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A very rare syndrome characterized by association of four clinical features, including craniosynostosis, exostoses, nevus, and epibulbar dermoids.

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Craniosynostosis, Exostoses, Nevus, Epibulbar Dermoids Syndrome.

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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 epibulbar dermoids.

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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.

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