Acute fatty liver of pregnancy (AFLP) is a dangerous condition of late pregnancy. Once diagnosed, or even suspected with a high degree of likelihood, it must be treated in a timely manner to ensure a good outcome. About three in every four cases will be delivered by Cesarean section within 48 hours of the diagnosis being made, but a few may have their pregnancy prolonged by up to 2 weeks.
Different stages of human liver illustration. Image Credit: BlueRingMedia / Shutterstock
The main facets of AFLP management include early diagnosis, prompt delivery, and comprehensive medical support by a multidisciplinary medical team in an intensive care setting. A high level of clinical suspicion is necessary to avoid delay in diagnosis.
The definitive treatment of AFLP is delivery. If possible, vaginal delivery is induced, but if the maternal or fetal condition do not permit this, operative delivery must be achieved as soon as safely possible. The mother’s condition should be stable with the airway, blood pressure, blood glucose, electrolyte, and clotting factors assessed and kept in normal condition. Mental status assessment is also essential, along with fetal evaluation.
After delivery the mother must be observed carefully for deterioration in the hemodynamic status because of the risk of clotting failure. Hypoglycemia, pancreatitis, pancreatic pseudocyst formation and subsequent infection, and retroperitoneal bleeding from an inflamed pancreas, are all possibilities which should be screened for carefully. Intravenous fluids, glucose infusions, and blood or blood component transfusions are all to be given as required. CT or MRI imaging may be useful in such assessments.
Supportive treatment also includes the management of hypertension and metabolic disturbances. Hepatic function should be carefully and repeatedly assessed at regular intervals.
Antibiotics may be necessary in almost all cases to prevent infection, while transfusions of blood or blood components are given in almost two-thirds of patients. More than 55% of patients will be admitted to the intensive care unit in view of impending multisystem derangements or the risk of a fatal outcome.
Liver transplants have been a last and rare resort in AFLP when women show irreparable liver failure even after delivery and despite intensive medical support, or hepatic encephalopathy, severe metabolic acidosis or worsening coagulopathy. Liver rupture with hepatocellular necrosis is another rare indication for a transplant. Rapid improvement has been noted in such patients.
Various complications must be looked for and managed.
Maternal Complications and Outcomes
The chief predictive factors for risk of serious complications, which occur in about 40% of women with AFLP, include:
- total and direct bilirubin levels
- prothrombin time (PT)
- internationalized normalized ratio (INR)
- fibrin degradation products (FDP)
Maternal death is usually due to multiorgan dysfunction, DIC, sepsis, aspiration or pancreatitis. The maternal mortality used to be in the range of 75% but with early delivery, it has come down to about 7%.
Fulminant hepatic failure is a high-risk factor, and leads to various complications. Ultrasound or CT scans of the liver are performed to assess the presence of hepatic failure and the risk of development of hepatorenal syndrome, but are not sensitive or specific enough. Liver biopsy is the definitive procedure but cannot be performed in patients at risk of coagulopathy, and is highly invasive.
The chief maternal complications include:
- Hypoproteinemia in 75%
- Acute renal failure in almost 40%
- Coagulopathy in almost 55%
- Ascites in more than 33%
- Disseminated intravascular coagulation (DIC) in 32%
- Preeclampsia in 20%
- Others include pancreatitis, encephalopathy, gastrointestinal bleeding due to ulceration of the stomach, and ascites
Fetal Complications and Outcomes
Risk factors for perinatal death in women born to women with AFLP include:
- Gestational term at onset of AFLP
- Gestational term when the fetus was born
- Total and direct bilirubin
- Blood glucose levels
Most of these babies have a gestational age of 35-40 weeks. About 70% of these babies will be preterm due to prompt delivery on diagnosis. The perinatal mortality is about 15%, down from about 90% in the past.
The most common neonatal complications are intrauterine fetal distress in 26%, and almost all babies having a low Apgar score at 1 minute.