Achalasia Treatment & Diagnosis

Achalasia is a rare disease involving the muscles of the esophagus (the “tube” that connects the mouth to the stomach). It interferes with the passage of food and drink down the esophagus, causing regurgitation, vomiting or even chest pain. 

The esophagus transports food from the mouth to the stomach. After swallowing, food and liquid are pushed down the esophagus by the muscles in the esophageal wall contracting in a coordinated way, called peristalsis. Once the food and liquid reach the end of the esophagus, they come up against a one-way muscular valve called the lower esophageal sphincter (LES). The LES senses that food is coming down the esophagus and opens to let it pass into the stomach. The LES then closes to prevent the food, liquid and digestive enzymes in the stomach from washing back up the esophagus.

People with achalasia have two problems:
  1. The muscles in the esophageal wall no longer contract in a coordinated fashion, or they stop contracting completely, keeping food and liquid from being pushed down into the stomach properly. 
  2. The LES does not open and relax when it is supposed to, causing food and fluid to get stuck in the lower esophagus, passing slowly (or not at all) into the stomach.

Symptoms of achalasia
People who have achalasia can have any or all of the following symptoms:
  • Backflow of food or liquid from the esophagus into the mouth (regurgitation)
  • Vomiting
  • Difficulty swallowing food or liquid (dysphagia)
  • A burning feeling in the lower chest or the upper abdomen just beneath the breast bone (heartburn)
  • Chest pain that gets worse after eating and can even be felt in the back (between the shoulder blades), neck or arms
  • Unplanned weight loss
The average amount of time people experience symptoms before they are diagnosed with achalasia is 4.6 years. Symptoms develop slowly and are often confused with acid reflux. In fact, many people diagnosed with achalasia have been told they have acid reflux, yet acid reflux actually is the opposite problem. With acid reflux, your LES is too loose or opens at the wrong time, allowing acid from the stomach to wash back up the esophagus, causing heartburn. Achalasia patients can get heartburn as well, but it is from food and fluid pooling in the lower esophagus (just above the LES) and irritating the esophageal lining.

Causes of achalasia
Nobody is exactly sure how achalasia develops. Possible causes include:
  • Infection/autoimmune: The most common theory is that a person gets an infection that causes the immune system to “turn on” and fight it. Unfortunately, the immune system can mistake the nerves in the esophagus as the cause of the infection and as a result destroy the nerves, causing achalasia. 
  • Genetic: Very rare, accounting for only 1 to 2 percent of patients.
  • Degenerative: A small percentage of people with achalasia, especially older patients, seem to get it from slow destruction of their nerves caused by an unknown neurological problem.

Why Houston Methodist Hospital?
Because achalasia is a rare disease, many doctors are unfamiliar with how to diagnose and treat it. This is why most patients with achalasia have had swallowing difficulties for years before they are diagnosed. It is important to be treated in a place like the Lynda K. and David M. Underwood Center for Digestive Disorders at Houston Methodist Hospital that has a team of specialists who are experts in esophageal diseases, and have the right tools to treat them. The team includes:
  • Experts in esophageal motility who understand how to do the manometry tests and who can read them properly
  • Gastroenterologist and surgical endoscopists who are skilled at performing upper endoscopies and recognizing the features of achalasia
  • Minimally invasive surgeons who perform laparoscopic myotomy surgery frequently

The proper tools to diagnose and treat achalasia are also important:
  • High resolution manometry – this is like the HD of manometry and important for making the right diagnosis in achalasia. Not every hospital has this technology.
  • Laparoscopic surgery – minimally invasive surgery for achalasia is an advanced procedure and even experienced surgeons may only do a handful a year. It is important to be treated at a hospital like Houston Methodist Hospital with the latest laparoscopic equipment and skilled surgeons who are familiar with that equipment and who can perform surgery with the least amount of pain and quickest recovery.
  • POEM – Houston Methodist Hospital is the only institution in the state of Texas that performs the POEM procedure for achalasia. It takes special training and instruments to perform and patients benefit by having a successful operation with no scars, minimal pain and quick return to full activity. 

DIAGNOSING ACHALASIA

Doctors primarily rely on two tests to determine if a patient has achalasia:
  1. Esophageal manometry: The best method for diagnosing achalasia, it measures how well the esophagus squeezes (peristalsis), the pressure on the lower esophageal sphincter (LES), and whether or not this LES relaxes when the patient swallows water. While patients must be awake so they can swallow water when they are asked to, manometry is not painful. 
  2. Upper endoscopy: This minimally invasive procedure allows either a gastroenterologist or a surgical endoscopist to look into the esophagus and stomach. While a patient is under anesthesia, the doctor passes a long flexible tube with a light and camera on the end of it (endoscope) through the mouth and into the esophagus and stomach, looking to see if there is food or fluid in the esophagus, whether or not the esophagus is dilated because the wall muscles are weak, if the LES appears tight and if there are any other causes behind the swallowing difficulties.

Other tests that help diagnose achalasia include:
  1. Barium swallow: Patients swallow a white liquid called barium that radiologists can see in an x-ray and use to trace the pathway from the esophagus into the stomach. Patients also may swallow a barium pill to see if it passes or not.
  2. CAT scan: A CAT (computer aided tomography) scan can show if there is something in the chest that is pushing on the esophagus and causing symptoms similar to achalasia. 

TREATMENT & PROCEDURES

Unfortunately, there are no medicines that work well in the treatment of achalasia. All treatments require a procedure or surgery and focus on one goal: getting the lower esophageal sphincter (LES) to open up and allow passage of food and liquid. 

It is important to be treated in a place like the Lynda K. and David M. Underwood Center for Digestive Disorders at Houston Methodist Hospital that has a team of specialists who are experts in esophageal diseases and have the right tools to treat them.

At the center, our experts treat achalasia in three ways:
  1. Botox injection: During an upper endoscopy, doctors can inject Botox (Botulinum toxin) into the LES to get it to relax. Unfortunately, Botox loses its effectiveness after two or three months, and it is less likely to work each time it is re-injected. Botox injections are usually only used as a temporary solution or in someone who is not fit to have a more permanent procedure.                                                                                                                                                
  2. Dilation: Specialists use x-ray guidance to pass a balloon into the esophagus and dilate the LES. The balloon stretches the muscle of the LES so dramatically that it permanently relaxes. Risks include:
    • The dilation balloon can rupture the esophagus, causing a hole. This happens in 2 to 3 percent of cases and often requires urgent surgery to fix. 
    • More than one dilation is often required to get a good result, which further increases the risk of esophageal rupture. 
    Because of these risks, many doctors have moved away from balloon dilation and now recommend minimally invasive surgery for their achalasia patients.             
  3. Surgery: Surgery has become the most common method used to treat patients with achalasia. The goal of surgery is to cut the muscular ring of the LES so it will relax, without putting a hole all the way through the wall of the esophagus. There are two layers to the esophageal wall: an inner layer called the mucosa, and an outer muscular layer separated by a flimsy layer of tissue called the submucosa. Surgery cuts the muscle layer of the esophagus without cutting the mucosa. This “breaks” the ring of the LES muscle, allowing it to open up and relax. The act of cutting the muscle is called a “myotomy.” There are two ways to perform a myotomy for achalasia — working from the outside of the esophagus (Heller myotomy) and working from the inside (POEM: Per Oral Endoscopic Myotomy).
    • Laparoscopic Heller myotomy: During this minimally invasive laparoscopic surgery where the patient is under general anesthesia, the surgeon cuts into the muscular layer of the lower esophagus and upper stomach without damaging the underlying mucosa. 
    • POEM (Per Oral Endoscopic Myotomy): This is a new procedure where the surgeon performs the myotomy while working completely inside the esophagus, making no incisions on the abdomen. 

Recovery
Both the Heller myotomy and POEM surgeries take from two to three hours. Heller myotomy patients will experience some mild pain at their abdominal wall incisions and may experience some mild chest pain. POEM patients tend to have less pain because there are no incisions. 

The day after surgery, many surgeons order a barium swallow to look for leaks from the esophagus and to make sure the liquid barium passes into the stomach. If the barium swallow looks okay, patients may start eating a liquid diet. Once patients demonstrate they can eat a liquid diet without problems, they can go home. For most patients, this is the day after surgery.

Most surgeons tell patients to follow a liquid or soft diet until their follow-up visit in one to two weeks. During this time they are encouraged to be physically active, but not to strain their abdominal muscles too much. After the first post-operative check-up, the patient’s surgeon will decide how soon the patient can eat regular food, typically within two to four weeks.

Can achalasia come back after surgery?
Both the Heller myotomy and POEM procedure are intended to be permanent fixes for achalasia. However, there is a chance that the cut muscle of the esophageal wall could scar together again, causing swallowing difficulties to return. Long-term follow-up of patients who have had a myotomy show that 80 to 90 percent of patients feel well more than two years after their surgery.  

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