Diffuse esophageal spasm

Changed by Mohammad Taghi Niknejad, 4 Dec 2023

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Disclosures - updated 12 Nov 2023: Nothing to disclose

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Diffuse/distal oesophageal spasm(DOS) is is a motility disorder of the oesophagus. On barium swallow, diffuse oesophageal spasm may appear as a corkscrew oesophagus, but this is uncommon. Manometry is the gold standard diagnostic test.

Diffuse oesophageal spasm differs from hypercontracting oesophagus (nutcracker oesophagus).

Epidemiology

Diffuse oesophageal spasm is an unusual cause of non-cardiac chest pain (2%) or or dysphagia (4%). It occurs most commonly in patients >50 years old but can occur at any age.

Clinical presentation

Chest pain and dysphagia are the primary complaints, but severity and occurrence are highly variable. Regurgitation may also be a feature (but less common than in achalasia) ⁵.

Pathology

Aetiology

Aetiology is unknown but may be related to loss of inhibitory neurones in the distal oesophagus.

Radiographic features

Fluoroscopy

only 60% of barium swallows will be abnormal
<5% will show "corkscrew oesophagus" or "rosary bead oesophagus" ~~where~~ where normal peristalsis is interrupted by many tertiary (non-propulsive) contractions occurring in the distal oesophagus
non-peristaltic contractions, pushing contrast in two directions, can be seen (sometimes the only feature) ⁵
sacculations and pseudodiverticula may be seen ⁵

Differential diagnosis

achalasia: diffuse oesophageal spasm may mimic achalasia in severe cases
non-specific oesophageal motility disorder
secondary oesophageal dysmotility from diabetic autonomic neuropathy

-<p><strong>Diffuse/distal oesophageal spasm</strong> (<strong>DOS</strong>) is a motility disorder of the <a href="/articles/oesophagus">oesophagus</a>. On <a href="/articles/barium-swallow">barium swallow</a>, diffuse oesophageal spasm may appear as a <a title="Corkscrew oesophagus" href="/articles/corkscrew-sign-diffuse-oesophageal-spasm">corkscrew oesophagus</a>, but this is uncommon. Manometry is the <a href="/articles/gold-standard">gold standard diagnostic test</a>.</p><p>Diffuse oesophageal spasm differs from <a href="/articles/hypercontracting-nutcracker-oesophagus">hypercontracting oesophagus (nutcracker oesophagus)</a>. </p><h4>Epidemiology</h4><p>Diffuse oesophageal spasm is an unusual cause of non-cardiac chest pain (2%) or <a href="/articles/dysphagia">dysphagia</a> (4%). It occurs most commonly in patients >50 years old but can occur at any age. </p><h4>Clinical presentation</h4><p>Chest pain and dysphagia are the primary complaints, but severity and occurrence are highly variable. Regurgitation may also be a feature (but less common than in <a href="/articles/achalasia">achalasia</a>) <sup>5</sup>.</p><h4>Pathology</h4><h5>Aetiology</h5><p>Aetiology is unknown but may be related to loss of inhibitory neurones in the distal oesophagus. </p><h4>Radiographic features</h4><h5>Fluoroscopy</h5><ul>
~~-<li>only 60% of barium swallows will be abnormal</li>~~
-<li><5% will show "<a title="Corkscrew oesophagus" href="/articles/corkscrew-oesophagus-3">corkscrew oesophagus</a>" or "rosary bead oesophagus" where normal peristalsis is interrupted by many tertiary (non-propulsive) contractions occurring in the distal oesophagus</li>
~~-<li>non-peristaltic contractions, pushing contrast in two directions, can be seen (sometimes the only feature) <sup>5</sup>~~
~~-</li>~~
~~-<li>sacculations and pseudodiverticula may be seen <sup>5</sup>~~
~~-</li>~~
~~-</ul><h4>Differential diagnosis</h4><ul>~~
~~-<li>~~
~~-<a href="/articles/achalasia">achalasia</a>: diffuse oesophageal spasm may mimic achalasia in severe cases</li>~~
~~-<li><a href="/articles/non-specific-oesophageal-motility-disorder">non-specific oesophageal motility disorder</a></li>~~
~~-<li>secondary oesophageal dysmotility from <a href="/articles/diabetic-autonomic-neuropathy">diabetic autonomic neuropathy</a>~~
~~-</li>~~
+<p><strong>Diffuse/distal oesophageal spasm</strong> (<strong>DOS</strong>) is a motility disorder of the <a href="/articles/oesophagus">oesophagus</a>. On <a href="/articles/barium-swallow">barium swallow</a>, diffuse oesophageal spasm may appear as a <a title="Corkscrew oesophagus" href="/articles/corkscrew-sign-diffuse-oesophageal-spasm">corkscrew oesophagus</a>, but this is uncommon. Manometry is the <a href="/articles/gold-standard">gold standard diagnostic test</a>.</p><p>Diffuse oesophageal spasm differs from <a href="/articles/hypercontracting-nutcracker-oesophagus">hypercontracting oesophagus (nutcracker oesophagus)</a>. </p><h4>Epidemiology</h4><p>Diffuse oesophageal spasm is an unusual cause of non-cardiac chest pain (2%) or <a href="/articles/dysphagia">dysphagia</a> (4%). It occurs most commonly in patients >50 years old but can occur at any age.  </p><h4>Clinical presentation</h4><p>Chest pain and dysphagia are the primary complaints, but severity and occurrence are highly variable. Regurgitation may also be a feature (but less common than in <a href="/articles/achalasia">achalasia</a>) <sup>5</sup>.</p><h4>Pathology</h4><h5>Aetiology</h5><p>Aetiology is unknown but may be related to loss of inhibitory neurones in the distal oesophagus. </p><h4>Radiographic features</h4><h5>Fluoroscopy</h5><ul>
+<li>only 60% of barium swallows will be abnormal</li>
+<li><5% will show "<a title="Corkscrew oesophagus" href="/articles/corkscrew-sign-diffuse-oesophageal-spasm">corkscrew oesophagus</a>" or "rosary bead oesophagus" where normal peristalsis is interrupted by many tertiary (non-propulsive) contractions occurring in the distal oesophagus</li>
+<li>non-peristaltic contractions, pushing contrast in two directions, can be seen (sometimes the only feature) <sup>5</sup>
+</li>
+<li>sacculations and pseudodiverticula may be seen <sup>5</sup>
+</li>
+</ul><h4>Differential diagnosis</h4><ul>
+<li>
+<a href="/articles/achalasia">achalasia</a>: diffuse oesophageal spasm may mimic achalasia in severe cases</li>
+<li><a href="/articles/non-specific-oesophageal-motility-disorder">non-specific oesophageal motility disorder</a></li>
+<li>secondary oesophageal dysmotility from <a href="/articles/diabetic-autonomic-neuropathy">diabetic autonomic neuropathy</a>
+</li>

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Image 9 Fluoroscopy (Frontal, lateral and oblique views) ( create )

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Case 7

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