What is the initial treatment for diffuse esophageal spasm?

Initial Treatment for Diffuse Esophageal Spasm

Start with proton pump inhibitors (PPIs) as first-line pharmacological therapy, particularly when symptoms overlap with gastroesophageal reflux disease (GERD), followed by smooth muscle relaxants such as calcium channel antagonists or nitrates if symptoms persist. 1

Diagnostic Workup Before Treatment

Before initiating therapy, confirm the diagnosis and rule out alternative pathology:

• Perform high-resolution manometry (HRM) to accurately diagnose and classify the motility disorder, as this is essential for distinguishing diffuse esophageal spasm from other conditions like achalasia or type III achalasia with spasm. 1
• Conduct upper GI endoscopy to exclude structural abnormalities and obtain biopsies if eosinophilic esophagitis is suspected, as this condition can mimic esophageal spasm but requires entirely different management. 1
• Consider ambulatory impedance-pH monitoring to rule out GERD, since failure to identify and treat concurrent reflux disease may lead to persistent symptoms despite appropriate spasm-directed therapy. 1

First-Line Pharmacological Management

Initial Medical Therapy

• Begin with PPIs as the initial therapeutic approach, especially when there is symptom overlap with GERD, which is common in esophageal spasm patients. 1
• If PPIs alone are insufficient, add smooth muscle relaxants including calcium channel antagonists (such as diltiazem or nifedipine) or nitrates (such as isosorbide dinitrate). 1, 2
  • These agents reduce esophageal contractile force and can provide symptomatic relief, though clinical benefit may be modest compared to manometric improvements. 2
  • One study showed only 1 of 9 patients improved with isosorbide dinitrate alone. 3

Neuromodulator Therapy

• Consider neuromodulators, particularly serotonin reuptake inhibitor antidepressants, as they can be highly effective for diffuse esophageal spasm. 1, 3
  • In one study, 8 of 9 patients (89%) improved with antidepressant therapy after failing nitrate treatment. 3
  • This approach addresses the psychosomatic component often present in esophageal spasm patients, who frequently have elevated anxiety and depression scores. 3
• Avoid metoclopramide as monotherapy or adjunctive therapy, as it is ineffective and potential harms outweigh benefits. 1

Second-Line Interventions

Endoscopic Botulinum Toxin Injection

• Botulinum toxin (BTX) injection is an effective treatment option when pharmacological therapy fails, with 78-89% of patients showing immediate symptom improvement. 1, 4
  • Inject 100 IU BTX diluted in 10 mL saline at multiple sites along the esophageal wall, beginning at the lower esophageal sphincter and moving proximally in 1- to 1.5-cm intervals. 4
  • Symptom relief typically lasts 6-12 months, and repeat injections are effective for recurrent symptoms. 4
  • This approach is particularly valuable for patients who are not surgical candidates. 4

Esophageal Dilation

• Pneumatic esophageal dilation can be considered for patients with associated strictures or when other therapies fail, though this is more commonly used in achalasia. 1, 5
  • One case report demonstrated successful management of DES in a 91-year-old high-risk patient using pneumatic dilation when pharmacological therapy failed. 5

Refractory Cases

Advanced Endoscopic and Surgical Options

• Per-oral endoscopic myotomy (POEM) is the preferred treatment for refractory distal esophageal spasm that fails medical and endoscopic therapies. 1
  • POEM should only be performed by experienced physicians in high-volume centers, as 20-40 procedures are needed to achieve competence. 1
  • Important caveat: There is a high risk (20-40%) of post-POEM reflux esophagitis requiring indefinite PPI therapy and/or surveillance endoscopy. 1

Critical Pitfalls to Avoid

• Do not overlook concurrent GERD, as this is a common cause of persistent symptoms despite appropriate spasm-directed therapy. 1
• Rule out eosinophilic esophagitis, which presents with similar symptoms but requires fundamentally different management with topical steroids and dietary elimination. 1
• Recognize the psychological component: Anxiety and depression are substantially elevated in DES patients, and addressing these factors with neuromodulators can be more effective than smooth muscle relaxants alone. 3
• Understand that "diffuse" esophageal spasm is actually "distal" esophageal spasm, as simultaneous contractions occur primarily in the smooth muscle portion of the distal esophagus (37% of swallows) rather than diffusely throughout the esophagus (only 2% in proximal esophagus). 6