JOURNAL OF THE LOUISIANA STATE MEDICAL SOCIETY
Complications of Poorly Controlled Sickle Cell Disease in a Patient Undergoing Cesarean Section
William Robinson, MD, Syem Barakzai
Background: Cesarean delivery in a patient with poorly controlled sickle cell disease can be complicated by chronic multiple organ damage.
Case: a 37-year-old woman, gravida 4 para 3, with poorly controlled sickle cell disease presented at 38 weeks gestation inactive labor. All previous pregnancies endedwithuncomplicatedvaginal deliveries. Due tobilateral hip osteonecrosis, she underwent cesarean section and post-operatively developed coagulopathy and shock. She was treatedmedically and surgically but eventually died of hemorrhagic, cardiogenic, and septic shock in the setting of florid hepatic and renal failure. Conclusion: Chronic liver injury secondary to poorly controlled sickle cell disease can result in coagulopathy and death in a patient undergoing cesarean section. Early recognition of liver disease may alter management and improve outcomes in such patients.
INTRODUCTION
dL), blood urea nitrogen (3 mg/dL), and creatinine (0.3 mg/dL). During the second presentation, her laboratory studies were significant for a urinalysis positive for nitrites, leukocyte esterase, and blood. She was treated with cefazolin for pyelonephritis. Her prothrombin time and platelets counts were within normal limits (12.1 s and 255,000 cells/mcL, respectively) during these admissions and she was serum negative for hepatitis B and C. Of note, she was documented to have scleral icterus at both presentations. She was admitted at 38 weeks estimated gestational age in latent labor. Her bilateral hip osteonecrosis made hip flexion and positioning for vaginal delivery very difficult. She was monitored and began to show non-reassuring fetal heart tones. She therefore underwent cesarean section with epidural anesthesia and bilateral tubal ligation. She delivered a 6 lb 12 oz male infant with Apgar scores of 8 and 9 without complications and an estimated blood loss of 600 mL. On post-operative day 1, she complained of light-headedness, shortness of breath, and was found to be tachycardic. Her laboratory studies showed severe anemia (5.9 g/dL), neutrophilia (82%, white blood cell count 54,600 cell/mcL), elevated liver enzymes (aspartate aminotransferase: 1010 U/L, alanine aminotransferase: 1529 U/L, alkaline phosphatase 430 U/L), hyperkalemia (7.2 mmol/L), and a blood smear with moderate sickle cells. Arterial blood gas showed metabolic acidosis (pH: 7.184) and her electrocardiogram showed peaked T waves consistent with hyperkalemia. She was transfused with 2 units of packed red blood cells and given IV fluids for shock and showed clinical improvement but remained anuric. She was also given
Patients with sickle cell anemia have an increased incidence of end organ damage and increased risks of complications from surgical procedures. A high index of suspicion and prophylactic management of sickle cell disease complications may aid in preventing catastrophic outcomes.
CASE REPORT
The patient is a 37-year-old Hispanic woman, gravida 4, parity 3003 with known sickle cell disease by hemoglobin electrophoresis. Her previous pregnancies ended in uncomplicated vaginal deliveries. In the years since her previous delivery she had suffered a significant functional decline. She had bilateral osteonecrosis of the femoral heads diagnosed by x-ray, limiting her mobility substantially, and required the use of a cane for mobility. During this pregnancy, she had sporadic prenatal care due to time spent in Honduras, and did not attend consultationswithmaternal-fetal-medicine. Shepresented twice in the third trimester for vaso-occlusive crises, with severe pain in her knees, elbows, and lower back, consistent with previous experiences. In both cases, she was treated supportively with hydromorphone (due to morphine allergy), intravenous (IV) fluids, and oxygen via nasal cannula. She was discharged in both cases after an inpatient maternal-fetal-medicine consult, but did not attend follow up appointments. Abnormal laboratory studies at the first presentation included elevated aspartate aminotransferase (104 U/L), lactate dehydrogenase (611 U/L), white blood cell count (13,700 cell/mcL), low hemoglobin (9.3 g/
128 J La State Med Soc VOL 169 SEPTEMBER/OCTOBER 2017
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