The Impact of MDO on Airway Outcomes in Patients with Pierre Robin Sequence

Abstract

Background: Pierre Robin sequence (PRS), characterized by the triad of retrognathia, glossoptosis, and airway obstruction, remains challenging to treat and no standard treatment protocols exist. Inadequate treatment may lead to obstructive sleep apnea and necessitate more invasive airway interventions. The purpose of this study is to present the results of a single center’s treatment protocol and the postoperative effect on airway status. Methods: A retrospective cohort study of consecutive patients with PRS treated with MDO from 2010 to 2020 was identified. Descriptive statistics and student’s t-tests were analyzed, and a P < .05 was considered statistically significant for all analyses. Results: Eighty-eight patients met inclusion criteria (50% female, 42% with syndromic diagnoses). Mean age at MDO was 151.2 days, with an average of 51.6 ± 34.3 months of post-operative follow up. For the entire cohort, apnea hypopnea index improved significantly from an average of 33.4 pre-operatively to 8.7 post-operatively, P < .0001. Obstructive apnea hypopnea index improved significantly from 27.6 pre-operatively to 5.3 post-operatively, P < .0001. Fourteen patients (15.9%), all with syndromic diagnoses, were tracheostomy dependent preoperatively, only 3 had a tracheostomy at most recent follow up. One patient with a very complex medical history and comorbid syndromic diagnosis required a tracheostomy postoperatively and remained ventilator dependent at most recent follow up, no other patients required supplemental oxygen. Conclusion: When managed with a rigorous protocol for preoperative workup and selection, treatment of patients with symptomatic PRS with MDO demonstrates great success in improving airway status as objectively measured by supine sleep studies and by successful avoidance of tracheostomy and decannulation.