Abstract
Safe airway management is essential, as even brief airway obstruction under anesthesia can lead to brain injury. Patients with PTEN hamartoma tumor syndrome (PHTS) undergo tonsillectomy more frequently than the general population. Although physical features of PHTS are associated with difficult intubation, prior literature has not addressed airway management in pediatric PHTS cases. This case highlights the importance of preoperative airway evaluation and planning to ensure safe airway management. The case was a four-year and three-month-old boy with PHTS who visited our hospital with a one-month history of cough and snoring. His tonsils were remarkably enlarged, and sleep apnea was observed. Tonsillectomy was planned, and preoperative nasopharyngolaryngoscopy revealed multiple polyps in the hypopharynx and larynx. He was previously diagnosed with difficult intubation because macrocephaly limited head movement, obstructing visualization of the glottic orifice during colorectal polypectomy. Therefore, we considered that multiple polyps in the hypopharynx and larynx could be another risk factor for difficult intubation. For safety management, fiberoptic nasotracheal intubation was selected, and airway complications were prevented. Preoperative airway assessment beyond bedside physical examination is necessary to accurately identify the risk of difficult intubation and consider safe airway management plans to avoid the worst-case scenario of airway management.