Pierre Robin

The Pierre Robin sequence (PRS) is characterized by mi- crognathia, glossoptosis, respiratory distress and optionally cleft palate (1). Approximately 40% of patients have PRS in combination with other malformations (2). The dominant clinical problem in PRS is severe upper airway obstruc- tion (UAO) (1,3) and failure to thrive (3,4). Various treat- ments have been developed to overcome the UAO (1,5). At our institution, a palatal plate has been developed that has a velar extension pulling the base of the tongue forward, thereby widening the pharyngeal space (the pre-epiglottic baton plate [PEPB]). In a controlled trial, this plate was shown to improve UAO in PRS (4).

Mental retardation has been reported in PRS (6,7). It is unclear, however, whether this is due to an underlying con- genital disorder involving both the brain and the mandible, as suggested by Abadie et al. (8), or a disturbed development resulting from intermittent hypoxia and/or sleep disturbance as seen in other cases of UAO early in life (9). If the latter were true, cognitive development should be within the nor- mal range if children with PRS had their UAO treated early. Children with craniofacial malformations may also have

an increased rate of behavioural disorders, internalizing and externalizing problems and reduced social abilities, and their self-concept and self-esteem may be negatively affected (10–

13). These impairments may continue or even worsen over time until adulthood (14,15). No study, however, has fo- cussed systematically on these psychosocial problems in chil- dren with PRS.

We, therefore, conducted a study investigating the cog- nitive and psychosocial development (self-concept, be- havioural and emotional problems) in children with PRS who had been treated with the PEBP mostly within their first 3 months of life.