What is the best management of diffuse esophageal spasm?

Management of Diffuse Esophageal Spasm

The best management of diffuse esophageal spasm begins with smooth muscle relaxants (calcium channel blockers or nitrates) as first-line pharmacotherapy, followed by endoscopic botulinum toxin injection for refractory cases, with per-oral endoscopic myotomy (POEM) reserved for severe, treatment-resistant patients. 1

Initial Diagnostic Confirmation

Before initiating treatment, confirm the diagnosis with high-resolution manometry (HRM), which is essential for accurate classification of esophageal motility disorders and to exclude achalasia (particularly type III achalasia, which can mimic diffuse esophageal spasm). 2, 1 Perform upper endoscopy to rule out structural abnormalities, strictures, and eosinophilic esophagitis, which can present with similar symptoms but require different management. 1 Consider ambulatory impedance-pH monitoring if gastroesophageal reflux disease (GERD) is suspected, as concurrent GERD is common and must be addressed. 1

First-Line Pharmacological Management

• Start with smooth muscle relaxants as initial therapy, including calcium channel blockers (diltiazem 60 mg three times daily) or nitrates, though evidence shows variable individual responses with some patients achieving substantial symptom relief while others show minimal benefit. 1, 3

• Initiate proton pump inhibitor (PPI) therapy if symptoms overlap with GERD, using standard once-daily dosing for 4-8 weeks, as GERD frequently coexists and untreated reflux will perpetuate symptoms despite appropriate spasm treatment. 1

• Consider neuromodulators such as tricyclic antidepressants for patients with associated esophageal hypervigilance or hypersensitivity, as these address the sensory component of symptoms. 1

• Baclofen may be effective for patients with predominant regurgitation or belching symptoms, though CNS and GI side effects limit its use. 1

Second-Line Endoscopic Intervention

For patients who fail pharmacological therapy after 8-12 weeks, endoscopic botulinum toxin injection is the best-studied and most effective non-surgical option. 1, 4 The technique involves injecting 100 IU of botulinum toxin 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. 5 This approach achieves symptom improvement in 78-89% of patients, with effects lasting 6-24 months before reinjection is needed. 5 Repeated injections remain effective for symptom relapse. 5

Esophageal dilation using balloon dilators or bougie dilators should be performed if strictures or narrowing are identified on endoscopy or barium studies, and can provide symptomatic relief even in the absence of obvious stricture in select elderly or high-risk patients who are not surgical candidates. 1, 6

Third-Line Surgical Management

Per-oral endoscopic myotomy (POEM) is the preferred surgical intervention for severe, refractory diffuse esophageal spasm, particularly when symptoms significantly impair quality of life despite maximal medical and endoscopic therapy. 1 POEM is especially effective for type III achalasia and select cases of distal esophageal spasm. 1 Traditional laparoscopic myotomy remains an option but is more invasive. 4

Adjunctive Behavioral Interventions

Provide cognitive behavioral therapy (CBT), esophageal-directed hypnotherapy, or diaphragmatic breathing exercises for patients with associated hypervigilance, reflux hypersensitivity, or anxiety-related symptom amplification. 1 These interventions address the psychological component that often perpetuates symptoms even after successful treatment of the motility disorder itself.

Critical Pitfalls to Avoid

• Do not use metoclopramide as it is ineffective for esophageal spasm and may cause harm. 1

• Do not miss concurrent eosinophilic esophagitis, which requires endoscopic biopsies and completely different treatment with topical corticosteroids. 1

• Do not proceed to invasive interventions without confirming the diagnosis with manometry and excluding achalasia, as treatment approaches differ fundamentally. 2, 1

• Do not ignore coexisting GERD, as failure to treat reflux will result in persistent symptoms regardless of spasm-directed therapy. 1

Treatment Algorithm Summary

1. Confirm diagnosis with HRM and exclude structural abnormalities with endoscopy 2, 1
2. Start smooth muscle relaxants (calcium channel blockers or nitrates) plus PPI if GERD coexists 1, 3
3. Add neuromodulators or behavioral therapy for hypervigilance/hypersensitivity 1
4. If no response after 8-12 weeks, proceed to botulinum toxin injection 1, 4, 5
5. Consider esophageal dilation if strictures present or in high-risk patients 1, 6
6. Reserve POEM for severe, refractory cases significantly impairing quality of life 1