Oral aspects of 49, XXXXY syndrome: a case report.

The 49, XXXXY syndrome is a rare chromosomal disorder. The purpose of this paper was to present the case of a 9-year-old boy with this karyotype. The most remarkable skeletal and dental anomalies were the absence of 9 permanent teeth, taurodontism of the permanent first molars, and the conical shape of permanent maxillary lateral incisors. Third molars were not visualized. Cephalometric analysis revealed a well-positioned maxilla, a slightly protruding mandible, and retroclined mandibular incisors. The surgery and dental rehabilitation are described. Given the specific characteristics of this syndrome, it is important to reassess the patient's skeletal and dental development and promote good dental hygiene practices.