ORAL SELF-MUTILATION IN THE LESCH-NYHAN SYNDROME : CASE REPORT

,; ,; ,; ,

Journal of the Korean Academy of Pediatric Dentistry 1999;26(1):151-156.
Published online February 28, 1999.

ORAL SELF-MUTILATION IN THE LESCH-NYHAN SYNDROME : CASE REPORT

Lesch-Nyhan syndrome 환아의 oral self-mutilation에 대한 증례보고

전진용, 이제호, 최형준, 최병재

연세대학교 치과대학 소아치과학교실

Abstract

Self-injurious behavior is defined as deliberate harm to one's own body without suicidal intent. It usually occurs as head banging or hitting, skin cutting, or finger biting and includes ocular, genital and oral self-mutilation. Lesch-Nyhan syndrome is a rare X-linked recessively inherited disorder, caused by complete absence of hypoxanthin-guanine phosphoribosyl transferase(HPRT) activity. Clinical presentation is characterized by mental retardation, chorea, athetosis, hyperuricemia, uricosuria and self-mutilating behavior. In these patients, the most typical feature is loss of tissue from biting themselves, even though they are not insensitive to pain. The dental management of self-mutilation includes treatment with appliances such as soft mouthguard or lip bumper, extraction of all the teeth, and orthognathic surgery. We report a 25-month-old boy who was a known case of Lesch-Nyhan syndrome and presented with severe self-mutilation wound on his lower lip. Vital pulpectomy and coronal resection was done as a more conservative approach than extracting all primary anterior teeth. Due to maintaining the root portion of the teeth in the bone, it is expected that the normal growth of the alveolar bone will be achieved.

Key Words: Lesch-Nyhan Syndrome, self-injurious behavior, self-mutilation