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. 2004;51(3):102–104.

Anesthetic management of a child with congenital central hypoventilation syndrome (CCHS, Ondine's curse) for dental treatment.

Hajime Ishibashi 1, Koji Umezawa 1, Sachiyo Hayashi 1, Koh Shibutani 1
PMCID: PMC2007484  PMID: 15497300

Abstract

Congenital Central Hypoventilation Syndrome (CCHS, also known as Ondine's Curse) is a rare syndrome characterized by apnea, cyanosis, and hypotonia. A 4-year-old, 90-cm, 12-kg girl with CCHS, mental retardation (MR), and Hischsprung's disease (HD) was treated under general anesthesia. Intravenous drugs were not used, but sevoflurane, a volatile anesthetic, was used. As a result, the recovery time from the end of the operation to returning to the ward was very short, only 18 minutes. There was no trouble during the perioperative period. We safely performed general anesthesia and dental treatment for a girl who had CCHS with HD and MR.

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Selected References

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  1. Bolk S., Angrist M., Xie J., Yanagisawa M., Silvestri J. M., Weese-Mayer D. E., Chakravarti A. Endothelin-3 frameshift mutation in congenital central hypoventilation syndrome. Nat Genet. 1996 Aug;13(4):395–396. doi: 10.1038/ng0896-395. [DOI] [PubMed] [Google Scholar]
  2. Brouwers M. J., Driessen J. J., Severijnen R. S. Clinical letter: epidural analgesia in a newborn with Hirschsprung's disease, associated with congenital central hypoventilation syndrome. Eur J Anaesthesiol. 2000 Dec;17(12):751–753. doi: 10.1046/j.0265-0215.2000.00771.x. [DOI] [PubMed] [Google Scholar]
  3. Croaker G. D., Shi E., Simpson E., Cartmill T., Cass D. T. Congenital central hypoventilation syndrome and Hirschsprung's disease. Arch Dis Child. 1998 Apr;78(4):316–322. doi: 10.1136/adc.78.4.316. [DOI] [PMC free article] [PubMed] [Google Scholar]
  4. Hamilton J., Bodurtha J. N. Congenital central hypoventilation syndrome and Hirschsprung's disease in half sibs. J Med Genet. 1989 Apr;26(4):272–274. doi: 10.1136/jmg.26.4.272. [DOI] [PMC free article] [PubMed] [Google Scholar]
  5. Kerbl R., Litscher H., Grubbauer H. M., Reiterer F., Zobel G., Trop M., Urlesberger B., Eber E., Kurz R. Congenital central hypoventilation syndrome (Ondine's curse syndrome) in two siblings: delayed diagnosis and successful noninvasive treatment. Eur J Pediatr. 1996 Nov;155(11):977–980. doi: 10.1007/BF02282890. [DOI] [PubMed] [Google Scholar]
  6. Khalifa M. M., Flavin M. A., Wherrett B. A. Congenital central hypoventilation syndrome in monozygotic twins. J Pediatr. 1988 Nov;113(5):853–855. doi: 10.1016/s0022-3476(88)80016-7. [DOI] [PubMed] [Google Scholar]
  7. Masumoto Kouji, Arima Tohru, Izaki Tomoko, Takahashi Yukiko, Honda Naotoshi, Toyoshima Satoshi, Shimotake Takashi. Ondine's curse associated with Hirschsprung disease and ganglioneuroblastoma. J Pediatr Gastroenterol Nutr. 2002 Jan;34(1):83–86. doi: 10.1097/00005176-200201000-00020. [DOI] [PubMed] [Google Scholar]
  8. Mellins R. B., Balfour H. H., Jr, Turino G. M., Winters R. W. Failure of automatic control of ventilation (Ondine's curse). Report of an infant born with this syndrome and review of the literature. Medicine (Baltimore) 1970 Nov;49(6):487–504. [PubMed] [Google Scholar]
  9. Rohrer Tilman, Trachsel Daniel, Engelcke Gabriele, Hammer Jürg. Congenital central hypoventilation syndrome associated with Hirschsprung's disease and neuroblastoma: case of multiple neurocristopathies. Pediatr Pulmonol. 2002 Jan;33(1):71–76. doi: 10.1002/ppul.10031. [DOI] [PubMed] [Google Scholar]
  10. Sritippayawan Suchada, Hamutcu Refika, Kun Sheila S., Ner Zarah, Ponce Monique, Keens Thomas G. Mother-daughter transmission of congenital central hypoventilation syndrome. Am J Respir Crit Care Med. 2002 Aug 1;166(3):367–369. doi: 10.1164/rccm.2112087. [DOI] [PubMed] [Google Scholar]
  11. Varma R. Raveendra, Narayanankutty P. K., Rajagopalan K., Rajeevan K. Hirschsprung's disease associated with Ondine's curse. Indian J Pediatr. 2002 Nov;69(11):987–988. doi: 10.1007/BF02726021. [DOI] [PubMed] [Google Scholar]
  12. Weese-Mayer D. E., Silvestri J. M., Huffman A. D., Smok-Pearsall S. M., Kowal M. H., Maher B. S., Cooper M. E., Marazita M. L. Case/control family study of autonomic nervous system dysfunction in idiopathic congenital central hypoventilation syndrome. Am J Med Genet. 2001 May 1;100(3):237–245. doi: 10.1002/ajmg.1249. [DOI] [PubMed] [Google Scholar]

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