CORNELIA DE LANGE SYNDROME: A CASE REPORT

Choi, Eun-Joo; Hyun, Hong-Keun; Kim, Young-Jae; Kim, Jung-Wook; Lee, Sang-Hoon; Kim, Chong-Chul; Hahn, Se-Hyun; Jang, Ki-Taeg

Journal of the Korean Academy of Pediatric Dentistry 2011;38(1):56-61.
Published online February 28, 2011.

CORNELIA DE LANGE SYNDROME: A CASE REPORT

Eun-Joo Choi, Hong-Keun Hyun, Young-Jae Kim, Jung-Wook Kim, Sang-Hoon Lee, Chong-Chul Kim, Se-Hyun Hahn, Ki-Taeg Jang

Department of Pediatric Dentistry, School of Dentistry, Seoul National University

Cornelia de Lange 증후군 환아의 구내증상과 치과치료: 증례보고

최은주, 현홍근, 김영재, 김정욱, 이상훈, 김종철, 한세현, 장기택

서울대학교 치의학대학원 소아치과학교실

Correspondence: Ki-Taeg Jang, Tel: 02-2072-2682, Email: jangkt@snu.ac.kr

Received: 5 October 2010 • Accepted: 15 December 2010

Abstract

Cornelia de Lange syndrome(CdLS) is a well-described multiple malformation syndrome typically involving proportionate small stature, developmental delay, specific facial features, major malformations, and behavioral abnormalities. Dental issues include micrognathia, crowding of teeth, small teeth, absent teeth, poor oral hygiene, and periodontal disease. We present a case of CdLS in a 10-year-old girl. Micrognathia, crowding of teeth, and bifid uvula are the characteristic features of this case. Dental treatments for this child including preventive and restorative procedures were performed under outpatient general anesthesia.

Key Words: Cornelia de Lange syndrome, Dental problem, General anesthesia