Journal of The Korean Dental Society of Anesthesiology (대한치과마취과학회지)

The Korean Dental Society of Anesthsiology (대한치과마취과학회)
권호 표지

Dental Treatment of a 3-Year-Old Male Patient with Haddad Syndrome under Outpatient General Anesthesia

Haddad 증후군 환아의 외래 전신마취 하 치과 치료

  • Jang, Jun-Hyuk (Department of Pediatric Dentistry, School of Dentistry, Seoul National University) ;
  • Shin, Teo-Jeon (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) ;
  • 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) ;
  • Hyun, Hong-Keun (Department of Pediatric Dentistry, School of Dentistry, Seoul National University)
  • 장준혁 (서울대학교 치의학대학원 소아치과학교실) ;
  • 신터전 (서울대학교 치의학대학원 소아치과학교실) ;
  • 김영재 (서울대학교 치의학대학원 소아치과학교실) ;
  • 김정욱 (서울대학교 치의학대학원 소아치과학교실) ;
  • 장기택 (서울대학교 치의학대학원 소아치과학교실) ;
  • 이상훈 (서울대학교 치의학대학원 소아치과학교실) ;
  • 김종철 (서울대학교 치의학대학원 소아치과학교실) ;
  • 현홍근 (서울대학교 치의학대학원 소아치과학교실)
  • Received : 2013.11.29
  • Accepted : 2013.12.18
  • Published : 2013.12.31
Download PDF
⟨ Previous Next ⟩

Abstract

The co-occurrence of congenital central hypoventilation syndrome (CCHS) and Hirschsprung's disease (HD) is termed Haddad syndrome, which is an extremely rare discorder. It was reported first by Haddad in 1978 and there are approximately 60 cases reported in the worldwide literature. Recent studies described that congenital central hypoventilation syndrome had deep relation to the mutation of the PHOX2B gene in its diagnosis and phenotype. This article presents a case report: Dental treatment of a 3-year-old male patient with Haddad syndrome under outpatient general anesthesia. The special considerations of dental care, especially caries theatment of the patient with Haddad syndrome are discussed.

Keywords

References

  1. Haddad GG, Mazza NM, Mellins RB, et al: Congenital failure of automatic control of ventilation, gastrointestinal motility and heart rate. Medicine (Baltimore) 1978; 57: 517-526. https://doi.org/10.1097/00005792-197811000-00003
  2. Brown LK: Hypoventilation syndromes. Clin Chest Med 2010; 31: 249-270. https://doi.org/10.1016/j.ccm.2010.03.002
  3. Trivedi A, Waters K, Nair R, et al: Congenital central hypoventilation syndrome: four families. Sleep Breath 2011; 15: 785-789. https://doi.org/10.1007/s11325-010-0439-z
  4. Croaker GD, Shie E, Cass DT, et al: Congenital central hypoventilation syndrome and Hirschsprung's disease. Arch Dis Child 1998; 78: 316-322. https://doi.org/10.1136/adc.78.4.316
  5. Amiel J, Laudier B, Attie-Bitach T, et al: Polyalanine expansion and frameshift mutations of the paired like homeobox gene PHOX2B in congenital central hypoventilation syndrome. Nat Genet 2003; 33: 459-461. https://doi.org/10.1038/ng1130
  6. Tomycz ND, Haynes RL, Kinney HC, et al: Novel neuropathologic findings in the Haddad syndrome. Acta Neuropathol 2010; 119: 261-269. https://doi.org/10.1007/s00401-009-0599-8
  7. D'Souza S, Khubchandani RP: Haddad syndrome- congenital central hypoventilation associated with Hirschsprung's disease. Indian J Pediatr 2003; 70: 597-599. https://doi.org/10.1007/BF02723168
  8. Haddad GG: Congenital central hypoventilation syndrome (Ondine curse). In: Behrman RE, Kliegman RM, Arvin AM (eds). Nelson Textbook of Pediatrics (18th ed). Philadelphia: W.B. Saunders Company; 2007: 1372-1373.
  9. Samdani PG, Samdani V, Goel A, et al: Congenital central hypoventilation syndrome. Indian J Pediatr 2007; 74: 953-955. https://doi.org/10.1007/s12098-007-0177-9
  10. Sasaki A, Kanai M, Hayasaka K, et al: Molecular analysis of congenital central hypoventilation syndrome. Hum Genet 2003; 114: 22-26. https://doi.org/10.1007/s00439-003-1036-z
  11. Weese-Mayer DE, Berry-Kravis EM, Marazita ML, et al: Idiopathic congenital central hypoventilation syndrome: analysis of genes pertinent to early autonomic nervous system embryologic development and identification of mutations in PHOX2b. Am J Med Genet A 2003; 123A: 267-278. https://doi.org/10.1002/ajmg.a.20527
  12. Matera I, Bachetti T, Ceccherini I, et al: PHOX2B mutations and polyalanine expansions correlate with the severity of the respiratory phenotype and associated symptoms in both congenital and late onset Central Hypoventilation syndrome. J Med Genet 2004; 41: 373-380. https://doi.org/10.1136/jmg.2003.015412
  13. Trochet D, O' Brien LM, Amiel J, et al: PHOX2B genotype allows for prediction of tumor risk in congenital central hypoventilation syndrome. Am J Hum Genet 2005; 76: 421-426. https://doi.org/10.1086/428366
  14. Sochala C, Deenen D, Ville A, et al: Heart block following propofol in a child. Paediatr Anaesth 1999; 9: 349-351. https://doi.org/10.1046/j.1460-9592.1999.00373.x
  15. Strauser LM, Helikson MA, Tobias JD: Anesthetic care for the child with congenital central alveolar hypoventilation syndrome (Ondine's curse). J Clin Anesth 1999; 11: 431-437. https://doi.org/10.1016/S0952-8180(99)00073-2
  16. Wiesel S, Fox GS: Anaesthesia for a patient with central alveolar hypoventilation syndrome (Ondine's Curse). Can J Anaesth 1990; 37: 122-126. https://doi.org/10.1007/BF03007492
  17. Movahed MR, Jalili M, Kiciman N: Cardiovascular abnormalities and arrhythmias in patients with Ondine's curse (congenital central hypoventilation) syndrome. Pacing Clin Electrophysiol 2005; 28: 1226-1230. https://doi.org/10.1111/j.1540-8159.2005.50194.x
  18. Weese-Mayer DE, Silvestri JM, Menzies LJ, et al: Congenital central hypoventilation syndrome: diagnosis, management, and long-term outcome in thirty two children. J Pediatr 1992; 120: 381-387. https://doi.org/10.1016/S0022-3476(05)80901-1
  19. Silvestri JM, Hanna BD, Volgman AS, et al: Cardiac rhythm disturbances among children with idiopathic congenital central hypoventilation syndrome. Pediatr Pulmonol 2000; 29: 351-358. https://doi.org/10.1002/(SICI)1099-0496(200005)29:5<351::AID-PPUL3>3.0.CO;2-Z
  20. Marcus CL, Jansen MT, Poulsen MK, et al: Medical and psychosocial outcome of children with congenital central hypoventilation syndrome. J Pediatr 1991; 119: 888-895. https://doi.org/10.1016/S0022-3476(05)83038-0