Place of Tracheotomy in Management of Air Way Obstruction for a Child with Hunter Syndrome Case Report

Dr Madi Sihem, Dr Zoheir Kermezli

Abstract: Mucopolysaccharidoses (MPS) are composed of inherited conditions coupled with errant mucopolysaccharide degradation. In connective tissues, since these substances are ubiquitous, the manifestations are large and challenging. Deficiencies in the lysosomal enzyme underlie the inadequate degradation of mucopolysaccharides and result in the accumulation in connective tissues of incompletely catabolized substances. There are 11 known deficiencies of enzymes that generate seven recognized MPS phenotypes. It has defined subtypes.11 In patients with type mucopolysaccharidosis (MPS), progressive multi-level upper airway obstruction can develop. Tracheostomy remains an effective technique to protect the airway where other procedures have failed or when the airway obstruction requires several locations, considering the particular challenges posed by these complex patients. The presence of airways is primarily responsible for the substantial anesthetic risk seen in MPS.

Keywords: Tracheostomy, Airway, Mucopolysaccharidoses, Hunter's syndrome, enzyme deficiencies