Velopharyngeal Outcomes After Palatoplasty for Patients With U-Shaped Cleft Palate and Pierre Robin Sequence

Background: Patients with Pierre Robin sequence (PRS) often present with a wider U-shaped cleft palate rather than a narrower V-shaped cleft palate. We investigate if PRS patients with a U-shaped cleft are more likely to require velopharyngeal insufficiency (VPI) repair after primary palatoplasty. Methods: We identified PRS patients born from 1990 to 2020 with ≥4 years of care at our institution and excluded the patients who were non-verbal, underwent primary palatoplasty at an outside institution, or had Veau I, III, or IV cleft palates. For each patient, we recorded syndromic status, surgeon notes, age at primary palatoplasty, hard and soft palate repair techniques, surgeon years in practice at primary palatoplasty, and VPI repair status. Results: Of the included 57 patients with Veau II cleft palate, 25 had a documented U-shaped cleft palate. Sixty percent of U-shaped and 28% of V-shaped cleft palate patients developed VPI (RR = 2.13, 95% CI 1.13-4.04, P = .020). Overall, 32% patients were syndromic (40% of U-shaped and 25% of V-shaped cleft palate, P = .262). Surgeon’s years in practice was greater in V-shaped (7.0 years) than U-shaped (5.0 years) cleft palate patients ( P = .041). There was no significant difference in age at primary palatoplasty or primary palatoplasty techniques between Ushaped and V-shaped cleft palate patients. A multivariate Cox regression model (−2 Log Likelihood = 151.154, P = .106) yielded an increased hazard ratio for VPI repair in PRS patients with U-shaped cleft palate (95% CI 1.236-8.279, P = .017). Syndromic diagnosis ( P = .713), surgeon years in practice ( P = .326), and age at primary palatoplasty ( P = .429) were not significant factors to VPI repair. Conclusion: Patients with Robin sequence and a U-shaped cleft palate have a higher relative risk of developing VPI after primary palatoplasty.