Pseudocholinesterase Deficiency in a Patient with Subglottic Stenosis

Abstract

Subglottic stenosis is a narrowing of the subglottic space, which extends from the inferior border of the vocal cords to the lower border of the cricoid cartilage. Patients often present with shortness of breath, which worsens with the degree of the stenosis. A common treatment for subglottic stenosis involves rigid bronchoscopy under general anesthesia. This chapter presents a case of a woman with subglottic stenosis who underwent a rigid bronchoscopy with balloon dilation under general anesthesia with propofol, fentanyl, and succinylcholine. Succinylcholine is a muscle relaxant of frequent choice for very short procedures as it is rapidly broken down by pseudocholinesterase, resulting in the short duration of clinical activity. At the conclusion of this case, the patient had prolonged weakness following the single dose of intravenous succinylcholine, which normally produces muscle relaxation for 7–10 min. The patient was supported until she recovered to baseline strength and was able to be successfully weaned from ventilatory support and sedation. Subsequent workup demonstrated a low level of pseudocholinesterase activity, explaining the prolonged muscle relaxation following succinylcholine administration. This chapter discusses the clinical manifestations and treatment options for subglottic stenosis, the anesthetic considerations for rigid bronchoscopy, the mechanism of action and side effects of neuromuscular blockade with succinylcholine, the management of a patient with residual neuromuscular blockade, and the pathology and genetics behind pseudocholinesterase deficiency.