Medical School of Nankai University, No. 94, Weijin Road, Naikai District, Tianjin, 300071, People's Republic of China.
Department of Neurosurgery, Chinese PLA General Hospital, No. 28, Fuxing Road, Haidian District, Beijing, 100853, People's Republic of China.
Acta Neurochir (Wien). 2020 Nov;162(11):2915-2921. doi: 10.1007/s00701-020-04358-x. Epub 2020 May 23.
Pure spinal epidural cavernous malformation (CM) is a rare hypervascular disease that is easily misinterpreted as other imaging-similar epidural lesions. The demographic characteristics, therapeutic strategies, and surgical outcomes associated with this vascular entity remains unclear.
A retrospective review of patients with pathologically proven, pure epidural CM from 2001 to 2018 was conducted. All data that included clinical manifestations, radiographic features, and treatment modalities were analyzed.
Twenty-three consecutive patients with an average age of 51.5 ± 8.4 years old (range 38-70 years old) were included; of these, 52.2% were female patients. Clinical manifestation included chronic progressive nerve root disturbance syndrome in 7 patients (30.4%) and myelopathy in 16 patients (69.6%). The CM level was predominately thoracic (n = 14, 60.9%) or lumbar (n = 6, 26.1%), with the dorsal epidural space (n = 14, 60.9%) the most common site. The initial clinical diagnoses were schwannoma (n = 11, 52.2%), meningioma (n = 5, 21.7%), angioma (n = 3, 13.1%), recurrent CMs (n = 2, 8.7%), and metastatic tumor (n = 1, 4.3%). Fifteen lesions (65.2%) were isointense on T1-weighted images, and all lesions were hyperintense on T2-weighted images, with homogenously strong enhancement observed in 17 lesions (73.9%). Total resection was achieved in 18 patients (78.3%) and usually resulted in excellent clinical outcomes (n = 21, 91.3%). No patients experienced recurrence of symptoms, and lesion relapse during follow-up.
Total surgical removal of epidural CM can usually achieve satisfactory outcomes in patients with a chronic clinical course and should be recommended. Subtotal removal of tumors can also benefit patients, and guaranteed a long recurrent free time after surgery. A good preoperative neurological condition usually leads to good outcomes.
单纯脊柱硬膜外海绵状畸形（CM）是一种罕见的高血管性疾病，很容易被误诊为其他影像学相似的硬膜外病变。与这种血管实体相关的人口统计学特征、治疗策略和手术结果仍不清楚。
对 2001 年至 2018 年期间经病理证实的单纯硬膜外 CM 患者进行回顾性分析。分析了所有包括临床表现、影像学特征和治疗方式的数据。
共纳入 23 例平均年龄 51.5±8.4 岁（38-70 岁）的连续患者；其中 52.2%为女性患者。临床表现包括 7 例（30.4%）慢性进行性神经根紊乱综合征和 16 例（69.6%）脊髓病。CM 水平主要为胸段（n=14，60.9%）或腰段（n=6，26.1%），最常见的部位为硬膜外背侧（n=14，60.9%）。最初的临床诊断为神经鞘瘤（n=11，52.2%）、脑膜瘤（n=5，21.7%）、血管瘤（n=3，13.1%）、复发性 CM（n=2，8.7%）和转移性肿瘤（n=1，4.3%）。15 个病灶（65.2%）在 T1 加权图像上呈等信号，所有病灶在 T2 加权图像上均呈高信号，17 个病灶（73.9%）呈均匀强化。18 例患者（78.3%）行肿瘤全切除，通常可获得良好的临床效果（n=21，91.3%）。无患者出现症状复发，随访期间无病灶复发。
慢性病程患者行硬膜外 CM 全切除通常可获得满意的结果，应予以推荐。肿瘤次全切除也可使患者受益，并保证术后较长的无复发时间。术前神经功能良好通常可获得良好的结果。
