Airway management of a child of Crouzon syndrome with midface distractor in situ

Dear Editor, The major anesthetic concern in Crouzon syndrome is related to bag-mask ventilation due to midface hypoplasia, proptosis, and presence of distractors after a correction surgery, since a mask may not fit properly.[1,2]An 8-year-old boy with Crouzon syndrome, weighing 20kg, had undergone midfacial distraction. Intubation was done following direct laryngoscopy with Cormack–Lehane grade 2 view. Twenty days later, he was posted for re-do osteotomy. On examination, mouth opening was 4cm with normal neck extension and midfacial distractor in place. Systemic examination and blood investigations were normal. The surgeons did not want to remove the distractor even temporarily, as the distraction surgery was done recently. Since removal of the distractor even for a short time soon after surgery could cause relapse of the distracted segment back to its original position. So, we planned a check video laryngoscopy under sedation, before proceeding with intravenous induction and intubation. The patient received midazolam 0.5mg, ketamine 20mg, and glycopyrrolate 0.2mg, and glottic opening was visible during check video laryngoscopy [Figure 1a]. Anesthesia was induced with propofol 40mg and fentanyl 40μg. Bag ventilation was attempted with nostrils pinched by one person and the other person holding an appropriate-sized face mask covering the mouth of the child horizontally [Figure 1b]. Neuromuscular paralysis was achieved with atracurium 10mg after confirming adequate mask ventilation.Videolaryngoscopy was performed after 3 min of mask ventilation, and the airway was secured with a preformed 5-size cuffed oral tube. The intraoperative period was uneventful. Surgery lasted for 2h, and the child was extubated when fully awake after reversing neuromuscular blockade, with the distractors in place [Figure 1c].Figure 1: (a) Check video laryngoscopy; (b) mask holding in a patient with midface distractor; (c) before extubationIn case of post-midface distraction, mechanical impingement on the reconstituted temporalis muscle, which encircles the distraction device, can cause fibrosis of the portion of the muscle above the device.[3] This may be an additional cause of difficult airway. Removal of midfacial distractors is usually done before induction of anesthesia, whenever difficult mask holding due to distractor is anticipated. This is advisable only if sufficient time has lapsed after the initial surgery and ossification of the distracted segment of the maxilla has taken place, which usually takes 3–4 months. Preoperative removal of distractor is possible with adult cooperative patients, but not in the pediatric population. By the technique described above, a child with midface distractor in situ can be ventilated through the mouth with closed nostrils and the distractor canbe removed after induction. We used atracurium instead of succinylcholine or rocuronium since mask ventilation was possible and check videoscopy showed glottis. Supraglottic device can be used for ventilation in case of difficult mask ventilation before intubation. Airway can be secured without muscle relaxant under deep plane of anesthesia also. The other option available for securing airway in pediatric patients with distractor in situ is fiberoptic bronchoscope-assisted intubation under sedation. It is concluded that mask ventilation is possible in pediatric patients with midface distractor in situ and a check laryngoscopy under sedation followed by induction and intubation can be considered a safer technique in these patients. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.