Surgical planning for restoring velopharyngeal function in velocardiofacial syndrome

Background

Velocardiofacial syndrome (VCFS) is one of the most common multiple anomaly syndromes in humans. Around 70% of the cases show velopharyngeal insufficiency (VPI), as a consequence of cleft palate. VPI is much more frequent due to special abnormal conditions inherent to VCFS including: platybasia, hypotrophy of adenoid, enlarged tonsils, hypotonia and abnormal pharyngeal muscles.

Objective

To evaluate the surgical treatment of VPI in VCFS patients.

Materials and methods

In the Hospital Gea Gonzalez at Mexico City, all cases of VCFS from January 2000 to July December 2007 were studied. All patients subjected to velopharyngeal surgery for correcting VPI were selected. Twenty-nine patients underwent velopharyngeal surgery. All operations were planned according to findings of videonasopharyngoscopy (VNP) and multiview video fluoroscopy (MVF).

Results

Twenty patients underwent pharyngeal flap operations, and 9 patients were operated on with a sphincter pharyngoplasty. After a pharyngeal flap, 17 cases (85%) improved to normal nasal resonance or mild hypernasality. Three flaps showed moderate hypernasality postoperatively. From the 9 sphincter pharyngoplasties, 6 cases (66%) improved to moderate hypernasality. Four patients (33%) persisted with severe hypernasality postoperatively. There were no complications.

Conclusions

Tailor-made pharyngeal flaps seem to be the best option for restoring velopharyngeal function in cases of VPI in VCFS patients. The use of VNP and MVF is useful for planning the operations for VPI, and they are also useful for indicating the removal of tonsils in cases with high risk of obstruction. Moreover, VNP is also useful for preventing damage to the internal carotids which are usually displaced in VCFS patients.