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Achalasia

(Cardiospasm; Esophageal Aperistalsis; Megaesophagus)

by Michael C. DiMarino, MD

Achalasia is a neurogenic esophageal motility disorder characterized by impaired esophageal peristalsis, a lack of lower esophageal sphincter relaxation during swallowing, and an elevation of lower esophageal sphincter resting pressure. Symptoms are slowly progressive dysphagia, usually to both liquids and solids, and regurgitation of undigested food. Evaluation typically includes barium swallow, endoscopy, and sometimes manometry. Treatments include dilation, chemical denervation, and surgical myotomy.

(See also the American College of Gastroenterology’s practice guidelines on the diagnosis and management of achalasia.)

Achalasia is thought to be caused by a loss of ganglion cells in the myenteric plexus of the esophagus, resulting in denervation of esophageal muscle. Etiology of the denervation is unknown, but viral and autoimmune causes are suspected, and certain tumors may cause achalasia either by direct obstruction or as a paraneoplastic process. Chagas disease, which causes destruction of autonomic ganglia, may result in achalasia.

Increased pressure at the lower esophageal sphincter (LES) causes obstruction with secondary dilation of the esophagus. Esophageal retention of undigested food is common.

Symptoms and Signs

Achalasia occurs at any age but usually begins between ages 20 and 60. Onset is insidious, and progression is gradual over months or years. Dysphagia for both solids and liquids is the major symptom. Nocturnal regurgitation of undigested food occurs in about 33% of patients and may cause cough and pulmonary aspiration. Chest pain is less common but may occur on swallowing or spontaneously. Mild to moderate weight loss occurs; when weight loss is pronounced, particularly in elderly patients whose symptoms of dysphagia developed rapidly, achalasia secondary to a tumor of the gastroesophageal junction should be considered.

Diagnosis

  • Barium swallow

  • Esophageal manometry

The preferred test is barium swallow, which shows absence of progressive peristaltic contractions during swallowing. The esophagus is dilated, often enormously, but is narrowed and beaklike at the LES. If esophagoscopy is done, there is dilation but no obstructing lesion. The esophagoscope usually passes readily into the stomach; resistance raises the possibility of an inapparent cancer or stricture. To exclude cancer, a retroflexed view of the gastric cardia, biopsies, and brushings for cytology should be obtained. Esophageal manometry is usually done and typically shows aperistalsis, increased LES pressure, and incomplete sphincteric relaxation during swallowing.

Achalasia must be differentiated from a distal stenosing carcinoma and a peptic stricture, particularly in patients with systemic sclerosis (see Systemic Sclerosis), in whom esophageal manometry may also show aperistalsis. Systemic sclerosis is usually accompanied by a history of Raynaud phenomenon and symptoms of gastroesophageal reflux disease (GERD—see Gastroesophageal Reflux Disease (GERD)) due to low or absent LES pressure.

Achalasia due to cancer at the gastroesophageal junction can be diagnosed by CT of the chest and abdomen or by endoscopic ultrasound.

Prognosis

Pulmonary aspiration and the presence of cancer are the determining prognostic factors. Nocturnal regurgitation and coughing suggest aspiration. Pulmonary complications secondary to aspiration are difficult to manage. Incidence of esophageal cancer in patients with achalasia may be increased; this point is controversial.

Treatment

  • Balloon dilation of the LES

  • Botulinum toxin injection

  • Sometimes surgical myotomy

No therapy restores peristalsis; treatment aims at reducing the pressure (and thus the obstruction) at the LES. Pneumatic balloon dilation of the LES is indicated initially. Results are satisfactory in about 85% of patients, but repeated dilations may be needed. Esophageal rupture and secondary mediastinitis requiring surgical repair occur in < 2% of patients. Nitrates (eg, isosorbide dinitrate 5 to 10 mg sublingually before meals) or Ca channel blockers (eg, nifedipine 10 to 30 mg po 30 to 45 min before a meal) are of limited effectiveness but may reduce LES pressure enough to prolong the time between dilations.

Achalasia can also be treated by chemical denervation of cholinergic nerves in the distal esophagus by direct injection of botulinum toxin type A into the LES. Clinical improvement occurs in 70 to 80% of patients, but results may last only 6 mo to 1 yr.

A Heller myotomy, in which the muscular fibers in the LES are cut, is usually reserved for patients who do not respond to dilation; its success rate is about 85%. It can be done via laparoscopy or, less commonly, thoracoscopy and is an alternative to dilation as primary therapy. Symptomatic GERD occurs after surgery in about 15% to 30% of patients. Some specialists recommend doing fundoplication after myotomy, which reduces the incidence of GERD.

Key Points

  • A viral- or autoimmune-induced loss of ganglion cells in the myenteric plexus of the esophagus decreases esophageal peristalsis and impairs relaxation of the lower esophageal sphincter (LES).

  • Patients gradually develop dysphagia for both solids and liquids, and about one third regurgitate undigested food at night.

  • Barium swallow shows absence of progressive peristaltic contractions during swallowing and a markedly dilated esophagus with beaklike narrowing at the LES.

  • Esophageal manometry and often endoscopy are also usually done.

  • No therapy restores peristalsis; treatment aims to reduce the pressure (and thus the obstruction) at the LES.

  • Treatment is typically balloon dilation or laparoscopic myotomy of the LES.

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  • ADALAT CC, PROCARDIA

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