Oral self-mutilation in the Lesch-Nyhan syndrome

Lesch-Nyhan Syndrome is a disorder caused by congenital absence of a purine metabolic enzyme, hypoxanthine-guanine phosphoribosyl fransferase (HPRT). This syndrome is characterized clinically by mental retardation, chorea, athetosis, hyperuricemia, uricosuria and self-mutilation. This report is of two children, who are cousins, both of whom have Lesch-Nyhan syndrome and presented with severe self-mutilation wounds on their lip(s). Vital pulpotomy and coronal resection was done as a more conservative approach than extracting all offending teeth. By maintaining the root portion of the teeth in the bone, it is expected that preservation of the alveolar bone will be achieved.