A 24-year-old primigravida with a twin pregnancy at 30 weeks gestation comes to the emergency department due to severe epigastric and right upper quadrant pain. The patient first developed the pain yesterday morning after eating breakfast. She took acetaminophen and an antacid but had no relief. She now has severe stabbing pain, has vomited 7 times today, and continues to have nausea. The patient has no chronic medical conditions or previous surgeries. Temperature is 37.2 C (99 F), blood pressure is 136/86 mm Hg, and pulse is 108/min. BMI is 23 kg/m². Fetal heart rate monitoring shows 2 fetal heart rates with baselines of 160/min and moderate variability. The patient appears pale and has scleral icterus. The abdomen has tenderness to palpation over the right upper quadrant and epigastric region, but no rebound. The uterus is nontender and has no palpable contractions. Which of the following is the best next step in management of this patient?

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11ec25c8_da0d_d8ac_bc81_313c68ae49b6_MD0003_00 This patient has acute fatty liver of pregnancy (AFLP), a rare but life-threatening condition that develops in the third trimester, particularly in patients with multiple gestation. AFLP is an intrinsic hepatic disease likely due to defective maternal-fetal fatty acid metabolism. It causes hepatic inflammation (eg, epigastric/right upper quadrant pain, leukocytosis, elevated aminotransferases) and subsequent fulminant liver failure (eg, scleral icterus, hyperbilirubinemia, profound hypoglycemia). As AFLP progresses, patients can develop multiorgan system failure, including disseminated intravascular coagulopathy (eg, hemolytic anemia, thrombocytopenia) and acute kidney injury (from hepatorenal syndrome). As the mother decompensates, the placenta hypoperfuses, which can result in fetal hypoxemia, acidosis, and subsequent death. Due to high maternal and fetal mortality rates, management of AFLP is with maternal stabilization and immediate delivery, regardless of gestational age. (Choice A) A 24-hour urine protein test evaluates for preeclampsia with severe features, which may also present in the third trimester with right upper quadrant pain and signs of end-organ damage (eg, elevated creatinine). This patient does not have hypertension (ie, systolic ≥140 mm Hg or diastolic ≥90 mm Hg), making this diagnosis less likely. (Choice B) Emergency cholecystectomy is performed for complicated acute cholecystitis (ie, gallbladder perforation or gangrene), which may present with postprandial right upper quadrant pain and an elevated alkaline phosphatase level. Acute cholecystitis does not cause hyperbilirubinemia or profound hypoglycemia. In addition, the source of this patient's elevated alkaline phosphatase level is likely the placenta. (Choices D and E) MRI and ultrasound are preferred imaging modalities in pregnancy due to low risk of fetal radiation exposure; they are performed in patients with an uncertain diagnosis. Neither is required to diagnose AFLP, and delaying delivery increases fetal and maternal mortality. Liver biopsy is not needed for the diagnosis of AFLP and increases this patient's bleeding risk due to thrombocytopenia and possible coagulopathy from liver dysfunction. Educational objective: Acute fatty liver of pregnancy presents in the third trimester with hepatic inflammation (eg, epigastric/right upper quadrant pain, leukocytosis, elevated aminotransferases) and fulminant liver failure (eg, profound hypoglycemia). Due to high maternal and fetal mortality rates, management is immediate delivery. References: Acute fatty liver disease of pregnancy: updates in pathogenesis, diagnosis, and management. (http://www.ncbi.nlm.nih.gov/pubmed/28291236) Pregnancy and liver disease. (http://www.ncbi.nlm.nih.gov/pubmed/26658682)

A 24-Year-Old Primigravida with a Twin Pregnancy at 30 Weeks