Achalasia

Achalasia is a problem at the bottom of the esophagus. Where the esophagus meets the stomach there is a second ring of muscle, the lower esophageal sphincter, which is supposed to relax every time you swallow. In achalasia, the nerves that tell it to relax are lost. The ring stays shut, and the waves that normally push food down the esophagus stop as well¹. Food and drink pile up above the closed ring and drain slowly, if at all.

Nobody knows what causes it^1,2. It is rare. A 2024 review put the number of new cases at a little under 1 per 100,000 people a year, and rising, probably because doctors are recognising it more often³. It affects men and women equally and most often starts between the ages of 30 and 60¹.

It has also been studied for a long time. The search used on this site finds 2,891 achalasia papers in PubMed from before 1990 alone⁴.

Food stops on the way down, somewhere behind the breastbone. It is usually solid food first, and then drinks too. It usually gets slowly worse over months or years^1,5.

Food comes back up, and it tastes like food rather than sour acid. There can be a lot of saliva with it⁵. Lying down makes this worse, so it can happen at night, and food that comes up while you sleep can be breathed in⁵. People often lose weight, usually 5 to 10 kg⁵.

Many people find their own tricks for getting food down: eating slowly, raising their arms, or arching their back⁵. Chest pain and heartburn are common as well¹.

From reflux. This is the big one. Between 27 and 42 percent of people with achalasia have heartburn, so many are first told they have reflux and given acid medicine, which cannot open a valve that will not relax. The wrong diagnosis often delays the right one¹. In a study of 300 patients, 42% had been given at least one wrong diagnosis first (26% one, 16% more than one), most often reflux⁶. The American College of Gastroenterology recommends testing for achalasia when suspected reflux does not get better with acid medicine¹.

From spasm. On manometry, achalasia is defined by a lower sphincter that fails to relax. In distal esophageal spasm the sphincter relaxes normally^7,8. One type of achalasia, type III, also has spasm-like squeezing, and some experts see the two as neighbours on the same spectrum⁹.

From the upper sphincter problems (A-CPD and Zenker's). Trouble with drinks as well as food points lower down. Where you feel it is only a rough guide, though, because achalasia can be felt as high as the neck¹⁰.

From something more serious. A cancer near the top of the stomach can imitate achalasia. It is more likely in people over 55 who have lost a lot of weight quickly over a short time. That is why everyone with suspected achalasia should have an endoscopy^1,2.

• High-resolution manometry. A thin tube goes through the nose and down into the stomach and measures pressure along the esophagus while you take small sips of water. It is the gold standard, and it also tells which of the three types of achalasia you have, which affects treatment^1,7.
• Barium swallow (esophagram). You drink barium and X-rays are taken, ideally at 1, 2 and 5 minutes, which is called a timed barium esophagram. The classic picture is a widened esophagus narrowing to a point at the bottom, often described as a bird's beak¹.
• Upper endoscopy. A camera on a flexible tube. It is mainly there to rule out cancer, and it often looks normal early in the disease².
• FLIP (functional lumen imaging probe). A newer test done during endoscopy that measures how well the bottom of the esophagus stretches open. It can help when manometry cannot be done¹.

The person to ask for is a gastroenterologist, and the test to ask about by name is high-resolution manometry.

None of the treatments cures achalasia. They all work by opening the bottom of the esophagus so food can drain¹.

• Pneumatic dilation. Under sedation, a balloon is placed across the sphincter and inflated to stretch and tear the muscle. It may need repeating. The risk of a tear through the wall is about 2%¹.
• Heller myotomy. Keyhole surgery through the abdomen that cuts the muscle of the sphincter, usually together with a partial wrap of the stomach (called a Dor or Toupet) to limit reflux afterwards¹.
• POEM (peroral endoscopic myotomy). The same cut made from the inside, through the mouth, with no cuts in the skin. Reflux afterwards is more common than with the other two, and some people need acid medicine long term¹.
• Botox. 100 units injected into the sphincter during an endoscopy. It wears off, so it is mainly for people who cannot have the other treatments¹.
• Pills. Calcium channel blockers or nitrates. These are the least effective option and often cause side effects such as headaches¹.

• Dilation and Heller myotomy work about equally well. In a randomized trial of 201 people, 86% after dilation and 90% after surgery were doing well at two years, which was not a real difference¹¹. At five years it was 82% and 84%¹².
• POEM matches Heller myotomy, with more reflux. In a randomized trial of 221 people, 83% after POEM and 82% after surgery were doing well at two years (difference 1.4 points, 95% CI -8.7 to 11.4)¹³, and 75% and 71% at five years¹⁴. At two years, 44% of the POEM group had reflux damage on endoscopy, against 29% after surgery¹³.
• POEM beat dilation in one trial. In 133 people, 92% after POEM and 54% after dilation were doing well at two years (difference 38 points, 95% CI 22 to 52)¹⁵, and 81% against 40% at five years¹⁶. The trial allowed only one round of dilation, while many centres dilate again with a larger balloon. Counting one extra dilation, success with dilation rose to 76%¹.
• Botox fades. About 79% have relief in the first month and about 41% still do at a year¹.
• It takes a long time to diagnose. In 278 patients in Italy the median wait from first symptom to diagnosis was 24 months¹⁷. In 300 patients in Germany the mean was 4.7 years⁶.
• There is a cancer risk, but it is small. Achalasia raises the risk of one kind of esophageal cancer many times over. The absolute risk is still low, and the ACG guideline does not recommend routine screening¹.

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