Geometrical Facial Deformation According to Posture and Non-invasive Ventilation Mask Virtual Fit in Amyotrophic Lateral Sclerosis

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. NIV treatment provides a better quality of life and is, therefore, strongly recommended; however, its use must consider the type of ALS. Severe bulbar dysfunction limits NIV treatment. 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. 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%. 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. Patients with bulbar ALS frequently have postural facial deformations, and these can lead to difficulties in wearing the NIV mask.

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.