Airway Morphological Changes in Pierre Robin Sequence: A Retrospective Study

Abstract

Objective: To investigate airway morphology changes in patients with Pierre Robin sequence (PRS) pre–/post–mandibular distraction osteogenesis (MDO) and to compare morphologic changes to age-matched controls. Design: Retrospective case–control study. Setting: Urban, academic, tertiary medical center. Patients, Participants: Fifteen patients with PRS after MDO to relieve upper airway obstruction (UAO) (2008-2018); age-matched controls for post-MDO patients. Interventions: Mandibular distraction osteogenesis, curvilinear internal mandibular distractors. Main Outcome Measures: (1) Physiologic improvement after MDO (apnea–hypopnea index; minimum oxygen saturation); (2) airway size (volume, surface area, length, mean/minimum cross-sectional area), shape (lateral:anterior–posterior ratio, cross-sectional area ratios, uniformity, sphericity), and changes with MDO; and (3) post-MDO airway size, shape versus age-matched controls. Results: Airway size increased after MDO (volume, P = .01; surface area, P = .02; length, P = .01), as did cross-sectional area (mean, P = .02; minimum, P = .02; minimum retropalatal, P = .05, mid-retroglossal, P = .02). Post-MDO PRS airways were larger than controls (volume, P < .01; surface area, P < .01; length, P < .01, cross-sectional area, P = .03). Airway shape remained nonuniform and flat post-MDO; control airways were round. Two syndromic patients required repeat MDO and had subphysiologic post-MDO airway cross-sectional area. Post-MDO PRS patients with supraphysiologic cross-sectional area along the entire airway had no UAO recurrence. Conclusions: In this small, heterogenous patient sample, MDO increases airway size, may preferentially affect the retropalatal airway, and often results in supraphysiologic airway dimensions. These retropalatal changes may be important in relieving severe UAO in patients with PRS. Generalizability of our results is limited by small cohort size and patient heterogeneity.

Creating Normative Growth Curves for Pediatric Upper Airway Volume

“…Expansion in particular segments of the upper airway including the retropalatal and retroglossal regions have been shown to correlate with physiologic response to airway expansion and the overall morphologic changes of the airway may provide a more predictive model than airway volume alone. 17 We have found that a small percentage of our patients, in particular those with an associated syndrome, have a recurrence in obstruction some time post-distraction. The airway volume growth charts may provide a clinical instrument to delineate how the airway of these patients has changed over time both relative to their own prior dimensions as well as compared to the general population.…”

“…Expansion in particular segments of the upper airway including the retropalatal and retroglossal regions have been shown to correlate with physiologic response to airway expansion and the overall morphologic changes of the airway may provide a more predictive model than airway volume alone. 17…”

“…Indeed, our group among others has published investigations about the utility of image-based airway volume calculation in guiding surgical planning and evaluating postoperative results. 12-17 In addition to showcasing the potential utility of 3D airway volume measurements, these and other reports also serve to highlight a conspicuous lack of reliable normative data relating to airway volume and how it changes during growth and development.…”

Impact of Cleft Palate on Tongue-Based Upper Airway Obstruction in Pierre Robin Sequence: Implications for Mandibular Distraction Osteogenesis and Timing of Cleft Palate Repair

“…Our previous work showed that the increase in airway size preferentially affects the retropalatal space, which can lead to supraphysiologic airway dimensions. 18 Over 90% of patients in that study had a CP, suggesting that morphologic changes in the cleft affected by MDO contributed to the observed changes in airway size. This is supported by Murage et al 19 who found that the absence of a CP was associated with failure of distraction.…”

Polysomnography in the Early Consolidation Phase of Mandibular Distraction as a Tool to Identify Residual Tongue-Based Airway Obstruction and Need for Further Distraction: A Follow-Up Study

“…17 However, these static predictions do not provide a physiologic measurement of the frequency and severity of OSA and do not correlate with physiologic improvements on post-distraction PSG. 22…”