ABSTRACT
Pierre Robin sequence (PRS) is originally named after the French dental surgeon Pierre Robin. He described micrognathia, glossoptosis, and respiratory distress in his first paper. Family members of PRS patients have a higher incidence of cleft lip and palate. The most common PRS syndromes are Stickler syndrome and velocardiofacial syndrome. PRS consists of three essential components: micrognathia or retrognathia; glossoptosis, possibly accompanied by airway obstruction; and cleft palate. The airway obstruction in PRS is due to the narrowing or complete obstruction of the pharyngeal space by the posteriorly displaced tongue. Endotracheal intubation serves as a short-term support if the nasopharyngeal airway is not successful or during resuscitation or anesthesia. Cleft palate is present in at least 80" of patients with PRS. A palatal plate can be used in patients with a cleft of the hard palate to improve feeding. The plate also corrects the tongue position by moving it anteriorly.
