PREVALENT ORAL MANIFESTATIONS IN PATIENTS WITH CLEIDOCRANIAL DYSPLASIA: LITERATURE REVIEW

Abstract

Cleidocranial Dysplasia or Cleidocranial Dysostosis is defined as an extremely rare, autosomal dominant genetic syndrome that involves mutations in the gene responsible for osteoblastic differentiation, resulting in late development and defects in bone, craniofacial and stomatognathic structures. The diagnosis is based on confirmation of the clinical and radiological features of the following pathognomonic triad: multiple supernumerary teeth, partial or total absence of clavicles and sagittal suture and open fontanelles. The objective of this work was to carry out a literature review about the oral manifestations resulting from cleidocranial dysplasia, aiming to understand how they are important for the conclusion of the diagnosis of the disease and how the dentist acts in the resolution of the same, through searches in the bases of data Scielo and Google Scholar. Because it is a disease that involves the oral cavity, leading to changes in maxillomandibular structures, such as the presence of supernumerary teeth, changes in the palate, delay in tooth eruption, among others, which affect the aesthetics and quality of life of patients, It is extremely important to study the oral manifestations of Cleidocranial Dysplasia, since commonly the dentist is the first health professional sought by the patient with the disease due to these changes. Therefore, knowledge of the characteristics of dysplasia, its etiology and its involvement with the oral cavity is essential for the professional to be increasingly able to recognize such manifestations and thus contribute to the early diagnosis and act in the development of the treatment plan. appropriate, contributing to the well-being of the patient.