Cognitive and psychosocial development of children with Pierre Robin sequence

Abstract

It is unclear whether cognitive impairment in Pierre Robin sequence (PRS) results from a primary disturbance affecting both the brain and the mandible or from recurrent upper airway obstruction (UAO). If the latter were true, cognitive impairment should be preventable by early treatment of UAO. We wanted to determine the cognitive and psychosocial outcome of children with PRS treated with a new device aimed at relieving UAO in infancy (pre-epiglottic baton plate). Thirty-four children with PRS and 34 healthy controls aged 4-11 years completed the Kaufman-Assessment Battery for Children (K-ABC) and a self-concept inventory. Parents rated their children's emotional and behavioural problems. Multi- and univariate analyses of covariance were performed, controlling for gender, age, parental education, family income and parental depression. The cognitive development of the PR children was within the reference range. Compared to healthy children, however, the children with PRS performed significantly poorer. There were no significant differences concerning self-concept, emotional or behavioural problems. These children with non-syndromic PRS who had received treatment of UAO in infancy performed worse in the K-ABC. However, this did not reflect a major cognitive impairment.