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Esophageal Motility Disorder/Eosinophilic Esophagitis


 

This information is intended only to provide general guidance. It does not provide definitive medical advice. It is important that you consult your doctor about your specific condition.


Difficulty swallowing (dysphagia) can occur if there is disturbance of muscular coordination (motility) in the esophagus or if there is an obstruction in the esophagus.

If the muscles at the back of the mouth and throat are not working in a coordinated fashion, called oropharyngeal dysphagia, there may be difficulty or choking when transferring food or liquids from the mouth into the esophagus or a feeling of food stuck in the throat. This most frequently occurs due to a neurologic disorder, such as a stroke, Parkinson's disease, ALS, or cerebral palsy. Treatments include adding thickening agents to drinks, physical/speech therapy, and medications for the neurological disorder.

The esophagus is a hollow, muscular tube that carries food from the back of the mouth down through the chest to the stomach. There are short valves, called the upper esophageal sphincter and the lower esophageal sphincter (LES) at the top and bottom of the esophagus. The portion of the esophagus between the two valves is called the esophageal body. Peristalsis is the coordinated muscular contraction that sweeps down the esophagus and pushes food and liquid down the esophagus into the stomach.

If there is an esophageal motility disorder, the muscles of the esophagus do not function with proper coordination, and you may sense food or liquids getting stuck or going down slowly in the throat or chest, or you may feel chest pain. Examples of esophageal motility disorders are achalasia, diffuse esophageal spasm, and nutcracker esophagus. These individual diseases will be discussed below.

Esophageal manometry, also called esophageal motility testing or esophageal function testing, is one of the most useful tests to evaluate swallowing disorders. Dr. Harary is one of the doctors who performs esophageal manometry in New York City. Esophageal manometry measures the pressure and coordination of the muscles of the lower esophageal sphincter and esophageal body, using a thin catheter with multiple sensors along its length. It is a safe procedure and has no significant risks. In addition to evaluating swallowing disorders, esophageal manometry can also be used to evaluate chest pain felt to be originating from the esophagus, as an additional test before doing ambulatory esophageal reflux pH monitoring, and before anti-reflux surgery is done.

Other tests that may be useful in evaluating swallowing disorders include upper GI endoscopy (EGD) and an X-ray test called a video-esophagram, in which barium, a thick liquid, is swallowed while X-rays are taken of the chest and esophagus as the barium passes through.


Achalasia

Achalasia is a severe disturbance of esophageal muscular coordination (motility) which causes difficulty swallowing, regurgitation and chest pain. In achalasia, there is a loss of peristalsis (the coordinated muscular contraction that moves down the esophagus) and a failure of relaxation of the lower esophageal sphincter (LES), so that food and fluid stay in the esophagus and do not move down into the stomach. Symptoms usually develop over several months, although some patients may have had symptoms for many years.

Achalasia is diagnosed with an x-ray test called a video-esophagram, upper GI endoscopy (EGD), and esophageal manometry. Achalasia can be treated in several ways:

  1. Pneumatic dilation, which New York City gastroenterologist Dr. Harary performs, is used in the treatment of achalasia. During this procedure, a large balloon at the end of a thin tube is passed through the mouth and inflated in the lower esophageal sphincter (LES), thus stretching and disrupting the muscle that does not properly relax and thus allowing food to leave the esophagus more easily. This is not the same type of esophageal dilation that is used to treat narrowing (stricture or rings) in the esophagus. Pneumatic dilation is an outpatient procedure that has a brief recovery period of only hours. There is a 2-4% chance of causing a tear through the wall of the esophagus, which may require emergency surgery if it occurs. A second pneumatic dilation may be required in 2-4 weeks, using a larger balloon, if the first balloon does not sufficiently relieve symptoms. Good results are obtained in 80-90% of achalasia patients.
  2. Laparoscopic Heller myotomy, in which a cut is made surgically in the muscle of the lower esophageal sphincterfollowed by a fundoplication, in which the upper part of the stomach is loosely wrapped around the lower end of the esophagus to prevent reflux from developing. The Heller myotomy is usually done using a laparoscope, a thin instrument that allows a much smaller incision and easer recovery than open surgery. It is about 90% effective for achalasia patients, but does require general anesthesia and carries the infrequent risks of surgery and anesthesia. Dr. Harary can refer you a surgeon who is skilled in doing laparoscopic Heller myotomy.
  3. Botulinum toxin (Botox) injection into the lower esophageal sphincter. Botox is injected through a needle at the end of a long catheter that is passed during an upper GI endoscopy (EGD). It helps 60-70% of achalasia patients, but the benefit usually lasts only 1 to 1-1/2 years. Therefore, it is usually reserved for achalasia patients with other health problems that make more invasive achalasia treatments too risky, or if the cause of the swallowing difficulty is unclear.
  4. POEM (per-oral endoscopic myotomy), in which the muscle of the lower esophageal sphincter (LES) is cut during an upper GI endoscopy (EGD), allowing the muscle which was not properly relaxing to now permit food and fluid to exit from the esophagus. This is a very new but promising technique. It appears to be very effective, but can cause complications and usually requires a 1-3 day stay in the hospital. Once the diagnosis of achalasia is made, Dr. Harary can refer you to one of the few gastroenterologists who performs POEM.

Once the diagnosis of achalasia is made, the choice of which procedure to use to treat achalasia is made after a discussion between the patient and the gastroenterologist.


Diffuse Esophageal Spasm

Diffuse esophageal spasm is an esophageal motility disorder that can cause difficulty swallowing or chest pain. In diffuse esophageal spasm, the entire muscle of the esophagus sometimes contracts at the same time instead of the normal peristalsis contractions that move down the esophagus. The diagnosis of diffuse esophageal spasm is made by esophageal manometry, which shows frequent simultaneous contractions mixed with normal peristalsis contractions. Diffuse esophageal spasm is usually treated with medications that relax the muscle of the esophagus, for example calcium channel blockers (diltiazem, nifedipine, and verapamil), anti-spasmodics (hyoscyamine, dicyclomine, propantheline, and methscopolamine), or nitroglycerin, which are either taken by mouth or dissolved under the tongue.


Nutcracker Esophagus

Nutcracker esophagus and jackhammer esophagus are esophageal motility disorders in which the esophageal muscle contracts in a coordinated fashion, but the contractions are too strong and often prolonged. They can cause either chest pain or difficulty swallowing. The diagnosis and treatment are the same as for diffuse esophageal spasm.


Hypertensive Lower Esophageal Sphincter

Hypertensive lower esophageal sphincter is an esophageal motility disorder in which the lower esophageal sphincter (LES) relaxes normally, but its resting pressure is abnormally high. Peristalsis contractions in the body of the esophagus are normal. Hypertensive lower esophageal sphincter may cause symptoms such as difficulty swallowing or chest pain. It can be treated with medications, Botox injection into the lower esophageal sphincter, and esophageal dilation.


Scleroderma And The Esophagus 

Esophageal motility disorders can also occur as a consequence of disease affecting other parts of the body. The most common is scleroderma or other collagen vascular disorders (lupus, mixed connective tissue disease, undifferentiated collagen vascular disorder). The smooth muscle of the esophagus is damaged and weakened with scleroderma, causing weakness of the lower esophageal sphincter and decreased or absent peristalsis contractions in the esophageal body. This can cause severe gastroesophageal reflux disease (GERD) symptoms, frequently complicated by scarring and narrowing of the esophagus, and impaired propulsion and clearance of food, fluid, and acid in the esophagus. Treatment is often the same as for other patients with gastroesophageal reflux disease (GERD), but esophageal dilation is often required if there is narrowing/scarring/stricture of the esophagus. A video-esophagram x-ray, upper GI endoscopy (EGD), and esophageal manometry are used in the evaluation of esophageal problems in scleroderma.


Obstruction Of The Esophagus

Obstruction of the esophagus can occur due to scarring and narrowing of the esophagus (stricture or stenosis), a lower esophageal ring (Schatzki ring), tumors, or eosinophilic esophagitis (see below). These conditions can cause the same symptoms as in esophageal motility disorders but involve structural and not functional abnormalities.

Esophageal stricture (stenosis): The passage of food can be slowed or blocked by narrowing in the esophagus. This can occur as a consequence of chronic gastroesophageal reflux disease (GERD), causing scarring and inflammation, by the ingestion of caustic substances, sometimes a long time in the past, by radiation therapy to the chest, or other rare diseases. The main treatment is usually esophageal dilation, which is quite effective.

Lower esophageal ring (Schatzki ring) is a common condition in which there is a very short, abrupt narrowing at the lower end of the esophagus. The typical symptom is either intermittent food impaction (food lodged in the lower esophagus) or chronic difficulty swallowing food. It is treated by esophageal dilation, which usually causes a dramatic improvement

Tumors of the esophagus (cancer) are uncommon causes of difficulty swallowing, but it is necessary to see a gastroenterologist to evaluate any new difficulty with swallowing because this could be due to a tumor. It is extremely important to diagnose esophageal cancer as early as possible. Esophageal cancer is more common in smokers, heavy alcohol users, and patients with Barrett's esophagus, but it can occur in anyone. It can be treated if diagnosed early. Esophageal cancer is usually diagnosed by upper GI endoscopy (EGD).


Eosinophilic Esophagitis

This is a common condition in which there is allergic type inflammation of the inner lining of the esophagus, resulting in obstruction to the passage of food through the esophagus. It occurs in both children and adults, and is more commonly seen in people who have other allergies, such as allergic rhinitis ("hay fever"), asthma, or atopic dermatitis. The most frequent symptom is difficulty swallowing. Gastroesophageal reflux disease (GERD) can sometimes cause a condition that mimics eosinophilic esophagitis; if GERD is the cause, the condition will improve when treated with acid-reducing medications.

Eosinophilic esophagitis is diagnosed by upper GI endoscopy (EGD), in which large numbers of eosinophils are found in biopsies of the lining of the esophagus. Eosinophils are type of white blood cells that are involved in the immune response and allergic conditions. Once this is diagnosed, a 6-8 week trial of proton pump inhibitors (acid-reducing medications) is the first treatment, and then another upper GI endoscopy (EGD) is done to distinguish between eosinophils in the esophagus due to GERD and due to allergic eosinophilic esophagitis. If eosinophils are still present on the second EGD, and then allergic eosinophilic esophagitis is present. Skin or blood allergy testing is not usually very useful, although it occasionally provides some hint as to the cause of the allergy.

Treatment may include medications (swallowing asthma corticosteroid inhaler mist), esophageal dilation, or elimination diets followed by gradual reintroduction of individual foods. Another upper GI endoscopy (EGD) is necessary after a period of treatment to check whether the eosinophils have been eliminated from the biopsies of the esophageal lining. Eosinophilic esophagitis should always be treated, both to relieve symptoms and to prevent more severe narrowing of the entire esophagus in the future.

Location

Albert M. Harary, MD
110 East 55th Street, 17th Floor
Midtown East/Upper East Side

New York, NY 10022
Phone: 212-702-0123

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