The Pierre robin syndrome (PRS) also known as the Pierre robin sequence is still a poorly defined syndrome. We did not find in the literature one classification that takes into consideration all of the aspects of PRS with a direct correlation with treatment planning. We are going to describe our thoughts on how to best manage children with PRS.PRS has always been defined as a triad, including micrognathia, glossoptosis and respiratory distress. The cleft palate was added later as an aggravating factor[1-3]. The syndrome might also include other malformations like cardio-vascular, limb, ocular, gastroenterological and urinary tract. In our literature review, we did not see anyone describing tracheo/laryngo-malacia as an inseparable part of the PRS pathology though our experience showed that it has a high prevalence in PRS patients. Therefore, in our opinion this entity should be included in the definition of PRS turning it from a triad into a "quartet".In our series, we always check for respiratory tract malformation using laryngoscopy and tracheoscopy, because we think that naso-pharyngoscopy does not go far enough to be able to make a good airway assessment. We found that there is a rather high percentage of airway obstruction due to either tracheo or laryngomalacia[4].
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