Development of Osteomyelitis of the Jaw in Patients with Congenital Insensitivity to Pain with Anhidrosis: A Case Study in a Sibling Pair

<p>Congenital insensitivity to pain with anhidrosis (CIPA) is a genetic disorder characterized by the loss of sensitivity to temperature and pain as well as dyshidrosis and intellectual disability. Dental complications associated with CIPA include bite wounds of the tongue and lips as well as osteomyelitis of the jaw. We describe here the case of osteomyelitis of the jaw in a sibling pair with CIPA.</p><p>The 16-year-old elder sister had poor oral hygiene and had developed fever and swelling of the right cheek 4 days after the extraction of 15. One week after the tooth extraction, we observed the formation and drainage of a gingival abscess in 16 on the cheek side. The mobility of 16 became significant by 28 days after the tooth extraction, and computed tomography revealed that the necrotic portion of the alveolar bone that corresponded to 16 had undergone spontaneous sequestration. The tooth was extracted based on these findings, and the necrotic bone was removed. Her younger brother, who was 5 years old, had poor oral hygiene and significant attrition throughout the jaws. He presented to our hospital with fever and swelling of the right mandibular cheek. Attrition had caused open pulpitis in 85, which led to gingival abscess formation. We performed procedures to open the pulp and administered medications. However, we observed the loss of (83, 84, 85) and the presence of necrotic bones after 3 weeks. Subsequently, we observed the loss of the necrotic bone and tooth germ of 45.</p><p>Based on our observations, we suspected that osteomyelitis of the jaw resulted from an infected socket of 15 in the elder sister and from the infection in the opening of the pulp due to attrition in the younger brother. In both patients, osteomyelitis rapidly progressed to the spontaneous sequestration of necrotic bones. The inflammatory response involved in tissue defense and repair is known to be distinct in CIPA patients, and our findings suggest that such a response may have played a role in the development of osteomyelitis.</p><p>Patients with CIPA do not have subjective symptoms due to the lack of pain sensation. This may result in a delay in detecting infections in CIPA patients, highlighting the importance of the preoperative and postoperative administration of antibiotics. Regular dental checkups should also be promoted in order to prevent and facilitate the early detection of dental disorders and to ensure maintenance of proper oral hygiene.</p>