17 - Cleft Palate and Velopharyngeal Dysfunction

Abstract

Cleft palate results from an aberration of fusion of the palatal shelves between weeks 7 and 12 of embryonic development. Cleft palate may occur in isolation or as a component of a named sequence or syndrome. The most commonly associated anomaly is Pierre Robin sequence, which includes micrognathia, glossoptosis, and respiratory distress. Cleft palate can also occur in a number of syndromes, including van der Woude, Stickler, Treacher Collins, and Apert syndromes, as well as oculo-auriculo-vertebral spectrum. There is general agreement that palate repair should be effected before 12 months of age. Velopharyngeal insufficiency may result following cleft palate repair or may herald the presence of an overt or occult submucous cleft palate. Identification of an occult submucous cleft palate or velopharyngeal dysfunction without evidence of a cleft is common in 22q11.2 deletion (velocardiofacial) syndrome. Treatment of velopharyngeal dysfunction includes palate re-repair with intravelar veloplasty, Furlow palatoplasty, pharyngeal flap, sphincter pharyngoplasty, or prosthetics.