Acute fatty liver of pregnancy: an experience in the diagnosis and management of fourteen cases.

OBJECTIVE

Our purpose was to investigate the diagnostic problems and maternal-perinatal outcome in cases of acute fatty liver of pregnancy.

STUDY DESIGN

Fourteen cases with acute fatty liver of pregnancy managed during the past 8-years were studied with emphasis on presenting symptoms, admitting diagnosis, laboratory findings, clinical course, maternal complications, and neonatal outcome.

RESULTS

The mean gestational age at onset was 34.5 weeks (range 28 to 39). Only seven patients had acute fatty liver of pregnancy as a definite or suspected diagnosis on admission. Computed tomography of the liver was performed on 10 patients, with only two positive results. There were no maternal deaths; however, maternal morbidity was frequent: four patients had hepatic encephalopathy, three pulmonary edema, three ascites, four respiratory arrest, two diabetes insipidus, and 10 had transfusion of blood or blood products to correct either disseminated intravascular coagulation or excessive bleeding. Coagulation abnormalities were common: hypofibrinogenemia (< 300 mg/dl) in 13 patients (93%), prolonged prothrombin time in 12 (86%), and prolonged partial thromboplastin time in 11 (79%). The corrected perinatal mortality was 6.6%.

CONCLUSION

Acute fatty liver of pregnancy should be suspected in all patients with symptoms of preeclampsia in the presence of hypoglycemia, low fibrinogen, and prolonged prothrombin time, particularly in the absence of severe abruptio placentae. Computed tomography of the liver has a high false-negative rate in patients with acute fatty liver of pregnancy. In spite of the literature's dismal prognosis, our findings indicate that maternal and perinatal outcomes appear favorable in well-managed patients.