Effective treatment of self-injurious oral trauma in Lesch-Nyhan syndrome: a case report.

摘要: Abstract –  Lesch–Nyhan syndrome (LNS) is an X-linked disorder originating from deficiency of the enzyme hypoxanthine guanine phosphoribosyl transferase. It characterized by neurological manifestations, including dramatic symptom compulsive self-mutilation, which results in destruction oral and perioral tissues. Several drug trials have been administered to improve severe self-destructive behaviour, with questionable effectiveness. Invasive treatment approaches, such as extraction teeth orthognathic surgery, suggested variable success. A conservative intraoral appliance serving prevent peri-oral self-injury presented this report. The patient was a 14-year-old boy demonstrating typical LNS self-biting, significant loss lip tongue tissue, spasticity involuntary movements. An acrylic maxillary designed constructed occlusal plate raising bite. retained two Adams’ clasps on first premolars, along three ball between incisors. Fabrication, insertion, maintenance were uncomplicated non-stressful patient. Periodic recall over 3-year period has confirmed effective healing lesions high level tolerance appliance.