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LETTER TO EDITOR
Year : 2011  |  Volume : 59  |  Issue : 2  |  Page : 294-296

Spinal epidural hematoma in a child with hemophilia B


Department of Neurosurgery, West China Hospital, Sichuan University, No. 37, GuoXueXiang, Chengdu, Sichuan Province, China

Date of Submission04-Nov-2010
Date of Decision04-Nov-2010
Date of Acceptance06-Nov-2010
Date of Web Publication7-Apr-2011

Correspondence Address:
Siqing Huang
Department of Neurosurgery, West China Hospital, Sichuan University, No. 37, GuoXueXiang, Chengdu, Sichuan Province
China
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.79150

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How to cite this article:
Zhong W, Chen H, You C, Huang S. Spinal epidural hematoma in a child with hemophilia B. Neurol India 2011;59:294-6

How to cite this URL:
Zhong W, Chen H, You C, Huang S. Spinal epidural hematoma in a child with hemophilia B. Neurol India [serial online] 2011 [cited 2019 Sep 18];59:294-6. Available from: http://www.neurologyindia.com/text.asp?2011/59/2/294/79150


Sir,

A 7-year-old boy was admitted with neck pain and progressive limb weakness of 2 days duration. There was no history of significant trauma except for a neck manipulation for wryneck 1 day prior. He had been diagnosed hemophilia B for 3.5 years and experienced hemorrhages several times. Neurological examination revealed motor weakness of 3/5 in the upper limbs and 2/5 in the lower limbs, sensory level below C3 segment, and bilateral extensor plantar response. Magnetic resonance imaging (MRI) of the spine revealed an epidural compressive lesion ventral to the cord, extending from C2 to T1. It was isointense on T1-weighted images and heterogeneous hyperintense on T2-weighed images [Figure 1], [Figure 2] and [Figure 3], consistent with acute epidural hematoma. The FIX activity was less than 11% and intracranial hemorrhage was excluded. The child was managed conservatively with prothrombin complex replacement (1500 units for 3 days and 600 units every other day for 2 weeks) and intravenous steroid. The patient showed gradual improvement in the neurological deficits over the next 3 days and returned to normal within 3 weeks. The epidural hematoma resolved over a period of 1 month.
Figure 1: Sagittal T1 MRI. The epidural lesion was isointense on T1- weighed imaging extending from C2 to T1

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Figure 2: Sagittal T2 MRI. The epidural lesion was of high signal on T2- weighed imaging

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Figure 3: Transverse T2 MRI. The lesion (arrow) was heterogeneous high signal T2 signal, and lying ventral to the spinal canal with spinal cord compression

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Spinal epidural hematoma is a rare neurosurgical complication of hemophilia and can result in significant disability or even death. The location is usually in the cervicothoracic region, often extensive and mostly in the dorsal epidural space, rarely in the ventral of the spine. [1] Patients with spinal epidural hematoma present with sudden severe back pain, followed by rapid and progressive signs and symptoms of spinal cord compression within minutes to days. However, the clinical presentation in the young can be at times vague and often results in delay in the diagnosis. [2] An MR imaging helps in establishing the diagnosis in suspected patients. The exact mechanism of spinal epidural hematoma is still unknown. Venous origin has been proposed. In our patient, the most probable mechanism is minor trauma to the neck, which might have directly resulted in the epidural venous plexus rupture, or the neck manipulation might have increased the intrathoracic pressure leading to the defective valves' epidural venous plexus rupture. Plasma concentration of the factor determines the risk of hemorrhage. Factor replacement has been recommended in every hemophilic patient with hematomas; the factor level should be up to 80-100% before any imaging studies and should be over 50% for at least 14 days. [3],[4] If the neurological deficits are progressive, surgical intervention can be considered. [1],[3] However, surgical decompression carries a high risk of operative re-bleeding and postoperative spinal deformity. Our patient made good functional recovery with conservative treatment, as has been reported earlier. [2],[3] The place of conservative approach seems in patients with mild neurological or no-progress neurologic deficits. A good preoperative neurological condition usually results in a good outcome. Our patient experienced a rapid and excellent outcome. It may be that in our patient the hematoma was not a solid clot and it might have resolved rapidly. It should also be reminded to the parents that excessive traction to the neck should be forbidden in hemophilic children to avoid hemorrhage.

 
  References Top

1.Rois PV, López MR, de Vergara BC, de la Lama Zaragoza A, García JG, Uxo JM. Spinal epidural hematoma in hemophilic children: Controversies in management. Childs Nerv Syst 2009;25:987-91.  Back to cited text no. 1
    
2.Iwamuro H, Morita A, Kawaguchi H, Kirino T. Resolution of spinal epidural haematoma without surgery in a haemophilic infant. Acta Neurochir (Wien) 2004;146:1263-5.  Back to cited text no. 2
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3.Balkan C, Kavakli K, Karapinar D. Spinal epidural haematoma in a patient with haemophilia B. Haemophilia 2006;12:437-40.  Back to cited text no. 3
[PUBMED]  [FULLTEXT]  
4.Heer JS, Enriquez EG, Carter AJ. Spinal epidural hematoma as first presentation of hemophilia A. J Emerg Med 2008;34:159-62.  Back to cited text no. 4
[PUBMED]  [FULLTEXT]  


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