Fatty Liver of Pregnancy, Acute

Key Features

• Acute liver failure in the third trimester of pregnancy

• Mortality of 7–23% with early delivery

• Likely cause is the result of poor placental mitochondrial function

• Many cases may be due to fetal long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency

• Fatty engorgement of hepatocytes seen on CT of liver and on biopsy

• Incidence of 1:14,000 deliveries

Clinical Findings

• Gradual onset of flu-like symptoms

• Progression to jaundice, encephalopathy, disseminated intravascular coagulation, and death

• Signs of liver failure are present on examination

• All etiologies of acute liver failure should be considered, but transaminase levels are lower (500–1000 units/mL) in acute fatty liver of pregnancy

Diagnosis

• Marked elevation of alkaline phosphatase

• Only moderate alanine aminotransferase and aspartate aminotransferase elevations

• Hypocholesterolemia and hypofibrinogenemia are typical

• Coagulopathy is also frequently seen with depressed procoagulant protein production

• Kidney function should be assessed for hepatorenal syndrome

• White blood cell count is elevated; platelet count is depressed

• Hypoglycemia may be extreme

Treatment

• Intensive supportive care with ICU-level observation is essential and typically includes administration of blood products and glucose as well as correction of acidemia

• Diagnosis mandates immediate delivery, preferably vaginally

• Rare cases of liver transplantation have been reported

• Resolution of encephalopathy occurs over days and laboratory derangements occurs over days with supportive care

• Recurrence rates are unclear