Introduction

The use of long term noninvasive respiratory support is expanding exponentially. The number of children with central nervous system (CNS) disorders treated with noninvasive ventilation (NIV) or continuous positive airway pressure (CPAP) in France has progressed by 155% over the past 20 years.

Aims

To describe the characteristics of children with CNS disorders treated with long-term noninvasive respiratory support in France.

Methods

Data were collected from 27 French paediatric university centres through an anonymous questionnaire filled for every child treated with noninvasive ventilatory support ? 3 months on June 1st, 2019.

Results

182 patients (55% boys, median age 10.2 (1 month-25 years) years) were included: 35 (19%) had non-tumoral spinal cord injury, 22 (12%) CNS tumours, 63 (35%) multiple disabilities, 26 (14%) central alveolar hypoventilation and 36 (20%) other CNS disorders. NIV was used in 75% of patients. The main investigations performed prior to noninvasive ventilatory support initiation were nocturnal gas exchange recordings and poly(somno)graphy (in 63% and 53% of the patients, respectively). CPAP/NIV was started in an acute setting in 10% of the patients. Mean adherence was good with 8.0±3.3 hours/use per night with 12% of patients using treatment < 4 hours/night. Nasal mask was the most common interface (70%). Airway clearance techniques were used by 31% patients.

Conclusion

Noninvasive ventilatory support in children with CNS disorders has increased in France over the last 2 decades. More information is needed on treatment efficacy and patient reported outcome measures (sleep quality, quality of life).