Journal of the korean academy of Pediatric Dentistry (대한소아치과학회지)

Korean Academy of Pediatric Dentistry (대한소아치과학회)
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EDWARDS SYNDROME : REPORT OF A CASE

에드워드 증후군 환아의 증례보고

  • Park, Ji-Eun (Dept. of Pediatric Dentistry, School of Dentistry, Dankook University) ;
  • Kim, Seung-Oh (Dept. of Anesthesiology, School of Dentistry, Dankook University) ;
  • Kim, Jong-Soo (Dept. of Pediatric Dentistry, School of Dentistry, Dankook University)
  • 박지은 (단국대학교 치과대학 소아치과학교실) ;
  • 김승오 (단국대학교 치과대학 치과마취학교실) ;
  • 김종수 (단국대학교 치과대학 소아치과학교실)
  • Published : 2008.05.30
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Abstract

Edward's syndrome is a genetic disorder of 18th chromosomal trisomy. Main clinical features of this syndrome include systemic conditions, such as mental retardation, growth retardation, dyspnea, congenital heart disease, and orthopedic disorders, such as flexion deformity and rocker-bottom feet. The characteristics of fetal abnormality are hydramnios, small placenta, common umbilical artery. Infants with Edward's syndrome have very low survival rate. Almost half of them die during fetal stage. Fifty percent of them survive 2 months, and 5 to 10 percent of them survive about 1 year. A 3-year and 7 month old girl visited our dental hospital for the treatment of dental caries. Considering her systemic disease, low body weight, medical history of cardiac surgery, and difficulty in airway management, dental procedure was performed under general anesthesia. We report this case with review of literatures.

에드워드 증후군은 18번 세염색체(trisomy)성 질환으로 신체의 모든 기관에 다수의 영향을 주며 정신 지체, 발육 지연, 호흡 곤란, 선천성 심장 질환 등의 전신 질환과 손가락의 굴곡변형과 족부후방돌출(rocker-bottom feet)의 소견을 보인다. 산모가 에드워드 증후군 환아를 임신했을 경우 양수과다, 작은 태반, 단일 제대 동맥의 소견을 보인다. 에드워드 증후군을 가진 환아는 생존율이 매우 낮다. 절반이 자궁 내에서 사망하며, 출생아의 50%는 생존율이 2개월이고, $5{\sim}10%$는 생존율이 1년 정도이다. 에드워드 증후군을 가진 환아가 충치 치료를 주소로 내원하였다. 환아의 전신 질환과 심장 수술 병력, 저체중, 기도확보 유지가 어려운 점을 고려하여 전신마취 하에 치과 치료를 시행하였다. 저자는 에드워드 증후군 환아의 치과 치료 후 다소의 지견을 얻었기에 보고하는 바이다.

Keywords

References

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