Algorithm for Airway Management in Patients with Pierre Robin Sequence

Purpose: Airway management in neonates with Pierre Robin sequence (PRS) can be challenging. The goal was to describe the algorithm developed by the authors over the past 8 years. Methods: A retrospective case series analyzing airway management in neonates with PRS admitted to the neonatal intensive care unit at a tertiary care pediatric hospital was performed. The utility of the proposed algorithm for airway management incorporating more consistent use of polysomnography (PSG), and airway assessment was assessed. Results: A total of 31 neonates with PRS (12 men, 19 women) with a mean gestational age of 38.2 weeks were analyzed. Thirteen (41.9%) patients had a named syndrome, chromosomal abnormality, or global delay. Twenty (64.5%) patients had pre-intervention PSG, and severe obstructive sleep apnea with an apnea-hypopnea index (AHI)≥10events/hour was identified in 19 (95.0%). Mandibular distraction osteogenesis was performed in 18 (58.1%) patients, and improved the AHI on post-operative PSGs. Direct assessment of the upper and lower airways was performed in 19 patients, and 13 (68.4%) were found to have secondary airway pathology. Presence of a concomitant syndrome was significantly associated with need for tracheostomy. Conclusion: The algorithm differs from previous ones in that it relies on rigorous pre- and post-intervention PSG (including with a nasopharyngeal airway), as well as that it allows flexibility between treatment options given the whole-patient clinical scenario and endoscopic findings. Results from these studies may be integrated to stratify patients into those who are most likely to benefit from conservative interventions or surgical procedures.