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  • • Dysphagia, substernal pain

    • Nervousness, intermittent symptoms

    • Fluoroscopic, cineradiographic, and manometric evidence of high-amplitude contractions

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Epidemiology

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  • • Characterized by nonperistaltic esophageal contractions, often associated with intermittent chest pain

    • The cause is not known, although stress can induce similar manometric findings in normal subjects, so stress and psychological disorders might play a role

    • The esophagogram is abnormal in 60% of patients

    • Sliding hiatal hernia and epiphrenic diverticula may be secondary complications of the uncoordinated and severe contractions of the esophagus

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Symptoms and Signs

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  • • Intermittent chest pain, which varies from slight discomfort to severe spasmodic pain that simulates the pain of coronary artery disease

    • Dysphagia for liquids and solids

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Imaging Findings

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  • Fluoroscopic studies

    • -Segmental spasms

      -Areas of narrowing

      -Irregular uncoordinated peristalsis

      -A small hiatal hernia or epiphrenic diverticulum may be present

    Manometry

    • -Repetitive, nonperistaltic (simultaneous) contractions after swallowing

      -The lower esophageal sphincter may fail to relax in response to swallows

      -Provocative testing during manometry is positive if the anticholinesterase edrophonium, which causes strong esophageal contractions, elicits pain similar to the spontaneous symptom

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  • • The diagnosis is made by the characteristic findings on manometry and by eliciting the symptoms with provocative tests

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Rule Out

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  • • Heart disease, particularly coronary ischemia

    • Mediastinal masses

    • Benign and malignant esophageal tumors

    • Scleroderma

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  • • Symptoms of dysphagia and intermittent substernal pain

    • Manometry and provocative testing

    • Esophagoscopy to confirm the absence of intraluminal lesions

    • 24-hour pH monitoring may demonstrate reflux, a common associated problem

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When to Admit

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  • • Severe symptoms prohibiting adequate enteral nutrition

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  • • Reassurance and symptomatic therapy are often sufficient

    • A soft diet taken in 5 or 6 small feedings per day may be required

    • Esophageal dilation is ineffective

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Surgery

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Indications

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  • • Esophageal myotomy for symptoms refractory to medical therapy

    • Esophagectomy for persistent symptoms after myotomy

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Medications

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  • • Hydralazine

    • Calcium channel blockers

    • Long-acting nitrates

    • Anticholinergic agents

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Complications

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  • • Sliding hiatal hernia

    • Epiphrenic diverticula

    • Regurgitation

    • Aspiration

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Prognosis

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  • • Esophageal myotomy is successful in 90% of cases

    • Postoperative relief is associated with reduced intraluminal pressure

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References

Storr M, Allescher HD. Esophageal pharmacology and treatment of primary motility disorders. Dis Esophagus. 1999;12:241.  [PubMed: 10770358]

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