https://scholars.lib.ntu.edu.tw/handle/123456789/634943
標題: | Endoscopic and Image Analysis of the Airway in Patients with Mucopolysaccharidosis Type IVA | 作者: | Lee, Yi-Hao Su, Chin-Hui CHE-YI LIN Lin, Hsiang-Yu Lin, Shuan-Pei Chuang, Chih-Kuang Lee, Kuo-Sheng |
關鍵字: | Morquio syndrome; T-tube; airway obstruction; computed tomography; fiberoptic bronchoscopy; glycosaminoglycans (GAGs); mucopolysaccharidosis (MPS); tracheal stenosis | 公開日期: | 9-三月-2023 | 卷: | 13 | 期: | 3 | 來源出版物: | Journal of personalized medicine | 摘要: | Mucopolysaccharidosis (MPS) is a hereditary disorder arising from lysosomal enzymes deficiency, with glycosaminoglycans (GAGs) storage in connective tissues and bones, which may compromise the airway. This retrospective study evaluated patients with MPS type IVA with airway obstruction detected via endoscopy and imaging modalities and the effects of surgical interventions based on symptoms. The data of 15 MPS type IVA patients (10 males, 5 females, mean age 17.8 years) were reviewed in detail. Fiberoptic bronchoscopy (FB) was used to distinguish adenotonsillar hypertrophy, prolapsed soft palate, secondary laryngomalacia, vocal cord granulation, cricoid thickness, tracheal stenosis, shape of tracheal lumen, nodular deposition, tracheal kinking, tracheomalacia with rigid tracheal wall, and bronchial collapse. Computed tomography (CT) helped to measure the deformed sternal angle, the cross-sectional area of the trachea, and its narrowest/widest ratio (NW ratio), while angiography with 3D reconstruction delineated tracheal torsion, kinking, or framework damage and external vascular compression of the trachea. The NW ratio correlated negatively with age (p < 0.01), showing that airway obstruction progressed gradually. Various types of airway surgery were performed to correct the respiratory dysfunction. MPS type IVA challenges the management of multifactorial airway obstruction. Preoperative airway evaluation with both FB and CT is strongly suggested to assess both intraluminal and extraluminal factors causing airway obstruction. |
URI: | https://scholars.lib.ntu.edu.tw/handle/123456789/634943 | ISSN: | 2075-4426 | DOI: | 10.3390/jpm13030494 |
顯示於: | 醫學院附設醫院 (臺大醫院) |
在 IR 系統中的文件,除了特別指名其著作權條款之外,均受到著作權保護,並且保留所有的權利。