Cleidocranial Dysplasia: Diagnosis, Surgical and Orthodontic Planning and Interventions in a Pediatric Patient

Francine Sumie Morikava; Rafaela Scariot; Imara de Almeida Castro Morosini; Aline Sebastiani; Delson João Costa; Fabian Calixto Fraiz & Fernanda Morais Ferreira

Summary

Cleidocranial Dysplasia (CCD) is an autosomal dominant bone disorder caused by a defect in the CBFA1 gene and characterized by skeletal, craniofacial and orodental anomalies. This paper describes the main aspects of a case of CCD from diagnosis and planning to the first step of the interventions. An 11-year-old male patient sought the pediatric dentistry clinic of the Federal University of Paraná (Brazil) with the complaint of the prolonged retention of nearly all his primary teeth. Clinical and imaging exams led to the diagnosis of CCD and the treatment plan was outlined. The first step consisted of the extraction of 4 primary, 2 permanent and 2 supernumerary teeth from the maxilla, followed by palate separation, traction of the impacted teeth and reverse traction of the maxilla. The patient remains in treatment. Clinical follow up as well as the awareness and motivation of the family are important factors in such cases.

KEY WORDS: cleidocranial dysplasia, bone diseases, tooth, supernumerary.

How to cite this article

MORIKAVA, F. S.; SCARIOT, R.; MOROSINI, I. A. C.; SEBASTIANI, A.; COSTA, D. J.; FRAIZ, F. C. & FERREIRA, F. M. Cleidocranial dysplasia: Diagnosis, surgical and orthodontic planning and interventions in a pediatric patient. Int. J. Odontostomat., 10(2):325-331, 2016.