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Begin a calcium channel blocker
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0/4
Begin botulinum toxin injections
Endoscopic balloon dilation of the lower esophageal sphincter
Upper endoscopy
100%
4/4
Myotomy with fundoplication
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In diagnosing achalasia, one must first rule out malignancy with an endoscopic evaluation. After a barium swallow and esophageal manometry suggest achalasia, then further management and definitive treatment can be started. Recall that achalasia is a motor disorder of the distal esophagus resulting from degeneration of Aurbach's plexus. It is the most common motility disorder and is often found in patients under 50. The lower esophageal sphincter fails to relax during swallowing. As a consequence, natural peristalsis is disrupted and the patient experiences dysphagia to solids and liquids, with liquids often being most problematic. A barium esophagram is helpful in making the diagnosis and should reveal the classic bird's beak tapering at the esophageal sphincter. This is the first step in management. Subsequently, diagnosis may be confirmed with esophageal manometry. Once endoscopy is completed, palliative treatment may begin. Treatment includes medical management consisting of calcium channel blockers, botulinum toxin injections, and surgical therapy may include endoscopic balloon dilation of the lower esophageal sphincter or a more invasive option, myotomy with fundoplication. Spieker discusses the evaluation of dysphagia. The differential is wide and includes stroke, gastroesophageal reflux disease, medication side effects, and malignancy as a few potential causes. Barium esophagram and gastroesophageal endoscopy can confirm the diagnosis. However, due to its cost effectiveness, barium esophagram should be the first step in management. Farrokhi and Vaezi discuss idiopathic (primary) achalasia. It is a rare disease with an annual incidence of only 1/100,000 and a prevalence of 1/10,000. It is similarly found in men and women and usually diagnosed between 25 and 60. Association of achalasia with viral infections and auto-antibodies against the myenteric plexus has been reported. Endoscopic examination is important to rule out malignancy as the cause of the achalasia. Treatment is palliative. Figure A depicts the classic birds beak appearance found in an esophagram in an achalasia patient. Incorrect Answers: Answers 1, 2, 3, and 5: All of these are potential treatments for achalasia. However, treatment should not begin until malignancy is ruled out with an upper endoscopy.
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