An 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) ≥ 10 events/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. Address correspondence and reprint requests to Jeffrey C. Rastatter, MD, Division of Otolaryngology – Head and Neck Surgery, Ann & Robert H. Lurie Children's Hospital of Chicago, 225 E Chicago Ave, Box 25, Chicago, IL 60611; E-mail: jrastatter@luriechildrens.org Received 18 August, 2017 Accepted 29 January, 2018 Presented at the American Academy of Otolaryngology – Head and Neck Surgery Annual Meeting, September 20, 2016, San Diego, CA. The authors report no conflicts of interest. © 2018 by Mutaz B. Habal, MD.

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