Primary Motility  Disorders of the  Esophagus
 The Esophageal
 Esophagogastric  Junction

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Volume: Primary Motility Disorders of the Esophagus
Chapter: Diffuse esophageal spasms (Corkscrew esophagus)

How is their idiopathic nature to be confirmed ?

A.C. Duranceau, C. Deschamps, E. Lafontaine (Montreal)

Symptomatic idiopathic diffuse esophageal spasm (SIDES) is a syndrome in which dysphagia, odynophagia and chest pain are produced by repetitive high amplitude simultaneous contractions of long duration in the smooth muscle portion of the esophagus. This condition has been repetitively defined and redefined with symptoms, radiologic changes and manometric abnormalities showing only a variable and intermittent association [1].

How does one conclude on the « primary » character of SIDES. How is it proven idiopathic ? It is of paramount importance to obtain a detailed history in patients suspected of having diffuse spasm since it is these complaints that will lead to a stronger diagnosis. Correlation with radiologic and manometric findings must be obtained to confirm the clinical history : This correlation when it is obtained shows that primary idiopathic diffuse esophageal spasm is in fact a rare condition.

The typical patient is a man or more often a woman of any age, usually over 50, with a particularly anxious and high-strung personality. The symptoms appear and are worsened by emotional stress. These symptoms are mainly moderate to severe substernal pain occurring with swallows with anterior and mid scapular irradiation. Bilateral jaw irradiation often causes considerable confusion with pain of cardiac origin. The absence of pain on exercise and the association of pain with swallowing and dysphagia may help to distinguish pain of cardiac or esophageal origin. Simultaneous recording of ECG esophageal contractions and pH values in the esophagus may help clarify the etiology of non cardiac chest pain and rule out primary esophageal spasm.

With strict manometric assessment, 24-hours pH studies and cineradiologic evaluation followed by endoscopic evaluation, other conditions that can be responsible of significant chest pain and esophageal dysfunction can be ruled out.

Criteria for manometric diagnosis of hypermotility disorders were summarized recently by Khan and Castell [4]. Diffuse esophageal spasm has been more clearly defined. Related hypermotility disorders include the « nutcracker esophagus » where elevated mean peristaltic amplitude is found (> 180 mmHg) in the distal esophagus, accompagnied by increased contraction duration (> 6.0 sec) and normal propulsion. The hypertensive lower esophageal sphincter shows LES resting pressure of >45 mmHg with normal peristalsis. Since there is a strong association between neuro psychiatric disorders and abnormal manometry in patients with chest pain [3], patients with these abnormalities need to be assessed from all facets of esophageal function : symptoms, radiologic abnormalities, liquid and solid scintiscan emptying, endoscopic findings with histologic and cytologic documentation of disease, manometric and pH studies form the basis of a good esophageal assessment. Computation of all these investigation methods should lead to a precise and objective diagnosis in the vast majority of patients. Despite this, a number of conditions may not be classifiable as a distinct disorder. Whenever this is the case, investigation and therapy

should include psychiatric evaluation and psychotropic treatment of behavioral modification prior to offering an operative approach for the motor disorder [2, 5].


1. Castell DO (1976) Achalasia and diffuse esophageal spasm. Arch Int Med, 136: 571-579.

2. Castell DO, Richter JE, Dalton CB (1987): Esophageal motility Testing. Elsevier Medical.

3. Clouse RE, Lustman PK (1983) Psychiatric illess and contraction abnormalities of the esophagus. NEJM 309: 1337-1342.

4. Khan A A, Castell DO (1988) Gastroesophageal reflux. Clinical features in: Surgery of the Esophagus. Jamieson Ed. Churchill-Livingstone. Edinburg, Melbourne.

5. Richter JE, Castell DO (1987) Surgical myotomy for nutcracker esophagus, to be or not to be. Dig Dis Sci 32 : 95-96.

Publication date: May 1991 OESO©2015