Achalasia Cardia: Symptoms, Causes, Treatment & Outlook - Healthline

Achalasia cardia is a swallowing disorder that falls into an unusual category. It's both very rare as well as one of the most common causes of motor-related swallowing difficulty.

The condition only affects about 1.6 people per 100,000 worldwide, although these numbers seem to be increasing.

This article will explore what happens in people with achalasia cardia, what causes this condition, and what can be done to treat it.

Achalasia cardia is a rare condition that affects the esophagus. This is the tube that moves food and liquids from your mouth to your stomach.

At the base of this tube, leading into the stomach, there is a ring-shaped muscle called the lower esophageal sphincter. This muscle is drawn tightly closed when you aren't eating to prevent food and gastric juices from bubbling up into the esophagus and causing tissue damage. When you are eating, this sphincter relaxes, allowing food to push past it and into the stomach for digestion.

In people with achalasia cardia, there is a problem with the way this muscle opens and closes. This causes swallowing problems, which can lead to a host of other symptoms and complications.

The main symptom of achalasia cardia is difficulty swallowing. The muscular opening that separates your throat from your stomach doesn't open and close when it's supposed to. This causes food to become trapped in the esophagus, where it can eventually pass back up into the mouth.

Not only can this swallowing difficulty lead to nutritional deficiencies, but it can also cause a number of other symptoms like:

  • mild chest pain that comes and goes
  • regurgitation of food from the esophagus back into the mouth
  • the movement of food from the esophagus into the lungs (aspiration)
  • coughing at night
  • dry mouth and eyes

Malnutrition and weight loss can result from these symptoms, but people with achalasia cardia may also develop problems like pneumonia from aspirating (inhaling) food into their lungs. The risk of developing esophageal cancer also seems to be higher in people with this condition.

No one's really sure what causes achalasia cardia, but it could be damage to the nerves that help the throat, stomach, and other parts of the digestive system function. What damages these nerves and causes them to lose function exactly is a mystery, but there are theories that genetics, autoimmune disease, or even infections might play a role.

In terms of who develops this disease, there are really no patterns. Achalasia cardia appears to mainly affect genders and races equally and is mostly a condition that occurs in adults. It's most common for this condition to develop between your 20s and 50s, with less than 2 to 5 percent of cases appearing in children under age 16.

When genetics are suspected as a cause in very rare forms of this condition, people assigned male at birth are twice as likely to be affected than people assigned female. People with the following characteristics are also more likely to be affected:

Other possible links to the development of this condition include things like:

Diagnosis of achalasia cardia may take some time, simply because of how rare the condition is. If you are having trouble swallowing or regurgitating undigested food — the two most common early symptoms of the condition —your doctor will start by asking you questions about your personal and family health history, as well as any new symptoms you're having.

A physical examination might be performed. With this condition, raising your arms up and other movements can sometimes help move food to the stomach.

A physical exam alone isn't usually enough to diagnose achalasia cardia, though. Typically, the following tests will be performed to get a better understanding of what's happening when you swallow:

  • X-ray imaging to examine the structure of your digestive system.
  • Imaging after drinking a barium solution (barium swallow study) to follow the path of swallowed foods and liquids.
  • Esophageal manometry to measure the force and efficiency of muscle contractions in the throat.
  • Upper gastrointestinal (GI) endoscopy for a direct view of your esophagus.

The main goal of treating achalasia cardia is to relieve swallowing problems and prevent complications. Since the root cause of this condition isn't completely understood, there really aren't any treatments aimed at truly preventing or correcting achalasia cardia.

Surgery and medications can be used depending on the severity of the condition and how well you might tolerate surgery.

Surgical treatments

For people who are at low risk of surgical complications, treatments can include the following procedures:

  • Pneumatic dilation. A balloon is inserted into the esophagus and expanded to widen the opening of the lower esophageal sphincter. People who undergo this procedure often need to have it repeated in time.
  • Laparoscopic Heller myotomy (LHM). A surgery called fundoplication that brings stomach tissue over the base of the esophagus, bypassing the lower sphincter. This is considered a first-line treatment for achalasia cardia, as up to 97 percent of patients respond to this procedure.
  • Peroral endoscopic myotomy (POEM). A tube is passed down through your mouth, and a small incision is made to open and loosen the lower esophageal sphincter. This procedure is effective but often reserved for specific types of achalasia cardia, including when other treatments have failed.

Each of these procedures can carry a risk of surgical complications, the need for repeated treatment, and the development of gastroesophageal reflux disease (GERD) from the loosening of the lower sphincter.

Medications

In some cases, medications like muscle relaxers and isosorbide or nifedipine are used to relieve achalasia cardia symptoms. Diet changes to the thickness and textures of foods and liquids may also help.

Botulinum toxin applications through an endoscopy tube are an option, too. This treatment is usually reserved for people who can't tolerate surgery or have a less favorable health outlook.

Achalasia cardia is considered an incurable, chronic neurologic disorder. Even with treatment, this condition will require lifelong monitoring and treatment.

Therapies like pneumatic dilation can help for a time, but 10 to 15 percent of people encounter overdilation. This can lead to the removal of all or part of the esophagus in 5 percent of people who receive this therapy.

Other surgeries don't have these exact results, but even LHM and POEM tend to fail or require repeated treatment after about 5 years.

Even after surgery, some lifestyle and diet changes may be needed, like:

  • trying to eat small meals
  • eating in an upright position
  • avoiding lying flat
  • keeping your head at a 30- to 45-degree angle to prevent aspiration

Although there's no cure for achalasia cardia, the overall prognosis is good for most people with the condition. A typical life expectancy is possible with lifelong monitoring and occasional treatments.

Achalasia cardia is a chronic condition in which the muscle at the base of the throat where food enters the stomach is too tight. This can cause food and liquids to back up in the throat.

Surgeries to loosen this muscle are an option for treatment but usually need to be repeated over time.

There's no cure for achalasia cardia, but people with this disorder can usually have a normal life span with regular monitoring and treatment.

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