Sharma Giriraj K, Kucia Elisa J, Spetzler Robert F
Department of Neurological Surgery, George Washington University, Washington, District of Columbia, USA.
Neurosurgery. 2009 Sep;65(3):E627-8; discussion E628. doi: 10.1227/01.NEU.0000350979.86196.FA.
Spontaneous hemorrhage of a spinal hemangioblastoma is a rare event. With limited reports in the literature, the risk of acute hemorrhage associated with these lesions is poorly understood.
A 39-year-old man presented with paraplegia and bilateral upper-extremity weakness related to an acute intramedullary hemorrhage from a thoracic spinal hemangioblastoma. Magnetic resonance imaging revealed an intramedullary hemorrhage from T3 to T6 with prominent flow voids along the dorsal aspect of the spinal cord from T6 to T10. Magnetic resonance angiography of the thoracic spine indicated a prominent enhancing vessel along the dorsum of the thoracic cord.
An emergency T3 to T8 laminoplasty was performed for evacuation of the hematoma and gross total resection of the lesion. Pathological analysis of the tumor biopsy confirmed the diagnosis of hemangioblastoma.
The risk of spontaneous hemorrhage from a spinal hemangioblastoma is low. Spinal hemangioblastomas presenting with intramedullary hemorrhage tend to cause severe neurological deficits and have a poorer long-term prognosis compared with subarachnoid hemorrhage and nonhemorrhagic lesions.
脊髓血管母细胞瘤自发性出血是一种罕见事件。鉴于文献报道有限,与这些病变相关的急性出血风险了解甚少。
一名39岁男性因胸段脊髓血管母细胞瘤急性髓内出血出现截瘫和双侧上肢无力。磁共振成像显示T3至T6有髓内出血,T6至T10脊髓背侧有明显的流空信号。胸椎磁共振血管造影显示胸段脊髓背侧有一条明显强化的血管。
急诊行T3至T8椎板成形术以清除血肿并对病变进行全切除。肿瘤活检的病理分析证实为血管母细胞瘤。
脊髓血管母细胞瘤自发性出血的风险较低。与蛛网膜下腔出血和非出血性病变相比,表现为髓内出血的脊髓血管母细胞瘤往往会导致严重的神经功能缺损且长期预后较差。