Acute Fatty Liver of Pregnancy

Summary

Acute fatty liver of pregnancy (AFLP) is a rare, life-threatening condition of the third trimester, caused by impaired fatty acid β-oxidation. It presents with jaundice, abdominal pain, nausea, vomiting, and may progress to encephalopathy, coagulopathy, and renal failure. Diagnosis relies on clinical findings supported by Swansea criteria, and management requires immediate delivery with supportive care. Maternal mortality is ~2% and fetal mortality 10–20%.

Definition

Acute fatty liver of pregnancy (AFLP) is a rare, idiopathic, and life-threatening obstetric emergency that usually develops in the third trimester. It is characterized by widespread microvesicular hepatic steatosis leading to acute liver failure. The incidence is approximately 1–3 per 10,000 pregnancies. Risk factors include multiple gestation, low maternal body mass index, and underlying defects in maternal–fetal fatty acid β-oxidation.

Clinical Features

Patients typically present with sudden-onset jaundice, right upper quadrant pain, nausea, vomiting, polyuria, and polydipsia. Advanced cases may progress to hepatic encephalopathy, coagulopathy, ascites, and renal impairment. Preeclampsia coexists in up to 50% of cases.

Diagnosis

The diagnosis is primarily clinical, supported by laboratory and imaging studies. The Swansea criteria are commonly applied, with the presence of six or more features suggesting AFLP. Ultrasound can exclude alternative diagnoses, while liver biopsy is rarely required unless the presentation is atypical or hepatic dysfunction persists postpartum. Other causes of acute liver failure, such as viral hepatitis, autoimmune hepatitis, Wilson disease, and drug-induced injury, must be excluded.

Swansea Criteria for Acute Fatty Liver of Pregnancy

Differential Diagnosis

• AFLP often overlaps with HELLP syndrome and severe preeclampsia, making differentiation difficult. 

• Hypoglycemia, hyperuricemia, and severe renal impairment are more characteristic of AFLP.

Management

The mainstay of treatment is immediate delivery, irrespective of gestational age, alongside aggressive supportive care. Management includes correction of hypoglycemia, coagulopathy, renal dysfunction, and close monitoring for multi-organ failure.

Complications and Prognosis

Major complications include:

• Acute liver failure

• Encephalopathy 

• Disseminated intravascular coagulation DIC 

• Acute renal failure 

• Pulmonary edema 

• Pancreatitis

• Sepsis. 

Despite improved outcomes with early recognition and delivery, maternal mortality remains around 2%, and fetal demise occurs in 10–20% of cases.