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Achalasia

WHAT IS ACHALASIA?

Achalasia is an uncommon condition that affects the esophagus, a muscular tube that joins the mouth to the stomach. The condition prevents the lower esophageal sphincter (LES) from relaxing, making it difficult for food and liquids to pass through into the stomach. Because the lower esophagus is unable to contract and push the food through the narrowed LES, swallowing gradually becomes a problem.  

At Loma Linda University Health, we treat a variety of digestive disease conditions. We have the ability to diagnose and treat achalasia in addition to other esophageal disorders.

WHAT ARE THE SYMPTOMS OF ACHALASIA?

Symptoms of achalasia may include:

  • Vomiting
  • Heartburn
  • Lump in the throat
  • Unintended weight loss

Sometimes, people compensate for achalasia in symptomatic ways. They may eat more slowly. They may use maneuvers such as lifting the neck or throwing the shoulders back to make swallowing easier. These may also be considered symptoms of achalasia.

WHAT CAUSES ACHALASIA?

Although the cause of achalasia is currently unknown, studies show it may be caused by degeneration of the nerve cells within the esophagus. Other researchers believe it may be related to a virus.

HOW IS ACHALASIA DIAGNOSED?

At Loma Linda University Health, diagnosing achalasia starts with a medical examination and discussing the patient’s health history. If a doctor suspects a patient may have achalasia, the following tests may be ordered:

Chest x-rays - A chest x-ray may reveal a dilated esophagus and absence of air in the stomach.

Barium swallow - This test involves swallowing a mixture of barium while x-rays are taken.

Esophageal manometry (also called esophageal motility study) - This test involves passing a thin tube through the mouth or nose into the esophagus.

Endoscopy - Endoscopy uses a thin, lighted, flexible tube to detect achalasia or a condition that may be mimicking achalasia (such as cancer of the upper portion of the stomach). The patient is normally sedated during the procedure.

Biopsy - A small sample of tissue (biopsy) may be obtained from the lower part of the esophagus to look for cancer cells. Having a biopsy taken during endoscopy is not painful.

HOW IS ACHALASIA TREATED?

Common treatments for achalasia may include:

Medications

Medications may be helpful for a short period of time in early achalasia. These include:

  • Nifedipine
  • Diltiazem
  • Nitroglycerin
  • Sildenafil

The use of medications may be limited by side effects.

Endoscopic Balloon Dilation

Endoscopic balloon dilation has been used for many years to treat achalasia. While the patient is under light sedation, a specially designed balloon is inserted through the lower esophagus and forceful inflation disrupts the muscle and widens the opening for food to enter the stomach. This procedure sometimes needs to be repeated to produce the desired result.

Surgery

The standard surgical treatment for achalasia is the Heller myotomy. In this operation, the muscles of the valve between the esophagus and the stomach are cut to allow food to pass through.

Minimally Invasive Surgery

Today, a minimally invasive approach that allows faster recovery is used for most patients. Using five small abdominal incisions, a Heller myotomy can be accomplished through a thin, lighted instrument that holds a tiny camera, referred to as a laparoscope. Coupled with a surgical procedure referred to as partial fundoplication, this recreates a functioning lower esophageal sphincter. This procedure allows food to move into the stomach while minimizing reflux. It usually requires a one-day hospital stay and general anesthesia. The majority of patients are successfully treated with surgery, although in rare cases the procedures may need to be repeated.

Botulinum Toxin Injections

Patients who have disabling medical conditions and who are not suitable for the remedies listed above may benefit from injections of botulinum toxin (Botox®). When injected into the lower esophagus, Botox® can relax the muscles and allow swallowed food to pass through. The injections generally give temporary relief and may need to be repeated regularly.

At Loma Linda University Health, we are using a new state-of-the-art device called the EndoFLIP (endoscopic functional luminal impedance probe). This device allows for more controlled balloon dilatation. The patient is usually discharged home after the procedure.

WHAT ARE THE COMPLICATIONS OF ACHALASIA? (Optional)

If achalasia is left untreated, most patients continue experiencing the symptoms of achalasia listed above. If it is untreated for a prolonged period of time, the esophagus may become enlarged and eventually stop working.

Researchers have noted that patients with untreated achalasia have 16 times the chance of developing a form of cancer known as esophageal squamous cell carcinoma (ESCC). Therefore, achalasia should be considered and treated as a precancerous condition.

There may be other complications depending on the treatment methods chosen.

Complications of Balloon Dilation

Chest pain immediately after balloon dilation, along with a fever, are expected. In 2% to 5% of patients, a small hole (perforation) may occur in the wall of the esophagus. Persistent or worsening pain after the procedure may indicate a perforation. Perforations are usually small and can be treated in the hospital with intravenous feeding and antibiotics. Large perforations may require surgery for repair. If you experience increasing pain after balloon dilation and develop a fever or chills, call your physician immediately.

A small percentage of patients may experience bleeding after balloon dilation. Symptoms of bleeding may include dizziness or fainting, vomiting blood, vomiting what looks like coffee grounds, and black or bloody stools. Notify your doctor immediately if you experience these symptoms.

About 2% of patients may develop gastroesophageal reflux disease (GERD) after balloon dilation. GERD is usually easily controlled with acid-reducing medications.

Complications of Heller Myotomy Surgery

Pain is normal after any surgery, and this is typically treated with pain medications. Rarely, there may be an unintentional puncture (perforation) or tearing in the lining of your esophagus. Other possible complications may include developing:

  • Infection in your esophagus (esophagitis)
  • Gastroesophageal reflux disease (GERD)
  • Barrett's esophagus
  • Esophageal cancer

Complications of Botulinum Toxin Injections (Botox®)

Although about 25% of patients may experience chest pain for a few hours after Botox® injections, and about 5% of patients may develop heartburn, Botox® injections are considered safer than both balloon dilation and Heller myotomy surgery because damage to the esophageal wall and lining are rare.

This greater short-term safety may make Botox® injections a better choice for patients of advanced age, or for patients with serious medical conditions such as heart or lung problems.

WHO IS AT RISK FOR ACHALASIA? (Optional)

Achalasia is very rare and doesn’t seem to affect people of any particular age, race or gender.  However, some studies show it usually occurs in people between 20 and 60 years old. It is extremely rare in children under 16 years old.

Although statistics indicate that achalasia occurs more frequently in patients with spinal cord injuries, these cases are usually related to damage of the cervical and thoracic vertebrae.

Those with anorexia nervosa may also be an increased risk of developing achalasia.

NEXT STEPS

  • Stay aware. If you are in the risk category for achalasia, stay aware of the symptoms and seek medical attention immediately if you begin experiencing them.  
  • Seek medical intervention. If left untreated, achalasia can lead to serious complications. If you are having difficulty swallowing or experiencing other symptoms of achalasia, request an evaluation at Loma Linda University Health. Contact your provider or schedule the appointment through MyChart.