Liang Chao-hui, Zhang Hong-qi, Zhi Xing-long, Zhang Peng, Li Meng, Ling Feng
Department of Neurosurgery, Xuanwu Hospital, Beijing 100053, China.
Zhonghua Yi Xue Za Zhi. 2010 Apr 6;90(13):882-5.
Cutaneous vertebral medullary angiomatosis, also known as Cobb's syndrome, is a part of spinal arteriovenous metameric syndromes (SAMs), is derived from the vascular malformation triad of skin, bone, and spinal cord involvement. It is poorly managed with current treatment modalities. We reviewed the treatment of Cobb's syndrome series and summarized the experiences.
A total of 61 cases of Cobb's syndrome with spinal cord dysfunction were treated at our department from February 2003 to December 2007. The treatment followed the same strategy: First step-investigating the pathogenic mechanisms by symptom onset, MRI, angiography and the response to initial treatment. Second step-treating the problematic parts of the lesions with embolization alone, surgery alone or combination of both. Forty-eight cases were embolized, 3 cases treated with surgery and 10 cases treated with a combination of both. Nidus involving pathogenic mechanism for spinal cord were eliminated, completely and nearly disappeared in 36 cases and partially in 25 cases. All patients were followed up regularly.
Among these 61 cases, the pathogenic mechanisms were identified as hemorrhage, mass effect, ischemia and venous hypertensive myelopathy. Two or more mechanisms could coexist in the same patient. More than 1-year's spinal cord function follow-up showed: excellent in 9 cases, good in 26, fair in 23 and worse in 3.
Not all lesions of Cobb's syndrome can be or should be cured anatomically. Pathogenic mechanism should be analyzed carefully and the treatment should focus on the special cord-affecting targets. Long-term improvement or stabilization may be achieved.
皮肤性椎体髓内血管瘤病，也称为科布综合征，是脊髓动静脉节段性综合征（SAMs）的一部分，源于皮肤、骨骼和脊髓受累的血管畸形三联征。目前的治疗方式对其疗效不佳。我们回顾了一系列科布综合征的治疗情况并总结经验。
2003年2月至2007年12月，我科共治疗61例伴有脊髓功能障碍的科布综合征患者。治疗遵循相同策略：第一步——通过症状发作、磁共振成像（MRI）、血管造影及初始治疗反应来探究致病机制。第二步——对病变的问题部位单独采用栓塞治疗、单独手术治疗或两者联合治疗。48例行栓塞治疗，3例行手术治疗，10例行两者联合治疗。涉及脊髓致病机制的瘤巢，36例完全消除且几乎消失，25例部分消除。所有患者均定期随访。
在这61例患者中，致病机制确定为出血、占位效应、缺血和静脉高压性脊髓病。两种或更多机制可在同一患者中共存。超过1年的脊髓功能随访结果显示：优9例，良26例，中23例，差3例。
并非所有科布综合征病变都能或都应在解剖学上治愈。应仔细分析致病机制，治疗应针对影响脊髓的特定靶点。可实现长期改善或稳定。
