AIRWAY MANAGEMENT STRATEGIES IN INFANTS BORN WITH PIERRE ROBIN SEQUENCE (PRS): A SYSTEMATIC REVIEW AND META-ANALYSIS OF EFFECTIVENESS

Abstract

Background: Pierre Robin sequence (PRS) is characterized by micrognathia, glossoptosis, and upper airway obstruction (UAO). Different management strategies exist, each with its own efficacy and clinical outcomes. Objective: To critically evaluate and compare the efficacy of conservative, orthodontic, and surgical airway interventions in neonates and infants with PRS, using polysomnographic (PSG) and clinical outcomes. Methods: A systematic review and meta-analysis were conducted per PRISMA guidelines. Databases (PubMed, Embase, Medline, etc.) were searched for studies (2017-2025) reporting pre- and post-intervention PSG data. Seventeen studies (n=1,291 patients) met the inclusion criteria. Risk of bias was assessed using the ROBINS-I tool. A random-effects meta-analysis was performed for Mandibular Distraction Osteogenesis (MDO) outcomes. Results: This study analysis reveals a critical finding: while all modalities can be effective, their efficacy is stratified. Conservative management (prone positioning, nasopharyngeal airway) achieved significant improvement, with 84.2% of patients attaining an AHI <5, supporting its role as a first-line strategy. However, MDO demonstrated superior and statistically significant efficacy in resolving severe obstruction, with meta analysis showing drastic improvements in OAHI (MD: -36.29, CI: -43.39 to -29.18, p<0.00001), AHI (p<0.00001), SpO₂ nadir (MD: +12.91%, p<0.00001), and end-tidal CO₂ (p<0.00001). Notably, MDO did not significantly improve weight gain (p=0.24) or sleep efficiency (p=0.51). Tongue-lip adhesion (TLA) was effective (89.5% achieved AHI<5) but was associated with higher recurrence rates than MDO. Orthodontic appliances (SOAP, PEBP) emerged as a highly promising non-surgical intervention, significantly reducing OAHI (e.g., from 39.3 to 12.2 events/h, p<0.001) and potentially obviating the need for surgery in selected cases. Conclusion: Management of PRS-related UAO requires a severity-based, stepwise algorithm. Conservative and orthodontic interventions are effective first-line options for a majority of patients. For severe, refractory cases, MDO is the most effective surgical intervention for rapidly securing the airway, though its impact on nutritional and sleep quality metrics remains limited.