Frontonasal encephalocele--"long nose hypertelorism".
Two varieties of frontonasal encephalocele are presented, with variations in the surgical technique necessitated by the different anatomical findings. The encephalocele pushes the nasal skeleton posteriorly and caudally and widens the distance between the medial orbital walls. This causes the long nose and the telecanthus. In this variety of frontonasal encephalocele, hypertelorism is uncommon. There may be absence of dura in relation to the encephalocele. The suggested method of correcting the deformity involves a combined intracranial and extracranial approach. The nasal skeleton is mobilized and placed in its correct position and the telecanthus is corrected. All skull defects are bone grafted. Correcting the deformity at an early age is recommended.
D002648:Child; D002675:Child, Preschool; D003394:Craniofacial Dysostosis / Q000601:surgery*; D004677:Encephalocele / Q000601:surgery*; D005004:Ethmoid Bone; D005624:Frontal Bone; D006801:Humans; D006972:Hypertelorism / Q000601:surgery*; D008297:Male; D009295:Nasal Bone; D009667:Nose Deformities, Acquired / Q000601:surgery*; D012225:Rhinoplasty; D013524:Surgical Flaps