History
Achalasia
Note the following:
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Progressive dysphagia for both solids and liquids is the hallmark of achalasia. Dysphagia for solids is more common than for liquids.
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Retrospectively, symptoms are present on average as long as 6 years prior to presentation.
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Regurgitation of food retained in the proximal dilated esophagus is a common occurrence, especially at night, requiring patients to sleep using multiple pillows or upright in a chair. This symptom worsens as the esophagus dilates with time.
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Chest pain may be another early symptom, characterized by a squeezing retrosternal pain radiating to the neck, jaw, arms, or back. The chest pain may worsen with food and can awaken patients from sleep.
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A sensation of heartburn may be reported by 30% of patients and is assumed to be related to retained food fermentation and lactic acid.
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Emotional stress or rapid eating may worsen all of the symptoms described above.
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Weight loss is common with achalasia; however, the loss is usually slight.
Spastic esophageal motility disorders
Chest pain is the hallmark of spastic esophageal motility disorders, although patients with spastic esophageal motility disorders also may report dysphagia. Similar to the chest pain of achalasia, it may mimic angina. The mechanism is not clear but may be related to transient esophageal muscle ischemia, luminal distension, or altered visceral sensation.
Dysphagia is not necessarily related to chest pain. Dysphagia for solids and liquids is a common symptom and especially seen in DES. Dysphagia may be intermittent and nonprogressive in nature, typically not prolonging mealtime or causing weight loss.
Patients also commonly report heartburn, regurgitation, or other esophageal complaints of reflux disease due to ineffective acid clearance from the esophagus.
Weight loss is common with achalasia; however, the loss is usually slight.
Scleroderma esophagus
Scleroderma involves the esophagus in more than 75% of patients, regardless of clinical type. Two forms of this disease exist–(1) progressive systemic sclerosis (PSS), characterized by diffuse scleroderma, and a more fulminant form with early involvement of internal organs or (2) CREST syndrome, characterized by calcinosis, Raynaud phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasia. The severity of esophageal involvement does not correlate necessarily with severity of involvement of other organs. In fact, dysphagia may be the presenting clinical symptom in some patients.
The esophageal symptoms of scleroderma usually reflect the severity of acid reflux disease, including heartburn, regurgitation, and dysphagia.
Erosive esophagitis is observed in as many as 60% of patients, and the incidence of Barrett esophagus and adenocarcinoma of the esophagus is increased.
Dysphagia usually is due to diminishing peristalsis, peptic strictures, or a combination of both.
Physical Examination
In patients with primary motility disorders, results of a physical examination often are unrevealing.
Clinical signs of scleroderma in the proper clinical setting must be noted, especially skin changes.
A bedside swallowing challenge may be performed with a glass of water.
Evaluate the patient's general nutrition and hydration status if significant dysphagia is reported.
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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).
