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Extubation and Decannulation of Unweanable Patients with Neuromuscular Weakness Disorders

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Noninvasive Mechanical Ventilation

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Abstract

Neuromuscular diseases (NMDs) encompass several syndromes and diseases, where respiratory muscle weakness is a major cause of morbidity and mortality. All patients with NMDs should therefore be evaluated for the need for chronic ventilatory support. The first approach to mechanical ventilation is usually with noninvasive positive pressure ventilation (NIV) strategies, whereas invasive ventilation could be required in two different settings: acute respiratory events or when NIV strategies become ineffective. In the first scenario, a trial of NIV might be useful to avoid unnecessary endotracheal intubation (ETT). Hopefully, ETT is successful in bridging the patient to independent respiratory function and extubation. Otherwise, tracheostomy and chronic invasive ventilation become necessary. However, the experience in highly specialized centers suggested successful weaning of NMDs patients, even if they were conventionally considered unweanable from ETT and tracheal mechanical ventilation.

In the second scenario, NMDs patients undergo tracheostomy when NIV strategies become either ineffective, especially in patients with severe bulbar disfunction, or unbearable for the patient. Consequently, the indication for tracheostomy is inability to cooperate with NIV or aspiration of airway secretions that cause a drop in SpO2 despite NIV. Generally, this only occurs in patients with advanced upper motor neuron bulbar such as amyotrophic ALS. In these patients, the skin-lined tracheostomy allows obtaining greater tracheostoma stability, related to epithelialized inlet between the trachea and the anterior cervical skin, with a reduction of the risk of granulation tissue, and an easier and safer home management of the cannula.

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Authors and Affiliations

  1. Department of Surgery, ENT Unit, Ravenna-Lugo Hospital, Health Local Agency of Romagna, Ravenna, Italy

    Andrea De Vito, Damiano Giardini, Emanuele Checcoli & Chiara Martone

  2. Department Thoracic Diseases, Pulmonary Operative Unit, Forlì Hospital, Health Local Agency of Romagna, Forlì, Italy

    Stefano Oldani & Serena Bensai

  3. Department of Surgery, Anaesthesia and Critical Care Unit, Ravenna Hospital, Health Local Agency of Romagna, Ravenna, Italy

    Massimo Terenzoni & Maurizio Fusari

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  1. Andrea De Vito
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  1. Murcia, Spain

    Antonio M. Esquinas

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De Vito, A. et al. (2023). Extubation and Decannulation of Unweanable Patients with Neuromuscular Weakness Disorders. In: Esquinas, A.M. (eds) Noninvasive Mechanical Ventilation. Springer, Cham. https://doi.org/10.1007/978-3-031-28963-7_65

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  • DOI: https://doi.org/10.1007/978-3-031-28963-7_65

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-031-28962-0

  • Online ISBN: 978-3-031-28963-7

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