title: Dentofacial growth in patients with Sotos syndrome.
abstract: Sotos syndrome is an overgrowth syndrome leading to peculiar facial characteristics, large hands and feet, and mental retardation. The maxillofacial characteristics are metopic protrusion, a high and narrow palate and a tapered mandible. In this study, we evaluated changes in maxillofacial growth in 2 patients with cerebral gigantism during the peripubertal period. Patient 1 was a boy aged 8 years at the first examination. The face showed midface retraction and a tapered mandible. Maxillary median diastema with an OJ of 2.5 mm and OB of 1.0 mm was observed, and the molar region showed mandibular mesial occlusion. Radiography revealed a lack of 15, 25, 37, 47, 14, 24, 34 and 44. Cephalometrics demonstrated maxillary and mandibular retrusion with an SNA of 68 degrees and an SNB of 70 degrees , and the patient had leptoprosopia with a mandibular plane of 38.0 degrees . This plane was 45 degrees at the time of re-examination when the patient was 14 years old, showing an increase in the lower facial height and decreases in facial axis and depth. Patient 2 was a boy aged 14 years at the first examination. The face showed mandibular retrusion and tapering. The occlusion was angle class II div. 1, OJ 14 mm, and OB -1 mm. Cephalometrics demonstrated maxillary and mandibular retrusion with an SNA of 74.5 degrees and an SNB of 69.5 degrees , and the patient had leptoprosopia with a mandibular plane of 37.0 degrees . At the time of re-examination, when the patient was 16 years old, the mandibular plane was 42.5 degrees , showing an increase in lower facial height and decreases in facial axis and depth. In this syndrome, excessive facial height without mandibular forward overgrowth is observed. Since the facial height tended to increase by growth during the peripubertal period, maxillofacial vertical growth is considered important in the treatment of this syndrome.
authors.affiliation: Division of Orthodontics, Department of Clinical Oral Health Science, Tokyo Dental College, Tokyo, Japan.
authors.forename: Kunio
authors.initials: K
authors.lastname: Takei
authors.forename: Kenji
authors.initials: K
authors.lastname: Sueishi
authors.forename: Hideharu
authors.initials: H
authors.lastname: Yamaguchi
authors.forename: Yumi
authors.initials: Y
authors.lastname: Ohtawa
mesh_terms: D000293:Adolescent; D001921:Brain / Q000002:abnormalities*; D002508:Cephalometry; D002648:Child; D019465:Craniofacial Abnormalities / Q000503:physiopathology*; D003970:Diastema / Q000209:etiology; D019066:Facies; D005500:Follow-Up Studies; D006130:Growth Disorders / Q000503:physiopathology*; D006801:Humans; D008297:Male; D008310:Malocclusion / Q000209:etiology; D008334:Mandible / Q000002:abnormalities; D008437:Maxilla / Q000002:abnormalities; D008445:Maxillofacial Development / Q000502:physiology*; D010159:Palate / Q000002:abnormalities; D013577:Syndrome; D014716:Vertical Dimension