Cobb syndrome

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Classification according to ICD-10
Q27.3 Arteriovenous malformation of the peripheral vessels Arteriovenous aneurysm
ICD-10 online (WHO version 2019)

The Cobb syndrome (also cutaneous mengiospinal angiomatosis ; spinal arteriovenous metameric syndrome (SAMS 1-31)) is a very rare, congenital vascular malformation with a combined occurrence of venous or arterio-venous angiomas in the skin, muscles, bones and in the spinal cord. Occurrence is limited to a spinal “metameric” segment (a specific body section). While hemangiomas in the skin can be completely harmless, in this syndrome they are an indication of possible hidden lesions such as AV malformations in the spinal cord, which can lead to considerable damage with failures and paralysis.

The disease is named after the author of a report on this syndrome from 1915.

diagnosis

Clinically, the skin changes are slight, the deeper angiomas can only be detected by imaging using sonography , magnetic resonance imaging, including MR angiography . A detailed representation also for possible therapy planning takes place by means of a vascular representation, an angiography . Changes can already be present before birth and can be recorded sonographically.

Differential diagnosis

The following are to be distinguished:

therapy

Treatment options for intraspinal hemangiomas or AV malformations are mainly embolization and spinal decompression by laminectomy , possibly also the administration of corticosteroids .

Complications of the disease can be subacute necrotizing myelitis (Foix-Alajouanine syndrome) or thrombosis in the angiomas if the treatment is not timely or sufficiently effective .

Individual evidence

  1. ↑ S. Cobb: hemangioma OF THE SPINAL CORD: ASSOCIATED WITH SKIN nevi OF THE SAME Metameric. In: Annals of surgery. Volume 62, Number 6, December 1915, pp. 641-649, ISSN  0003-4932 . PMID 17863459 . PMC 1406820 (free full text).
  2. ↑ M. Jagła, I. Szymońska, P. Kruczek: Sonographic findings in a neonate with Cobb syndrome. In: Journal of clinical ultrasound: JCU. Volume 41, Number 4, May 2013, pp. 258-260, ISSN  1097-0096 . doi: 10.1002 / jcu.21892 . PMID 22331559 .
  3. ↑ A. Soeda, N. Sakai, K. Iihara, I. Nagata: Cobb syndrome in an infant: treatment with endovascular embolization and corticosteroid therapy: case report. In: Neurosurgery . Volume 52, Number 3, March 2003, pp. 711-715, ISSN  0148-396X . PMID 12590699 .
  4. ^ FP Wirth, KD Post, G. Di Chiro: Foix-Alajouanine disease. Spontaneous thrombosis of a spinal cord arteriovenous malformation: a case report. In: Neurology. 1970, vol. 20, pp. 1114-1118.

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