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Introduction
Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease that eventually affects the respiratory muscles. In most cases of the slow progression of respiratory failure, noninvasive ventilation (NIV) is the first treatment option.1 NIV treatment provides a better quality of life and is, therefore, strongly recommended; however, its use must consider the type of ALS.2 Severe bulbar dysfunction limits NIV treatment.3 Aside from additional NIV options in ALS, including mouthpiece ventilation and intermittent abdominal pressure ventilation, prolonged use of a ready-made face mask is impractical for several reasons. Cognitive impairments can affect the maintenance of NIV.4 Hypersalivation and bronchial secretion disrupting ventilation can lead to poor NIV adherence. Pressure injuries are another clinical issue. The incidence of pressure injuries associated with NIV masks ranges from 10–31%.5 Long-term use of NIV face masks can result in pressure injuries at the contact area, and skin-protective patches may not prevent skin problems.6 Patients with bulbar ALS frequently have postural facial deformations, and these can lead to difficulties in wearing the NIV mask.7 No previous study has analyzed morphological differences in facial soft tissue according to posture in ALS.
The aim of this study was to compare the deformations of the face in different postures in subjects with bulbar ALS and healthy participants. This study also virtually fitted an NIV mask to understand how the NIV mask interface affects the face in different postures.
Methods
This study was an observational study approved by the institutional review board of a local ethical committee (Pusan National University Yangsan Hospital, 05-2021-034). The participants had no history of facial surgery or facial palsy. Participants with neurologic diseases other than ALS were excluded. Fourteen healthy volunteers and 14 subjects with bulbar ALS were recruited. All participants gave written informed consent.
The face was …
Correspondence: Sungchul Huh, Department of Rehabilitation Medicine, Pusan National University Yangsan Hospital, 20 Geumo-ro, Mulguem, Yangsan, Gyeongnam 50612, Republic of Korea. E-mail: dr.huhsc{at}gmail.com
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