Fulminant hepatic failure (FHF) is defined as the development of hepatic necrosis with encephalopathy occurring within 12 weeks of onset of jaundice in the absence of pre existing symptomatic liver disease. This is further sub classified depending on the interval between the onset of jaundice and encephalopathy into hyperacute liver failure (interval 0-1 week), acute liver failure (interval 1-4 weeks) and subacute hepatic failure (interval 4-12 weeks). This classification has important prognostic implications with the most favorable prognosis for hyperacute liver failure. Viral hepatitis A is the commonest cause of FHF in children in India.

The essential aspects of treatment are - prevention and treatment of complications, provision of hepatic support and hepatic replacement, if need be. The general management includes fluid restriction to 75% of maintenance to prevent cerebral edema, prevention of dyselectrolytemia and hypoglycemia. The circulating volume needs to be maintained with crystalloid and colloid solutions. Prevention of gastrointestinal bleed using ranitidine and sucralfate is recommended.

The children in FHF are prone to developing sepsis hence the use of broad-spectrum antibiotics is warranted. In non G-6PD deficient children intravenous vitamin-K is useful in controlling coagulopathy. However, severe coagulopathy requires fresh frozen plasma.

The management of encephalopathy includes protein restriction, oral lactulose along with fluid restriction and intra venous mannitol. Elective ventilation is required in grade III/IV. N-acetyl cysteine has been shown to be useful even in non-paracetamol induced FHF by enhancing oxygen delivery by augmenting intracellular cysteine and glutathione, thus improving survival.

Artificial liver support using porcine hepatocytes or hepatoma cell lines has improved coagulopathy and reduced encephalopathy in limited studies. Recently MARS (Molecular Absorbent Regenerating system), which is a cell free extracorporeal liver assist device, has been used to replace the detoxification function liver. Although useful as a "bridge to transplantation", long term outcome and survival has not been affected by these modalities.

Liver transplantation (LT) is the only solution for children who do not respond to above measures. The questions that need to be answered are whether or not spontaneous recovery is possible. If not, the feasibility of such a procedure needs to be assessed as presence of irretrievable complications is a definite contraindication. The King's criteria are useful in identifying the candidates for LT. Younger age (<10 yrs), rising bilirubin and prothrombin time along with worsening encephalopathy are indications for urgent referral to a specialized center with facilities for LT.

NCPCC 2005 - Conference Abstracts.Pediatric Oncall [serial online] 2006 [cited 15 May 2006(Supplement 5)];3. Available from:
http://www.pediatriconcall.com/fordoctor/Conference_abstracts/
Fulminant_hepatc_failure.asp