Management of difficult airway in a patient with mucopolysaccharidosis type 6 [Mukopolisakkaridoz tip 6 hastasinda zor hava yolu yönetimi]

Mucopolysaccharidosis type 6 or maroteaux-lamy syndrome is a lysosomal storage disease which is characterized by the accumulation of chondroitin sulfate and dermatan sulfate in the tissue due to the lack of the n-acetyl galactosamine 4-sulfatase enzyme. causes such as short neck,macroglossia,gingival hyperplasia,mandibular abnormalities,airway obstruction,cervical instability makes the airline dominance difficult. accompanying obstructive/restrictive lung damage and cardiovascular problems as the cause of hemodynamic instability after intubation are the possible difficulties in anesthesia sustainment. in this case report,anesthetic management in patients who underwent adenotonsillectomy and attached bilateral ventilation tube with mucopolysaccharidosis type 6 are discussed.