Oral self‐mutilation by a 17‐month‐old child with Lesch‐Nyhan syndrome

Abstract

We report a 17-month-old boy who was a known case of Lesch-Nyhan syndrome and presented with self-inflicted oral ulcerations of his lips and cheeks. He had a normal complement of caries-free deciduous teeth. Initially a conservative approach was planned and a bite plate made, but as a result of poor compliance and persistent ulceration and after consultation with his parents it was decided to extract all deciduous teeth. Introductionpurine metabolism. HPRT catalyses the condensation

Lesch-Nyhan syndrome: The saga of metabolic abnormalities and self-injurious behavior

“…SIB has also been observed in congenital insensitivity to pain with anhidrosis, mental retardation, and syndromes as Cornelia de Lange syndrome, Munchausen syndrome, Moebius syndrome, Gilles de la Tourette syndrome, and Rett syndrome (5). Since the oral cavity is the earliest primary focus and the means of interaction with environment, it falls prey to any attempt at self-injury, with sharp teeth being the preferred tool (5,6). After the eruption of the primary teeth, SIB is displayed, as was noted in the current case.…”

“…Conservative modalities such as a mouthguard, bite guard, lip bumpers, or bite planes have been tried in the past but they have not been effective (9)(10)(11). Extraction of both primary and permanent teeth may be the ultimate solution in most cases (2,5,6). In the current case, the patient's pattern of self-biting changed after a bite guard was affixed and later when the primary mandibular incisors were extracted.…”

Consequences of Delayed Dental Extraction in Lesch‐Nyhan Disease

“…First, SIB is a chronic disorder and long‐term use of such appliances is impractical. Several investigators have commented on poor tolerability of the appliances, related to the difficulty they create with daily dental hygiene and eating that require frequent removals and reinsertions, as in cases DN and DB . A related problem is the need for frequent revisions as the child's mouth grows and additional teeth erupt.…”

“…Several investigators have commented on poor tolerability of the appliances, related to the difficulty they create with daily dental hygiene and eating that require frequent removals and reinsertions, as in cases DN and DB. 9,11,14,15,20,22,23 A related problem is the need for frequent revisions as the child's mouth grows and additional teeth erupt. It is sometimes impossible to acquire good oral impressions needed for the devices because of the patient's refusal to open the mouth.…”

Evaluation of Epidemiology and Etiology of Dental Injuries Observed in Children [Cocuklarda Gorulen Dis Yaralanmalarinin Etiyoloji ve Epidemiyolojilerinin Degerlendirilmesi]

“…Süt dentisyon tamamlandığında genellikle travmanın şiddeti de artar [52,53]. Mental retardasyonu olan çocukların koordinasyona dayanan kompleks hareketleri yapamadıklarından ve kendi kendine zarar verme eğiliminde olduklarından düşme ve çarpma sonucu dental yaralanmalara normal çocuklara göre daha sık maruz kalırlar [54,55].…”