What is achalasia?
In people with achalasia, the muscular activity of the esophagus is impaired. The sphincter muscle to the stomach remains tense, does not relax and therefore does not open properly. This is where achalasia gets its name from: The “A” stands for “not”, and the Greek word “chalasie” means something like “slackening, slackening”. At the same time, the muscle pump of the esophagus, which is necessary for the transportation of food, no longer functions adequately. As a result, those affected find it increasingly difficult to swallow food. Even if most people don’t think about it, swallowing is a complex, sophisticated process in which the muscles and nerves in the esophagus have to work well together. If this cooperation does not function smoothly, the chyme comes to a standstill and is not transported any further towards the stomach.
Achalasia is characterized by difficulty swallowing and pain behind the breastbone. In addition, many of those affected regurgitate undigested food residues. Over time, the symptoms become more and more severe. Although achalasia cannot be cured, there are effective therapies, in particular endoscopic procedures such as dilation or surgery. All treatments aim to open the lower esophageal muscle and thus ensure better passage of food.
Causes of primary or secondary achalasia
The cause of primary achalasia is an abnormal reaction of the immune system. For example, viruses in respiratory tract infections, in which nerve cells are destroyed and thus lose their function. This is not reversible.
There is also secondary achalasia, in which other diseases are to blame for the malfunction of the esophagus. These include esophageal cancer and stomach cancer, when the tumor narrows the passage between the esophagus and stomach.
Achalasia – frequency and age
Achalasia is a very rare clinical picture. Experts estimate that only one in 100,000 people contract the disease. People between the ages of 25 and 60 are usually affected, but achalasia can occur at any age. Women fall ill about as often as men.
Great experience
Achalasia is a rare disorder of the motor function of the esophagus, which usually manifests itself in swallowing disorders. There are several recognized treatment methods that must be adapted to the individual situation. At the University Hospital Zurich, we have extensive experience with this clinical picture and can offer modern and high-quality diagnostics and treatment thanks to the close interdisciplinary cooperation between gastroenterology and surgery.
What happens with achalasia?
Two mechanisms are at work in achalasia:
- The nerves in the lower part of the esophagus lose their function. If the nerve function fails, the muscles also receive no impulse to move, to tense and relax. As a result, the esophagus no longer transports the food pulp quickly towards the stomach, as is the case in healthy people.
- In addition, the sphincter muscle at the end of the esophagus no longer relaxes sufficiently – it remains tense and can therefore no longer open properly. The food gets “stuck” in the esophagus.
Symptoms: Achalasia causes problems with swallowing
The symptoms of achalasia are usually only mild at the beginning and only occur occasionally. Over time, however, they become more and more pronounced and can severely impair everyday life and enjoyment of life (especially the pleasure of eating). The following symptoms are observed in achalasia:
- Swallowing disorders: Those affected have problems swallowing food. Many describe a feeling as if the food is stuck in their throat. They drink a lot so that the food slides down better. Initially, only swallowing solid food is difficult, but later liquid food also becomes a problem.
- Ejection of undigested food components (regurgitation): The food components do not reach the stomach but flow back towards the mouth. This can be particularly dangerous when lying down and at night. Sometimes those affected inhale the food residue and it ends up in the windpipe and airways, which can lead to pneumonia.
- Pain behind the breastbone, cramps in the chest – many people misinterpret this as heart disease.
- Weight loss – many sufferers can no longer eat and swallow sufficiently.
- Pain when swallowing if the food remains in the esophagus for longer and causes inflammation there.
Achalasia: Diagnosis with us
Always consult a doctor if you have problems swallowing – even if they are less pronounced at first. We will first ask you about your medical history in an anamnesis interview. The focus is on the symptoms, the intensity and duration, but also on underlying illnesses, the medication you are taking and your lifestyle.
Investigations
- Pressure measurement (manometry): High-resolution esophageal manometry in functional diagnostics in gastroenterology is the most important examination in the diagnosis of achalasia. We determine the pressure conditions in the esophagus. The values provide an indication of the muscular activity of the esophagus. Manometry also shows whether the lower esophageal muscle remains tense during swallowing and does not open completely.
- Endoscopy of the oesophagus and stomach (gastroscopy): This involves the use of an endoscope, a thin tube with a light source and camera chip. Inflammation and other changes to the mucous membrane of the esophagus can be detected in this way. Gastroscopy also provides indications of constrictions.
- EndoFLIP: This is a highly specialized examination performed by specialists in functional diagnostics during endoscopy of the esophagus and stomach. It provides additional information on the distensibility of the transition from the esophagus to the stomach and residual function of the esophagus.
- X-ray examination/fluoroscopy: Patients are given a contrast medium to drink while dynamic X-ray images are taken. The transportation of fluid via the esophagus is thus made visible. If the achalasia is already more advanced, the esophagus looks like a champagne glass on the X-ray – there is a typical narrowing at the transition to the stomach, while the area above it is widened.
Achalasia: prevention, early detection, prognosis
Unfortunately, there are no preventive measures against achalasia. However, you can reduce the risk of consequences and complications of achalasia, such as esophagitis or esophageal cancer, through your lifestyle. Avoid alcohol and smoking as much as possible. As the risk of oesophageal cancer is slightly increased with achalasia, you should also have your oesophagus examined regularly by your doctor – this allows changes to be detected at an early stage.
Course and prognosis of achalasia
Achalasia is a chronic disease that progresses if left untreated. The problems with swallowing decrease temporarily, which unfortunately means that the esophagus continues to expand, and then increase again as emptying becomes increasingly difficult. However, adequate treatment as early as possible can alleviate the symptoms and delay the progression of achalasia. Without treatment, the esophagus becomes increasingly dilated and, in the worst case, loses its function. Pneumonia is also a common consequence if you inhale regurgitated food residue and it gets into your lungs. In addition, the esophagus can become inflamed if the food remains there for a longer period of time, followed by bleeding or ulcers.
Achalasia that has existed for years means an increased risk of esophageal cancer. Regular checks are therefore important for us.
Achalasia: Treatments
Achalasia can be treated well. With the right therapies, we can alleviate the symptoms. However, there is no treatment that directly addresses the cause – namely the loss of nerve function. Achalasia is therefore not curable. The treatments aim to reduce the resistance in the esophageal sphincter. Which treatment is suitable depends on the extent of the symptoms, but also on your general state of health, age and individual wishes. In general, a definitive therapy should always be sought – medication and Botox do not help well or only in the short term and are only useful in older patients with many accompanying illnesses.