title: Orthodontic treatment of a patient with Stickler syndrome.
abstract: Stickler syndrome (MIM 108300, 604841, 184840) is an autosomal dominant disease characterized by midfacial flattening and variable disorders of vision, hearing and articulation. There are three types of the syndrome caused by mutations in different genes (type 1, COL2A1; type 2, COL11A1; and type 3, COL11A2). About 20% of type 1 patients have cleft palate or bifid uvula, but there have been no case reports of orthodontic treatment of this syndrome so far. The Japanese female patient presented here with Stickler syndrome was characterized by a flat midface and had high myopia, sensorineural hearing loss, enlarged joints, and cleft of the soft palate. She had fairly small SNA and SNB angles and a steep mandibular plane with an enlarged gonial angle. The incisors of both arches were retroclined, and a large overjet and overbite were noted. Orthodontic treatment was initiated at 11 years of age using a lingual arch appliance followed by an edgewise multibracket appliance. Stable functional occlusion was obtained after the treatment. Most of the other seven Stickler syndrome patients exhibited pretreatment characteristics of small SNA and SNB angles, steep mandibular planes, enlarged gonial angles, and retroclined incisors of both arches, demonstrating the characteristic skeletal and occlusal features of this syndrome.
authors: Department of Maxillofacial Reconstruction and Function, Graduate School, Tokyo Medical and Dental University, Japan. n-suda.mort@tmd.ac.jp
authors: Naoto
authors: N
authors: Suda
authors: Sachiko
authors: S
authors: Handa
authors: Norihisa
authors: N
authors: Higashihori
authors: Takuya
authors: T
authors: Ogawa
authors: Michiko
authors: M
authors: Tsuji
authors: Kimie
authors: K
authors: Ohyama
mesh_terms: D000015:Abnormalities, Multiple; D000848:Anodontia / Q000000981:diagnostic imaging / Q000235:genetics / Q000628:therapy; D002508:Cephalometry; D002648:Child; D004948:Esotropia; D005260:Female; D006319:Hearing Loss, Sensorineural; D006801:Humans; D007592:Joint Diseases; D008310:Malocclusion / Q000000981:diagnostic imaging / Q000235:genetics / Q000628:therapy*; D009971:Orthodontics, Corrective / Q000379:methods*; D011859:Radiography; D013577:Syndrome