Lesch-Nyhan syndrome and the lower lip guard.
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.
Evans
J
J
Department of Oral and Maxillofacial Surgery, the Royal Gwent Hospital, Newport, South Wales, U.K.
Sirikumara
M
M
Gregory
M
M
D002648:Child; D006801:Humans; D007926:Lesch-Nyhan Syndrome / Q000503:physiopathology*; D008046:Lip / Q000293:injuries*; D008297:Male; D009064:Mouth Protectors; D012652:Self Mutilation / Q000517:prevention & control*