体节性皮肤-肌肉-脊柱脊髓血管瘤病的治疗

Trealment of cutaneous vertebral medullary angiomatosis

目的探讨体节性皮肤．肌肉-脊柱脊髓血管瘤病（Cobb’s综合征）的血管构筑特点及其治疗方法。方法回顾2003年2月至2007年12月在首都医科大学宣武医院治疗的61例Cobb’s综合征患者的临床和影像学资料，根据患者的发病症状、临床表现、病变部位、脊髓MRI和脊髓血管造影，总结该病的血管构筑学特点和致病机制，并分析治疗方法和疗效。方法包括栓塞和手术，以及二者的结合，其中栓塞治疗48例，单纯手术治疗3例，术前栓塞＋手术切除10例。影响到脊髓的畸形部分的解剖疗效：完全或次全消除36例，部分消除25例。结果Cobb’s综合征由于其畸形的血管构筑学特点和解剖关系决定了致病机制，包括压迫、盗血、出血、静脉高压性淤血等。治疗的目标是影响脊髓功能的畸形部分，基于脊髓功能的临床疗效：随访1年以上的，优9例，良26例，尚可23例，差3例。结论Cobb’s综合征的血管构筑学复杂，术前需深入分析其致病机制，合理利用手术和栓塞相结合的方法，可以取得较好的疗效。

Objective 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. Methods 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 treatmenL 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. Results 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. Conclusion 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.