Skip to main content Accessibility help

Failure to control the airway in a patient with Hunter's syndrome

  • Luis Gaitini (a1), Milo Fradis (a1), Sonia Vaida (a1), Geoffrey Collins (a1), Millian Croitoru (a1), Mustafa Somri (a1), Zvi Borochovltz (a1) and Avishai Golz (a1)...


Hunter's syndrome is due to recessively inherited mucopolysaccharide storage diseases. The anaesthetic complications in this syndrome are related to the organs involved in the disease, but pertain especially to upper airway obstruction problems. We report a patient with Hunter's syndrome who, while under general anaesthesia for a simple procedure (the introduction of bilateral grommets), suffered a fatal outcome.


Corresponding author

Address for correspondence: Milo Fradis, M.D., Department of Otolaryngology – Head and Neck Surgery, Bnai Zion Medical Center, P.O.B. 4940, 31048 Haifa, Israel.


Hide All
American Society of Anesthesiologists (1993) Practice guidelines for management of the difficult airway. A report by the American Society of Anesthesiologists Task Force on Management of the Difficult Airway. Anesthesiology 78: 597601.
Baines, D., Keneally, J. (1983) Anaesthetic implications of the mucopolysaccharidoses: a 15-year experience in a children's hospital. Anaesthesia and Intensive Care 11: 198:202.
Bredenkamp, J. K., Smith, M. E., Dudley, J. P., Williams, J. C., Crumley, R. L., Crockett, D. M. (1992) Otolaryngologic manifestations of the mucopolysaccharidoses. Annals of Otology, Rhinology and Laryngology 101: 472478.
Herrick, I. A., Rhine, E. J. (1988) The mucopolysaccharidoses and anaesthesia: a report of clinical experience. Canadian Journal of Anaesthesia 35: 6773.
Kempthorne, P. M., Brown, T. C. K. (1983) Anaesthesia and the mucopolysaccharidoses: a survey of techniques and problems. Anaesthesia and Intensive Care 11: 203207.
Moores, C., Rogers, J. G., McKenzie, I. M., Brown, T. C. (1996) Anaesthesia for children with mucopolysaccharidoses. Anaesthesia and Intensive Care 24: 459463.
Semenza, G. L., Pyeritz, R. E. (1988) Respiratory complications of mucopolysaccharidestorage disorders. Medicine 67: 209219.
Shapiro, J., Strome, M., Crocker, A. C. (1985) Airway obstruction and sleep apnea in Hurlers and Hunter's syndromes. Annals of Otology, Rhinology and Laryngology 94: 458461.
Woolley, M. M., Morgan, S., Hays, D. M. (1967) Heritable disorders of connective tissue. Surgical and anesthetic problems. Journal of Pediatric Surgery 2: 325331.
Young, I. D., Harper, P. S. (1982) Mild form of Hunter's syndrome: clinical delineation based on 31 cases. Archives of Disease in Childhood 57: 828836.
Zlotogora, J., Schaap, T., Zeigler, M., Bach, G. (1991) Hunter's syndrome in Jews in Israel: further evidence for prenatal selection favouring the Hunter allele. Human Genetics 86: 531533.



Full text views

Total number of HTML views: 0
Total number of PDF views: 0 *
Loading metrics...

Abstract views

Total abstract views: 0 *
Loading metrics...

* Views captured on Cambridge Core between <date>. This data will be updated every 24 hours.

Usage data cannot currently be displayed