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Cutaneous Ectopic Brain

  • Author: Camila K Janniger, MD; Chief Editor: Dirk M Elston, MD  more...
 
Updated: Jun 24, 2016
 

Background

Extracranial brain tissue without a direct connection to the brain itself may be an isolated cutaneous embryonic defect, usually on occipital or parietal areas of the scalp. It is also known as heterotopic brain tissue or cutaneous ectopic brain (CEB).

Lee and Mclaurin[1] described CEB in 1955 in a 1-year-old girl with a flat, almost perfectly circular, bluish-red plaque approximately 3 cm in diameter on the posterior midline of her scalp. Microscopically, heterotopic glial tissue in a pattern suggestive of abortive gyri and sulci was evident within the dermis. Additional patients with CEB have since been described.

Heterotopic brain tissue is a rare developmental abnormality that usually has no effect on neurological development.

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Pathophysiology

CEB may be an isolated embryonic rest or a congenital herniation through the skull with an eventual loss of connection. Perhaps the neural tube initially overgrows, preventing closure of the cranial or spinal coverings. Thus, its pathogenesis is uncertain.

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Epidemiology

Many congenital cutaneous disorders of the scalp have been described, all of which are quite uncommon. CEB is less common than encephalocele.

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Prognosis

The prognosis for patients with cutaneous ectopic brain (CEB) generally is good because no communication exists with underlying structures and few, if any, serious associated anomalies occur.

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Contributor Information and Disclosures
Author

Camila K Janniger, MD Clinical Professor of Dermatology, Clinical Associate Professor of Pediatrics, Chief of Pediatric Dermatology, Rutgers New Jersey Medical School

Camila K Janniger, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Specialty Editor Board

David F Butler, MD Section Chief of Dermatology, Central Texas Veterans Healthcare System; Professor of Dermatology, Texas A&M University College of Medicine; Founding Chair, Department of Dermatology, Scott and White Clinic

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

Disclosure: Nothing to disclose.

Robert A Schwartz, MD, MPH Professor and Head of Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, Rutgers New Jersey Medical School; Visiting Professor, Rutgers University School of Public Affairs and Administration

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, New York Academy of Medicine, American Academy of Dermatology, American College of Physicians, Sigma Xi

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

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

Disclosure: Nothing to disclose.

Additional Contributors

Albert C Yan, MD Section Chief, Associate Professor, Department of Pediatrics, Section of Dermatology, Children's Hospital of Philadelphia and University of Pennsylvania School of Medicine

Albert C Yan, MD is a member of the following medical societies: American Academy of Dermatology, Society for Investigative Dermatology, Society for Pediatric Dermatology, American Academy of Pediatrics

Disclosure: Nothing to disclose.

References
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  15. Altissimi G, Ascani S, Falcetti S, Cazzato C, Bravi I. Central nervous system tissue heterotopia of the nose: case report and review of the literature. Acta Otorhinolaryngol Ital. 2009 Aug. 29(4):218-21. [Medline]. [Full Text].

  16. Ali MJ, Kamal S, Vemuganti GK, Naik MN. Glial Heterotopia or Ectopic Brain Masquerading as a Dacyrocystocele. Ophthal Plast Reconstr Surg. 2014 Jan 31. [Medline].

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