What are the management options for Acute Fatty Liver of Pregnancy (AFLP) based on Swansea's (Swansea's) criteria?

Management of Acute Fatty Liver of Pregnancy Based on Swansea Criteria

The management of Acute Fatty Liver of Pregnancy (AFLP) requires prompt recognition using the Swansea criteria, maternal stabilization, expedited delivery, and intensive supportive care to improve maternal and fetal outcomes. 1, 2

Diagnosis Using Swansea Criteria

The Swansea criteria are used to diagnose AFLP, requiring ≥6 of the following findings in the absence of another cause:

• Vomiting 1
• Abdominal pain 1
• Polydipsia/polyuria 1
• Encephalopathy 1
• Elevated transaminases (AST or ALT >42 IU/L) 1
• Elevated bilirubin (>0.8 mg/dL) 1
• Hypoglycemia (<72 mg/dL) 1
• Leukocytosis (>11 × 10^6/L) 1
• Elevated uric acid (>5.7 mg/dL) 1
• Elevated ammonia (>42 IU/L) 1
• Ascites or bright liver on ultrasound 1
• Renal impairment (creatinine >1.7 mg/dL) 1
• Coagulopathy (PT >14 seconds or PTT >34 seconds) 1
• Microvesicular steatosis on biopsy (if performed) 1

Management Algorithm

Step 1: Maternal Stabilization and Monitoring

• Admit patients with encephalopathy, elevated serum lactate, MELD score >30, or Swansea criteria >7 to intensive care 2
• Correct coagulopathy and metabolic derangements before delivery 2, 3
• Monitor for and treat hypoglycemia, which is common in AFLP (53-75% of cases) 1, 3, 4
• Manage coagulopathy with fresh frozen plasma, cryoprecipitate, or platelets as needed 4
• Monitor for complications including renal failure, ascites, and gastrointestinal hemorrhage 4

Step 2: Expedited Delivery

• Expedite delivery once coagulopathy and metabolic derangements have been treated 1, 2
• Decisions about mode of delivery should be made jointly by obstetricians, hepatologists, and the multidisciplinary team 2, 5
• Cesarean section is often performed (57-75% of cases) due to maternal condition and urgency 3, 4
• Anesthesia selection should be individualized based on coagulation status - general anesthesia with rapid-sequence induction may be preferred for patients with severe coagulopathy 3

Step 3: Post-Delivery Management

• Provide intensive supportive care including correction of hypoglycemia, coagulopathy, and electrolyte abnormalities 1, 2
• Consider plasma exchange in severe cases to improve maternal disease severity and decrease recovery time 2
• N-acetylcysteine may be considered in women requiring intensive care admission, although evidence is limited 2
• Monitor for postpartum hemorrhage, which is a common complication 4

Step 4: Liver Transplantation Consideration

• Early referral to a transplant center should be made for women with severe hepatic impairment or acute liver failure 1, 2
• AFLP complicated by acute liver failure should prompt transfer to a transplant center for evaluation 1
• Postpartum transplantation has occasionally been necessary in severe cases 1

Special Considerations

• Screen newborns of mothers with AFLP for long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency 1, 2
• Provide genetic counseling for affected mothers and their newborns 1
• AFLP has a strong association with fetal mitochondrial fatty acid oxidation disorders 1, 6
• Differentiate AFLP from HELLP syndrome, which can present similarly but has distinct features:
  • AFLP more commonly presents with hypoglycemia, jaundice, and encephalopathy 6
  • HELLP syndrome more commonly presents with hypertension, proteinuria, and headache 6

Monitoring and Follow-up

• Monitor liver function tests until normalization, which typically occurs within 1-2 weeks post-delivery 2, 7
• If liver function tests remain abnormal after 6 weeks, investigate for underlying chronic liver disease 1, 2
• Maternal mortality has improved from 92% before 1970 to less than 10% in recent years with early recognition and prompt delivery 1, 7

Pitfalls and Caveats

• Liver biopsy is rarely indicated unless the diagnosis is in doubt and would influence management 1
• The Swansea criteria have high sensitivity but low specificity in women with severe AFLP with features of acute liver failure 1
• Do not delay delivery for extensive diagnostic workup when AFLP is strongly suspected 1, 7
• AFLP can be confused with other pregnancy-related liver disorders; focus on supportive care and preparation for delivery while the diagnosis is being established 1