Tracheobronchial Involvement of Mucopolysaccharidosis Type VI.

A 20-year-old male with Mucopolysaccharidosis type VI (Maroteaux-Lamy syndrome) presented with severe mitral valve stenosis, obstructive sleep apnea, and progressive respiratory compromise due to extensive tracheobronchial involvement. Chest CT revealed diffuse tracheal-bronchial wall thickening, fixed tracheal angulation, and significant airway narrowing consistent with glycosaminoglycan accumulation characteristic of MPS VI.