Achalasia in Northern Virginia

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Achalasia is a rare disorder of the esophagus in which the muscular valve at the bottom of the esophagus does not relax when you swallow, and the esophagus itself stops pushing food downward. Food and liquid collect above the valve instead of passing into the stomach. Because its early symptoms overlap with acid reflux, achalasia is often treated as reflux for months or years before it is recognized. This guide explains what achalasia is, how it differs from other swallowing problems, how it is diagnosed, and how the main treatments compare.

This article is general education, not medical advice. The right tests and treatment depend on your symptoms, your test results, and your overall health, so talk with your physicians about your own situation.

What achalasia is

The esophagus is the muscular tube that carries food from the throat to the stomach. Normally a wave of muscle contractions, called peristalsis, moves each swallow downward, and a ring of muscle at the bottom, the lower esophageal sphincter, relaxes to let it through. The American College of Gastroenterology (ACG), in its 2020 clinical guideline on achalasia (Vaezi and colleagues), which remains the ACG's current guideline on the topic, describes achalasia as a disorder in which both steps fail: the sphincter does not relax enough, and peristalsis is abnormal or absent.

The ACG explains that the underlying cause is still unknown. The leading explanation is a loss of the nerve cells in the lower esophagus that tell the muscle to relax, which leaves the signals that tighten the muscle unopposed. MedlinePlus, from the National Library of Medicine, notes that in some people the problem may be inherited.

How common achalasia is

Achalasia is uncommon. The ACG guideline estimates that only 20,000 to 40,000 people in the United States have it. It affects men and women equally, shows no racial pattern, and is most often diagnosed between ages 30 and 60. Most gastroenterologists see far more reflux, strictures, and eosinophilic esophagitis than achalasia, which is part of why it can take time to identify.

Symptoms of achalasia

The ACG lists the classic symptoms of achalasia as:

• Difficulty swallowing both solids and liquids. This is the hallmark. A narrowing such as a stricture usually affects solid food first, while achalasia tends to affect liquids as well, and it gradually worsens.
• Regurgitation. Undigested food or saliva comes back up, sometimes hours after a meal or when lying down at night. It is different from vomiting, because the food never reached the stomach.
• Chest pain. MedlinePlus notes it may increase after eating and can be felt in the back, neck, or arms.
• Heartburn. The ACG reports heartburn in 27 to 42 percent of people with achalasia.
• Weight loss. Varying degrees of weight loss or nutritional deficiency can develop as eating becomes harder.

MedlinePlus also lists cough among the symptoms. Regurgitated food can be breathed into the lungs, which the ACG identifies as a risk of untreated or advanced achalasia.

New or severe chest pain should always be treated as a possible heart problem first. If you have chest pain with shortness of breath, sweating, or pain spreading to the arm or jaw, call 911 rather than waiting for a digestive evaluation.

Why achalasia is often mistaken for acid reflux

Heartburn, chest discomfort, and food coming back up are also the main symptoms of GERD, which is far more common. The ACG guideline notes that people with achalasia are frequently diagnosed with reflux at first and treated with acid-reducing medicines called proton pump inhibitors (PPIs), and that this often delays the correct diagnosis significantly.

For that reason, the ACG recommends that patients who are thought to have GERD but do not respond to acid-suppressing treatment be evaluated for achalasia. If you have been taking a PPI as directed and still have regurgitation or trouble swallowing, that is worth raising with a physician. Our guide to GERD that is not responding to medication walks through the full list of reasons and the usual order of testing.

How achalasia is diagnosed

The ACG describes three tests that work together: upper endoscopy, a barium swallow X-ray, and esophageal manometry. No single symptom confirms achalasia, so the diagnosis rests on these results.

Upper endoscopy

The ACG states that anyone with suspected achalasia should always have an upper endoscopy. A thin, flexible camera is passed through the mouth, usually with sedation, to look at the esophagus and stomach. In achalasia, the physician may see a widened esophagus holding saliva or food and a tight opening into the stomach. The guideline notes that these signs are more obvious in advanced disease and can be absent early on, so a normal-looking endoscopy does not rule achalasia out.

Endoscopy has a second purpose: making sure nothing else is blocking the lower esophagus. A tumor near the junction of the esophagus and stomach can mimic achalasia, a situation called pseudoachalasia. The ACG advises particular caution when an older adult has lost significant weight over a short time, and notes that a CT scan or endoscopic ultrasound may be used in those cases. Biopsies taken during endoscopy can also identify eosinophilic esophagitis, another cause of difficulty swallowing.

Barium swallow

For a barium swallow, also called an esophagram, you drink a chalky liquid that shows up on X-ray. In achalasia, the images can show a widened esophagus that narrows to a point at the bottom, a shape radiologists call a bird's beak. A timed version of the test takes images at 1, 2, and 5 minutes to measure how much barium remains in the esophagus. The ACG notes that early achalasia can be missed on a barium study or misread as a reflux-related narrowing.

Esophageal manometry

The ACG calls high-resolution manometry the gold standard test for confirming achalasia. A thin, flexible tube lined with pressure sensors is passed through the nose into the esophagus while you are awake, and you take a series of small sips of water. The sensors record how the esophagus squeezes and whether the lower sphincter relaxes. Unlike endoscopy, it is done without sedation.

A newer tool, the functional lumen imaging probe (FLIP), measures how easily the lower sphincter opens using a balloon placed during a sedated endoscopy. The ACG describes its role as still evolving and notes it may help people who cannot tolerate manometry or whose manometry results are unclear.

The three types of achalasia

Manometry results are read using the Chicago Classification, an international system for esophageal motility disorders that is now in version 4.0. It divides achalasia into three types. In all three the lower sphincter fails to relax; they differ in what the rest of the esophagus does.

• Type I. The esophagus shows no contractions and little pressure. The ACG reports it accounts for 20 to 40 percent of cases.
• Type II. There is no normal peristalsis, but the whole esophagus builds pressure at once during a swallow. This is the most common type, at 50 to 70 percent of cases.
• Type III. The lower esophagus has spastic contractions. It is the least common type, about 5 percent of cases.

The type matters for planning. The ACG notes that people with type II achalasia tend to do very well whichever treatment is chosen, while type III achalasia responds better to a longer cut in the muscle than to treatments aimed only at the sphincter. The Chicago Classification also notes that opioid pain medicines are associated with a type III pattern, and recommends that people be tested off opioids when possible. Bring a full medication list to any motility test.

Treatment options for achalasia

The ACG is direct about what treatment can and cannot do. Achalasia is a chronic condition, and no current treatment restores the lost nerve function or brings back normal peristalsis. Every treatment works the same way: by weakening or opening the lower sphincter so food can pass by gravity and the remaining pressure in the esophagus. The goals the guideline sets are relief of symptoms, better emptying of the esophagus, and preventing the esophagus from widening further.

The ACG considers three procedures to be definitive treatments, and recommends that all of them be performed at centers with adequate volume and experience.

Pneumatic dilation

Pneumatic dilation is done during an endoscopy with sedation. A balloon is positioned across the lower sphincter and inflated with air to stretch and partly tear the muscle fibers. The ACG notes that the standard dilators used for an ordinary stricture are not effective for achalasia. The ACG describes a graded approach: starting with a 3.0 centimeter balloon, reassessing after 4 to 6 weeks, and moving to a larger size if symptoms persist. The guideline calls serial pneumatic dilation the most effective non-surgical treatment for achalasia.

The main risk is a tear through the wall of the esophagus. The ACG reports a median perforation rate of 1.9 percent in experienced hands and states that anyone having the procedure should understand that surgery may be needed if a tear occurs. Reflux develops afterward in 15 to 35 percent of patients.

Laparoscopic Heller myotomy

A Heller myotomy is a surgical procedure, usually done through small abdominal incisions, in which the surgeon cuts the muscle fibers of the lower sphincter. Because that removes the barrier to reflux, the ACG recommends pairing it with a partial wrap of the stomach around the esophagus, called a fundoplication. In a European trial of 201 patients cited by the guideline, pneumatic dilation and Heller myotomy had similar success at two years (86 percent and 90 percent) and at five years (84 percent and 82 percent). The ACG considers the two equivalent for patients who are candidates for either.

Peroral endoscopic myotomy (POEM)

POEM accomplishes a myotomy with an endoscope passed through the mouth, with no external incisions. The physician tunnels beneath the lining of the esophagus and cuts the muscle from the inside. In a randomized trial cited by the ACG, clinical success two years after treatment was 83 percent with POEM and 82 percent with Heller myotomy. Because the length of the cut can be tailored, the ACG recommends POEM or a tailored Heller myotomy over dilation for type III achalasia.

The trade-off is reflux. POEM does not include a fundoplication, and in a pooled analysis cited by the guideline, abnormal acid exposure was found in 39 percent of patients after POEM compared with about 17 percent after surgical myotomy with fundoplication. The ACG advises that people considering POEM be told that long-term acid-suppressing medication may be needed, and that it may be prudent to check afterward for reflux damage, including Barrett's esophagus.

Botulinum toxin and medications

Botulinum toxin (Botox) can be injected into the lower sphincter during an endoscopy to relax it. It is low risk, but the benefit fades. In a review cited by the ACG, about 79 percent of patients had relief within the first month and about 41 percent still had relief at one year. The guideline recommends it as the first choice for people whose other health conditions make dilation, surgery, and POEM unsuitable, and not as a first treatment for people who are fit for those procedures.

Oral medicines that relax smooth muscle, such as calcium channel blockers and nitrates, are described by the ACG as the least effective option, with a short duration of action and side effects that include headache and low blood pressure. The guideline reserves them for people who cannot have a definitive procedure and have not been helped by botulinum toxin. It recommends against using stents for long-term management.

How the choice is made

For type I and type II achalasia, the ACG considers dilation, Heller myotomy, and POEM comparably effective, and states that the choice depends on the achalasia type, the patient's preference, and the expertise available. Age, sex, other health conditions, how you weigh a repeat procedure against a single longer one, and how you feel about long-term reflux medication all belong in that conversation with your physicians.

Life after achalasia treatment

Because treatment relieves the blockage without restoring normal muscle function, follow-up continues over the long term. The ACG notes that symptoms can return over time: failure rates after myotomy range from 5 to 30 percent within one to three years, and the guideline describes repeat treatment as safe and appropriate. Dilation can be used after a myotomy, and POEM can be used after dilation or surgery.

When symptoms return, the guideline recommends a timed barium swallow as the first test, since symptoms alone do not always show whether the esophagus is emptying. Endoscopy helps tell recurrent achalasia apart from reflux-related inflammation or narrowing, which is treated differently.

The ACG also addresses cancer risk plainly. The risk of esophageal cancer is higher in people with achalasia than in the general population, but the overall number of cases is low, and the guideline recommends against routine endoscopic screening for cancer on that basis alone. It notes that many experts still favor periodic endoscopy or imaging once the condition has been present for more than 10 to 15 years, partly to watch for widening of the esophagus. Your physician can explain what follow-up schedule fits your situation.

Esophageal motility disorders other than achalasia

Achalasia is the most clearly defined esophageal motility disorder, but manometry can show other patterns. The Chicago Classification version 4.0 groups them into problems with the opening into the stomach and problems with peristalsis:

• Esophagogastric junction outflow obstruction. The lower sphincter relaxes poorly, but peristalsis is still present. Some cases are an early form or variant of achalasia. The classification notes that more than one-third may have no clinical importance and can reflect a mechanical cause, opioid use, or a measurement artifact.
• Distal esophageal spasm. Contractions in the lower esophagus arrive too early and out of sequence.
• Hypercontractile esophagus. Peristalsis is present but far stronger than normal.
• Absent contractility. The esophagus does not contract, but the lower sphincter relaxes normally.
• Ineffective esophageal motility. Contractions are weak or frequently fail.

An important point in version 4.0 is that several of these are patterns on a test, not automatically diseases. The classification states that outflow obstruction, spasm, and hypercontractile esophagus matter clinically only when a person also has relevant symptoms, mainly difficulty swallowing or chest pain that is not from the heart, and in some cases supporting evidence from a barium swallow or FLIP. An unexpected manometry finding is a reason for a careful conversation, not necessarily for a procedure.

When to see a gastroenterologist about possible achalasia

MedlinePlus advises contacting a health care provider if you have trouble swallowing or painful swallowing. Achalasia is one of the less common explanations, but the symptoms that suggest it deserve evaluation whatever the cause:

• Difficulty swallowing liquids as well as solids, or swallowing that is steadily getting worse.
• Food or saliva coming back up, especially at night.
• Reflux symptoms that have not improved despite taking a PPI as directed.
• Coughing or choking at night, or repeated chest infections.
• Unintended weight loss along with any of the above.

If food becomes completely stuck and you cannot swallow your own saliva, seek emergency care. Our guide to when to see a gastroenterologist covers how to tell a scheduled concern from an urgent one. For more detail on achalasia itself, the ACG clinical guideline on achalasia and the MedlinePlus achalasia overview are both freely available.

Evaluation for achalasia in Northern Virginia with GC of NoVA

Gastroenterology Center of Northern Virginia sees adults across Arlington, Annandale, and the surrounding communities for swallowing problems, regurgitation, and reflux that has not responded to treatment. Evaluation begins with a detailed history and, in most cases, an upper endoscopy to look for narrowing, inflammation, or anything blocking the esophagus. When the findings point toward achalasia or another motility disorder, our physicians arrange the additional testing needed to confirm it, explain what the results mean, and coordinate referral to a center experienced in achalasia procedures when treatment is needed. We see patients at our Arlington office, next to VHC Health, and our Annandale office, in the Woodburn Medical complex.

If this is your first visit, our guide to your first gastroenterologist visit explains what to bring. For swallowing problems, it helps to note whether solids, liquids, or both are affected, how long symptoms have been present, any weight change, and every medication you take, including pain medicines. To schedule, contact our Arlington office at (703) 522-7476 or our Annandale office at (703) 560-6106, or learn more at gcofnova.com.

This article is for general education and is not medical advice. Talk with a qualified gastroenterologist about your symptoms, diagnosis, and treatment options.