Critical Care Physician Advocate Christ Medical Center, Illinois
Disclosure information not submitted.
Introduction: Acute liver failure (ALF) in pregnancy may result from infectious, autoimmune, or metabolic disease. Acute fatty liver of pregnancy (AFLP) is a rare, life-threatening cause of ALF. It can overlap with hemolysis, elevated liver enzymes, and low platelets (HELLP) syndrome, making diagnosis challenging.
Description: A 22-year-old female at 23 weeks’ gestation with ulcerative colitis presented with abdominal cramping, diarrhea, and lower extremity edema. She was initially tachycardic, normotensive, and alert. Labs showed transaminitis and impaired liver function. She also had hyperammonemia, acidosis, thrombocytopenia, and refractory hypoglycemia, with normal fibrinogen. She progressed to multiorgan injury including acute kidney injury (AKI). Workup for viral hepatitis, HIV, systemic lupus erythematosus, and Budd Chiari syndrome was negative. Given clinical concern for AFLP, OB/GYN, gastroenterology, and MICU teams proceeded with emergent cesarean delivery. Postpartum, she developed small bowel obstruction, clostridium difficile colitis, Escherichia coli bacteremia, Pyoderma gangrenosum, and poor wound healing requiring wound VAC. After weeks of supportive care, liver function recovered and she was discharged in stable condition.
Discussion: AFLP presents in the third trimester, although earlier cases have been noted. Patients may report nausea, vomiting, and abdominal pain. Labs can show transaminitis and hyperbilirubinemia, acidosis, elevated INR, low fibrinogen, and hyperammonemia. This patient had many of these features but a normal fibrinogen. HELLP syndrome presents similarly. Studies have seen higher bilirubin and AKI in AFLP, whereas HELLP patients tend to have hypertension. Other causes of ALF such as viral hepatitis, congestive hepatopathy, drug toxicity should also be considered. AFLP management is supportive, focusing on correcting hypoglycemia, coagulopathy, and acidosis. Key complications include encephalopathy and disseminated intravascular coagulation. Though patients often improve after delivery, some may develop refractory liver failure marked by profound acidosis, coagulopathy, hypotension, and toxin buildup leading to cerebral edema, encephalopathy, and renal failure. Consultation with transplant teams in anticipation of this is vital to help save patient lives.
