Journal of the korean academy of Pediatric Dentistry (대한소아치과학회지)

Korean Academy of Pediatric Dentistry (대한소아치과학회)
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Oral Features in a Child with Noonan Syndrome : A Case Report

누난 증후군 환자의 구강 내 특징 : 증례 보고

  • Hwang, Inkyung (Division of Pediatric Dentistry, Department of Dentistry, Ewha Womans University Mokdong Hospital) ;
  • Lee, Yeonju (Division of Pediatric Dentistry, Department of Dentistry, Ewha Womans University Mokdong Hospital) ;
  • Sim, Dohee (Division of Pediatric Dentistry, Department of Dentistry, Ewha Womans University Mokdong Hospital) ;
  • Mah, Yonjoo (Division of Pediatric Dentistry, Department of Dentistry, Ewha Womans University Mokdong Hospital)
  • 황인경 (이화여자대학교 의과대학 목동병원 소아치과학교실) ;
  • 이연주 (이화여자대학교 의과대학 목동병원 소아치과학교실) ;
  • 심도희 (이화여자대학교 의과대학 목동병원 소아치과학교실) ;
  • 마연주 (이화여자대학교 의과대학 목동병원 소아치과학교실)
  • Received : 2017.06.26
  • Accepted : 2017.08.29
  • Published : 2018.02.28
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Abstract

Noonan syndrome is characterized by distinctive facial features, short stature, and congenital heart disease. It is a congenital genetic disorder with a prevalence of between 1/1,000 and 1/2,500 in both genders. An 11-year-old boy with Noonan syndrome visited the hospital with an ectopically positioned tooth. A pulmonary stenosis was diagnosed and his growth and development were delayed. In many cases of this diseases there is obvious hemostasis, which he was not experiencing. His facial appearance showed characteristic features of Noonan syndrome. The patient showed a dental class II relationship, labioversion of the upper anterior teeth, and a shallow overbite. Radiographic examination revealed that the upper right canine was ectopically positioned, which led to root resorption of the upper right lateral incisor. A lateral cephalometric radiograph revealed a craniofacial pattern that was within normal limits. Surgical opening and button attachment on the impacted upper right canine were performed and traction was applied on the impacted tooth using a removable appliance. This patient was mildly affected by Noonan syndrome and showed some dental problems. However, few studies have reported the oral characteristics of Noonan syndrome despite its high incidence. Thus, this case report describes the oral features and management of Noonan syndrome.

누난 증후군은 특이한 얼굴 모습, 작은 키, 선천적 심장질환을 보이는 유전 질환으로 성별에 관계없이 신생아 1000 - 2500명 당 1명의 유병률을 보인다. 누난 증후군으로 진단된 11세 소년이 상악 우측 견치의 위치이상을 주소로 내원하였다. 그는 선천적 폐동맥 협착증을 진단받았으며, 성장발달 지연을 보였다. 지혈에는 문제가 없었으며, 얼굴은 누난 증후군의 특징적 모습을 보였다. 구강검사 시 치성 2급 부정교합, 전방돌출 된 전치, 얕은 피개교합을 보였다. 방사선학적 검사상 상악 우측 견치의 위치이상 및 맹출 경로의 이상으로 상악 우측 측절치의 치근 흡수가 관찰되었다. 측모두부 방사선사진상 상하악의 골격적 패턴은 정상범주에 있었다. 가철성 교정장치를 이용하여 매복치를 견인했다. 본 증례의 누난 증후군 증상 및 구강 내 이상 소견은 심하지 않으나 유병률에 비해 많이 다루어지지 않아 본 증례발표를 통해 누난 증후군 환자의 구강 내 특징 및 치과적 관리 방법에 대해 언급하고자 하였다.

Keywords

References

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