Guideline for Management of Acute Fatty Liver of Pregnancy

Immediate Assessment and Risk Stratification

ICU admission (level 2–3) is recommended for women with acute fatty liver of pregnancy who develop encephalopathy, serum lactate > 2.8 mg/dL, or a MELD score > 30, indicating high‑risk disease. (strong recommendation) [1][2][3][4]5
The Swansea criteria can aid diagnosis, but clinicians should be aware of its limited ability to distinguish AFLP from other pregnancy‑related liver disorders. (moderate evidence) 6

Typical presentation occurs in the third trimester or postpartum and includes abdominal pain and vomiting. (observational data) [6][7]
Coagulopathy (prolonged prothrombin time) and disseminated intravascular coagulation are present in > 75 % of cases, underscoring the need for rapid correction before delivery. (high prevalence) 6

Maternal stabilization—correction of coagulopathy, hypoglycemia, and metabolic/electrolyte disturbances—must be achieved before proceeding to delivery. (standard of care) [1][2][3][4]5

Expedited delivery is the definitive therapy once stabilization is achieved; timing should be individualized but generally occurs on the day of diagnosis or within a few days. (strong recommendation) [1][2][3][4]5
The decision between vaginal and cesarean delivery should be made jointly by obstetricians, hepatologists, and other members of the multidisciplinary team. (strong recommendation) [1][2]3
Choice of delivery mode depends on gestational age, fetal condition, severity of maternal disease, and cervical status. (moderate evidence) 7
Because thrombocytopenia and coagulopathy often preclude safe regional anesthesia, general anesthesia is usually required for cesarean sections. (clinical consensus) 7

Therapeutic plasma exchange may be considered after delivery in women with severe hepatic impairment to accelerate recovery; recommendation is weak due to limited data from small case series. (weak recommendation) 1
N‑acetylcysteine can be considered for patients requiring ICU care; although no AFLP‑specific trials exist, benefits in other non‑acetaminophen liver failures provide a rationale. (conditional recommendation) [1][3]

Early referral to a liver transplant center is mandatory for patients showing signs of irreversible hepatic failure despite delivery. (strong recommendation) [1][2][3][4]5

Patients should remain under close observation in an ICU or high‑dependency unit during the recovery phase to monitor hepatic, renal, and coagulation parameters. (standard practice) 3
Ascites develops in approximately 40 % of cases and should be anticipated and managed accordingly. (observational data) 3

Maternal mortality has decreased dramatically with modern management, now ranging from 7 % to 18 %. (epidemiologic data) 6
Fetal/neonatal mortality ranges from 9 % to 23 %, with outcomes influenced by gestational age at diagnosis and prematurity‑related complications. (epidemiologic data) [6][4]

A coordinated approach involving obstetrics, hepatology, anesthesia, and intensive care teams is essential for optimal maternal and fetal outcomes. (expert consensus) [1][2]3

The Swansea criteria can aid the diagnosis of AFLP but have limited ability to distinguish it from HELLP syndrome or preeclampsia, necessitating reliance on clinical judgment and additional testing. 8

Immediate referral to a liver‑transplant center is advised for patients with AFLP who show signs of irreversible hepatic failure despite delivery, particularly when encephalopathy worsens or multi‑organ failure fails to resolve within the expected post‑delivery timeframe. 8

Delivery must not be postponed while attempting to differentiate AFLP from HELLP syndrome; both conditions require prompt delivery, and liver biopsy should be reserved only for situations where the result would change the therapeutic approach. 8