Achalasia

Achalasia is a disorder of the oesophagus in which the muscular ring at the junction with the stomach stops opening on cue, while the oesophagus itself loses the ability to push food downwards. Whatever is swallowed stalls above the narrowing and sooner or later comes back up. The condition is rare, roughly one new case per 100,000 people a year, and it advances slowly, so several years often pass between the first trouble with swallowing and the diagnosis.

What stops working in the oesophagus

The oesophagus is not a passive tube but a muscular pump. After each swallow a wave of contractions travels down it, and the muscular ring at the bottom, the lower oesophageal sphincter, relaxes at exactly the right moment to let food into the stomach.

In achalasia the nerve cells in the oesophageal wall that carry the instruction to relax die off. The ring stays shut and the wave never forms. Food descends only under its own weight and under the pressure of whatever has already piled up above it. Over time the oesophagus stretches, holds more and more content and empties less and less well.

How it feels

The main sign is that food struggles to go down. One detail matters a great deal: in achalasia solids and a glass of water are equally difficult. When a tumour or a scar is blocking the passage, solids fail first and liquids keep going through for a while.

Alongside this there may be:

• undigested food coming back up without a sour taste, sometimes an hour or two after a meal;
• a night-time cough, a wet pillow, the sense of choking while asleep;
• pressing or burning pain behind the breastbone, easily mistaken for heartburn or for the heart itself;
• a lump-in-the-throat feeling that eases on standing up, walking about, straightening the back or sipping warm water;
• slow weight loss with no dieting behind it.

People adapt for years without noticing: eating slowly, washing down every mouthful, giving up meat and bread, avoiding company at meals. That adaptation blurs the picture and delays the first appointment.

When waiting for a routine appointment is not an option

Seek help straight away if:

• nothing goes down at all, not food, not water, not even saliva;
• severe chest pain begins right after vomiting or after an endoscopic procedure, especially with breathlessness and a crackling feeling under the skin of the neck, which is how a tear in the oesophageal wall shows itself;
• a night-time episode of regurgitation is followed by fever, a productive cough and shortness of breath, because the contents may have entered the airways;
• there has been blood in the vomit or the stools have turned black;
• weight is dropping fast, several kilograms over a month or two.

A separate situation: swallowing became difficult after the age of 55, everything developed within a few months and weight fell noticeably. The doctor must then first rule out a tumour where the oesophagus meets the stomach, because it produces exactly the same picture; this is called pseudoachalasia. That is why endoscopy is done for everyone, even when the diagnosis looks obvious.

Why the nerves stop responding

No exact cause can be named yet. Most of the evidence points to an autoimmune process: the immune system mistakenly destroys the nerve networks of the oesophagus, and in some people the corresponding antibodies are found. A previous viral infection in someone already predisposed is considered a plausible trigger.

Inherited forms are rare. In children achalasia is occasionally part of a congenital syndrome that also involves adrenal insufficiency and an absence of tears.

One cause is worth remembering after travel or a move abroad: in Latin America the same picture is produced by Chagas disease, a parasitic infection that destroys those very nerve networks. If you have lived or spent long periods there, say so explicitly, because the work-up will be different.

What does not cause achalasia is stress, eating in a hurry or spicy food. Those explanations only postpone the investigation.

What the tests show

• High-resolution manometry measures pressure along the whole length of the oesophagus. It is the key test: it confirms the diagnosis and sorts the disease into types that directly determine which treatment is chosen.
• Upper endoscopy lets the doctor look at the lining from inside and rule out a tumour, a scarred narrowing and inflammation.
• A barium study shows the contrast standing as a column and trickling downwards in a thin stream, with the narrowing taking its characteristic bird-beak shape. A repeat image a few minutes later shows how far the oesophagus manages to empty.

Computed tomography is added if a tumour is suspected.

What restores the passage

Lost nerve cells cannot be brought back, so treatment sets a different goal: to weaken the closed ring so that food reaches the stomach. The method is chosen according to the type found on manometry, your age and any other conditions.

• Balloon dilation: under endoscopic control a balloon is placed in the sphincter and inflated. Several sessions are often needed and there is a small risk of tearing the wall.
• Peroral endoscopic myotomy: the muscle fibres are divided from the inside, with no cuts in the skin.
• Laparoscopic myotomy: the same fibres are divided from outside through small abdominal incisions, usually together with a wrap that limits reflux.
• Botulinum toxin injected into the sphincter: the effect lasts months, so it is reserved for people for whom a procedure is risky because of age or other illness.
• Medicines from the nitrate and calcium channel blocker groups relax the ring weakly and briefly; they serve as a stopgap while definitive treatment is arranged.

Every method that opens the sphincter has a flip side: stomach contents gain a route back up. Heartburn after treatment is common and is managed with acid-lowering medicines and follow-up.

Everyday habits that make eating easier

• Eat sitting upright, in small portions, chewing thoroughly.
• Sip warm water with meals: cold drinks often go down worse.
• Finish eating three hours before bed and raise the head of the bed. This lowers the risk of night-time regurgitation and of the contents reaching the airways.
• Weigh yourself regularly rather than now and then: weight is the most honest measure of whether you are getting enough food.
• Give up smoking and strong alcohol, which further irritate a lining already suffering from retained food.

No diet cures achalasia. Follow-up remains necessary even after a successful procedure, because symptoms sometimes return years later. An oesophagus altered over many years slightly raises the risk of oesophageal cancer; the probability is low, but it is one more reason to stay in touch with a doctor.

Online consultation

An Oladoctor doctor can assess whether your complaints fit achalasia or point to another cause of difficult swallowing, explain which tests are needed first, and help you read manometry or endoscopy reports you already have.

Prepare for the conversation: when swallowing first became hard, whether solids or liquids go down worse, whether food comes back up at night, how much weight you have lost and over what period, which medicines you take.

A remote appointment does not replace endoscopy or manometry, but it saves time otherwise lost on scattered tests and helps you reach the right specialist sooner. If any of the warning signs listed above appear, call your local emergency number, 112 across Europe, instead of booking a consultation.

This material is for information only and does not replace medical advice.