Airway Management for Treacher Collins Syndrome with Limited Mouth Opening

Patients with Treacher Collins Syndrome (TCS) present unique airway management problems for anesthesiologists due to mandibular micrognathia, the small oral aperture, and temporomandibular joint anomalies. We describe the case of a pediatric TCS patient with limited mouth opening who experienced severe airway obstruction during deep inhalation anesthesia (sevoflurane following i.v. midazolam) for routine dental work. When difficult airway management is expected, intubation of conscious patients is a well-recognized technique in adults; however, it is rarely appropriate for pediatric patients who usually do not cooperate. According to general anesthesia algorithms for pediatric patients with difficult airways, in most pediatric patients, tracheal intubation is performed after the induction of general anesthesia and some authors have reported the usefulness of LMA for maintaining airway patency in patients with TCS. However, in our case LMA could not be used because of severe limitation of mouth opening. In addition, the LMA is so bulky that it is impossible to insert a LMA into patients with narrow airway anatomy. We initially planned to carry out fiber-optic intubation while awake and under sedation if the airway patency could not be secured after the induction of anesthesia. The patient was sedated properly with midazolam and sevoflurane, and awake fiberoptic intubation was performed uneventfully. Our experience in this case highlighted that careful planning of backup contingencies is important in achieving fiberoptic intubation and maintaining airway patency in pediatric TCS patients with limited mouth opening, and that awake intubation can be successful even in pediatric patients.