Background: Patients with velocardiofacial syndrome often present with refractory velopharyngeal incompetence. Whereas the high wide pharyngeal flap has been advocated for these patients, poor visualization makes high inset of the flap technically difficult. We present a consecutive series of patients with velocardiofacial syndrome and severe velopharyngeal incompetence treated with high inset pharyngeal flap achieved by through-and-through dissection of the soft palate.
Methods: All patients with velocardiofacial syndrome in whom secondary surgical management was recommended for treatment of severe velopharyngeal incompetence over the last 7 years were treated with a high inset pharyngeal flap achieved by through-and-through dissection of the soft palate (to be described). All patients had nasendoscopy and videofluoroscopy preoperatively and underwent perceptual speech assessment preoperatively and at least 12 months postoperatively.
Results: Eight patients met the inclusion criteria with a mean age at presentation of 7 years, 3 months. No patient had a history of cleft palate. All patients had minimal lateral pharyngeal wall motion and a medium to large velopharyngeal gap (>6 mm). Velopharyngeal incompetence, as measured by perceptual speech score, improved from a mean of 10.3 (range, 9 to 12 on a maximum scale of 13) to a mean of 1.9 (range, 0 to 4), achieving velopharyngeal competence (p < 0.01) without nasal airway obstruction in any patient.
Conclusions: The present report demonstrates restoration of velopharyngeal competence in a consecutive series of patients with velocardiofacial syndrome who presented with severe velopharyngeal incompetence. We attribute this outcome to the development of a reliable technique for high inset of the pharyngeal flap using through-and-through dissection of the soft palate, enabling direct visualization of flap placement.