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.

Report of a case of Cobb syndrome: multimodality imaging

“…The dermatomal distribution of the cutaneous lesions is important, as this may raise the suspicion of Cobb syndrome. 5 The deep vascular lesions occurring at the same body metamere can be within the spinal cord itself (intramedullary), outside the cord (intraspinal extramedullary), vertebral or extraspinal, including the paraspinal soft tissues. 5 Under the new International Society for the Study of Vascular Anomalies classification system, vascular lesions which were previously described as Cobb syndrome would now be better characterized as vascular malformations rather than true hemangiomas.…”

“…5 The deep vascular lesions occurring at the same body metamere can be within the spinal cord itself (intramedullary), outside the cord (intraspinal extramedullary), vertebral or extraspinal, including the paraspinal soft tissues. 5 Under the new International Society for the Study of Vascular Anomalies classification system, vascular lesions which were previously described as Cobb syndrome would now be better characterized as vascular malformations rather than true hemangiomas. Cobb syndrome is usually associated with spinal high-flow vascular malformations; spinal arteriovenous vascular malformations are classified into four subtypes, Cobb Syndrome is associated with Type 3 arteriovenous malformations.…”

Pooling of lupus can "catch" early skin cancerous: "Rapid Progression in DNA Damage"

“…By Duplex ultrasonography as arteriovenous malformations (AVMs) were not available, Parkes Weber's syndrome was excluded. As the baby had not spinal or paraspinal (AVMs), seizure or scoliosis, CLOVE syndrome (Congenital lipomatous overgrowth, vascular malformations, and epidermal nevi) and Cobb syndrome were ruled out [5,6]. The clinical picture of the baby did not match the diagnosis of Sturge Weber syndrome as there was not facial port wine stain and brain ultrasound was normal [7].…”

Cobb syndrome (cutaneomeningospinal angiomatosis)

“…The patient was diagnosed with Cobb syndrome, a rare congenital condition characterised with arteriovenous malformations (AVMs) and vascular lesions in the same dermatome with associated spinal angiomas or AVMs 1. Treatment considerations centre on preventing or treating neurological symptoms.…”