A New Look at the Management of the Oculo-Mandibulo- Facial Syndrome

Abstract

The authors review the literature on the oculo-mandibulo-facial syndrome and present the case of a six-year-old boy with congenital cataracts, microphthalmos, nystagmus, failure to thrive, dysmorphic features with a tiny pinched nose, mandibular hypoplasia, microstomia, double chin, chronic snoring, recurrent respiratory infections and dental problems. Chronic obstructive sleep apnoea with decreased oxygen saturation was present. Optimal medical management of OMFS-patients is described.

Key Words:

• oculo-mandibulo-facial syndrome
• Hallermann-Streiff-François syndrome
• François syndrome
• dyscephaly
• oculomandibulodyscephaly
• mandibular hypoplasia
• chronic obstructive sleep apnoea
• rnicrophthalmos
• congenital cataracts
• dental anomalies
• alopecia