Nutcracker Esophagus (Hypertensive Peristalsis)?

Nutcracker esophagus is a form of esophageal spasm. It is in the same category of esophageal motility disorders as jackhammer esophagus and diffuse esophageal spasms. In the U.S. about 1 in 100,000 individuals are diagnosed with an esophageal motility disorder per year, however, the true incidence is thought to be much higher since it is often misdiagnosed as acid reflux.

Esophageal spasms may be more common in Caucasian people and occur more frequently in women than men. The older you become the more likely you are to get esophageal spasms.

The characterizing features of nutcracker esophagus include high pressures and spasms in the esophagus that occur in a coordinated manner. Spasms of the esophageal muscles can cause food to become stuck or lodged in the esophagus. With nutcracker esophagus, however, because the spasms still occur in a coordinated and organized manner it is more likely to experience chest pain than difficulty swallowing.

It is not known exactly what causes nutcracker esophagus. Some theories are that the condition is closely related to gastroesophageal reflux disorder (GERD) and may be caused by it. Another theory is that it is caused by a nerve disorder, and some people believe it is caused by an abnormal response to the substance acetylcholine.

Symptoms of Nutcracker Esophagus

Nutcracker esophagus may cause a combination of some or all of the following symptoms:

• difficulty swallowing (dysphagia) which occurs with both solid and liquid food and may occur only sometimes
• chest pain
• regurgitation of food
• reflux

It is also possible for nutcracker esophagus to be asymptomatic (not cause noticeable symptoms). Some research suggests that people with nutcracker esophagus may have an esophagus that is hypersensitive and stiff.

The symptoms of other motility disorders (jackhammer esophagus and diffuse esophageal spasm) are almost the same as nutcracker esophagus so special testing must be done to differentiate between these disorders.

Diagnosing Nutcracker Esophagus

The following tests can help your healthcare provider to diagnose nutcracker esophagus:

1. A barium swallow is a test that involves swallowing a substance called barium sulfate. Barium sulfate shows up on x-rays so it's possible to visualize the path of barium sulfate through the digestive system. In addition to esophageal spasms, this test can help to diagnose problems like difficulty swallowing or narrowing of the esophagus. You should not have this test if you are allergic to barium sulfate but generally speaking, the test has a good safety record. However, barium sulfate is known to cause constipation so you may need to drink a lot of water or use an over the counter stool softener after the test.
2. A CT scan may show thickening of the esophageal wall (greater than 3mm) in individuals who have nutcracker esophagus. However, this can also occur in other conditions including cancer of the esophagus. If your CT scan shows thickening of the esophageal wall your healthcare provider will need to order further testing to confirm your diagnosis.
3. High-frequency ultrasound can be helpful in distinguishing between nutcracker esophagus, jackhammer esophagus, and diffuse esophageal spasm.
4. Esophageal Manometry is a very good test for diagnosing esophageal spasms and causes of non-cardiac chest pain. The test involves the insertion of a thin tube into your nose, down your throat and esophagus and into your stomach. The tube is pressure sensitive and can measure the strength and pressure of muscle contractions in the esophagus while you swallow. This test can cause some discomfort which is eased with a spray that numbs the inside of the nose and sometimes the top of the throat. 
5. (EGD)Esophagogastroduodenoscopy is a procedure that cannot specifically diagnose nutcracker esophagus but may be useful in ruling out other similar disorders such as a hiatal hernia or GERD.

Treatment of Nutcracker Esophagus

You and your healthcare provider may choose to use some of the following treatments to help manage the symptoms of nutcracker esophagus. 

Several medications are used to treat this condition and include calcium channel blockers, nitrates, and phosphodiesterase inhibitors. Injections of botulinum toxin above the lower esophageal sphincter can temporarily relieve symptoms by blocking the release of acetylcholine.

Proton pump inhibitors can help to relieve symptoms related to acid reflux. One of the most effective medications for esophageal motility disorders including nutcracker esophagus is tricyclic antidepressants.

Balloon dilation of the esophagus is a procedure that stretches the diameter of the esophagus and can relieve symptoms of nutcracker esophagus. The procedure is usually done on an outpatient basis and under sedation.

You will receive special instruction about not eating and drinking for a certain time period before your procedure. Risks of balloon dilation include side effects or allergic reactions to the anesthesia type medications used or rarely, perforation of the esophagus.

Surgery May Be Warranted If Treatments Are Ineffective

In extreme cases that haven't responded well to other treatment, a surgical procedure called a myotomy may be warranted. This procedure is also called a Heller myotomy or esophageal myotomy and is most often used to treat achalasia but can be used to treat nutcracker esophagus as well. It is used as a last resort because in some cases it has actually caused the muscle contractions associated with nutcracker esophagus to become worse.

The procedure can be done laparoscopically or as an open procedure but the laparoscopic method is associated with less risk and shorter recovery time. A small incision is made just above the belly button and then the outer muscle layers of the esophagus are cut to help prevent them from contracting. Additionally, weakening the muscles at the gastroesophageal junction causes the sphincter between the stomach and esophagus to remain open.

The Risks Involved With Surgery

The risks involved in esophageal myotomy include damage to surrounding organs including the spleen, liver, or stomach as well as post-operative infection. As with any surgical procedure, there is also a risk of an allergic reaction or side effects of anesthesia. 

As previously mentioned when used to treat nutcracker esophagus there is a risk that symptoms will worsen. Even if symptoms do subside after a period of years it is possible that the positive effects of this procedure will wane.

In addition to the aforementioned treatments research suggests that nutcracker esophagus seems to get better on its own within a few years.