Acute fatty liver of pregnancy is a rare and life-threatening disease associated with a defect in fatty acid metabolism in the fetus that causes liver disease in the mother. Prompt diagnosis and management are critical to the outcome of both the mother and the fetus and require involvement of several medical specialties, including hepatology, obstetrics, and, possibly, critical care. The included case study describes a woman with acute fatty liver of pregnancy decompensating to acute liver failure complicated by encephalopathy, cerebral edema, and intracranial hypertension. Subsequent management of these conditions, including the woman’s progression to liver transplant, is provided.
A normal pregnancy can be a time filled with great expectation and joy. In contrast, a pregnancy with medical complications can be overwhelming for the expectant mother, the family, and the health care team. Acute fatty liver of pregnancy (AFLP) is a pregnancy-related disease caused by a metabolic defect in the fetus that results in acute damage of the mother’s liver, putting both the expectant mother and the fetus in jeopardy.
When AFLP was first described in 1940, the mortality rate was 85%.1 Maternal mortality was 0.13 per 100000 live births for the period 1983 to 2006 in a large retrospective population-based study in the Netherlands, and 3.2 per 100000 deliveries for 2004 to 2006.1 Rare and potentially fatal, AFLP usually becomes evident in the third trimester of pregnancy but can occur as early as 26 weeks’ gestation and as late as the postpartum period.