title: Dentomaxillofacial characteristics of ectodermal dysplasia.
abstract: The aim of this retrospective hospital-based study was to elucidate the dentomaxillofacial characteristics of ectodermal dysplasia. Six Japanese individuals (one male and five female; age range, 12.7-27.2 years) underwent comprehensive examinations, including history recording, cephalometric analysis, panoramic radiography, and analysis of dental models. All the subjects had two or more major manifestations for clinical diagnosis of ectodermal dysplasia (e.g., defects of hair, teeth, nails, and sweat glands). They presented hypodontia (mean number of missing teeth, 9.5; range, 5-14), especially in the premolar region, and enamel dysplasia. Five subjects had bilateral molar occlusion, whereas one subject had unilateral molar occlusion. The common skeletal features were small facial height, maxillary hypoplasia, counterclockwise rotation of the mandible, and mandibular protrusion. Interestingly, the maxillary first molars were located in higher positions and the upper anterior facial height was smaller than the Japanese norm. The results suggest that vertical and anteroposterior maxillary growth retardation, rather than lack of occlusal support due to hypodontia, leads to reduced anterior facial height in individuals with ectodermal dysplasia.
authors.affiliation: Maxillofacial Orthognathics, Department of Maxillofacial Reconstruction and Function, Division of Maxillofacial/Neck Reconstruction, Graduate School, Tokyo Medical and Dental University, Tokyo, Japan.
authors.forename: Yumiko
authors.initials: Y
authors.lastname: Nakayama
authors.forename: Yoshiyuki
authors.initials: Y
authors.lastname: Baba
authors.forename: Michiko
authors.initials: M
authors.lastname: Tsuji
authors.forename: Hiroki
authors.initials: H
authors.lastname: Fukuoka
authors.forename: Takuya
authors.initials: T
authors.lastname: Ogawa
authors.forename: Mizue
authors.initials: M
authors.lastname: Ohkuma
authors.forename: Keiji
authors.initials: K
authors.lastname: Moriyama
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D004476:Ectodermal Dysplasia / Q000000981:diagnostic imaging*; D005260:Female; D006801:Humans; D008297:Male; D019767:Maxillofacial Abnormalities / Q000000981:diagnostic imaging*; D011859:Radiography; D012189:Retrospective Studies; D014071:Tooth Abnormalities / Q000000981:diagnostic imaging*; D055815:Young Adult
keywords: ectodermal dysplasia; hypodontia; maxillary hypoplasia