Management of Acute Fatty Liver of Pregnancy
The cornerstone of AFLP management is immediate obstetric consultation and expeditious delivery once maternal coagulopathy and metabolic derangements have been corrected, as this is the only definitive treatment that prevents maternal and fetal mortality. 1
Immediate Recognition and Risk Stratification
Clinical Diagnosis
Use the Swansea criteria for diagnosis, requiring ≥6 of the following: vomiting, abdominal pain, polydipsia/polyuria, encephalopathy, elevated transaminases (>42 U/L), elevated bilirubin (>14 μmol/L), hypoglycemia (<4 mmol/L), leukocytosis (>11×10⁶/L), elevated uric acid (>340 μmol/L), elevated ammonia (>47 μmol/L), ascites or bright liver on ultrasound, and renal impairment (creatinine >150 μmol/L). 2
Hypoglycemia is pathognomonic and should be actively monitored and corrected. 2, 3
Look for the classic triad: jaundice, coagulopathy, and low platelets, often accompanied by hypoglycemia. 1
Severity Assessment and ICU Admission Criteria
Admit to intensive care immediately if any of the following are present: 2
- Encephalopathy (a hallmark finding in AFLP) 3
- Elevated serum lactate
- MELD score >30
- Swansea criteria score >7
Step-by-Step Management Algorithm
Step 1: Maternal Stabilization (Pre-Delivery)
Correct metabolic derangements before delivery: 2
- Hypoglycemia: Continuous IV dextrose infusion with frequent glucose monitoring (every 1-2 hours)
- Coagulopathy: Administer fresh frozen plasma to correct PT/INR and fibrinogen levels 4
- Hypertension (if present in ~50% of cases): Use labetalol, nifedipine, or methyldopa 5
- Seizure prophylaxis: Administer magnesium sulfate if severe hypertension or preeclampsia features are present 5
Monitor for complications: 6
- PT/INR (identified as a risk factor for fatal complications; OR=1.558)
- Fibrinogen levels (typically <245 mg/dL)
- Renal function (acute renal failure occurs in 39-40% of cases) 6, 4
- DIC markers (occurs in >75% of AFLP cases) 3
Step 2: Expeditious Delivery
Deliver promptly once coagulopathy and metabolic derangements are treated. 1, 2, 5
Mode of delivery decisions should be made jointly by obstetricians, hepatologists, and the multidisciplinary team. 2
Anesthetic considerations: The choice between general and regional anesthesia must balance the risk of worsening hepatic encephalopathy with general anesthesia against the risks of regional anesthesia in the presence of coagulopathy. 7
Do not delay delivery waiting for definitive diagnosis—focus on supportive care and preparing for delivery. 3
Step 3: Post-Delivery Management
Recovery is typically rapid after delivery, with supportive care being the primary treatment. 1
Consider advanced therapies in severe cases: 2
- Plasma exchange post-delivery may improve maternal disease severity and decrease recovery time in severe cases
- N-acetylcysteine may be considered in women requiring ICU admission, although evidence is limited
Continue intensive monitoring for: 2, 5
- Persistent hypoglycemia
- Worsening coagulopathy
- Hepatic encephalopathy progression
- Renal function deterioration
Step 4: Liver Transplantation Consideration
In women with severe hepatic impairment, make early referral to a transplant center. 2, 3
- Postpartum transplantation has occasionally been necessary when recovery does not occur after delivery. 1
Critical Pitfalls to Avoid
Do not perform liver biopsy unless it will change management, as AFLP requires expeditious delivery regardless of histologic confirmation. 3 The Oil-red O staining technique best demonstrates hepatic steatosis if biopsy is performed. 1
Do not miss herpes simplex virus as a cause of acute liver failure in third trimester pregnancy—treat with acyclovir if suspected. 1
Screen newborns for LCHAD deficiency (long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency), as there is a strong association between AFLP and this fetal metabolic disorder. 2, 3
Ensure normalization of liver function tests within 3 months post-delivery; if abnormal, investigate for underlying liver disease. 2, 5
Distinguishing AFLP from HELLP Syndrome
While both require expeditious delivery, key distinguishing features include: 3
- AFLP: Encephalopathy, polydipsia/polyuria, hypoglycemia, DIC (>75%), fatty infiltration on imaging, preeclampsia in only ~50%
- HELLP: Preeclampsia in 95%, neurological symptoms (headache, visual changes), platelets <100×10⁹/L, ALT typically <500 U/L, no ascites