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Anesthesiology:
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Laryngeal Mask Airway and Fiberoptic Endoscopy in an Infant with Schwartz‐Jampel Syndrome

Theroux, Mary C. MD; Kettrick, Robert G. MD; Khine, Henry H. MD

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To the Editor:—A 20‐day‐old male infant weighing 2.5 kg with Schwartz‐Jampel syndrome was scheduled for gastrostomy tube placement and circumcision under general anesthesia. Schwartz‐Jampel syndrome is characterized by micrognathia, skeletal anomalies, dwarfism, blepharophimosis, and myotonia. [1–4] Tracheal intubation was known to be difficult because of severe micrognathia and a very anterior larynx. A complete cleft of both hard and soft palate and glossoptosis causing partial airway obstruction when awake was present as well. Awake intubation was planned for three reasons: (1) anticipated difficulty with ventilating the lungs after induction of anesthesia due to inability to displace the tongue along with severe micrognathia leading to improper mask placement; (2) reluctance to use succinylcholine because of case reports of muscle rigidity, hyperthermia, and elevated creatinine phosphokinase in a patient with Schwartz‐Jampel syndrome under general anesthesia [5]; and (3) presence of myotonia further complicating any anticipated use of succinylcholine in the event of an airway obstruction. [6].
After intravenous access was obtained and routine monitors were placed, fiberoptic endoscopy (2.2 mm) was attempted orally. Vocal cords were visualized several times, but attempts to enter the larynx with the fiberoptic endoscope failed. Failure, we believe, was due to the acute anterior flexion needed to visualize the vocal cords, preventing it from entering the larynx. In other words, acute anterior flexion rendered the scope no longer streamlined, forcing it to act like a coiled rope.
Having failed to intubate the trachea using fiberoptic intubation, two attempts were made to intubate the trachea under direct laryngoscopy using a #1 Miller blade, which failed and caused bloody secretions to obscure the airway. At this time, a size‐1 laryngeal mask airway was introduced and easily seated. The infant had clear and equal breath sounds along with loss of retractions present before laryngeal mask airway was introduced. The fiberoptic endoscope was prepared by threading serially two 3.0‐mm uncuffed endotracheal tubes without their connector adaptors. [7] The tip of the distal tube was wedged into the base of the proximal tube, essentially creating a long endotracheal tube. The fiberoptic scope was introduced through the laryngeal mask airway and easily advanced through the vocal cords. The assembly of the two endotracheal tubes was advanced over the fiberoptic scope, and thus the trachea was intubated. The junction of the two endotracheal tubes disappeared into the shaft of the laryngeal mask airway, but the presence of the distal endotracheal tube fitting snugly into the proximal endotracheal tube allowed stabilizing the endotracheal tube assembly and removing the laryngeal mask airway.
Use of the laryngeal mask airway to guide intubation of the trachea using fiberoptic endoscope has been reported. [8–10] The case presented above is the first case of laryngeal mask airway and fiberoptic intubation in an awake infant. Resuscitation of newborns using laryngeal mask airway was recently reported, [11] although intubation of the trachea was not attempted in those situations. This case illustrates that combined use of laryngeal mask airway and fiberoptic endoscopy can be performed safely and effectively in an awake infant to secure its airway, even when the infant is as small as 2.5 kg.
Mary C. Theroux, M.D.; Robert G. Kettrick, M.D.; Henry H. Khine, M.D.; Department of Anesthesiology; Alfred I. duPont Institute of Nemours Foundation; 1600 Rockland Road; P. O. Box 269; Wilmington, Delaware 19899.
(Accepted for publication November 10, 1994.)
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REFERENCES

1. Aberfeld DC. Hinterbuchner LP, Schneider M: Myotonia, dwarfism, diffuse bone disease, and unusual ocular and facial abnormalities (a new syndrome). Brain 88:313-326, 1965.

2. Edwards WC, Root AW: Chondrodystrophic myotonia (Schwartz-Jampel syndrome): Report of new case and follow-up of patients initially reported in 1969. Am J Med Genet 13:51-56, 1982.

3. Fowler WM Jr, Layzer RB, Taylor RG, Eberle ED, Sims GE, Munsat TL, Philipart M, Wilson BW: The Schwartz-Jampel syndrome: Its clinical, physiological, and histological expressions. J Neurol Sci 22:127-146, 1974.

4. Schwartz O, Jampel RS: Congenital blepharophimosis associated with a unique generalized myopathy. Arch Ophthalmol 68:82-87, 1962.

5. Seay AR, Ziter FA: Malignant hyperpyrexia in a patient with Schwartz-Jampel syndrome. J Pediatr 93:83-84, 1978.

6. Mitchell MM, Ali HH, Savarese JJ: Myotonia and neuromuscular blocking agents. ANESTHESIOLOGY 49:44-48, 1978.

7. Zagnoev M, McCloskey JJ, Martin TM: Fiberoptic intubation via the laryngeal mask airway. Anesth Analg 78:813-814, 1994.

8. Maekawa N, Mikawa K, Obara H: Laryngeal mask may be a useful device for fiberoptic airway endoscopy in pediatric anesthesia. ANESTHESIOLOGY 75:169-170, 1991.

9. Asai T: Use of the laryngeal mask for tracheal intubation in patients at increased risk of aspiration of gastric contents. ANESTHESIOLOGY 77:1029-1030, 1992.

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11. Paterson SJ, Byrne PJ, Molesky MG, Seal RF, Finucane BT: Neonatal resuscitation using the laryngeal mask airway. ANESTHESIOLOGY 80:1248-1253, 1994.

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