Treacher Collins syndrome has features of mandibulo-facial dysostosis because of a first branchial arch defect (1). Because of these anatomical features, patients with Treacher Collins syndrome are difficult to intubate. In cases such as these, it is important to evaluate the upper airway before the induction of anesthesia to safely manage the patient’s airway.We found that three-dimensional computed tomography (3D-CT) images were useful in the preoperative evaluation of the patient’s airway and these images guided our airway management in a patient with Treacher Collins syndrome.
A 13-year-old girl, 148 cm tall and 29.2 kg weight, with Treacher Collins syndrome was scheduled for plastic surgery on the left auricle. She had undergone plastic surgery four times and had been difficult to intubate. In the past, after a slow induction of anesthesia with sevoflurane, an intubating laryngeal mask airway (Fastrach®, Jersey Island, UK) was inserted and ventilation was initiated through the laryngeal mask. Intubation was attempted with the use of a bronchoscope through a Fastrach. With this technique, the glottis was barely visible and more than an hour was required to successfully intubate the trachea.
To evaluate the upper airway and to formulate an anesthetic management plan, a preoperative 3D-CT-guided image of the patient’s airway was performed. The 3D-CT produced images that showed: (a) the mandible was hypoplastic (Fig. 1A), the mesopharynx was narrow (Fig. 1B), and (b) the median axis of the larynx was left of the median axis of the pharynx (Figs. 1C and D).
Because the patient was relatively uncooperative, an awake intubation was avoided. Because of the findings of (a), we decided that an inhaled induction with sevoflurane posed a high risk of obstructing the upper airway. Therefore, we decided to insert an oral airway after an IV induction in order to better facilitate airway patency. From the previous anesthetic information and the finding (b), we decided to intubate the trachea with a bronchoscope, but to avoid the use of a laryngeal mask. The absence of a laryngeal mask airway would allow the unrestricted movement of the bronchoscope and better enable the vocal cords to be visualized. To secure ventilation during the induction, we used two more devices: an intubating pharyngeal airway (8 cm, Berman Intubating Pharyngeal Airway, Vital signs, Totowa, NJ) and an endoscopy mask (Endoscopy Mask, VBM Medizintechnik, Sulz, Germany) (Fig. 2). The anesthesia emergency airway set was immediately available.
No premedication was administered, and anesthesia was induced with fentanyl, propofol, and atropine sulfate. No muscle relaxants were given. We inserted the intubating pharyngeal airway immediately after the induction of anesthesia and ventilation was supported manually with a bag and the endoscopy mask. In consideration of the deviation between the axis of the larynx and oral cavity (Fig. 1C), we positioned the tip of the intubating pharyngeal airway toward the left and tilted the patient’s head slightly to the right. A bronchoscope was inserted through a slit in the endoscopy mask and through the intubating pharyngeal airway; the glottis could then be visualized. After topical anesthesia of the larynx with lidocaine was performed, the bronchoscope was easily passed into the trachea. It took approximately 10 min to complete the intubation and no desaturation and/or adverse cardiac events were observed. The remainder of the anesthesia was uneventful.
Treacher Collins syndrome is a congenital disease characterized, in part, by mandibulo-facial dysostosis because of the first branchial arch defect (1). Because of these congenital abnormalities, this syndrome causes a difficult airway. There are some reports that showed that bronchoscope-guided intubation through a laryngeal mask airway was a useful technique for intubating patients with Treacher Collins syndrome (2–5). In a previous operation on this patient, bronchoscope-guided intubation through a Fastrach was attempted, but the glottis was barely visible. We evaluated the plain CT images of seven patients with Treacher Collins syndrome at our hospital and noted that the present patient’s deviation of the trachea from the midvertebral line was 10.0 mm (Fig. 1D). This deviation was more than those of the other patients (mean 3.6 mm, range from 2.4 to 5.2 mm). Deviation of the trachea from the midvertebral line is one major reason for the difficulties experienced during attempts to intubate through the Fastrach in this patient’s prior anesthetics. The Fastrach usually fits the oral cavity, but because the median axis of the larynx was deviated to the median axis of the oral cavity, the glottis did not lie in front of the opening of the Fastrach. Thus, we recommend taking 3D-CT when a patient’s larynx shifts considerably in the lateral direction. Although we evaluated seven patients with plain CT images, only two patients were evaluated with 3D-CT. Thus, we cannot provide any definitive statement about the prediction of the direction of deviation from the patient’s appearance, such as the asymmetry of the mandible.
In conclusion, although a careful risk/benefit analysis will need to be considered in younger patients when sedation may be needed to perform 3D-CT-guided images, preoperative anatomical evaluation of the airway using 3D-CT images in a patient who is predicted to be difficult to intubate contributes to safe airway management.
The authors thank Dr. Jiro Maegawa for providing information about other Treacher Collins syndrome patients.
1. Posnick JC, Ruiz RL. Treacher Collins syndrome: current evaluation, treatment, and future directions. Cleft Palate Craniofac J 2000;37:434
2. Nilsson E, Ingvarsson L, Isern E. Treacher Collins syndrome with choanal atresia: one way to handle the airway. Paediatr Anaesth 2004;14:700–1
3. Takita K, Kobayashi S, Kozu M, Morimoto Y, Kemmotsu O. Successes and failures with the laryngeal mask airway (LMA) in patients with Treacher Collins syndrome—a case series. Can J Anaesth 2003;50:969–70
4. Inada T, Fujise K, Tachibana K, Shingu K. Orotracheal intubation through the laryngeal mask airway in paediatric patients with Treacher-Collins syndrome. Paediatr Anaesth 1995;5: 129–32
5. Ebata T, Nishiki S, Masuda A, Amaha K. Anaesthesia for Treacher Collins syndrome using a laryngeal mask airway. Can J Anaesth 1991;38:1043–5