Variety of spinal vascular pathology seen in adult Cobb syndrome

Abstract

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.