Distal esophageal spasm in high-resolution esophageal pressure topography: defining clinical phenotypes

The manometric diagnosis of distal esophageal spasm (DES) uses "simultaneous contractions" as a defining criterion, ignoring the concept of short latency distal contractions as an important feature. Our aim was to apply standardized metrics of contraction velocity and latency to high-resolution esophageal pressure topography (EPT) studies to refine the diagnosis of DES.

Two thousand consecutive EPT studies were analyzed for contractile front velocity (CFV) and distal latency to identify patients potentially having DES. Normal limits for CFV and distal latency were established from 75 control subjects. Clinical data of patients with reduced distal latency and/or rapid CFV were reviewed.

Of 1070 evaluable patients, 91 (8.5%) had a high CFV and/or low distal latency. Patients with only rapid contractions (n = 186 [17.4%] using conventional manometry criteria; n = 85 [7.9%] using EPT criteria) were heterogeneous in diagnosis and symptoms, with the majority ultimately categorized as weak peristalsis or normal. In contrast, 96% of patients with premature contraction had dysphagia, and all (n = 24; 2.2% overall) were ultimately managed as spastic achalasia or DES.

The current DES diagnostic paradigm focused on "simultaneous contractions" identifies a large heterogeneous set of patients, most of whom do not have a clinical syndrome suggestive of esophageal spasm. Incorporating distal latency into the diagnostic algorithm of EPT studies improves upon this by isolating disorders of homogeneous pathophysiology: DES with short latency and spastic achalasia. We hypothesize that prioritizing measurement of distal latency will refine the management of these disorders, recognizing that outcomes trials are necessary.