Post-extubation airway obstruction following uvulopalatopharyngoplasty in an undiagnosed Pierre Robin sequence: A case report

Pierre Robin Sequence (PRS) is a rare congenital condition characterized by micrognathia, glossoptosis, and often cleft palate, predisposing patients to severe airway management difficulties. While typically diagnosed in infancy, an undiagnosed presentation in adulthood poses a unique and critical challenge for the anesthesiologist, especially when complicated by procedures such as uvulopalatopharyngoplasty (UPPP). We report a case of a 25-year-old man with cognitive impairment and severe obstructive sleep apnea (OSA) who underwent elective UPPP. Preoperative examination identified micrognathia and high arched palate. Post-extubation, the patient developed acute airway obstruction leading to severe hypercapnia (EtCO 2 > 60 mmHg) and critical desaturation (SpO 2 70 %). Rescue efforts, including laryngeal mask airway (LMA) insertion, were hampered by anatomical abnormalities, specifically the high arched palate and severe glossoptosis. The airway was successfully secured via re-intubation using a video laryngoscope (Glidescope ® ). Subsequent investigation confirmed the suspicion of PRS with a balanced translocation between the short arm of the X-chromosome (Xp) and the long arm of chromosome 2 (2q) on karyotyping. This case underscores the necessity of a heightened index of suspicion and meticulous preoperative airway assessment in adult patients presenting with micrognathia and OSA, even in the absence of a formal PRS diagnosis. Failure to recognize syndromic features can lead to life-threatening post-extubation airway failure following upper airway surgery.