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  • • Dysphagia

    • Retention of ingested food in the esophagus

    • Radiologic evidence of absent primary peristalsis, dilated body of the esophagus, and a conically narrowed cardioesophageal junction

    • Absent primary peristalsis by manometry and cineradiography

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Epidemiology

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  • • Achalasia is a neuromuscular disorder; esophageal dilation and hypertrophy occur without organic stenosis

    • Primary peristalsis is absent and the cardioesophageal sphincter fails to relax in response to swallowing; the circular muscle layer hypertrophies

    • There is absence, atrophy, or disintegration of the ganglion cells of Auerbach myenteric plexuses and a reduction in nerve fibers within the wall of the esophagus

    • The cause is unknown, but 2 theories exist:

    • 1. A degenerative disease of the neurons

      2. Infection of neurons by a virus (eg, herpes zoster) or other infectious pathogen

    • Achalasia affects males more often than females and may develop at any age; peak incidence ranges from 30 to 60 years

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Symptoms and Signs

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  • • Dysphagia is dominant symptom

    • Weight loss is not usually marked despite the functional obstruction

    • Pain is infrequent

    • Regurgitation of retained esophageal contents is common, especially during the night while the patient sleeps in a recumbent position

    • A variant called vigorous achalasia is characterized by chest pain and esophageal spasms that generate nonpropulsive high-pressure waves in the body of the esophagus

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Imaging Findings

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  • Contrast radiography and endoscopy

    • -Narrowing at the cardia

      -The dilated body of the esophagus blends into a smooth cone-shaped area of narrowing 3-6 cm long

      -As the disease progresses, the esophagus dilates further and becomes tortuous

    Manometry

    • -The body of the esophagus is devoid of primary peristaltic waves, but simultaneous disorganized muscular activity may be present

      -Pressure in the gastroesophageal sphincter is increased; relaxation after swallowing is incomplete or absent

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  • • Symptoms should prompt contrast radiographic or endoscopic studies

    • Endoscopy is essential for establishing the diagnosis and excluding other causes of symptoms

    • Manometry is useful for confirming diagnosis

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Rule Out

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  • • Benign strictures of the lower esophagus

    • Carcinoma at or near the cardioesophageal junction

    • Diffuse esophageal spasm

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  • • Gastroesophageal contrast radiography

    • Upper GI endoscopy

    • Esophageal manometry

    • pH study, particularly if symptoms of reflux are present or fundoplication is planned

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  • • Goal is to relieve the functional obstruction either by pneumatic dilation or longitudinal division of all the esophageal muscular layers (Heller myotomy).

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Surgery

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Indications

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  • • Advanced disease

    • Failed dilation

    • If no reflux symptoms exist, the need to add an antireflux procedure has not been established

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Treatment Monitoring

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  • • Measure rate of esophageal passage of a technetium Tc 99m-labeled solid meal

    • Periodic esophagoscopy; treatment of achalasia does not lessen the increased risk of squamous cell carcinoma

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