Chapter 83

• Fulminant hepatic failure (FHF) is defined as the onset of hepatic encephalopathy (HE) within 8 weeks of the onset of liver-related symptoms.
• Common complications in patients with FHF include encephalopathy, cerebral edema, cardiovascular instability, renal failure, and infection.
• Supportive management of FHF consists of (1) airway protection, (2) intracranial pressure monitoring in the sickest patients, and (3) treatment of complications and vigilance for infection.
• Orthotopic liver transplantation (OLT) is the definitive therapy for FHF. Extracorporeal liver support remains investigational.

Fulminant hepatic failure (FHF) describes a clinical syndrome of progressive hepatocyte necrosis resulting in the dysfunction of virtually every major organ system. Consequently, management of patients with FHF is complicated and unpredictable, where many of the therapies initiated have not been proven effective in controlled clinical trials. Management of this syndrome requires early recognition and treatment of organ system deterioration because overall patient survival is inversely related to the number and extent of extrahepatic organ dysfunction. Therefore, treatment of these organ failures should be highly individualized and tailored toward anticipation and prevention of multisystem organ failure while waiting for recovery of hepatic function or liver transplantation.

Perhaps the most difficult dilemma in treating patients with FHF is the inability to predict which patients will recover adequate liver function and survive an episode of FHF without liver transplantation. Among patients with FHF who have progressed to grade 3 or 4 encephalopathy, expected recovery with medical management alone is approximately 10% to 40%.1 The advent of liver transplantation has improved survival markedly to 60% to 80% and is currently considered the treatment of choice for progressive FHF.1 Thus the overall treatment strategy has evolved into early stabilization of the FHF patient, optimization of the patient's general condition, and early and continued evaluation for liver transplantation.

Trey and Davidson2 originally proposed the term fulminant hepatic failure, which describes a condition of massive hepatic cell necrosis (Fig. 83-1) that is clinically evident by the progression to encephalopathy within 8 weeks of the onset of jaundice. Since this initial definition, numerous alterations and terms have been proposed that often complicate rather than clarify understanding of this disease process. A more concise definition proposed by O'Grady and colleagues3 attempts to precisely categorize liver failure into three types based on the time interval between the onset of jaundice and the emergence of encephalopathy: hyperacute (0 to 7 days), acute (8 to 28 days), and subacute (29 days to 12 weeks). The utility of this terminology is supported by the different clinical features and associated mortalities within each category (Table 83-1). Patients with hyperacute FHF tend to have rapidly progressive cerebral edema, to improve with medical management, and to have an overall favorable prognosis. Patients with acute FHF also have rapidly progressive FHF, but they tend to have a worse prognosis without liver transplant. Subacute FHF patients have a lower incidence of cerebral edema but an overall poor ...

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