Background: Pierre Robin sequence (Robin sequence) is defined as the triad of micrognathia, glossoptosis, and airway obstruction. It is frequently associated with palatal clefting. In recent years, increased interest in speech outcomes of cleft patients diagnosed with Robin sequence has been shown. Methods: Speech outcomes of cleft patients with Robin sequence were assessed at age 5 in comparison with a cleft palate–only cohort. Speech parameters were evaluated according to the Cleft Audit Protocol for Speech-Augmented and analyzed using the National Audit Standards for Speech (United Kingdom). All patients were treated in the same institution during the same period (2005 to 2012). Subjects who needed nasopharyngeal airway support and those whose airway was managed by positioning only were eligible. Results: Fifty-one cleft patients diagnosed with Robin sequence were included in this study. Outcomes were compared to those of 128 nonsyndromic cleft palate–only patients. Patients with Robin sequence were shown to present with a significantly higher rate of cleft speech characteristics in comparison to the reference cohort (p = 0.001). Furthermore, it was shown that Robin sequence is associated with a significantly higher rate of secondary speech surgery for velopharyngeal dysfunction before the age of 5 (p = 0.016). Robin sequence patients with a nasopharyngeal airway presented with a higher rate of cleft speech characteristics compared to Robin sequence patients managed with positioning only. Conclusion: Cleft patients with Robin sequence are more likely to need further surgery to correct velopharyngeal dysfunction before the age of 5 and are more prone to present with cleft speech characteristics at the age of 5. CLINICAL QUESTION/LEVEL OF EVIDENCE: Risk, II.
Objective: To report speech outcomes following Orticochea pharyngoplasty in 43 patients with cleft palate and noncleft velopharyngeal dysfunction. Design: A retrospective surgical audit of patients undergoing Orticochea pharyngoplasty between 2004 and 2012, with speech as a primary outcome measure. Setting: Patients known to a regional UK cleft center. Methods: Forty-three patients underwent Orticochea pharyngoplasty by a single surgeon in a UK regional cleft center. Twenty-one patients had undergone a prior procedure for velopharyngeal dysfunction. Pre- and postoperative speech samples were assessed blindly using the Cleft Audit Protocol for Speech-Augmented by a specialist cleft speech and language therapist, external to the team. Speech samples were rated on the following parameters: hypernasality, hyponasality, audible nasal emission, nasal, turbulence, and passive cleft speech characteristics. Statistical differences in pre- and postoperative speech scores were tested using the Wilcoxon matched-pairs signed-ranks test. Inter- and intrareliability scores were calculated using weighted Cohen κ. Results: Whole group: A statistically significant difference in pre- and postoperative scores for hypernasality ( P < .001), hyponasality ( P < .05), nasal emission ( P < .01), and passive cleft speech characteristics ( P < .01) were reported. Patients with cleft diagnoses: A statistically significant difference in scores for hypernasality ( P < .001), nasal emission ( P < .01), and passive cleft speech characteristics ( P < .01) were reported for this group of patients. Patients with noncleft diagnoses: The only parameter to demonstrate a statistically significant difference was hypernasality ( P < .01) in this group. Conclusions: Orticochea pharyngoplasty is a successful surgical procedure in treating velopharyngeal dysfunction in both the cleft and noncleft populations.
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