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

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.

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FACE
Published
2025-11-22
DOI
https://doi.org/10.1177/27325016251387010
Primary Topic
Cleft Lip and Palate Research
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article
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article

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

Casey Zhang, Nicolás M. Kass, Tiffany Jeong, John Smetona et al.
FACE
Cleft Lip and Palate Research
article

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

Casey Zhang, Nicolás M. Kass, Tiffany Jeong, John Smetona, Janina Kueper, Justin Beiriger, Jesse A. Goldstein, Joseph Mocharnuk, Mike Hernández, Anne E. Glenney, Joseph E. Losee, M. Ford, Angel Dixon, Tobi Somorin
article en

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.

FACEVol. 7(1)
University of Pittsburgh Medical Center (US)
Openalex Percentile: Top 61%
Cleft Lip and Palate Research
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