A healthy 50-year-old white woman presented to the emergency department with acute-onset
stridor and dyspnea. Although she had been diagnosed with achalasia 12 years earlier,
she had declined therapy and had experienced minimal dysphagia. On arrival at the
hospital, she was found to be cyanotic and bradycardic, and required emergency endotracheal
intubation. She was extubated soon afterwards, but required nebulized racemic epinephrine
because of persistent expiratory stridor. Computed tomography showed dilatation of
the proximal cervical esophagus that did not impinge on the trachea ([Figure 1]). However, a more distal computed tomographic slice showed the esophagus to be severely
dilated and compressing the posterior wall of the trachea ([Figure 2]). Upper endoscopy revealed a dilated esophagus with normal gastric and esophageal
mucosa. After dilation of the lower esophageal sphincter with a 20-mm balloon, the
patient felt dramatically better, with complete resolution of her stridor. She subsequently
underwent an uncomplicated Heller myotomy with Dor fundoplication.
Figure 1 Computed tomographic image showing the cervical esophagus at a level where it was
not impinging on the trachea.
Figure 2 Thoracic computed tomographic image showing severe tracheal compression resulting
from a massively dilated esophagus.
Airway obstruction due to achalasia has been reported primarily in women beyond their
fifth decade of life. Several mechanisms have been proposed. Early reports suggested
that dysfunction of the cricopharyngeal muscle, associated with cephalad movement
of a dilated esophagus, causes progressive esophageal dilatation [1]. More recently, a defective upper esophageal sphincter belch reflex has been implicated
[2]
[3]. Therapy is first directed at securing the airway. Patients with respiratory failure
due to achalasia frequently require endotracheal intubation or emergency esophageal
decompression. We recommend that stable patients are cared for in a highly monitored
environment with facilities for rapid endotracheal intubation prior to esophageal
decompression, as oropharyngeal manipulation can exacerbate a delicate airway situation.
Heller’s myotomy is the most common long-term decompression strategy, but good results
have also been reported with injection of botulinum toxin (Botox) into the lower esophageal
sphincter [4]
[5].
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