An algorithm for application of furlow palatoplasty to the treatment of velocardiofacial syndrome-associated velopharyngeal insufficiency.

Abstract

Treatment of velocardiofacial syndrome (VCFS)-associated velopharyngeal insufficiency is controversial. Palatoplasties have variable success, and pharyngeal flaps (PPF) increase the obstructive sleep apnea risk. Our center uses Furlow palatoplasties to treat overt clefts and kinetic submucous cleft palates. PPFs are employed to treat akinetic palates and recurrent/persistent velopharyngeal insufficiency after palatoplasty. A retrospective review was performed of patients with VCFS treated according to this algorithm. Twenty-seven patients with VCFS were included: 3.7% (n = 1) had overt clefts; 81.4% (n = 22) underwent Furlow palatoplasties for kinetic submucous cleft palates; 14.8% (n = 4) underwent primary PPFs for akinetic palates. The algorithm was successful in 21 patients (77.7%). Furlow palatoplasty achieved ultimate success in 45% of patients. Secondary PPF was successful in all 7 patients, as was primary PPF in all 4 patients. Furlow palatoplasty represents a first step in treating appropriate VCFS patients that avoids the risk of sleep apnea, but the potential for secondary pharyngoplasty must be considered.