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Achalasia

Overview

Achalasia refers to esophageal achalasia, a rare motility disorder that happens due to the defective nervous transmission of the smooth muscles of the esophagus- the food pipe. Your esophagus consists of smooth muscles which contract and relax (peristalsis) to push your food from the mouth to the stomach. The lower part of the esophagus has a ring of smooth muscles called the lower esophageal sphincter (LES) that acts like a gate that can open to allow the food to go down in the stomach and close so that the acidic contents from the stomach do not come back to the esophagus. In achalasia, the peristaltic movements of the esophagus are impaired, the LES remains tight and relax inadequately to let the food go down. Hence, the food remains in the esophagus, and also, some of the stomach contents can regurgitate back in the esophagus. Symptoms include difficulty in swallowing food, regurgitation, heartburn, and pain in the chest. Surgical and non-surgical treatments are available, although none cure the disease. Primary Achalasia can occur by itself or may result from other esophageal disorders like cancer, in which case it is called secondary achalasia. 

Epidemiology

In the U.S., Achalasia affects about 1 in every 100,000 people annually. Adults between the ages of 25 and 60 are most commonly diagnosed with achalasia. However, it can occur in any age group, and less than 5% of cases are found in children.  It has no predilection for any race and affects men and women equally. Very rarely, a form of achalasia can be inherited. 

Causes

The cause of achalasia is not understood well. It is thought to result from an abnormal immune reaction of your body which may be triggered by a virus. The nerve cells found between the muscle layers of the esophagus are attacked and damaged by immune cells. These nerve cells that control the muscular movements degenerate slowly, resulting in achalasia. 

Signs And Symptoms

The symptoms of achalasia appear gradually and deteriorate over time. They may include:

·         Inability to swallow solids and later liquids also.

·         Returning of food or saliva in the mouth

·         Heartburn

·         Recurrent chest pain

·         Cough

·         Vomiting

·         Pneumonia or lung infections due to aspiration of food in the lungs

·         Weight loss

Diagnosis

Due to the gradual appearance of symptoms, the diagnosis is often difficult to make and is usually made late. Some of the tests used to diagnose achalasia are;

Barium swallow: In this test, you will be given a barium liquid to swallow, and its movement is recorded through X-Rays. If you have achalasia, the report will show a beak-shaped narrowing of the distal end of the esophagus.

Upper GI endoscopy: This test is used to visualize the inside of your esophagus by passing a narrow flexible tube with the camera through your mouth into the esophagus. You may be sedated during this test. It can rule out cancer or strictures and check for the development of Barrett's esophagus as well.

Manometry: This test is used to measure the pressure in the muscles of the esophagus made during the contraction and relaxation of the muscles, especially the lower esophageal sphincter, to check its ability to open and close or work properly. It is the best diagnostic test for achalasia.

Differential Diagnosis

·         GERD (Gastroesophageal reflux disease)

·         Esophageal stricture

·         Esophageal cancer

·         Disorders causing nervous dysfunction involved in swallowing like multiple sclerosis, Parkinson’s disease, stroke, or amyotrophic lateral sclerosis (ALS). 

Treatment

The goal of treatment is to relieve the symptoms and relax the LES to allow the passage of food. This is done either by medicines, minimally invasive procedures, or surgery.

Medicines: Some medicines that can relax muscles are used like nitroglycerine, calcium channel blockers (nifedipine)

Botox injections: Some people who may not be ideal candidates for surgery may benefit from botulinum toxin injections in the LES to paralyze it to keep it open. They need to be repeated after every six months.

Pneumatic dilatation: In this procedure, the LES is stretched open by passing a balloon attached to the endoscope through LES and inflated to open it. It is performed as an outpatient or a day case, and you are sedated during this procedure. It is a pretty successful procedure, but it may need to be repeated after five years in some people.

Surgery: Heller myotomy is a surgical procedure in which a surgeon makes a cut in the LES. It can be performed laparoscopically or via an endoscope called Peroral Endoscopic Myotomy (POEM). As it predisposes a patient to gastric reflux disease, it may be accompanied by fundoplication in which a surgeon wraps around a part of the stomach to the esophagus to make a valve-like action to prevent reflux. 

Prognosis

Achalasia is a rare disease but can lead to troublesome symptoms. Untreated disease can cause the aspiration of food contents into the lungs leading to pneumonia or lung infections. The treatment is palliative, and it does not cure the disease. Even after pneumatic dilatations or surgery, the function of swallowing can deteriorate over time. You may need to follow up with the doctor, who will continue to monitor the relapse of symptoms. The regurgitation of acidic contents from the stomach can irritate the esophagus and result in the development of a pre-cancerous condition called Barrett's esophagus. During the follow-ups, the doctor also monitors the risks of cancer development. Some people may need a revision of the procedures. 

Lifestyle Modifications

Achalasia is a long-term disease. The treatment procedures only ease the severity and relieve the symptoms but do not cure achalasia. You may need to know about the results of each procedure and choose the right one after discussing it with your doctor. You may need to make some lifestyle changes to cope with the condition like:

·         Take small bites of food, chew well, and eat slowly in an upright position to let the gravity pull the food down.

·         Drink enough water with the food.

·         Avoid food at bedtime.

·         Keep the head side of the bed elevated to avoid gastric reflux.

·         Avoid foods like ketchup, chocolates, citrus, alcohol, and caffeine as they may increase gastric reflux.

·         Both before and after treatment, your doctor may prescribe you some medicines that decrease the release of stomach acid.