title: Three-dimensional CT and histopathological findings of airway malacia in Hunter syndrome.
abstract: A 19-year-old man with known Hunter syndrome presented with dyspnea, and was admitted to our hospital. Bronchoscopy revealed tracheal narrowing with excessive granulation tissue formation in the trachea. Three-dimensional CT clearly demonstrated severe stenosis in the trachea and both main bronchi. Autopsy showed granulomatous tissue proliferation and deposition of mucopolysaccharide in the tracheal wall. We demonstrated the clinico-radiological-pathological correlation of bronchial lesions in Hunter syndrome, and emphasized that three-dimensional CT is helpful in deciding upon therapeutic strategy to treat stenosis in the large airway.
authors.affiliation: Division of Neurology, Department of Internal Medicine, Tokai University School of Medicine, Isehara, Kanagawa, Japan.
authors.forename: Reiko
authors.initials: R
authors.lastname: Nagano
authors.forename: Shunya
authors.initials: S
authors.lastname: Takizawa
authors.forename: Naoki
authors.initials: N
authors.lastname: Hayama
authors.forename: Shinobu
authors.initials: S
authors.lastname: Umemura
authors.forename: Tsuyoshi
authors.initials: T
authors.lastname: Uesugi
authors.forename: Satoko
authors.initials: S
authors.lastname: Nakagawa
authors.forename: Sari
authors.initials: S
authors.lastname: Okamoto
authors.forename: Noriharu
authors.initials: N
authors.lastname: Yanagimachi
authors.forename: Shigeharu
authors.initials: S
authors.lastname: Takagi
mesh_terms: D017809:Fatal Outcome; D006801:Humans; D021621:Imaging, Three-Dimensional / Q000379:methods*; D008297:Male; D016532:Mucopolysaccharidosis II / Q000000981:diagnostic imaging* / Q000473:pathology*; D014057:Tomography, X-Ray Computed / Q000379:methods*; D014135:Tracheal Stenosis / Q000000981:diagnostic imaging* / Q000473:pathology*; D055815:Young Adult