Speech Therapy in Velocardiofacial Syndrome After Palatopharyngeal Pharyngoplasty.

Velocardiofacial syndrome (VCFs) is a rare congenital disease with an incidence of 1:4000 to 1:6000. Previous studies have found that the abnormality is associated with 22qDS. As reported at the international VCFs conference held in 2006, >180 phenotypes have been identified with this syndrome. Accordingly, there is a wide range of clinical manifestations including congenital defects of the heart and palate, immune deficiencies, psychiatric illness, and speech, learning, and cognitive disabilities Among all the symptoms above, however, pharyngopalatine dysplasia has been observed in nearly every patient. Patients with VCFs often have velopharyngeal insufficiency, which may cause serious disorders in functional speech, with poor intelligibility, a glottal stop with hypernasality and a pharyngeal fricative, and dropping and weakening of consonants. Most also show defects in language learning. Therefore, specialized speech therapy is essential for patients with VCFs, mainly focused on correction of abnormal pronunciation. Long-term clinical experience has shown that speech therapy is based on 2 components: velopharyngeal function and behavior therapy. Our study focused mainly on establishing a corrective behavior guidance model of pronunciation for patients with VCFs.