Lesch-Nyhan syndrome is a rare X-linked recessively inherited disorder, caused by complete absence or decrease
in activity of hypoxanthine guanine phosphoribosyl transferase(HPRT), an enzyme involved in purine
metabolism. This enzyme deficiency gives rise to nephropathy symptoms, such as hyperuricosuria and hyperuricemia
by excessive uric acid production and neuropathy symptoms, such as mental retardation, choreoathetosis
and self mutilation behavior. Patients with Lesch-Nyhan syndrome have tendency to bite their lip, tongue
and finger. In severe cases, partial or even total amputation of tongue or finger occur. Self-inflicted bites are often
complicated by secondary infection to the injured site as well as pain. Furthermore tissue loss by biting results
in esthetic problems.
The dental management of self mutilation includes treatment with appliances such as soft mouth guard or lip
bumper, extraction of all the teeth, and orthognathic surgery.
We report a case of a 13 year-old boy with Lesch-Nyhan syndrome, who severely injured himself on his
tongue. At first, conservative treatment using soft mouth guard was considered, but it could not prevent trauma
on his tongue. Therefore, extraction of the lower anterior and posterior teeth was carried out.