Fulminant hepatic failure (FHF) is usually defined as the severe impairment of hepatic functions or severe necrosis of hepatocytes in the absence of preexisting liver disease. However, unlike in adults, encephalopathy may be absent, late, or apparent in children only at the terminal stages. Thus, the emphasis in children is placed on the presence of significant coagulopathy in the absence of sepsis or disseminated intravascular coagulation that is not correctable by the administration of parenteral vitamin K within 8 hours.
The exact etiology remains unidentified in many cases of pediatric FHF. Likewise, the pathophysiologic mechanism that leads to hepatic encephalopathy in children with FHF has not been fully defined (see Pathophysiology and Etiology).
FHF affects previously healthy children with no recognized risk factors for liver disease. Children usually present with a hepatitis-like clinical picture; jaundice is the presenting symptom in most patients. Children with FHF are critically ill, and symptoms and level of consciousness rapidly deteriorate. Over a few days to weeks, the condition progresses to coma, with development of ascites, cerebral edema, and decorticate and decerebrate posturing (see Presentation).
A range of laboratory studies is required to determine the etiology, severity, and prognosis in pediatric patients with FHF. Liver biopsy is usually an essential procedure to consider in the management of this condition (see Workup).
Symptomatic treatment and life support should be provided to patients. Direct treatment is toward the specific cause of FHF when an identifiable etiology is found. An intensive care unit (ICU) and pediatric hepatology setting with facilities for liver transplantation should be available for proper diagnosis and management. Orthotopic liver transplantation remains the only effective mode of treatment of FHF (see Treatment and Management).
Pharmacologic intervention is usually directed at causative agents or the treatment of complications (see Medication).
See Acute Liver Failure for more information on this topic.