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来自：双语学影像；本期病例选自AJNR Case of the Week Katsunari Namba, MD,1 Ayuho Higaki, MD,2 Shigeru Nemoto, MD31 Division of Neuroendovasclar ；urgery - Endovascular Therapy, Jichi Medical University, Tochigi Japan；2 Department of Neurosurgery, Jichi Medical University, Tochigi, Japan；3 Department of Endovascular Surgery, Tokyo Medical and Dental University, Tokyo, Japan 
DescriptionA 10-year-old boy without known medical history presented with sudden onset of headache followed by low back pain. Neurological examination was intact except for neck stiffness. On physical examination, a subtle nevus was observed on his back. 10岁男孩，既往史不详，突然发作头痛，随后出现下腰痛。神经系统检查：颈部僵硬，余（-）。体检颈背部隐约可见痣。
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LegendsA photograph of the upper back demonstrates a subtle capillary malformation on the patient’s back, at the C4-7 dermatomal level (arrowheads). CT in axial (B, top) and sagittal (B, bottom) planes demonstrate subarachnoid hemorrhage, predominantly in the posterior fossa and upper cervical spine (arrowheads). Sagittal T1WI MRI of the cervical area in the sagittal plane reveals extensive vascular malformation involving the C4-5 spinal cord (arrowheads) and C5-7 vertebral body and paraspinal tissues (arrows). Fused angiogram of the bilateral vertebral arteries and left subclavian artery in AP view demonstrates extensive arteriovenous malformations affecting the C4-5 spinal cord (arrowheads) and C5-7 vertebral body and paraspinal tissues (arrows). 患者背部照片可见约C4-7水平颈背部细微毛细血管畸形。 CT横断位及矢状位重建示后颅凹和上颈椎蛛网膜下腔出血。
MR矢状位T1WI示广泛血管畸形，主要累及C4-5水平脊髓和C5-7水平椎体和椎旁软组织。
双侧椎动脉和左侧锁骨下动脉造影示：约C4-5水平脊髓和C5-7水平椎体和椎旁软组织血管畸形。
Cobb Syndrome (Spinal Arteriovenous Metameric Syndrome)
节段性脊髓血管畸形综合征
Cobb syndrome is a complex of vascular malformations affecting the spinal cord, epidural space, vertebral body, paraspinal soft tissue, muscle, subcutaneous tissue, and skin that are derived from the same spinal metameric segment. For this reason, Cobb syndrome is also referred to as spinal arteriovenous metameric syndrome. Cobb综合征是一种累及脊髓、硬膜外腔、椎体、椎旁软组织、肌肉、皮下组织、皮肤的复杂血管畸形，病变位于同一脊髓节段，又称为节段性脊髓血管畸形综合征。 Clinical features could be related to spinal cord hemorrhage, subarachnoid hemorrhage, mass effect of the spinal cord and roots from dilated arteries and veins, venous hypertension, and vascular steal phenomenon. 临床症状与脊髓出血、蛛网膜下腔出血、动静脉扩张所致的占位效应和神经根症状、静脉压增高、血管盗血相关。
Key Diagnostic FeaturesThe finding of vascular malformations affecting more than two tissues in a given spinal metamere confirms the diagnosis. However, involvement of all of the tissue elements in the metamere is rare. Skin vascular malformation can manifest as a distinct nevus or a subtle discoloration, and may be the first recognizable sign of this complex disease. The vascular malformations often affect more than one spinal metameric segment.
同一脊髓节段两种以上组织的血管畸形可以证实Cobb综合征的诊断，但同时累及一个节段的所有组织相对少见。皮肤血管畸形表现为明显的痣或轻微的颜色改变，可能是本病复杂血管畸形的首要标志。血管畸形可累及多个脊髓节段。
DDx
Spinal cord AVM, spinal cord AVF 脊髓动静脉畸形、动静脉瘘
Rx
Palliative endovascular embolization. Due to the diffuse and extensive nature of the malformation, surgical excision or endovascular embolization aiming complete cure is difficult. Targeted embolization of the symptomatic lesion may be the choice of therapy. 一般选择姑息性血管内栓塞治疗。由于血管畸形较为广泛，手术切除或彻底血管内栓塞相对困难。对症的靶向血管内栓塞可能是治疗的首选。
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