Johnson Walter D, Petrie Michelle M
Department of Neurosurgery, Loma Linda University, Loma Linda, CA, USA.
J Neurosurg Spine. 2009 May;10(5):430-5. doi: 10.3171/2009.1.SPINE08334.
Cobb syndrome is a rare clinical entity that includes the combination of a vascular skin nevus and an angioma in the spinal canal present at identical dermatomal level(s) (cutaneomeningospinal angiomatosis). To date, 38 cases have been reported, only 18 of which are in adults (> 18 years of age). The majority of these cases have been described in the era predating current neuroimaging techniques, and most authors have assumed that each case involves similar vascular pathology. This report highlights 2 patients presenting with similar thoracic cutaneous vascular nevi yet with markedly differing spinal vascular pathology. A 29-year-old man presented with cutaneous hemangioma and a progressive paraparesis and paresthesia of the lower extremities. A 20 x 20-cm port-wine stain over his right upper midback (T6-10) correlated precisely with MR imaging that demonstrated an enhancing epidural mass between T-6 and T-10 causing compression of the cord and cord edema. A 34-year-old man also presented with progressive myelopathy and a 15 x 20-cm port-wine stain within the same dermatomal region as a Type III spinal arteriovenous malformation. Workup for each patient included pre- and postoperative contrast-enhanced MR imaging with vascular sequencing and spinal angiography. The first patient was treated with bilateral laminectomy at the T6-10 levels, with significant postoperative improvement in motor strength. The second patient underwent coil embolization of an intranidal aneurysm, with follow-up embolization 8 years later. Cobb syndrome is an unusual entity in the adult population and should be considered when there is a constellation of cutaneous manifestation and underlying neurological deficit. The vascular skin nevus associated with Cobb syndrome is accompanied by a wide variety of vascular pathologies.
科布综合征是一种罕见的临床病症，其特征为在相同皮节水平出现皮肤血管痣和椎管内血管瘤（皮肤脊髓血管瘤病）。迄今为止，已报告38例，其中仅18例为成人（>18岁）。这些病例中的大多数是在当前神经影像技术出现之前的时代描述的，大多数作者认为每个病例都涉及相似的血管病变。本报告重点介绍了2例表现出相似胸部皮肤血管痣但脊髓血管病变明显不同的患者。一名29岁男性出现皮肤血管瘤以及下肢进行性轻瘫和感觉异常。他右中背部上方（T6 - 10）有一个20×20厘米的葡萄酒色斑，与磁共振成像精确对应，该成像显示T6至T10之间有一个强化的硬膜外肿块，导致脊髓受压和脊髓水肿。一名34岁男性也出现进行性脊髓病，在同一皮节区域有一个15×20厘米的葡萄酒色斑，伴有III型脊髓动静脉畸形。对每位患者的检查包括术前和术后的对比增强磁共振成像及血管序列检查和脊髓血管造影。第一名患者在T6 - 10水平接受了双侧椎板切除术，术后运动力量有显著改善。第二名患者接受了瘤巢内动脉瘤的弹簧圈栓塞术，并在8年后进行了随访栓塞。科布综合征在成人中是一种不寻常的病症，当出现一系列皮肤表现和潜在神经功能缺损时应予以考虑。与科布综合征相关的皮肤血管痣伴有多种血管病变。
