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Cutaneomeningospinal angiomatosis
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Cutaneomeningospinal angiomatosis

Contributors: Lucy Ledyard MD, Keith Morley MD, Susan Burgin MD
Other Resources UpToDate PubMed

Synopsis

Cobb syndrome, also known as cutaneomeningospinal angiomatosis, is a rare sporadic condition characterized by meningeal or spinal arteriovenous malformations (AVMs) most commonly affecting the thoracic or lumbar spine with or without an overlying cutaneous vascular malformation in the same segment. The shared segmental distribution between the spinal vascular malformation and overlying skin findings is thought to be due to the embryologic origin of the blood supply to the vertebrae and spinal cord, which originates from the segmental dorsal arteries.

The vascular anomalies are present at birth. The cutaneous vascular malformation is typically a segmental capillary malformation (CM). Occasionally, there is thickening of the surface of the CM, which can mimic angiokeratomas. Angiolipomas and microcystic lymphatic malformations occurring near the CM have been described.

Neurological symptoms most commonly occur in late childhood to early adulthood but may present at any age. These symptoms are thought to be secondary to cord compression (and consequent ischemia) from the spinal vascular lesions, vascular steal syndrome, or venous hypertension. Symptoms range from weakness to monoparesis to full quadriplegia and can develop over the course of weeks to a year. Bowel and bladder incontinence is a common manifestation. Less frequently, headache, meningismus, and gluteal and limb hypertrophy have been reported.

Codes

ICD10CM:
Q87.89 – Other specified congenital malformation syndromes, not elsewhere classified

SNOMEDCT:
254774003 – Cobb's syndrome

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Last Reviewed:07/02/2020
Last Updated:07/27/2020
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Cutaneomeningospinal angiomatosis
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Cutaneomeningospinal angiomatosis
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