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Cobb syndrome

MedGen UID:
91079
•Concept ID:
C0346068
•
Disease or Syndrome
Synonyms: Cobb Syndrome; Cobb's Syndrome; Cobb's syndrome; Cutaneomeningospinal angiomatosis; cutaneomeningospinal angiomatosis; SAMS 1-31; spinal arteriovenous metameric syndrome; Spinal arteriovenous metameric syndrome
SNOMED CT: Cobb syndrome (254774003); Cobb's syndrome (254774003)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0018893
Orphanet: ORPHA53721

Definition

Cobb syndrome is defined by the association of vascular cutaneous (venous or arteriovenous), muscular (arteriovenous), osseous (arteriovenous) and medullary (arteriovenous) lesions at the same metamere or spinal segment. This segmental distribution may involve one or many of the 31 metameres present in humans. Only 16% of the medullary lesions are multiple and have a clearly metameric distribution. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCobb syndrome
Follow this link to review classifications for Cobb syndrome in Orphanet.

Professional guidelines

PubMed

Diagnosis and endovascular treatment of pediatric spinal arteriovenous shunts.
Cullen S, Krings T, Ozanne A, Alvarez H, Rodesch G, Lasjaunias P
Neuroimaging Clin N Am 2007 May;17(2):207-21. doi: 10.1016/j.nic.2007.02.004. PMID: 17645971

Recent clinical studies

Etiology

Angiokeratoma Circumscriptum Naeviforme Presenting as a Dark Warty Plaque on the Leg.
Abbenante D, Raone B, Baraldi C, Anna Carpanese M, Patrizi A
Acta Dermatovenerol Croat 2021 Dec;29(3):169-170. PMID: 34990348
Cutaneomeningospinal Angiomatosis (Cobb Syndrome) in a Young Patient.
Putilina M, Teplova N, Dvornikov A
CNS Neurol Disord Drug Targets 2021;20(10):888-893. doi: 10.2174/1871527320666210218083550. PMID: 33602108
Obliteration of a metameric spinal arteriovenous malformation (Cobb syndrome) using combined endovascular embolization and surgical excision.
Schirmer CM, Hwang SW, Riesenburger RI, Choi IS, David CA
J Neurosurg Pediatr 2012 Jul;10(1):44-9. Epub 2012 Jun 15 doi: 10.3171/2012.3.PEDS11530. PMID: 22702329
Spinal arteriovenous malformation associated with spinal metameric syndrome: a treatable cause of long-term paraplegia?
Linfante I, Tari Capone F, Dabus G, Gonzalez-Arias S, Lau PE, Samaniego EA
J Neurosurg Spine 2012 Apr;16(4):408-13. Epub 2012 Jan 6 doi: 10.3171/2011.12.SPINE11636. PMID: 22225485
Diagnosis and endovascular treatment of pediatric spinal arteriovenous shunts.
Cullen S, Krings T, Ozanne A, Alvarez H, Rodesch G, Lasjaunias P
Neuroimaging Clin N Am 2007 May;17(2):207-21. doi: 10.1016/j.nic.2007.02.004. PMID: 17645971

Diagnosis

Cutaneomeningospinal Angiomatosis (Cobb Syndrome) in a Young Patient.
Putilina M, Teplova N, Dvornikov A
CNS Neurol Disord Drug Targets 2021;20(10):888-893. doi: 10.2174/1871527320666210218083550. PMID: 33602108
Cobb syndrome: case report and review of the literature.
Dilmé-Carreras E, Iglesias-Sancho M, Márquez-Balbás G, Sola-Ortigosa J, Umbert-Millet P
Dermatology 2010;221(2):110-2. Epub 2010 Jun 8 doi: 10.1159/000313510. PMID: 20805687
Cobb syndrome: a case report and systematic review of the literature.
Clark MT, Brooks EL, Chong W, Pappas C, Fahey M
Pediatr Neurol 2008 Dec;39(6):423-5. doi: 10.1016/j.pediatrneurol.2008.08.001. PMID: 19027589
Diagnosis and endovascular treatment of pediatric spinal arteriovenous shunts.
Cullen S, Krings T, Ozanne A, Alvarez H, Rodesch G, Lasjaunias P
Neuroimaging Clin N Am 2007 May;17(2):207-21. doi: 10.1016/j.nic.2007.02.004. PMID: 17645971
Cobb syndrome.
Jessen RT, Thompson S, Smith EB
Arch Dermatol 1977 Nov;113(11):1587-90. PMID: 931402

Therapy

Monitoring Arteriovenous Malformation Response to Genotype-Targeted Therapy.
Edwards EA, Phelps AS, Cooke D, Frieden IJ, Zapala MA, Fullerton HJ, Shimano KA
Pediatrics 2020 Sep;146(3) doi: 10.1542/peds.2019-3206. PMID: 32859736
Anesthetic Considerations in Cobb Syndrome: A Case Report.
Minagar M, Dehghan-Tezerjani M, Khan ZH
A A Pract 2019 Aug 15;13(4):137-139. doi: 10.1213/XAA.0000000000001016. PMID: 30973350
Cobb syndrome in an infant: treatment with endovascular embolization and corticosteroid therapy: case report.
Soeda A, Sakai N, Iihara K, Nagata I
Neurosurgery 2003 Mar;52(3):711-5; discussion 714-5. doi: 10.1227/01.neu.0000048483.21777.b7. PMID: 12590699

Prognosis

Spinal arteriovenous malformation associated with spinal metameric syndrome: a treatable cause of long-term paraplegia?
Linfante I, Tari Capone F, Dabus G, Gonzalez-Arias S, Lau PE, Samaniego EA
J Neurosurg Spine 2012 Apr;16(4):408-13. Epub 2012 Jan 6 doi: 10.3171/2011.12.SPINE11636. PMID: 22225485
COBB syndrome treated by staged intravascular embolisation and surgery.
Sayuthi S, Moret J, Pany A, Sobri A, Shafie M, Abdullah J
Med J Malaysia 2006 Jun;61(2):239-41. PMID: 16898321

Clinical prediction guides

Classification of spinal cord arteriovenous shunts: proposal for a reappraisal--the Bicêtre experience with 155 consecutive patients treated between 1981 and 1999.
Rodesch G, Hurth M, Alvarez H, Tadié M, Lasjaunias P
Neurosurgery 2002 Aug;51(2):374-9; discussion 379-80. PMID: 12182775
Neurocutaneous vascular hamartomas mimicking Cobb syndrome. Case report.
Wakabayashi Y, Isono M, Shimomura T, Tajima A, Terashi H, Asada Y, Izumi T, Hori S
J Neurosurg 2000 Jul;93(1 Suppl):133-6. doi: 10.3171/spi.2000.93.1.0133. PMID: 10879770

Recent systematic reviews

Cobb syndrome: case report and review of the literature.
Dilmé-Carreras E, Iglesias-Sancho M, Márquez-Balbás G, Sola-Ortigosa J, Umbert-Millet P
Dermatology 2010;221(2):110-2. Epub 2010 Jun 8 doi: 10.1159/000313510. PMID: 20805687
Cobb syndrome: a case report and systematic review of the literature.
Clark MT, Brooks EL, Chong W, Pappas C, Fahey M
Pediatr Neurol 2008 Dec;39(6):423-5. doi: 10.1016/j.pediatrneurol.2008.08.001. PMID: 19027589

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