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.

Introduction: Mucopolysaccharidosis (MPS) type IVA usually results in airway obstruction due to thoracic cage deformity and crowding of intrathoracic structures, causing tracheal compression by the tortuous innominate artery. Objectives: To offer an alternative and effective method in dealing with the challenged deformity of the airway in patients with MPS type IVA. Methods: We present 3 patients with MPS type IVA who underwent airway stenting using Montgomery ^® T-tube stents. Three-dimensional reconstructed computed tomography was essential to design the T-tube and evaluate the anatomical relationship between the innominate artery and the trachea. The Y-shaped Montgomery ^® Pediatric Safe-T-Tube™ is more suitable for MPS type IVA. Regular follow-ups using fiberoptic bronchoscopy are necessary to evaluate the complications. Results: All 3 patients had good outcomes during the follow-ups until present, despite the complication of granulation formation, which was resolved by revising the limbs of the T-tube. Conclusions: T-tube stents placed below the vocal cord may restore airway patency and preserve laryngeal function, including respiration, phonation, and swallowing, in patients with MPS type IVA.