[Diagnostic and therapeutic problems in a clinical case of oculoauricular dysplasia associated with mandibule-facial dysostosis (Franceschetti-Goldenhar syndrome)].
The authors present a case of oculo-auricular dysplasia (type Goldenhar) associated with France-schetti's syndrome (mandibulo-facial dysostosis) and complicated by paralytic strabismus and genital malformations. Complete investigation failed to establish a precise aetiology; one suggests an external disturbance in the course of the organogenesis of the branchial arches. Aesthetic improvement was undertaken--removal of epibulbar dermoids, correction of the squint, and ablation of the preauricular appendages; functional orthodontic treatment continues.
D000328:Adult; D002675:Child, Preschool; D003884:Dermoid Cyst / Q000601:surgery; D005134:Eye Neoplasms / Q000601:surgery; D005260:Female; D006053:Goldenhar Syndrome / Q000150:complications* / Q000175:diagnosis / Q000628:therapy; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D008342:Mandibulofacial Dysostosis / Q000150:complications* / Q000175:diagnosis / Q000628:therapy; D008875:Middle Aged; D009971:Orthodontics, Corrective; D010375:Pedigree; D012878:Skin Neoplasms / Q000601:surgery
Problèmes de diagnostic et de traitement dans un cas clinique de dysplasie oculo-auriculaire associée à la dysostose mandibulo-faciale (syndrome de Franceschetti-Goldenhar).