Journal of the korean academy of Pediatric Dentistry (대한소아치과학회지)

Korean Academy of Pediatric Dentistry (대한소아치과학회)
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Dental Management in a Patient with Infantile Osteopetrosis : A Case Report with a 7-Year follow-up

유아기형 골화석증 환자의 치과적 관리 : 7년간의 경과 관찰

  • Cheon, Minkyoung (Department of Pediatric Dentistry, School of Dentistry, Chonnam National University) ;
  • Yang, Sunmi (Department of Pediatric Dentistry, School of Dentistry, Chonnam National University) ;
  • Kim, Jaehwan (Department of Pediatric Dentistry, School of Dentistry, Chonnam National University) ;
  • Choi, Namki (Department of Pediatric Dentistry, School of Dentistry, Chonnam National University) ;
  • Kim, Seonmi (Department of Pediatric Dentistry, School of Dentistry, Chonnam National University)
  • 천민경 (전남대학교 치과대학 소아치과학교실) ;
  • 양선미 (전남대학교 치과대학 소아치과학교실) ;
  • 김재환 (전남대학교 치과대학 소아치과학교실) ;
  • 최남기 (전남대학교 치과대학 소아치과학교실) ;
  • 김선미 (전남대학교 치과대학 소아치과학교실)
  • Received : 2017.10.11
  • Accepted : 2017.11.23
  • Published : 2018.05.31
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Abstract

Osteopetrosis is characterized by impaired osteoclast function and increased bone density. Infantile osteopetrosis is a severe form of the disease and has characteristics such as diffusely sclerotic skeleton, pancytopenia, cranial nerve entrapment, infection susceptibility, and abnormal craniofacial appearance. Patients with infantile osteopetrosis often experience developmental delay, and may have a short life span. A 14-month-old girl with osteopetrosis presented to the department of pediatric dentistry. Incipient caries on deciduous incisors were observed. The patient revisited 4 years of age. Besides medical problems, oral complications such as growth retardation, narrow upper arch, crowding, dental caries, and abnormal tooth development were observed. After consultation with her pediatrician, dental treatments were performed on the deciduous molars under sedation after a prophylactic antibiotic injection. At a periodic follow-up, multiple deciduous teeth were treated and extracted, and oralrehabilitation with a removable partial denture was initiated. Patient with osteopetrosis are highly susceptible to infection because of their compromised immune system and problems associated with wound healing that lead to osteomyelitis or sepsis development. Active participation in dental care for sugar intake management and proper oral hygiene are obligatory.

골화석증은 파골세포의 기능 장애 및 증가된 골 밀도를 보이는 질환으로 그 중 유아기형 골화석증은 심각한 유형이다. 전신의 골경화와 범혈구감소증, 두개 신경 협착, 높은 감염위험성, 두부와 안모의 변형 등 다양한 증상을 유발한다. 대부분의 유아기 골화석증 환자는 발달 지연과 왜소증을 보이며, 조기에 사망에 이를 수 있다. 14개월의 여성 환아가 유전치부위에 초기 우식병소를 주소로 전남대학교 치과병원 소아치과에 내원하였다. 환아는 4세에 재 내원 하였으며 interferon-gamma, erythropoietin 치료를 받고 있었다. 성장 지연, 골격 변형, 좁은 상악궁, 총생, 선천적 영구치 결손, 우식증을 보였다. 소아과 의사와 협진하여 예방적 항생제 투여와 진정요법 후 치과 치료를 진행하였다. 이후 감염이 발생한 다수 유구치를 발치 후 상악에 가철성 연성 의치를 이용하여 구강 재건(rehabilitation)을 시행하였다. 골화석증 환자의 경우, 저하된 면역기능으로 인해 감염에 매우 취약하며, 출혈이나 발치와 연관된 골수염이나 패혈증이 유발될 수 있으므로 소아과 의료진의 협조와 예방적 항생제의 사용에 관한 고려가 간단한 치과시술 시에도 필수적이다. 또한, 당분섭취 제한 및 구강위생관리를 위한 의료진의 적극적인 개입이 필요하다.

Keywords

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