Anaesthetic management of a infant with Pierre robin sequence

Abstract

Pierre Robin sequence (PRS) is characterized by the clinical triad of micrognathia (small mandible), glossoptosis (backward, downward displacement of the tongue), U or V shaped cleft palate and airway obstruction defines the Pierre Robin sequence (PRS). Airway obstruction and respiratory distress are clinical hallmarks. It occurs in 1:8500 live births with an equal male-to-female ratio. The mechanical theory which states that initiating event is mandibular hypoplasia which keeps the tongue high within the oral cavity; this in turn causes a cleft palate by preventing the closure of the palatal shelves, this syndrome is now called as Pierre robin sequence. These cases pose a challenge to the anesthetist as they present at a very young age, may have associated congenital anomalies, varying degrees of difficult airway, need repeated corrective surgeries, and lastly the airway has to be shared with the surgeons.