JOURNAL OF THE LOUISIANA STATE MEDICAL SOCIETY
IV calcium, sodium bicarbonate, and insulin with 50% glucose solution for hyperkalemia. Blood, urine, and vaginal cultures were taken and she was started on vancomycin and piperacillin- tazobactam for possible sepsis. She was also transferred to the intensive care unit. In the intensive care unit, she continued to have symptoms and lab results consistent with acute shock as well as hypoxemic respiratory failure necessitating intubation and sedation. A chest x-ray showed lung consolidations consistent with acute chest syndrome. She was also having significant vaginal bleeding with prolonged coagulation studies (prothrombin time: 29.0 s, international normalized ratio: 2.80). An abdominal ultrasound showed free blood in the abdomen, and the decision was made to perform an exploratory laparotomy. Intraoperatively, the uterus appeared boggy and bleeding was noted from the uterine incision and vagina. Hysterectomy was performed using standard surgical techniques. After the hysterectomy was completed, she continued to have multiple areas of bleeding in the pelvis consistent with a coagulopathy. During abdominal exploration, the liver was noted to be firm, dense, and nodular. Multiple applications of gelatin-thrombin matrix sealant and oxidized cellulose polymer were placed over all visible sites of bleeding. Multiple laparotomy pads were then tied end-to- end and placed tightly in the pelvis as a pack. The end of the pack was then brought through the vaginal opening created by the hysterectomy such that the pack could be removed via the perineum without performing an additional laparotomy. A Jackson-Pratt drain was placed over the pack and brought out through a separate stab incision in the left lower quadrant. It was noted that the pelvic bleeding was generally controlled by the tamponade created by the pack. During the surgery, several units of packed red blood cells and fresh frozen plasma were administered, and blood clots were seen to be forming at bleeding sites not covered by the pack. On post-operative day two from the cesarean delivery, she required packed red blood cell transfusions to maintain hemoglobin levels above 8.0. She had minimal urine output, as well as severe elevation of liver function tests (aspartate aminotransferase > 10,000 U/L). Her coagulation studies were again considerably elevated (prothrombin time: 26.1, international normalized ratio: 2.70), and her cardiac function deteriorated. An echocardiogram showed biventricular failure. She was sent to the Cardiac catheterization lab for insertion of a ventricular function assistance device and was placed on 4 anti- hypotensive agents. On postoperative day three, 100 cc/hr of bloody serous fluid was noted coming from her intraabdominal drain. For this reason, as well as the unstable blood pressure, the pelvic pack was left in place. Her laboratory studies and clinical appearance indicated multiple organ system failure. She continued to receive maximum doses of anti-hypotensive agents and left ventricular assistance without significant improvement. Due to increasing edema and kidney failure, she began continuous bedside renal dialysis to remove 200 cc/hr of fluid.
Over the next few days, she showed a continued decline in liver function tests (aspartate aminotransferase: > 10,000 U/L, alanine aminotransferase: 1528 U/L) and a right upper quadrant abdominal ultrasoundwas consistent with cirrhotic liver without portal vein thrombosis. On postoperative day seven, she developed cardiac asystole and was pronounced dead in the intensive care unit. Her final diagnoses/cause of death was listed as hemorrhagic, cardiogenic, and septic shock in the setting of florid hepatic and renal failure, as well as acute respiratory distress syndrome.
DISCUSSION
Pregnant patients with sickle cell disease require multiple unique considerations. Sickle cell-related vaso-occlusive crises and anemia occur with higher frequency in pregnancy and may increase with gestational age. These patients may also experience higher rates of systemic inflammatory response syndrome, sepsis, and mortality during pregnancy. 1 Patients considered high-risk due to sickle cell disease, may benefit from prophylactic transfusions throughout pregnancy. 2 This case demonstrates that a history of uncomplicated pregnancies may not predict future outcomes, particularly when cesarean section is the method of delivery. There is a higher incidence of cardiomyopathy in patients with sickle cell disease including diastolic dysfunction, left atrial enlargement, and pulmonary hypertension. 3 This combination places sickle cell patients at elevated risk for surgery and should prompt preoperative assessment of cardiac function. Hepatic dysfunction in sickle cell disease can come from multiple causes including increased rates of hepatitis C virus, iron overload from transfusion, acute ischemia, and cholestasis. Hepatic damage may result from sinusoidal obstruction and ischemia of hepatic veins, eventually leading to perisinusoidal fibrosis. 4,5,6 Liver function tests may have less diagnostic value in sickle cell disease since patients have elevated total bilirubin and aspartate aminotransferase with hemolysis. This patient had increased total bilirubin, aspartate aminotransferase, and scleral icterus at presentation. Since these findings can point to chronic hemolysis, liver disease, or both; preoperative hepatic evaluation (such as a right upper quadrant ultrasound) may have facilitated the diagnosis of chronic liver failure, which would then have prompted further assessments and preparations for parturition. Patients with underlying liver disease have increased risk of both clotting and bleeding from derangements in clotting factor production, thrombocytopenia, increased fibrinolysis, and platelet dysfunction. One study showed 40% of patients admitted for decompensated cirrhosis had bleeding (half of which was not variceal) and 7% had deep venous thromboembolic disease. In such patients, prothrombin time, international normalized ratio, and partial thromboplastin time may not accurately reflect the risk of altered hemostasis. 7 Patients with such fragile hepatic derangements are likely at
J La State Med Soc VOL 169 SEPTEMBER/OCTOBER 2017 129
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