Diffuse esophageal spasm and nutcracker esophagus

INTRODUCTION

The clinical relevance of esophageal motility abnormalities such as diffuse esophageal spasm (DES) and the nutcracker esophagus is best understood when working from a classification system for these abnormalities. Prior to discussing the current classification system, it is helpful to first briefly review the history and controversy surrounding attempts to understand the pathophysiology of these disorders.

An esophageal motility disorder (EMD) is defined as motility that differs significantly from accepted normal variations. In the early years of esophageal motility testing, these "disorders" were primarily descriptions of motility findings in patients with otherwise unexplained symptoms, usually dysphagia or chest pain. As an example, William Osler first described esophageal spasms in 1892 in hypochondriacal patients with unexplained chest pain [1]. The older radiology literature also used terms such as "presbyesophagus," reflecting abnormal peristalsis that was thought to be age-related [2]. This term should not be seriously considered since there is scant evidence that any esophageal dysmotility occurs as a direct result of aging. More recently, classifications of esophageal motility abnormalities have been based more specifically upon motility findings that exceeded two standard deviations from those found in a large group of normal subjects [3].

There is considerable controversy concerning the clinical significance of abnormal esophageal motility patterns. The most important questions are whether they represent actual disorders or are only manometric abnormalities and whether they cause or explain the patient's symptoms. With the exception of achalasia, no specific pathology or pathophysiology has been described for any of these dysmotility patterns, including the more generally accepted "disorder" of DES.

This topic review will attempt to characterize the relevant information on these motility abnormalities without the implication that they are actual disease entities, excluding the one true motility disorder, achalasia. (See "Clinical manifestations and diagnosis of achalasia".) An overview of chest pain of esophageal origin is discussed separately. (See "Chest pain of esophageal origin".)

CLASSIFICATION OF ESOPHAGEAL MOTILITY ABNORMALITIES

Esophageal motility abnormalities are generally classified into those that are an isolated phenomenon (primary) (table 1) and those associated with generalized disease (secondary) (table 2). Within each of these major categories, the motility abnormalities can usually be subcategorized into hypercontracting, hypocontracting, and discoordinated motility.