Diffuse esophageal spasm (DES) is a rare motility disorder.
1 The term and first clinical
description is attributedn to Osgood, who in 1889 reported six patients suffering from sudden attacks of constriction in the epigastrium and dysphagia. 2 In 1934, Moersch and Camp were the first to describe the radiographic appearance based on observations in eight patients.3 Creamer et al. in 1958 reported the original manometric features as a pattern characterized by simultaneous contractions (SCs) and prolonged rise of pressure in the distal esophagus.4 In 1984, Richter and Castell, after studying 95 healthy controls5 and reviewing the existing literature at the time, confirmed that the manometric criteria of SCs in greater than 10% of wet swallows, but less than 100%, was consistent withDES and allowed differentiation from achalasia. 6 Most series published after the implementation of Richter and Castells paper comprise small number of patients; we found only four studies including more than 50 patients.710 Furthermore, these series have addressed selected aspects of the disease. For instance, Dalton et al. in 1991 reported that the prevalence of DES at a tertiary center was uncommon. Of the 1480 patients referred to esophageal motility, only 4% had DES. They also underscored that high-amplitude contractions were uncommon.7 Allen and DiMarino in 1996 evaluated 60 symptomatic patients according to their SC amplitude, concluding that a diagnosis of DES should not be made in those with distal contraction amplitude of less than 30 mm Hg.8 In 2003, Sperandio et al. found that the SCs that characterize DES are rarely seen in the proximal esophagus. In their study, only 3 out of 53 patients (5.6%) had SC in the proximal esophagus. As a result, they proposed that the term diffuse esophageal spasm was a misnomer, and instead it should be relabeled distal esophageal spasm.9 Tutuian et al. in 2006 focused on the esophageal bolus transit time using impedancemanometry in 71 patients. They noted that bolus transit for liquids and viscous swallows was abnormal in 25% of the patients and found higher distal esophageal amplitude (DEA) and higher percentage of complete bolus transit of liquid swallows in patients with chest pain compared with patients with dysphagia. 10 The said studies have shed light into selected aspects of DES. Yet several areas remain insufficiently understood such as: Are there specific gender or demographic features in DES? What are the clinical characteristics of these patients? What is the time from symptom onset to diagnosis? What is the contribution of radiology, endoscopy, or ambulatory pH testing to the diagnosis of these patients? The purpose of our study is to address these questions in a large sample of patients at a single tertiary center.