Abstract
Objective: Analyze outcomes of operations for velopharyngeal insufficiency (VPI) in patients with velocardiofacial syndrome (VCFS). We hypothesize that patients with more severe phenotypic features of VCFS have greater VPI and are more likely to require revision VPI surgery.
Method: Retrospective chart review. Demographic data, VCFS characteristics, anatomical variations, perceptual speech investigation, nasal endoscopy, acoustic nasometry, and operative intervention data were collected for each patient with VCFS and VPI at Vanderbilt University Medical Center. Sixteen patients with VCFS were reviewed.
Results: Average age was 7.38 years, with 8 female patients. Surgery was necessary in 12 of 16 patients (75%). Eight patients (50%) underwent pharyngeal flap, 2 patients (12.5%) underwent Furlow palatoplasty, and 2 patients (12.5%) underwent sphincter pharyngoplasty. Six patients had heart anomalies (37.5%). Eight patients (50%) had hearing loss (average loss of 31 dB), and 3 patients had significant hypocalcemia (18.8%). Two patients had submucous clefts (12.5%), while 1 patient had cleft palate (6.3%). Two patients (12.5%) required revision operations. Both patients requiring revision had Tetralogy of Fallot, mild/moderate conductive hearing loss, and moderate to severe hypernasality preoperatively.
Conclusion: VPI is common in VCFS, and most patients require surgical intervention. VCFS patients presenting with a more severe phenotype as exhibited by increasing severity of heart disease, hearing loss, and hypernasality are more likely to require revision surgery to correct VPI.
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