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Achalasia

What is achalasia?

Achalasia, also known as cardiospasm, oesophageal aperistalsis or mega-oesophagus, is a disorder of the oesophagus (the tube that carries food from your mouth to your stomach).

Under normal circumstances, when you swallow, food is passed down the oesophagus by waves of muscle contractions and through a special valve into the stomach.

stomach and oesophagus

Lower oesophageal sphincter

A ‘valve’, which doctors call a sphincter, controls the entry of food from the lower end of the oesophagus into the stomach. This particular sphincter is known as the lower oesophageal sphincter. It is a band of muscle that opens to allow the food to pass from the oesophagus down into your stomach and then closes again to prevent the acidic stomach contents from coming back up.

In achalasia the lower oesophageal sphincter doesn’t relax properly with swallowing, which means that food is not pushed down into the stomach. Instead, it becomes lodged in the oesophagus. This happens because achalasia affects the nerves that control the sphincter muscles.

Another feature of achalasia is that the normal rhythmic contractions of the oesophagus, which propel food down it towards the stomach (doctors call this peristalsis), are lacking. Doctors think that this may be due to a malfunction of the nerves that encase the oesophagus.

Achalasia can happen at any age, even as young as 25, but the risk increases with age. It starts gradually, beginning with difficulty swallowing solid food and progressing to difficulty swallowing liquids.

What are the symptoms of achalasia?

  • Difficulty swallowing food or liquids.
  • Chest pain.
  • Regurgitation of food or liquids that have become caught in the oesophagus.
  • Regurgitation of undigested food while asleep.
  • Coughing at night due to regurgitation of food.
  • Weight loss.

How is it diagnosed?

Barium meal or swallow

Achalasia can be diagnosed in a number of ways. One is a barium meal or swallow examination, where you drink a thick liquid containing barium, which coats the inside of the oesophagus and stomach, making them show up on an X-ray. This test is particularly useful for seeing if the oesophagus has become stretched and enlarged due to food backing up in it. The specialist will also be able to see whether the muscle contractions of peristalsis are working.

Oesophageal manometry

Another investigation that is useful in the diagnosis of achalasia is oesophageal manometry. In this test a thin tube with pressure gauges along its surface is inserted into the oesophagus to measure pressures along the oesophagus and at the lower oesophageal sphincter as you swallow small sips of water. In a person with achalasia the muscular contractions that normally pass food along the oesophagus will be missing and the closing pressure of the lower oesophageal sphincter will be either normal or higher than normal. Also, the relaxation of the lower oesophageal sphincter during swallowing to allow food into the stomach will not happen or will not be normal.

Oesophagoscopic examination

A flexible viewing tube (an oesophagoscope) can be put down the throat to examine the oesophagus. This may show widening of the oesophagus in people with achalasia. It would also show if there were any obstructions in the oesophagus. The doctor can take a sample of tissue for examination (a biopsy) if necessary.

What are the complications or risks with achalasia?

Most people with achalasia may have no discernible symptoms for months or years before a diagnosis is actually made. This is because they may not experience any symptoms that cause any major discomfort and do not seek medical attention.

It’s estimated that about one-third of people with achalasia regurgitate food that is undigested while they are asleep. This can cause problems if these substances are accidentally inhaled into the lungs, as this can lead to pneumonia, an abscess of the lung or damage to the bronchial tubes.

If you have achalasia it’s important that it’s identified as soon as possible, as doctors believe the condition may slightly increase the risk of cancer of the oesophagus (although this association remains unproven).

How is it treated?

Mechanical widening of the lower oesophageal sphincter

Often one of the first approaches used to treat achalasia is the dilation or stretching of the oesophagus. This is done by inflating a small balloon in the lower oesophageal sphincter to widen it and reduce the pressure.

Unfortunately, there is a risk when dilating or stretching the oesophagus, as this technique has been known to cause the oesophagus to rupture. The dilation process may also need to be repeated as it may not work sufficiently the first time and this also increases the risk of rupture.

Medications

Medications such as nitrates (put under the tongue before meals) or calcium channel blockers (e.g. nifedipine) reduce the lower oesophageal sphincter pressure and can postpone future dilations. Except in mild forms of the disease, medications are rarely adequate treatment on their own.

Botulinum toxin

Botulinum toxin (Botox) is a nerve toxin that has become well-known recently for its use in relaxing facial frown lines and wrinkles. Botulinum toxin has now been gaining acceptance in the treatment of achalasia. The toxin is injected directly into the lower oesophageal sphincter via an endoscope. The toxin acts on nerves to block nerve impulses to the muscles which has the end result of relaxing the muscles of the lower oesophageal sphincter. The procedure is gaining popularity because it does not carry the risk of perforation that can happen with balloon stretching of the oesophagus. However, botulinum toxin only gives temporary relief (usually only 3 to 12 months) and, as yet, the effects of long-term use are not yet known.

Surgery

In serious cases, or in cases where the achalasia does not respond to dilation with the balloon, surgery may be necessary. This takes the form of cutting the lower oesophageal sphincter muscle to release the tension. This operation is successful in about 85 per cent of cases, but up to 20 per cent of people develop gastro-oesophageal reflux after surgery.


 

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