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

Korean Association for Disability and Oral Health (대한장애인치과학회)
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DENTAL TREATMENT OF A PATIENT WITH HADDAD SYNDROME UNDER GENERAL ANESTHESIA: CASE REPORT

Haddad syndrome 환아의 전신마취 하 치아우식 치료: 증례보고

  • Suh, Heewon (Department of Pediatric Dentistry, School of Dentistry, Seoul National University) ;
  • Song, Ji-Soo (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) ;
  • Jang, Ki-Taeg (Department of Pediatric Dentistry, School of Dentistry, Seoul National University)
  • 서희원 (서울대학교 치의학대학원 소아치과학교실) ;
  • 송지수 (서울대학교 치의학대학원 소아치과학교실) ;
  • 신터전 (서울대학교 치의학대학원 소아치과학교실) ;
  • 현홍근 (서울대학교 치의학대학원 소아치과학교실) ;
  • 김영재 (서울대학교 치의학대학원 소아치과학교실) ;
  • 김정욱 (서울대학교 치의학대학원 소아치과학교실) ;
  • 이상훈 (서울대학교 치의학대학원 소아치과학교실) ;
  • 장기택 (서울대학교 치의학대학원 소아치과학교실)
  • Received : 2017.06.03
  • Accepted : 2017.06.21
  • Published : 2017.06.30
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Abstract

Haddad syndrome, the association of Congenital Central Hypoventilation Syndrome (CCHS) and Hirschsprung's disease (HD), is a disease caused by mutation of PHOX2B gene. This disease, which occurs very rarely worldwide, is characterized by adequate ventilation during wakefulness and hypoventilation during sleep due to decreased ventilatory sensitivity. In this case report, we report a case of dental treatment under general anesthesia in a child with Haddad syndrome. A 3-year-old child with Haddad syndrome visited Seoul National University Dental Hospital for dental treatment. Dental treatment was planned under general anesthesia because of his medical condition. Treatment was successfully done without any postoperative complications. This case suggests that general anesthesia may be advantageous for dental treatment due to the risk of respiratory depression in a child with Haddad syndrome.

Haddad 증후군은 Congenital central hypoventilation syndrome과 Hirschsprung's disease가 함께 나타나는 질환으로 수면 시 호흡 저하를 특징으로 하나, 증상이 심할 경우 깨어있을 때에도 호흡 저하가 나타날 수 있다. 따라서 전신 마취 시, 중추성 억제 약물 사용에 주의를 기울여야 하고, 적절한 환기가 이루어지도록 하는 것이 중요하다. 또한 술 후에도 호흡 저하가 일어나지 않도록 주의를 기울여야 한다.

Keywords

References

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