title: Consequences of Delayed Dental Extraction in Lesch-Nyhan Disease.
abstract: Patients with Lesch-Nyhan disease (LND) often engage in self-injurious biting. This problem requires difficult management choices, sometimes including removal of the teeth. Although many health care professionals are reluctant to remove teeth in a child because of the permanent negative cosmetic consequences of the edentulous state, disfigurement of the face and tongue from self-biting can be worse. We analyzed the records of 5 LND patients who used mouth guards to spare the teeth. Success was variable, and dental extraction ultimately was required in 4 cases. We also reviewed previously published cases on the use of dental devices to spare teeth in LND. Various devices have been recommended, but failure rates are high, and tooth extraction often is still needed. Although dental extraction is not required in all cases, it should not be delayed when biting is severe.
authors.affiliation: Department of Neurology, Emory University, Atlanta, Georgia 30322 USA.
authors.forename: Emily M
authors.initials: EM
authors.lastname: Goodman
authors.affiliation: Division of Clinical Biochemistry, Hospital Universitario La Paz, IdiPaz, Madrid, Spain.
authors.forename: Rosa J
authors.initials: RJ
authors.lastname: Torres
authors.affiliation: Division of Internal Medicine, Hospital Universitario La Paz, IdiPaz, Madrid, Spain.
authors.forename: Juan G
authors.initials: JG
authors.lastname: Puig
authors.affiliation: Departments of Neurology, Human Genetics & Pediatrics, Emory University, Atlanta, Georgia 30322 USA.
authors.forename: H A
authors.initials: HA
authors.lastname: Jinnah
keywords: Lesch-Nyhan disease; developmental disorders; inherited metabolic disease; review; self-biting; self-injurious behavior