부산대학교 도서관

Methyl CpG binding protein 2 (MECP2) duplication syndrome is a rare genetic disorder caused by duplication of the chromosomal region containing the MECP2 gene. This genetic disease is characterized by early-onset hypotonia, progressive spasticity, severe intellectual disability, recurrent respiratory infections, and refractory epilepsy. Here, we report an experience of daily dental treatment and perioperative management for tooth extraction in a patient with MECP2 duplication syndrome for 6 years from the age of 12. Because he was uncooperative with dental treatment, we focused on training and oral hygiene instruction to improve his acceptance of dental practice. However, because of his strong resistance to dental treatment, we performed psychosedation or intravenous sedation. At the age of 18, extraction of a right impacted third molar under general anesthesia was scheduled. He showed intractable epilepsy, mental retardation, autism disorder and hypoplasia in the middle to lower face, which were characterized by MECP2 duplication syndrome. In addition, he had a history of anaphylaxis to various allergens including foods and drugs. Therefore, we planned to minimize the use of drugs in the perioperative period, and did not use muscle relaxants as much as possible according to previous reports and his medical history. Moreover, we prepared for the possibility of transfer to the intensive care unit in cooperation with the emergency department in case of emergency. In conclusion, we performed the dental treatment using psychosedation or intravenous sedation, because we could not obtain the patient’s cooperation with the dental treatment. If surgical treatment is required, sufficient preparation to prevent perioperative complications is needed for safety management.