The Journal of Korea Assosiation for Disability and Oral Health (대한장애인치과학회지)

Korean Association for Disability and Oral Health (대한장애인치과학회)
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DENTAL MANAGEMENT OF THE RUSSELL-SILVER SYNDROME: CASE REPORT

Russell-Silver Syndrome 환아의 치과적 관리: 증례 보고

  • Kim, Jun-Hhewk (Department of Pediatric Dentistry, College of Dentistry, Yonsei University) ;
  • Sohn, Hyung-Kyu (Department of Pediatric Dentistry, College of Dentistry, Yonsei University) ;
  • Kim, Seung-Hye (Department of Pediatric Dentistry, College of Dentistry, Yonsei University) ;
  • Choi, Hyung-Jun (Department of Pediatric Dentistry, College of Dentistry, Yonsei University) ;
  • Lee, Jae-Ho (Department of Pediatric Dentistry, College of Dentistry, Yonsei University)
  • 김준혁 (연세대학교 치과대학 소아치과학교실) ;
  • 손흥규 (연세대학교 치과대학 소아치과학교실) ;
  • 김승혜 (연세대학교 치과대학 소아치과학교실) ;
  • 최형준 (연세대학교 치과대학 소아치과학교실) ;
  • 이제호 (연세대학교 치과대학 소아치과학교실)
  • Received : 2010.11.25
  • Accepted : 2010.12.22
  • Published : 2010.12.31
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Abstract

Russell-Silver syndrome is a genetic disorder characterized by intrauterine and/or postnatal growth restriction and typical facies. The clinical feature is various due to heterogeneous genetic characters. Their common findings are short stature without catch-up growth, normal head size for age, a distinctive triangular face with prominent forehead and a pointed chin, low set ears and clinodactyly of the fifth fingers. Intraoral features of the syndrome are microdontia, delayed tooth eruption, hypodontia, and crowding. More than 400 case have been reported in the literature, and estimated incidence is from 1 in 3000 to 1 in 100,000. In this case we performed caries treatment under the general anesthesia for the patient with Russell-Silver syndrome. Dentist have to consider microstomia for the management of patients with Russell-Silver syndrome.

1. 러셀-실버 증후군 환아는 특징적인 소구증 및 유아기의 섭식 장애를 보이므로, 출생 후 필요시설소대 절제술을 고려해야 하며 특별히 고안된 구강위생 용품이 구강위생 증진에 도움이 될 수 있다. 2. 러셀-실버 증후군 환아의 우식 치료 시 개구량이 확보되지 않으므로 근육 이완을 위해 전신마취를 고려해야 하며, 소형의 핸드피스 및 버와 같은 기구들을 사용하여 접근하는 것이 치료를 수월케 하며 환아의 불편감을 감소시킬 수 있다.

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