Cutaneous Ectopic Brain Clinical Presentation
- Author: Camila K Janniger, MD; Chief Editor: Dirk M Elston, MD more...
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. 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.
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. 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. 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.
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.
The cause is unknown. CEB may be an isolated embryonic rest or a congenital herniation through the skull with an eventual loss of connection.
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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