Cobb syndrome: A rare cause of paraplegia

Abstract

Cobb syndrome is an exceedingly rare clinical condition defined by the presence of a vascular skin nevus and an angioma in the spinal canal at the same metamere. We report the case of a 14-year-old boy who presented with sudden onset paraplegia. Physical examination showed port-wine stains over buttock and thigh. Magnetic resonance (MR) angiogram of the dorso-lumbar spine revealed a large arteriovenous malformation (AVM) at D11-D12 to L2-L3 levels. These concurrent findings led to the diagnosis of Cobb's syndrome. The patient received orally administered prednisolone therapy and underwent endovascular embolization of spinal angioma. Cobb's syndrome is a rare disease entity and literature search revealed only a few case reports and series mentioning this condition to date. The importance lies in the recognition that cutaneous vascular lesions may clue to an associated spinal cord angioma or AVM that may lead to weakness or paralysis.

Keywords: Cobb syndrome; arteriovenous malformation; embolization; magnetic resonance angiogram; spine; surgery.

Fig. 1:

Large port-wine stains on his buttock (A) and over the anterior aspect of right thigh (B).

Fig. 2:

Magnetic resonance imaging scan of dorso-lumbar spine. T2 weighted sagittal imaging: (1 = Lower dorsal and upper lumbar cords showing heterogenous T2 hyperintensities suggestive of developing syrinx; 2 = Vascular flow voids on ventral aspect of thecal sac). Conus appears to be low down and expanded with internal heterogenous signal intensity (3 = Resolving cord hematoma.).

Fig. 3:

(A). MR angiogram (MIP image) showing a dilated and tortuous feeding artery of the AVM arising from lower intercostal arteries (yellow arrow); (B). Axial fat-suppressed post-contrast T1 weighted image showing multiple intra thecal dilated vessels (yellow arrow) situated both dorsal and ventral to the cauda and conus.