Report of a case of Cobb syndrome: multimodality imaging

Abstract

Cobb syndrome is a rare vascular disorder characterized by vascular skin lesions distributed in a dermatomal pattern, with corresponding muscular, osseous, paraspinal, and/or spinal vascular lesions occurring at the same body somite (metamere). We present a case of a 25-year-old man who presented with a history of right upper limb paresthesia followed by bilateral progressive upper and lower limb weakness and heaviness. Physical examination showed large cutaneous port wine stains on the right side of the chest, the nape, and along the whole right upper limb in a dermatomal distribution, with no corresponding limb hypertrophy or asymmetry. MRI and CT scan of the cervical spine showed aggressive vertebral hemangiomas involving the right side of C1 down to C4 vertebrae associated with extraosseous epidural lesion causing cervical cord compression, in addition to right paraspinal muscular low flow vascular malformations. Digital subtraction angiography of the neck vessels showed corresponding vascular blush and delayed contrast pooling in the affected regions. Cobb syndrome was diagnosed based on the dermatomal distribution of the cutaneous vascular lesions and the corresponding vertebral, epidural, and paraspinal vascular lesions occurring at the same metamere. The patient underwent a decompressive laminectomy at C2–C6 levels with removal of the epidural lesion, after which his symptoms had improved.