Awake airway endoscopy in mucopolysaccharidosis: a case report

Fernandes, Sofia; Borges, João Batista; Martins, M. de Pinho

doi:10.1016/j.bjane.2021.06.005

Cited by 1 publication

(1 citation statement)

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“…While previous studies described several types of examinations for airway evaluation in MPS patients, the present study relied on the results of CT and endoscopy for most findings in patients with MPS IVA. FB is the most widely used examination method for both upper and lower airway evaluation and is able to support preoperative decision making and postoperative follow-up [ 6 , 9 , 21 , 22 , 23 , 24 , 25 ]. FB is an office-based procedure that can be performed by an experienced otorhinolaryngologist or pulmonologist transnasally under local anesthesia, with the patient in a sitting position.…”

Section: Discussionmentioning

confidence: 99%

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

Lee

Lin

et al. 2023

JPM

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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.

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