A very rare syndrome characterized by association of
four clinical features, including craniosynostosis, exostoses, nevus, and
Craniosynostosis, Exostoses, Nevus, Epibulbar Dermoids
Clinical features include mental retardation,
conductive deafness, craniofacial anomalies (frontal bossing, thickened
skull, sutural synostosis, facial structural asymmetry, flared nostrils,
broad nose), myopia, speech defect, multiple exostoses, pigmented nevi, and
Direct laryngoscopy and tracheal
intubation can be difficult because of malformation and has to be evaluated carefully
(clinical, radiograph). Careful intraoperative positioning is necessary.
Aminoglycosides should be used cautiously because of their otologic effects.