合并脊髓静脉高压综合征的脊髓血管母细胞瘤的手术治疗

Microsurgical treatment of spinal cord hemangioblastoma associated with spinal hypertensive myelopathy

目的总结合并脊髓静脉高压综合征(VHM)的脊髓血管母细胞瘤的诊治经验。方法回顾性分析2005~2019年手术治疗的10例脊髓血管母细胞瘤的临床资料,结合术前MRI T2像及病人临床表现评估VHM,并总结脊髓血管母细胞瘤合并VHM的诊治经验。结果单发肿瘤9例,其中肿瘤位于颈段3例、胸段4例、腰段2例;多发1例(颈段、胸段及腰段均有)。术前存在VHM 6例,术前MRI均可见病变节段以外的脊髓中央区MRI T2高信号,存在明显的脊髓功能损伤症状,如肢体运动功能障碍或大小便功能障碍等。10例肿瘤均全切除,其中1例多发肿瘤一次切除3个胸段肿瘤。无手术死亡病例。结论脊髓血管母细胞瘤为富含血管的血管性肿瘤,往往有高流量的血流通过,引流静脉常迂曲扩张并同脊髓表面正常引流静脉沟通导致VHM,有症状者应早期手术,避免出现不可逆的神经功能损伤,肿瘤切除后VHM自然治愈。

Objective To summarize the experience in diagnosis and treatment of spinal angioblastomas complicated with spinal hypertensive myelopathy(VHM).Methods The clinical data of 10 patients with spinal angioblastomas who underwent microsurgery from 2005 to 2019 were analyzed retrospectively.The VHM was diagnosed according to the preoperative MRI T2 weighted image combined with clinical manifestations of patients.Results There were multiple tumors in one patient and only one tumor in 9 patients of whom the tumor was located in cervical segment of 3 patients,in thoracic segment of 4 and lumbar segment of 2.There were 6 patients with VHM before operation.Preoperative MRI showed high T2 signal in the central region of the spinal cord other than the diseased segment.There were obvious symptoms of spinal cord injury,such as limb motor dysfunction or dysfunction.The tumors were completely resected in 10 patients.Three thoracic tumors were resected at one time in the patient with multiple tumors.There were no patients died from the operation.Conclusions The spinal angioblastoma is a type of vascular-rich tumor.The tortuous and dilated drainage veins of the spinal angioblastomas are often communicated with the normal draining veins on the surface of the spinal cord,resulting in VHM.The patients who were associated with the spinal injury symptoms should be operated as early as possible in order to avoid irreversible neurological damage.The VHM can be cured after the total resection of tumors.