Distal esophageal spasm: Update on diagnosis and management in the era of high-resolution manometry

Abstract

Distal esophageal spasm (DES) is a rare major motility disorder in the Chicago classification of esophageal motility disorders (CC). DES is diagnosed by finding of ≥ 20% premature contractions, with normal lower esophageal sphincter (LES) relaxation on high-resolution manometry (HRM) in the latest version of CCv3.0. This feature differentiates it from achalasia type 3, which has an elevated LES relaxation pressure. Like other spastic esophageal disorders, DES has been linked to conditions such as gastroesophageal reflux disease, psychiatric conditions, and narcotic use. In addition to HRM, ancillary tests such as endoscopy and barium esophagram can provide supplemental information to differentiate DES from other conditions. Functional lumen imaging probe (FLIP), a new cutting-edge diagnostic tool, is able to recognize abnormal LES dysfunction that can be missed by HRM and can further guide LES targeted treatment when esophagogastric junction outflow obstruction is diagnosed on FLIP. Medical treatment in DES mostly targets symptomatic relief and often fails. Botulinum toxin injection during endoscopy may provide a temporary therapy that wears off over time. Myotomy through peroral endoscopic myotomy or via surgical Heller myotomy can provide long term relief in cases with persistent symptoms.

Keywords: Distal esophageal spasm; High-resolution manometry; Esophagus; Functional lumenal imaging probe; Spastic achalasia; Esophageal motility

Core tip: Distal esophageal spasm (DES) is an esophageal motor disorder that is diagnosed using high-resolution manometry and is classified as a major motility disorder in the Chicago classification of esophageal motility disorder. While the criteria for diagnosis have been revised overtime to achieve a homogenous clinical entity, presentation of DES continues to be heterogenous. This has led to the usage of multiple pharmacological treatment options such as nitrates, phosphodiesterase inhibitors, calcium channel blockers, and tricyclic antidepressants, often resulting in poor symptom management. DES pathophysiology falls within the spectrum of spastic motility disorders, and it may represent a stage in the process of evolution to achalasia, more likely type 3. Patients who have continued symptoms despite medical management might benefit from endoscopic procedures such as botulinum injection and peroral endoscopic myotomy.