SURGICAL-TREATMENT FOR SCHEIES-SYNDROME (MUCOPOLYSACCHARIDOSIS TYPE I-S) - REPORT OF 2 CASES

Scheie's syndrome (mucopolysaccharidosis type I-S) is a rare genetic l ysosomal storage disease affecting mucopolysaccharide metabolism, and is known to include cardiovascular disease. Surgical treatment was car ried out in 2 patients with Scheie's syndrome. Patient 1 was a 56-year -old man with triple-vessel coronary artery disease, who successfully underwent coronary artery bypass grafting. Patient 2 was a 52-year-old man with aortic and mitral valve stenosis, who successfully underwent combined aortic and mitral valve replacement. The literature on Schei e's syndrome associated with valvular and coronary artery disease is a lso reviewed.