Journal of the korean academy of Pediatric Dentistry (대한소아치과학회지)

Korean Academy of Pediatric Dentistry (대한소아치과학회)
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A CASE REPORT AND DENTAL TREATMENT CONSIDERATIONS OF FIBRODYSPLASIA OSSIFICANS PROGRESSIVA PATIENT

Fibrodysplasia ossificans progressiva 환자의 증례보고 및 치과 치료시 고려 사항

  • Kweon, Young-Sun (Department of Pediatric Dentistry, School of Dentistry, Seoul National University) ;
  • Hyun, Hong-Keun (Department of Pediatric Dentistry, School of Dentistry, Seoul National University) ;
  • Kim, Young-Jae (Department of Pediatric Dentistry, School of Dentistry, Seoul National University) ;
  • Jang, Ki-Taeg (Department of Pediatric Dentistry, School of Dentistry, Seoul National University) ;
  • Lee, Sang-Hoon (Department of Pediatric Dentistry, School of Dentistry, Seoul National University) ;
  • Kim, Chong-Chul (Department of Pediatric Dentistry, School of Dentistry, Seoul National University) ;
  • Hahn, Se-Hyun (Department of Pediatric Dentistry, School of Dentistry, Seoul National University) ;
  • Kim, Jung-Wook (Department of Pediatric Dentistry, School of Dentistry, Seoul National University)
  • 권영선 (서울대학교 치과대학 소아치과학교실) ;
  • 현홍근 (서울대학교 치과대학 소아치과학교실) ;
  • 김영재 (서울대학교 치과대학 소아치과학교실) ;
  • 장기택 (서울대학교 치과대학 소아치과학교실) ;
  • 이상훈 (서울대학교 치과대학 소아치과학교실) ;
  • 김종철 (서울대학교 치과대학 소아치과학교실) ;
  • 한세현 (서울대학교 치과대학 소아치과학교실) ;
  • 김정욱 (서울대학교 치과대학 소아치과학교실)
  • Received : 2010.11.20
  • Accepted : 2011.01.10
  • Published : 2011.02.28
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Abstract

Fibrodysplasia ossificans progressiva(FOP) is characterized by episodes of permanent heterotopic ossifications of soft tissues throughout the body. FOP is inherited in an autosomal dominant pattern, but most cases result from new mutations in the ACVR1 gene. Even minimal trauma can cause permanent ossifications of soft tissues and give rise to complications following routine dental care. Dental block anesthesia, severe stretching of the jaw and biopsies are all contraindicated in children with FOP. There is no effective treatment. Since the prevalence of FOP is very low and most patients with FOP are misdiagnosed during childhood, they undergo dangerous and unnecessary treatment that can lead to permanent harm. For patients with FOP, early diagnosis and prevention of complications are most important. This study aims at contemplating the characteristic features and consideration factors for the dental treatment of FOP patients in relation to the case of an eight years-and-one month old boy who was referred to Pediatric Dentistry due to prolonged retention of mandibular right and left deciduous central incisors after being diagnosed with sporadic FOP at Pediatric Orthopaedics, Seoul National University Hospital, and received dental treatment without the exacerbation of the FOP symptoms.

Fibrodysplasia ossificans progressiva(FOP)는 연조직의 진행성 이소성 골경화를 특징으로 한다. 상염색체 우성 유전을 따르지만 대부분의 FOP 환자는 ACVR1 유전자의 새로운 돌연변이로 인해 나타난다. 아주 작은 외상조차도 연조직의 영구적인 골경화를 유발할 수 있기 때문에 일반적인 치과치료에 의해서도 합병증이 생길 수 있다. FOP 환자의 치과치료 시 전달마취, 과도한 근육의 스트레칭, 생검 등은 모두 금기이다. 현재까지 알려진 FOP의 효과적인 치료법은 없다. FOP는 유병률이 매우 낮으며 초기에는 오진되는 경우가 많아 부적절한 처치로 인해 증상을 더욱 악화시키는 경우가 많다. 따라서 조기진단 및 합병증 발생을 줄이기 위한 예방적 조치가 가장 중요하다. 본 증례는 서울대학교병원 소아정형외과에서 특발성 FOP로 진단을 받고 하악 좌, 우측 유중절치의 만기잔존을 주소로 소아치과에 의뢰된 8세 1개월의 남아에서 증상의 악화없이 치과치료를 시행하였기에 이와 관련하여 FOP환자의 특징적 소견 및 치과치료 시 고려사항을 고찰하기 위함이다.

Keywords

References

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