Teaching NeuroImage: Clinical and Imaging Features of Cobb Syndrome

A 17-year-old boy with typical neurodevelopment presented with 3 months of lower-limb weakness without a history of trauma. Physical examination, plain radiography and a T2-weighted MRI revealed a port-wine stain over the lumbar region, significant scoliosis below the cervical segment, and a multifocal flow void sign (steal phenomenon) from epidural space to dorso-lumbar spine, respectively (Figure 1). CT angiography identified an enhancing dorsal vascular mass with involvement of the spinal canal including destruction of the vertebral body determined to be extensive arteriovenous shunts on digital subtraction angiography (DSA) (Figure 2).