time of transplantation were independently and significantly correlated with patient survival, whereas no association was found between survival and grade of encephalopathy, prior abdominal surgery, recipient weight, pretransplantation values for total bilirubin or prothrombin time, ABO match, allograft type, peak posttransplantation aspartate aminotransferase levels, or the presence of posttransplantation hepatic artery thrombosis. Non-ventilator-dependent patients demonstrated a 96% 1-, 3-, and 5-year survival as compared with only 56% at these same time points for those children requiring ventilator support at the time of transplantation (P < .001). At the time of most recent follow-up, median values for total bilirubin and aspartate aminotransferase concentrations were 10.3 micromol/L (0.6 mg/dL) and 56 U/L, respectively, in the 40 surviving patients.
Conclusions: In children undergoing liver transplantation for fulminant hepatic failure: (1) overall results are comparable to those achieved for less emergent non-neoplastic indications in this same age group; (2) ventilator dependency prior to transplantation is the strongest predictor of ultimate survival, followed by recipient age; (3) 5-year survival exceeds 90% in recipients who are ventilator independent immediately prior to liver transplantation but is significantly compromised once the need for mechanical ventilation supervenes, particularly in those younger than 4 years; and (4) prompt referral and timely liver replacement are the cornerstones of optimal outcome.