SYMPTOMS OF CHILDREN WITH RETT SYNDROME:A CASE REPORT

레트 증후군 환아의 제증상에 관한 증례보고

Rett syndrome is a progressive neurological disorder that occurs exclusively in females. The syndrome is characterized by regression of language, motor development, and stereotypic hand movement. Autistic behavior, breathing irregularities, gait dyspraxia, scoliosis, and seizure are also accompanied. The cause of Rett syndrome is unknown, however, it is believed that the X-chromosome might playa significant role in the development of the syndrome. Patients with this syndrome have unusual oral and/or digital habits such as abnormal chewing pattern, bruxism, hypersalivation, micrognathia, high vaulted palate, tongue protrusion with lower posture of tongue, hand biting, digit-hand sucking. Dentists who are aware of distinct manifestations of Rett syndrome will be able to aid in early diagnosis and treatment of the syndrome. Prior to dental treatment for a patient with the Rett syndrome under sedation or general anesthesia, one should assess the degree of hypersalivation, apnea, severity of autism, expected life span. Early recognition of the syndrome and also dental treatment with established strict preventive guidelines for patients with the Rett syndrome may obviate the necessity of sedation or general anesthesia. Two cases with the Rett syndome were reported. Both patients had most of the above mentioned typical manifestations of the syndrome. Dental treatment for the case 1(8-year-old) including caries control, stainless steel crown, sealant application was performed under general anesthesia. The case 2 could not be undergone the dental treatment due to poor general conditions.

저자는 치아우식증을 주소로 경희의료원 소아치과에 내원한 레트 증후군 환아를 전신마취 하에서 치료를 시행하고 다음과 같은 치의학적 소견을 얻었다. 1. 본 증례에 보고된 레트 증후군 환아들은 비정상적인 저작, 이갈이, 과다한 타액분비, 높고 깊은 상악 구개, 혀의 전방 변위 등의 구강 증상을 보였다. 2. 본 증례에서 보고된 레트 증후군 환아들은 입과 손을 사용하는 습관-손깨물기, 손등을 핥거나 입술로 문지르기, 손가락빨기-을 갖고 있으며 흔히 손을 씻는 듯한 특징적인 상동증 사이에 이러한 동작을 반복하였다. 3. 이상과같은 진단적 요건을 숙지한다면 레트 증후군의 조기 발견에 도움이 될 수 있으며, 치과적 처치시에도 조기치료 및 예방술식을 시행할 수 있어 진정요법이나 전신마취의 필요성을 감소시킬 수 있을 것으로 판단된다.