Diffuse Esophageal Spasm-
Esophagus Nutcracker

Clinical picture

Diffuse esophageal spasm (DES) and oesophageal reflux disease (NE) are clinically characterized by recurrent chest pain and dysphagia. Chest pain can range from mild to overwhelming, extending to the back and jaw and lasting from seconds to minutes. Pain with DES does not always occur with swallowing. Reflux is rare. Dysphagia in patients with DES can be due to solids or liquids and often occurs more often with ingestion of either very cold or very hot foods. In both DES and NE the symptoms may be intermittent, appear or not appear with food and are usually not progressive.

Diagnosis

Manometry.

The classic abnormality observed during esophageal manometry in patients with DES is a pattern of abnormal concurrent contractions of the esophageal body. These must be present in more than 30% of swallows.

Other manometric diagnostic criteria for DES include frequent, repetitive contractions of the esophagus (= 3) wide (> 180 mm Hg) that are prolonged (> 6 seconds). LES may occasionally have high relaxation pressure but is usually normal, as is the upper esophageal sphincter.

The Esophageal Nutcracker is manometrically characterized by an average peripheral width of the esophageal peristalsis greater than 180 mm Hg. Other criteria for NE include repetitive contractions (> 2) that are prolonged (> 6 seconds). LES relaxes normally but has an increased relaxation pressure greater than 40 mm Hg.

Radiological findings.

The classic finding of DES, which is most often seen during the barium swallowing study, is the appearance of a "corkscrew" or "rosary" of the esophagus due to concomitant contractions. Primary (normal) peristalsis is often seen in the upper third of the esophagus and the so-called tertiary (abnormal) activity is observed in the area of the spasm. Tertiary activity may occasionally occur in normal individuals. Episodes of pain are not always associated with spastic contractions of the esophagus and patients may experience severe pain even when swallowing appears radiographically normal.

In NE, the findings from barium ingestion are often normal. Various non-specific forms of tertiary activity have been reported. However, NE is a manometric, not radiological, diagnosis.

Endoscopic findings.

The esophagus is usually normal in patients with DES and NE, but a thorough search for GERD (esophagitis, ulcers, stenosis, etc.) should be performed during gastroscopy. 

Treatment

General Instructions.

It is important to note that both DES and NE are usually non-progressive disorders that are not associated with more serious medical problems. Treatment should aim at symptomatic relief once heart disease is definitively ruled out through formal examinations. Patients may experience spontaneous improvement or resolution of symptoms in the absence of treatment.

The incidence of psychiatric disorders is increased in patients with spastic esophageal abnormalities. In particular, anxiety, depression and somatoform disorders are more common in this group of patients.

Pharmacology.

Muscle relaxants such as nitrates, calcium channel blockers, and even botulinum toxin have been used to treat DES and NE, with varying results. Although many small series and anecdotal reports have described a good clinical response to these factors, there are no long-term outcome studies confirming their effectiveness. Side effects (hypotension, headache, etc.) can be serious. Trazodone anxiolytic is the only factor that has been shown to improve the symptoms of esophageal spasticity in a prospective, blinded, controlled study. 

Because DES and NE may represent GOP events and GOP treatment has been shown to be beneficial. For these patients, unexplained chest pain has been shown to decrease, regardless of whether the patients had mobility abnormalities.  

Endoscopic Myotomy (POEM)

Oral endoscopic myotomy can be applied to treatment-resistant patients. Studies to date have shown encouraging results as the myotomy is performed not only at the level of the lower esophageal sphincter but also in the esophagus itself.

Surgery.

The traditional surgical approach to esophageal spastic disorders was esophageal myotomy. Myotomy performed either laparoscopically or thoracoscopically is mainly intended for patients with DES or NE in whom medical treatment has failed. There are several important points to keep in mind when considering surgical myotomy in patients with DES or NE. First, myotomy will only reduce the intensity of esophageal contractions, not the frequency. Thus, the symptoms may appear even after the surgery. Second, myotomy can lead to a stimulatory esophagus, which can lead to dysphagia (a symptom that surgery is intended to improve). Third, chest pain with DES and NE responds better to myotomy than the dysphagia associated with these diseases.