Delayed encephalopathy in fulminant hepatic failure in the pediatric population and the role of liver transplantation.

BACKGROUND: Liver transplantation is the therapeutic choice for fulminant hepatic failure in children.
METHODS: All 66 cases of fulminant hepatic failure in the pediatric population seen at UCLA from May, 1985 to November, 1993 were reviewed to determine changes in survival rates since the advent of liver transplantation. We evaluated the clinical course and events leading to the exclusion of surgical management of nonsurvivors, who otherwise would have benefited from a liver transplant. We also compared the latter's clinical course with the nontransplant survivors to determine parameters for screening patients for liver transplantation.
RESULTS: Fifty-one patient (77%) were put on the transplant list initially but eventually, only 38 (58%) patients underwent orthotopic liver transplantation (OLT) and of these 30 (79%) patients survived. Of the remaining 29 (42%) patients who did not undergo liver transplantation, only 10 (36%) patients survived. Nine patients died while waiting for a donor liver secondary to complications of hepatic failure. The majority of nonsurvivors in the OLT and no-OLT groups succumbed because of irreversible neurologic deterioration. In the no-OLT group, comparisons between survivors and non-survivors were made. There were no significant demographic differences. It took a mean of 8 days (+/-8) versus 22 days (+/-15), (p = 0.009), from onset of illness to first hospital admission for survivors and nonsurvivors, respectively. Time to reach stage II encephalopathy was a mean of 5 days (+/-5) for survivors versus 18 days (+/-16), (p = 0.05) for nonsurvivors. Nonsurvivors were transferred to the transplant center at a mean of 12.2 days (+/-12) after being first admitted elsewhere as compared to a mean of 1.9 days (+/-18) for survivors, (p = 0.02). Mean prothrombin time decreased by a mean of 13.4 s/day (+/-16) for survivors as against 2.25 s/day (+/-6) for nonsurvivors, (p = 0.06). Mean peak total bilirubin for nonsurvivors was 460 mumol/L (27 mg/dl) versus 220 mumol/L (13 mg/dl) for survivors, (p = 0.06). Nonsurvivors died at a mean of 30 days (+/-19) from onset and survivors' liver tests started to improve at a mean of 11 days (+/-9) from onset.
CONCLUSIONS: From these studies, we conclude that liver transplantation remains the therapeutic choice for fulminant hepatic failure in children. Early referral and closer follow-up is necessary for timely admission to liver transplant centers to enable screening and proper preparation of these patients for liver transplantation.