Efficacy and outcomes of long-term non-invasive ventilation in children with Robin sequence.

Purpose: To describe the clinical characteristics and outcomes of children with Robin sequence (RS) using non-invasive ventilation (NIV), including continuous and bilevel positive airway pressure, and to compare these parameters to other children using this technology.

Methods: This study is a sub-study of a multicenter retrospective 10-year cohort of children using long-term NIV. Children with RS were identified from medical chart review and matched by age, sex, and year of initiation to other children in the cohort. Clinical characteristics, NIV technology, and treatment effect and outcomes were extracted from the medical chart.

Results: From 622 children in the NIV cohort, 13 had RS and were matched to 39 comparators. Age at NIV initiation and comorbidities did not differ between groups. Use of gastrostomy/nasogastric tubes was higher in children with RS (OR 3.0, 95% CI 1.17-7.69). Neither the proportion of children with obstructive sleep apnea or obstructive apnea-hypopnea index ≥ 10 events/h differed between groups. Improvements in respiratory, oxygen, and carbon dioxide parameters between the diagnostic and treatment polysomnography were similar for children with RS and the comparison group. NIV start location was predominantly at home and for used during sleep in both groups. Children with RS used NIV for a median of 1.45 (IQR 1.85) years. The most common reason for stopping NIV in both groups was an improvement in the underlying condition.

Conclusions: Children with RS have similar characteristics and outcomes to other children using NIV. A high proportion of children with RS cease NIV use because of improvements in the underlying primary condition leading to NIV.