Parents or physicians usually notice a cystic nodule at birth or shortly thereafter. The plaque or cyst may be skin colored, 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. CEB may be an isolated embryonic rest or a congenital herniation through the skull with an eventual loss of connection. Consider the possibility of a dermal sinus or a direct connection to the meninges or central nervous system when assessing congenital defects.
Encephaloceles usually are in the midline scalp, either hairless or with a collarette of hair.
The nasal glioma, like CEB, may have no connection to underlying central nervous system structures. It appears on the nose as a smooth, often polypoid, tumor. Caution is obligatory, since skin biopsy or needle aspiration might lead to retrograde infection should the lesion communicate with 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. The overlying epidermis in CEB is normal or somewhat atrophic due to the ectopic brain tissue impinging upon it.