Airway clearance techniques in neuromuscular disorders: A state of the art review.

This is a unique state of the art review written by a group of 21 international recognized experts in the field that gathered during a meeting organized by the European Neuromuscular Centre (ENMC) in Naarden, March 2017. It systematically reports the entire evidence base for airway clearance techniques (ACTs) in both adults and children with neuromuscular disorders (NMD). We not only report randomised controlled trials, which in other systematic reviews conclude that there is a lack of evidence base to give an opinion, but also include case series and retrospective reviews of practice. For this review, we have classified ACTs as either proximal (cough augmentation) or peripheral (secretion mobilization). The review presents descriptions; standard definitions; the supporting evidence for and limitations of proximal and peripheral ACTs that are used in patients with NMD; as well as providing recommendations for objective measurements of efficacy, specifically for proximal ACTs. This state of the art review also highlights how ACTs may be adapted or modified for specific contexts (e.g. in people with bulbar insufficiency; children and infants) and recommends when and how each technique should be applied.

Noninvasive ventilation as an alternative to endotracheal intubation during tracheotomy in advanced neuromuscular disease.

OBJECTIVE: To compare conventional tracheotomy with endotracheal intubation to tracheotomy with noninvasive positive-pressure ventilation (NPPV) in advanced neuromuscular disease. METHODS: This was a retrospective study of a historical cohort of patients tracheotomized while sedated and intubated versus patients tracheotomized under NPPV and local anesthesia. We recorded previous intubation difficulties, complications (eg, aspiration pneumonia), and hospital stay. RESULTS: Conventional tracheotomy was performed in 7 patients. We performed tracheotomy during NPPV with local anesthesia in 13 patients. All but 3 patients had risk factors for difficult intubation. Hospital stay was 23.3 +/- 10.3 d in the conventional group and 25.3 +/- 12.9 d in the NPPV group (p = 0.87). The number of pneumonias was higher in the conventional-tracheotomy group (4 vs 1, p = 0.03). CONCLUSION: In neuromuscular patients, performing tracheotomy with NPPV and local anesthesia may help avoid endotracheal intubation and reduce morbidity.