Pierre Robin sequence

Abstract

Pierre Robin Sequence (PRS) is a rare condition described in the early twentieth century as the triad of micrognathia, glossoptosis and upper airway obstruction occurring in infants. Up to 90% of infants affected will have a cleft palate. The severity of the condition varies widely. The airway obstruction may be immediately life threatening, or associated with difficulties with feeding and faltering growth which can also be severe. More commonly the airway obstruction is more mild, and can often be managed by conservative intervention such as positioning of the child. Investigation and management regimes vary widely, but the specific definitions of PRS used in published literature are numerous making comparison between treatments difficult. Recently a consensus document has been published, the aim of which is to improve consistency in definition of PRS and thus improve the evidence base for intervention. This chapter describes the condition, and outlines management options and outcomes.