Achalasia is an esophageal disorder of unknown cause. Certain nerve fibers in the esophagus are destroyed, with two results: (1) the esophagus loses its ability to push, and (2) the valve at the lower end of the esophagus – the lower esophageal sphincter – does not relax properly. Achalasia can happen at any age, more frequently middle age. It affects men and women equally.
The result of these two problems is that the esophagus does not empty properly. Achalasia is a lifelong disorder, and there is no cure. Therapies are designed to help manage symptoms, primarily by enabling the lower esophageal sphincter (LES) to relax or open up better.
Symptoms of achalasia may include dysphagia (difficulty swallowing or food sticking), chest pain, and regurgitation (sensation of food or fluid coming back up – in this case food that never made it to the stomach). Occasionally, people can also experience heartburn. In primary achalasia, the cause is not well understood.
Diagnosis of Achalasia
Achalasia may be suspected on the basis of an upper GI endoscopy.
A barium swallow or cine esophagram is performed to evaluate the degree of esophageal distention and extent of narrowing of the lower esophageal sphincter.
An esophageal motility study (HRIM) is almost always required to make the diagnosis of achalasia as there are various subtypes that respond differently to therapy.
The goal of achalasia treatment is to improve the quality of life as much as possible, knowing there is not a cure for this disease. No interventions restore the motility of the esophagus, but interventions to help the lower esophageal sphincter can be beneficial to patients.
Swallowing can often be improved with interventions to help the lower esophageal sphincter stay open; chest pain and discomfort generally do not respond as well to therapy.
Medications: there are a few medications that may help the valve relax. These medications often do not work very well and would need to be taken for life.
Botox: Botox can be injected into the valve between the stomach and esophagus. The botox relaxes the sphincter muscle and shortterm improvement is seen in 2/3 of patients. However, long-term results of botox are poor and botox is not currently considered the best achalasia treatment option.
Balloon Dilations: During an endoscopy, a balloon is placed through the mouth and down to the valve in between the stomach and esophagus. The balloon is then forcefully inflated and this inflation tears the muscles in the valve. This is not as longterm as surgery, but may provide some improvement in the ability to swallow. There are small risks of tearing the esophagus that would require surgery. This is the best nonsurgical option for achalasia, and should be considered for patients who would not tolerate surgery.
Surgery: Surgery has demonstrated the most success longterm in the treatment of achalasia. The surgery, called a Heller myotomy, involves laparoscopically (small incisions in the abdomen) cutting the muscle of the valve and then rebuilding the valve to prevent reflux. Most patients notice improvement in swallowing immediately. There are small risks of recurrent swallowing problems and reflux/heartburn.
A new surgical therapy for achalasia is called per oral endoscopic myotomy (POEM). In this procedure, a special endoscope is placed through the mouth and tunneled under the lining of the esophagus. The same muscle layer that is cut during a Heller myotomy is cut from the inside of the esophagus. There are no incisions in the abdomen. However, there is a higher incidence of heartburn and GERD after this procedure than the Heller myotomy. There is also no longterm data as of yet, and most insurances consider the procedure investigational, and do not cover POEM.
Effectiveness and Downsides of Achalasia Surgery
Longterm, achalasia surgery is very effective in maintaining swallowing to a point where it is not a significant impact on your overall quality of life. Certain foods, such as steak, dry chicken, or bread may be off the menu for life.
Some patients (10-15%) develop excess acid exposure (GERD) after a Heller myotomy.
Patients with achalasia have a slight increased risk of developing esophageal cancer. The reason for this is not entirely clear. If you have achalasia, it is recommended that you have an endoscopy and biopsy periodically (no current recommendations on how often).
Before and After Achalasia Surgery
Before surgery: You will be on a clear liquid diet for 48 hours prior to your surgery. This is done because we do not want any food in your esophagus at the time of surgery. Most people with achalasia are otherwise healthy, and there is not much preparation needed, other than testing and confirming the diagnosis. Surgery takes about an hour to an hour and a half.
After surgery: This is usually an outpatient procedure and patients can go home the same day. After achalasia surgery, most patients notice an immediate improvement in the ability to swallow. You will be on a special diet for a few weeks after the surgery until the swelling goes down. Generally, there are no activity restrictions after surgery.