Surgical management of velopharyngeal incompetence in velocardiofacial syndrome.
OBJECTIVE
To analyze the results of surgery for velopharyngeal incompetence (VPI) in velocardiofacial syndrome.


METHODS
Prospective data collection, with randomized, blind assessment of speech and velopharyngeal function on lateral videofluoroscopy and nasendoscopy.


METHODS
Two-site, tertiary referral cleft unit.


METHODS
Forty-two consecutive patients with the 22q11 deletion underwent surgery for symptomatic VPI by a single surgeon.


METHODS
Intraoral examinations, lateral videofluoroscopy (+/- nasendoscopy) and intraoperative evaluation of the position of the velar muscles through the operating microscope. Based on these findings, either a radical dissection and retropositioning of the velar muscles (submucous cleft palate [SMCP repair]) or a Hynes pharyngoplasty (posterior pharyngeal wall augmentation pharyngoplasty) was performed. As anticipated, a proportion of patients undergoing SMCP repair subsequently required a Hynes. The aim of this staged approach was to maximize velar function, thereby enabling a less obstructive pharyngoplasty to be performed. Thus, there were three surgical groups for analysis: SMCP alone, Hynes alone, and SMCP+Hynes.


METHODS
Blind perceptual rating of resonance and nasal airflow; blind assessment of velopharyngeal function on lateral videofluoroscopy and nasendoscopy; and identification of predictive factors.


RESULTS
Significant improvement in hypernasality in all three groups. The SMCP+Hynes group also showed significant improvement in nasal emission. There were significant improvements in the extended and resting velar lengths following SMCP repair and a trend toward increased velocity of closure.


CONCLUSIONS
Depending on velopharyngeal anatomy and function, there is a role for SMCP repair, Hynes pharyngoplasty, and a staged combination of SMCP+Hynes, all of which are procedures with a low morbidity.
Mehendale
Felicity V
FV
Great Ormond Street Hospital for Children, London, United Kingdom.
Birch
Malcolm J
MJ
Birkett
Louise
L
Sell
Debbie
D
Sommerlad
Brian C
BC
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D002872:Chromosome Deletion; D002892:Chromosomes, Human, Pair 22; D002972:Cleft Palate / Q000235:genetics; D005260:Female; D006330:Heart Defects, Congenital / Q000235:genetics; D006801:Humans; D008297:Male; D019647:Oral Surgical Procedures / Q000379:methods*; D010156:Palatal Muscles / Q000601:surgery; D010614:Pharynx / Q000601:surgery; D011446:Prospective Studies; D011897:Random Allocation; D016037:Single-Blind Method; D013068:Speech Production Measurement; D013577:Syndrome; D014681:Velopharyngeal Insufficiency / Q000235:genetics / Q000601:surgery*; D014741:Video Recording; D014832:Voice Disorders / Q000175:diagnosis