Montgomery© T-tubes in the management of multilevel airway obstruction in mucopolysaccharidosis

Soni-Jaiswal, Archana; Penney, Susannah; Jones, Simon A.; Walker, R; Rothera, M P; Bruce, Iain

doi:10.1016/j.ijporl.2014.06.015

Cited by 7 publications

(5 citation statements)

References 16 publications

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“…According to Yeung et al [7], clinically significant airway obstruction was found in 70% of patients, being tracheostomy required in 11%. For Soni Jaswiel et al [11], more than 16% of MPS children will require tracheostomy, either as a consequence of complete airway obstruction or extubation failure following general anesthesia. In these patients, tracheostomy is associated with significant morbidity, which may in part be attributed to tracheobronchial abnormalities (narrow trachea, short neck, involvement of the cervical spine and difficulty in neck extension).…”

Section: Laryngeal Tracheal and Bronchial Disease In The Mucopolysamentioning

confidence: 99%

Laryngeal, Tracheal, and Bronchial Disease in the Mucopolysaccharidoses: Endoscopic Study

et al. 2020

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Mucopolysaccharidoses (MPS) are genetically determined diseases, leading to a deficiency of enzymes in the glycosaminoglycan (GAG) degradation pathway. The accumulation of GAG occurs in connective tissue in various organs and systems of the body, including the larynx, trachea, and bronchi. Respiratory symptoms are common and severe in these patients, and respiratory disease is a frequent cause of death. A cross-sectional study with flexible bronchoscopy was conducted in 30 MPS patients (6 MPS I, 8 MPS II, 2 MPS III, 3 MPS IV-A, and 11 MPS VI). Only four patients (13.33%) had a normal airway; nine (30%) had mild to moderate disease, 12 (40%) moderate to severe, and five patients (16.67%) had severe disease. Of particular interest, neuronopathic MPS II had the largest proportion of tracheostomized patients who died due to respiratory complications; in MPS IV-A, all patients had significant tracheobronchial deformity with associated tracheomalacia, despite lacking laryngeal involvement. Laryngotracheobronchial disease (LTBD) was associated to longer disease history and was significantly more severe in older patients. Longer use of enzyme replacement therapy did not prevent the progression of LTBD, although the age of therapy introduction may be a crucial factor in lower airway involvement.

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Section: Laryngeal Tracheal and Bronchial Disease In The Mucopolysamentioning

confidence: 99%

Laryngeal, Tracheal, and Bronchial Disease in the Mucopolysaccharidoses: Endoscopic Study

et al. 2020

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“…Surgical intervention most commonly takes the form of adenotonsillectomy but may include microlaryngeal surgery, tracheostomy, or Montgomery t-tube insertion in severe cases. Nocturnal intervention involves support with continuous positive airways pressure (CPAP 13,14 ).…”

Section: Introductionmentioning

confidence: 99%

Obstructive Sleep Apnea in MPS

Pal

Brown

Jones

et al. 2015

Journal of Inborn Errors of Metabolism and Screening

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The mucopolysaccharidoses (MPSs) are a group of inherited, metabolic disorders characterized by progressive multisystem accumulation of partially degraded glycosaminoglycans. This manifests with multilevel airway obstruction, presenting with obstructive sleep apnea (OSA). We systematically reviewed the literature to determine the severity and prevalence of OSA in MPS based on polysomnography analysis. Fifteen studies with 294 participants met the inclusion criteria for review. The pretreatment prevalence of OSA in MPS was 81% with a mean apnea-hypopnea index (AHI) of 10.4. Patients with MPS I are most significantly affected, with 75% suffering with moderate to severe OSA (mean AHI, 16.6). Enzyme replacement therapy (ERT) results in an almost significant reduction in OSA in MPS I (P ¼ .06), while adenotonsillar surgery significantly improves AHI (P ¼ .002). Obstructive sleep apnea least affects MPS III. There is a lack of long-term post-ERT and hematopoietic stem cell transplant data relating to OSA outcomes in this population, with further prospective studies required to determine the ongoing response to treatment.

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“…However, most patients had only short-term benefits from this type of stent, and their responses were complicated by considerable granulation formation due to mucosal inflammation and GAGs deposition [ 37 , 38 ]. The T-tube is a good alternative stenting material first used in patients with MPS I, II, and VI by Soni-Jaiswal et al [ 40 ]. Our team also first applied the T-tube in patients with MPS type IVA (patients No.…”

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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Montgomery© T-tubes in the management of multilevel airway obstruction in mucopolysaccharidosis

Cited by 7 publications

References 16 publications

Laryngeal, Tracheal, and Bronchial Disease in the Mucopolysaccharidoses: Endoscopic Study

Laryngeal, Tracheal, and Bronchial Disease in the Mucopolysaccharidoses: Endoscopic Study

Obstructive Sleep Apnea in MPS

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

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