Hearing loss and speech difficulty are often associated with PRS. Cleft palate (incomplete closure of the roof of the mouth) is present in the majority of patients. PRS is characterized by an unusually small mandible, posterior displacement or retraction of the tongue, and upper airway obstruction.
#Pierre robin sequence adults series#
PRS is not merely a syndrome, but rather it is a sequence-a series of specific developmental malformations which can be attributed to a single cause. A wide, U-shaped cleft palate is commonly also present.
The three main features are micrognathia (abnormally small mandible), which causes glossoptosis (downwardly displaced or retracted tongue), which in turn causes breathing problems due to obstruction of the upper airway. Pierre Robin sequence ( / p j ɛər r ɔː ˈ b æ̃/ abbreviated PRS) is a congenital defect observed in humans which is characterized by facial abnormalities. Intrauterine compression of fetal mandible or de-novo mutations (on chromosomes 2, 4, 11, or 17)Ĭraniofacial surgery, oral and maxillofacial surgery Micrognathia, glossoptosis, obstruction of the upper airway, sometimes cleft palate Pierre Robin syndrome, Pierre Robin malformation, Pierre Robin anomaly, Pierre Robin anomalad
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