What is the recommended management for a third‑trimester pregnant woman diagnosed with acute fatty liver of pregnancy?

Management of Acute Fatty Liver of Pregnancy

For a third-trimester pregnant woman diagnosed with acute fatty liver of pregnancy, expeditious delivery after maternal stabilization is the definitive treatment, with consultation from obstetrical services being mandatory. 1

Immediate Recognition and Stabilization

Critical Assessment Upon Diagnosis

• Admit to intensive care if any of the following are present: encephalopathy, elevated serum lactate, MELD score >30, or Swansea criteria score >7 2
• Correct coagulopathy and metabolic derangements before proceeding to delivery 2
• Monitor continuously for hypoglycemia, which is a characteristic and common finding in AFLP 2
• Establish strict fluid balance monitoring with urinary catheter placement 3

Pre-Delivery Stabilization Steps

• Administer fresh frozen plasma to correct coagulopathy as needed 3
• Maintain euglycemia with intravenous dextrose infusions 2
• Consider central venous access for invasive monitoring in severe cases 3
• Perform abdominal imaging (ultrasound or CT) to rule out intrahepatic hemorrhage or hepatic rupture, which constitute rare emergent situations requiring rapid resuscitation 1

Definitive Management: Expeditious Delivery

Timing and Decision-Making

• Early recognition and prompt delivery are critical in achieving good outcomes 1
• Delivery should be expedited once coagulopathy and metabolic derangements have been treated 2
• Decisions about mode of delivery should be made jointly by obstetricians, hepatologists, and the multidisciplinary team 2

Common pitfall to avoid: Do not delay delivery waiting for laboratory values to normalize, as AFLP only resolves after delivery 3

Mode of Delivery Considerations

• Cesarean section rates are typically high due to maternal instability and fetal compromise 3
• General anesthesia may be required if platelets are <100,000/mm³, precluding safe regional anesthesia 3
• Regional anesthesia (epidural/spinal) should not be used with platelets <100,000/mm³ due to risk of epidural hematoma 3

Post-Delivery Management

Supportive Care and Monitoring

• Recovery is typically rapid after delivery, with supportive care being the primary treatment required 1
• Continue intensive monitoring for at least 24-48 hours postpartum, as some cases may worsen in the immediate postpartum period 3
• Monitor liver function tests, coagulation parameters, and glucose levels closely 2

Advanced Interventions for Severe Cases

• Plasma exchange post-delivery may be considered to improve maternal disease severity and decrease recovery time in severe cases 2
• N-acetylcysteine may be considered in women requiring intensive care admission, although evidence is limited 2
• In the subset of women with severe hepatic impairment, early referral to a transplant center should be made, as postpartum transplantation has occasionally been necessary 1, 2

Special Considerations and Follow-Up

Neonatal Screening

• Newborns of mothers with AFLP should be screened for long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency, as there is a strong association between AFLP and this fetal metabolic disorder 2, 4

Maternal Follow-Up

• Ensure normalization of liver function tests within 3 months post-delivery 2
• If liver function tests remain abnormal beyond 3 months, investigate for underlying liver disease 2

Differential Diagnosis Considerations

• Pregnancy (especially in the third trimester) increases the risk of acute liver failure due to herpes virus, which should be treated with acyclovir 1
• Keep in mind that acute liver failure in pregnant women may be caused by entities not necessarily related to the pregnant state 1
• AFLP can be distinguished from HELLP syndrome by the presence of prolonged PT, low fibrinogen, hypoglycemia, and encephalopathy 5

Key Clinical Pearls

• The triad of jaundice, coagulopathy, and low platelets may occasionally be associated with hypoglycemia in AFLP 1
• Features of pre-eclampsia such as hypertension and proteinuria are common in AFLP 1
• Steatosis documented by imaging studies supports the diagnosis 1
• Intrahepatic hemorrhage and/or hepatic rupture constitute rare emergent situations requiring rapid resuscitation and intervention 1