title: Patient with oculo-facio-cardio-dental syndrome treated with surgical orthodontics.
abstract: Oculo-facio-cardio-dental (OFCD) syndrome is a rare syndrome characterized by ocular, facial, cardiac, and dental disorders. Only about 20 cases have been reported to date. The most prominent of the various features of this syndrome is canine radiculomegaly. Other features include a long and narrow face, a high nasal bridge, a broad and pointed nose, a bifid nose, ear deformity, cleft palate or submucous cleft palate, maxillary growth retardation, a large gonial angle, open apices, delayed eruption, persistent deciduous teeth, extreme overbite, and constricted maxilla. Orthodontic and prosthodontic treatment has been reported for several patients, but surgical-orthodontic treatment for OFCD has not been reported. An 18-year-old woman with skeletal Class III malocclusion and OFCD syndrome was treated with edgewise appliance therapy combined with orthognathic surgery. We applied a light force during the treatment so as not to induce ankylosis. At the end of the surgical and orthodontic treatments, functional occlusion and an improved facial profile were achieved. After the retention period, stomatognathic function was improved. The results of this treatment suggest that surgical-orthodontic treatment is an effective method for improving skeletal disharmony, facial profile, occlusion, and stomatognathic function in patients with OFCD.
authors.affiliation: Department of Orthodontics, Field of Developmental Medicine, Health Research Course, Graduate School of Medical and Dental Sciences, Kagoshima University, Kagoshima, Japan.
authors.forename: Katsuyoshi
authors.initials: K
authors.lastname: Sakaguchi
authors.forename: Takakazu
authors.initials: T
authors.lastname: Yagi
authors.forename: Junko
authors.initials: J
authors.lastname: Nagata
authors.forename: Takeshi
authors.initials: T
authors.lastname: Kubota
authors.forename: Kazumasa
authors.initials: K
authors.lastname: Sugihara
authors.forename: Shouichi
authors.initials: S
authors.lastname: Miyawaki
mesh_terms: D000293:Adolescent; D001641:Bicuspid / Q000002:abnormalities; D001921:Brain / Q000002:abnormalities / Q000503:physiopathology / Q000601:surgery; D002386:Cataract / Q000503:physiopathology / Q000628:therapy / Q000151:congenital; D002508:Cephalometry; D003481:Cuspid / Q000002:abnormalities; D005260:Female; D006330:Heart Defects, Congenital / Q000503:physiopathology / Q000601:surgery* / Q000628:therapy; D006343:Heart Septal Defects; D006801:Humans; D008310:Malocclusion / Q000628:therapy; D008313:Malocclusion, Angle Class III / Q000601:surgery / Q000628:therapy*; D008409:Mastication; D008850:Microphthalmos / Q000503:physiopathology / Q000601:surgery* / Q000628:therapy; D009811:Odontometry; D009971:Orthodontics, Corrective; D059229:Osteotomy, Sagittal Split Ramus; D010155:Palatal Expansion Technique; D014071:Tooth Abnormalities; D014092:Tooth Root / Q000002:abnormalities*; D055815:Young Adult