title: Tracheobronchial stents in mucopolysaccharidosis.
abstract: BACKGROUND
  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.
  
  
  METHODS
  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.
authors.affiliation: University Children's Hospital, Dr. von Haunersches Kinderspital, Ludwig-Maximilians University, Munich, Germany. Electronic address: karl.reiter@med.uni-muenchen.de.
authors.forename: Reiter
authors.initials: R
authors.lastname: Karl
authors.affiliation: University Children's Hospital, Dr. von Haunersches Kinderspital, Ludwig-Maximilians University, Munich, Germany.
authors.forename: Schoen
authors.initials: S
authors.lastname: Carola
authors.affiliation: Research Center, University Children's Hospital, Dr. von Haunersches Kinderspital, Ludwig-Maximilians University, Munich, Germany; Experimental Pediatrics, Department of General Pediatrics, Neonatology and Pediatric Cardiology, University Children's Hospital, Heinrich Heine University Düsseldorf, Düsseldorf, Germany.
authors.forename: Ensenauer
authors.initials: E
authors.lastname: Regina
authors.affiliation: University Children's Hospital, Dr. von Haunersches Kinderspital, Ludwig-Maximilians University, Munich, Germany.
authors.forename: Nicolai
authors.initials: N
authors.lastname: Thomas
authors.affiliation: Division of Respiratory Medicine and Thoracic Oncology, Ludwig-Maximilians University, Munich, Germany.
authors.forename: Rudolf M
authors.initials: RM
authors.lastname: Huber
mesh_terms: D000293:Adolescent; D000402:Airway Obstruction / Q000209:etiology / Q000601:surgery*; D001995:Bronchography; D002648:Child; D006801:Humans; D008297:Male; D016532:Mucopolysaccharidosis II / Q000150:complications / Q000601:surgery*; D009087:Mucopolysaccharidosis VI / Q000150:complications / Q000601:surgery*; D011788:Quality of Life; D015607:Stents; D014057:Tomography, X-Ray Computed; D014132:Trachea / Q000473:pathology / Q000601:surgery; D014135:Tracheal Stenosis / Q000209:etiology / Q000601:surgery*; D014139:Tracheostomy / Q000009:adverse effects / Q000379:methods*
keywords: Airway stenosis; Bronchoscopy; Mucopolysaccharidosis; Tracheal stent