BACKGROUND: Craniofacial microsomia (CFM) is characterized by malformations of facial structures that are derived from the first and second brachial arches. Although hearing, occlusion, and facial paralysis are the most typical functional considerations for CFM, abnormalities in speech, including velopharyngeal insufficiency (VPI), have also been sparingly reported in several studies. As a result, the purpose of this multicenter study was to analyze the prevalence and management of VPI in patients with CFM. Furthermore, this study sought to investigate the effects of concomitant cleft lip and/or palate (CL/P) on VPI in CFM patients. METHODS: A retrospective chart review of patients from the University of California, Los Angeles Craniofacial Clinic and the Orthopaedic Institute for Children in Los Angeles was conducted. Inclusion criteria included patients who were diagnosed with CFM, first and second brachial arch syndrome, oculo-auriculo-vertebral sequence, facio-auriculo-vertebral syndrome, or Goldenhar syndrome. Patients with isolated microtia were excluded from this study. Included CFM patients were stratified based on the presence or absence of CL/P, and all patients were evaluated for VPI, methods of diagnosis, speech therapy, and surgery. All patients received at least 1 thorough evaluation by a speech pathologist and were diagnosed clinically with VPI if they were noted to have any hypernasal speech, nasal air emission, or nasal turbulence. Chi-square tests and Levene’s test (P ≤ 0.05) were used for analysis. RESULTS: Overall, 78 patients with CFM (48 males, 61.5%) were assessed for VPI, aged 4–34 years old at time of review. In the entire cohort, 22 (28.2%) patients were found to be diagnosed with VPI. Of the 78 patients, 8 (10.3%) patients had concomitant CL/P. Of the 70 CFM patients without CL/P, 14 (20.0%) had VPI. Eight (57.1%) of these patients were recommended for nasoendoscopy, whereas none of these patients required any corrective VPI surgery. Comparatively, all 8 CFM with CL/P patients were diagnosed with VPI and recommended for nasoendoscopy, significantly higher rates than those without CL/P. Furthermore, 6 (75.0%) of these patients eventually underwent VPI surgery, significantly more than those without CL/P. CONCLUSIONS: This study establishes an overall rate of VPI in CFM at 28.2%, with the largest cohort of CFM looking into VPI in the literature. All of the CFM patients with CL/P had VPI, with a majority requiring surgery, compared with only a fifth of CFM patients without CL/P having VPI and none needing surgery. Findings from this study highlight VPI as a common problem in CFM patients who should be diagnosed and managed early on. In addition, this study displays CL/P as a risk and severity factor for VPI.
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