What is the management and treatment of acute fatty liver of pregnancy?

Management and Treatment of Acute Fatty Liver of Pregnancy

Expedited delivery after correcting coagulopathy and metabolic derangements is the definitive treatment for acute fatty liver of pregnancy, with intensive supportive care before and after delivery. 1, 2

Diagnosis and Initial Assessment

Use the Swansea criteria for diagnosis, with ≥6 criteria highly correlating with clinical AFLP 1. Key diagnostic features include:

• Hypoglycemia is characteristic and should be actively monitored 1, 2
• Coagulopathy with prolonged PT/INR (PT is a risk factor for fatal complications) 3
• Elevated liver enzymes (ALT ~262 U/L, AST ~261 U/L, LDH ~1012 U/L) 3
• Elevated direct bilirubin (~86 μmol/L) 3
• Presentation typically occurs in late third trimester (mean 34.6 weeks) with nausea, vomiting, abdominal pain, malaise, and jaundice 4, 3

Management Algorithm

Step 1: Determine Need for ICU Admission

Admit to intensive care if any of the following are present: 1, 2

• Encephalopathy develops
• Elevated serum lactate
• MELD score >30
• Swansea criteria >7

Step 2: Maternal Stabilization (Pre-Delivery)

Correct coagulopathy and metabolic derangements before proceeding to delivery: 1, 2

• Administer fresh frozen plasma for coagulopathy (large dose infusions improve prognosis) 4
• Aggressively treat hypoglycemia with glucose infusions 1, 2
• Correct electrolyte abnormalities
• Monitor for and manage renal failure (occurs in 39-40% of cases) 4, 3

Common maternal complications requiring management: 4, 3

• Hypoproteinemia (75%)
• Coagulopathy (50-54%)
• Acute renal failure (39-40%)
• Encephalopathy (30%)

Step 3: Expedited Delivery

Once coagulopathy and metabolic derangements are treated, proceed immediately to delivery 1, 2. This is critical because:

• Early recognition and prompt delivery are essential for good outcomes 1, 2
• Recovery is typically rapid after delivery with supportive care 1, 5
• Mortality has decreased from 80% to 10% with prompt delivery and supportive care 6

Mode of delivery decisions should be made jointly by obstetricians, hepatologists, and the multidisciplinary team 1, 2. The choice between cesarean section and vaginal delivery depends on obstetric indications and maternal stability 4.

Step 4: Post-Delivery Management

Supportive care is the primary treatment after delivery: 5

• Plasma exchange post-delivery may be considered in severe cases to improve maternal disease severity and decrease recovery time 1
• N-acetylcysteine may be considered for women requiring ICU admission, though evidence is limited 1
• Continue monitoring hypoglycemia and coagulopathy (coagulation defect resolves over 1-2 days) 6
• Hepatic and renal function typically restore soon after delivery 6

Step 5: Liver Transplantation Consideration

For severe hepatic impairment, make early referral to a transplant center 1, 2. Postpartum transplantation has occasionally been necessary in severe cases 1, 2.

Critical Monitoring and Follow-up

Newborn screening: Screen all newborns for long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency due to strong association with AFLP 1, 5

Maternal follow-up: 5

• Monitor liver function tests until complete normalization
• Liver function tests should normalize within 3 months post-delivery
• If abnormalities persist beyond 3 months, investigate for underlying liver disease

Common Pitfalls to Avoid

• Do not delay delivery once coagulopathy is corrected – this is the definitive treatment 1, 2
• Do not miss hypoglycemia – it is characteristic of AFLP and requires aggressive monitoring 1, 2
• Do not perform regional anesthesia without correcting coagulopathy – weigh risks of general anesthesia on encephalopathy versus bleeding risk with neuraxial techniques 7
• Do not forget to screen the newborn for LCHAD deficiency 1, 5
• Do not assume all liver abnormalities will resolve – ensure active monitoring until normalization 5

Anesthetic Considerations

When delivery is required, the anesthesiologist faces a dilemma between general anesthesia (which may worsen hepatic encephalopathy) versus regional anesthesia (risky with coagulopathy) 7. Successful outcomes have been reported with different techniques after correcting coagulopathy 7.