Outcome of paediatric domiciliary mask ventilation in neuromuscular and skeletal disease

Noninvasive positive pressure ventilation delivered by nasal mask or facema sk has been used widely in the last decade to manage chronic ventilatory fa ilure in adults with neuromuscular and chest wall disease, However, it has been thought that paediatric patients would not be able to tolerate masks, and previous anecdotal reports on the paediatric application of mask ventil ation have not assessed the effects on nocturnal and arterial blood gas con trol. Domiciliary mask ventilation has been used in 40 children,vith ventilatory insufficiency due to congenital neuromuscular and skeletal disease aged 9 m onths-16 yrs, Eighteen patients had symptomatic nocturnal hypoventilation, 17 had diurnal ventilatory failure, three were referred for weaning and two had frequent chest infections associated,vith sleep-disordered breathing. Thirty eight of the 40 patients tolerated mask ventilatory support long-ter m. Diurnal mean+/-SD, oxygen tension in arterial blood (Pa,oz) increased fr om 8.5+/-1.8- 10.9+/-1.7 kPa (p<0.001) and meansd carbon dioxide tension in arterial blood (Pa,CO2) fell from 7.0+/-1.6-5.9+/-0.8 kPa (p=0.01) followi ng initiation of ventilatory support. Mean and minimum nocturnal Pa,Oz and peak transcutaneous carbon dioxide tension (Ptc,CO2) (n=21) improved signif icantly. Mask ventilation can be used successfully in young children and reverses ve ntilatory insufficiency due to congenital neuromuscular and skeletal diseas e.