Introduction: Intracranial epidural hematomas (EDH) typically occur in the setting of trauma. Acute non-traumatic intracranial epidural hematomas are very rare, occurring in the contexts of pregnancy, dialysis, Paget’s disease, coagulopathy, metastases to dura, infection, vascular malformation, and sickle cell disease. While epidemiologically the incidence of combined ischemic and hemorrhagic strokes in the pediatric population is about 1 case per 100,000 children, in SCD patients the frequency is about 200 per 100,000 children. We report a rare case of a spontaneous EDH complicated by the development of disseminated intravascular coagulopathy (DIC) in a pediatric sickle cell disease (SCD) patient.

Methods: We present the first ever reported case of spontaneous EDH complicated by DIC in the setting of pediatric SCD. We discuss possible mechanisms and report on a review of the pertinent literature.

Results: An 18-year-old African American male with SCD, in the midst of an an acute sickle cell crisis, spontaneously developed acute bifrontal EDHs that required emergent evacuation. However, he also subsequently developed DIC. Despite successful evacuation of the EDH, the patient’s EDH reaccumulated. After multiple transfusions, the patient underwent a repeat EDH evacuation along with craniectomy and placement of an intracranial pressure monitor. With medical therapy and ICP management, the patient’s neurologic status improved slightly. Due to family’s wishes, no further intervention was pursued, and the patient expired from respiratory complications. Literature describes (1) acute vaso-occlusive infarction of a craniofacial bone, and (2) acute bone marrow hyperplasia from acute anemia, as mechanisms of EDH in SCD.

Conclusions: SCD presents a unique hematological landscape by which hemorrhagic sequelae such as intracranial EDH can occur more frequently and by atypical mechanisms. In our case report, we discuss unique mechanisms in SCD that can lead to EDH, and we describe how DIC affected our overall management of the patient.

Patient Care: Understanding the two proposed mechanisms of EDH in SCD can help practitioners and researchers develop new methods of identifying skull bone infarctions or acute medullary hematopoeisis prior to the development of an associated hemorrhage. This could potentially lead to closer monitoring of affected patients and/or more rapid interventions when necessary, potentially leading to better patient outcomes.

Learning Objectives: By the conclusion of this session, participants should be able to: 1) understand the epidemiology of acute cerebral events specific to SCD, 2) describe the proposed underlying mechanisms of spontaneous EDH formation specific to SCD, 3) identify the grave complications that a coagulopathy such as DIC can cause in a SCD patient.

References: 1. Dahdaleh N, Lindley TE, Kirby PA, Oya H, Howard MA (2009) A "neurosurgical crisis" of sickle cell disease. J Neurosurg Pediatrics. 4:532-535. 2. Wood D (1978) Cerebrovascular complications of sickle cell anemia. Stroke. 9:73-75. 3. Sarnaik S, Lusher JM (1982) Neurological complications of sickle cell anemia. Am J Pediatr Hematol Oncol. 4:386-94. 4. Bullock M, Chesnut R, Ghajar J, Gordon D, Harti R, Newell DW, Sevadei F, Walters BC, Wilberger JE (2006) Surgical management of acute epidural hematomas. Neurosurgery. 58: 5. Besenski N (2002) Traumatic injuries: imaging of head injuries. Eur Radiol. 12:1237. 6. Babatola B, Salman YA, Abiola AM, Okezie KO, Oladele AS (2012) Spontaneous Epidural Haematoma in Sickle Cell Anemia: Case Report and Literature Review. J Surg Tech Case Rep. 4:135-137. 7. Szkup P, Stoneham G (2004) Case report: spontaneous spinal epidural haematoma during pregnancy: case report and review of the literature. Br J Radiol. 77:881. 8. Jea A, Moza K, Levi AD, Vanni S (2005) Spontaneous spinal epidural hematoma during pregnancy: case report and literature review. Neurosurgery. 56:E1156. 9. Shimokawa S, Hayashi T, Anegawa S, Nakashima S, Tsutsumi K, Nakagawa S, Furukawa Y, Sugiyama T, Higashi H, Hori Y (2003) Spontaneous epidural hematoma in a patient undergoing hemodialysis: a case report. No To Shinkei. 55:163. 10. Takahashi K, Koiwa F, Tayama H, Satomi A, Akizawa T, Ideura T (1999) A case of acute spontaneous epidural haematoma in a chronic renal failure patient undergoing haemodialysis: successful outcome with surgical management. Nephrol Dial Transplant. 14:2499. 11. Martínez-Lage J, Saez V, Requena L, Martínez-Barba E, Poza M (2000) Cranial epidural hematoma in Paget's disease of the bone. Intensive Care Med. 26:1582. 12. Ng W, Yeo TT, Seow WT (2004) Non-traumatic spontaneous acute epidural haematoma--report of two cases and review of the literature. J Clin Neurosci. 11:791-3. 13. McIver J, Scheithauer BW, Rydberg CH, Atkinson JL (2001) Metastatic hepatocellular carcinoma presenting as epidural hematoma: case report. Neurosurgery. 49:447. 14. Kelly D, Smith JM (1968) Epidural Hematoma Secondary to Frontal Sinusitis Case Report. Journal of Neurosurgery. 28:67-9. 15. Matsumoto K, Akagi K, Abekura M, Tasaki O (2001) Vertex epidural hematoma associated with traumatic arteriovenous fistula of the middle meningeal artery: a case report. Surg Neurol. 55:302. 16. Naran A, Fontana L (2001) Sickle cell disease with orbital infarction and epidural hematoma. Pediatr Radiol. 31:257-9. 17. Hettige S, Sofela A, Bassi S, Chandler C (2015) A review of spontaneous intracranial extradural hematoma in sickle cell disease. Acta Neurochir. 157:2025-2029. 18. Kossorotoff M, Brousse V, Grevent D, Naggara O, Brunelle F, Blauwblomme T, Gaussem P, Desguerre I, De Montalembert M (2015) Cerebral haemorrhagic risk in children with sickle-cell disease. Dev Med Child Neurol. 57:187-93. 19. Yogarajah M, Agu CC, Sivasambu B, Mittler MA (2105) HbSC Disease and Spontaneous Epidural Hematoma with Kernohan's Notch Phenomena. Case Reports in Hematology. Article ID: 470873:3. 20. Watanabe M, Saito N, Nadgir RN, Liao JH, Flower EN, Steinberg MH, Sakai O (2013) Craniofacial bone infarcts in sickle cell disease: clinical and radiological manifestations. J Compur Assist Tomogr. 37:91-7. 21. Arends S, Coebergh JA, Kerkhoffs JL, van Gils A, Koppen H (2011) Severe unilateral headache caused by skull bone infarction with epidural haematoma in a patient with sickle cell disease. Cephalalgia. 31:1325-8. 22. Ganesh A, Al-Zuhaibi S, Pathare A, William R, Al-Senawi R, Al-Mujaini A, Hussain S, Wali Y, Alkindi S, Zachariah M, Knox-Macaulay H (2008) Orbital infarction in sickle cell disease. Am J Ophthalmol. 146:595-601. 23. Karacostas D, Artemis N, Papadopoulou M, Chistakis J (1991) Case report: epidural and bilateral retroorbital hematomas complicating sickle cell anemia. Am J Med Sci. 302:107-9. 24. Resar L, Oliva MM, Casella JF (1996) Skull infarction and epidural hematomas in a patient with sickle cell anemia. J Pediatr Hematol Oncol. 18:413-5. 25. Alli N, Wainwright RD, Mackinnon D, Poyiadjis S, Naidu G (2007) Skull bone infarctive crisis and deep vein thrombosis in homozygous sickle cell disease- case report and review of the literature. Hematology. 12:169-174. 26. Ilhan N, Acipayam C, Aydogan F, Atci N, Ilhan O, Coskun M, Daglioglu MC, Tuzcu EA (2014) Orbital compression syndrome complicated by epidural hematoma and wide cephalohematoma in a patient with sickle cell disease. Journal of AAPOS. 18:189-91. 27. Janssens C, Claeys L, Maes P, Boiy T, Wojciechowski M (2015) Orbital wall infacrtion in child with sickle cell disease. Acta Clin Belg. 70:451-2. 28. Tony J, Subramanya G, Kallur KG, Chalapathy AV, Sheshadri S, Lakhkar B (1995) Proptosis, skull infarction and epidural haematoma in sickle thalassemia. Postgrad Med J. 71:445. 29. Kotb M, Tantawi WH, Elsayed AA, Damanhouri GA, Malibary HM (2006) Brain M.R.I. CT findings in sickle cell disease patients from Western Saudi Arabia. Neurosciences (Riyadh). 11:28-36. 30. Kalala Okito J, Van Damme O, Calliauw L (2004) Are spontaneous epidural haematoma in sickle cell disease a rare complication? A report of two new cases. Acta Neurochir (Wien). 146:407-10. 31. Serarslan Y, Aras M, Altas M, Kaya H, Urfali B (2014) Non-traumatic spontaneous acute epidural hematoma in a patient with sickle cell disease. Neurocirugia. 25:128-31. 32. Bo¨lke E, Scherer A. (2012) Sickle cell disease. CMAJ 184:E201. 33. Niebanck A, Pollock AN, Smith-Whitley K, Raffini LJ, Zimmerman RA, Ohene-Frempong K, Kwiatkowski JL (2007) Headache in Children with Sickle Cell Disease: Prevalence and Associated Factors. Journal of Pediatrics. 151:67-72.