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手术切除Cobb综合征巨大脊髓硬膜外海绵状血管瘤的治疗:病例说明

Surgical treatment to resect giant intraspinal epidural cavernous hemangioma of Cobb syndrome: illustrative case.

作者信息

Ying Yu-Zhe, Wang Gui-Huai, Saberi Hooshang, Sharma Hari Shanker, Sun Zhen-Xing

机构信息

Department of Neurosurgery, Beijing Tsinghua Changgung Hospital, School of Clinical Medicine, Tsinghua University, Beijing, China.

Department of Neurosurgery, Imam Khomeini Hospital Complex, Tehran University of Medical Sciences, Tehran, Iran.

出版信息

J Neurosurg Case Lessons. 2025 Jun 23;9(25). doi: 10.3171/CASE24838.

Abstract

BACKGROUND

Cobb syndrome is a rare nonfamilial neurocutaneous syndrome. It is characterized by vascular malformations of the skin, spine, spinal membrane, and spinal cord of the same body segment. However, epidural cavernous hemangioma as the main manifestation of intravertebral canal space is extremely rare, and the treatment is often more complex and difficult. Only a few cases have been reported.

OBSERVATIONS

Here, the author reported a case of Cobb syndrome with a large intraspinal cavernous hemangioma in a 23-year-old man with paresthesia and paralysis of both lower limbs. The authors highlight the case presentation, operative technique, and postoperative course. Following surgery, there were no immediate surgical complications, and the patient noted clinical improvement in neurological function.

LESSONS

This report presents a case of Cobb syndrome involving a large spinal epidural cavernous hemangioma, with the aim to enhance the understanding of this rare condition. Although delayed treatment still yielded favorable recovery in this case, early MRI screening (in patients with cutaneous hemangiomas and neurological symptoms) could prevent irreversible spinal cord damage. An ultrasonic bone cutter should be considered the instrument of choice for epidural hypervascular lesions, particularly in reoperations or high-bleeding-risk scenarios, due to its hemostatic mechanical effect, which minimizes intraoperative risks (dural injury or major hemorrhage). https://thejns.org/doi/10.3171/CASE24838.

摘要

背景

科布综合征是一种罕见的非家族性神经皮肤综合征。其特征是同一身体节段的皮肤、脊柱、脊膜和脊髓出现血管畸形。然而,以椎管内海绵状血管瘤为主要表现极为罕见,且治疗往往更为复杂困难。仅有少数病例报道。

观察

在此,作者报告了一例23岁男性科布综合征患者,伴有巨大脊髓内海绵状血管瘤,出现双下肢感觉异常和瘫痪。作者重点介绍了病例表现、手术技术及术后过程。术后未出现即刻手术并发症,患者神经功能有临床改善。

经验教训

本报告展示了一例涉及巨大脊髓硬膜外海绵状血管瘤的科布综合征病例,旨在增进对这种罕见病症的了解。尽管该病例延迟治疗仍取得了良好恢复,但早期MRI筛查(对于有皮肤血管瘤和神经症状的患者)可预防不可逆的脊髓损伤。由于其止血机械效应可将术中风险(硬脊膜损伤或大出血)降至最低,对于硬膜外高血运病变,尤其是再次手术或高出血风险情况,应考虑选择超声骨刀。https://thejns.org/doi/10.3171/CASE24838

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1603/12184525/e767c6bd7742/CASE24838_figure_1.jpg

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