The Journal of Korea Assosiation for Disability and Oral Health (대한장애인치과학회지)

Korean Association for Disability and Oral Health (대한장애인치과학회)
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MESIODENS EXTRACTION OF A PATIENT WITH ROBINOW SYNDROME UNDER GENERAL ANESTHESIA

로비노 증후군(Robinow syndrome) 환자의 전신마취 하 과잉치 발치

  • Park, Sung-Hee (Department of Pediatric Dentistry, School of Dentistry, Seoul National University) ;
  • Shin, Teo-Jeon (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) ;
  • Kim, Jung-Wook (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) ;
  • Jang, Ki-Taeg (Department of Pediatric Dentistry, School of Dentistry, Seoul National University)
  • 박성희 (서울대학교 치의학대학원 소아치과학교실) ;
  • 신터전 (서울대학교 치의학대학원 소아치과학교실) ;
  • 현홍근 (서울대학교 치의학대학원 소아치과학교실) ;
  • 김영재 (서울대학교 치의학대학원 소아치과학교실) ;
  • 김정욱 (서울대학교 치의학대학원 소아치과학교실) ;
  • 이상훈 (서울대학교 치의학대학원 소아치과학교실) ;
  • 김종철 (서울대학교 치의학대학원 소아치과학교실) ;
  • 장기택 (서울대학교 치의학대학원 소아치과학교실)
  • Received : 2016.04.15
  • Accepted : 2016.06.20
  • Published : 2016.06.30
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Abstract

Robinow syndrome is skeletal dysplasia with both autosomal dominant and recessive inheritance patterns. It is characterized by short-limbed dwarfism, abnormalities in the head and face, as well as vertebral segmentation. A 2-year-7-month old boy with Robinow syndrome had visited Seoul National University Dental Hospital, for the evaluation of tooth palatal eruption on maxilla. He had micrognathia, delayed tooth eruption, cleft lip with bifid uvula. He also had an erupted mesiodens on the palatal side of maxillary primary incisors, which was tuberculated and 8mm in major diameter. The patient was scheduled for mesiodens extraction under general anesthesia. He was a young child with delayed development, so general anesthesia was inevitable. General anesthesia was induced and maintained with inhalation agent, Sevoflurane. There were no postoperative complications related to anesthesia and dental treatment. Robinow syndrome patients have craniofacial dysmorphism and eruption disorders. Therefore, he requires regular check-ups as well as dental managements.

저자는 과잉치로 인한 불편감을 호소하며 서울대학교치과병원 소아치과에 내원한 2세 7개월의 로비노 증후군 증례를 치료하였기에 문헌 고찰과 함께 보고하는 바이다. 환자의 전신 소견으로는 대두증, 전두부 돌출, 양안격리증, 넓은 안검렬, 들창코, 콧등 함몰, 삼각형의 입술을 포함하는 특이한 안모와 짧은 손,발가락, 소음경을 포함한 잠복고환 및 발달지연이 관찰되었다. 구내 소견으로는 수술받은 구순열과 하악골 저성장, 치아맹출지연, 치은비대, 과잉치가 관찰되었다. 로비노 증후군 환아는 전신마취 하에서 성공적으로 치과 치료가 가능했다. 치과의사는 치과 치료에 의한 스트레스로 인해 근육의 긴장저하, 발달, 호흡, 섭식 등 의 전신적인 상태를 항상 고려해야 한다. 또한, 로비노증후군 환자가 두개 안면부의 이형성과 맹출장애를 가진다는 사실을 알고 접근해야 하며, 환자가 성장함에 따라 적절한 시기에 필요한 치료를 할 수 있도록 주기적인 관찰이 필요하다.

Keywords

References

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