Achalasia: when the oesophagus won't open

Achalasia: When the oesophagus won't open

Achalasia is a rare condition of the oesophagus where the sphincter muscle, which allows food into the stomach, fails to relax. Food becomes trapped, and over months or years, the oesophagus can become so stretched that it resembles a pouch. Achalasia is rare and can begin at any age.

The condition mimics many other ailments, which is one reason why several years often pass between the onset of the first symptoms and diagnosis.

What are the symptoms?

The first sign is usually difficulty swallowing. This typically starts with solid foods and progresses slowly. Later, it also affects liquids, which is one of the signs that can differentiate achalasia from more common conditions where only solid food causes problems.

Other symptoms you might experience:

• Food and mucus regurgitating into the mouth, especially when lying down
• Chest pain behind the breastbone, often associated with meals
• Heartburn that does not improve with acid-reducing medication
• Unexplained weight loss
• Night-time coughing, due to contents from the oesophagus flowing up into the throat and down into the airways

Some patients develop pneumonia as a result. Repeatedly having contents in the airways is not without risk.

Why does it happen?

In a normal oesophagus, nerves send signals that cause muscles to contract in waves, pushing food down. The lower sphincter muscle should open to allow food into the stomach. In achalasia, the nerve cells in the lower part of the oesophagus gradually degenerate. As a result, the wave-like contractions disappear, and the sphincter muscle fails to relax.

In the vast majority of cases, the cause is unknown. In rare instances, it is seen as a consequence of parasitic infection (Chagas disease), which is almost exclusively found in South America.

How is it diagnosed?

Three investigations are used in combination:

1. Gastroscopy to rule out cancer or a stricture as another cause of oesophageal dysfunction. In achalasia, the mucous membrane often appears normal, and this is part of the point: to exclude dangerous conditions.
2. Barium swallow (contrast X-ray). The classic, though rarely perfectly typical, finding is a bird's beak-shaped area at the bottom of the oesophagus, where the contrast slowly trickles down.
3. Manometry. A thin probe measures pressure at different points in the oesophagus as you swallow. This is the investigation that establishes the diagnosis itself and classifies achalasia into three subtypes. This classification is important because it guides treatment choices.

Treatment

Achalasia cannot be cured. Treatment focuses on opening the lower sphincter muscle so that food can pass through. There are four options, and the choice depends on your age, general health, and the subtype of achalasia you have:

• Pneumatic dilation: A balloon is inserted into the sphincter muscle and inflated, causing a controlled tear in the muscle. The effect is rapid, but its efficacy diminishes in some patients, and the treatment can be repeated.
• Heller myotomy: A surgeon cuts through the sphincter muscle. The operation is performed using keyhole surgery. The choice between myotomy and the other options rests on an individual assessment, and results vary from person to person. It is often combined with a partial wrap of the stomach to prevent reflux afterwards.
• POEM (Peroral Endoscopic Myotomy): A relatively new method where the surgeon performs the myotomy from within via a gastroscope, without external incisions. The results are similar to Heller myotomy.
• Botulinum toxin injected into the sphincter muscle works for a while, but the effect is short-lived. It is typically used for elderly patients or those who cannot tolerate surgery.

Medication (calcium channel blockers, nitrates) rarely helps and is largely no longer used as a primary treatment.

When should you see a doctor?

Contact your GP if you have:

• Difficulty swallowing that lasts more than a couple of weeks
• Difficulty swallowing liquids, not just solid food
• Food or mucus regurgitating into your mouth at night
• Unexplained weight loss combined with swallowing problems
• Repeated pneumonia without another explanation

Achalasia is rare, and most people with swallowing difficulties have another cause. However, precisely because diagnosis is often delayed, it is worth getting it investigated.

Frequently asked questions

Can achalasia lead to cancer? The risk of oesophageal cancer is slightly elevated in patients with achalasia, especially if the condition has been untreated for many years. There is, however, no established programme of routine cancer surveillance by gastroscopy for everyone with achalasia. Whether and how often you need a check-up gastroscopy depends on disease duration, how dilated the oesophagus is and your symptoms, and is agreed with the treating department.

Does achalasia go away on its own? No. The nerve cells do not regenerate. Treatment can alleviate symptoms, but the sphincter muscle will never function normally again.

Do I need to change my diet? Most people find it helpful to eat slowly, chew thoroughly, drink water with meals, and avoid lying down immediately after eating. Many avoid dry and crumbly foods like rice and bread, which are difficult to swallow.

How long after surgery can I eat normally? How the diet is built up after surgery is agreed with the operating team and depends on the procedure and on how swallowing works afterwards.

References

1. Vaezi MF, Pandolfino JE, Yadlapati RH, Greer KB, Kavitt RT. ACG Clinical Guidelines: Diagnosis and Management of Achalasia. Am J Gastroenterol. 2020;115(9):1393-1411. PubMed
2. Boeckxstaens GE, Zaninotto G, Richter JE. Achalasia. Lancet. 2014;383(9911):83-93. PubMed
3. Sundhed.dk. Akalasi (lægehåndbogen).
4. Werner YB, Hakanson B, Martinek J, et al. Endoscopic or Surgical Myotomy in Patients with Idiopathic Achalasia. N Engl J Med. 2019;381(23):2219-2229. PubMed

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This article provides general information and does not replace a medical consultation. Contact your GP if you experience symptoms.