What is Diffuse Esophageal Spasm?

Diffuse esophageal spasm (DES) is an unusual condition that impacts how the esophagus, the tube connecting your mouth and stomach, functions. This involves disorganized or quickly spreading contractions that are normally strong and often result in difficulty swallowing. This is clinically established as occurrences of abnormal contractions in the esophageal smooth muscle, alternating with regular contractions in over 20% of instances when swallowing liquids with contraction strength greater than 30 mmHg.

However, with the use of advanced esophagus pressure mapping techniques, the identifying criteria for DES have changed. Now, the diagnosis typically involves at least two untimely contractions happening rapidly – in less than 4.5 seconds – functioning normally to relax the opening between the esophagus and stomach.

What Causes Diffuse Esophageal Spasm?

The cause of diffuse esophageal spasm, a condition which affects the muscles in the esophagus, is not fully understood. There are many different theories, but a general consensus is that it may be due to a lack of balance in the nerve pathways that control how food is moved down the esophagus. There may also be an excess growth of muscle in the lower part of the esophagus, which makes up about two-thirds of it. We don’t know what sets off these problems, but it’s thought that there could be an increase in a chemical called acetylcholine.

Other theories suggest possible causes could be: a problem with the function of nerve cells due to a chemical known as nitric oxide, stomach acid reflux, or it could be a nerve or muscle disorder. These could all contribute to the abnormal movement of food down the esophagus, which is seen in this condition.

Other factors could be acid exposure, which can lead to spasms in the esophagus, or heartburn, which can cause the esophagus to contract.

Some researchers have also suggested that the condition might be related to high cholesterol levels or body mass index (BMI). Additionally, blood sugar levels and BMI may affect the function of the muscle at the bottom of the esophagus, known as the lower esophageal sphincter.

Risk Factors and Frequency for Diffuse Esophageal Spasm

Diffuse esophageal spasm is a condition that affects around 1 person in 100,000 each year. It tends to be more common in white people and is seen more often in women. The likelihood of experiencing this condition increases with age and it’s rarely found in children. Based on information from referral centers, the condition affects about 4 to 7% of people.

Signs and Symptoms of Diffuse Esophageal Spasm

Diffuse esophageal spasm (DES) is a condition that affects the muscles in the esophagus (the tube that carries food from your mouth to your stomach). People with DES often experience several symptoms, including:

  • Trouble swallowing (‘dysphagia’) food and drinks
  • A feeling of something stuck in the back of the throat, known as ‘Globus hystericus’
  • Chest pain that is not from the heart, often felt behind the breastbone and can sometimes reach to the back
  • The occasional return of undigested food from the stomach
  • Heartburn, often making the diagnosis more complicated

These symptoms may come and go, and they may occur when eating quickly or drinking hot or cold beverages. Additionally, people with DES are commonly dealing with other health problems, such as anxiety and depression.

Testing for Diffuse Esophageal Spasm

When it comes to examining chest pain that’s not caused by heart issues in older patients, particularly men and white individuals, or those with swallowing difficulties, doctors often use a method called esophageal function testing. Swallowing difficulties, or dysphagia, can be consistently identified during these tests.

A test called a twenty-four-hour manometry is the most effective way to determine if the cause of the chest pain is Diffuse Esophageal Spasm (DES). To confirm the cause and check for any inflammation or abnormal growths in the esophagus, doctors usually pair this test with an endoscopy and a barium swallow test.

A manometry test will show the following results for Diffuse Esophageal Spasm:

  • More than 30% of wet swallows show no coordinated muscle movement (aperistalsis).
  • 20% of muscle movements occur all at once (simultaneous contractions).
  • There is greater strength of contractions in the last three-fifths of the esophagus (more than 30% of the force).

While manometry tests typically help in making the diagnosis, endoscopy and barium swallow tests are also useful to exclude any inflammation or tumor growths. A barium test can show a specific corkscrew or rosary beads appearance, which is a classic sign of DES, resulting from abnormal contractions leading to coiling and partitioning of the esophagus.

The development of techniques such as high-resolution manometry (HRM) and esophageal pressure topography (EPT) has significantly improved the diagnosis of DES. Studies using an ultrasound imaging technique conducted via a catheter have demonstrated an increase in muscle depth in people with DES.

To make a clear diagnosis, other conditions like diabetes, which share similar symptoms, should also be ruled out using glucose and HbA1C level tests in the blood.

Treatment Options for Diffuse Esophageal Spasm

Current treatments for esophageal spastic disorders include various medications such as nitrates, calcium-channel blockers, painkillers for the digestive tract like tricyclic agents or SSRIs, and procedures to widen the esophagus. The first choice of treatment usually includes calcium channel blockers and nitrates. If these don’t provide enough relief, doctors might recommend esophageal botox injections or a procedure to mechanically widen the esophagus.

If a patient also suffers from acid reflux disease, doctors will treat that condition as well, especially if tests reveal critical levels of acid reflux. Starting a medication called a proton pump inhibitor (PPI), taken half an hour before meals, can help ease symptoms. If PPIs work for the patient, they will generally continue this treatment for three months.

In some cases, botox injections into the esophagus are considered a safe and effective way to relieve symptoms in the short term. This option is usually only available for patients who have serious medical conditions.

In specific cases, doctors might recommend a procedure that includes a lengthwise cut (myotomy) in the esophagus or a less invasive procedure known as peroral endoscopic myotomy. These methods relax the muscles of the esophagus to help relieve symptoms. If a patient’s main issue is difficulty swallowing, they often improve after a myotomy.

For patients who don’t respond well to medication, a procedure known as a Heller myotomy combined with a surgery to prevent acid reflux may be an option.

If patients suffering from esophageal disorders also suffer from mental health conditions like anxiety and depression, they might benefit from a type of antidepressant called a tricyclic antidepressant. These drugs can help relieve both esophageal and psychological symptoms, leading to better overall outcomes.

Once a treatment is initiated or changed, it’s important to have follow-up care with a doctor. It’s crucial to monitor the effectiveness of the medication and spot any serious side effects so they can be managed properly. If medication doesn’t work, the patient might have to consider surgical treatment under a thoracic surgeon’s guidance.

When a doctor is trying to identify the root cause of severe esophageal spasms, which are painful contractions within the food pipe, several health conditions may come to mind as potential culprits. These include:

  • Achalasia (an issue with the food pipe that disrupts swallowing)
  • Angina pectoris (chest pain due to reduced blood flow to the heart)
  • Scleroderma (a rare disease causing hardening and tightening of the skin and connective tissues)
  • Vascular anomalies (abnormalities in the blood vessels)
  • Chagas disease (an infection spread by insects)
  • Esophageal cancer
  • Esophageal diverticula (pouches created by weak spots in the food pipe’s wall)
  • Gastroesophageal reflux (stomach bile flowing back into the food pipe)
  • Hypertensive LES (lower esophageal sphincter that cannot relax)
  • Esophageal motility disorder (swallowing problems caused by weak food pipe muscles)
  • Esophageal stricture (narrowing of the food pipe)
  • Ineffective esophageal motility (weak food pipe contractions)
  • Myocardial infarction (heart attack)
  • Esophageal webs and rings (extra tissues in the food pipe)
  • Esophagitis (inflammation in the food pipe)

Your doctor will consider all these possibilities in order to properly diagnose and treat your condition.

What to expect with Diffuse Esophageal Spasm

Diffuse esophageal spasm (DES) generally has a moderate prognosis, with noticeable improvements in symptoms over a three-year period. However, as chest pain symptoms can resemble those of heart, lung, and rheumatological diseases, it’s essential that a thorough medical examination is carried out to confirm a DES diagnosis.

While the risk of death from DES is low, it can significantly impact a patient’s quality of life and lead to other health complications. Some issues that can arise include:

  1. A reduced ability to eat due to associated pain.
  2. Severe pain that can limit the patient’s normal activities. This can also cause emotional and physiological stress.
  3. A significant impact on the patient’s nutritional state.

One of the key aspects of helping a patient with DES is offering counseling. Addressing the emotional and social challenges that come with this disorder is as critical as treating the physical symptoms. Patients should be reassured about the positive outcomes possible with appropriate symptom monitoring, aiming to improve their quality of life.

Possible Complications When Diagnosed with Diffuse Esophageal Spasm

Therapeutic interventions can sometimes cause unwanted reactions, which is why regular check-ups for patients are crucial.

A feared complication is the perforation (or rupture) of the esophagus after the procedure to widen it. If this happens, it may require a hospital stay and potentially surgery. If there’s suspicion of a ruptured esophagus, with symptoms such as severe vomiting of blood, the presence of air in the abdominal cavity, abnormal levels of body salts and minerals, and possible damage to the vagus nerve, urgent surgery is required to repair the rupture.

Before starting to eat and drink normally again, the patient should have a test where they swallow a contrast substance. This is to check if there’s any leak, which would need immediate treatment.

The potentials complications that might require medical attention are:

  • Wound infection
  • Atelectasis (collapse of part of a lung)
  • Pneumonia
  • Leak of air after the operation

It’s important to stay alert to these possibilities and treat them appropriately if they do occur.

Preventing Diffuse Esophageal Spasm

Teaching patients about their disease, its symptoms, and treatment options is crucial. The success of any treatment largely depends on the patient’s understanding and participation. This education helps patients identify what’s happening with their health and lets them know about the available options for treating their illness.

Frequently asked questions

Diffuse Esophageal Spasm is an unusual condition that affects the functioning of the esophagus, causing disorganized or quickly spreading contractions, resulting in difficulty swallowing.

Diffuse esophageal spasm affects around 1 person in 100,000 each year.

The signs and symptoms of Diffuse Esophageal Spasm (DES) include: - Trouble swallowing ('dysphagia') food and drinks. - A feeling of something stuck in the back of the throat, known as 'Globus hystericus'. - Chest pain that is not from the heart, often felt behind the breastbone and can sometimes reach to the back. - The occasional return of undigested food from the stomach. - Heartburn, often making the diagnosis more complicated. These symptoms may come and go, and they may occur when eating quickly or drinking hot or cold beverages. Additionally, people with DES are commonly dealing with other health problems, such as anxiety and depression.

The cause of diffuse esophageal spasm is not fully understood, but it may be due to a lack of balance in the nerve pathways that control how food is moved down the esophagus. Other possible causes include excess muscle growth in the lower part of the esophagus, problems with nerve cells due to a chemical called nitric oxide, stomach acid reflux, or nerve and muscle disorders. Other factors such as acid exposure, heartburn, high cholesterol levels, body mass index (BMI), and blood sugar levels may also affect the function of the esophageal muscles.

The other conditions that a doctor needs to rule out when diagnosing Diffuse Esophageal Spasm include: - Achalasia - Angina pectoris - Scleroderma - Vascular anomalies - Chagas disease - Esophageal cancer - Esophageal diverticula - Gastroesophageal reflux - Hypertensive LES - Esophageal motility disorder - Esophageal stricture - Ineffective esophageal motility - Myocardial infarction - Esophageal webs and rings - Esophagitis

The tests needed for Diffuse Esophageal Spasm (DES) include: 1. Twenty-four-hour manometry: This test is the most effective way to determine if the cause of chest pain is DES. It measures muscle movement in the esophagus during swallowing and can identify aperistalsis (lack of coordinated muscle movement) and simultaneous contractions. 2. Endoscopy: This test is used to check for any inflammation or abnormal growths in the esophagus. It involves inserting a flexible tube with a camera into the esophagus to visualize the lining. 3. Barium swallow test: This test can confirm the cause of DES and check for any abnormal contractions. It involves swallowing a liquid containing barium, which coats the esophagus and allows abnormalities to be seen on X-ray. In addition to these tests, glucose and HbA1C level tests may be performed to rule out other conditions like diabetes that share similar symptoms.

Diffuse Esophageal Spasm is treated with various medications such as nitrates, calcium-channel blockers, painkillers for the digestive tract like tricyclic agents or SSRIs, and procedures to widen the esophagus. The first choice of treatment usually includes calcium channel blockers and nitrates. If these don't provide enough relief, doctors might recommend esophageal botox injections or a procedure to mechanically widen the esophagus.

The prognosis for Diffuse Esophageal Spasm (DES) is generally moderate, with noticeable improvements in symptoms over a three-year period. However, it is important to conduct a thorough medical examination to confirm the diagnosis, as chest pain symptoms can resemble those of other diseases. While the risk of death from DES is low, it can significantly impact a patient's quality of life and lead to other health complications.

A gastroenterologist.

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