What is the management and treatment of Acute Fatty Liver of Pregnancy (AFLP)?

Management and Treatment 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 Acute Fatty Liver of Pregnancy to reduce maternal mortality and morbidity. 1

Diagnosis and Initial Assessment

• AFLP typically presents in the third trimester with:

  • Nausea, vomiting, abdominal pain (right upper quadrant/epigastric)
  • Jaundice, malaise, headache
  • Hypoglycemia (characteristic finding)
  • Features of pre-eclampsia (hypertension, proteinuria) in some cases 1

• Laboratory findings include:

  • Elevated transaminases (AST/ALT)
  • Coagulopathy (prolonged PT, decreased fibrinogen)
  • Hypoglycemia
  • Elevated bilirubin
  • Thrombocytopenia
  • Elevated lactate dehydrogenase

• Swansea criteria can be used for diagnosis: presence of ≥6 findings in absence of another cause strongly correlates with AFLP 1

Management Algorithm

Step 1: Maternal Stabilization

• Admit to high-level care setting
  • Women with encephalopathy, elevated serum lactate (>2.8 mg/dl), MELD score >30, or Swansea criteria >7 require level 2/3 care (intensive care) 1
• Correct hypoglycemia (common and dangerous complication)
• Manage coagulopathy with fresh frozen plasma
• Correct electrolyte abnormalities
• Monitor for and treat renal failure

Step 2: Expedite Delivery

• Delivery is the definitive treatment and should be expedited once maternal coagulopathy and metabolic derangements are corrected 1
• Mode of delivery should be determined jointly by obstetricians, hepatologists, and the multidisciplinary team 1
• Cesarean section may be necessary in cases of maternal deterioration or fetal distress

Step 3: Post-Delivery Management

• Continue intensive supportive care

• Monitor for complications:

  • Hepatic encephalopathy
  • Renal failure (occurs in ~40% of cases) 2
  • Coagulopathy (occurs in ~50-54% of cases) 3, 2
  • Hypoproteinemia (occurs in ~75% of cases) 3

• Consider plasma exchange for severe cases:

  • May improve maternal disease severity and decrease recovery time in women with severe hepatic impairment
  • Should be performed at clinical centers with expertise in plasma exchange in high-dependency or intensive care settings 1

• Consider N-acetylcysteine:

  • May be beneficial based on experience with other causes of non-paracetamol-induced liver failure
  • Can be considered for women requiring intensive care admission 1

Step 4: Transplant Evaluation

• Early referral to a transplant center should be made for women with:
  • Severe hepatic impairment
  • Signs of liver failure that may require transplantation 1

Special Considerations

• Newborns of mothers with AFLP should be screened for long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency, as there is a strong association between this fetal metabolic disorder and AFLP 1

• Genetic counseling is indicated for affected mothers and their newborns 1

• Prothrombin time (PT) and INR are significant risk factors for fatal complications in patients with AFLP 3

Prognosis

• With early recognition and prompt management, maternal outcomes have significantly improved
• Recovery is typically rapid after delivery with supportive care 1
• Perinatal mortality remains high (reported up to 66% in some series) 2

Pitfalls to Avoid

1. Delayed diagnosis: Maintain high index of suspicion for AFLP in pregnant women with nausea, vomiting, abdominal pain, and abnormal liver function in the third trimester

2. Confusing AFLP with HELLP syndrome: Both can present with similar features, but AFLP typically has more pronounced hypoglycemia, jaundice, and encephalopathy

3. Delaying delivery: Once AFLP is diagnosed, delivery should not be delayed after maternal stabilization

4. Inadequate post-delivery monitoring: Despite delivery, patients can still deteriorate and require continued intensive care

5. Failure to refer to transplant center: Early referral is essential when signs of liver failure are present

The management of AFLP requires a coordinated multidisciplinary approach involving obstetricians, hepatologists, intensivists, and neonatologists to optimize maternal and fetal outcomes.