Anesth Analg 2006;103:1611-1613
© 2006 International Anesthesia Research Society
doi: 10.1213/01.ane.0000246588.32148.0a
LETTER TO THE EDITOR
Editor-in-Chief Steven L. Shafer
Anesthetic Issues with a Hyperextended Cervical Spine in a Child with Emery-Dreifuss Syndrome
Dinesh K. Choudhry, MD, FRCA, and
William G. Mackenzie, MD
Department of Anesthesiology; Alfred I. DuPont Hospital for Children; Nemours Children’s Clinic; Wilmington, DE; Jefferson Medical College; Philadelphia, PA (Choudhry)
Department of Orthopaedics; Alfred I. DuPont Hospital for Children; Nemours Children’s Clinic; Wilmington, DE; Jefferson Medical College; Philadelphia, PA (Mackenzie)
To the Editor:
Emery-Dreifuss syndrome, a childhood-onset, slowly progressive, humeroperoneal, muscular dystrophy with contractures and cardiomyopathy with arrhythmias, has significant anesthetic implications (1,2). We report our experience in managing a 16-year-old teenager with EDS, weighing 58 kg, who had a severely hyperlordotic cervical spine (Fig. 1). He was scheduled for posterior cervical release with halo and vest application using Ilizarov equipment.
Routine examination revealed a cooperative teenager with no history of palpitations or shortness of breath. He had trivial mitral regurgitation and Holter indicated atrial arrhythmia. He had Class I airway and had bilateral good air entry with mild restrictive lung disease. His extremities were contracted at the elbows, hips, and knee joints.
In view of his unusually severe extension deformity of the cervical spine (110°), we prepared for a potentially difficult tracheal intubation. Following placement of routine monitors and IV line, we administered oxygen to him and induced anesthesia with propofol, placing the patient in the left lateral decubitus position. After confirming that easy mask ventilation was possible, we administered rocuronium. With direct laryngoscopy (DL), we could visualize only the posterior comissures. We successfully inserted a styleted, reinforced, endotracheal tube (ETT) into the trachea and secured it with tape with the 23-cm mark on the incisors. The patient remained stable during the surgical procedure. After tracheal extubation in the operating room, we transferred him to the intensive care unit with oxygen administered by facemask. The subsequent postanesthetic course was uneventful.
We considered several relevant anesthetic issues with our patient. First, because performing endotracheal intubation was difficult in a supine position, we placed the patient in the left lateral decubitus position to obviate having to elevate his entire trunk to accommodate for his extreme backward head position and to move his tongue to the left, thereby facilitating visualization of the larynx. We succeeded in intubating our patient on the first attempt.
Second, the larynx moves posterocephalad with flexion and anterocaudad with extension of the cervical spine (3). In our patient, we noticed via DL that the larynx was quite anterior with only posterior comissures in view (Fig. 3). Therefore, we chose a styleted, reinforced, flexible, nonkinking ETT instead of a regular, "kyphotic" ETT. Because the kyphotic ETT does not fit the hyperlordotic tracheal axis, it can cause not only pharyngeal injury, but also tracheal and esophageal perforation (4,5).
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Figure 3. Computed tomography scan showing hyperlordotic trachea conforming to the curvature of the cervical spine.
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Third, displacement of the ETT toward the vocal cords by neck extension and toward the carina by neck flexion is well-documented (6,7). To avoid accidental tracheal extubation, we inserted an ETT of longer length, an action that does increase the risk of the ETT advancing endobronchially, when the cervical lordosis is corrected. Therefore, one must pay special attention to the ETT’s position in such patients. Finally, positioning our patient prone was quite challenging, as shown in Figure 2. Head placement was especially difficult because of excessive neck extension (Fig. 2).
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Figure 2. Patient positioned prone for surgery. Note the head position with only his chin in contact with the prone headrest.
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REFERENCES
- Emery AE. Emery-Dreifuss syndrome. J Med Genet 1989;26:637–41.[Abstract/Free Full Text]
- Aldwinckle RJ, Carr AS. The anesthetic management of a patient with Emery-Dreifuss muscular dystrophy for orthopedic surgery. Can J Anaesth 2002;49:467–70.[Abstract/Free Full Text]
- Brechner VL. Unusual problems in the management of airways. I. Flexion-extension mobility of the cervical vertebrae. Anesth Analg 1968;47:362–73.[Free Full Text]
- Johnson KG, Hood DD. Esophageal perforation associated with endotracheal intubation. Anesthesiology 1986;64:281–3.[ISI][Medline]
- Tsujimoto S, Yamazaki K, Kato H. Pharyngeal perforation following endotracheal intubation in a patient with rheumatoid arthritis. J Anesth 1993;7:113–15.[Medline]
- Sugiyama K, Yokoyama K. Displacement of the endotracheal tube caused by change of head position in pediatric anesthesia: evaluation by fiberoptic bronchoscopy. Anesth Analg 1996;82:251–3.[Abstract]
- Jin-Hee K, Ro YJ, Seong-Won M, et al. Elongation of the trachea during neck extension in children: implications of the safety of endotracheal tubes. Anesth Analg 2005;101:974–7.[Abstract/Free Full Text]
