title: Multiple, supernumerary retained teeth in the course of cleido-cranial dysplasia. A case report.
abstract: Cleido-cranial dysplasia, often referred to as Scheuthauer-Marie-Sainton syndrome, is an autosomal dominant disorder of the musculo-skeletal system. Patients with cleido-cranial dysplasia are characterized by short stature, frequent varus or valgus hip, kyphoscoliosis, underdevelopment of the scapulas and the sternum, incorrect number of ribs. The most characteristic feature is unilateral or bilateral, partial or total underdevelopment of clavicles. Mental development is not affected in this syndrome. Malocclusion, occlusal irregularities, multiple supernumerary teeth, impacted teeth, and persistent milk teeth are found in the stomatognathic system. Teeth often have abnormal anatomy. Gothic palate, cleft hard and soft palate are diagnosed. The aim of this paper is to present a case of a 12-year-old boy diagnosed with irregularities in the masticatory system involving an additional number of retained teeth. The boy was referred by an orthodontist for surgical and orthodontic team therapy. The case presented confirms the observations of other authors that only the multi-specialty collaboration of a pediatrician, a geneticist, an orthopedist, an orthodontist, a maxillofacial surgeon, an implant prosthetic surgeon and a physiotherapist can provide proper diagnosis and treatment.
authors.affiliation: Clinical Department of Maxillo-Facial Surgery, Provincial Specialist Hospital in Rzeszów, ul. Chopina 2, 35-005 Rzeszów, Phone 17 866 62 60, 605-547-070, e-mail: boglewandowski@wp.pl.
authors.forename: Bogumił
authors.initials: B
authors.lastname: Lewandowski
authors.forename: Katarzyna
authors.initials: K
authors.lastname: Martula-Gala
authors.forename: Robert
authors.initials: R
authors.lastname: Brodowski
authors.forename: Barbara
authors.initials: B
authors.lastname: Zych
mesh_terms: D000293:Adolescent; D002973:Cleidocranial Dysplasia / Q000150:complications* / Q000000981:diagnostic imaging; D006801:Humans; D007569:Jaw Abnormalities / Q000000981:diagnostic imaging* / Q000209:etiology* / Q000601:surgery; D008297:Male; D008437:Maxilla / Q000503:physiopathology*; D019647:Oral Surgical Procedures; D011859:Radiography; D014081:Tooth Extraction; D014095:Tooth, Impacted / Q000209:etiology; D014096:Tooth, Supernumerary / Q000000981:diagnostic imaging / Q000209:etiology*; D016896:Treatment Outcome