The incidence rate of CHARGE syndrome is presumed to be 0.1-1.2/10,000 live births [
3]. Coloboma and other ocular abnormalities are reported in 75-90% of CHARGE syndrome patients. Cardiac malformation is reported in 75-85% of all cases, while choanal atresia is reported in 65% of all cases [
4]. Craniofacial anomalies, micrognathia, anterior larynx, cleft palate, and enlarged tonsils and adenoid often accompany CHARGE syndrome, leading to many airway problems. Upper airway collapse or subglottic stenosis due to laryngomalacia can also occur [
5]. Tracheostomy is necessary for 29% of patients [
6]. As seen above, in treating CHARGE syndrome patients, general anesthesia and perioperative airway management are persistent challenges. Many attempts have been made to overcome these issues. Hara et al. [
7] have tried the laryngeal mask airway (LMA) in CHARGE syndrome patients and found it to be successful. They have reported that since CHARGE syndrome patients have smaller pharynges and larynges than normal children, a smaller LMA should be used first. However, in some types of surgery, successful LMA insertion is difficult. We also had to turn the head to a lateral position for the cochlear implant. The use of LMA was limited to due to the long operation time. In our hospital, it took three hours for the right cochlear implant and three hours 55 minutes for the left cochlear implant. Shimizu et al. [
8] have reported a successful intubation case using GlideScope
® (Verthon, USA). They mentioned that this tool is useful for difficult airway management due to an upper airway malformation. Use of a GlideScope
® for intubation in CHARGE syndrome patients has also been reported to be successful [
9]. We used C-MAC
® with a D-blade for pediatrics, which is similar to a GlideScope
®. The use of the C-MAC
® video laryngoscope in the setting of a predicted difficult airway has resulted in an improved laryngeal image and a higher success rate of tracheal intubation than in direct laryngoscopy [
10]. Teoh et al. [
11] has reported that C-MAC
® has the easiest blade insertion. It gave the best clarity of laryngeal views, prevented fogging of the lens, and offers automatic white balance. The D-blade, which was essentially designed for management of difficult airways, is half-moon shaped with increasing blade angulations from 18° to 40°. The high blade angulation enables optimal glottic visualization in almost all patients [
12]. It may also prevent the need for additional maneuvers and further extension of the head [
13]. However, it has limitations, such as longer intubation time and difficult tube passage. Due to the anterior location of the larynx, large tonsils, acute angle of C-MAC
® video laryngoscope, and failure of BURP maneuver, several attempts may be needed for successful intubation, even with the use of C-MAC
®. Since the blade is angled more, the tip of the intubation tube has to be directed to the larynx at a right angle. This leads to difficulty in passage or in navigation of the endotracheal tube toward the larynx, despite adequate visualization of the glottis [
13]. Sun et al. [
14] have found that the hockey-stick-like J-curvature of the stylet at the end of the tube, with the tube passed from the lateral side of the patient’s mouth, is more successful for placing the tip of the endotracheal tube in the glottis. We also made a tube with a stylet shaped like a hockey stick. However, the intubation failed. After we rotated the tube clockwise, we intubated successfully. Despite these limitations, a better view was obtained with the C-MAC D-blade than with the direct laryngoscope, and we achieved excellent visualization of the glottic opening, resulting in a high success rate.
In conclusion, many surgical interventions are required for CHARGE syndrome patients. Since airway abnormality is present in many cases, preoperative airway evaluation is very important. For difficult intubation, it should be kept in mind that LMA or video laryngoscope could be used. Though tracheostomy is safe, it could be invasive. Therefore, we recommend using a video laryngoscope since it is non-invasive and useful. It seems to be the best option for successful intubation in patients with CHARGE syndrome.