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The Maroteaux-Lamy syndrome (Mucopolysaccharidosis type VI) is a rare, inherited metabolic disease that results in progressive tissue accumulation of dermatan-sulfated glycosaminoglycans and inflammatory consequences that almost always affects the heart valves. From the anesthesia point of view, managing the airway and ventilation might be a serious challenge due to specific features of the syndrome. Additionally, it is more than probable that the surgical team will perform a non-straightforward procedure. A 42-year-old male with Marotea
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