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Year : 2012  |  Volume : 7  |  Issue : 1  |  Page : 77-78

A rare case of spontaneous bilateral extradural hematoma in a sickle cell disease child

Department of Neurosurgery, S.C.B. Medical College, Cuttack, Orissa, India

Date of Web Publication28-Jun-2012

Correspondence Address:
Sunil K Patra
Qr. No: B-1/75, Lingaraj Vihar, Pokhariput, Bhubaneswar, Orissa - 751 020
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/1817-1745.97636

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How to cite this article:
Patra SK, Mishra SS, Das S. A rare case of spontaneous bilateral extradural hematoma in a sickle cell disease child. J Pediatr Neurosci 2012;7:77-8

How to cite this URL:
Patra SK, Mishra SS, Das S. A rare case of spontaneous bilateral extradural hematoma in a sickle cell disease child. J Pediatr Neurosci [serial online] 2012 [cited 2023 May 30];7:77-8. Available from: https://www.pediatricneurosciences.com/text.asp?2012/7/1/77/97636

Dear Sir,

Primary hemorrhagic stroke is a known but uncommon complication of sickle cell disease (SCD) in childhood, but extradural hemorrhage (EDH) is a more rare complication. EDH mostly develops after head injury with dramatic neurological deterioration. Among various presentations of complications (Primary hemorrhagic stroke mostly subarachnoid or intraventricular) of SCD, EDHs are rare clinical entities that may occur in children. We present a rare case of spontaneous bilateral EDH with SCD who presented with mild on and off headache in a 13-year-old boy and review the possible suggested mechanisms of its occurrences.

A 13-year-old boy presented with on and off headache for 2 years with vomiting and severe headache for 2 days without any history of trauma. He was a known SCD patient and was initially managed conservatively. CT scan revealed bilateral EDH in posterior parietal area [Figure 1]. Bilateral osteoplastic craniotomy and evacuation of hematoma [Figure 2] were done under general anesthesia. Postoperative course was uneventful. Postoperative CT scan revealed total removal of hematoma with satisfactory re-expansion of brain [Figure 3].
Figure 1: Closer view of bilateral extradural hemorrhage (EDH)

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Figure 2: Intraoperative removal of extradural hemorrhage (EDH)

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Figure 3: Postoperative noncontrast computed tomography (CT)

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Case series of EDH almost exclusively demonstrate trauma, sinus infection or arteriovascular malformations as the etiology. [1],[2],[3],[4] The only previous report of EDHs in sickle cell disease was from a French group. [5] There are many proposed risk factors for hemorrhagic stroke. The Cooperative Study of Sickle Cell Disease identified three significant risk factors for first hemorrhagic stroke: older age, low steady-state hemoglobin concentration, and high steady-state leukocyte count. [4] Other proposed risk factors include previous ischemic stroke, moyamoya lesions, cerebral aneurysms, acute chest syndrome, acute hypertension, hypertransfusion, [6],[7],[8],[9],[10] and antecedent treatment with steroids. Investigation showed that hypoxia significantly increased sickle RBC adherence to vascular endothelium when compared with the normoxia controls. However, hypoxia had no effect on the adherence of normal RBCs. Evaluation regarding the effects of hypoxia on the expression of EC surface receptors involved in RBC adherence to macrovascular ECs, including vascular cell adhesion molecule-1 (VCAM-1), intracellular adhesion molecule-1 (ICAM-1), and the vitronectin receptor (VnR) showed upregulation of the expression of both VCAM-1 and ICAM-1, whereas no effect on VnR and the whole effect is more pronounced in SCD. In the reported case, the child has normal leukocyte count and hypertension was detected, but it was difficult to comment whether it was the cause or the effect; moreover, the patient had a couple of transfusions but was not on hypertransfusion regimen and his steady-state hemoglobin was low (8-9 g/dl), with S. feritin 676.8 ng/ml. The mild derangement of coagulation profile is an accompanying feature of SCD. However, it is difficult to implement preventive measures or screening because no structural etiology was found for this EDH. We may speculate that the occurrence of EDH in this child suggests a possible inherited risk factor, perhaps a mutation that accentuates the "elastorrhexis" of the vascular wall seen in hemoglobinopathies. [11] But genetic studies would be greatly aided by report in other relatives. [2]

In summary, this is an unusual case of EDH (nontraumatic) in a child with SCD, from a sickle cell belt of Orissa. The child survived after extensive supportive care and then evacuation of bilateral EDH after osteoplastic craniotomy and was making progress in rehabilitation 3 months later. Reports of similar cases may provide new insights on the etiologies of EDH in SCD. EDH is among the emerging challenges in SCD care, because screening and preventive transfusions are reducing ischemic stroke, and therefore, more attention should be directed toward the nuance of hemorrhagic catastrophe.

   References Top

1.Hene-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;91:288-94.  Back to cited text no. 1
2.Tsomi K, Karagiorga-Lagana M, Karabatsos F, Fragodimitri C, van Vliet-Konstantinidou C, Premetis E, et al. Arterial elastorrhexis in beta-thalassaemia intermedia, sickle cell thalassaemia and hereditary spherocytosis. Eur J Haematol 2001;67:135-41.  Back to cited text no. 2
3.Browne GJ, Lam LT. Isolated extradural hematoma in children presenting to an emergency department in Australia. Pediatr Emerg Care 2002;18:86-90.  Back to cited text no. 3
4.Fullerton HJ, Wu YW, Zhao S, Johnston SC. Risk of stroke in children: Ethnic and gender disparities. Neurology 2003;61:189-94.  Back to cited text no. 4
5.Malik NK, Makhdoomi R, Indira B, Shankar S, Sastry K. Posterior fossa extradural hematoma: Our experience and review of the literature. Surg Neurol 2007;68:155-8.  Back to cited text no. 5
6.Stockman JA, Nigro MA, Mishkin MM, Oski FA. Occlusion of large Cerebral vessels in sickle cell anemia. N Engl J Med 1972;287:846-9.  Back to cited text no. 6
7.Royal JE, Sealer RA. Hypertension, Convulsions and cerebral hemorrhage in sickle cell anemia patients after blood transfusions. Lancet 1978;2:1207.  Back to cited text no. 7
8.Powars D, Adams RJ, Nicholas FT, Milner P, Charache S, Sarnaik S. Delayed Intracranial hemorrhage following cerebral infarction in sickle cell anemia. J Assoc Acad Phys 1990;1:79-82.  Back to cited text no. 8
9.Cabon I, Hladky JP, Lambillotte A, Cotton A, Dhellemmes P. Uncommon etiology of extradural hematoma. Neurochirurgie 1997;43:173-6.  Back to cited text no. 9
10.Dobson SR, Holder KR, Niettert PJ, Laver JH, Disco D, Abboud MR. Moyamoya syndrome in Childhood sickle cell disease: Predictive factors for recurrent Cerebrovascular events. Blood 2002;99:3144-50.  Back to cited text no. 10
11.Keyrouz SG, Diringer MN. Clinical review: Prevention and therapy of vasospasm in subarachnoid hemorrhage. Crit Care 2007;11:220.  Back to cited text no. 11


  [Figure 1], [Figure 2], [Figure 3]

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