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)

The numerous causes which may give rise to DES call for caution in the diagnosis of an idiopathic disorder

H.J. Stein, T.R. DeMeester, R.R. Klingman (Omaha)

Since its first description in 1889 by Osgood, diffuse esophageal spasm has been thought to be the classic esophageal motor abnormality causing dysphagia and non-cardiac chest pain[l]. The diagnosis of diffuse esophageal spasm is usually based on the findings of an increased frequency of simultaneous, repetitive, and spontaneous contractions on standard manometric evaluation of the esophagus [2]. Currently, there is considerable confusion concerning the required frequency and significance of these manometric abnormalities to establish the diagnosis.

In our laboratory, a criterion of 20 p.cent or more simultaneous contractions following ten wet swallows is considered diagnostic if at least one sequence of normal peristalsis is observed (figure I). Associated manometric findings may include contractions of high amplitude or long duration and a high frequency of spontaneous contractions, but these findings are not necessary for the diagnosis.


Figure 1. Computer generated plot of the incidence of simultaneous contractions at the various levels of the esophageal body in a patient with diffuse esophageal spasm.

True idiopathic esophageal spasm is, however, a rare condition and manometric abnormalities similar to those of diffuse esophageal spasm can also occur in patients with obstructive esophageal lesions, endocrine and neuromuscular disorders, chronic alcoholism, presbyesophagus, and most frequently secondary to gastroesophageal reflux [3]. Making the diagnosis of idiopathic diffuse esophageal spasm is complicated further by the finding that even normal asymptomatic volunteers can have an occasional simultaneous contraction following a swallow when studied under the unphysiologic conditions of a manometric study in the esophageal laboratory. Therapeutic success is based on the principle that accurate diagnosis precedes therapy.

Consequently, the inclusion of individuals with esophageal motor abnormalities secondary to other underlying conditions will lead to less than satisfactory results of pharmacologic or surgical treatment of primary diffuse esophageal spasm [4]. The disappointing results reported could be explained by this inaccuracy in current diagnostic techniques and the lack of a generally accepted definition of the disease.

We have recently applied the new technology of 24-hour ambulatory esophageal motility monitoring to identify an esophageal motility pattern that would indicate the presence of idiopathic diffuse esophageal spasm. Discriminate analysis showed that a composite score, based on ten of 144 analyzed parameters of the circadian esophageal motor profile, could completely differentiate patients with primary diffuse esophageal spasm from asymptomatic volunteers (table I) [5].

Table 1. Selected parameters used in discriminant analysis





Stand, coeff.

Unstand. coeff.


1. area under the curve (channel 1)





2. w/e non-peristaltic





3. ratio peristaltic/ non-peristaltic


- 0.308



4. w/e double peaked





5. w/e triple peaked



- 0.323


6. w/e above 180 mm Hg





7. w/e above 7 seconds



- 2.524



8. w/e non-peristaltic





9. ratio peristaltic/ non-peristaltic



- 0.674

- 0.53

10. w/e above 180 mm Hg





w/e : waves per event (128 seconds)

F-Ratio : Univariate F-Ratio of the Wilks procedure.

Corr. equation : Correlation coefficient to the prediction equation (motility score)

Stand, coeff. : Standardized canonical discriminant function coefficient

Unstand. coeff. : Unstandardized canonical discriminant function coefficient (to be multiplied with the

individual patient's data)

When applied prospectively, the score obtained on the basis of the 24-hour ambulatory motor pattern showed a specificity and a positive predictive value to diagnose the presence of idiopathic diffuse esophageal spasm of 100 p. cent. Subsequent

application of this technique in 29 symptomatic patients with non-specific abnormalities on standard manometry showed that in 52 p. cent of these patients, the marker of diffuse esophageal spasm was present (figure 2). When ambulatory esophageal motility monitoring is performed simultaneously with 24-hour esophageal pH monitoring, gastroesophageal reflux disease as the cause of the esophageal motor abnormality can be excluded [6].

These data suggest that combined ambulatory 24-hour esophageal motility and pH monitoring is superior to standard manometry in the diagnosis of primary esophageal motor disorders. We believe that this new technique will improve identification of patients who will benefit from pharmacologic or surgical therapy of primary diffuse esophageal spasm.


Figure 2. Development, validation, and application of a scoring system based on 24-hour ambulatory motility monitoring to identify patients with the manometric markers of primary diffuse esophageal spasm. Sixty-nine percent of patients with hypercontracting esophagus and 38 percent with nonspecific motility abnormalities on standard manometry were reclassified as diffuse spasm after 24-hour ambulatory motility test. DES : diffuse esophageal spasm Hyp. contr. : hypercontracting esophagus Non-spec. : non-specific esophageal motility disorder


1. Osgood H (1889) A peculiar form of oesophagismas. Boston Medical and Surgical Journal 120: 401-405.

2. Castell DO, Richter JE, Dalton CB (Eds) (1987) Esophageal Motility Testing. Elsevier : New York.

3. Bennett JR. Hendrix TR (1970) Diffuse esophageal spasm: A disorder with more than one cause. Gastroenterology 59 : 273.

4. DeMeester TR (1982) Surgery for esophageal motor disorders. Ann Thorac Surg 34 : 225-229.

5. Eypasch EP, Stein HJ, DeMeester TR. Ambulatory 24-hour esophageal motility monitoring : A new technique to define and clarify esophageal motor disorders. Am J Surg (In press).

6. Stein HJ, Eypasch EP, DeMeester TR et al (1990) Circadian esophageal motor function in patients with gastroesophageal reflux disease. Surgery 108 : 769-778.

Publication date: May 1991 OESO©2015