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Table of Contents    
Year : 2011  |  Volume : 59  |  Issue : 2  |  Page : 301-302

Spontaneous epidural hematoma: A rare complication of sickle cell anemia

1 Department of Medicine, Byramjee Jeejeebhoy Medical College & Sassoon General Hospitals, Pune, India
2 Department of Neurosurgery, Byramjee Jeejeebhoy Medical College & Sassoon General Hospitals, Pune, India

Date of Submission20-Dec-2010
Date of Decision20-Dec-2010
Date of Acceptance21-Dec-2010
Date of Web Publication7-Apr-2011

Correspondence Address:
Shashikala A Sangle
Department of Medicine, Byramjee Jeejeebhoy Medical College & Sassoon General Hospitals, Pune
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.79156

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How to cite this article:
Sangle SA, Lohiya RV, Karne SS, Chugh A. Spontaneous epidural hematoma: A rare complication of sickle cell anemia. Neurol India 2011;59:301-2

How to cite this URL:
Sangle SA, Lohiya RV, Karne SS, Chugh A. Spontaneous epidural hematoma: A rare complication of sickle cell anemia. Neurol India [serial online] 2011 [cited 2022 Jan 23];59:301-2. Available from:


In sickle cell anemia spontaneous extradural hematoma is a rare complication and can be fatal. We are reporting one such case.

A 15-year-old boy, first product of non-consanguineous marriage and known case of sickle cell anemia, was admitted for moderate grade fever associated with headache and pains in the hands and feet. There was no history of seizures, limb weakness, abdominal pain or trauma. On admission his vitals were: temperature 99ºF, pulse 100 beats per minute and blood pressure 110/70 mm Hg. Patient was drowsy and could obey vocal commands and had no focal neurological deficit. Investigations revealed: hemoglobin of 9.7 gm/dL, leukocyte count 9600/, hematocrit of 31% and platelets 2.1 lakh/ Peripheral blood smears showed microcytic normochromic red blood cells (RBC) and sickle-shaped RBCs.

Over the next 12 hours, the patient developed rapid deterioration of consciousness with left eye congestion and proptosis. His GCS rapidly deteriorated from a score of 12 to 4. An emergent magnetic resonance imaging (MRI) of brain with MR-venography revealed multiple large extradural hematomas (7.5 Χ 4.6 Χ 7.8 cm) in left frontal region causing severe mass effect, subfalcine and uncal herniation and also a small (3.4 Χ 1.6 Χ 3.7 cm) right frontal extradural hematoma [Figure 1]. MR-venography was normal. He had an urgent decompression surgery. A bicoronal skin flap was raised followed by bilateral frontal craniotomies and evacuation of the extradural hematomas. The skull bone was soft and thinned out. The bone flaps were of normal color and consistency. Histopathology of blood clot showed presence of sickle cell RBCs with precursors while bone fragment examination showed thinned out bony trabeculae with hyper-proliferative bone marrow [Figure 2].
Figure 1: T1-weighted MR axial image showing heterogenous isointense biconvex lesions in left and right frontal region s/o extradural hematoma with midline shift to right

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Figure 2: Photomicrograph of bony fragment showing hypercellular marrow with no evidence of bony infarct (H and E stain, ×10)

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Postoperatively patient was mechanically ventilated and managed in the intensive care unit. The patient's general condition however deteriorated with decrease in urine output. Patient developed acute renal failure and succumbed.

Sickle cell anemia is associated with various complications and the average life expectancy is 42 years in males and 48 years in females. [1] Acute neurological complications in patients of sickle cell anemia can be ischemic or hemorrhagic. Cerebral ischemic complications are most common accounting for two-third of all neurological complications [2] and are due to vaso-occlusive phenomenon in sickle cell anemia. Hemorrhagic complications are uncommon; intracerebral hemorrhage is common and subarachnoid hemorrhage and spontaneous epidural hematomas are very rare. [3]

Spontaneous extradural hematomas as a complication of sickle cell anemia have been reported mostly secondary to bone infarction. [4] Possible mechanisms for such hematomas are: [4],[5] (1) periosteal elevation secondary to bone infarction with disruption of the cortical bone margin, and bleeding into the epidural space; (2) involvement of epidural vessels in the vicinity of infracted bone can lead to spontaneous rupture of these vessels and formation of hematomas; (3) poor venous drainage leading to venous congestion and rupture of these thin-walled veins and (4) expanding hematopoietic tissue disrupting the inner and outer skull tables with bleeding into subgaleal and epidural spaces. In our patient there was no bone infarction and MR-venography was normal thus the likely pathogenesis is expansion of hematopoietic tissue and disruption of bony margins.

  References Top

1.Platt OS, Brambilla DJ, Rosse WF, Milner PF, Castro O, Steinberg MH, et al. Mortality in sickle cell disease. Life expectancy and risk factors for early death. N Eng J Med 1994;330:1639-44.   Back to cited text no. 1
2.Wood DH. Cerebrovascular complications of sickle cell anaemia. Stroke 1978;9:73-5.  Back to cited text no. 2
3.Anson JA, Koshy M, Ferguson L, Crowell RM. Subarachnoid haemorrhage in sickle-cell disease. J Neurosurg 1991; 75:552-8.  Back to cited text no. 3
4.Dahdaleh NS, Lindley TE, Kirby PA, Oya H, Howard MA 3rd. A neurosurgical crisis of sickle cell disease. J Paediat Neurosurgery 2009;4:532-5.  Back to cited text no. 4
5.Naran AD, Fontana L. Sickle cell disease with orbital infarction and epidural hematoma. Pediatr Radiol 2001;31:257-9.  Back to cited text no. 5


  [Figure 1], [Figure 2]

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