Adenoid hypoplasia in the velo-cardio-facial syndrome.

The velo-cardio-facial syndrome is one of the most comprehensively studied congenital malformation syndromes of clefting. More than 20 features have been described to date. The purpose of this investigation is to report evidence for congenital hypoplasia of the adenoids in over 80% of the cases studied. Radiographic and nasopharyngoscopic studies of 30 children with velo-cardio-facial syndrome were reviewed along with 30 age- and sex-matched children with nonsyndromic cleft palate to determine the size of their adenoid masses. Eighty-three percent of the subjects with velo-cardio-facial syndrome were found to have small or absent adenoids, whereas only 20% of the controls were found to have small adenoids. None of the control sample had total adenoid absence. These findings may have significant implications for the hypernasal speech patterns often found in individuals with velo-cardio-facial syndrome and may also be related to histories of frequent upper respiratory illnesses in this group.