Abstract
Prader-Willi syndrome is caused by an abnormality in chromosome 15 and characterized by hypotonia in infancy, poor weight gain due to suckling disorder, overeating and obesity that becomes prominent from early childhood, ambiguous external genitalia, almond-like palpebral fissures, a fishlike triangular mouth, and mental retardation. Although affected individuals have a friendly personality, they have difficulty in suppressing their emotions, and problems with their surroundings frequently occur due to character disorders and abnormal behavior. In particular, the character disorders become more difficult to manage with age.As for oral manifestations, enamel hypoplasia, saliva with high viscosity, and dry mouth along with other are generally observed, with tooth decay prone to occur from overeating and poor oral hygiene. We have been conducting oral management of several Prader-Willi syndrome patients since early childhood. However, they gradually became non-cooperative when reaching school age and treatment becomes difficult after adolescence. Even though behavior modification was attempted in these cases,cooperation could not be obtained and dental treatment was conducted under general anesthesia.In our experience, Prader-Willi syndrome patients may first seem compliant, leading practitioners to conclude that they will cooperate with various management techniques. However, these patients often do not understand the explanations or results provided by the practitioner. In order to solve these problems, we think it important to perform treatment according to individual circumstances by keeping intravenous sedation and general anesthesia in mind as possible options, so as not to cause discomfort and/or stress to patients, as well as in cooperation with other medical departments to fully understand the current physical and mental state of the patient.
