title: Dental and maxillofacial characteristics of six Japanese individuals with ectrodactyly-ectodermal dysplasia-clefting syndrome.
abstract: Objective : Ectrodactyly-ectodermal dysplasia-clefting syndrome is a congenital anomaly characterized by ectodermal dysplasia, ectrodactyly, cleft lip and palate, and lacrimal duct anomalies. Because this syndrome is frequently accompanied by a congenital lack of teeth, narrow palate, and malocclusion, comprehensive orthodontic intervention is required. Design : To highlight the specific dental and maxillofacial characteristics of ectrodactyly-ectodermal dysplasia-clefting syndrome, six Japanese individuals diagnosed with the syndrome are described here. Patients : The subjects consisted of two boys and four girls (age range, 6.0 to 13.9 years) diagnosed with ectrodactyly-ectodermal dysplasia-clefting syndrome by medical and dental specialists. Their conditions included ectodermal dysplasia (hypodontia, microdontia, enamel hypoplasia, and abnormalities in hair and nails), cleft lip and/or palate, and ectrodactyly. Cephalograms, panoramic x-rays, and dental casts were taken; systemic complications were recorded at the first visit to our dental hospital. Results : All individuals had severe oligodontia with 9 to 18 missing teeth. The missing teeth were mainly maxillary and mandibular incisors and second bicuspids, arranged in a symmetrical manner. Cephalometric analysis showed retruded and short maxilla due to cleft lip and/or palate. It is interesting that all individuals showed a characteristically shaped mandibular symphysis with a retruded point B. It is likely that this unusual symphyseal morphology is due to the lack of mandibular incisors. Conclusions : This study demonstrates the presence of severe oligodontia in the incisal and premolar regions and describes a characteristic maxillary and mandibular structure in Japanese individuals with ectrodactyly-ectodermal dysplasia-clefting syndrome.
authors.forename: Erika
authors.initials: E
authors.lastname: Okamura
authors.forename: Naoto
authors.initials: N
authors.lastname: Suda
authors.forename: Yoshiyuki
authors.initials: Y
authors.lastname: Baba
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: Nozomi
authors.initials: N
authors.lastname: Ahiko
authors.forename: Akihiro
authors.initials: A
authors.lastname: Yasue
authors.forename: Toshimoto
authors.initials: T
authors.lastname: Tengan
authors.forename: Momotoshi
authors.initials: M
authors.lastname: Shiga
authors.forename: Michiko
authors.initials: M
authors.lastname: Tsuji
authors.forename: Keiji
authors.initials: K
authors.lastname: Moriyama
mesh_terms: D000848:Anodontia; D002508:Cephalometry; D004476:Ectodermal Dysplasia; D006801:Humans; D013577:Syndrome