A Modified Intraoral Resin Mouthguard to Prevent Self-Mutilations in Lesch-Nyhan Patients

Abstract

Lesch-Nyhan syndrome, described in 1964 by Lesch and Nyhan, is a X-linked recessive disorder, occurring in 1 : 100000 to 1 : 380000 live births. LNS is characterized by a decrease in activity of hypoxanthine guanine phosphoribosyl transferase, an enzyme involved in purine metabolism, resulting in overproduction of uric acid. Hyperuricemia and neurological features including choreoathetoid spasticity, self-mutilation, and mental retardation clinically characterize this syndrome. In LNS patients the typical feature is loss of tissue from biting themselves with partial or complete amputation of fingers, lips, and tongue. The self-mutilation compares with the eruption of the deciduous teeth. Several drugs trials have been administered to improve self-destructive behavior and invasive treatment approaches, such as extractions of teeth and orthognathic surgery, have been suggested with variable effectiveness. Nowadays prevention is, therefore, the standard of care. The role of dentistry is essential in the management of the self-mutilating behavior, because the teeth represent the main self-injury instrument. This report presents a revision of various therapeutic approaches to manage self-destruction, highlighting the effectiveness of a preventive treatment. It describes a new technique: a resin mouthguard, realized at Gaslini Hospital, to obtain immediate healing of the oral lesions, confirmed in the follow-up period.

Lesch-Nyhan syndrome: The saga of metabolic abnormalities and self-injurious behavior

“…However, oral injuries, and especially those of the lower lip and tongue, can still occur. Conservative modalities such as a mouthguard, bite guard, lip bumpers, or bite planes have been tried in the past but they have not been effective (9)(10)(11). Extraction of both primary and permanent teeth may be the ultimate solution in most cases (2,5,6).…”

Case Report of a Severe Recurrent Tongue Self-Injury in an Infant With Dystonia

“…Odontologists recommended installing of a bite guard, a system that has shown efficacy in selfinjuries due to other neurologic disorders such as Lesch-Nyhan syndrome. 8 The first attempt to discontinue NMB involved applying resin composites to the sharp temporary teeth. This noninvasive technique eliminated the sources of cuts and was successful for 8 days.…”

Overcoming the oral aspects of ­self‐mutilation: description of a method

“…These devices need to address the patient' s penchant for biting his tongue, lips, cheeks, and oral mucosa, in addition to hands and shoulders. Numerous designs have been reported in the literature, 31,32 most of which have enjoyed limited success when considered in the context of dayto-day management, particularly in those suffering from intellectual disability and those with powerful, involuntary, and sudden movements into occlusion. None have succeeded in satisfying all of the above criteria and much of the blame for this is the difficulty in achieving adequate retention of the appliance.…”