Endoscopic and Image Analysis of the Airway in Patients with Mucopolysaccharidosis Type IVA

Abstract

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.

Airway management of a child with mucopolysaccharidosis undergoing cervical spine surgery: A case report

“…In MPS disorder, the abnormal storage of GAGs in connective tissues and bones may compromise the airway. [ 1 ] A varying degree of airway obstruction was detected via endoscopy and imaging modalities described by Yi-Hao Lee, et al . [ 1 ] The data of 15 MPS cases were reviewed in detail.…”

“…[ 1 ] A varying degree of airway obstruction was detected via endoscopy and imaging modalities described by Yi-Hao Lee, et al . [ 1 ] The data of 15 MPS cases were reviewed in detail. FOB was used to distinguish adeno-tonsillar 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.…”

Partial Obstruction of the Endotracheal Tube by a Part of the Metallic Stylet; Case Report and Review of the Literature

“…Several case reports and retrospective studies have documented partial obstruction of the endotracheal tube due to the metallic stylet becoming dislodged or malpositioned inside the tube. 14–16 These reports underscore the importance of vigilance during intubation and the need to recognize this rare but critical complication early.…”