Acute Fatty Liver of Pregnancy Complicated by Quadruplets

Abstract

Poster Presentation Background The diagnosis of acute fatty liver of pregnancy (AFLP) prior to surgical incision is critical. Case A 26‐year‐old G2P1 woman pregnant with quadruplets at 32 weeks gestation presented to triage with general malaise, pain in the right thigh, and feeling unwell. The woman had normal range blood pressures and no complaint of visual changes, epigastric pain, or jaundiced appearance. She was admitted for observation to rule out deep vein thrombosis (DVT). Routine labs were ordered with a comprehensive metabolic panel that indicated high enzymes, low platelets, and low hemoglobin and hematocrit (H&H). The decision to proceed with delivery was made due to diagnosis of HELLP syndrome. Clotting studies and a disseminated intravascular coagulation (DIC) panel were ordered and drawn at this time. The woman was sent to the operating room (OR) for delivery and prepped for an epidural. The diagnosis of AFLP was made in the OR. The procedure was stopped prior to epidural placement, and the woman was moved back to the obstetric intensive care unit for correction of coagulopathy. Consultation was initiated with transfusion medicine and transfusion of 12 units apheresis platelets, two units of packed red blood cells (PRBC), 16 pooled cryoprecipitated anti‐hemophilic factor (cyro), two jumbo fresh frozen plasma (FFP) units, and six liquid plasma units was ordered. We increased fibrinogen from 60 to 347 during the course of 6 hours, and the decision was made to move the woman to the OR and proceed with the cesarean with an intraoperative transfusion of three apheresis platelets, two units of PRBCs, six pooled cyros, and two jumbo FFP units. The delivery of the quadruplets and delivery of placenta were successful with good hemostasis. The woman experienced a total estimated blood loss of 1400 ml. After birth, the quadruplets were transported to the NICU, and the women was ventilated and placed in the obstetric intensive care unit. Careful evaluation for renal failure, hepatic encephalopathy, and/or other sequelae of AFLP was ongoing. The woman was extubated 24 hours postoperatively with stable renal function and urine output. Conclusion On postoperative day 2, the woman began to experience agitation, deterioration in mental status, and decreased ability to communicate. Fibrinogen decreased from 327 to 225, international normalized ratio (INR) increased from 2.2 to 2.5, ammonia increased from 33 to 51, and creatinine was 2.60. A care team including renal specialists, critical care providers, maternal/fetal medicine and obstetric intensive care unit nurses agreed to transfer the woman to a long‐term acute care facility with an in‐house live transplant team for ongoing support and care. The diagnosis of AFLP prior to surgical incision proved critical to this woman's survival. The multidisciplinary care team worked together to provide optimal care and prevented what could have been a dire outcome.