Read more about this disease, some with Classification – Types – Signs and symptoms – Genetics – Pathophysiology – Diagnosis – Screening – Prevention – Treatment and management – Cures and much more, some including pictures and video when available.
Achalasia, also known as esophageal achalasia, achalasia cardiae, cardiospasm, dyssynergia esophagus, and esophageal aperistalsis, is an esophageal motility disorder: The smooth muscle layer of the esophagus loses normal peristalsis (muscular ability to move food down the esophagus), and the lower esophageal sphincter (LES) fails to relax properly in response to swallowing.[1]
Achalasia is characterized by difficulty swallowing, regurgitation, and sometimes chest pain. Diagnosis is reached with esophageal manometry and barium swallow X-ray studies. Various treatments are available, although none cure the condition completely. Certain medications or Botox may be used in some cases, but more permanent relief is brought by esophageal dilatation and surgical cleaving of the muscle (Heller myotomy).
The most common form is primary achalasia, which has no known underlying cause. However, a small proportion occurs as a secondary result of other conditions, such as esophageal cancer or Chagas disease (an infectious disease common in South America).[2] Achalasia affects about one person in 100,000 per year.[3][2]
The main symptoms of achalasia are dysphagia (difficulty in swallowing), regurgitation of undigested food and chest pain. The dysphagia tend to be progressively worse over time, and to involve fluids and solids simultaneously. Some also experience coughing, especially when lying in a horizontal position. Food and liquid, including saliva, are retained in the esophagus and may be inhaled into the lungs (aspiration),[2] potentially leading to aspiration pneumonia. If the swallowing problems are severe, the inadequate intake of nutrients may lead to weight loss.
Due to the similarity of symptoms, achalasia can be mistaken for more common disorders such as gastroesophageal reflux disease (GERD), hiatus hernia, and even psychosomatic disorders.
Specific tests for achalasia are barium swallow and esophageal manometry. In addition, a CT scan of the chest and endoscopy of the esophagus, stomach and duodenum (esophagogastroduodenoscopy or EGD), with or without endoscopic ultrasound, are typically performed to rule out the possibility of cancer.[2] The internal tissue of the esophagus generally appears normal in endoscopy, although a “pop” may be observed as the scope is passed through the non-relaxing lower esophageal sphincter with some difficulty.
The patient swallows a barium solution, with continuous fluoroscopy (X-ray recording) to observe the flow of the fluid through the esophagus. Normal peristaltic movement of the esophagus is not seen. There is acute tapering at the lower esophageal sphincter and narrowing at the gastro-esophageal junction, producing a “bird’s beak” or “rat’s tail” appearance. The esophagus above the narrowing is often dilated (enlarged) to varying degrees as the esophagus is gradually stretched, and it may contain food debris.[2] An air-fluid margin is often seen over the barium column due to the lack of peristalsis. A five-minute timed barium swallow can provide a useful benchmark to measure the effectiveness of treatment.
Because of its sensitivity, manometry (esophageal motility study) is considered the key test for establishing the diagnosis. A thin tube is inserted through the nose, and the patient is instructed to swallow several times. The probe measures muscle contractions in different parts of the esophagus during the act of swallowing.
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