We investigated the factors that cause maladapted behavior in people with autism spectrum disorders at the first visit. The subjects were 32 patients with autism spectrum disorders who visited the clinic from April 2019 to December 2021 for the first time.

We surveyed age, gender, developmental age, disability characteristics, and past dental and medical department experience. We observed and evaluated behaviors in the order of “1. Entering the examination room,” “2. Sitting in a dental chair,” “3. Supine on the dental chair,” “4. Caregiving toothbrushing,” and “5. Oral examination.” Statistical analyses were performed by using Fisher’s exact test and decision tree analysis. It was found that the dental consultation behavior at the first visit and the developmental age were related in all situations. “Entering the examination room” required the language comprehension of at least 1 year and 10.5 months, and “Supine on the dental chair” required a developmental age of at least 3 years. The optimal age for the categories was 7 years for “Entering the examination room” and “Sitting in a dental chair,” and at least 11 years for “Supine on the dental chair.” It was suggested that it is difficult to deal with young patients at the first visit. “Presence or absence of medical restraint experience” was extracted as an effective item for determining adaptive behavior such as “Sitting in a dental chair,” “Supine on the dental chair,” “Caregiving toothbrushing,” and “Oral examination.” An unpleasant experience not only in dentistry but also in the medical department affected the behavior on the first visit. It is necessary to be careful not to make people with autism spectrum disorders feel uncomfortable at medical institutions.

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.

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.

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.

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.

Ataxia-telangiectasia (AT) is an autosomal recessive disorder primarily characterized by telangiectasia, immunodeficiency and an increased risk of malignancies. It is necessary to consider the use of radiation due to DNA repair disorder and to pay attention to respiratory infection in case of dental treatment. We report the dental treatments of a patient with AT under intravenous sedation.

The patient was an 18-year-old male with AT. Dental caries were found when he initially visited our dental clinic. We scheduled dental treatment under intravenous sedation because of intellectual disability and involuntary movements. Thereafter, we recommended regular check-ups every three months. However, he returned to our dental office for the first time in one year and eight months due to forgetting his appointments and poor physical condition. This period without our management was enough time to cause secondary caries and so we scheduled second dental treatment. We needed to consult with his family doctor in advance due to the limitations of X-rays and the dose of antibiotics after dental treatment for this AT patient who had undergone immunoglobulin therapy. No symptoms such as infectious disease or cancer have been observed since dental treatment was finished.

Dental treatment for patients with AT must be planned while taking into consideration the limitations of X-rays, involuntary movements and immunodeficiency. Even after treatment, long-term discontinuation should be avoided as much as possible. Regular check-ups are necessary so that treatment can be minimally invasive.

Joubert syndrome is an extremely rare disorder that presents with dysplasia of the cerebellum and brainstem, intellectual disability, hypotonia, movement disorders, and respiratory problems in the neonatal period. We present a case of Joubert syndrome with self-mutilation of the lower lip. The patient was a boy aged 3 years and 2 months old with Joubert syndrome. At the age of 3 years and 0 month, the patient suffered a penetrating wound due to severe biting of the lower lip. The lower lip was sutured under general anesthesia at the oral surgery department of a hospital, and soft-type mouth guards were placed in the upper and lower jaws. However, the patient was referred to our hospital because of poor fit after about 1 month. He complained of severe lower lip injury due to his self-mutilation at the age of 3 years and 7 months. Therefore, we made a mandibular mouth guard for him, but it could not prevent the self-mutilation. At the age of 3 years and 8 months, we made a maxillary mouth guard at the request of his parents. At the age of 3 years and 10 months, the patient still had a bite wound on the lower lip. To prevent mandibular lip entrapment, we made a mouth guard with augmentation on the mandibular anterior teeth on the labial side. As a result, new severe lower lip injury was not observed. At present, no recurrence of the self-mutilation behavior has been observed, but the possibility of recurrence cannot be ruled out because the cause is not clear. It is important to continue to support him and his family.