Cobb syndromeQ27.3

Author:Prof. Dr. med. Peter Altmeyer

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Last updated on: 28.11.2022

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Synonym(s)

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

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HistoryThis section has been translated automatically.

Cobb, 1915

DefinitionThis section has been translated automatically.

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.

Occurrence/EpidemiologyThis section has been translated automatically.

So far, < 150 cases have been described in the literature. Both sexes are equally frequently affected.

EtiopathogenesisThis section has been translated automatically.

The syndrome is not clustered in affected families, and chromosomal abnormalities have not been described. The beginnings of the disease are in early embryogenesis, before migration of vascular precursor cells to their definitive segments (skin, bone, peripheral nerves, spinal cord). When two consecutive segments are affected, a multimetameric form of the disease results.

Clinical featuresThis section has been translated automatically.

Since birth existing, 1 or 2 dermatomas following nevus flammeus lateralis. Neurological symptoms beginning in schoolchildhood or adolescence in the form of sudden onset of paralysis or progressive neurological deficits, reminiscent of an intraspinal tumor. Of note is the association of Cobb syndrome and segmental neurofibromatosis (NF1) (Pascual-Castroviejo I et al. 2008).

DiagnosisThis section has been translated automatically.

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

TherapyThis section has been translated automatically.

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

Note(s)This section has been translated automatically.

In a recent analysis of the syndrome, the designation Spinal Arterio-venous Metameres Syndrome 1-31 (SAMS 1-31)

LiteratureThis section has been translated automatically.

  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

Authors

Last updated on: 28.11.2022

Author: Prof. Dr. med. Peter Altmeyer