Speech outcomes following pharyngeal flap in patients with velocardiofacial syndrome.

BACKGROUND

Velocardiofacial syndrome is the most common defined disorder associated with palatal insufficiency. The authors' purpose is to evaluate one surgeon's experience with correction of velopharyngeal insufficiency in velocardiofacial syndrome using a tailored pharyngeal flap.

METHODS

The authors reviewed the records of all children with velocardiofacial syndrome and velopharyngeal insufficiency who were managed with a pharyngeal flap between 1983 and 2009. Data collected included age at operation, preoperative videofluoroscopic findings, speech outcomes, complications, and need for a secondary operation.

RESULTS

The authors identified 33 patients with velocardiofacial syndrome and velopharyngeal insufficiency who had postoperative speech evaluations. Velopharyngeal insufficiency was diagnosed at a median age of 5 years. Palatal findings were: Veau type I (n = 4), overt submucous (n = 6), or occult submucous (n = 23). Median preoperative lateral pharyngeal wall movement was 22 percent (range, 0 to 90 percent). Successful correction of velopharyngeal insufficiency was achieved in 29 of 33 patients (88 percent). One patient had a medially displaced right internal carotid artery, and evidenced intraoperative bleeding and required a blood transfusion. One patient developed obstructive sleep apnea.

CONCLUSION

A tailored pharyngeal flap is highly effective for correction of velopharyngeal insufficiency in velocardiofacial syndrome with few complications.