Achalasia and Failure of Lower Esophagus Sphincter Relaxation (failure of the lower esophageal sphincter (LES) to open)

 

This is a rare functional motor disorder (neuro-muscular) disease of the entire esophagus due to degeneration of the autonomous nerve fibres, resulting in the lack of regular contractions of the esophagus towards the stomach and failure of the lower esophageal sphincter to open. (The lower esophagus sphincter is the muscle which opens and closes the end of the esophagus at its junction with the stomach).  

 

Symptoms

Difficulty in swallowing, more pronounced with liquids than with solids. Pains behind the breastbone, especially after a meal, halitosis, chocking on food with pneumonia as a complication.

 

Diagnostic procedures 

X-rays of the esophagus are taken after a contrast medium (barium) is swallowed. Endoscopy is done to exclude the presence of tumors, and if indicated tissue samples are taken. Manometry to measure the pressure of the lower sphincter.

 

Treatment

The preferred treatment is one with medication and a careful endoscopic dilatation of the lower oesophageal sphincter. If repeated dilatations fail to produce the desired results, surgery is indicated. Surgery also becomes necessary in unclear cases as a malignant tumor can sometimes develop in these cases. 

 

Surgical Therapy

A cardiomyotomy (myotomy = a surgical procedure in which a muscle is cut) is done after the upper abdomen has been opened by a vertical incision. The junction of the esophagus and stomach is exposed and an incision in made through the musculature (sphincter) of the lower esophagus to the stomach, taking utmost care not to injure the mucous membrane. The cramped muscle is thus relaxed. Afterwards a fundoplication is done: the top portion of the stomach (fundus) is wrapped around the back of the esophagus where the incision was made. The portion of the fundus which is now on the right side of the esophagus is sutured (sewn) to the portion on the left side to keep the wrap in place (the esophagus is in the middle with the fundus thus wrapped around it like a necktie). This prevents stomach acid from flowing into the esophagus. In our hospital this surgery is usually performed laparoscopically and its purpose is to make it easier for the food to pass into the stomach.

 

Complications

A long term undetected or insufficiently treated achalasia can present a higher risk of esophagus cancer. Endoscopic dilatation therapy has the risk of perforation of the esophageal wall. Surgical therapy has a slight risk of injury to the vagus nerve (a nerve which originates in the brainstem and controls heart rate, intestinal movement gastric acid secretion etc.). In some cases there is an insufficient closing of the lower esophagus sphincter resulting in reflux disease with acid indigestion. 

 

Aftercare

Because of an increased carcinoma (tumor) risk, it is advisable to undergo regular endoscopic examinations and if these show anything suspicious, tissue samples should be taken.