Pierre Robin Sequence (PRS) consisting of Micrognathia, Retrognathia, Glossoptosis and cleft palate present with airway obstruction and feeding difficulties with or without other congenital anomalies. These patients come into category of difficult ventilation and difficult intubation. We present a case report of a 10 days old female child, weighing 2.5kg, suffering from PRS with cleft palate, Micrognathia, Retrognathia, Glossoptosis, airway obstruction, feeding and breathing difficulty posted for tongue lip adhesion. Thorough preoperative airway assessment was done. Use of transparent round mask and two hand jaw thrust were beneficial for ventilation of the child with receding chin. Inhalational induction was performed with Sevoflurane in oxygen. The child was ventilated with facemask. PRS patients are at risk of postoperative airway obstruction and chronic hypoxia. Use of long acting opioids was avoided and patient was kept under observation in NICU.
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