Achalasia – diagnosis and treatment

You may have been diagnosed with a condition called achalasia. This is a problem which affects the nerves and muscles of the oesophagus.

In achalasia, the bottom of the oesophagus (the lower oesophageal sphincter) loses its ability to open. Also, the oesophagus itself does not function properly depending on which type of achalasia you have. It might not move at all or be associated with spasm. As a result, the oesophagus loses its ability to push food and drink into the stomach properly. Achalasia does not affect other parts of the body.

Achalasia is a rare condition that affects around two in every 100,000 people.  It can be diagnosed in individuals of all ages. There is no particular race or sex that is affected more. No one knows why it happens and there is nothing a person could have done to stop it from happening.

People with achalasia have difficulty passing food and drink into the stomach. At first large pieces of food, such as meat and bread, may be difficult to pass, but even drinking water may become difficult as the condition progresses. You may develop chest pain, and even regurgitation of your food and drink. You might also lose weight as a consequence.

Timed barium swallow

This is a test where you drink a liquid (barium or gastrograffin) which shows up on an X-ray. You will then have a series of X-rays which show the outline of your oesophagus and valve, how it works and how much barium is held up. This is different to a standard barium swallow test which you might have had before and is used as a baseline test which will need to be repeated after treatment. Please therefore do not cancel this procedure because you have had a similar test in the past. This is an important test so please attend if it is arranged. 

Endoscopy

The procedure starts with a camera to examine the oesophagus and stomach. If the person doing the procedure sees something that needs to be sampled, a small biopsy can be taken. If there is too much food or fluid in the oesophagus that cannot be removed, the procedure might be abandoned, and you might be asked to fast for longer. The procedure might be undertaken just to have a look; to do some tests or it might be done for treatment (see below). Usually this is performed with medication to help you relax so that you are comfortable having it done.

All endoscopy procedures that require sedation will require that you bring someone with you to take you home. You will not be able to receive medication that relaxes you for the endoscopy if you have no one to take you home. If you have no one available to take you home, we will need to know in advance as we will need to arrange that you stay in hospital overnight.

Endoflip

During the endoscopy, you might also have another test called Endoflip. This is a device that measures how easy it is to open the valve by inflating a balloon This balloon is passed from the mouth to the valve and is slowly filled with water. This test might be done at the same time as the endoscopy, or you might be brought back for another endoscopy for this procedure to be undertaken. The person doing the endoscopy will decide and explain this to you. Doing Endoflip adds approximately 5 minutes to a standard endoscopy. This procedure is normally performed with medication that helps you relax, so you will need to bring someone with you to take you home afterwards.  

Oesophageal manometry

Oesophageal manometry measures pressures inside your oesophagus whilst you swallow. It is also known as High Resolution Manometry (HRM). It is a very important test to measure how your oesophagus works. A thin tube is passed into your nose and down your oesophagus in order to investigate how the oesophagus moves (oesophageal motility). This test is done with you awake so that you can swallow whilst the tube is in place. You do not need to bring anyone to take you home for this procedure. This test can confirm if you have Achalasia and what type of Achalasia you have. It can also identify other oesophageal motility disorders. 

Once the tube is passed you will be asked to swallow water and rice. The test takes approximately 10 minutes. When done the tube will come out and you will be able to go home. The test is analysed fully later, after you leave. 

Even if you have had this test done before, if you are invited to do this test please attend and don’t cancel. The test may be done differently to show more information and/or may show if the type of achalasia you have has changed, which will influence how you are treated.

There is a separate page called ‘Oesophageal manometry’ which gives more information. If you have been invited to have this test and have not received the ‘Oesophageal Manometry’ information, please request one for more information.

Achalasia can be treated in a variety of ways. All treatments aim to open up the passage between the oesophagus and stomach so that food and drink can pass through more easily. It is important to note that achalasia will never get better on its own. It will only stay the same or become worse. It is for this reason that you are being investigated and offered treatment. 

Botulinum toxin (Botox®)

This is a treatment in which Botox® is injected into the lower oesophageal sphincter (the valve) and, sometimes also into the oesophagus itself during endoscopy. Botox® paralyses the muscles in the oesophagus in an attempt to allow food to pass into the stomach more easily. However, treatment may need to be repeated in the future as it wears off. 

Pneumatic dilatation

In this treatment a balloon is used to stretch the valve. You will need to receive medication that relaxes you to have this procedure done. There is a possibility that you will need this procedure performed once or twice a few weeks apart. The doctor will be able to tell you after the procedure is done. There is a 2% risk of a bleed or tear through the oesophagus every time the stretch is performed. Normally if this happens, we try to close the tear or stop the bleeding on the spot. Very rarely you will need to be admitted to hospital, have antibiotics, more scans (such as CT scan) and have more endoscopy procedures to try to close the tear.  

The doctor referring you or the person doing the endoscopy will explain which type of balloon stretch you are having. Regardless, normally a barium test is done before and approximately one month after the final stretch:   

A. Pneumatic dilatation with X-ray

During endoscopy, a wire is passed through the top of the camera, out the other end and into the stomach. The camera is then taken out and the wire is left in place. Then a deflated balloon is passed over the wire and into the oesophagus such that the balloon sits across valve. The balloon is then inflated with fluid and air until it is full. This opens the valve and relaxes the muscle. Very low radiation X-ray is normally used to confirm this. The balloon is then deflated and everything is removed. The camera is then passed down once more to make sure that everything is ok before the procedure is finished. Most patients will go home on the same day of their treatment and be permitted to try to drink and eat. If there is a complication, you may need to stay in hospital overnight. 

B. Esoflip

After endoscopy is done, the camera is taken out and another type of balloon called Esoflip is passed. The Esoflip balloon is then inflated with water until the valve is seen to be sufficiently open. No X-ray is needed for this as the balloon provides measurements directly onto the computer screen. The balloon is then deflated and removed.  After the Esoflip balloon is removed, the camera is passed again to make sure that everything is okay.

Esoflip is normally preceded by an Endoflip test (see above) to acquire measurements across the valve and oesophagus first. After Esoflip, commonly Endoflip is put down again to acquire measurements in order to compare the result and determine if the stretch has been effective. Based on this, a decision will be made if you will need to come back on another day for another stretch with a bigger balloon or if one stretch was enough.

Surgery

For this you will need to be referred to the surgeons. The procedure is done under general anaesthetic and commonly needs an overnight stay in hospital. The muscles in the lower oesophageal sphincter are cut during an operation called a Heller myotomy. Normally this is performed using keyhole surgery (laparoscopy) but sometimes an open cut is necessary. To reduce the risk of severe acid reflux, an anti-reflux procedure, known as partial wrap or fundoplication is commonly performed at the same time. There is a 2% risk of a tear, infection or bleeding after. If you choose to have this procedure, the surgeons will need to see you in clinic first and discuss the details and risks associated with this procedure in more detail first.

Peroral endoscopic myotomy (POEM)

This is an endoscopy treatment that is similar to the surgery. The same muscles are cut but it is done during Endoscopy instead of in an operating theatre. 

Under general anaesthetic, after passing a camera down the oesophagus, a small hole is created in the side of the oesophagus. Then a tunnel is created such that the skin of the oesophagus (mucosa) is on one side and the muscle is on the other. This tunnel goes down to the valve and continues to the top of the stomach. The muscle that is not relaxing is then cut. In most cases, just the muscle of the valve is cut, but in patients with the spasm type of achalasia, muscle is also cut up the oesophagus depending on the extent of the spasm measured during the high resolution manometry (see above); the doctor will have explained beforehand which procedure you will be having. The hole is then closed with clips which fall off on their own over time and are passed into the toilet.  

Endoflip is commonly used during POEM both at the start and at the end of the procedure. This is done to make sure that the appropriate amount of muscle is cut. It only adds 5 – 10 minutes to the procedure but allows us to tailor the treatment more accurately. POEM will not leave any external scars on the abdomen or chest as everything is done from inside the oesophagus. Patients normally stay just one night in hospital. The following day, if all is well when you drink you will be sent home. Sometimes patients will be permitted to leave in the same day. 

There is a 2% risk of a tear or bleeding from POEM. Patients might experience pain for a few days after. There might be a risk of acid reflux symptoms. You will need acid reducing medications (such as omeprazole) after the procedure for at least one month, but some patients need to continue this medication longer. To avoid infection, antibiotics will be provided for 3 days. Please tell us if you have an allergy to penicillin beforehand.

You will be asked to be on a liquid diet for 1 week (yogurt, milk shake, juice, soup) then a soft diet for 1 week after that (pasta, mash). Then you will be permitted to eat and drink whatever you like as tolerated. This diet protocol will be primarily to prevent the clips being knocked off.  

Approximately one month later, you will likely need to have another barium swallow. Please make sure you attend.  

Diet days before your achalasia treatment or your first endoscopy: 

Before you come for your first endoscopy or first treatment for achalasia, you will need to be on a liquid diet for at least 3 days and water only in the last 24 hours. Liquid diet includes juices, nutritional supplements (like Ensure and Fortisip), milkshakes, soups (without bits). It is important to make sure that your oesophagus is not full of food because this will increase the risk of infection if food comes up in which case the procedure may have to be abandoned and rescheduled. There may be exceptions to this rule. If you are not sure or do not think this is possible, please speak with your doctor.

Achalasia is a well-defined medical condition of the oesophagus and you can find information about it is available online. 

• Patient.co.uk
• NHS website

UCLH cannot accept responsibility for information provided by other organisations. 

Pathway Co-ordinator (Monday to Friday 09:00-17:00)

Direct line: 020 3447 7488 

Switchboard: 0845 155 5000 / 020 3456 7890 ext. 77488

E-mail: uclh.gimedicineenquiries@nhs.net 

Address: GI Physiology Unit, Lower Ground Floor, EGA Wing, University College Hospital, 25 Grafton Way, London, WC1E 6DB