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表现为马尾蛛网膜下腔出血的硬脊膜内外哑铃形血管母细胞瘤

Intra-extradural dumbbell-shaped hemangioblastoma manifesting as subarachnoid hemorrhage in the cauda equina.

作者信息

Nishimura Yusuke, Hara Masahito, Natsume Atsushi, Takemoto Masaya, Fukuyama Ryuichi, Wakabayashi Toshihiko

机构信息

Department of Neurosurgery, Nagoya University School of Medicine, Nagoya, Aichi, Japan.

出版信息

Neurol Med Chir (Tokyo). 2012;52(9):659-65. doi: 10.2176/nmc.52.659.

Abstract

A 56-year-old man presented with a rare intra-extradural dumbbell-shaped hemangioblastoma in the lumbar spine associated with von Hippel-Lindau (VHL) disease manifesting as subarachnoid hemorrhage. The tumor, which was exiting at the right L2-3 intervertebral foramen, was removed successfully by total facetectomy and posterior spinal fusion at the L2-3 segment. Nine years later, a recurrent tumor due to VHL was also totally removed with minimal neurological complications. Accurate diagnosis of this vascular-rich lesion is essential for developing an adequate surgical strategy. The dumbbell-shaped tumor requires total facetectomy and spinal reconstruction, and care should be taken to preserve the entire nerve root origin by only identifying the affected nerve fascicles at the origin, if possible. Postoperative adhesion must be minimized for second surgery in patients with genetic disease such as VHL, who are likely to suffer recurrence.

摘要

一名56岁男性患者,患有罕见的腰椎硬膜内-外哑铃形血管母细胞瘤,与表现为蛛网膜下腔出血的冯·希佩尔-林道(VHL)病相关。肿瘤经右侧L2-3椎间孔穿出,通过L2-3节段全椎板切除术和后路脊柱融合术成功切除。9年后,因VHL复发的肿瘤也被完全切除,神经并发症极少。准确诊断这种富含血管的病变对于制定合适的手术策略至关重要。哑铃形肿瘤需要全椎板切除术和脊柱重建,如有可能,应仅识别起源处受影响的神经束,小心保留整个神经根起源。对于像VHL这种可能复发的遗传性疾病患者,二次手术时必须尽量减少术后粘连。

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