Evans J, Sirikumara M, Gregory M
Department of Oral and Maxillofacial Surgery, the Royal Gwent Hospital, Newport, South Wales, U.K.
Oral Surg Oral Med Oral Pathol. 1993 Oct;76(4):437-40. doi: 10.1016/0030-4220(93)90009-s.
Lesch-Nyhan syndrome, a rare inborn error of metabolism, is characterized by mental retardation and factitious oral lesions, which cause immense difficulties in its management. A case of an 11-year-old boy, the elder of two siblings with this condition, who had severe self-inflicted injuries to oral and perioral tissues is presented highlighting the different treatment modalities considered. A tailor-made lipguard proved to provide the best cosmetic result short term.