Distal Esophageal Spasm

Abstract

Distal esophageal spasm (DES) is an uncommon esophageal motility disorder associated with dysphagia and/or chest pain. Its pathophysiology implies an impairment of esophageal inhibitory neural function. Using conventional manometry, DES was defined by the presence of simultaneous esophageal contractions. With the introduction of high-resolution manometry and esophageal pressure topography (EPT) in clinical practice, rapidly propagated contractions are nonspecific of esophageal spasm. Hence, a more physiological and clinically relevant definition was proposed. Distal latency (DL) measures the period of inhibition that precedes contraction in the distal esophagus immediately proximal to the esophagogastric junction (EGJ). Premature contractions, defined as reduced DL, appeared to be much more specific for DES in EPT. Premature contractions with normal EGJ relaxation constitute DES, while premature contractions with impaired EGJ relaxation are diagnostic of spastic achalasia. Because of the interaction between DES and gastroesophageal reflux disease, 24-h esophageal pH monitoring should also be considered in patient evaluation. Medical treatment of DES aims to compensate for the deficient inhibitory neural function. Sildenafil, which blocks nitric oxide degradation and thus prolongs esophageal muscle relaxation, is a promising treatment. Endoscopic injection of botulinum toxin in the esophageal muscle is also an interesting therapeutic option. Finally, extended surgical myotomy might be discussed in extreme cases after failure of other therapeutic options.

Sildenafil improves clinical signs and radiographic features in dogs with congenital idiopathic megaoesophagus: a randomised controlled trial

“…Manometric studies have found a normal tone and functioning of the lower oesophageal sphincter (LOS) in dogs with idiopathic megaoesophagus (Diamant and others 1973), unlike in other oesophageal motility disorders in humans, such as achalasia or diffuse oesophageal spasm, where a hypertonicity of sphincter muscle is present (Pohl and Tutuian 2007, Roman and Kahrilas 2012). However, a failure by the LOS to relax in response to intraluminal balloon distension has been observed (Tan and Diamant 1987), further supporting the hypothesis of a functional defect of oesophageal sensory innervation.…”

Factors Determining the Inter-observer Variability and Diagnostic Accuracy of High-resolution Manometry for Esophageal Motility Disorders

“…This finding was based on spastic esophageal disorders, including achalasia (especially Type III achalasia), jackhammer esophagus and DES, sharing common pathophysiologic characteristics, such as loss of inhibitory ganglionic neuron function and excess cholinergic drive in the distal esophagus, resulting in simultaneous contraction or hypercontraction of the distal esophagus and incomplete deglutitive EGJ relaxation. 11,15,[18][19][20][21] Although the clinical significance remains under evaluation, this classification provided a theoretical basis for the importance of early diagnosis and an active management strategy, such as endoscopic or surgical treatment for major motility disorders such as achalasia, DES and jackhammer esophagus. [2][3][4]22,23 Nevertheless, few existing studies are available that have analyzed the reliability and diagnostic accuracy for distinguishing major esophageal motility disorders from minor motility disorders or a normal state or differential diagnosis among major disorders.…”

“…1,13 Second, although CFV was excluded from the key metrics for the diagnosis of motility disorders in the updated classification scheme, 1 a certain proportion of cases with clinical features suggestive of DES, such as typical symptoms and simultaneous contraction on barium esophagography, showed rapid contraction (CFV > 9 cm/sec) with normal DL (≥ 4.5 seconds). [14][15][16] Finally, in cases in which an automatically calculated DL by commercial software was not available, especially cases combined with bolus transport disturbances, the interpreters were obliged to calculate the DL by themselves. All these factors together may have contributed to a low level of inter-observer agreement for the diagnosis of DES with HRPT format images, especially among less experienced interpreters, in the present study.…”

Esophageal Hypomotility and Spastic Motor Disorders: Current Diagnosis and Treatment

“…These techniques have introduced new tools that have improved the identification of DES, and they include (1) contractile deceleration point (CDP), (2) distal latency (DL), and (3) contractile front velocity (CFV). CDP is "the inflection point along the 30 mmHg isobaric contour where propagation velocity slows, demarcating the tubular esophagus from the phrenic ampulla," DL is "the interval between upper esophageal sphincter (UES) relaxation and the CDP," and CFV is "the slope of the tangent approximating the 30 mmHg isobaric contour between the proximal pressure at the transition zone and the CDP" [2••, 18,19] (Fig. 1a).…”