What Is Corkscrew Esophagus?

When it comes to problems with the esophagus, heartburn or acid reflux, are the conditions that usually come to mind. While acid reflux is common, there is another rare condition that can cause similar symptoms. A case report in the New England Journal of Medicine featured an elderly woman with a very puzzling diagnosis—what doctors call a "corkscrew esophagus."

According to the report, the 83-year-old woman sought treatment at a gastroenterology clinic for dysphagia (aka, swallowing difficulties) and regurgitation after every meal. The woman also reportedly suffered from chest pain after eating. For years she suffered from difficulty swallowing both solids and liquids, but her condition got worse in the year before she sought treatment. Over the course of that year, she also reported a weight loss of about 20 pounds.

A barium esophagogram—essentially a special type of X-ray—showed a corkscrew pattern in the woman's esophagus. She was eventually diagnosed with type III (spastic) achalasia (more on that later) that manifested with a corkscrew esophagus.

What Exactly Is a Corkscrew Esophagus?

To understand corkscrew esophagus, also called rosary bead esophagus, you need to know how the esophagus should work, said Scott Gabbard, MD, a gastroenterologist at Cleveland Clinic. When a person with a healthy esophagus swallows, different parts of the esophagus squeeze at different times. The top squeezes, then the middle, then the bottom (this process is technically known as peristalsis), and this helps propel food through the organ.

When you have corkscrew esophagus, "The entire esophagus squeezes at once, and you end up with this corkscrew appearance," said Dr. Gabbard, adding that the esophagus also squeezes too fast. That happens because the body's immune system has attacked the nerves that release nitric oxide, which causes the esophagus to relax. Without that relaxing agent, the esophagus can spasm painfully.

What Are the Symptoms?

Corkscrew esophagus appears in conditions affecting the movements of the esophagus. In this woman's case, it was a condition called achalasia, a condition that results from nerve damage and makes it difficult for the body to pass food and liquid to the stomach. Other times, corkscrew esophagus can occur in a condition called diffuse esophageal spasm (DES), which is another disorder of how the esophagus moves and is often accompanied by difficulty swallowing. Both achalasia and DES are very rare, however.

Chest pain, dysphagia (difficulty swallowing), and gastrointestinal reflux disease (GERD) can all be symptoms of corkscrew esophagus. And because it can make swallowing and eating difficult, a corkscrew esophagus may also be accompanied by weight loss over time.

How Is It Treated?

The good news: There are a few treatment options for this condition, the main one being surgery, said Dr. Gabbard. The surgery—which is known as a myotomy and can also be performed endoscopically—involves making an incision in the lining of the esophagus, said Dr. Gabbard. "It stops the spastic contractions [and] relaxes that bottom valve so patients can swallow," explained Dr. Gabbard.

Another possible treatment, which the woman in the case report opted for, is an injection of boltulinum toxin (botox) into the esophagus. Botox can be effective in the short term because it stops nerves in the esophagus from releasing a substance that causes muscle spasms. However, these injections only work for about six months, said Dr. Gabbard.

Five months after being treated with endoscopic botulinum injections, the patient with achalasia was doing better: She wasn't regurgitating food, but she was still experiencing difficulty swallowing now and then.

Cases of DES can be treated with medicines like calcium blockers and proton pump inhibitors. But they may not always be effective, as was the case for an 84-year-old man diagnosed with DES reported in The American Journal of Medicine. After the medicines failed, the man was treated with repeated endoscopic balloon dilatation, a procedure where a balloon is inserted into the esophagus and inflated, and saw a significant improvement in his symptoms.

What Causes Corkscrew Esophagus?

Healthcare providers don't know what triggers corkscrew esophagus. One theory is that the condition is caused when the immune system attacks the nerves that release nitric oxide after the body fights off a virus. However, healthcare providers don't know which virus that is, and, again, they aren't positive that's what triggers the condition.

Also worth repeating is that these two esophageal conditions are extremely rare. Achalasia occurs in about one in 100,000 people per year. "The spastic variant is less than a fifth of achalasia [cases]," said Dr. Gabbard. However, "we certainly do see this," added Dr. Gabbard. DES is equally rare and occurs in around one in 100,000 people per year.

Dr. Gabbard has seen cases of corkscrew esophagus that were misdiagnosed for months or years. A healthcare provider who isn't a specialist might hear the symptoms and assume it's a reflux condition. For this reason, it's essential to visit a center that has the testing capabilities needed to diagnose corkscrew esophagus if you continue to have difficulty swallowing.

A Quick Review

Corkscrew esophagus is a rare condition that occurs as a result of esophagus conditions like achalasia or DES. Treatment options are available and may include surgery, botox, or medications. Corkscrew esophagus is difficult to diagnose and may be misdiagnosed as acid reflux. So, if you are having symptoms, consult your healthcare provider for more information.