ORAL ALTERATIONS IN PATIENTS WITH SPONDYLOCOSTAL DYSOSTOSIS: A CASE REPORT

Spondylocostal dysostosis is a rare genetic disease characterized by skeletal malformations, such as multiple segmentation defects of the vertebrae, rib misalignment with variable points of intercostal fusion, and reduction in the number of ribs. In the literature, we did not find any reports of oral manifestations related to this condition. This is the report of a 23-year-old white woman with spondylocostal dysostosis, with short stature, short trunk and neck, prominent thorax, moderate scoliosis, umbilical hernia, and cognitive deficit. In addition, we observed nodules on the upper lip and tongue, generalized enamel hypoplasia, and gemination of the crown of tooth 32. Radiographic examination revealed presence of supernumerary teeth in the mandible and gyroversion of the tooth 31. We guided and motivated dental hygiene, performed periodontal therapy, restored the anterior teeth, and performed the endodontic treatment of the tooth 46. The aesthetic and functional rehabilitation performed proved to be effective