Perioperative airway complications in infants and children with Crouzon and Pfeiffer syndromes: A single-center experience

Background: Crouzon and Pfeiffer syndromes are rare genetic disorders characterized by craniosynostosis, exorbitism, and maxillary hypoplasia. Patients with these syndromes frequently require general anesthesia for various diagnostic and surgical procedures and may present a challenge to anesthetists with regard to airway management.

Aims: The primary aim of this study was to determine the incidence, timing, and management of perioperative upper airway obstruction in infants and children with Crouzon and Pfeiffer syndromes. The secondary aim was to determine the degree of difficulty in performing endotracheal intubation.

Methods: A retrospective review of 812 anesthetic encounters in 67 patients was conducted. The following were recorded: timing and management of episodes of perioperative upper airway obstruction, from induction of anesthesia to discharge from recovery, degree of difficulty with laryngoscopy using the Cormack-Lehane grading system and number of intubation attempts required, patient demographics, respiratory comorbidity, surgical procedure, and anesthetic airway management techniques.

Results: Upper airway obstruction at induction of anesthesia was very common, with an incidence of 31% (167/542 anesthetic encounters affecting 54 patients). In a quarter of these incidents, bag-valve-mask ventilation was challenging, but a laryngeal mask airway was almost always effective. Upper airway obstruction on emergence from anesthesia was less common, with an incidence of 2.7% (14/515 anesthetic encounters affecting 10 patients). Contributing factors included patient comorbidity (obstructive sleep apnea, nasal stenosis) and the nature of surgery (craniofacial or airway procedures). Intubation was rarely difficult in this cohort, with 85% of laryngoscopies rated Cormack-Lehane grade 1 or 2 (n = 373), and 89% of intubations successful on the first attempt (n = 306).

Conclusions: Upper airway obstruction at induction of anesthesia is common in patients with Crouzon and Pfeiffer syndrome. These patients are likely to present some difficulties with perioperative airway management, especially bag-valve-mask ventilation, but rarely endotracheal intubation.