Surgical outcomes for velopharyngeal insufficiency in velocardiofacial syndrome and nonsyndromic patients.

OBJECTIVE

To compare speech outcomes after operative intervention for velopharyngeal insufficiency between velocardiofacial syndrome patients and nonsyndromic patients.

DESIGN

Retrospective cohort study.

SETTING

Tertiary academic center.

PATIENTS: Cohorts of 14 velocardiofacial syndrome and 15 nonsyndromic patients without overt cleft palate who underwent operative procedures to correct velopharyngeal insufficiency. All velocardiofacial syndrome patients were positive for 22q11.2 microdeletion by fluorescent in situ hybridization and possessed phenotypic features of velocardiofacial syndrome.

INTERVENTIONS: Operative procedures, including sphincter pharyngoplasty, Furlow palatoplasty, or both, were selected based on preoperative endoscopic assessments of velopharyngeal motion and residual gap size and shape, as well as velocardiofacial syndrome status. Five single and 9 combined procedures were performed in the velocardiofacial syndrome group, whereas 13 single and 2 combined procedures were performed in the nonsyndromic group.

OUTCOME MEASURES: Pre- and post-op evaluation was conducted by a speech pathologist. Assessment parameters scored on a numerical scale included speech intelligibility, resonance, nasal air emissions, and overall severity of velopharyngeal insufficiency. Postoperative complications were recorded.

RESULTS

Most velocardiofacial syndrome patients and nonsyndromic patients demonstrated significant improvements in all parameters. Comparison of the two groups demonstrated similar improvements in both. Changes in speech resonance were significantly different between the two groups, whereas other speech parameters did not reach significance. There was no difference in airway complications between groups.

CONCLUSIONS

Velocardiofacial syndrome patients may have comparable outcomes to nonsyndromic patients in selective surgical management of velopharyngeal insufficiency. In addition, the data demonstrate the efficacy of a single-stage combined procedure without increased morbidity.