Dental Management of Cornelia De Lange Syndrome: Case Report and Literature Review Cornelia De Lange Syndrome

Cornelia de Lange syndrome (CLS) occurs in one out of 10.000 individuals. It is mainly characterized by a delay in growth and development, hirsute, facial dysmorphia, upper-extremity malformation, cardiac defects and dental problems. The aim of this paper is to describe the dental management of a 20 year-old male with CLS, who had multiple dental problems such as caries, periodontal disease, malocclusion and an impacted premolar in the maxilla which produce pain and self-injurious behavior. Physical findings were similar to those previously described by the literature. Dental treatment was carried out under sedation due to the patient's inability to cooperate during dental treatment. After 1 year of follow-up there were no new caries, the periodontal health had improved and the auto aggressive behavior was considerably reduced.