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)

What is the incidence of associated hiatus hernia or gastroesophageal reflux ?

A. G. Little (Las Vegas)

We must first acknowledge the controversy that exist in the definition of diffuse esophageal spasm (DES) [1]. Whether one defines DES as a motility disorder characterized by high pressure and prolonged duration esophageal body contractions or, as is most commonly accepted today, as a motility disorder characterized by frequent simultaneous contractions, conclusions can be made regarding the incidence of hiatus hernia and gastroesophageal reflux (GER) in these patients.

GER itself can produce abnormalities of esophageal motility. Although described as nonspecific abnormalities which can be identified both radiologically and manometrically, these abnormal motility patterns usually include the presence of simultaneous contractions during manometric analysis and tertiary or simultaneous contractions radiologically [2]. These patients, however, have the standard symptoms of GER with heartburn as the primary component. Dysphagia, in the absence of a reflux structure, is usually absent and is rarely a clinically significant complaint. As is well known, most patients with symptomatic GER will also have a radio-logically demonstrable hiatal hernia.

In contrast, patients with DES have a symptom complex which is predominated by a crushing type of chest pain which is quite different in nature from heartburn. If anything, the difficulty in these patients is distinguishing the esophageal pain from myocardial pain, rather than from the heartburn of GER.

One very well carried out clinical investigation identified 31 patients with manometric evidence of DES, i.e., frequent simultaneous contractions [3]. In seven of these patients, the symptoms were vague and esophagograms were normal, leaving the etiology unclear. In eleven patients the symptoms, manometric findings and esophagograms were compatible with DES. In a separate group of eleven patients, however, there were varied radiologic changes which were not characteristic

of spasm and nine of these 11 patients had a radiologically demonstrable hiatus hernia and radiologic reflux.

These patients did not have a crushing or squeezing type of substernal pain but had heartburn as their major complaint. In contrast, patients who have symptomatic, manometric and radiologic findings of classic DES do not have evidence of either a radiographic hiatus hernia or of gastroesophageal reflux. Few of these patients have actually been definitively studied with pH monitoring of the esophagus but a combination of clinical and radiologic investigations, which fail to identify GER in DES patients, supports the contention that DES is a primary motor disorder which is not associated with incompetence of the lower sphincter and reflux [1,3, 4]. In fact, manometrically the lower esophageal sphincter in these patients is typically normal.

In summary, although occasional patients with disordered motility secondary to GER can mimic manometrically the findings associated with DES, a distinction can be made on the basis of symptoms and radiology. Patients with DES represent a primary motor disorder which is not associated with either an anatomic hiatus hernia or GER.


1. Richter JE, Castell DO (1984) Diffuse esophageal spasm: a reappraisal. Ann Int Med 100: 242-245.

2. Henderson RD, Mugashe FL, Jeejeebhoy KN et al (1974) The motor defect of esophagitis. Can J Surg 17: 112-116.

3. Gonzalez G (1973) Diffuse esophageal spasm. Am J Roentgenol Radium Ther Nucl Med 117 : 251-258.

4. Roth HP, Fleshier B (1964) Diffuse esophageal spasm. Ann Int Med 61 : 914-923.

Publication date: May 1991 OESO©2015