Annick Frapin

Long-term non-invasive ventilation in children.

Use of long-term non-invasive ventilation is increasing exponentially worldwide in children of all ages. The treatment entails delivery of ventilatory assistance through a non-invasive interface. Indications for use of non-invasive ventilation include conditions that affect normal respiratory balance (eg, those associated with dysfunction of the central drive or respiratory muscles) and disorders characterised by an increase in respiratory load (eg, obstructive airway or lung diseases). The type of non-invasive ventilation used depends on the pathophysiological features of the respiratory failure. For example, non-invasive ventilation will need to either replace central drive if the disorder is characterised by an abnormal central drive or substitute for the respiratory muscles if the condition is associated with respiratory muscle weakness. Non-invasive ventilation might also need to unload the respiratory muscles in case of an increase in respiratory load, as seen in upper airway obstruction and some lung diseases. Technical aspects are also important when choosing non-invasive ventilation-eg, appropriate interface and device. The great heterogeneity of disorders, age ranges of affected children, prognoses, and outcomes of patients needing long-term non-invasive ventilation underline the need for management by skilled multidisciplinary centres with technical competence in paediatric non-invasive ventilation and expertise in sleep studies and therapeutic education.

Continuous Positive Airway Pressure for Upper Airway Obstruction in Infants with Pierre Robin Sequence.

Summary: The aim of the study was to evaluate noninvasive continuous positive airway pressure as first-line treatment for upper airway obstruction in neonates with Pierre Robin sequence. Forty-four neonates were enrolled and classified in 4 groups: ventilator-dependent patients (severe upper airway obstruction group), patients with clinical upper airway obstruction, an abnormal (moderate upper airway obstruction group) or normal polygraphy (mild upper airway obstruction group), and those with no clinical upper airway obstruction (no upper airway obstruction group). Five neonates in the severe upper airway obstrction group (n = 9) were successfully managed by noninvasive continuous positive airway pressure and 4 required a tracheotomy. The moderate upper airway obstruction group (n = 4) was successfully managed by noninvasive continuous positive airway pressure and the other patients by prone positioning. Continuous positive airway pressure represents an effective treatment of upper airway obstruction in Pierre Robin sequence and may be recommended in selected patients. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

Long term continuous positive airway pressure (CPAP) and noninvasive ventilation (NIV) in children: Initiation criteria in real life

Long term noninvasive continuous positive airway pressure (CPAP) and noninvasive ventilation (NIV) are increasingly used in children but limited information is available on the criteria and conditions leading to the initiation of these treatments. The aim of the study is to describe the objective overnight respiratory parameters and clinical situations that led to the initiation of CPAP/NIV in a pediatric NIV unit.

Obstructive sleep apnea in Down syndrome: Benefits of surgery and noninvasive respiratory support

Children with Down syndrome are at increased risk of obstructive sleep apnea (OSA). The aim of the study was to describe the management of OSA in a large cohort of children with Down syndrome. A retrospective analysis of sleep studies and consequent management was performed for all consecutive Down syndrome patients evaluated between September 2013 and April 2016. The data of 57 patients were analyzed: 51/53 had an interpretable overnight polygraphy and 4 the recording of nocturnal gas exchange. Mean age at baseline sleep study was 6.2 ± 5.9 years. Eighteen patients (32%) had prior upper airway surgery. Mean apnea‐hypopnea index (AHI) was 14 ± 16 events/hr with 41 of the 51 (80%) patients having OSA with an AHI >1 event/hr and 20 patients (39%) having an AHI ≥10 events/hr. Consequently, eight patients (14%) had upper airway surgery. OSA improved in all patients except two who needed noninvasive respiratory support. Nineteen (33%) patients required noninvasive respiratory support. Mean age at noninvasive respiratory support initiation was 7 ± 7 years. On 11 patients with objective adherence data available, mean compliance at 2 ± 1 years of treatment was excellent with an average use per night of 8 hr46 ± 3 hr59 and 9 patients using the noninvasive respiratory support >4 hr/night. Noninvasive respiratory support was associated with an improvement of nocturnal gas exchange. The prevalence of OSA is high in Down syndrome. Upper airway surgery is not always able to correct OSA. Noninvasive respiratory support represents then an effective treatment for OSA and good compliance may be achieved in a majority of patients.

Weaning from long term continuous positive airway pressure or noninvasive ventilation in children

A significant number of children are able to discontinue long term continuous positive airway pressure (CPAP) or noninvasive ventilation (NIV) but the underlying disorders, weaning criteria, and outcome of these children have not been studied.

Outpatient initiation of long-term continuous positive airway pressure in children

Current guidelines recommend initiating continuous positive airway pressure (CPAP) in children during an overnight in‐hospital titration study. Due to a shortage of hospital beds and economic constraints, we started a program for outpatient initiation of CPAP in selected children with obstructive sleep apnea (OSA).