Cobb syndrome Q87.8

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Angiomatosis cutaneo-meningo-spinal; Angiomatosis metamere; Cutaneo-meningo-spinal angiomatosis

History
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Cobb, 1915

Definition
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Neurocutaneous angiodysplasia syndrome: Combination of an angioma of the spinal cord vessels with a lateral flammeus nevus of the dermatome corresponding to the affected spinal cord segment. No familial occurrence.

Clinical features
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Since birth existing, 1 or 2 dermatomes of the following nevus flammeus lateralis. Neurological symptoms beginning in schoolchildren or adolescents in the form of sudden paralysis or progressive neurological deficits reminiscent of an intraspinal tumour.

Diagnosis
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X-ray of the spine, CT, NMR, selective angiography of the spinal vessels.

Therapy
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If possible, surgical removal of the angioma, see below nevus flammeus lateralis, cooperation with neurologists and neurosurgeons. Treatment of the flammeus nevus.

Literature
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  1. Clinton TS et al (2003) Cobb syndrome associated with a verrucous (angiokeratomalike) vascular malformation. Cutis 71: 283-287
  2. Cobb S (1915) Haemangioma of the spinal cord associated with skin naevi of the same metamere. Ann Surg 62: 641-649
  3. Jessen TR, Thompson S, Smith EB (1977) Cobb Syndrome. Arch Dermatol 113: 1587-1590
  4. Soeda A et al (2003) Cobb syndrome in an infant: treatment with endovascular embolization and corticosteroid therapy: case report. Neurosurgery 52: 711-715

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Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 29.10.2020