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

Lesch-Nyhan syndrome 환아의 oral self-mutilation에 대한 증례보고

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.

1. 하순에 심한 self-mutilation wound를 야기한 Lesch-Nyhan syndrome 환아에서 상하악 유전치에 치관부 절단 및 치수 절제술을 시행하여 치아에 의한 자해를 방지하였다. 2. 장치를 이용한 치료가 어려운 경우 발치가 고려되기도 하나 본 증례에서는 발치보다 보존적인 술식으로 만족할 만한 결과를 얻었다.