Anesthetic Management of a Patient With Pierre Robin Sequence: A Case Report

Pierre Robin Sequence (PRS) is a rare congenital disorder characterized by a triad of micrognathia, glossoptosis, and airway obstruction. This case report presents a rare instance of PRS marked by congenital airway obstruction, which significantly complicated endotracheal intubation with vocal cord motion and a smaller airway to navigate. It will describe what can be expected from such airways and the approaches taken to manage them. The patient was a five-month-old female with PRS, undergoing removal of bilateral mandibular distractors for treatment of micrognathia. Preoperative evaluation revealed a well-nourished, full-term, American Society of Anesthesiologists (ASA) Class II infant in no distress, breathing spontaneously on room air with previously placed bilateral mandibular distractors. Anesthesia induction utilized 8% sevoflurane in 100% oxygen, maintaining spontaneous ventilation. Video laryngoscopy showed a large U-shaped cleft palate and a Cormack-Lehane class II view. The uneventful procedure included dexamethasone, acetaminophen, ketorolac, propofol, and local anesthesia (lidocaine with epinephrine and marcaine). Total IV fluids were 350 ml, blood loss was minimal, and extubation was successful with stable recovery. Video documentation of the intubation sequence is included within this report. This report demonstrates effective airway management strategies tailored to PRS-related anatomical challenges and highlights the vital role of anesthesiologists in clinical decision-making teams for the management of complex airway scenarios.