Abstract

 

Hurler Syndrome is an inherited and congenital disease, disorder of mucopolysaccharide metabolism α-L

iduronidase enzyme deficiencyis the causeof the disease which effects many organs and tissues.

Present abnormalities accompany to the syndrome frequently cause serious complications during anesthesia which

is performed for surgical or diagnostic approaches. One of the major problem for anesthesia is airway difficulty.

Multiple factors are present in the mucopolysaccharidoses which make the airway management and trachael

intubation potentially hazardous. Cardiovasculary and respiratory pathologies may be the cause of death.

In this report we would like to share our experiences, discuss the anesthetic risks and the management of HS

patients in the light of literature by presenting our anesthetic approaches towards a 3-year-old patient who is

prediagnosed with Hurler Syndrome (Type I Mucopolysaccharidosis).

Key Words: Hurler Syndrome, Anaesthesia, Magnetic Rezonans Imaging (MRI), Intubation Difficulties