Comparison of Velopharyngeal Outcomes Between Patients with Pierre Robin Sequence and Non-syndromic Isolated Cleft Palate

Abstract

Introduction:

Cleft palates in patients with Pierre Robin Sequence (PRS+CP) are wide and U-shaped, making palatoplasty challenging. Velopharyngeal insufficiency (VPI) is a common finding after primary palatoplasty, often necessitating secondary speech surgery. The purpose of this study was to evaluate long-term Pittsburgh Weighted Speech Scores (PWSS) and the need for secondary speech surgery for VPI in patients with PRS + CP after primary palatoplasty compared to patients with isolated cleft palate (iCP).

Methods:

A retrospective cohort study included patients diagnosed with syndromic and non-syndromic PRS+CP who underwent primary cleft palate repair at a tertiary care children’s hospital between 2001 and 2019. The control group included iCP patients matched by Veau Class I or II cleft palate. Data collected included demographics, clinical data, and speech outcomes. Outcomes included PWSS and secondary operations for VPI.

Results:

88 patients with PRS + CP (58.0% female) and 48 controls with iCP (54.2% female) met the inclusion criteria. Most recent PWSS did not differ between groups (3.9 ± 1.0 syndromic PRS + CP, 3.5 ± 1.6 non-syndromic PRS + CP, 3.7 ± 0.9 iCP; P = .55). Secondary speech surgery rates were significantly higher in patients with syndromic PRS + CP versus iCP (37.9% vs 10.4%, P = .008), and non-syndromic PRS + CP versus iCP (32.2% vs 10.4%, P = .01).

Conclusion:

Although PRS + CP patients have higher rates of secondary palate surgery for persistent VPI, appropriate intervention resulted in similar long-term speech outcomes when compared to patients with iCP.