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Reiter, Karl; Schoen, Carola; Ensenauer, Regina; Nicolai, Thomas; Huber, Rudolf M. (2016): Tracheobronchial stents in mucopolysaccharidosis. In: International Journal of Pediatric Otorhinolaryngology, Vol. 83, No. 4: pp. 187-192
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Introduction: The mucopolysaccharidoses are a group of hereditary disorders pathologically characterized by tissue accumulation of glycosaminoglycans due to deficient lysosomal metabolism which often leads to progressive airway stenosis and respiratory insufficiency. Relentless and treatment-refractory narrowing of the lower airways with ensuing severe limitation of quality of life is a challenging problem in mucopolysaccharidoses. Case reports: We report 2 cases of MPS (Hunter's and Maroteaux-Lamy's syndrome resp.) in whom tracheal stents were placed to relieve severe tracheal obstruction. The first patient could be weaned from mechanical ventilation after stent placement but showed significant long-term stent-related morbidity. The second patient suffered a severe procedure-related complication due to positioning problems typical for MPS. Conclusions: Very good short-term success can be achieved with airway stent placement in patients with MPS and severe lower airway stenosis but a high risk of severe complications and important long-term morbidity have to be weighed against potential individual benefit. (C) 2016 ELSEVIER. All rights reserved.