Achalasia: What is it?
Your esophagus is impacted by the dangerous disorder known as achalasia. The tube that moves food from the throat to the stomach is called the esophagus. The muscular ring known as the lower esophageal sphincter (LES) separates the esophagus from the stomach. Your LES should expand up while swallowing if you have achalasia, but it doesn't. This causes food to back up in your esophagus. This condition may be brought on by esophageal nerve injury. It might also be a result of LES damage.
Acetylcholine, substance P, and inhibitory neurotransmitters like nitric oxide and vasoactive intestinal peptide control LES pressure and relaxation. Lack of noradrenergic, non-cholinergic, inhibitory ganglion cells in achalasia patients leads to an imbalance in
Background
Sir Thomas Willis described achalasia in 1672. In 1881, von Mikulicz described the disease as a cardiospasm to indicate that the symptoms were due to a functional problem rather than a mechanical one. In 1929, Hurt and Rake realized that the disease was caused by a failure of the lower esophageal sphincter (LES) to relax. They coined the term achalasia, meaning failure to relax.
Epidemiology
Achalasia is an uncommon disorder with an annual incidence of approximately 1.6 cases per 100,000 individuals and prevalence of 10 cases per 100,000 individuals [1]. Men and women are affected with equal frequency. The disease can occur at any age, but onset before adolescence is rare. Achalasia is usually diagnosed in patients between the ages of 25 and 60 years. Achalasia may occur in association with adrenal insufficiency and alacrima in patients with triple A syndrome or Allgrove syndrome, a rare autosomal recessive genetic disorder
Causes
Achalasia can happen for different reasons. It can be difficult for your doctor to find a specific cause. This condition may be hereditary, or it may be the result of an autoimmune condition. With this type of condition, your body’s immune system mistakenly attacks healthy cells in your body. The degeneration of nerves in your esophagus often contributes to the advanced symptoms of achalasia.
Other conditions can cause symptoms similar to achalasia. Cancer of the esophagus is one of these conditions. Another cause is a rare parasitic infection called Chagas’ disease. This disease occurs mostly in South America.
Possible Risk factors
Allgrove syndrome, also known as triple-A syndrome (AAA), or as Achalasia-Addisonianism-Alacrimia syndrome,
Herpes infection
Measles infection
Autoimmune diseases
HLA type 2
Symptoms and signs of Achalasia
The symptoms of achalasia include:
Difficulty swallowing food
Difficulty swallowing both solid and liquid food
Regurgitation of food that is retained in the esophagus. If this happens at night, food may be aspirated into the lungs, a serious medical problem
Chest discomfort from esophageal dilation and/or retained food
Sharp chest pain usually of unclear cause
Heartburn; however, the heartburn is not characteristic of heartburn and is not helped by treatment for heartburn
Loss of weight due to reduced intake of food
Frequent coughing
Feeling as if there is always a lump in your throat
Unintentional weight loss and/or malnutrition
Complications of achalasia
Aspiration Pneumonia: This is the most serious complication of achalasia cardia but rare. In some cases due to sudden regurgitation process, gastric contents from the stomach enter in to the lungs leading to bronchopneumonia. Patient may present with sudden breathlessness, choking, vomiting episodes and respiratory distress.
GERD- Long term immobility of the esophagus causing weakening of LES can lead to frequent regurgitation of the food contents to the mouth causing acid reflux disease. Patients normally present with epigastric pain, fullness after meals, water brash and indigestion.
Esophagitis- Constant collection of food and gastric contents in the esophagus can irritate the mucosal lining causing inflammation of esophagus known as esophagitis.
Perforation of esophagus- Chronic inflammation and irritation can also cause ulceration leading to formation of a hole in esophagus called esophageal perforation.
Esophageal cancer: There is not much significant evidence medically about the correlation of esophageal cancer with achalasia cardia. However some patients who have been diagnosed with adenocarcinoma of esophagus have undergone dilatation procedure for achalasia conditions.
Malnutrition: Prolonged weight loss due to inability to swallow liquids and solids can lead to significant weight loss which can affect the nutritional status of the individual.
Diagnosis and testing
Physician may suspect achalasia based on symptoms and physical examination. Achalasia should be suspected if patient have difficulty swallowing both solids and liquids and also regurgitation that has not resolved despite treatment with proton pump inhibitors.
Esophageal manometry. This involves placing a tube in esophagus while swallowing. The tube records the muscle activity and makes sure your esophagus is functioning properly.
An X-ray or similar exam of your esophagus may also be helpful in diagnosing this condition. Other doctors prefer to perform an endoscopy. In this procedure, doctor will insert a tube with a small camera on the end into esophagus to look for problems.
Endoscopy also is helpful in the diagnosis of achalasia although it can be normal early in achalasia. Endoscopy is a procedure in which a flexible fiberoptic tube with a light and camera on the end is swallowed. The camera provides direct visualization of the inside of the esophagus.
Barium swallow. The patient swallows a barium preparations (Liquid or other form) and its movement through the esophagus is evaluated using X-Ray.
Diagnosis of Achalasia patient by Barium swallow
Treatment and Medications
Treatment aims at opening or relaxing the lower esophageal sphincter muscles for easy passage of food into the stomach. Some of the options for relieving symptoms include:
POEM: POEM or Per Oral Endoscopic Myotomy is a new treatment being offered to allow a myotomy to be performed using a gastroscope rather than having an operation. This latest treatment is offered by Mr Crosthwaite
Medications: Medications include nitrates, such as isosorbide dinitrate (Isordil), and calcium channel blockers (CCBs), such as nifedipine (Procardia) and verapamil, serve to relax the lower esophageal sphincter muscles and offer momentary relief (Calan).
Injections of botulinum toxin can be given to help relax the sphincter muscles. It only offers transient relief and must be repeated a few months or years later.
Pneumatic balloon dilatation: To enlarge the opening, a tiny balloon is placed at the lower esophageal sphincter and inflated. While this operation helps with swallowing, it is not a long-term fix and must be repeated.
Surgery called myotomy involves severing the sphincter muscle to widen the esophagus. This procedure offers a long-lasting remedy.
Prevention
Achalasia has a number of unpreventable causes. However, the disorder's therapy might help to avert consequences.
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