Sphincter pharyngoplasty for the surgical management of speech dysfunctionassociated with velocardiofacial syndrome

There are no reports in the literature that document the effectiveness of s phincter pharyngoplasty as a surgical alternative to pharyngeal flap for ma nagement of velopharyngeal dysfunction in patients with velocardiofacial sy ndrome. A retrospective review of patients with velocardiofacial syndrome w as undertaken at our tertiary cleft care centre. All patients were managed between 1984 and 1996 at the Cleft Palate and Craniofacial Deformities Inst itute, St Louis Children's Hospital. Subjects (n = 19) underwent velopharyn geal surgical management on the basis of perceptual speech evaluations and instrumental assessments of inadequate velopharyngeal closure. All patients had a molecular diagnosis of velocardiofacial syndrome based on fluorescen t in situ hybridisation analysis of peripheral blood lymphocytes and indepe ndent evaluation by a medical geneticist. Surgical outcome was classified a s successful if perceptual speech assessment indicated elimination of hyper nasality, nasal emission and turbulence, and instrumental assessment indic ated 100% velopharyngeal closure. Results showed that 18 of 19 patients wer e managed successfully with sphincter pharyngoplasty. Our data corroborate that sphincter pharyngoplasty is a reasonable alternative to pharyngeal fla p in patients with velopharyngeal dysfunction secondary to velocardiofacial syndrome. (C) 1999 The British Association of Plastic Surgeons.