Diffuse esophageal spasm differential diagnosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Madhu Sigdel M.B.B.S.[2]

Overview

Diffuse esophageal spasm must be differentiated from other diseases that cause dysphagia, chest pain and weight loss such as angina, reflux esophagitis, esophageal carcinoma, systemic sclerosis, nutcracker esophagus, hypertensive LES, esophageal web/stricture, pseudoachalasia, stroke, esophageal candidiasis and Chagas disease etc.

Differential Diagnosis

Diffuse esophageal spasm must be differentiated from other diseases that cause dysphagia, chest pain and weight loss such as angina, reflux esophagitis, esophageal carcinoma, systemic sclerosis, nutcracker esophagus, hypertensive LES, esophageal web/stricture, pseudoachalasia, stroke, esophageal candidiasis and Chagas disease etc.

Disease	Signs & Symptoms	Findings on barium esophagogram	Findings on endoscopy	Other findings
Reflux esophagitis	Dysphagia (from peptic stricture) Heartburn Hoarseness	Poor clearance Free reflux of barium	Peptic stricture (advanced cases)	Lower esophageal pH studies will demonstrate pathologic gastroesophageal reflux A hiatus hernia may be present below the stricture Manometry shows decreased tone of lower esophageal sphincter
Angina	Chest pain related to exertion Dysphagia	Normal	Normal	ST segment elevation on ECG
Esophageal carcinoma	Dysphagia (initially for solids, liquids develops with advanced disease.) Weight loss Lymphadenopathy Cachexia	Irregular stricture Pre-stricture dilatation	Endoscopy with biopsy is the most accurate test for diagnosis and tumor histology. It may be used to depict: Esophageal obstruction Staging of disease	CT scan and PET scan of the chest and abdomen is an optional test for staging of the disease
Systemic sclerosis	Dysphagia Muscle and joint pain Raynaud's phenomenon Skin changes (rash, skin thickening)	Dysmotility Patulous esophagus	Mucosal damage Peptic stricture (advanced cases)	Positive serology for Antinuclear antibodies Rheumatoid factor Creatine kinase ESR
Pseudoachalasia	Dysphagia Weight loss Lymphadenopathy Appetite changes Cachexia Older patients Underlying malignancy that mimics idiopathic achalasia. Patients tend to be older, duration of symptoms shorter, and weight loss greater and more rapid.	More marked mucosal irregularity Temporary patency of LES	Endoscopy with biopsy is the most accurate test for diagnosis and tumor histology. It may be used to depict: Esophageal obstruction Staging of disease	Gastroscopic biopsy of gastroesophageal junction and cardia may demonstrate malignancy. Findings at endoscopy, barium swallow, and manometry may be indistinguishable from achalasia.
Chagas disease	Dysphagia Toxic megacolon Myocarditis Blepharitis	Esophageal dilatation Stasis of barium	Dilated esophagus Thickened LES (muscular ring)	Giemsa stain will show Trypanosoma cruzi. PCR may be done to determine trypanosome subtype
Pharyngitis	Dysphagia Fever Throat pain	Normal	Inconclusive	Rapid antigen detection test positive for group A streptococccus Tonsillar hypertrophy may cause severe narrowing of the pharynx Physical exam may show: Erythema, edema and/or exudates of the pharynx Lymphadenopathy
Esophageal candidiasis	Dysphagia Immunocompromised History of corticosteroid	Shaggy" appearance (plaques) Irregular contours in the lower third	Ulceration Plaques and pseudomembranes Tiny nodules, polypoid folds (advanced cases)	Creamy white or yellowish plaques (thrush) in oropharynx
Stroke	Progressive dysphagia Dysarthria Limb weakness Fatigue	Pooling of contrast in the pharynx Aspiration of barium contrast into the airway.	Reduced opening of upper esophageal sphincter Reduced larynx elevation	CT without contrast is the best initial test to differentiate between ischemic and hemorrhagic stroke MRI is more specific and sensitive than a CT scan but is more time consuming.

			Manifestations		Diagnostic tools
	Achalasia		Dyspnea Dysphagia for solids and liquids is the most common feature, being seen in 91 % and 85% of patients respectively Regurgitation of undigested food occurs in 76-91% of patients Cough mainly when lying down in 30%		Esophagogastroduodenoscopy findings include a dilated esophagus with residual food fragments, normal mucosa and occasionally candidiasis (due to the prolonged stasis). Barium swallow shows the characteristic bird's beak appearance. Barium swallow showing bird's beak appearance - By Farnoosh Farrokhi, Michael F. Vaezi. - Idiopathic (primary) achalasia. Orphanet Journal of Rare Diseases 2007, 2:38(http://www.pubmedcentral.nih.gov/articlerender.fcgi?artid=2040141), CC BY 2.0, https://commons.wikimedia.org/w/index.php?curid=2950922
	GERD		Retrosternal burning chest pain. Cough and hoarseness of voice. May present with complications such as strictures and dysphagia.		Upper GI endoscopy shows the complications such as esophagitis and barret esophagus. Esophageal manometry may show decreased tone of the lower esophageal sphincter. 24-hour esophageal pH monitoring may be done to confirm the diagnosis. Barret's esophagus - By Samir धर्म - taken from patient with permission to place in public domain, Copyrighted free use, https://commons.wikimedia.org/w/index.php?curid=1595945
	Esophageal carcinoma		Dysphagia Odynophagia- fluids and soft foods are usually tolerated, while hard or bulky substances (such as bread or meat) cause much more difficulty Weight loss Pain, often of a burning nature, may be severe and worsened by swallowing, and can be spasmodic in character Nausea and vomiting		Upper GI endoscopy and esophageal biopsy the gold standard for the diagnosis of esophageal CC BY-SA 3.0, https://commons.wikimedia.org/w/index.php?curid=2587715
	Esophageal stricture		Patient may present with the symptoms of the underlying GERD. Dysphagia and odynophagia.		Barium esophagography provides information about the site and the diameter of the stricture before the endoscopic intervention. Peptic stricture - By Samir धर्म - From en.wikipedia.org, Public Domain, https://commons.wikimedia.org/w/index.php?curid=1931423
	Plummer-Vinson syndrome		Common symptoms of Plummer-Vinson syndrome include: Difficulty swallowing (more for solids) Weakness Pain Burning sensation in mouth Dry tongue Painful cracks in the angles of a dry mouth Pale color of the skin Less common symptoms Cold intolerance Reduced resistance to infection Altered behavior Craving for for unusual items (such as ice or cold vegetables)		Lab tests are consistent with the diagnosis of iron deficiency anemia. Findings on an x-ray (barium esophagogram) suggestive of esophageal web/strictures associated with Plummer-Vinson syndrome appear as either: Thin projections on the anterior esophageal wall. Multiple upper (cervical) esophageal constrictions consistent with esophageal webs. Plummer-Vinson syndrome (Source: Case courtesy of Dr Hani Salam, <a href="https://radiopaedia.org/">Radiopaedia.org</a>. From the case <a href="https://radiopaedia.org/cases/14029">rID: 14029</a>)