Achalasia - Causes, Symptoms, Diagnosis, and Treatment

What Is Achalasia?

Achalasia is a rare esophageal disorder that affects the motility of the esophagus, this disorder leads the food and liquid to be unmovable in the stomach. The esophagus is a muscular tube that transports food from the mouth to the stomach, and the point where the esophagus and stomach meet is made up of the ring called LES (lower esophageal sphincter). This sphincter closes tightly to prevent the backing of the food contents. In achalasia, LES does not relax and prevents food from moving into the stomach. It can occur at any age and commonly affects 25 to 60 years of age. Rarely is the condition inherited.

What Causes Achalasia?

The exact cause of achalasia is unknown. It is suspected that it may be caused by nerve cell loss in the esophagus. There are theories about the cause of the condition, and suspected theories are autoimmune responses and viral infection. Rarely may it be caused by an inherited genetic disorder and infection. In some cases, the body's own immune system attacks nerve cells of muscle layers of the esophagus and at the lower end sphincter. Excessive contractions in the LES occur as a result, and if achalasia occurs LES fails and liquid can't pass through the esophagus in the stomach.

How Serious Is Achalasia?

When achalasia is not treated, it can lead to various health complications like:

• Esophagitis.

• Megaesophagus.

• Aspiration pneumonia.

• Esophageal perforation.

• Esophageal cancer.

There is no cure for achalasia, so even with treatment, symptoms of achalasia do not fade away completely. Several lifestyle changes are to be made like:

• Avoid foods that cause heartburn.

• Having smaller meals.

• Sleeping elevated instead of sleeping flat.

• Quit smoking.

What Are the Signs and Symptoms of Achalasia?

Signs and symptoms of achalasia generally appear gradually and get worse over time.

• Heartburn.

• Regurgitation of saliva or food.

• Dysphagia (Inability to swallow food).

• Coughing at night.

• Chest pain that comes and goes.

• Belching.

• Nausea.

• Vomiting.

• Weight Loss.

• Pneumonia.

What Are the Complications of Achalasia?

Complications of achalasia are a result of food backing into the esophagus and trachea, which leads to lungs. The complications are:

• Lung infections.

• Pneumonia.

• Esophageal cancer- achalasia increases the risk of cancer.

How Is Achalasia Diagnosed?

The tests that are used to diagnose achalasia are:

• Barium Swallow: For this procedure barium containing liquid is asked to swallow, and the esophagus is evaluated using x-rays. This test reveals narrowing of the esophagus at the LES.

• Manometry: A test that measures the strength and timing of esophageal contractions and relaxations of the lower esophageal sphincter. Failure of LES on the walls of the esophagus is a positive test for achalasia. This is the gold standard test.

• Upper Endoscopy: A narrow flexible tube with a camera head is inserted into the esophagus. This camera takes images of the esophagus onto a screen for evaluation. Helps in ruling out cancerous lesions as assessed for achalasia.

What Is the Treatment for Achalasia?

Treatment of achalasia depends on stretching open and relaxing the lower esophageal sphincter, which would help the food and liquid pass through. Specific treatments depend on medical and health conditions, age, and severity of the disease. There are mainly two approaches:

Non-surgical method:

• Medication: Healthcare providers may recommend muscle relaxants like nifedipine (Procardia) and nitroglycerine (Nitrostat). These medications have severe side effects and minimal treatment effects. The individuals are considered for a medicational approach only if they are not candidates for surgery or pneumatic dilation and botox also has no effect.

• Botox: This type of muscle relaxant is injected into the esophageal sphincter with an endoscopic needle. Repeat injections are given, which makes it more difficult to perform surgery. Botox is recommended for the individuals in whom the surgery and pneumatic dilation is not done due to overall age health. A great improvement by botox administration helps in confirming the diagnosis of achalasia.

• Pneumatic Dilation: In this procedure, a balloon is inserted in the esophageal sphincter, and the opening is inflated. It is a procedure where the esophageal sphincter does not stay open. Overall, one-third of individuals who are treated with balloon dilation require treatment to be repeated within five years. This procedure is done under sedation.

Surgery:

• POEM (Peroral Endoscopic Myotomy): In this procedure, the health care provider uses an endoscope that is inserted through the mouth into the esophagus and creates an incision inside the lining of the esophagus. Then, following the same technique as Heller myotomy, the provider cuts the muscle at the LES (lower end sphincter). This technique can also be combined with later fundoplication that helps in preventing gastroesophageal reflux. In some cases, where individuals who have POEM and develop gastroesophageal reflux are being treated with daily oral medication.

• Heller Myotomy: In this procedure, the health care provider cuts the lower esophageal sphincter and helps the food to pass through easily. This procedure can be done non-invasively. People who have Heller myotomy have a tendency to develop GERD later in life. To avoid this, a procedure called fundoplication is performed along with heller myotomy. In this technique, the provider wraps the top end of the stomach around the lower esophagus; this creates an anti-reflux valve and thus prevents GERD. Fundoplication is done as a minimally invasive procedure.

What Are the Complications of Treatment of Achalasia?

Complications of treatment of achalasia are:

• Bloating.

• Gastroesophageal reflux disease.

• Formation of a hole in the esophagus.

• Lack of success of treatment and recurrence of achalasia symptoms.

What Is the Differential Diagnosis of Achalasia?

The differential diagnosis of achalasia are:

• Stricture: Abnormal tightening or narrowing of the esophagus.

• Schatzki Ring: A circular mucosa and submucosa that forms the squamocolumnar junction of the distal esophagus.

• Hiatal Hernia: It is part of the stomach that is pushed through the diaphragm muscle.

• Scleroderma: Hardening and tightening of the skin and connective tissue.

• Gastroesophageal Reflux Disorder: A chronic condition where the bile and stomach acid irritate the esophagus lining.

• Diffuse Esophageal Spasm: This is a rare esophageal motility disorder.

• Paraesophageal Hernia: Condition where the lower part of the esophagus and other organs move to the chest.

Conclusion

Achalasia is a rare esophageal disorder with no cure. Treatment remains palliative currently. Care between gastroenterologists, nutritionists, and thoracic surgeons is done by primary care. Most cases are managed outpatient, but in cases of severe malnutrition, dehydration, and electrolyte imbalance, hospitalization is needed. Even after successful treatment, lifelong follow-up is necessary.