LAUSR

Use of chronic non-invasive-positive-pressure ventilation (NIPPV) in individuals with neuromuscular disease allows for increased independence and mobility in this population. Optimal mask fit is imperative due to the chronic and extensive device use. Commonly cited side effects of improper mask fit include air leak, dermatitis, skin breakdown, nasal discomfort, and claustrophobia. This is a case report of a 46-year-old female with rigid spine muscular dystrophy leading to chronic hypercarbic respiratory failure secondary to neuromuscular weakness and thoracic cage abnormalities. The patient is dependent on continuous use of NIPPV with a PLV 100 device (Philips Respironics; discontinued model). The patient prefers this model because of its unique ventilation delivery mode, which allows her to pause breaths to speak. The patient developed a significant air leak with her nasal mask and was unable to be fitted properly with commercially available nasal masks. In order to minimize her air leak her husband used an innovative approach using 3D printing technology. He created a 3D print of her facial and nose features and then used this to print a 3D mask. Additionally he 3D printed silicone nose clips, that reinforced the seal on the outside of her nose. The patient had a significant decrease in her air leak and subjectively reported improved comfort with use of the 3D printed mask. This represents a case where application of ingenuity and innovative technology improved treatment efficacy and compliance with NIPPV. The combination of 3D custom fit mask (currently available from a limited number of vendors), with custom fit nasal clips may possibly be applied to a wider category of patients with similar complaints of nasal discomfort and frequent air leaks.