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DENTAL CHARACTERISTICS OF A PATIENT WITH MCCUNE-ALBRIGHT SYNDROME

McCune-Albright syndrome 환자의 치과적 특성

  • Lim, Jae-Young (Department of Pediatric Dentistry, College of Dentistry, Yonsei University) ;
  • Song, Je-Seon (Department of Pediatric Dentistry, College of Dentistry, Yonsei University) ;
  • Lee, KoEun (Department of Pediatric Dentistry, College of Dentistry, Yonsei University) ;
  • Lee, Jae-Ho (Department of Pediatric Dentistry, College of Dentistry, Yonsei University)
  • 임재영 (연세대학교 치과대학 소아치과학교실) ;
  • 송제선 (연세대학교 치과대학 소아치과학교실) ;
  • 이고은 (연세대학교 치과대학 소아치과학교실) ;
  • 이제호 (연세대학교 치과대학 소아치과학교실)
  • Received : 2018.06.15
  • Accepted : 2018.06.28
  • Published : 2018.06.30

Abstract

McCune-Albright syndrome (MAS) is a rare disease characterized by fibrous dysplasia (FD), Cafe-au-lait spots, and endocrine disorder. A 4-year-old girl with MAS visited the clinic with a chief complaint of facial asymmetry and bruxism without any pain. Facial asymmetry and many dental problems such as midline deviation, "ground glass appearance" on the entire jaw, thinned cortical bone, loss of lamina dura and ectopic germs were found. Because of severely displaced tooth germs and FD affected jaw, there is a high possibility of malocclusion during mixed/permanent dentition. It is necessary to observe the eruption pattern periodically. If there are clinical symptoms like an abnormal eruption pattern, facial asymmetry or high caries susceptibility, appropriate interventions of dentist are required.

본 증례에서는 MAS 환자의 치과적인 특성에 관하여 논하였다. 본 환아는 현재 특히 하악 우측골의 팽융과 안면 비대칭 소견을 보이고 있었으며 치아의 교모, 변위, 맹출 지연, 맹출 경로 이상 등의 특징을 보이고 있었다. MAS 환자들에게서는 이러한 증상뿐 아니라 앞서 언급되었던 치아 우식에 이환될 위험성을 높이는 다른 여러 증상들이 발현될 수 있다. 따라서 환아에게 해당 증상이 발생할 수 있음을 보호자에게 설명하였고, 주기적인 관찰을 시행하고 있다. 결론적으로 MAS 환자에서 가장 중요한 것은 정기적인 검진이며, 이러한 검진 과정에서 임상적인 증상들이 나타난다면 치과의사의 교정치료나 충치치료 등의 적절한 개입이 필요하고 환자에게도 교육의 중요성이 더욱 강조된다. 장기적인 검진은 본 환아의 병소에 대한 이해를 높일 것이고 치료 결과의 질을 높여줄 것이라고 판단된다. 소아의 파노라마 방사선 사진 판독 시 치아와 주변 골조직의 주의 깊은 관찰이 동반된다면 이러한 질환의 조기 진단에 도움이 될 것이다.

Keywords

References

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