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Cutaneous Ectopic Brain Clinical Presentation

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

History

Parents or physicians usually notice a cystic nodule at birth or shortly thereafter. A retrospectively review of the demographic, clinical, and histopathologic records of 11 infants with 12 heterotopic neural nodules of the scalp showed all lesions were located in the parietal or occipital region.[2] All but one had overlying alopecia surrounded by a ring of long, coarse hair (the hair collar sign), and 9 of 11 children had a capillary stain surrounding the lesion. Careful clinical evaluation and MRI of the brain should be performed prior to biopsy or excision of these scalp lesions.[3]

Heterotopic brain tissue is most often seen in the nasal region; other locations for ectopic brain tissue are less common, with a few observations on the face or neck.[4] Heterotopic brain tissue is rarely seen in the orbit. Orbital ectopic glial and glioneuronal brain tissue has been described.[5, 6] Heterotopic brain tissue has also been described in a cleft palate; this is a rare developmental anomaly.[7] Progressive proptosis in a neonate was described as a result of an ectopic cerebral rest in the orbit in the absence of a formed eye.[8]

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Physical

CEB is usually seen at birth as a 2-4 cm diameter, solitary, circular, bald scalp plaque or cyst, which may be compressible.

The plaque or cyst may be the color of skin, erythematous, or bluish, and tends to be on the midline occipital or parietal scalp.

A collar of hypertrophic hair may be evident surrounding the plaque or cyst (the hair collar sign[9, 10] ). A cutaneous marker for neural tube closure defects of the scalp (hair collar sign) consists of a ring of long, dark, coarse hair surrounding a midline scalp nodule. This sign should alert the physician to the possibility of ectopic neural tissue in the scalp or underlying central nervous system malformations.

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Causes

The cause is unknown. CEB may be an isolated embryonic rest or a congenital herniation through the skull with an eventual loss of connection.

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Complications

Caution is obligatory because skin biopsy or needle aspiration might lead to retrograde infection should the lesion communicate with the brain. If a congenital defect overlies a large blood vessel such as the sagittal sinus, removal of what appears to be a crust of dried serum may produce a fatal hemorrhage.

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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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  2. Rogers GF, Mulliken JB, Kozakewich HP. Heterotopic neural nodules of the scalp. Plast Reconstr Surg. 2005 Feb. 115(2):376-82. [Medline].

  3. Stevens CA, Galen W. The hair collar sign. Am J Med Genet A. 2008 Feb 15. 146(4):484-7. [Medline].

  4. Kurban Y, Sahin I, Uyar I, Deveci S, Gul D. Heterotopic brain tissue on the face and neck in a neonate: a rare case report and literature review. J Matern Fetal Neonatal Med. 2013 Apr. 26(6):619-21. [Medline].

  5. Ghose S, Balasubramaniam ST, Mahindrakar A, Sharma V, Sen S, Sarkar C, et al. Orbital ectopic glial tissue in relation to medial rectus: a rare entity. Clin Experiment Ophthalmol. 2005 Feb. 33(1):67-9. [Medline].

  6. Meyer P, Arnold Wörner N, Goldblum D, Bruder E. [Heterotopic glioneuronal brain tissue in the orbit: case report]. Klin Monbl Augenheilkd. 2013 Aug. 230(8):829-31. [Medline].

  7. Giannas JE, Bayat A, Davenport PJ. Heterotopic nasopharyngeal brain tissue associated with cleft palate. Br J Plast Surg. 2005 Sep. 58(6):862-4. [Medline].

  8. Grover AK, Chaudhuri Z, Popli J. Clinical anophthalmia with orbital heterotopic brain tissue. Ophthalmic Surg Lasers Imaging. 2007 Mar-Apr. 38(2):148-50. [Medline].

  9. Commens C, Rogers M, Kan A. Heterotropic brain tissue presenting as bald cysts with a collar of hypertrophic hair. The 'hair collar' sign. Arch Dermatol. 1989 Sep. 125(9):1253-6. [Medline].

  10. Drolet BA, Clowry L Jr, McTigue MK, Esterly NB. The hair collar sign: marker for cranial dysraphism. Pediatrics. 1995 Aug. 96(2 Pt 1):309-13. [Medline].

  11. Ramos L, Coutinho I, Cardoso JC, Garcia H, Cordeiro MR. Frontal cutaneous meningioma - Case report. An Bras Dermatol. 2015 Jun. 90 (3 Suppl 1):130-3. [Medline].

  12. Avecillas-Chasin JM, Saceda-Gutierrez J, Alonso-Lera P, Garcia-Pumarino R, Issa S, López E, et al. Scalp Metastases of Recurrent Meningiomas: Aggressive Behavior or Surgical Seeding?. World Neurosurg. 2015 Jul. 84 (1):121-31. [Medline].

  13. Held I, Rose C, Hamm H, Folster-Holst R. The hair collar sign - a possible indication of cranial dysraphism. J Dtsch Dermatol Ges. 2011 Feb. 9(2):136-8. [Medline].

  14. Ma C, Li X, Li Y, Qu X. Primary Ectopic Meningioma of the Tongue: Case Report and Review of the Literature. J Oral Maxillofac Surg. 2016 May 2. [Medline].

  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].

  17. Modarresifar H, Ho L. Brain heterotopia. Clin Nucl Med. 2009 Mar. 34(3):151-2. [Medline].

  18. Battistella M, Guedj N, Fallet-Bianco C, Bodemer C, Brousse N, Fraitag S. The histopathological spectrum of cutaneous meningeal heterotopias: clues and pitfalls. Histopathology. 2011 Sep. 59(3):407-20. [Medline].

 
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