Esophageal spastic disorders that are idiopathic abnormalities of neural processes of the esophagus. They can be categorized into 3 types.
- Diffuse esophageal spasm (DES)
- Hypercontracting esophageal disorder of hypertensive peristalsis (nutcracker esophagus)
- Hypertensive lower esophageal sphincter (HLES)
The best study to differentiate the three types of disorder is via manometry studies. Endoscopy and Barium swallow may show same results, unless there is an episode of spasm.
Etiology and Associations
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The cause of esophageal spastic disorder is unknown (idiopathic) but they can be precipitated by drinking cold water or beverages. They can also be simulated with ergonovine (used for diagnosis) during manometry.
Diffuse esophageal spasm (DES) is associated with impairment of inhibitory innervation which results in both premature and rapidly propagating or simultaneous contraction in the distal esophagus.
Nutcracker esophagus and Hypertensive LES are both associated with premature excitatory innervation or smooth muscle response to excitatory nerves.
Symptoms and Clinical Presentation
The 3 esophageal disorder can all be symptomatic but yet does not correlate with manometry studies (which are most diagnostic at differentiating the three). This is because the symptoms can be due to acid reflux disease (GERD).
Diffuse esophageal spasm are usually symptomatic and present as intermittent chest pain in a patient without risk factors of coronary artery disease (CAD). Chest pain can simulate that of myocardial infarction, but has no relationship with exertion or and not necessary only after eating (this rules out odynophagia which is classic to pill/infectious esophagitis that only occurs during swallowing whereas DES can occur even without eating).
Dysphagia to solid and liquid food are common. In DES, the chest pain is non-cardiac in nature with a normal EKG, Stress test and coronary angiography. This is unlike prinzmetal variant angina where patients will have ST elevation on EKG.
In Hypercontacting LES and Nutcrackers esophagus, only a small portion of patients have symptoms.
Diagnosis
The most accurate and diagnostic test for esophageal spasm is manometry studies that show high intensity disorganized non-peristalsis contractions in Diffuse esophageal spasm (DES) versus normal sequential peristalsis but abnormal pressure in HLES and Nutcracker esophagus. Other diagnostic test include Barium swallow which will show corkscrew at the time of spasm in DES described as rosary beak or corkscrew.
Barium swallow show normal sequential peristalsis in the other 2 disorder. On upper endoscopy (EGD), they appear normal. Cardiac workup like EKG, stress test, coronary angiography usually are all normal.
Diagnosis should be suspected if patient develop refractory GERD, non-cardiac chest pain and esophageal dysphagia. Dysphagia patient usually are evaluated with barium swallow if there are no alarming symptoms. If there are alarming symptoms, they warrant upper endoscopy and biopsy if necessary.
If both barium swallow and upper endoscopy are normal, manometry is warranted. It is important to rule out GERD, esophageal stricture, esophagitis and esophageal webs, rings before considering esophageal motility disorder. Most can be rule out by upper endoscopy.
Treatment
Esophageal spasm are usually treated initially with calcium channel blockers (CCBs) like diltiazem or Tricyclic antidepressant (TCA) like imipramine. If patient is non-responsive to CCBs, they can consider botulinum toxin or NO based medication like sildenafil. Treatment approach with calcium channel blockers and nitrates are the same as the treatment for prinzmetal’s angina.