Management of Upper Airway Obstruction by Non-Invasive CPAP in Neonates with Pierre Robin Sequence

Background: Neonates with Pierre Robin Sequence (PRS) may present upper airway obstruction (UAO) which is generally managed by surgery. Noninvasive continuous positive airway pressure (CPAP) could be an effective treatment for severe UAO in PRS. Aim of the study: To retrospectively analyse over a one-year period a standardized protocol of UAO management in a national PRS reference centre with the use of CPAP as first line treatment for severe UAO. Material and Methods: All neonates with PRS requiring a hospitalization for respiratory or feeding problems were analyzed and divided in 3 groups; those dependent on continuous ventilatory support: the neonatal intensive care unit (NICU group); those with less severe UAO in whom a polygraphy (PG) could be performed (UAO group); and those with normal breathing and overnight pulse oximetry (no UAO group). CPAP was used in the NICU group and in those with an abnormal PG. Results: The data of 37 neonates were analyzed. The NICU group comprised 9 neonates; 5 were successfully managed by CPAP and 4 required a tracheotomy because of total ventilatory dependence or CPAP failure. The UAO group comprised 11 neonates; 7 had a normal PG and gas exchange with prone positioning and 4 an abnormal PG (apnea-hypopnea index 19-42/h, oxygen desaturation index 18-137/h). These 4 patients were successfully managed by CPAP. CPAP patients were successfully discharged home. Evolution was favorable in all patients. Conclusion: CPAP represent an effective non-invasive treatment of moderate and severe UAO in neonates with PRS and reduces the need for a tracheotomy. As such, CPAP may be recommended in a selected group of patients with PRS before surgical procedures.