Achalasia

What is achalasia?

Achalasia is a rare disorder of the esophagus, the muscular tube that carries food and liquid from the mouth to the stomach. In people with achalasia, two things go wrong at the same time. First, the ring of muscle at the bottom of the esophagus, called the lower esophageal sphincter, fails to relax properly when food arrives. This sphincter normally acts like a one-way valve, opening to let food into the stomach and closing to keep stomach contents from flowing back up. Second, the coordinated wave-like muscle contractions of the esophagus, known as peristalsis, weaken or disappear. Together, these problems mean that food and liquid have difficulty passing into the stomach and may sit in the esophagus instead.

Many people first search for “what is achalasia” after being told they have difficulty swallowing that is not explained by a blockage or a tumor. In simple terms, achalasia is a swallowing disorder caused by faulty nerve signals to the muscles of the esophagus, not by a physical obstruction. Over time, if it is not treated, the esophagus can stretch and widen because food repeatedly collects inside it.

Achalasia can affect people of any age, but it is most often diagnosed in adults between roughly 25 and 60 years of age. It affects men and women at similar rates. It is considered uncommon, and because its early symptoms can resemble more familiar conditions such as acid reflux, diagnosis is sometimes delayed. The condition is coded as K22.0 in the ICD-10 classification system used by doctors and hospitals worldwide.

Symptoms of achalasia

Achalasia symptoms usually develop gradually, often over months or years, and tend to worsen slowly. Because the changes are gradual, many people adapt without realizing it, for example by eating more slowly, drinking large amounts of liquid with meals, or avoiding certain foods.

Common achalasia symptoms include:

• Difficulty swallowing (dysphagia): a sensation that food or liquid is sticking in the chest or throat. In achalasia, this typically affects both solids and liquids, which helps distinguish it from blockages that mainly affect solid food.
• Regurgitation: undigested food or saliva coming back up into the mouth, sometimes hours after eating, and often without the sour taste of stomach acid.
• Chest pain or discomfort: pressure or pain behind the breastbone, which may occur during or after meals and can sometimes be mistaken for heart-related pain.
• Heartburn-like symptoms: burning in the chest that may not improve with standard acid-reducing medicines, because it is caused by retained food fermenting in the esophagus rather than by acid reflux.
• Unintended weight loss: often gradual, as eating becomes slower and less comfortable.
• Coughing or choking, especially at night: retained food or liquid can flow back into the throat when lying down and may be inhaled into the airways, sometimes causing nighttime coughing or repeated chest infections.
• Belching difficulty and hiccups: some people notice they cannot burp easily or have frequent hiccups.

Symptoms can differ depending on how advanced the condition is and on its subtype. In the earlier stages, swallowing difficulty may be intermittent and mild, and some people notice it mainly with cold drinks or when eating quickly or under stress. As the disease progresses and the esophagus dilates, regurgitation, weight loss, and nighttime symptoms often become more prominent. Doctors also classify achalasia into three types based on pressure measurements of the esophagus. Type I tends to involve a weak, dilated esophagus with little muscle activity. Type II involves pressure building up across the whole esophagus and often responds well to treatment. Type III involves spastic, uncoordinated contractions and is more often associated with chest pain. Your care team may mention these types because they can influence which treatment is likely to work best.

Causes and risk factors

The exact achalasia causes are not fully understood. What is well established is that the condition results from damage to, or loss of, the nerve cells in the wall of the esophagus that coordinate swallowing. These nerve cells normally tell the esophageal muscles when to contract and tell the lower esophageal sphincter when to relax. When they are lost, the sphincter stays tight and the normal wave of contractions fails.

Why these nerve cells degenerate is still being studied. Leading explanations include:

• Autoimmune reaction: in many cases, the body’s immune system appears to mistakenly attack the nerve cells of the esophagus. Achalasia is seen somewhat more often in people who have other autoimmune conditions.
• Viral triggers: some researchers believe that a prior viral infection may set off the immune response in people who are genetically susceptible, although no single virus has been proven to cause achalasia.
• Genetic susceptibility: achalasia does not usually run in families, but rare inherited syndromes can include achalasia as one feature, and certain genetic factors may increase risk.
• Chagas disease: in parts of Central and South America, infection with the parasite Trypanosoma cruzi can damage the same nerve cells and produce a condition very similar to achalasia.

It is important to know that achalasia is not caused by anything you ate, by stress, or by lifestyle habits, and it is not something you could have prevented. There are few clearly proven risk factors beyond age (most diagnoses occur in mid-adulthood) and, in endemic regions, exposure to Chagas disease. Achalasia is not contagious.

Doctors also distinguish true achalasia from a look-alike condition called pseudoachalasia, in which a tumor or another disorder at the junction of the esophagus and stomach mimics the same symptoms. This is one reason thorough testing is important, particularly in older adults or when symptoms appear rapidly.

Diagnosis

Achalasia diagnosis usually begins with a careful conversation about your symptoms, followed by specific tests. Because swallowing difficulty can have many causes, doctors need to both confirm achalasia and rule out other explanations, especially narrowing from scarring or a tumor. Diagnosis and ongoing care are typically managed by a gastroenterology department, where specialists in digestive disorders perform and interpret these studies.

The main tests include:

• Esophageal manometry: this is the gold-standard test for confirming achalasia. A thin, flexible tube with pressure sensors is passed through the nose into the esophagus. It measures how the muscles contract when you swallow and whether the lower esophageal sphincter relaxes. Modern high-resolution manometry shows the characteristic pattern of absent peristalsis and a sphincter that fails to relax, and it identifies the achalasia subtype (type I, II, or III).
• Barium swallow (esophagram): you drink a chalky liquid containing barium, which shows up on X-rays. In achalasia, the esophagus often appears dilated with a smooth, tapered narrowing at the bottom, sometimes described as a “bird’s beak” appearance. A timed version of this test can also show how slowly the esophagus empties.
• Upper endoscopy: a thin, flexible camera is passed through the mouth to look directly at the lining of the esophagus and stomach. Endoscopy is essential to exclude other causes of the same symptoms, such as strictures (scar-related narrowing) or tumors, and it may show retained food or a dilated esophagus.

In many cases, doctors use all three tests together, because each answers a different question: endoscopy rules out structural problems, the barium study shows the shape and emptying of the esophagus, and manometry confirms the abnormal muscle function that defines achalasia. Additional imaging, such as a CT scan or endoscopic ultrasound, may be suggested if your doctor suspects pseudoachalasia.

Treatment options for achalasia

There is currently no treatment that restores the lost nerve cells, so achalasia treatment focuses on relieving the tight lower esophageal sphincter so that food can pass into the stomach more easily. With appropriate treatment, most people experience substantial and lasting improvement in swallowing, although follow-up over time is usually recommended because symptoms can return. An overview of how this condition is evaluated and managed is also available on the dedicated achalasia treatment page; at Acibadem, this condition is managed within the gastroenterology and gastrointestinal surgery services.

Watchful waiting and lifestyle measures. Achalasia is progressive in most people, so purely waiting is generally not recommended once the diagnosis is confirmed. However, while treatment is being planned, measures such as eating slowly, chewing thoroughly, drinking fluids with meals, choosing softer foods, and staying upright after eating can make symptoms more manageable. Sleeping with the head of the bed raised may reduce nighttime regurgitation.

Medications. Drugs that relax smooth muscle, such as nitrates or calcium channel blockers, can modestly lower the pressure of the lower esophageal sphincter. In practice, their effect is often limited and temporary, and side effects such as headache or low blood pressure are common. Medication is therefore usually reserved for people who cannot undergo, or are waiting for, more definitive treatment.

Botulinum toxin injection. During an endoscopy, botulinum toxin can be injected into the lower esophageal sphincter to relax it. This often relieves symptoms, but the effect typically wears off after some months and repeat injections tend to become less effective. It is most often used for people who are not good candidates for procedures or surgery because of frailty or other medical conditions.

Pneumatic (balloon) dilation. In this endoscopic procedure, a special balloon is positioned in the lower esophageal sphincter and inflated to stretch and partially tear the tight muscle fibers. It is an established and effective treatment, though some people need repeat dilations over time. The main serious risk is a tear (perforation) of the esophagus, which is uncommon but may require urgent treatment.

Peroral endoscopic myotomy (POEM). POEM is a newer procedure performed entirely through the mouth with an endoscope. The doctor creates a tunnel within the wall of the esophagus and cuts the tight muscle fibers of the sphincter, and often part of the esophageal muscle above it, from the inside. POEM leaves no external incisions and has become a widely used option, particularly for type III achalasia, where a longer muscle cut can be helpful. Acid reflux afterward is relatively common, because the procedure does not include an anti-reflux step, so some people need long-term acid-reducing medication or monitoring.

Surgical myotomy (Heller myotomy). In this operation, usually performed laparoscopically through small abdominal incisions, the surgeon cuts the muscle fibers of the lower esophageal sphincter from the outside. It is often combined with a partial fundoplication, in which the top of the stomach is loosely wrapped around the lower esophagus to reduce acid reflux afterward. Heller myotomy is a well-established, durable treatment for many people with achalasia.

Esophagectomy. In a small minority of people with very advanced, long-standing achalasia and a severely dilated, poorly functioning esophagus, removal of part or all of the esophagus may be discussed. This is major surgery and is considered only when other treatments cannot help.

The best choice depends on your achalasia type, age, overall health, the shape and dilation of your esophagus, prior treatments, and personal preferences. Your doctor may recommend one option first and keep others in reserve, since treatments can be combined or repeated over time.

Living with achalasia and outlook

Achalasia is a chronic condition, meaning it does not go away on its own and current treatments manage it rather than cure it. That said, the outlook for most people is reassuring: with effective treatment, many people eat comfortably, regain lost weight, and return to their usual activities. Some people remain symptom-free for many years after a single procedure, while others need repeat or additional treatments.

Long-term follow-up is generally advised for several reasons. Symptoms can gradually return as the sphincter tightens again. Acid reflux can develop after treatment, sometimes without obvious symptoms, and may need medication or monitoring. In addition, people with long-standing achalasia have a somewhat higher than average risk of developing esophageal cancer over decades, although the absolute risk remains low. Your doctor can advise whether periodic endoscopic checks are appropriate in your situation.

Day to day, many people find it helpful to eat smaller, more frequent meals, chew food well, drink liquids during meals, avoid eating close to bedtime, and identify foods that tend to stick, which vary from person to person. If weight loss or nutritional concerns arise, a dietitian can help design an eating plan that maintains adequate nutrition while symptoms are being brought under control.

Frequently asked questions

What is achalasia in simple terms?

Achalasia is a swallowing disorder in which the valve at the bottom of the esophagus does not relax and the normal muscle waves that push food downward are lost. As a result, food and liquid pass into the stomach slowly or incompletely and may come back up. It is caused by damage to the nerves that control the esophageal muscles, not by a physical blockage.

Can achalasia heal on its own?

Achalasia does not resolve on its own, and in most people symptoms slowly worsen without treatment. The nerve damage that causes it cannot currently be reversed. However, treatments that relax or cut the tight muscle at the bottom of the esophagus are effective for most people and can control symptoms for many years, sometimes with occasional repeat procedures.

How serious is achalasia?

Achalasia is not usually immediately life-threatening, but it should not be ignored. Untreated, it can lead to significant weight loss, malnutrition, aspiration of food into the lungs with repeated chest infections, and progressive widening of the esophagus that makes later treatment more difficult. There is also a modestly increased long-term risk of esophageal cancer, which is one reason ongoing medical follow-up is often recommended.

What are the first symptoms of achalasia?

The earliest and most common symptom is difficulty swallowing that affects both solids and liquids, often described as food sticking in the chest. Regurgitation of undigested food, chest discomfort, heartburn that does not respond well to acid-reducing medicines, and gradual weight loss are also frequent early features. Because symptoms develop slowly, many people adapt their eating habits before seeking care.

How is achalasia diagnosed?

Doctors confirm achalasia mainly with esophageal manometry, a pressure test that shows the sphincter failing to relax and the loss of normal contractions. A barium swallow X-ray often shows a dilated esophagus with a narrow “bird’s beak” at the bottom, and an upper endoscopy is performed to rule out other causes such as scarring or tumors. In many cases, all three tests are used together.

What is the best treatment for achalasia?

There is no single best treatment for everyone. Pneumatic balloon dilation, peroral endoscopic myotomy (POEM), and laparoscopic Heller myotomy are all established options with good results for many people, and the choice depends on the achalasia subtype, your overall health, and local expertise. Botulinum toxin injections and muscle-relaxing medicines are usually reserved for people who cannot undergo these procedures. Your doctor can explain which approach fits your situation.

How long is recovery after achalasia treatment?

Recovery is generally short for endoscopic treatments; many people go home the same day or after a brief hospital stay following balloon dilation or POEM and return to normal activities within days. Laparoscopic Heller myotomy usually involves a short hospital stay and a somewhat longer recovery, often a few weeks. After any procedure, a gradual return from liquids to soft foods and then a normal diet is commonly advised, following your care team’s instructions.

When to see a doctor

Make an appointment with a doctor if you have ongoing difficulty swallowing, food or liquid regularly coming back up, unexplained chest discomfort with meals, or unintended weight loss. These symptoms have many possible causes, and proper testing is the only way to find the reason.

Seek urgent medical care if you experience any of the following red-flag warning signs:

• Sudden inability to swallow anything, including your own saliva, or a sensation that food is completely stuck.
• Severe chest pain, especially if it is new, intense, or accompanied by sweating, shortness of breath, or pain spreading to the arm or jaw, since heart problems must be ruled out.
• Vomiting blood or passing black, tarry stools, which can indicate bleeding in the digestive tract.
• Choking episodes, coughing when lying down, fever with cough, or repeated chest infections, which may suggest food or liquid entering the lungs.
• Rapid, unintended weight loss or signs of dehydration such as dizziness, very dark urine, or inability to keep fluids down.
• Sudden severe pain after an endoscopic procedure or dilation, particularly with fever or difficulty breathing, which could signal a tear in the esophagus and needs emergency assessment.

Early evaluation matters. Achalasia is very treatable, and the sooner it is identified, the easier it usually is to relieve symptoms and protect the esophagus from long-term damage.