493 - Lesch-Nyhan Syndrome in a Pediatric Patient

Introduction: Lesch-Nyhan Syndrome is an X-linked recessive genetic disorder most often found in males. It is characterized as a neurological and behavior condition with an abnormal build up of uric acid levels in the body, often causing arthritis and kidney stones. Behavioral disturbances experienced by these patients include muscle tensing, jerking, and flailing of the limbs. Self-mutilation, including biting and head banging, is often a result of these disturbances.

Case Report: A 10-year-old male presented to Riley Children’s Hospital dental clinic for evaluation.  Parent’s chief concern is lip and finger biting. His health history included Lessch-Nyhan syndrome, learning delay and having a g-tube. The patient exhibited self-mutilative behaviors and was wheelchair bound with wrist restraints. This report will include different treatment options performed prior to completely edentulating the patient, as well as the sequencing of extractions both chair side and under general anesthesia, in order to reduce self-harm.