Cornelia de Lange 증후군 환아의 구내증상과 치과치료: 증례보고 = CORNELIA DE LANGE SYNDROME: A CASE REPORT

국문 초록 (Abstract)

Cornelia de Lange 증후군(Cornelia de Lange Syndrome)은 성장지연, 정신지체, 골격과 외모의 이상을 특징으로 하는 선천성 희귀병이다. 대표적 합병증으로는 위-식도 역류증이 있는데, 이로 인한 구토증은 기도를 폐쇄하여 질식의 우려가 있으 므로 전신마취 시 특별한 주의가 요구된다. 10세 4개월 Cornelia de Lange 증후군 환아가 치아우식증을 주소로 서울대학교 치과병원 소아치과에 내원하였다. 임상적 으로 하악 왜소증, 치열 총생, 왜소치, 치아우식증, 치아부식증, 구개수열의 소견을 보였으며, 중증의 정신지체 및 청각장애로 인해 환자 협조도가 부족하여 외래 전신마취 하에 치과치료를 시행하였기에 보고하는 바이다.

다국어 초록 (Multilingual 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.

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