Traumatic lesions from congenital insensitivity to pain with anhidrosis in a pediatric patient: dental management.
Congenital insensitivity to pain with anhidrosis is a rare autosomal-recessive disorder characterized by unexplained fever episodes, anhidrosis, pain insensitivity, self-mutilating behavior, and mental retardation. The lack of sensitivity to pain results in traumatic lesions, such as ulcers, fractures, burns, bites, scars, and digital amputations. Several methods have been suggested to treat these patients; however, appropriate management is difficult, especially when the mutilation is particularly severe. This report describes the case of a 2-year-old female patient who had severe self-mutilating injuries to her tongue, hands, lips, and oral mucosa caused by biting. The patient presented digital amputation and also a premature loss of a permanent tooth germ during the treatment. The dental management is described and discussed. It is important to include the dentist on the multidisciplinary team to reduce the frequency and severity of the self-inflicted lesions in these patients, also to prevent complications.
Neves
Beatriz Gonçalves
BG
Department of Pediatric Dentistry and Orthodontics, Federal University of Rio de Janeiro, Rio de Janeiro, Brazil.
Roza
Rosemere Teixeira
RT
Castro
Gloria Fernanda
GF
D001734:Bites, Human / Q000150:complications / Q000209:etiology* / Q000517:prevention & control; D002675:Child, Preschool; D003241:Consanguinity; D005260:Female; D005383:Finger Injuries / Q000209:etiology; D006801:Humans; D007007:Hypohidrosis / Q000150:complications; D008046:Lip / Q000293:injuries; D009061:Mouth Mucosa / Q000293:injuries; D019226:Oral Ulcer / Q000209:etiology*; D000699:Pain Insensitivity, Congenital / Q000150:complications*; D010348:Patient Care Team; D012652:Self Mutilation / Q000209:etiology; D013577:Syndrome; D014059:Tongue / Q000293:injuries; D014081:Tooth Extraction