Fulminant, or acute, hepatic failure is defined as severe hepatocyte dysfunction resulting in rapid elevation of aminotransferases, encephalopathy, coagulopathy and multiorgan failure in an otherwise healthy individual without preexisting liver disease. Acute liver failure (ALF) has an incidence of 1–2/100,000 people in the United States or approximately 3,000–6,000 cases per year with nearly 30% of patients requiring a liver transplantation. ALF is fundamentally different and should not be confused with acute or chronic liver failure or decompensated cirrhosis, as the etiology of ALF is the most important determinant of transplant-free survival.

Key Learning Points

1. 1. Hepatorenal syndrome (HRS) is the end result of progressive impairment of renal perfusion secondary to worsening hepatic failure.

2. 2. HRS is a diagnosis of exclusion of acute kidney injury in chronic liver disease.

3. 3. Both forms of HRS (types 1 and 2) have a poor prognosis, although HRS 1 typically leads to death within weeks.

4. 4. Hepatopulmonary syndrome (HPS) develops from transpulmonary shunting, with resultant hypoxaemia.

This chapter provides anoverview of the surgical treatment for biliary atresia, the Hepatic Portoenterostomy or Kasai Procedure. The pathophysiology of biliary atresia is reviewed as it related to the organ systems effected. The signs and symptoms of neonatal liver failure are discussed including the development of hepatorenal and hepatopulmonary syndromes. The chapter provides anoverview of the surgical aspects and anesthetic concerns related to the Kasai procedure. The chapter provides a segue into the chapter on liver transplantation.