ACHALASIA ('difficulty swallowing')

• What causes Achalasia?
• Symptoms of Achalasia
• Complications of Untreated Achalasia
• How is Achalasia Treated?
  • Pneumatic Dilatation
  • Botulinum toxin injection
• Laparoscopic Surgical Therapy
• Possible Complications of Laparoscopic Surgery

What is Achalasia?

Achalasia is a disease of the esophagus in which that organ's ability to move food down and into the stomach is severely reduced or restricted. In this condition, the lower esophageal sphincter, a muscular barrier between the esophagus and stomach, is unable to relax sufficiently to allow food an liquid to pass normally into the stomach. Only after sufficient pressure can be provided by the weight of the column of the food in the esophageal tube, will food enter the stomach. Patients experience progressive and severe difficulty swallowing solids and liquids. They also complain of retro-sternal chest pain or discomfort, regurgitation of undigested food, aspiration (inhalation) of gastric and esophageal contents in the lungs which leads to pneumonia. This is a progressive disease, and generally gets worse, not better. For patients with achalasia, swallowing is difficult and unpleasant. Substantial weight loss is common and lung infections due to aspiration (inhalation) of swallowed food material is not unusual. Symptoms frequently do not improve spontaneously.

What causes Achalasia?

There are primary and secondary causes of this disease. It is felt that patients with primary Achalasia lack a specific nerve complex in the muscular wall of the esophagus that is responsible for relaxation of the LES during meals. Degenerative changes can occur in the entire chain of nerves that begins in the brain and extends through the brain stem, spinal cord, esophagus, and stomach. This is confirmed by pathology that has found that there is a loss of ganglion cells in the myenteric plexus (nerve connections that stimulate the esophagus). Another finding is active destruction of the nerve cells by another cell type called 'lymphocytes'. It is not understood why this destruction of the nerve cells occurs.

At any rate, it is this absence of neural impulses that creates a functional lack of control in the esophagus and LES. This lack of nerve stimulation causes the LES to remain tight and unable to relax.

Other disease entities can cause achalasia as well: esophageal cancer, scleroderma, and diabetes, among others.

Symptoms of Achalasia

The most frequent presenting symptoms are difficulty swallowing (dysphagia). It is insidious and intermittent in the beginning but tends to become progressively worse. It is rare that it leads to an abrupt loss of ability to eat or drink. The symptoms are subtle in its onset and most describe "fullness".

• Heartburn occurs in 25 to 45% of patients.
• Coughing and lung infections from food particles trapped in bronchial tubes (aspiration of food) occur in 10% of patients. Some patients have coughing at night from food aspirating into the trachea from food in the esophagus. Elevating the head of the bed may often help these people.
• Over 90% of patients have regurgitation of undigested foods.
• Chest spasms or pains, resembling heart pain (angina), occur in 30-50% of patients.
• Hoarseness, drooling, and belching may also occur. Because of these symptoms, the diagnosis may often be missed. Most commonly, many are misdiagnosed as having gastroesophageal reflux disease or GERD.

Eventually, the dysphagia occurs with both liquids and solids, this distinguishes achalasia from a mechanical obstruction know as peptic stricture. The difficulty swallowing is more pronounced with solid food, and patients then start to avoid meats, leafy vegetable, pastas, breads and liquids. Eventually some weight loss may develop.

The patient's eating habits change by eating slower, chewing longer, arching the back or raising their arms to swallowing by gravity. At this point, the esophagus is only a passive tube unable to contract.

Complications of Untreated Achalasia

Due to the inability to eat, severe weight loss and malnutrition may develop. Repeated lung infections or pneumonia from frequent aspiration of food may lead to permanent lung injury. The most feared complication of untreated achalasia is cancer of the esophagus. This occurs in 41 per 100,000 patients-ten times that of the general population.

How is Achalasia Treated?

Pneumatic Dilatation

In this procedure, usually performed on an outpatient basis, a guide wire is passed into the stomach with the aid of an endoscope. After the endoscope is removed, a pneumatic balloon is passed over the guide wire, half above and half below the LES. The balloon is then rapidly inflated for 30-60 seconds, expanding the circular muscles around the diaphragm, causing them to rupture slightly.

Pneumatic dilatation is successful in 60% to 95% of patients and can last for ten years. The procedure also seems to be more effective for people over 45.

One of the downsides of this procedure is that the inflation of the balloon can be extremely painful. Another is that the esophagus can be perforated (torn), which-depending on the extent of the tear-may require an emergency chest operation for repair and drainage. Perforations occur about 5% of cases. Other complications can include acid reflux in the esophagus, bleeding, and aspiration.

This procedure can be repeated, but effectiveness drops about 50% with each subsequent treatment.

Botulinum toxin injection.

This treatment involves injecting the esophageal musculature with a neurotoxin that results in lowering relaxation of the LES. There is an initial success rate of approximately 75%. However, studies demonstrate that after 2.5 years, practically all patients have recurrence of their symptoms, necessitating repeat injections every 10 months or so.

Surgical Therapy - Minimally Invasive (Laparoscopic) Myotomy

This procedure has a 95% success rate in alleviating the symptoms of achalasia. The "Heller Myotomy" (see diagram) was originally performed through a large abdominal incision, but due to recent advances in endoscopic surgery, it can now be accomplished with smaller incisions, allowing for a faster recovery and an earlier return to work.

During the procedure, the patient is put under general anesthesia. Five small incisions are made on the abdominal wall and a laparoscope is inserted into the area around the stomach. After the lower end of the esophagus is found and moved into position, the muscular ring surrounding the LES is cut, allowing it to open more easily. This is done while a gastroenterologist has inserted an endoscope. This is done to evaluate the length and adequacy of the of the myotomy and search for any perforation of the esophageal wall. If a hiatal hernia exits, it is closed. An anti-reflux procedure is usually not performed because it may cause postoperative obstruction to swallowing.

However, in some cases, an anti-reflux procedure is required. A procedure called the Toupet Fundoplication is performed by passing the left upper part of the stomach behind the esophagus and anchoring it to the right and left sides of the esophagus.

Possible Complications of Heller Laparoscopic Myotomy

Tearing and leakage of the esophagus may occur from performing the myotomy. If the repair is too tight, obstruction at the esophago-gastric junction that may lead to a swallowing disorder similar to Achalasia. Regurgitation of acid or food from the stomach and into the esophagus (GERD) may happen because of any incompetent LES.

All these procedures-medication, pneumatic dilatation, and laparoscopic Heller myotomy- are intended to relax the LES; none have any effect on the normal muscular function and propagation of food and liquids of the main esophagus function of the main esophagus.