SPONTANEOUS EPIDURAL BLEED: A DIRE COMPLICATION OF SICKLE CELL CRISIS

Abstract

SESSION TITLE: Case Report Semifinalists 1 SESSION TYPE: Fellow Case Report Posters PRESENTED ON: 10/21/2019 02:30 PM - 03:15 PM INTRODUCTION: Sickle cell disease (SCD) is caused by a single amino acid substitution on the beta globin chain resulting in the propensity of the hemoglobin molecule to polymerize in deoxygenated state. Neurologic manifestation secondary to SCD is common and affects almost 35% of patients. About 54% of these patients suffer from ischemic strokes and 34% from intracranial bleeding. Isolated non-traumatic spontaneous epidural hematoma (EDH) is an exceedingly rare complication of SCD. Other reported etiologies of spontaneous SDH are infectious, coagulation abnormalities associated with end stage renal disease and hemodialysis, dural metastasis and Langerhans cell histiocytosis. CASE PRESENTATION: A 20-year-old African American female with SCD presented with severe generalized pain. She was hypertensive, tachycardic, tachypneic with an oxygen saturation 98% on room air. Physical examination revealed poor bilateral air entry on lung auscultation due to splinting and a normal neurological examination. Blood work showed leukocytosis of 12,800 with 53% neutrophil and 1% band, hemoglobin 7.5 gm/dL, platelet 181,000/dL, reticulocyte count 13%, LDH 1144 IU/L. Liver function tests were normal except a total bilirubin level of 16.2 mg/dL. The patient was started on IV hydration, analgesia was achieved by IV narcotics. About 24 hours after hospital admission, the patient suddenly became unresponsive. An emergent CT scan of the head revealed a large right sided fronto-parietal epidural hematoma with midline shift, subfalcine and uncal herniation. No noticeable bone infarction overlying the hematoma. The patient underwent emergent hematoma evacuation but died 24 hours post-surgery. DISCUSSION: Spontaneous non-traumatic EDH is a rare manifestation of SCD. Only 30 cases have been reported in the literature. The pathophysiology is not completely understood. Three explanations have been proposed over the years. 1) Vaso-occlusion of the haematopoietically active calvarial diploic bone resulting in bone infarction and subsequent leaking of blood in the periosteal and subsequently epidural or in the subgaleal space 2) Acute rapid expansion of hematopoiesis with resultant microfracture of already thinned inner cortex and extravasation of blood and hematopoietic tissue 3) Sludging of sickle cells in the diploic veins hampering venous drainage and oozing of blood due to vascular injury and elevated back pressure.Presence of coagulopathy or platelet dysfunction is also likely responsible for hematoma expansion and worse clinical outcome. Overlying bony infarction is associated with better prognosis. CONCLUSIONS: The understanding regarding this rare disease entity is rapidly evolving in the era of advanced technology and improved awareness among health care providers. We believe this report will shed more light on the clinical as well as the pathophysiologic aspect of the disease process. Reference #1: Hettige S, Sofela A, Bassi S, Chandler C. A review of spontaneous intracranial extradural hematoma in sickle-cell disease. Acta Neurochirurgica. 2015;157(11):2025-9. Reference #2: Ohene-Frempong K, Weiner SJ, Sleeper LA, Miller ST, Embury S, Moohr JW, et al. Cerebrovascular accidents in sickle cell disease: rates and risk factors. Blood. 1998;91(1):288-94. Reference #3: Banerjee C, Yowtak J, Fridlyand D, Alleyne C, Jr. Acute spontaneous intracranial epidural haematoma and disseminated intravascular coagulation in a paediatric sickle cell patient. BMJ case reports. 2018;2018. DISCLOSURES: No relevant relationships by Hau Chieng, source=Web Response No relevant relationships by Boris Medarov, source=Web Response No relevant relationships by Biplab Kumar Saha, source=Web Response No relevant relationships by Aditi Saha, source=Web Response