Management of Acute Fatty Liver of Pregnancy (AFLP)
Immediate delivery should be expedited once coagulopathy and metabolic derangements have been treated, as this is the definitive treatment for AFLP to reduce maternal mortality and morbidity. 1
Diagnosis
AFLP is a rare but serious obstetric emergency occurring in approximately 0.01% of pregnancies, typically in the third trimester or early postpartum period 2. Diagnosis is based on:
Clinical Presentation
- Characteristic "AFLP-Triad" 3:
- Symptoms: Nausea/vomiting, jaundice, abdominal pain
- Laboratory abnormalities: Renal dysfunction, coagulopathy, liver function abnormalities, hypoglycemia
- Complications: Renal failure, coagulopathy, ascites, encephalopathy
Diagnostic Criteria
- Swansea criteria: Presence of ≥6 of the following findings strongly correlates with AFLP diagnosis 2, 1:
- Vomiting
- Abdominal pain
- Polydipsia/polyuria
- Encephalopathy
- Elevated transaminases (AST or ALT >42 IU/L)
- Elevated bilirubin (>0.8 mg/dL)
- Hypoglycemia (<72 mg/dL)
- Leukocytosis (>11 × 10^6/L)
- Elevated uric acid (>5.7 mg/dL)
- Elevated ammonia (>42 IU/L)
- Ascites or bright liver on ultrasound
- Renal impairment (creatinine >1.7 mg/dL)
- Coagulopathy (PT >14 seconds or PTT >34 seconds)
- Microvesicular steatosis on biopsy (if performed)
Differential Diagnosis
- HELLP syndrome
- Preeclampsia
- Intrahepatic cholestasis of pregnancy
- Viral hepatitis
Management Algorithm
Step 1: Initial Stabilization
- Transfer to high-dependency or intensive care unit for women with:
- Encephalopathy
- Elevated serum lactate
- MELD score >30
- Swansea criteria >7 1
Step 2: Correct Metabolic Derangements
- Treat hypoglycemia (present in up to 94.2% of cases) 3
- Correct coagulopathy with fresh frozen plasma and blood products 2
- Manage renal dysfunction with fluid management and renal replacement therapy if needed
Step 3: Expedite Delivery
- Delivery is the definitive treatment and should be performed once maternal coagulopathy and metabolic derangements are corrected 2, 1
- Mode of delivery should be determined by a multidisciplinary team including obstetricians and hepatologists 1
- For fetal demise, expedited vaginal induction could be considered instead of cesarean section 2
Step 4: Post-Delivery Management
- Continue intensive monitoring for:
Step 5: Advanced Therapies for Severe Cases
- Consider plasma exchange for severe cases to improve maternal disease severity and decrease recovery time 1
- N-acetylcysteine may be beneficial for women requiring intensive care admission 1
- Evaluate for liver transplantation in patients who progress to acute liver failure, especially those who have already given birth but do not improve clinically 2
Postpartum Care and Follow-up
Maternal Follow-up
- Monitor liver function tests until normalization
- Complete recovery of hepatic function is expected in most cases 4
Neonatal Care and Screening
- Screen newborns for long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency 2, 1
- Provide genetic counseling for affected mothers and their newborns 2
- Newborns with LCHAD deficiency can be treated with dietary modifications to reduce morbidity and mortality 2
Pitfalls and Caveats
- Delayed diagnosis is common and dangerous; AFLP is often diagnosed on the second hospital day after laboratory results indicate coagulopathy, renal insufficiency, and liver function abnormalities 4
- Liver biopsy is rarely needed and should not be obtained unless it will affect management 2
- Radiologic studies generally do not aid in diagnosis 4
- AFLP can be difficult to distinguish from HELLP syndrome; approximately 50% of AFLP patients may have features of pre-eclampsia 2
- Maternal mortality has improved from historical rates of up to 92% to less than 10% in recent years with prompt diagnosis and management 2
Early recognition and prompt delivery remain the cornerstones of successful management of AFLP, with multidisciplinary care essential to optimize maternal and fetal outcomes.