Conservative management of oral self-mutilation in Lesch–Nyhan syndrome enhanced by a digital workflow: a case report

Abstract Introduction Lesch-Nyhan Syndrome (LNS) is a rare X-linked disorder characterized by the triad of hyperuricemia, neurological dysfunction, and compulsive self-injurious behavior, often involving oral self-mutilation. Objective This report describes the conservative, multidisciplinary management of severe oral self-mutilation in a hospitalized child with LNS, combining botulinum toxin and a digitally fabricated customized bite plate. Case Report A 10-year-old male with LNS was admitted to the Intensive Care Unit due to gastrostomy-related perforation and sepsis. Oral examination revealed complete permanent dentition, generalized dental calculus and gingivitis, cicatricial lesions on the tongue, and partial loss of the lower lip due to chronic self-biting. Previous provisional bite plates had proven ineffective. Methods The patient underwent periodontal treatment, botulinum toxin application to the bilateral masseter and orbicularis oris muscles, and placement of a customized bite plate fabricated via digital intraoral scanning and 3D printing. Patients with additional neurological conditions might potentially benefit from it. Results Post-intervention, parafunctional movements decreased, soft tissue lesions partially healed, and oral function was preserved without adverse effects. Conclusion This case report reveals that combining tailored care, botulinum toxin therapy, and digital splint manufacture creates a realistic and patient-centered alternative to extreme interventions, preserving oral function and contributing to enhanced quality of life.