title: [Fatal stenosis of trachea and main bronchus due to compression by the spine and sternum in three patients with severe motor and intellectual disabilities].
abstract: Three patients with severe motor and intellectual disabilities developed fatal respiratory insufficiencies caused by stenosis of the trachea and main bronchus due to compression by the spine and trachea. The onset of respiratory insufficiency was by 20, 16, and 29 years of age. Chest computed tomography demonstrated deformation and narrowing of the trachea and main bronchus, and shortening of the distance between spine and sternum. Although respiratory insufficiency was temporarily relieved by insertion of a stent into the main bronchus in patient 1, he died at the age of 21 due to proliferation of granulation tissue. Patient 2 died of bleeding from the granulation tissue around the window of tracheotomy at the age of 18 years, and patient 3 died of recurrent pneumonia at the age of 34 years. In conclusion, stenosis of the trachea and bronchus observed in these patients was caused by progressive deformation of the thorax. The stenosis may result in sudden death or recurrent respiratory infections in patients with severe motor and intellectual disabilities.
authors.affiliation: Department of Pediatrics, Shizuoka Higashi Hospital, Shizuoka.
authors.forename: M
authors.initials: M
authors.lastname: Tanaka
authors.forename: Y
authors.initials: Y
authors.lastname: Watanabe
authors.forename: K
authors.initials: K
authors.lastname: Fukushima
authors.forename: T
authors.initials: T
authors.lastname: Fujiwara
authors.forename: K
authors.initials: K
authors.lastname: Yagi
authors.forename: S
authors.initials: S
authors.lastname: Ohta
mesh_terms: D000293:Adolescent; D000328:Adult; D001982:Bronchial Diseases / Q000209:etiology*; D002547:Cerebral Palsy / Q000150:complications*; D003251:Constriction, Pathologic; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D008297:Male; D012131:Respiratory Insufficiency / Q000209:etiology; D013131:Spine / Q000473:pathology*; D013249:Sternum / Q000473:pathology*; D014135:Tracheal Stenosis / Q000209:etiology*