SPINAL ARTERIOVENOUS METAMERIC SYNDROME (SAMS) / COBB SYNDROME.

Warda Sattar, Shazia Kadri, Rab Nawaz

Abstract


 

ABSTRACT:

Spinal arteriovenous metameric syndrome (SAMS), also known as Cobb syndrome, is a rare embryonic metameric syndrome, in which cutaneous, muscular, and or bony vascular lesions as well as para spinal and or spinal vascular lesions are found in the same metamere. Less than 80 cases with this syndrome have been reported in the literature and only one case was reported from Indian subcontinent. Classically, a birthmark (a cutaneous venocapillary malformation) and a spinal lesion (arteriovenous malformation or arteriovenous fistula) are found in the same or neighboring metameres.  It should alert the physician and radiologist for early imaging of spine as early diagnosis and timely intervention would be favorable and may save patient from permanent neurological sequelae.

KEYWORDS:

Spinal arteriovenous metameric syndrome (SAMS), Cobb syndrome, pigmented cutaneous nevus, lymphangiomacircumscriptum, neurocutaneous disorder.


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References


REFRENCE:

Edelstein S1, Naidich TP, Newton T. The rare phakomatoses. Neuroimaging Clin N Am. 2004;14:185–217.

Pascual-Castroviejo I, Di Rocco C, editors. Neurocutaneous disorders phakomatoses and hamartoneoplastic syndromes. Wien: Springer; 2008.

Nandigam K, Mechtler L, Smirniotopoulos J. Neuroimaging of neurocutaneous diseases. NeurolClin. 2014;32:159–92.

Abdel Razek AA. Vascular neurocutaneous disorders: neurospinal and craniofacial imaging findings. Jpn J Radiol (2014) 32:519–528. PMID: 25078149

Krings T, Geibprasert S, Luo CB, Bhattacharya JJ, Alvarez H, Lasjaunias P. Segmental neurovascular syndromes in children. Neuroimaging Clin N Am 2007;17(2):245–258.

Bhattacharya JJ, Luo CB, Suh DC, Alvarez H, Rodesch G, Lasjaunias P. Wyburn-Mason or Bonnet-Dechaume-Blanc as cerebrofacial arteriovenous metameric syndromes (CAMS): a new concept and a new classification. IntervNeuroradiol 2001;7(1):5–17.

MatsumaruY,PongpechS,LaothamasJ,etal.Multifocalandmetamericspinal cord arteriovenous malformations: review of 19 cases. IntervNeuroradiol 1999;5:27–34

Spetzler RF, Detwiler PW, Riina HA, et al. Modified classification of spinal cord vascular lesions. J Neurosurg 2002;96:145–56

Dilme-Carreras, E., Iglesias-Sancho, M., Marquez-Balbas, G., Sola-Ortigosa, J., &Umbert-Millet, P. (2010). Cobb syndrome: case report and review of the literature. Dermatology, 221(2), 110-112. doi: 10.1159/000313510

Komiyama M1, Ishiguro T, Terada A, Watanabe Y, Nakajima H, Ohata Y, Matsusaka Y. Spinal arteriovenous metameric syndrome in a neonate presenting with congestive heart failure: case report. Childs NervSyst 2014 Sep;30(9):1607-11. PMID: 24845229

Sardana K, Kabir, Sehgal, Virendra N., Mahajan, Supriya, &Bhushan, Premanshu. Cobb Syndrome in an Indian Girl. Skinmed, 2006 Jan-Feb;5(1):51-3, PMID: 16522989

Berenstein A, Lasjaunias P, terBrugge K. Spinalarteriovenousmalformations. In:Berenstein A, LasjauniasP,terBrugge K. SurgicalNeuroangiography.2nded. Berlin, Germany: Springer-Verlag; 2004:737–847

Niimi, Y., et al. (2013). Spinal arteriovenous metameric syndrome: clinical manifestations and endovascular management. AJNR Am J Neuroradiol 34(2): 457-463, PMID: 22859288.

Shim JH, Lee DW, Cho BK. A case of Cobb syndrome associated with lymphangiomacircumscriptum.Dermatology. 1996;193:45–7. [PubMed]

Maramattom BV, Cohen-Gadol AA, Wijdicks EF. et al. Segmental cutaneous hemangioma and spinal arteriovenous malformation (Cobb syndrome). Case report and historical perspective. J Neurosurg Spine.2005;3:249–52. [PubMed]

Miyatake S, Kikuchi H, Koide T. et al. Cobb’s syndrome and its treatment with embolization. J Neurosurg. 1990;72:497–9. [PubMed]

Mulliken JB, Glowacki J (1982) Hemangiomas and vascular malformations in infants and children: a classification based on endothelial characteristics. PlastReconstrSurg 69:412–420

Pal P, Ray S, Chakraborty S,Dey S and Talukdar A. Cobb syndrome: A rare cause of paraplegia. Ann Neurosci. 2015 Jul; 22(3): 191–193


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