ABSTRACT
Mucopolysaccharidoses are rare lysosomal storage diseases resulting from defects in lysosomal enzymes involved in degradation of glycosaminoglycans. Different mucopolysaccharidoses are caused by different enzyme deficiencies The anesthetic complications are related to the organs involved. Patients with mucopolysaccharidoses are rare, and few anesthetists encounter such patients. We experienced a case of mucopolysaccharidoses type II. Several endotracheal intubation attempts were tried, but we experienced failed endotracheal intubation. And we decided to proceed with surgery under bag-mask ventilation because of the short operation time. There’s no desaturation time. And the patient’s spontaneous ventilation was recovered and awakened. We have also briefly discussed the pathophysiology, clinical features, and possible airway management options for patients with mucopolysaccharidoses type II.