Severe acute fatty liver in pregnancy: a diagnostic dilemma in clinical practice

Background: Acute fatty liver of pregnancy (AFLP) is an uncommon complication in the third trimester of pregnancy. Differential diagnosis between severe cases of AFLP and others conditions remains challenging since there is no specific diagnostic test for this condition and the diagnosis is made by clinical and laboratory findings.

Objective: To evaluate the clinical presentation, laboratory findings, and clinical outcome in patients with acute fatty liver of pregnancy.

Material and Method: A retrospective study was carried out in all hospitalized pregnant patients who presented with hepatitis in the third trimester at King Chulalongkorn Memorial Hospital (KCMH), between January 2001 and March 2011. The diagnosis of AFLP had been made by clinical symptoms, laboratory evidence of acute hepatitis in the third trimester of pregnancy and by exclusion of other causes.

Results: Of 102,989 deliveries, there was five AFLP, giving an incidence of 1 in 20,598 pregnancies. The mean maternal age and gestational age was 33.6 years and 36 weeks, respectively. The mean length of stay in hospital was 12 days (range 8 to 20 days). Nausea and jaundice were the most common symptoms. It is of interest that one case of AFLP coexisted with the syndrome, which is a combined medical feature of “H” for hemolysis, “EL” for elevated liver enzymes, and “LP” for low platelet count (HELLP). Hypoglycemia was found in all patients requiring continuous infusion of dextrose solution. Acute renal failure was also found in all cases. Initial serum creatinine varied from 1.5 to 3.7 mg/dL. None of the patients required hemodialysis and renal function returned to normal at discharge. Two cases were associated with DIC, which caused postpartum hemorrhage. Liver function tests became normal within 7 to 43 days. There was one case of perinatal death of the fetus and no maternal deaths.

Conclusion: AFLP is an emergency. Multiple organ failures could develop even after delivery. In our experience, some cases of AFLP could overlap with HELLP syndrome or masquerade as TTP in the setting of pregnancy. Careful analysis of the clinical progression is important in the recognition of AFLP and prompt termination of the pregnancy is required to improve maternal and perinatal outcomes.