Upper airway changes in Pierre Robin sequence from childhood to adulthood

Abstract

OBJECTIVES: To investigate pharyngeal airway changes in patients with Pierre Robin sequence (PRS) longitudinally from childhood to adulthood. SETTING AND SAMPLE POPULATION: Cleft Lip and Palate Unit, Clinic of Orthodontics, University of Zurich. Twenty-four patients born between 1970 and 1990 with non-syndromic PRS. MATERIALS AND METHODS: Lateral cephalograms at age 5 (T1), 10 (T2), 15 (T3) and 20 (T4) years were available. Variables describing pharyngeal airway dimensions, soft palate morphology, tongue and hyoid position, skeletal morphology and head posture were assessed. RESULTS: A significant increase in nasopharyngeal depth was found over the entire observation period (T1 10.7 to T4 19.1 mm, p < 0.001), especially between T2 and T3 (change 3.8 mm, p < 0.001), and was mainly due to adenoid recession (r = -0.75, p < 0.001; variation explained by 56%). Increase in velopharyngeal depth mainly took place between T3 and T4 (change 2.3 mm, p < 0.01). It was due to more anterior tongue posture (r = 0.65, p < 0.001; 42.5% of variation explained), in turn allowing the soft palate to take a more vertical position (r = -0.52, p < 0.001). Increase in oropharyngeal depth was associated with head extension and anterior mandibular positioning (36% of variation explained). However, significance was not reached (T1 8.3 to T4 9.8 mm, p > 0.05). CONCLUSIONS: Upper airway dimensions in children with PRS improve with time, except for the oropharyngeal airway. Despite large interindividual variation, the mean remained in the lower reaches of normality described in other studies. Thus, further research should investigate the prevalence of obstructive sleep apnoea in adults with PRS.