Open AccessVelopharyngeal Insufficiency Repair Outcomes in Patients with Velocardiofacial Syndrome
Author(s) -
Hall Joseph E.,
Muckala Jennifer,
Sowder Derrick,
Wooten Christopher,
Goudy Steven L.
Publication year - 2011
Publication title -
otolaryngology–head and neck surgery
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.232
H-Index - 121
eISSN - 1097-6817
pISSN - 0194-5998
DOI - 10.1177/0194599811415823a377
Subject(s) - medicine , velopharyngeal insufficiency , surgery , conductive hearing loss , hearing loss , tetralogy of fallot , retrospective cohort study , laryngomalacia , sphincter , heart disease , audiology , middle ear , airway , stridor
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.