Abstract

MACHADO, Cíntia de Vasconcellos; PASTOR, Iandira Maria Oliveira  and  ROCHA, Maria Celina Barreiros Siquara da. Clinicoradiological features in cleidocranial dysplasia: case report. RFO UPF [online]. 2010, vol.15, n.3, pp. 302-306. ISSN 1413-4012.

OBJECTIVE: The purpose of thid study is describe a case of cleidocranial dysplasia. A cleidocranial dysplasia (CCD) is a rare autossomal dominant skeletal disease (1:1.000.000) that affects generally clavicular and cranial bones, caused by a defect in the CBFA1 gene, that regulates differentiation of osteoblasts and appropriate bone formation. CASE REPORT: this study presents a case of a 9-year-old boy with CCD, who has being accompanied for 3 years in the ambulatory of the discipline of Pediatric Dentistry, Faculty of Dentistry, Federal University of Bahia. Clinical and radiographic abnormalities like short stature, hipoplastic clavicle of the right side, presence of an enlarged cranium, hypertelorism, hipoplastic maxillary bones as well as delayed permanent tooth eruption and the presence of supernumerary teeth confirmed the diagnosis of CCD. After the necessary restorative and preventive procedures were done, an orthopedic/orthodontic and surgical treatment was proposed. FINAL CONSIDERATIONS: the early detection of this disease is essential for a proper treatment, in order to reduce the extent of surgical and orthodontic interventions in future.

Keywords : Cleidocranial dysplasia; Maxilla; Clavicle; Hypoplasia; Pediatric dentistry.