Cobb Syndrome, A Rare Disease – Case Report


  • Krushikumar Soladhra SVP hospital, Ahmedabad, India
  • Arvind Kumar Verma SVP hospital, Ahmedabad, India
  • Jaimin Modh SVP hospital, Ahmedabad, India
  • Dharmik Velani SVP hospital, Ahmedabad, India



Cobb, Spinal arteriovenous metameric syn-drome, Ulnar claw hand, Vertebral hemangioma, Spinal arteriovenous metameric syndrome


Cobb syndrome is a rare genetic disorder characterized by dermatomal pattern, with corresponding muscular, osseous, paraspinal, and/or spinal vascular lesions occurring at the same body somite (metamere). It is also known as spinal arteriovenous metameric syndrome (SAMS) and cutaneous meningospinal angiomatosis. We present a case of a 36 years old male presented to OPD with Complain of progressive weakness of Right upper limb for 5 years. On examination, Patient had large cutaneous port wine stain on the right side of the chest, nape of neck, and along the whole right upper limb in a dermatomal distribution with ulnar claw hand wasting of thenar muscles on right side. MRI cervical spine with contrast showed aggressive vertebral hemangioma involving anterior and posterior arch of C1 vertebra, vertebral body of C2 to C5, right half of vertebral body of C2 and C6 and right pedicle of C2 to C5 vertebra. Abnormal thickened and T2WI /STIR hyperintense skin and subcutaneous tissue involving right half of scalp and right half of neck which showing post contrast enhancement, possibility of subcutaneous hemangioma. Cobb syndrome was diagnosed based on the dermatomal distribution of the cutaneous vascular lesions and the corresponding vertebral, epidural, and paraspinal vascular lesions occurring at the same metamere. Treatment decision is guided by the patient’s symptoms and imaging features.


Elkordy A, Endo T, Sato K, Sonoda Y, Takahashi A, Tominaga T. Exclusively epidural spinal metameric arteriovenous shunts: case report and literature review. Spine J 2015; 15: e15-22. Doi: PMid:25450654

Cobb S. Haemangioma of the spinal cord: associated with skin naevi of the same metamere. Ann Surg 1915; 62: 641-9. Doi: PMid:17863459

Komiyama M, Ishiguro T, Terada A, Watanabe Y, Nakajima H, Ohata Y, et al.. Spinal arteriovenous metameric syndrome in a neonate presenting with congestive heart failure: case report. Childs Nerv Syst 2014, 30: 1607‐-11. Doi: PMid:24845229

Choi IS. Spinal arteriovenous metameric syndrome: angioar-chitecture and their prognosis. AJNR Am J Neuroradiol 2013; 34: 464-5. Doi: PMid:22936097

Pal P, Ray S, Chakraborty S, Dey S, Talukdar A. Cobb syn-drome: a rare cause of paraplegia. Ann Neurosci 2015; 22: 191-3. Doi: PMid:26130930

Nozaki T, Nosaka S, Miyazaki O, Makidono A, Yamamoto A, Niwa T, et al.. Syndromes associated with vascular tumors and malformations: a pictorial review. Radiographics 2013; 33: 175-95. Jan-Feb. Doi: PMid:23322836

Clinton TS, Cooke LM, Graham BS. Cobb syndrome associated with a verrucous (angiokeratomalike) vascular malformation. Cutis 2003; 71: 283-7. [ PubMed ] [ Google Scholar ]

Johnson WD, Petrie MM. Variety of spinal vascular pathology seen in adult cobb syndrome. J Neurosurg Spine 2009; 10: 430-5. Doi: PMid:19442004

Lee EJ, Kang SW, Shinn KS. Cobb's syndrome: a case report. J Korean Radiol Soc 1997; 36: 33-6. Doi:

Wan L, Ge W-R, Shi X-Y, Wang J, Hu L-Y, Zou L-P, et al. Cobb syndrome manifesting as repetitive seizures in a 10-year-old girl: a case report and literature review. Front Neurol 2019; 10: 1302. Doi: PMid:31866938

Saraf-Lavi E, Bowen BC, Quencer RM, Sklar EML, Holz A, Fal-cone S, et al.. Detection of spinal dural arteriovenous fistulae with MR imaging and contrast-enhanced Mr angiography: sensitivity, specificity, and prediction of vertebral level. AJNR Am J Neuroradiol 2002; 23: 858‐-67. [ PMC free article ] [ PubMed ] [ Google Scholar ]

Krings T, Geibprasert S. Spinal dural arteriovenous fistulas. AJNR Am J Neuroradiol 2009; 30: 639-48. Doi: PMid:19213818

Farb RI, Kim JK, Willinsky RA, Montanera WJ, terBrugge K, Derbyshire JA, et al.. Spinal dural arteriovenous fistula localiza-tion with a technique of first-pass gadolinium-enhanced MR angiography: initial experience . Radiology 2002; 222: 843-50. Doi: PMid:11867811

Gilbertson JR, Miller GM, Goldman MS, Marsh WR. Spinal du-ral arteriovenous fistulas: Mr and myelographic findings. AJNR Am J Neuroradiol 1995; 16: 2049-57. [ PMC free article ] [ PubMed ] [ Google Scholar ]

Miyatake S-ichi, Kikuchi H, Koide T, Yamagata S, Nagata I, Minami S-suke, et al.. Cobb's syndrome and its treatment with embolization. J Neurosurg 1990; 72: 497-9. Doi: PMid:2406383

Linfante I, Tari Capone F, Dabus G, Gonzalez-Arias S, Lau PE, Samaniego EA. Spinal arteriovenous malformation associated with spinal metameric syndrome: a treatable cause of long-term paraplegia? J Neurosurg Spine 2012; 16: 408‐-13. Doi: PMid:22225485

Soeda A, Sakai N, Iihara K, Nagata I. Cobb syndrome in an in-fant: treatment with endovascular embolization and cortico-steroid therapy: case report. Neurosurgery 2003; 52: 711-5. Doi: PMid:12590699




How to Cite

Soladhra, K., Verma, A. K., Modh, J., & Velani, D. (2024). Cobb Syndrome, A Rare Disease – Case Report. National Journal of Medical Research, 14(01), 35–40.



Case Report