Management and Prevention of Oral Self-Injuries in Lesch–Nyhan Syndrome

Abstract

Lesch-Nyhan syndrome (LNS) is a rare X-linked recessive disorder with an incidence of 1/100,000-380,000 live births. It is characterized by neurological manifestations, including symptoms of compulsive self-mutilation, which result in the destruction of oral and perioral tissues. This report describes a case of a four-year-old boy diagnosed with LNS, who was referred for evaluation and treatment of self-injury behaviour (SIB). The parents requested the prevention of self-mutilation of the lower lip and tongue by the child's own teeth. After a thorough discussion with the parents, it was agreed that a conservative approach, avoiding extraction, should be followed initially. A removable dental appliance was fabricated. The parents were instructed and trained about insertion, removal, and cleaning of the appliance. The child was re-examined after one week: biting of the lips and tongue improved immediately after the insertion of the appliances. Initial healing of the lesion was observed. After two and four weeks, positive results were seen. The lesion had resolved completely. In conclusion, appropriate preventive methods have to be developed for each individual patient on the basis of the observation of each single case. Oral appliances represent a conservative solution for SIB and an alternative to more invasive approaches. They can be the initial solution for the management of oral self-injury in LNS patients.