Will this patient have a “difficult airway?” What are the contributing reasons that you would be concerned about? Why has she been in traction? Is it likely that she has to remain in traction, or can this be released for induction? Should the halo device be removed, or should it remain? Are you surprised by the patient’s comorbidities? What developmental explanation can you offer?

Does this patient require further evaluation or consultation, and if so, with whom? Any other studies you would like?

This patient is highly likely to have a difficult airway for several reasons. First of all, she has Klippel-Feil syndrome as a result of fusion of several cervical vertebrae. Klippel-Feil syndrome severity is typically classified as type I, when patients have a single-level fusion; type II, when patients have multiple, noncontiguous fused elements; and type III, with multiple, contiguous fused segments. It may also be associated with branchial arch anomalies like Goldenhar syndrome, fetal alcohol syndrome, and anomalies of the extremities. Preoperative traction has been known to relieve sensorimotor impairment and improve the quality of the fusion repair. Depending on the type of halo fixation device, it may very well be released or portions released in order to get to the face and the patient’s head held carefully in traction by the spine surgeon. The halo in all likelihood will be replaced by Gardner-Wells tongs or a similar device for the procedure. The comorbidities of renal anomalies often occur with Klippel-Feil syndrome; hearing impairment is not uncommon as well. Sacral agenesis and a tethered cord are not typically associated, even though they represent neural crest migration defects.

It would be advisable to consult with the ORL service in case their help is needed for securing the airway or for emergency tracheotomy. Soft tissue imaging studies, such as a soft tissue x-ray of the neck, would help define the anatomic relationships, especially the relationship of the posterior pharyngeal wall, which is likely to have moved anteriorly, thus narrowing the pharyngeal cross-sectional diameter and the larynx. This is also an important consideration for the patient’s airway status postoperatively.

What monitors will you choose? Why? Does this patient need an arterial line? Why? Does this procedure require any other special monitors? Would a precordial Doppler be a reasonable choice? Why or why not?

What are your considerations for anesthetic induction? Your colleague stops by and suggests an awake intubation? What do you think? You select an intramuscular preinduction technique in the pre-op holding area with ketamine because of the extreme separation anxiety, and the patient obstructs within 30 s in the mother’s arms and begins to turn blue. What do you do next? Will an oral airway help? Is this patient a difficult intubation? Should you continue with the case?

What will be your primary anesthetic technique? Why? Will you use nitrous oxide? Why/why not? What is your choice of muscle relaxant, if any? Why? What would you consider optimal fluid management for the case? Choice of fluid? Volume?

During surgery, the patient suddenly develops a drop in blood pressure, bradycardia, and a drop in end-tidal CO₂. What is your differential diagnosis? How can you go about narrowing the possibilities? What would you do? Why? Blood pressure is 60/40 with a heart rate of 60; how would you manage his depth of anesthesia? Why?

Routine noninvasive monitoring plus an arterial line to follow mean arterial pressure. In addition, there is a chance of air embolism because as bone is decorticated, the potential point of air entry is superior to the venous system and the right atrium of the heart. The Doppler should be placed in the typical position on the anterior chest wall, but should not be allowed to compress the skin with undue pressure.

Depending on the airway assessment and the patient, anesthetic induction might precede intubation of the trachea or follow intubation. An “awake” (actually, sedated, with topical and/ or local anesthetic) can be accomplished in almost any age patient with adequate planning and time. Topical lidocaine can be nebulized or gargled, specific nerve blocks for the glossopharyngeal and superior laryngeal nerves can be administered, a transtracheal injection of lidocaine can be delivered, and intravenous sedation can supplement the entire procedure. At that point, either a direct laryngoscopy, a video laryngoscopy, or a fiber-optic intubation (transnasal or transoral) can be accomplished. The patient can also undergo an inhalation induction with preservation of spontaneous breathing. Neuromuscular blockade can also be utilized once the ability to ventilate by mask is ensured. All of these possibilities are ultimately determined by the anticipated difficulty of the airway as well as the maturity, cooperation, and willingness of the patient and family.