Neurology India Medknow Publications on behalf of the Neurological Society of India ISSN: 0028-3886 EISSN: 1998-4022 Vol. 59, Num. 2, 2011, pp. 294-296

Neurology India, Vol. 59, No. 2, March-April, 2011, pp. 294-296

Letter to Editor

Spinal epidural hematoma in a child with hemophilia B

Weiying Zhong, Haifeng Chen, Chao You, Siqing Huang

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

Correspondence Address: Siqing Huang, Department of Neurosurgery, West China Hospital, Sichuan University, No. 37, GuoXueXiang, Chengdu, Sichuan Province, China, huangsiqing028@163.com

Date of Submission: 04-Nov-2010
Date of Decision: 04-Nov-2010
Date of Acceptance: 06-Nov-2010

Code Number: ni11079

PMID: 21483138

DOI: 10.4103/0028-3886.79150

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.

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.

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