TREACHER COLLINS SYNDROME : A CASE REPORT

Park, Ji-Hyun; Kim, Sueng-Hae; Song, Je-Seon; Kim, Seong-Oh; Lee, Jae-Ho

Journal of the Korean Academy of Pediatric Dentistry 2010;37(3):374-380.
Published online August 31, 2010.

TREACHER COLLINS SYNDROME : A CASE REPORT

Ji-Hyun Park, Sueng-Hae Kim, Je-Seon Song, Seong-Oh Kim, Jae-Ho Lee

Department of Pediatric Dentistry and Oral Science Research Center, College of Dentistry, Yonsei University

Treacher Collins 증후군 환아의 증례보고

박지현, 김승혜, 송제선, 김성오, 이제호

연세대학교 치과대학 소아치과학교실 및 구강과학연구소

Correspondence: Jae-Ho Lee, Tel: 02-2228-3173, Email: leejh@yuhs.ac

Received: 2 April 2010 • Accepted: 20 June 2010

Abstract

Treacher Collins syndrome(TCS) is a rare, incurable condition occurring in approximately 1 of 25,000 to 50,000 births. It may occur as a spontaneous mutation out of genetically normal parents or it may be manifested as an autosomal dominant pattern. TCS is characterized by facial deformities such as, underdevelopment of the maxilla, mandible and zygoma, malocclusion, bilateral deformities of auricles, antimongoloid slant of the palpebral fissures. The syndrome is often associated with cleft lip and palate, ear malformations and hearing loss, short stature, and anomalies of the heart and skeleton. Respiratory difficulty associated with air way obstruction may also be observed, and there is considerable difficulty in airway management during general anesthesia. It is necessary that dentists provide safe dental treatments and guidelines to TCS patients by providing adequate understanding about the characteristics of the syndrome and proper ways of managements. The purpose of this study is to report the dental and medical characteristics of the patient who visited the Department of Pediatric Dentistry, Yonsei University for multiple dental caries treatment and to review the literatures of TCS.

Key Words: Treacher Collins syndrome, Facial deformities, Respiratory difficult