Three-dimensional CT and histopathological findings of airway malacia in Hunter syndrome

Reiko Nagano; Shunya Takizawa; Naoki Hayama; Shinobu Umemura; Tsuyoshi Uesugi; Satoko Nakagawa; Sari Okamoto; Noriharu Yanagimachi; Shigeharu Takagi

Three-dimensional CT and histopathological findings of airway malacia in Hunter syndrome

Tokai J Exp Clin Med. 2007 Jul 20;32(2):59-61.

Authors

Reiko Nagano¹, Shunya Takizawa, Naoki Hayama, Shinobu Umemura, Tsuyoshi Uesugi, Satoko Nakagawa, Sari Okamoto, Noriharu Yanagimachi, Shigeharu Takagi

Affiliation

¹ Division of Neurology, Department of Internal Medicine, Tokai University School of Medicine, Isehara, Kanagawa, Japan.

PMID: 21319059

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.

Publication types

Case Reports

MeSH terms

Fatal Outcome
Humans
Imaging, Three-Dimensional / methods*
Male
Mucopolysaccharidosis II / diagnostic imaging*
Mucopolysaccharidosis II / pathology*
Tomography, X-Ray Computed / methods*
Tracheal Stenosis / diagnostic imaging*
Tracheal Stenosis / pathology*
Young Adult