Lesch-Nyhan syndrome is a genetic abnormality disease related to metabolic enzymes of uric acid, and the main symptom is self-mutilation behavior. We report here a patient with Lesch-Nyhan syndrome whose injuries caused by self-mutilation behavior decreased after continuous use of a mouth-guard（MG）under a regional alliance. The patient was a 4-year-old male diagnosed with Lesch-Nyhan syndrome with mental retardation（severe）. His mother brought him to our clinic to receive MG treatment for prevention of self-mutilation behavior. Biting his hands and lips had been observed since the age of 6 months and had recently worsened. Multiple self-mutilation wounds were observed on his hands and lips as well as attrition on the teeth. We produced a soft-type MG. After use of the MG, no remarkable wounds caused by self-mutilation were observed. At the age of 7 years, a large wound caused by self-mutilation was observed on the lower lip, and then our examination showed that the MG had become incompatible, because his teeth were undergoing replacement with permanent teeth. We repeated taking impressions and produced a new MG, after which the behavior was significantly reduced. Until the age of 12 years, we continued to produce a new MG every 1-2 months through corporation with a local dental clinic and performed only minimum tooth grinding. When the patient was 12 years old, a serious wound was found which led us to consider the benefits and feasibility of extracting the teeth that could cause serious wounds. On the other hand, however, several reports have noted that self-mutilation behavior is reduced as age increases in patients with Lesch-Nyhan syndrome. Thus, we decided to avoid tooth extraction and produced a new MG. The MG was a hard type, because permanent dentition was completed, and the soft-type MG were soon broken due to severe self-mutilation behavior. A temporary MG was used for 2 weeks until the new device was delivered to the patient. The hard-type MG was broken when the patient was 13 years old and he again wore a soft-type MG. However, his mother noticed that a gagging reflex gradually worsened at the age of 15 years, and it made it impossible for him to keep wearing any type of MG. Now, the patient is 16 years old, and severe self-mutilation behavior has not been recently observed. Cooperation will be needed with a local dental clinic again if we find oral wounds caused by self-mutilation.

Here, we report our experience with a case of wisdom tooth extraction performed under general anesthesia in a cerebral palsy patient with intellectual disability who had severe mitral insufficiency and atrial fibrillation.

The patient was a 32-year-old woman, who was referred to N University Hospital because the need for wisdom tooth extraction was noted. Since birth, the patient had mitral valve prolapse, which has currently progressed to severe mitral insufficiency. In selecting behavior management, due to the fact that the surgical invasiveness of wisdom tooth extraction was relatively high and the patient had cerebral palsy, general anesthesia was considered to be indicated. Taking the patient’s coexistent severe mitral insufficiency into consideration, selection of general anesthesia was performed in accordance with the guidelines for perioperative cardiovascular evaluation and management for noncardiac surgery. Although transient reduction in blood pressure was observed upon introduction of anesthesia, by performing rapid infusion using cardiac output, stroke volume, and changes in stroke volume using FloTrac^® sensor as an indicator, management was successfully performed without serious complications.

In order to reduce refractory epilepsy seizures, vagus nerve stimulation therapy is sometimes carried out as an adjuvant seizure therapy. Here we report a case of perioperative general anesthesia management for tooth extraction in a patient with severe intellectual disabilities who was implanted with a vagus nerve stimulator（VNS） for intractable epilepsy.

The patient is a 26-year-old man. At 4 years old, he became severely intellectually disabled with intractable epilepsy due to sequelae of herpes encephalitis, and at 23 years old, he was implanted with VNS via corpus callosotomy in our hospital in the neurosurgery department. Although he takes multiple antiepileptic drugs, he still has frequent epileptic seizures every day. He was counseled by his home dentist to have his wisdom teeth extracted and introduced to the department of oral surgery in our hospital. We planned to extract 4 wisdom teeth under general anesthesia because we could not expect his cooperation during the surgery.

To prevent his perioperative seizures, we prepared benzodiazepine diazepam and a magnet which can temporarily apply a strong electric stimulus by holding it over the VNS at the time of epilepsy seizure aura. During status epilepticus, we planned to let him be examined by a doctor of neurosurgery. Propofol and remifentanil were used as general anesthetics, but no epileptic seizures occurred during the operation. After the operation, he had a slight fever, and the day after the operation, a diazepam suppository was administered due to tonic seizure. For two days after the operation, as his body temperature rose to 38°C, we referred him to the department of infectious diseases. However, pneumonia and other infection diseases were not present, and he was discharged three days after the operation.

The patient suffered from frequent seizures despite VNS being implanted, and careful handling was required for perioperative seizures. In addition, as patients with epilepsy may suffer from heat retention, it is difficult to distinguish this from infectious fever, and we also had to pay attention to body temperature management.

Introduction：Congenital insensitivity to pain with anhidrosis (CIPA), a rare disorder classified as hereditary sensory and autonomic neuropathy type IV, is considered to show autosomal recessive inheritance with many uncertainties in the pathology. In the present article, we report a 9-month-old girl diagnosed with this disorder on the basis of abnormal ulceration of the tongue.

Case and course：Patient：A 9-month-old girl. First visit：March, 2015. Chief complaint：Ulceration of the tongue and feeding difficulty. Family history：Not applicable. Medical history：In November 2014 when she was 5 months old, she was presented at our pediatrics clinic with aggravation of erosion and swelling on the skin of the bilateral thumbs and left second finger due to finger sucking. History of present illness：On March, 2015, she was admitted to our pediatrics clinic for dehydration due to feeding difficulty and referred to us for close examination of ulceration and bleeding of the tongue. Intraoral findings：The lingual body was rather swelled, and a large ulceration with surrounding crust was observed near the lingual apex. General condition：Dehydration was observed. Right second and third finger nails were peeled, and bites were observed on the bilateral hand fingers. Palpation revealed hand skin dryness. Clinical course after admission：Fluid replacement was started. Her body temperature was 38°C when she was presented due to bleeding of the tongue. Then we started oral care and intervention for ulceration, such as applying dexamethasone ointment and grinding sharp edges of teeth. This patient was referred to pediatrics for close examination of CIPA, suspecting self-injurious behavior leading to abnormal ulceration. The body temperature was reduced after cooling. Dehydration was corrected with fluid replacement. Ulceration was improved, gradually allowing better feeding；therefore, she was discharged on March. Later, the disorder was diagnosed in pediatrics based on the sweating test and clinical symptoms. Now a follow-up is being conducted among pediatrics, orthopedics, and us. A developmental test was performed in pediatrics, indicating mild developmental disorder. She has presented to the orthopedics clinic with a fracture of the lower extremity. Our treatment was discontinued because of a stable intraoral condition, and then regular oral maintenance is performed at a primary dental clinic.

Conclusion：CIPA could be diagnosed on the basis of abnormal ulceration of the tongue. Early detection of and intervention for this disorder should be done even by dentists for improving QOL of a patient and his/her family members.

The 9p trisomy syndrome is a chromosomal abnormality that is clinically characterized by short stature, microcephaly, and delayed bone development, including craniofacial and digital dysmorphic features, as well as varying degrees of intellectual disability. We describe here a 22-year-old woman with 9p trisomy syndrome who exhibited an upper labial ulcer induced by twisted and delayed eruption of the upper right first incisor. She demonstrated a high-arched palate, shortened superior labial frenum, malocclusion, three supernumerary teeth, and three impacted teeth, as well as moderate periodontitis. She had a clinical history of hypocalcemia induced by pseudohypoparathyroidism at the age of 6, resulting in an enamel hypoplasia line on both first upper premolars.

To treat the ulcer, it seemed effective for this patient with disability to modify the shape of the upper incisor, in order to relive pressure between the upper lip and upper incisor. When the ulcer had recovered, improvement of the twisted upper incisor via orthodontic therapy could follow. To prevent aggravation, maintenance of oral hygiene by her guardians was also needed.

After several attempts at training her to adapt to dental care, we determined that her cooperation might be inadequate for dental treatment. We provided a glass ionomer cement covering to widen the surface of the upper first incisor and asked her guardian to keep her mouth clean by daily brushing. At first, the ulcer showed repeated episodes of improvement and aggravation. Thus, to further reduce the pressure between the lip and teeth we again covered both the upper first and second incisors with glass ionomer cement. As a result, the ulcer recovered and did not recur. Although orthodontic therapy could not be applied because of the patient’s inadequate cooperation, she accepted professional mechanical cleaning of the teeth. Her oral hygiene gradually improved. Regular dental check-ups are necessary for maintenance of oral hygiene and for early detection of oral disease.

Patients with Rett syndrome（RTT）face problems such as difficulty with conscious dental treatment, respiratory abnormalities, and difficulty with airway management. We received a request for domiciliary dental treatment, but there would be difficulty with conscious dental treatment. Herein, we report on perioperative management at home including that of dental treatment under hospitalization with general anesthesia of severely mentally and physically disabled persons with Rett syndrome.

The patient was a 40-year-old woman. She had severe mental retardation, epilepsy, and cerebral paralysis. She had been diagnosed with RTT at the age of 18 years. She had difficulty keeping a sitting position, needed total help with daily living activities, had no verbal communication ability, and had light scoliosis. Four months after initial diagnosis, she received dental treatment under general anesthesia by a different dental office, but the dental treatment was stopped because of distrust. We received a request for domiciliary dental treatment by decreasing dietary intake and for untreated caries teeth. Because there would be difficulty in conscious dental treatment, we undertook such treatment including extraction, conservative treatment, and prosthodontic treatment under hospitalization with general anesthesia. Subsequently, we managed her prophylactic oral treatment in the outpatient clinic under intravenous anesthesia, with home visit guidance by the dental hygienist.

It seems important to have safe dental treatment by anesthesia management, product dental prosthesis considering the function and cleanability, and making sure of guidance together with simple treatment administered via home visits that enables overall regional dental care.

We report the case of a myotonic dystrophy patient who underwent a jaw bone osteotomy under general anesthesia and who developed aspiration pneumonia by dysphagia after the operation. A 28-year-old male visited our hospital because he needed a jaw bone osteotomy. He was given blood tests which showed a very high creatine kinase score, and he was diagnosed with myotonic dystrophy. However, a neurologist determined that he was able to undergo the operation using general anesthesia, and so the patient underwent a sagittal splitting ramus osteotomy. After the operation, he developed a fever, and we suspected aspiration pneumonia based on X-ray imaging. We consulted an otolaryngologist and the patient underwent swallowing video endoscopy. Invasion by remaining saliva on the epiglottis vallecular was identified. We restricted eating and drinking and started the patient on swallowing rehabilitation with a speech-language-hearing therapist. His swallowing function improved and we restarted his eating and drinking. He continued with his rehabilitation, was cured of the aspiration pneumonia and had no fever；he was discharged from the hospital 20 days after the operation. Patients with myotonic dystrophy usually have weak breathing and swallowing due to declining muscular strength. Therefore, we need to carefully assess the breathing and swallowing functions of patients with muscular dystrophy before they undergo a head and neck operation.

We encountered a patient with maxillary left central incisor root fracture in whom a bridge was mounted after extraction but it became detached after about one month and we had difficulty in resolving the issue.

The patient was a 46-year-old male with autism spectrum disorder, and he visited our clinic with the chief complaint of maxillary left central incisor mobility. Although the resin veneer metal crown bridge was mounted from the maxillary right central incisor to the left lateral incisor after premedication with diazepam and extraction of the maxillary left central incisor under nitrous oxide inhalation sedation, the bridge became detached in one month. It was found that the patient had a habit of biting and pulling a handkerchief. Pulpectomy of the abutment and attachment of a retainer were performed, but detachment, fracture, and loss frequently occurred thereafter, and finally we abandoned prosthetic treatment. Self-injurious behavior, such as hitting his jaw with his fist, was noted and crown fracture of other teeth occurred.

The patient lives with his elderly mother and the detailed past medical history and state of the patient could not be obtained from her. Although the habit and self-injurious behavior were suspected before treatment, these could not be confirmed. This case highlights the difficulty of performing prosthetic treatment in disabled persons and the necessity of sufficient surveillance of the patient’s living situation and behavior in order to deal with such patients.

The Tsuyama Center for Oral Health was founded in 1988 as a core dental care site for individuals with disabilities in the northern part of Okayama prefecture. While Yakushiji reported on the state of and regional activities related to dental care for disabled individuals at this Center in 2005, no studies have investigated the recent state of such care. Therefore, we investigated the state of new patients who visited the Center over the past 6 years in order to determine the current state and how to respond to continuously changing social conditions, as well as to contribute to future activities.

Of the new patients who visited our Center between 2010 and 2015, we gathered data such as age, disease, and questions from the medical records and medical history forms recorded at the initial visit for 71 patients treated at the Disabled Individual Dental Treatment Department.

Of the subjects, 63％ lived in Tsuyama city, while the remaining patients came from nearby regions. The same trend was seen for age distribution, with approximately half of the patients aged 20 years or younger. In terms of disabilities and diseases of the patients, intellectual disorders accounted for approximately 25％, which was approximately the same percentage as the previous investigation, while autism increased from 16％ to 24％. Disabilities related to the limbs and trunk decreased to fewer than 10％ of patients. Fewer subjects （36％） answered that they visited our Center due to a mental/physical disorder or systemic disease than in the previous investigation （52％）, while an increasing number answered that the reason was wanting to undergo regular checkup or due to being referred.

These results suggested that activities conducted at our Center are highly evaluated regionally, and that the Center should be enhanced as a secondary medical facility.

Objective：In dysphagia during infancy, tube feeding is often selected because congenital disease and immaturity make oral intake difficult. In the development of feeding and swallowing functions, the ability to chew and eat foods is acquired by about one year of age, and therefore to promote the acquisition of feeding and swallowing functions, it is important to provide dysphagia rehabilitation at an early stage. In the present study, we investigated the recent status of dysphagia rehabilitation for infants.

Subjects and methods：The subjects included patients younger than one year of age with dysphagia who consulted our center in fiscal 2015 and 2016. Examination items included disease, age at initial examination, sex, chief complaint, nutrition intake method （at the initial examination and at the outcome）, and outcome.

Results and discussion：The subjects were 77 patients （mean age 5.7±3.1 months, including 47 boys and 30 girls）. Conditions present in a high ratio included heart disease, Down syndrome and neurological disease.

The chief complaints were ‘presence of aspiration’ in the age group from birth up to 4 months, and ‘How to eat baby food?’ for those aged over 5 months. Regarding nutrition intake method, tube nutrition accounted for over half at the initial visit, but this ratio had reduced to 19％ at the time of outcome.

The need for continued dysphagia rehabilitation was observed in 80％ of patients overall, suggesting the importance of cooperation with hospitals as well as regional clinics.

In recent years, the need for professional dental treatment for patients with disabilities has increased, and thus the necessity of training dentists to treat them has also increased.

The Special Care Dentistry Division of the Osaka University School of Dentistry has been giving second-year dental students the opportunity to visit a nursing home for persons with disabilities as the first step in studying special care dentistry.

We verified the effect of this educational visit by qualitatively examining the students’ reports after the visit and evaluating their learning and change of perspective of disabled people through the visit.

The students’ learning and perspective of disabled people, derived from the qualitative research of the reports, can be summarized as follows. 1） Emotional changes through the educational visit, 2） Formation of students’ perspective of disabled people, 3） Learning about the oral condition of persons with disabilities, and 4） Awareness of special care dentistry. Many students’ feelings changed from negative to positive through the visits and some of them described how to interact with persons with disabilities and what disabilities are.

These results showed that many students have prejudice due to lack of knowledge about disabilities. However, the students may have learned about various communication methods and the characteristics of persons with disabilities through the education. Furthermore, it is thought that the students could understand the difficulty of daily oral care for persons with disabilities and discovered for themselves how to interact with persons with disabilities and problems such as the necessity of visiting dental treatment.

It is suggested that it is important to give dental students the opportunity to visit nursing homes for persons with disabilities in order to understand such disabilities. It is also suggested that such opportunities motivate students to study special care dentistry and to discover the dental problems of persons with disabilities by themselves.