Non-extraction solution for self-injurious behavior in children with Lesch-Nyhan syndrome: a retrospective case series with up to 14 years of follow-up.

Lesch-Nyhan syndrome (LNS) is a rare X-linked disorder characterized by hyperuricemia, neurologic impairment, and severe self-injurious behavior (SIB), frequently involving the oral tissues. Dental management of oral SIB remains challenging, and irreversible tooth extraction is still commonly employed. This retrospective case series describes four unrelated patients with LNS (aged 2.5 to 16 years) treated over a 20-year period using a customized dual-laminate thermoplastic intraoral appliance. The primary outcome was cessation of oral self-injury; secondary outcomes included tissue healing, appliance tolerance, and adverse effects. Follow-up ranged from 16 months to 14 years. All patients demonstrated complete clinical cessation of oral self-biting, with healing of soft tissues and no need for tooth extraction. The appliances were well tolerated, easily fabricated, and demonstrated durable performance without reported complications during long-term follow-up. Conclusions: This conservative, fully intraoral approach may represent an effective and reversible alternative to irreversible dental extractions for managing oral SIB in patients with LNS, particularly when implemented in experienced clinical settings. The technique appears most appropriate for dental practitioners trained in special care or hospital-based dentistry to ensure safety, comfort, and long-term functional outcomes.