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Achalasia cardia
Last reviewed: 04.07.2025
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Achalasia of the cardia (cardiospasm, aperistaltic esophagus, megaesophagus) is a disease of the esophagus characterized by the absence of reflex opening of the cardia during swallowing and accompanied by impaired peristalsis and decreased tone of the thoracic esophagus (A. L. Grebenev, V. M. Nechaev, 1995), as a result of which the evacuation of food into the stomach is disrupted.
Achalasia is a neurogenic disorder of the esophagus that is characterized by impaired peristalsis and inadequate relaxation of the lower esophageal sphincter during swallowing. Symptoms of achalasia include slowly progressive dysphagia, usually to liquids and solids, and regurgitation of undigested food. Evaluation typically includes barium swallow, endoscopy, and sometimes manometry. Treatment of achalasia includes esophageal dilation, drug denervation, and surgical myotomy.
Most often, the disease achalasia cardia occurs at the age of 25-50 years, and women are more often affected than men. The prevalence of achalasia cardia is 0.5-0.8 per 100,000 population (Mayberry, 1985).
ICD-10 code
K22.0 Achalasia of the cardiac part.
What causes achalasia of the cardia?
Achalasia of the cardia is thought to be due to a decrease in the number of ganglion cells in the intermuscular plexus of the esophagus, leading to denervation of the esophageal musculature. The etiology of the denervation is unknown, although a viral etiology is suspected; some tumors can cause achalasia by direct obstruction of the esophagus or as a paraneoplastic process. Chagas disease, which involves destruction of the autonomic ganglia, can lead to achalasia.
Increased pressure in the lower esophageal sphincter (LES) causes its obstruction with secondary dilation of the esophagus. Typical is the retention of undigested food in the esophagus with the development of congestive chronic esophagitis.
Symptoms of achalasia cardia
Achalasia of the cardia may develop at any age, but usually begins between the ages of 20 and 40 years. Onset is sudden, with gradual progression over months to years. The cardinal symptom is dysphagia for both solids and liquids. Nocturnal regurgitation of undigested food occurs in approximately 33% of patients and may cause cough and lead to pulmonary aspiration. Chest pain is less common but may be present with swallowing or spontaneous. Patients have mild weight loss; if weight loss occurs, particularly in older patients with rapid onset of dysphagia, achalasia secondary to a tumor at the gastroesophageal junction should be considered.
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Diagnosis of achalasia of the cardia
The main investigation is fluoroscopy with a barium swallow, which reveals the absence of progressive peristaltic contractions of the esophagus during swallowing. The esophagus is often significantly dilated, but in the area of the LES it is narrowed like a bird's beak. Esophagoscopy reveals dilation of the esophagus without any pathological formations, but the endoscope easily passes into the stomach; difficult advancement of the device raises suspicion of an asymptomatic course of a tumor or stricture. To exclude malignancy, examination of the posteriorly curved cardiac part of the stomach, biopsy, and mucosal scraping samples for cytologic examination are necessary. Esophageal manometry is usually not performed, but characteristically shows the absence of peristalsis, increased pressure of the LES, and incomplete relaxation of the sphincter during swallowing.
Achalasia of the cardia is differentiated from carcinoma stenotic to the distal esophagus and peptic stricture, particularly in patients with scleroderma, in whom manometry may also reveal esophageal aperistalsis. Systemic sclerosis is usually accompanied by a history of Raynaud's phenomenon and features of gastroesophageal reflux disease (GERD).
Achalasia of the cardia due to cancer of the esophagogastric junction can be diagnosed by chest CT, abdominal CT, or endoscopic ultrasonography.
Diagnosis of achalasia of the cardia
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Treatment of achalasia cardia
There is no therapy to restore peristalsis; treatment is aimed at reducing the pressure (and thus the obstruction) of the LES. Pneumatic balloon dilation of the LES is usually indicated. Satisfactory results are seen in approximately 85% of patients, but repeated dilations are often necessary. Esophageal rupture and secondary mediastinitis requiring surgical treatment occur in <2% of patients. Nitrates (eg, isosorbide dinitrate 5–10 mg sublingually before meals) or calcium channel blockers (eg, nifedipine 10 mg orally 3 times daily) have limited efficacy but may reduce LES pressure sufficiently to prolong the recovery period between dilations.
Chemical denervation of the cholinergic nerves of the distal esophagus by direct injection of botulinum toxin into the LES can be used in the treatment of achalasia cardia. Clinical improvement occurs in 70-80% of patients, but the results may last from 6 months to a year.
Heller myotomy, which involves cutting the muscle fibers of the LES, is usually used in patients in whom dilation is ineffective; the success rate is approximately 85%. The procedure can be performed laparoscopically or thoracoscopically and may be a definite alternative to dilation in primary therapy. Symptomatic GERD develops in approximately 15% of patients after surgery.
What is the prognosis for achalasia cardia?
With timely treatment, achalasia of the cardia has a favorable prognosis for life, despite the fact that the disease is fundamentally incurable. With the help of therapeutic measures, symptomatic improvement is usually achieved, but lifelong observation in a specialized hospital is necessary. With pneumocardiodilation or cardiomyotomy, remission lasts longer than with the use of botulinum toxin.
Pulmonary aspiration and the presence of cancer are strong prognostic factors. Nocturnal regurgitation and cough suggest aspiration. Secondary pulmonary complications from aspiration are difficult to treat. The number of patients with esophageal cancer and achalasia may be increasing; however, this view is controversial.