Surgical correction of velopharyngeal insufficiency in children with velocardiofacial syndrome.

BACKGROUND: The surgical management of velopharyngeal insufficiency in children with velocardiofacial syndrome is inherently more difficult, with the need for revision being high. The purpose of this report was to evaluate and document the authors' experience with sphincter pharyngoplasty in the management of velopharyngeal insufficiency in children with velocardiofacial syndrome, and compare outcome.
METHODS: In part I, 32 patients with velocardiofacial syndrome underwent sphincter pharyngoplasty for velopharyngeal insufficiency between January of 1987 and March of 2001. There were 18 girls and 14 boys, with a mean age at primary sphincter pharyngoplasty of 6.7 years. Pharyngoplasty revision was defined as any secondary surgical revision of the sphincter as determined by clinical evaluation and objective speech assessment. In part II, comparisons were made to 218 non-velocardiofacial syndrome patients with velopharyngeal insufficiency who underwent sphincter pharyngoplasty (cleft palate, n = 127; velopharyngeal insufficiency alone, n = 63; submucous cleft, n = 15; other, n = 13). There was no significant difference in the average age or gender in the two groups. All patients underwent screening of velopharyngeal function, which included perceptual speech evaluation, clinical screening of velopharyngeal closure, and oral examination.
RESULTS: In part I, success of the primary sphincter pharyngoplasty was demonstrated in 78 percent of the velocardiofacial syndrome patients (n = 25), with a revision rate of 22 percent. Patients who required revision were slightly older, 8.6 versus 6.3 years (p = not significant). Preoperative nasometry scores were significantly higher in patients who required a pharyngoplasty revision (69 versus 54; p = 0.002). Patients who required revision of the pharyngoplasty were more likely to have larger velopharyngeal areas (30 mm versus 22 mm). In part II, the revision rate in patients with velocardiofacial syndrome was significantly higher than in those patients in the original cohort without velocardiofacial syndrome (22 percent versus 11 percent; p < 0.05). Preoperative objective speech data demonstrated significantly greater velopharyngeal incompetence in all categories (nasometry scores, pressure flow measurements, and radiographic measurements) for patients with velocardiofacial syndrome, and age at initial sphincter repair was slightly older (8.5 versus 7.7 years; p = not significant).
CONCLUSIONS: The management of velopharyngeal insufficiency using sphincter pharyngoplasty in children with velocardiofacial syndrome is safe and effective. The higher need for surgical revision in velocardiofacial syndrome patients is most likely attributable to a greater degree of preoperative nasalance and a slightly later age of presentation. This should provide insight into various technique modifications in an attempt to minimize pharyngoplasty revision.