TONGUE INJURIES BY SELF MUTILATION IN LESCH-NYHAN SYNDROME PATIENT: A CASE REPORT

Kim, Ji-Hee; Choi, Byung-Jai; Kim, Seong-Oh; Choi, Hyung-Jun; Son, Heung-Kyu; Lee, Jae-Ho

Journal of the Korean Academy of Pediatric Dentistry 2008;35(3):532-538.
Published online August 31, 2008.

TONGUE INJURIES BY SELF MUTILATION IN LESCH-NYHAN SYNDROME PATIENT: A CASE REPORT

Ji-Hee Kim, Byung-Jai Choi, Seong-Oh Kim, Hyung-Jun Choi, Heung-Kyu Son, Jae-Ho Lee

Department of Pediatric Dentistry, College of Dentistry and Oral Science Research Center, Yonsei University

Lesch-Nyhan 증후군 환아의 자해에 의한 혀의 외상

김지희, 최병재, 김성오, 최형준, 손흥규, 이제호

연세대학교 치과대학 소아치과학교실 및 구강과학연구소

Correspondence: Jae-Ho Lee, Tel: 02-2228-8800, 3173, Email: leejh@yuhs.ac

Received: 21 January 2008 • Accepted: 3 July 2008

Abstract

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.

Key Words: Lesch-Nyhan syndrome, Self mutilation, Tongue wound, Mouth guard, Extraction of teeth