Approach Considerations
A diagnosis of achalasia should be considered when a patient presents with dysphagia, chest pain, and refractory reflux symptoms after an endoscopy does not reveal a mechanical obstruction or an inflammatory cause of esophageal symptoms. [11]
The Chicago classification is a system that separates achalasia into three subtypes of esophagogastric junction outflow disorders. The manometric findings common to all types of achalasia include impaired relaxation of the lower esophageal sphincter (LES) (residual pressure or integrated relaxation pressure of ≥10 mmHg) and absent peristalsis in a patient without mechanical obstruction near the LES. The types are differentiated as follows [12] :
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Type I: Incomplete relaxation of the LES with aperistalsis and absence of esophageal pressurization
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Type II: Incomplete relaxation of the LES with aperistalsis and panesophageal pressurization in at least 20% of swallows
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Type III: Incomplete relaxation of the LES with premature (spastic) contractions with distal contractility integral (DCI) >450 mmHg s cm with ≥20% of swallows
The American College of Gastroenterology released new guidelines for the diagnosis and management of achalasia in September, 2020. [13] Recommendations for the proper diagnosis of achalasia include the following:
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Evaluate patients with suspected GERD who do not respond to treatment for achalasia
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Esophageal pressure topography is preferred to conventional line tracing
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Classify achalasia subtypes by the Chicago Classification to help inform prognosis and treatment plan
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Symptomatic patients with suspected achalasia should undergo upper endoscopy to rule out pseudoachalasia and exclude other pathology
Laboratory studies are generally noncontributory.
Esophageal pressure topography (EPT) is the preferred assessment modality of esophageal motility over conventional line tracings (CLT). [13, 14] Six attending gastroenterologists and six gastroenterology fellows from three academic centers interpreted each of the 40 studies using both EPT and CLT formats. Among all raters, the odds of an incorrect exact esophageal motility diagnosis were 3.3 times higher with CLT than with EPT, and the odds of incorrect identification of a major motility disorder were 3.4 times higher with CLT than with EPT. [14]
Perform an esophagogastroduodenoscopy (EGD) to rule out cancer of the gastroesophageal junction or fundus. If a tumor is suspected, perform an endoscopic ultrasound at the same time.
Imaging Studies
Barium swallow
The esophagus appears dilated, and contrast material passes slowly into the stomach as the lower esophageal sphincter (LES) opens intermittently. The distal esophagus is narrowed and has been described as resembling a bird's beak (see the image below).
Achalasia. Barium swallow demonstrating the bird-beak appearance of the lower esophagus, dilatation of the esophagus, and stasis of barium in the esophagus.
Other Tests
High resolution esophageal manometry (see the images below) is the criterion standard in helping to diagnose the classic findings of achalasia. [15] These findings include the following:
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Incomplete relaxation of the lower esophageal sphincter (LES) in response to swallowing
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High resting LES pressure
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Absent esophageal peristalsis
Achalasia. High-resolution manometry of patient with achalasia type I. Top one third (in purple) represents fluid-filled esophagus by impedance, with incomplete emptying between swallows. Below this, upper horizontal dark-red band represents upper esophageal sphincter; orange band at bottom with interspersed dark-red areas represents lower esophageal sphincter. In between these two bands, it can be noted that there is no panesophageal pressurization and no peristalsis in the esophageal body. Image courtesy of R Matthew Gideon, Albert Einstein Medical Center.
Achalasia. High-resolution manometry of patient with achalasia type II. Top one third (in purple) represents fluid-filled esophagus by impedance. Bottom two thirds (in orange) represents pressurized esophagus. Dark-red band at top of orange area represents upper esophageal sphincter (UES); dark-red band at bottom represents lower esophageal sphincter (LES). Note isobaric simultaneous contractions and elevated intraesophageal pressure (orange area) along with impaired LES relaxation (high resting pressure and incomplete relaxation). Image courtesy of R Matthew Gideon, Albert Einstein Medical Center.
Achalasia. High-resolution manometry of patient with achalasia type III. Top one third represents impedance portion of study. In bottom two thirds, in between orange horizontal bars representing upper and lower esophageal sphincters, vertical bands that represent esophageal body contractions can be seen. Note areas that are equivalent to spastic contraction, which can even obliterate esophageal lumen. Image courtesy of R Matthew Gideon, Albert Einstein Medical Center.
Prolonged esophageal pH monitoring is important for the following reasons:
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To rule out gastroesophageal reflux disease (GERD)
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Achalasia. Barium swallow demonstrating the bird-beak appearance of the lower esophagus, dilatation of the esophagus, and stasis of barium in the esophagus.
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Achalasia. Manometric evaluation of the esophagus in a patient with achalasia. Pertinent findings include absence of propulsive peristalsis in the body of the esophagus (note simultaneous contractions), elevated resting lower esophageal sphincter (LES) pressure, and the absence of LES relaxation.
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Achalasia. Heller myotomy extending 1.5 cm onto the gastric wall.
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Achalasia. Dor fundoplication, left row of sutures (after division of short gastric vessels).
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Achalasia. Completed Dor fundoplication.
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Achalasia. High-resolution manometry of patient with achalasia type I. Top one third (in purple) represents fluid-filled esophagus by impedance, with incomplete emptying between swallows. Below this, upper horizontal dark-red band represents upper esophageal sphincter; orange band at bottom with interspersed dark-red areas represents lower esophageal sphincter. In between these two bands, it can be noted that there is no panesophageal pressurization and no peristalsis in the esophageal body. Image courtesy of R Matthew Gideon, Albert Einstein Medical Center.
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Achalasia. High-resolution manometry of patient with achalasia type II. Top one third (in purple) represents fluid-filled esophagus by impedance. Bottom two thirds (in orange) represents pressurized esophagus. Dark-red band at top of orange area represents upper esophageal sphincter (UES); dark-red band at bottom represents lower esophageal sphincter (LES). Note isobaric simultaneous contractions and elevated intraesophageal pressure (orange area) along with impaired LES relaxation (high resting pressure and incomplete relaxation). Image courtesy of R Matthew Gideon, Albert Einstein Medical Center.
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Achalasia. High-resolution manometry of patient with achalasia type III. Top one third represents impedance portion of study. In bottom two thirds, in between orange horizontal bars representing upper and lower esophageal sphincters, vertical bands that represent esophageal body contractions can be seen. Note areas that are equivalent to spastic contraction, which can even obliterate esophageal lumen. Image courtesy of R Matthew Gideon, Albert Einstein Medical Center.
