Achalasia is the best defined primary motility disorder and the only one with an established pathology. The predominant neuropathologic process of achalasia involves the loss of ganglion cells from the wall of the esophagus, starting at the LES and developing proximally. The degree of ganglion cell loss parallels the disease duration such that, at 10 years, ganglion cells are likely completely absent. At the LES, the loss of inhibitory nerves is demonstrated by loss of nitric oxide synthase and vasoactive intestinal peptide (VIP) immunohistochemistry staining. Variable amounts of inflammatory cells have been described within the myenteric plexus along with the disappearing nerves. In the peristaltic esophageal body, achalasia is characterized by a loss of intrinsic acetylcholine-containing nerves.
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The typical picture of achalasia. Note the "bird-beak" appearance of the lower esophageal sphincter (LES), with a dilated, barium-filled esophagus proximal to it. Image courtesy of Andrew Taylor, MD, Professor, Abdominal Imaging, Department of Radiology, University of Wisconsin Medical School, Madison.
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The response to amyl nitrate (a smooth muscle relaxant), with partial relaxation of the lower esophageal sphincter (LES), allows some barium to pass through it into the stomach. Image courtesy of Andrew Taylor, MD, Professor, Abdominal Imaging, Department of Radiology, University of Wisconsin Medical School, Madison.
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Esophagram of a 65-year-old man with rapid-onset dysphagia over 1 year. Although esophagram shows a typical picture of achalasia, this patient had adenocarcinoma of the gastroesophageal junction. This is an example of pseudoachalasia, which reinforces the absolute need for esophagogastroduodenoscopy (EGD) in patients with radiologic diagnosis of achalasia. Image courtesy of Andrew Taylor, MD, Professor, Abdominal Imaging, Department of Radiology, University of Wisconsin Medical School, Madison.
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An esophagram demonstrating the corkscrew esophagus picture observed in a patient with manometry confirmed findings of diffuse esophageal spasm (DES). Image courtesy of Andrew Taylor, MD, Professor, Abdominal Imaging, Department of Radiology, University of Wisconsin Medical School, Madison.
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Response to amyl nitrate, with disappearance of the spasm on esophagram. Image courtesy of Andrew Taylor, MD, Professor, Abdominal Imaging, Department of Radiology, University of Wisconsin Medical School, Madison.
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Normal manometry results show normal esophageal body peristalsis with normal lower esophageal sphincter (LES) pressure and relaxation. The LES pressure tracing is at the level of the sleeve (tracing 6).
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Achalasia manometry picture Note the nonrelaxing lower esophageal sphincter (LES) and the absence of esophageal body peristalsis. The LES pressure tracing is at the level of the sleeve (tracing 6).
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Manometry demonstrates diffuse esophageal spasm with simultaneous contractions of the esophagus observed throughout the tracing. The lower esophageal sphincter (LES) pressure tracing is at the level of the sleeve (tracing 6).
