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.[ 1] [ 2] [ 3] [ 4] [ 5] [ 6] [ 7] [ 8] [ 9] [ 10] [ 11]
Disease
Signs & Symptoms
Findings on barium esophagogram
Findings on endoscopy
Other findings
Reflux esophagitis
Dysphagia (from peptic stricture)
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
Esophageal carcinoma
Dysphagia (initially for solids, liquids develops with advanced disease.)
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
Skin changes (rash, skin thickening)
Peptic stricture (advanced cases)
Positive serology for
Pseudoachalasia
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
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
Thickened LES (muscular ring)
Giemsa stain will show Trypanosoma cruzi . PCR may be done to determine trypanosome subtype
Pharyngitis
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
History of corticosteroid
Shaggy" appearance (plaques) Irregular contours in the lower third
Plaques and pseudomembranes
Tiny nodules, polypoid folds (advanced cases)
Creamy white or yellowish plaques (thrush) in oropharynx
Stroke
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
Dysphagia for solids and liquids is the most common feature, being seen in 91 % and 85% of patients respectively[ 2] Regurgitation of undigested food occurs in 76-91% of patients[ 2] Cough mainly when lying down in 30%[ 2]
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.[ 3]
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[ 4] Weight loss Pain , often of a burning nature, may be severe and worsened by swallowing, and can be spasmodic in characterNausea and vomiting [ 4]
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.[ 7]
Barium esophagography provides information about the site and the diameter of the stricture before the endoscopic intervention.[ 8] 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:[ 9] [ 10] [ 11] 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 cmmon 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:
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>)
References
↑ Ferri, Fred (2015). Ferri's clinical advisor 2015 : 5 books in 1 . Philadelphia, PA: Elsevier/Mosby. ISBN 978-0323083751 . ↑ 2.0 2.1 2.2 2.3 Boeckxstaens GE, Zaninotto G, Richter JE (2013). "Achalasia" . Lancet . doi :10.1016/S0140-6736(13)60651-0 . PMID 23871090 . ↑ 3.0 3.1 Badillo R, Francis D (2014). "Diagnosis and treatment of gastroesophageal reflux disease" . World J Gastrointest Pharmacol Ther . 5 (3): 105–12. doi :10.4292/wjgpt.v5.i3.105 . PMC 4133436 PMID 25133039 . ↑ 4.0 4.1 4.2 Napier KJ, Scheerer M, Misra S (2014). "Esophageal cancer: A Review of epidemiology, pathogenesis, staging workup and treatment modalities" . World J Gastrointest Oncol . 6 (5): 112–20. doi :10.4251/wjgo.v6.i5.112 . PMC 4021327 PMID 24834141 . ↑ Matsuura H (2017). "Diffuse Esophageal Spasm: Corkscrew Esophagus". Am. J. Med . doi :10.1016/j.amjmed.2017.08.041 . PMID 28943381 . ↑ Lassen JF, Jensen TM (1992). "[Corkscrew esophagus]". Ugeskr. Laeg. (in Danish). 154 (5): 277–80. PMID 1736462 . ↑ 7.0 7.1 Ruigómez A, García Rodríguez LA, Wallander MA, Johansson S, Eklund S (2006). "Esophageal stricture: incidence, treatment patterns, and recurrence rate". Am. J. Gastroenterol . 101 (12): 2685–92. doi :10.1111/j.1572-0241.2006.00828.x . PMID 17227515 . ↑ 8.0 8.1 Shami VM (2014). "Endoscopic management of esophageal strictures" . Gastroenterol Hepatol (N Y) . 10 (6): 389–91. PMC 4080876 PMID 25013392 . ↑ 9.0 9.1 López Rodríguez MJ, Robledo Andrés P, Amarilla Jiménez A, Roncero Maíllo M, López Lafuente A, Arroyo Carrera I (2002). "Sideropenic dysphagia in an adolescent". J. Pediatr. Gastroenterol. Nutr . 34 (1): 87–90. PMID 11753173 . ↑ 10.0 10.1 Chisholm M (1974). "The association between webs, iron and post-cricoid carcinoma" . Postgrad Med J . 50 (582): 215–9. PMC 2495558 PMID 4449772 . ↑ 11.0 11.1 Larsson LG, Sandström A, Westling P (1975). "Relationship of Plummer-Vinson disease to cancer of the upper alimentary tract in Sweden". Cancer Res . 35 (11 Pt. 2): 3308–16. PMID 1192404 . ↑ Ferri, Fred (2015). Ferri's clinical advisor 2015 : 5 books in 1 . Philadelphia, PA: Elsevier/Mosby. ISBN 978-0323083751 .
