title: Evaluation of the maxillofacial morphological characteristics of Apert syndrome infants.
abstract: Apert syndrome is a rare craniosynostosis syndrome characterized by irregular craniosynostosis, midface hypoplasia, and syndactyly of the hands and feet. Previous studies analyzed individuals with Apert syndrome and reported some facial and intraoral features caused by severe maxillary hypoplasia. However, these studies were performed by analyzing both individuals who had and those had not received a palate repair surgery, which had a high impact on the maxillary growth and occlusion. To highlight the intrinsic facial and intraoral features of Apert syndrome, five Japanese individuals with Apert syndrome from 5 years and 2 months to 9 years and 10 months without cleft palate were analyzed in this study. A concave profile and a skeletal Class III jaw-base relationship caused by severe maxillary hypoplasia were seen in all patients. The patients exhibited anterior and posterior crossbites possibly due to a small dental arch of Maxilla.
authors.affiliation: Clinical Department of Orthodontics, Hokkaido University Hospital, Sapporo, Japan.
authors.forename: Hitomi
authors.initials: H
authors.lastname: Kakutani
authors.affiliation: Department of Orthodontics, Division of Oral Functional Science, Graduate School of Dental Medicine, Hokkaido University, Sapporo, Japan.
authors.forename: Yoshiaki
authors.initials: Y
authors.lastname: Sato
authors.affiliation: Clinical Department of Orthodontics, Hokkaido University Hospital, Sapporo, Japan.
authors.forename: Yuri
authors.initials: Y
authors.lastname: Tsukamoto-Takakusagi
authors.affiliation: Department of Orthodontics, Division of Oral Functional Science, Graduate School of Dental Medicine, Hokkaido University, Sapporo, Japan.
authors.forename: Fumio
authors.initials: F
authors.lastname: Saito
authors.affiliation: Clinical Department of Plastic Surgery, Hokkaido University Hospital, Sapporo, Japan.
authors.forename: Akihiko
authors.initials: A
authors.lastname: Oyama
authors.affiliation: Department of Orthodontics, Division of Oral Functional Science, Graduate School of Dental Medicine, Hokkaido University, Sapporo, Japan.
authors.forename: Junichiro
authors.initials: J
authors.lastname: Iida
mesh_terms: D000168:Acrocephalosyndactylia / Q000175:diagnosis* / Q000601:surgery; D002675:Child, Preschool; D003724:Dental Arch / Q000002:abnormalities; D019066:Facies; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D019767:Maxillofacial Abnormalities / Q000175:diagnosis* / Q000601:surgery; D010641:Phenotype; D011859:Radiography; D014071:Tooth Abnormalities
keywords: Apert syndrome; maxillary hypoplasia; skeletal Class III jaw-base relationship