Cobb Syndrome Clinical Presentation

  • Author: Stephen J Krivda, MD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Nov 21, 2011
 

History

Patients typically present with sudden onset of back or lower extremity radicular pain associated with numbness that can be localized below a specific dermatome. Less commonly, weakness or bowel/bladder dysfunction may be presenting symptoms.

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Physical

  • Cutaneous lesions may be distributed anywhere in the dermatome, from midline back to abdomen. See images below.Cutaneous angioma. Courtesy of L. Cooke, MD. Cutaneous angioma. Courtesy of L. Cooke, MD. Cutaneous angioma. Courtesy of L. Cooke, MD. Cutaneous angioma. Courtesy of L. Cooke, MD.
  • Midline back lesions, on rare occasions, are associated with spina bifida.
  • Unilateral lesions provide a clue to the location of the feeding artery in the spinal canal.
  • The cutaneous lesion may be very faint and may be more pronounced when the patient performs a Valsalva maneuver. The increased abdominal pressure causes preferential filling of the cutaneous angioma.
  • Neurological examination reveals weakness or paralysis and numbness or decreased sensation with a sharp upper cutoff.
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Causes

Although Cobb syndrome is thought to be noninherited, two case reports exist of inherited cutaneous angiomas in patients with Cobb syndrome. The forebears had no clinical evidence of spinal lesions. It has been postulated that Cobb syndrome, Wyburn-Mason syndrome, and PHACE (posterior fossa, hemangioma or other vascular birthmark present either on the outside or inside, arterial defect in the head and or neck area, cardiac problems, eye problems) syndrome are segmental vascular syndromes that differ only in the timing and target of an error in neural plate migration.[5]

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Contributor Information and Disclosures
Author

Stephen J Krivda, MD  Assistant Professor of Dermatology, Uniformed Services University of the Health Sciences; Chief of the Integrated Department of Dermatology, Chief of Dermatology Service, Director of Dermatopathology, Staff Dermatopathologist, Walter Reed Army Medical Center; Head, Department of Dermatology, Staff Dermatologist and Dermatopathologist, National Naval Medical Center

Stephen J Krivda, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, Association of Military Dermatologists, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Specialty Editor Board

Abby S Van Voorhees, MD  Assistant Professor, Director of Psoriasis Services and Phototherapy Units, Department of Dermatology, University of Pennsylvania School of Medicine, Hospital of the University of Pennsylvania

Abby S Van Voorhees, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, National Psoriasis Foundation, Phi Beta Kappa, Sigma Xi, and Women's Dermatologic Society

Disclosure: Amgen Honoraria Consulting; Abbott Honoraria Consulting; Merck Salary Management position; Abbott Honoraria Speaking and teaching; Amgen Honoraria Review panel membership; Centocor Honoraria Consulting; Leo Consulting; Merck None Other

David F Butler, MD  Professor of Dermatology, Texas A&M University College of Medicine; Chair, Department of Dermatology, Director, Dermatology Residency Training Program, Scott and White Clinic, Northside Clinic

David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Association of Military Dermatologists, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Rosalie Elenitsas, MD  Herman Beerman Associate Professor of Dermatology, University of Pennsylvania School of Medicine; Director, Penn Cutaneous Pathology Services, Department of Dermatology, University of Pennsylvania Health System

Rosalie Elenitsas, MD is a member of the following medical societies: American Academy of Dermatology and American Society of Dermatopathology

Disclosure: Lippincott Williams Wilkins Royalty Textbook editor; DLA Piper Consulting fee Consulting

Catherine M Quirk, MD  Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania

Catherine M Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD  Director, Ackerman Academy of Dermatopathology, New York

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous author, Christopher Norwood, MD, MS, to the development and writing of this article.

References

  1. Cobb S. Haemangioma of the spinal cord associated with skin naevi of the same metamere. Annals Surgery. 1915;62:641-9.

  2. Clinton TS, Cooke LM, Graham BS. Cobb syndrome associated with a verrucous (angiokeratomalike) vascular malformation. Cutis. Apr 2003;71(4):283-7. [Medline].

  3. Shim JH, Lee DW, Cho BK. A case of Cobb syndrome associated with lymphangioma circumscriptum. Dermatology. 1996;193(1):45-7. [Medline].

  4. Wirth FP, Post KD, Di Chiro G. Foix-Alajouanine disease. Spontaneous thrombosis of a spinal cord arteriovenous malformation: a case report. Neurology. 1970;20:1114-18.

  5. Krings T, Geibprasert S, Luo CB, Bhattacharya JJ, Alvarez H, Lasjaunias P. Segmental Neurovascular syndromes in children. Neuroimaging Clin N Am. May 2007;17:245-58. [Medline].

  6. Miyatake S, Kikuchi H, Koide T, Yamagata S, Nagata I, Minami S, et al. Cobb's syndrome and its treatment with embolization. Case report. J Neurosurg. Mar 1990;72(3):497-9. [Medline].

  7. Soeda A, Sakai N, Iihara K, Nagata I. Cobb syndrome in an infant: treatment with endovascular embolization and corticosteroid therapy: case report. Neurosurgery. Mar 2003;52(3):711-5; discussion 714-5. [Medline].

  8. Spiotta AM, Hussain MS, Masaryk TJ, Krishnaney AA. Combined endovascular and surgical resection of a giant lumbosacral arteriovenous malformation in a patient with Cobb syndrome. J Neurointerv Surg. Sep 2011;3(3):293-6. [Medline].

  9. Aminoff MJ, Logue V. Clinical features of spinal vascular malformations. Brain. Mar 1974;97(1):197-210. [Medline].

  10. Aminoff MJ, Logue V. The prognosis of patients with spinal vascular malformations. Brain. Mar 1974;97(1):211-8. [Medline].

  11. Brant AJ, James HE, Tung H. Cutaneomeningospinal angiomatosis (Cobb syndrome) with tethered cord. Pediatr Neurosurg. Feb 1999;30(2):93-5. [Medline].

  12. Doppman JL, Wirth FP Jr, Di Chiro G, Ommaya AK. Value of cutaneous angiomas in the arteriographic localization of spinal-cord arteriovenous malformations. N Engl J Med. Dec 25 1969;281(26):1440-4. [Medline].

  13. Jessen RT, Thompson S, Smith EB. Cobb syndrome. Arch Dermatol. Nov 1977;113(11):1587-90. [Medline].

  14. Kaplan P, Hollenberg RD, Fraser FC. A spinal arteriovenous malformation with hereditary cutaneous hemangiomas. Am J Dis Child. Dec 1976;130(12):1329-31. [Medline].

  15. Karshner R, Rand C, Reeves D. Epidural hemangioma associated with hemangioma of the vertebrae. Report of a case. Arch Surgery. 1939;39:942-51.

  16. Laredo JD, Reizine D, Bard M, Merland JJ. Vertebral hemangiomas: radiologic evaluation. Radiology. Oct 1986;161(1):183-9. [Medline].

  17. Mercer RD, Rothner AD, Cook SA, Alfidi RJ. The Cobb syndrome: association with hereditary cutaneous hemangiomas. Cleve Clin Q. 1978;45(2):237-40. [Medline].

  18. Wakabayashi Y, Isono M, Shimomura T, Tajima A, Terashi H, Asada Y, et al. Neurocutaneous vascular hamartomas mimicking Cobb syndrome. Case report. J Neurosurg. Jul 2000;93(1 Suppl):133-6. [Medline].

 
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Cutaneous angioma. Courtesy of L. Cooke, MD.
Cutaneous angioma. Courtesy of L. Cooke, MD.
Cutaneous angioma. Courtesy of L. Cooke, MD.
MRI of spinal angioma. Courtesy of L. Cooke, MD.
 
 
 
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