血友病患儿的脊髓硬膜外血肿：治疗中的争议
Spinal epidural hematoma in hemophilic children: controversies in management.
作者信息
Rois Pilar Varela, López Manuel Rodríguez, de Vergara Beatriz Cabanas López, de la Lama Zaragoza Adolfo, García Jesús González, Uxo Jorge Mañas
机构信息
Department of Neurosurgery, Xeral-Cies Hospital, Pizarro Street, Vigo 36204, Pontevedra, Spain.
出版信息
Childs Nerv Syst. 2009 Aug;25(8):987-91; discussion 993, 995. doi: 10.1007/s00381-009-0861-3. Epub 2009 Apr 10.
INTRODUCTION AND OBJECTIVE
Spinal epidural hematoma (SEH) is an uncommon complication in hemophilic children. It can produce rapidly progressive neurological deficits. We aim to discuss the different management options for these patients.
CASE REPORT
A 13-year-old boy with a history of hemophilia A was admitted with acute onset of localized spine pain and weakness. No trauma was reported on review of the history. Recombinant factor VIII aggressive replacement therapy was started. Spinal magnetic resonance imaging revealed an extradural mass lesion extending from D5 to D6 level. Emergency hemilaminectomies of D5 and D6 and evacuation of the clot were done. The patient made excellent recovery following surgery.
CONCLUSION
Early diagnosis and immediate aggressive replacement therapy are mandatory in the management of SEH. Prompt surgical decompression to avoid any permanent neurological deficit is a safe and effective treatment option for an SEH in selected hemophilic children.
引言与目的
脊髓硬膜外血肿（SEH）是血友病儿童中一种罕见的并发症。它可导致迅速进展的神经功能缺损。我们旨在探讨这些患者的不同治疗选择。
病例报告
一名有甲型血友病病史的13岁男孩因急性发作的局部脊柱疼痛和无力入院。回顾病史未报告有外伤史。开始进行重组凝血因子VIII积极替代治疗。脊髓磁共振成像显示一个硬膜外肿块病变，从D5延伸至D6水平。对D5和D6进行了急诊半椎板切除术并清除了血块。患者术后恢复良好。
结论
在SEH的治疗中，早期诊断和立即积极的替代治疗是必不可少的。对于部分血友病儿童的SEH，及时进行手术减压以避免任何永久性神经功能缺损是一种安全有效的治疗选择。
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