What is Diffuse Esophageal Spasm?
The esophagus is a muscular tube that connects your mouth to your stomach. When you swallow food, the muscles of the esophagus contract and relax in a coordinated sequential manner from top to bottom to push the food down to your stomach. In diffuse esophageal spasm (DES)the muscles behave spastically and their uncoordinated and non-sequential motion is unable to propel the food properly. This disease may be chronic and progressive in nature or the symptoms may come and go.
What is the Cause of Diffuse Esophageal Spasm?
The direct cause of DES is still unknown. Gastric Reflux Disorder is a proposed cause of DES. Pathology specific to esophageal muscle or nerve cells has also been proposed to explain this disease. In DES there is a disruption of the nerves that control the muscular action of swallowing which causes an increased amount of a chemical named acetylcholineto be released. Acetylcholine is a muscle-stimulating chemical that causes hyperactivity of the esophageal muscles and it is this hyperactivity and inability to effectively swallow food that produces the symptoms of DES.
What are the Symptoms of Diffuse Esophageal Spasm?
The most common symptom of DES is a difficulty in swallowing both solid foods and liquids. Patients may have to swallow repeatedly or change positions in order to swallow food. Painful swallowing is also a symptom of DES. Because of all the muscular spasms in the esophagus, DES patients can have chest pain that is very severe. The pain may involve the entire chest and even move down the arms and up to the jaw just as the pain of a heart attack does. Many times patients that present with these symptoms are worked up first to ensure that they are not experiencing a heart attack. Other symptoms of DES include: heartburn, retching, regurgitating food, and feeling as if something is stuck in your throat.
How is Diffuse Esophageal Spasm Diagnosed?
As discussed above, often patients are worked up for a heart attack (or coronary artery disease) before being worked up for DES. Once that work-up excludes the heart for coronary artery disease, an esophagogram is often done as the next step. That is, if your doctor thinks of this diagnosis! This test is an x-ray of the esophagus taken while the patient swallows Barium. Barium is a liquid contrast material that appears white on the x-ray and enables the doctor to visualize the shape of the esophagus as the muscles contract to push the food towards the stomach. In DES patients the esophagus does not look like a tube, but rather like a corkscrew or a rosary beadpattern. These patterns represent the lack of coordination of the esophageal muscles.
Manometry is the most accurate test used to diagnose DES. Manometry involves placing a balloon in the esophagus to measure the pressure in different segments. Measurement of the pressures allows the physician to determine if the muscles in the esophagus are contracting too much or out of sequence, two phenomenon that can occur in DES. A positive manometry test for DES will reveal more than two uncoordinated esophageal contractions in ten attempts at swallowing.
A CT Scan may also be requested by the doctor to evaluate the disease in the esophagus. In DES, however, a CT Scan will reveal fairly non-specific findings. Often the only abnormality seen on CT Scan (if any) is a thickened wall of the esophagus.
What is the Treatment of Diffuse Esophageal Spasm?
The simplest treatment of DES is avoidance of very hot or very cold foods as they are thought to precipitate the painful muscle contractions that are the hallmark of this disease. Aside from food avoidance, some medications may offer relief to DES patients. Medications include:
1. Nitrates-These drugs come in both short (delivered under the tongue) and long-acting forms. An example of a nitrate is nitroglycerin. Nitrates act to inhibit the spasm of the blood vessels in the chest that can contribute to chest pain.
2. Calcium Channel Blockers- These drugs decrease the intensity of the muscle contractions in the esophagus and thus diminish the chest pain experienced by DES patients.
3. Tricyclic Antidepressants- tricyclic antidepressants, originally marketed for their effective relief of depression, are also a treatment for DES. Imipramine is a tricyclic antidepressant that has been demonstrated by an unknown mechanism to decrease the chest pain felt by individuals experiencing esophageal spasm.
4. Botulinum Toxin- Botulinum toxin (commonly known as Botox) has also been used for DES. Injections of the toxin bind to nerve endings and prevent the release of the chemical Acetylcholine. By blocking Acetylcholine, the toxin is able to prevent the muscle spasms from occurring. This treatment is not perfect, however, and the inhibitory cost and the tolerance the body develops to the injected toxin make it a temporary relief of the pain of DES.
Aside from medications, two other routes are available for DES treatment. Esophageal dilatation with a balloon is a procedure that may provide temporary relief of the muscles spasms. Another option is reserved for severe cases of DES associated with extreme chest pain or DES that has not been controlled with medications and diet modification. A surgery called a esophageal myotomy is the most radical treatment option available. In this procedure, a surgeon cuts the muscles along the entire length of the esophagus to relieve the spasms. In addition to the myotomy, most surgeons also perform another procedure to reduce the risk of esophageal obstruction and reflux. The Lower Esophageal Sphincter (a circular muscle connecting the esophagus to the stomach) is cut to prevent spasm that would lead to the inability of food to pass from the esophagus into the stomach. However, when the Lower Esophageal Sphincter is cut, the chance of refluxing food from the stomach back into the esophagus is increased. In order to prevent reflux a third surgical procedure may be done in which part of the stomach is wrapped and secured around the esophagus (Nissen fundoplication). Aside from general surgical complications such as infection, difficulty swallowing is a complication of the myotomy surgery that may occur.
What is the Prognosis of Diffuse Esophageal Spasm?
Many patients with DES have to try several of the previously discussed therapies for relief. Some patients will have complete resolution of their symptoms, but most will have a lessening of the symptoms.
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