Journal of the korean academy of Pediatric Dentistry (대한소아치과학회지)

Korean Academy of Pediatric Dentistry (대한소아치과학회)
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Dental and Skeletal Characteristics and Behavioral Aspects of the Patient with Floating-Harbor Syndrome Compared with Twin Sister

Floating-Harbor 증후군 환자와 쌍둥이 여동생의 치성 및 골격성 특성과 행동 양상 비교

  • Jonghwa, Lim (Department of Pediatric Dentistry, School of Dentistry, Kyungpook National University) ;
  • Gimin, Kim (Department of Pediatric Dentistry, School of Dentistry, Kyungpook National University) ;
  • Jaesik, Lee (Department of Pediatric Dentistry, School of Dentistry, Kyungpook National University) ;
  • Soonhyeun, Nam (Department of Pediatric Dentistry, School of Dentistry, Kyungpook National University) ;
  • Hyunjung, Kim (Department of Pediatric Dentistry, School of Dentistry, Kyungpook National University)
  • 임종화 (경북대학교 치의학대학원 소아치과학교실) ;
  • 김기민 (경북대학교 치의학대학원 소아치과학교실) ;
  • 이제식 (경북대학교 치의학대학원 소아치과학교실) ;
  • 남순현 (경북대학교 치의학대학원 소아치과학교실) ;
  • 김현정 (경북대학교 치의학대학원 소아치과학교실)
  • Received : 2021.10.08
  • Accepted : 2021.11.10
  • Published : 2022.05.31
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Abstract

Floating-Harbor syndrome (FHS) is a rare genetic disorder. This report introduced in a patient with FHS. Distinctive facial characteristics, severe skeletal class 3 malocclusion with underdeveloped maxilla and protruded mandible, congenital missing teeth, microdontia and ectopic positions of maxillary teeth were presented in the patient. In his twin sister, mild skeletal class 3 malocclusion with protruded mandible was observed but congenital missing teeth and microdontia were not observed. High-arched palate, narrow V-shaped maxillary arch compared to wide and ovoid mandibular arch and inverse relationship between the maxillary and mandibular intermolar width resulted in posterior crossbite were confirmed by model analysis of the patient. These were not observed in the twins. Behaviorally, poor cooperation during dental treatment because of mental retardation was observed in the patient.

Floating Harbor 증후군 (FHS)은 드문 유전질환이다. 본 증례는 FHS를 가진 환아에 대해 소개하였다. 환아는 독특한 얼굴 형태, 저성장된 상악과 전돌된 하악을 동반한 심한 골격적 3급 부정교합, 결손치, 왜소치, 상악 치아의 이소맹출을 보였다. 쌍둥이 여동생은 하악 전돌을 동반한 경미한 골격성 3급 부정교합은 보였으나 결손치와 왜소치는 보이지 않았다. 높은 구개궁, 넓은 난형의 하악궁에 비해 좁은 V형의 상악궁, 역의 관계의 상하악 구치간 폭경으로 인한 구치부 반대교합이 환자의 모델 분석을 통해 확인되었다. 이러한 특성은 쌍둥이에서는 나타나지 않았다. 행동면에서 환아는 경미한 정신지체로 인해 치과 치료 중 낮은 협조도를 보였다.

Keywords

References

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