A Case Report of the Dental Procedure in a Patient with PURA Syndrome

<p>PURA syndrome is a type of PURA-related neurodevelopmental disorder which is characterized by moderate to severe delay in intellectual and movement development. In addition, hypotonia, hypothermia, hypersomnia, dysphagia, excessive hiccups, apneas, epileptic seizures, abnormal nonepileptic movements （e.g., dystonia）, and vision impairment are identified as clinical features of this syndrome. There is a risk of complication of congenital heart diseases, urogenital malformations, skeletal abnormalities and endocrine disorders, however, these are reported to be infrequent. In addition, there are several characteristic facial features such as myopathic face and oversized incisors.</p><p>In this study, we report on the dental caries treatment, periodontal treatment and oral hygiene management under nitrous oxide inhalation sedation while administering midazolam for a patient with PURA syndrome.</p><p>The patient was a 10-year-old boy. He had refused dental treatment at General Dentistry, so he was referred to the Oral Health Center of the Kumamoto Dental Association.</p><p>We performed dental treatment and oral hygiene management by nitrous oxide inhalation sedation while administering midazolam, taking into consideration his hypotonia, dysphagia, excessive hiccups. The patient currently visits our Center every two months to receive dental training and oral hygiene management.</p><p>It has been reported that this syndrome is the cause of about 0.3-0.5％ of cases of delayed intellectual development. Clinical diagnostic criteria have not been officially published, yet the number of cases is increasing gradually due to widespread recognition that genome analysis is required for a definitive diagnosis.</p><p>Many children with this syndrome have relatively good receptive language skills and are expected to respond somewhat to dental disease prevention training. Therefore, it is necessary to collaborate with medical departments earlier and educate parents regarding dental caries and oral hygiene management.</p>