title: Management of Life-Threatening Tracheal Stenosis and Tracheomalacia in Patients with Mucopolysaccharidoses.
abstract: Several different lysosomal storage diseases, mainly mucopolysaccharidosis (MPS) type I, II, and VI, are complicated by severe obstruction of the upper airways, tracheobronchial malacia, and/or stenosis of the lower airways. Although enzyme replacement therapies (ERTs) are available, the impact of these on tracheobronchial alterations has not been reported. By extending the life expectancy of MPS patients with ERTs, airway problems may become more prevalent at advanced ages. These airway abnormalities can result in severe, potentially fatal, difficulties during anesthetic procedures. Usually, upper airway obstruction is treated by tracheostomy. However, with lower airway malacia and/or stenosis, there are no procedures available to date to address these difficulties. We report the first cases using a new technique of tracheal stenting in patients with MPS type VI (Maroteaux-Lamy syndrome) and type II (Hunter syndrome) who had almost complete tracheal occlusion and total airway collapse. An updated literature review is also reported.
authors.affiliation: Center for Diseases in Childhood and Adolescence and Villa Metabolica, Mainz Medical University, Mainz, Germany. kampmann@mail.uni-mainz.de.|Zentrum für Kinder- und Jugendmedizin, Universitätsmedizin Mainz, Langenbeckstrasse 1, 55131, Mainz, Germany. kampmann@mail.uni-mainz.de.
authors.forename: Christoph
authors.initials: C
authors.lastname: Kampmann
authors.affiliation: Center for Diseases in Childhood and Adolescence and Villa Metabolica, Mainz Medical University, Mainz, Germany.
authors.forename: Christiane M
authors.initials: CM
authors.lastname: Wiethoff
authors.affiliation: Center for Diseases in Childhood and Adolescence and Villa Metabolica, Mainz Medical University, Mainz, Germany.
authors.forename: Ralf G
authors.initials: RG
authors.lastname: Huth
authors.affiliation: Department of Pediatric Radiology, Clinic for Radiology, Mainz Medical University, Mainz, Germany.
authors.forename: Gundula
authors.initials: G
authors.lastname: Staatz
authors.affiliation: Center for Diseases in Childhood and Adolescence and Villa Metabolica, Mainz Medical University, Mainz, Germany.
authors.forename: Eugen
authors.initials: E
authors.lastname: Mengel
authors.affiliation: Center for Diseases in Childhood and Adolescence and Villa Metabolica, Mainz Medical University, Mainz, Germany.
authors.forename: Michael
authors.initials: M
authors.lastname: Beck
authors.affiliation: Center for Diseases in Childhood and Adolescence and Villa Metabolica, Mainz Medical University, Mainz, Germany.
authors.forename: Stefan
authors.initials: S
authors.lastname: Gehring
authors.affiliation: ENT Clinic, Mainz Medical University, Mainz, Germany.
authors.forename: Torsten
authors.initials: T
authors.lastname: Mewes
authors.affiliation: Center for Diseases in Childhood and Adolescence and Villa Metabolica, Mainz Medical University, Mainz, Germany.
authors.forename: Tariq
authors.initials: T
authors.lastname: Abu-Tair