Dental management of 49,XXXXY syndrome with taurodontism: A case report

A Japanese male with a karyotype of 49,XXXXY was presented for dental treatments from 6 years to 20 years of age. The patient had pulmonary stenosis as well as mental retardation, hypogonadism, fifth finger and toe clinodactyly, genu valgus, pes cavus and mandibular prognathism. Dental manifestations revealed multiple caries and taurodontism of both primary and permanent dentitions and hypodontia in permanent dentition. Dental management of a patient with 49,XXXXY syndrome is important to accomplish both controlling behavioral problems and medical considerations for preventing infective endocarditis.