Long-term Oral Management of a Case of Congenital Insensitivity to Pain with Anhidrosis

<p>Congenital insensitivity to pain with anhidrosis (CIPA) is an autosomal recessive inherited disease characterized by an absence of thermal sensation and pain, anhidrosis and intellectual disabilities. The dental problems in cases with CIPA are bite wounds on the tongue, lips, buccal mucosa and finger tips ; osteomyelitis of the upper and lower jaws ; and pathological fracture of the jawbone. In infancy, protective plates are applied to the patient to prevent severe bite wounds, but they are difficult to make and use.</p><p>In this study, we report a patient who underwent long-term oral management from infancy to adulthood, and the dentition model measurements at the time of 2 years and 7 months, 5 years and 1 month, and 27 years and 6 months.</p><p>1. In infancy, protective plates were used to prevent bite wounds on the tongue and buccal mucosa from 10 months of age for the mandible and from 1 year for the maxilla, to 4 years and 8 months.</p><p>2. The use of protective plates from the early stage of eruption of deciduous teeth is an effective method to prevent early tooth extraction and tooth loss before the roots are formed completely, and to prevent bite wounds.</p><p>3. In model analysis, there was no evidence of dental arch narrowing in both the upper and lower jaws in the permanent dentition, and the use of protective plates in the deciduous dentition phase had no obvious effect on the permanent dentition.</p><p>4. In the permanent dentition, sudden mobility of the maxillary bilateral first and second molars occurred, and the left first and second molars were extracted because they were difficult to preserve. The right first and second molars, with sequestrum, fell out as a single mass which was presumed to be due to osteomyelitis resulting from periodontitis.</p>