Definitions^[1][2]

Based on manometry:

• Diffuse oesophageal spasm (DES) is defined by the presence of at least 20% of swallows showing simultaneous contractions in the distal oesophagus.
• Nutcracker oesophagus (NE) is defined as normal peristalsis of the oesophageal body with an average distal oesophageal amplitude exceeding 180 mm Hg.

In DES, most simultaneous contractions are present only in the distal oesophagus; therefore, some authors have proposed renaming this condition 'distal oesophageal spasm'.

Aetiology

The precise cause is unknown. Possible factors involved are: a defect in the nitric oxide pathway; abnormalities in the nervous system; visceral hypersensitivity; gastro-oesophageal reflux and smooth muscle thickening in the oesophageal wall.^[3]

Diffuse oesophageal spasm (DES) has been reported to be found in conjunction with other disorders, including anxiety and depression, diabetes mellitus, alcoholic neuropathies, pseudo-obstruction, amyloidosis and scleroderma.^[2]

Epidemiology

• The prevalence is unknown.^[3]
• Diffuse oesophageal spasm (DES) can can occur at any age, but is most most often found in patients aged over 50 years. It is found in about 3-10% of patients undergoing oesophageal manometry.^[2]

Presentation

May be diagnosed following investigation of non-cardiac chest pain or dysphagia.

Symptoms:^[2]

• Chest pain:^[4]
  • Because the heart and oesophagus are in such close proximity, distinguishing oesophageal pain from cardiac pain can be difficult and oesophageal spasm is often initially diagnosed as angina pectoris. Oesophageal spasm can cause episodes of severe, crushing, retrosternal pain.
  • Oesophageal pain may be gripping, boring, pressing or stabbing. It is usually felt in the anterior chest, throat or epigastrium and can radiate to the neck, back or upper arms, as with cardiac chest pain.
• Dysphagia
• Reflux-related symptoms, eg heartburn, regurgitation, cough and hoarseness.

Differential diagnosis

• Myocardial ischaemia or infarction
• Gastro-oesophageal reflux
• Oesophageal web, ring or stricture
• Other oesophageal motility disorders, eg achalasia
• Oesophageal perforation/mediastinitis (acutely)
• Oesophageal carcinoma
• Other causes of chest pain

Investigations

Note: remember that oesophageal and cardiac problems can coexist and diagnosis of one does not exclude the other. Also, any tests create anxiety and can lead to medical dependence.^[4]

• Oesophageal manometry:
  • Diagnosis of oesophageal spasms is made on oesophageal manometry - this is the preferred investigation.^[1]
  • 24-hour ambulatory manometry may be more useful than standard, laboratory-based manometry.^[5]
  • For examples of oesophageal manometry traces demonstrating diffuse oesophageal spasm (DES) and nutcracker oesophagus (NE), see the emedicine link under Document references, below.
• Oesophageal pH studies:
  • Allow assessment of concurrent gastro-oesophageal reflux disease.
• Oesophageal provocation tests
  • Edrophonium injected during oesophageal manometry can provoke abnormal contractions.^[4] Limitations are that patients may anticipate symptoms and that it can produce contractions in a normal oesophagus.
• Barium swallow:
  • This may suggest oesophageal spasm; for example, from the appearance of a 'corkscrew oesophagus' or 'rosary bead' appearance. However, these findings are not specific or sensitive for diagnosing oesophageal spasm.^[1]
  
  
  
  
  Barium swallow showing oesophageal spasm
  
  
  
• Ultrasound:
  • High-frequency intraluminal ultrasound can assess the sensory and motor function of the oesophagus and help to differentiate DES from NE.^[6]
• Cardiac investigations:
  • Are often indicated (or will have already been done) to rule out cardiac causes of chest pain.

Management^[1][3]

There is a lack of both evidence and controlled trials in this area.

Nondrug treatment

• Reassurance that this is not heart disease and that no significant progression occurs.
• Dietary modification.
• Avoiding cold fluids, and taking hot liquids with meals, helps some patients.

Drug treatment

• Rule out gastro-oesophageal reflux (trial of proton pump inhibitor) - this has been suggested as a first step by some authors.
• Subsequent options are:
  • Nitrates.
  • Calcium-channel blockers, eg nifedipine or diltiazem.
  • Antidepressants, eg trazodone, imipramine or sertraline - these may act as 'visceral analgesics'.
  • Phosphodiesterase inhibitors (sildenafil, etc.) - gave symptom relief in one small study.
  • Peppermint oil - improved manometric findings in one small study.
  • Theophylline - improved non-cardiac chest pain in one trial, and may relax the oesophageal wall.

Invasive or surgical treatment

• Botulinum toxin injection:
  • This involves the injection of Botox® at the gastro-oesophageal junction ± at several levels in the oesophagus.
  • In two studies of this treatment, the authors reported favourable results, but controls were lacking. Repeated injections may be needed.
• Surgical treatment - this is considered as rather drastic by some authors.^[1] Options are:
  • Oesophageal dilatation.
  • Oesophageal myotomy - published results suggest that this option could be considered in patients with symptomatic DES. However, these results are from highly specialised centres.^[1] Surgery seems less effective for NE.^[7]

Prognosis^[3]

The prognosis is probably good, in that the (limited) evidence so far suggests that most patients have an improvement in symptoms over time, and that diffuse oesophageal spasm (DES) and nutcracker oesophagus (NE) are unlikely to progress to the more severe condition of achalasia.

However, the evidence suggests that oesophageal spasm is difficult to treat; only a minority of patients found that a specific treatment was helpful.

Areas of debate

• The relationship between manometric findings (diffuse oesophageal spasm (DES) or nutcracker oesophagus (NE)) and symptoms (chest pain and/or dysphagia) has been questioned.^[2]
• It is unclear whether DES and NE are specific and separate diagnoses, or whether they are part of a continuous spectrum of oesophageal motility disorders.^[2]
• The current definition of DES has been challenged and alternative criteria proposed.^[5]