Approach Considerations
Usually, the workup and treatment are performed in an outpatient setting. Patients in whom medical management fails and who require operative intervention should have routine postoperative care based on the procedure performed.
Calcium channel blockers and nitrates are first-line therapy.
Medical Care
Sildenafil, a phosphodiesterase inhibitor, is a smooth muscle relaxant that can lower the LES pressure and spastic contractions of the esophagus in nutcracker esophagus.
Botulinum toxin binds receptors in the nerve endings, thereby decreasing the release of acetylcholine. By endoscopically injecting botulinum toxin above the LES, symptoms may improve. [22] The effect is temporary, and the response decreases with repeated injections.
Balloon dilatation is commonly used for achalasia, but it has been also used to treat diffuse esophageal spasm and nutcracker esophagus. [23] The studies are small, the relief is not uniform, and symptoms recur. Dilation with mercury-filled bougies has been used in the past. [24]
Proton pump inhibitors effectively reduce or alleviate the symptoms of gastroesophageal reflux disease, which may mimic diffuse esophageal spasm. [25] A trial of acid-lowering therapy may be undertaken prior to instituting other treatments. Although treatment is often ineffective, the symptoms from diffuse esophageal spasm and nutcracker esophagus usually improve over time.
Tricyclic antidepressants have produced much success in the treatment of many patients with esophageal motility disorders. Some of these patients may have associated psychiatric illnesses, and concomitant treatment of those conditions may improve outcomes.
Diet
Diet-induced symptoms are patient-specific. Dietary restriction, even to pureed foods, can decrease symptoms temporarily.
Outpatient monitoring
Patients need close follow-up care upon the initiation of therapy or with a change in therapy. Patients should be monitored for improvements in symptoms and for adverse effects of the medications.
Patients in whom medical management fails should be referred to a thoracic surgeon for possible operative intervention.
Surgical Care
For extreme cases, operative treatment usually involves a myotomy. Myotomy relieves symptoms by eliminating the effectiveness of the contractions. Traditionally, a thoracotomy was required to obtain access to the esophagus, but now, a thoracoscopic approach can be used. In rare, recalcitrant cases, esophagectomy can relieve symptoms.
Myotomy is effective for treating diffuse esophageal spasm. [26] The myotomy should extend the entire length of the involved segment, which should be determined preoperatively with manometry. Furthermore, the myotomy should extend through the lower esophageal sphincter (LES) to help prevent dysphagia postoperatively by preventing outlet obstruction. Finally, an antireflux procedure should be performed concomitantly, by either a partial wrap or a Nissen fundoplication.
Myotomy should be used with caution in patients with nutcracker esophagus because it may worsen the symptoms. Myotomy reduces the amplitude of the contractions, but this does not consistently improve symptoms, especially if the primary complaint is pain. Furthermore, dysphagia can develop or worsen after myotomy because the effectiveness of the propagative waves is eliminated, leaving gravity to propel food caudally.
As a last resort, esophagectomy can be used to relieve symptoms. The esophagus is resected, and the stomach, small intestine, or colon is used to restore the continuity of the GI tract. Morbidity and mortality of esophagectomy are substantial; therefore, this procedure should be performed only after other treatments have been exhausted.
Overall, surgical therapy is reserved for those refractory to medical therapy. Filicori et al concluded that chest pain and dysphagia can be effectively palliated with per oral endoscopic myotomy (POEM) in patients with non-achalasia disorders of the esophagus. [27]
A thoracoscopy with a long myotomy from the arch of the aorta across the LES with an antireflux surgery is a commonly performed procedure in this setting.
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Barium swallow demonstrates diffuse uncoordinated contractions of the esophagus in a patient with diffuse esophageal spasm.
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This is normal esophageal manometry tracing with normal amplitude of the contractions. The contractions are coordinated because the contractions in the proximal esophagus (top of image) occur before the contractions further distal in the esophagus.
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Esophageal manometry tracing demonstrates diffuse esophageal spasm. Note the multiple uncoordinated contractions in the third tracing from the distal esophagus.
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Esophageal manometry tracing demonstrates nutcracker esophagus. Note the excessive amplitude of the contractions.
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High-resolution manometric display of a normal esophageal swallow using esophageal pressure topography. Image courtesy of Wikimedia Commons.
