一名小儿血友病患者发生自发性脊髓硬膜外血肿。
Spontaneous spinal epidural hematoma in a pediatric hemophiliac.
作者信息
Scalia Gianluca, Porzio Massimiliano, Costanzo Roberta, Iacopino Domenico Gerardo, Galvano Gianluca, Nicoletti Giovanni Federico, Umana Giuseppe Emmanuele
机构信息
Department of Head and Neck Surgery, Neurosurgery Unit, Garibaldi Hospital, Catania, Italy.
Department of Experimental Biomedicine and Clinical Neurosciences, School of Medicine, Postgraduate Residency Program in Neurological Surgery, Neurosurgical Clinic, AOUP "Paolo Giaccone", Palermo, Italy.
出版信息
Surg Neurol Int. 2023 Feb 24;14:69. doi: 10.25259/SNI_82_2023. eCollection 2023.
BACKGROUND
Spontaneous spinal epidural hematomas (SSEH), unrelated to trauma, epidural anesthesia, or surgery, are rare in the pediatric population. Here, a 1-year-old male with hemophilia presented with a magnetic resonance (MR)-documented SSEH and was successfully treated with a C5-T10 right hemilaminectomy.
CASE DESCRIPTION
A 1-year-old male with hemophilia presented with quadriparesis. The holo-spine magnetic resonance imaging with contrast showed a posterior cervicothoracic compressive epidural lesion extending from C3 to L1 consistent with an epidural hematoma. He underwent a C5 to T10 right-sided hemilaminectomy for clot removal, following which his motor deficits fully resolved. A literature review of SSEH attributed to hemophilia revealed that 28 of 38 cases were effectively treated conservatively, while only 10 cases warranted surgical decompression.
CONCLUSION
Select patients with SSEH attributed to hemophilia with severe MR-documented cord/cauda equina compromise and significant accompanying neurological deficits may require emergent surgical decompression.
背景
自发性脊髓硬膜外血肿（SSEH）与创伤、硬膜外麻醉或手术无关，在儿科人群中较为罕见。在此，一名患有血友病的1岁男性出现经磁共振成像（MR）确诊的SSEH，并通过C5 - T10右侧半椎板切除术成功治疗。
病例描述
一名患有血友病的1岁男性出现四肢瘫痪。增强全脊柱磁共振成像显示从C3至L1有一个颈胸段后方压迫性硬膜外病变，符合硬膜外血肿表现。他接受了C5至T10右侧半椎板切除术以清除血块，术后其运动功能障碍完全恢复。对血友病所致SSEH的文献回顾显示，38例中有28例经保守治疗有效，仅有10例需要手术减压。
结论
对于经MR证实脊髓/马尾严重受压且伴有明显神经功能缺损的血友病所致SSEH患者，部分可能需要紧急手术减压。
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