Management of Acute Fatty Liver of Pregnancy in a Third-Trimester Primigravida
Expedited delivery is the definitive treatment for acute fatty liver of pregnancy (AFLP) once coagulopathy and metabolic derangements have been corrected, with consultation from obstetrics and hepatology required immediately upon diagnosis. 1, 2
Immediate Recognition and Stabilization
Diagnosis should be established using the Swansea criteria (≥6 of the following): vomiting, abdominal pain, polydipsia/polyuria, encephalopathy, elevated transaminases (>42 IU/L), elevated bilirubin (>0.8 mg/dL), hypoglycemia (<72 mg/dL), leukocytosis (>11×10⁶/L), elevated uric acid (>5.7 mg/dL), elevated ammonia (>42 IU/L), ascites or bright liver on ultrasound, renal impairment, and coagulopathy. 2, 3
Critical red flags requiring ICU admission include:
- Development of encephalopathy 2, 3
- Elevated serum lactate (>2.8 mg/dL) 3
- MELD score >30 2, 3
- Swansea criteria score >7 2, 3
Before delivery, correct the following:
- Coagulopathy with fresh frozen plasma and blood products 2, 4
- Hypoglycemia with continuous dextrose infusion (this is characteristic of AFLP and requires vigilant monitoring) 1, 2, 3
- Metabolic derangements including electrolyte abnormalities 2
Delivery Planning
The mode of delivery should be determined jointly by the multidisciplinary team (obstetrician, obstetric anesthetist, hepatologist, obstetric physician) based on gestational age, fetal condition, severity of maternal condition, and cervical status. 1, 2
Anesthetic considerations are critical:
- Maternal thrombocytopenia or coagulopathy may preclude regional anesthesia (spinal or epidural), necessitating general anesthesia for cesarean section 1
- For vaginal delivery when regional anesthesia is contraindicated, systemic opiates (e.g., remifentanil with patient-controlled administration) are the only safe alternative for pain relief 1
Post-Delivery Management
Recovery is typically rapid after delivery with supportive care as the primary treatment. 1, 2
For severe cases requiring ICU admission, consider:
- Plasma exchange post-delivery to improve maternal disease severity and decrease recovery time 2
- N-acetylcysteine in women requiring intensive care admission, though evidence is limited 1, 2
Early referral to a transplant center is mandatory for women with severe hepatic impairment who do not show rapid improvement after delivery, as postpartum transplantation is occasionally necessary. 1, 2
Critical Post-Delivery Actions
Screen the newborn for long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency, as there is a strong association between AFLP and this fetal metabolic disorder. 2
Monitor for normalization of liver function tests within 3 months post-delivery. 2 If liver function tests remain abnormal beyond this timeframe, investigate for underlying chronic liver disease such as primary biliary cholangitis, primary sclerosing cholangitis, ABCB4 deficiency, or chronic hepatitis C. 1
Common Pitfalls to Avoid
Do not delay delivery while attempting medical management alone - early recognition and prompt delivery are critical for achieving good outcomes and have dramatically improved maternal morbidity and mortality. 1, 4, 5
Do not confuse AFLP with HELLP syndrome. Key distinguishing features of AFLP include: prolonged PT, low fibrinogen, hypoglycemia, and encephalopathy (if present, highly suspicious for AFLP over HELLP). 3 HELLP syndrome typically presents with hemolysis, thrombocytopenia often <50,000/μL, and is associated with preeclampsia. 1
Do not overlook the possibility of concurrent conditions - approximately 50% of AFLP patients have features of preeclampsia. 1