Esophageal Spasm

Updated: Oct 24, 2017
  • Author: Ahmad Malas, MD; Chief Editor: BS Anand, MD  more...
  • Print
Overview

Background

Broadly, esophageal spasm can be divided into 2 major variants that are distinct entities: (1) diffuse esophageal spasm and (2) hypertensive peristalsis.

Diffuse esophageal spasm is characterized by contractions that are of normal amplitude but are uncoordinated, simultaneous, or rapidly propagated (see image below).

Barium swallow demonstrates diffuse uncoordinated Barium swallow demonstrates diffuse uncoordinated contractions of the esophagus in a patient with diffuse esophageal spasm.

Hypertensive peristalsis, also known as nutcracker esophagus, is diagnosed when contractions proceed in a coordinated manner but the amplitude is excessive. Hypercontractile esophagus, also known as jackhammer esophagus, is an extreme phenotype of hypertensive contractions in which contractions are of very high amplitude, involving the majority of the esophagus, and whose duration occurs for a prolonged period with a jackhammer-type appearance on high-resolution manometry.

Symptoms can include dysphagia, regurgitation, and noncardiac chest pain. Because of the vague symptoms and difficulty in diagnosis, esophageal spasm is often underdiagnosed and therefore not adequately treated. In many patients, manometric and radiologic abnormalities may not correlate with symptom presentation.

Currently, high-resolution manometry is the best diagnostic modality. Treatment includes calcium channel blockers, botulinum toxin, nitrates, tricyclic antidepressants, sildenafil, dilatation, myotomy, and esophagectomy. Research is ongoing to determine the underlying causes to improve diagnostic capabilities and therapeutic regimens in the future. [1]

Next:

Pathophysiology

Etiology of esophageal spasm is unknown. Increased release of acetylcholine appears to be a factor (sensitive to cholinergic stimulation), but the triggering event is not known. Other theories include gastric reflux or a primary nerve or motor disorder. Body mass index (BMI) and total cholesterol may be highly predictive factors for esophageal body contractility, whereas BMI and glucose may be predictive factors for lower esophageal sphincter contractile function. [2]

The oropharyngeal swallow with primary and secondary peristaltic contractions of the esophageal body usually transports solid and liquid food boluses from the mouth to the stomach within 10 seconds. Motor abnormalities may not be present with every swallow, so dysphagia may seem to wax and wane. Solid and liquid food dysphagia suggests a neuromuscular disorder, whereas solid food dysphagia only suggests a structural problem causing mechanical obstruction. Very hot or cold liquids, loud noises, and stress may exacerbate dysphagia from esophageal spasm by stimulating muscular contractions.

The connection between unexplained chest pain and esophageal spasm was first discovered by William Osler in 1892. Nutcracker esophagus was first diagnosed in the 1970s. [3, 4]

The esophagus is composed of 2 layers of muscle, the inner circular and the outer longitudinal layers. The esophagus can be divided into 3 zones, each with separate yet integrated anatomy and physiology.

The function of the upper and lower esophageal sphincters is coordinated with the oropharynx, esophageal body, and stomach. Within the swallowing process, voluntary and involuntary control mechanisms act together. In the esophagus, the activity of the 2 types of muscle, striated (voluntary) and smooth (involuntary), is intimately coordinated. This distinction has functional significance because most esophageal motor abnormalities involve the smooth muscle portion.

A number of mechanisms for the initiation and control of esophageal motor activity are located at different levels within the central nervous system, as well as peripherally within the intramural nerves and muscles. This redundancy has implications for the operation of reserve mechanisms when a primary control mechanism is damaged or dysfunctional.

Esophageal zones

The upper zone is composed entirely of striated muscle. This zone initiates the contractions that propel the food bolus down the esophagus. The upper esophageal sphincter (UES), named the cricopharyngeus muscle, is located in the upper zone.

The middle zone is composed of striated and smooth muscle. The inner circular muscle layer and the outer longitudinal muscle layer work in conjunction to propel the food bolus down the esophagus.

The lower zone contains the lower esophageal sphincter (LES). This sphincter is a thickening of the smooth muscle that is contracted tonically to prevent reflux. At rest, the pressure in the LES is usually 15-25 mm Hg. For food to pass into the stomach, the LES relaxes at the appropriate time.

Upper esophageal sphincter

When functioning properly, the esophagus can detect the presence of a food bolus at the UES and then coordinate the progression of the food down the esophagus to the stomach. When this does not occur in a coordinated fashion, the patient can develop symptoms of esophageal spasm or oropharyngeal dysphagia.

The UES is contracted tonically. Manometric evaluation of the UES reveals constant spiking activity. As food is sensed at the UES, the laryngeal muscles contract to move the cricoid cartilage anteriorly. The tonic contraction of the UES is inhibited, opening the UES to allow the passage of food. The inner circular muscles and longitudinal muscles of the remainder of the upper zone then propel the food. To propel the food, the longitudinal muscles must contract, followed immediately by contraction of the circular muscles. At the end of the upper zone, the initial wave dies out as another wave starts, propelling the food down to the middle zone. The nucleus solitarius in the brainstem controls swallowing in the upper zone.

Esophageal middle zone

The middle zone propels the food bolus from the upper zone to the lower zone. This segment consists of 2 muscle layers, an inner circular layer and an outer longitudinal layer.

In the middle zone, the striated muscle transitions to the smooth, or involuntary, muscle. The wave propagates down the esophagus by coordinated contractions. Again, the longitudinal muscles must contract before the circular muscle contracts. Furthermore, the contraction of the muscles must proceed caudally in an organized manner. If the muscle contraction is not orderly, the food bolus cannot progress effectively.

Two forces propel the food from cephalad to caudad. First, gravity pulls the food caudally. Second, the organized contractions of the muscles propel the food caudally. If a myotomy is performed, the contractions will be ineffective. Only gravity forces the food caudally. Thus, patients who have had a myotomy can be more likely to report dysphagia.

Lower esophageal sphincter

The lower zone contains the LES. This is a condensation of the smooth muscles. Tonically, this muscle is contracted and must relax to allow food to pass. Failure of the LES to relax to allow a food bolus to pass is termed achalasia.

Diffuse esophageal spasms

Simplistically, diffuse esophageal spasms occur when the propagative waves do not progress correctly. Usually, several segments of the esophagus contract simultaneously, preventing the propagation of the food bolus. In addition, diffuse esophageal spasms can be characterized by rapid wave progression down the esophagus or distinguished by a nonperistaltic response to swallowing. The usual presentation is intermittent dysphagia with occasional chest pain. Myotomy, which is performed only in extreme cases, can relieve the uncoordinated contractions. [5, 6, 7, 8, 9]

Nutcracker and jackhammer esophagus

Nutcracker and jackhammer esophagus occur when the amplitude of the contractions exceed 2 standard deviations from normal. The contractions proceed in an organized manner, propelling food down the esophagus. These patients present with chest pain more often than they present with dysphagia, as they experience less dysphagia than patients with diffuse esophageal spasm.

Because the progression of the contractions occurs normally, patients often do not benefit from a myotomy. Even though the increased amplitude of the contractions can be demonstrated using manometry, the symptoms often do not correlate with the manometrically documented contractions.

The symptoms of diffuse esophageal spasm, nutcracker esophagus, and jackhammer esophagus may overlap and can be distinguished only by a motility study.

Previous
Next:

Epidemiology

United States statistics

The incidence of esophageal spasm is about 1 case in 100,000 population per year. [4] The symptoms range from mild to severe. Patients with mild symptoms often do not seek medical attention. Because of the similarity of symptoms of reflux disease and esophageal spasm, many patients may be misdiagnosed with reflux. Furthermore, reflux and spasm can occur concomitantly.

International statistics

Because the symptoms are mild (or even absent) in many patients, the true incidence is not known.

Race-, sex-, and age-related statistics

Esophageal spasm seemingly is more common in whites, and it may be more common in women than in men. However, esophageal spasm is rare in children, and its incidence increases with age.

Previous
Next:

Prognosis

Prognosis is moderate. Symptom scores improve over time (3 y or longer) in diffuse esophageal spasm and nutcracker esophagus. The mortality rate is minuscule, but the morbidity rate is high.

No treatment is effective in all patients. Some patients do not respond to any treatment. In most patients, symptoms are controllable with a combination of treatment modalities.

Mortality/morbidity

Mortality is very rare, but morbidity can be significant.

Morbidity arises from an inability to eat, secondary to the pain, and the subsequent decline in the nutritional status. The pain can be incapacitating, preventing normal activity and leading to considerable psychological challenges and impairment in the patient's quality of life. The chest pain can mimic cardiac, pulmonary, or rheumatological chest pain, instigating appropriate workup.

Primary esophageal motility disorders rarely progress and are not known to be fatal.

Complications

Potential problems are based on the therapy. All medications have possible adverse effects, and patients should be monitored.

Esophageal perforation can occur with esophageal dilatation, leading to admission to the hospital, time lost from work, and possible surgery.

Operative complications are the same as for any operation on the esophagus. Esophageal perforation can occur during myotomy. If a perforation of the mucosa occurs, the defect should be closed and the patient should have a contrast-swallowing test prior to resuming oral feeding. Vagal injury can occur during the dissection. Other postoperative complications include wound infection, atelectasis, pneumonia, and persistent air leak. Any complication associated with a thoracic operation or an esophageal operation can occur.

Previous
Next:

Patient Education

Patients should be educated about the symptoms and treatment options for the disease. Patient involvement and education are crucial to the success of any treatment modality.

For patient education resources, see Botox Injections.

Previous