title: [Cleidocranial dysplasia: report of 2 cases and literature review].
abstract: Cleidocranial dysplasia or dysostosis involves dental anomalies, bone abnormalities with membranous ossification (clavicles, cranium, face, pelvis), rarely of the spine and the remainder of the skeleton. We report 2 new cases and describe the different clinical aspects of this disorder and the orthopedic problems that it can pose. The clinical demonstrations of this disease are highly variable and inconsistent, which explains the diversity of circumstances of discovery. Abnormalities of the face and clavicles, as well as of pelvic ossification are most frequent and can be regarded as major signs. These clinical demonstrations do not require treatment in the majority of the cases. Dental anomalies, coxa vara and scoliosis require regular monitoring and treatment in the event of progressive aggravation. The incomplete penetrance of this autosomal dominant disease and its good tolerance explain the frequency of undiagnosed forms, whose clinical expression is discrete.
authors.affiliation: Service de chirurgie orthopédique et traumatologique, CHU Habib Bourguiba, faculté de médecine, 3029 Sfax, Tunisie. dr_moez_trigui@yahoo.fr
authors.forename: M
authors.initials: M
authors.lastname: Trigui
authors.forename: K
authors.initials: K
authors.lastname: Ayadi
authors.forename: M Ould
authors.initials: MO
authors.lastname: Elhassan
authors.forename: M
authors.initials: M
authors.lastname: Zribi
authors.forename: I
authors.initials: I
authors.lastname: Chabchoub
authors.forename: H
authors.initials: H
authors.lastname: Keskes
mesh_terms: D002675:Child, Preschool; D002973:Cleidocranial Dysplasia / Q000175:diagnosis*; D005260:Female; D006801:Humans; D008297:Male