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==Differential Diagnosis==  
==Differential Diagnosis==  
Plummer-Vinson syndrome must be differentiated from other diseases that cause dysphagia such as [[reflux esophagitis]], [[esophageal carcinoma]], [[systemic sclerosis]], [[esophageal spasm]], [[pseudoachalasia]], [[stroke]], [[esophageal candidiasis]] and [[Chagas disease]].
Plummer-Vinson syndrome must be differentiated from other diseases that cause dysphagia such as [[reflux esophagitis]], [[esophageal carcinoma]], [[systemic sclerosis]], [[esophageal spasm]], [[pseudoachalasia]], [[stroke]], [[esophageal candidiasis]] and [[Chagas disease]].<ref>{{cite book | last = Ferri | first = Fred | title = Ferri's clinical advisor 2015 : 5 books in 1 | publisher = Elsevier/Mosby | location = Philadelphia, PA | year = 2015 | isbn = 978-0323083751 }}</ref><ref name="pmid23871090">{{cite journal| author=Boeckxstaens GE, Zaninotto G, Richter JE| title=Achalasia. | journal=Lancet | year= 2013 | volume=  | issue=  | pages=  | pmid=23871090 | doi=10.1016/S0140-6736(13)60651-0 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23871090  }}</ref><ref name="pmid23871090">{{cite journal| author=Boeckxstaens GE, Zaninotto G, Richter JE| title=Achalasia. | journal=Lancet | year= 2013 | volume=  | issue=  | pages=  | pmid=23871090 | doi=10.1016/S0140-6736(13)60651-0 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23871090  }} </ref><ref name="pmid23871090">{{cite journal| author=Boeckxstaens GE, Zaninotto G, Richter JE| title=Achalasia. | journal=Lancet | year= 2013 | volume=  | issue=  | pages=  | pmid=23871090 | doi=10.1016/S0140-6736(13)60651-0 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23871090  }} </ref><ref name="pmid25133039">{{cite journal |vauthors=Badillo R, Francis D |title=Diagnosis and treatment of gastroesophageal reflux disease |journal=World J Gastrointest Pharmacol Ther |volume=5 |issue=3 |pages=105–12 |year=2014 |pmid=25133039 |pmc=4133436 |doi=10.4292/wjgpt.v5.i3.105 |url=}}</ref><ref name="pmid24834141">{{cite journal |vauthors=Napier KJ, Scheerer M, Misra S |title=Esophageal cancer: A Review of epidemiology, pathogenesis, staging workup and treatment modalities |journal=World J Gastrointest Oncol |volume=6 |issue=5 |pages=112–20 |year=2014 |pmid=24834141 |pmc=4021327 |doi=10.4251/wjgo.v6.i5.112 |url=}}</ref><ref name="pmid24834141">{{cite journal |vauthors=Napier KJ, Scheerer M, Misra S |title=Esophageal cancer: A Review of epidemiology, pathogenesis, staging workup and treatment modalities |journal=World J Gastrointest Oncol |volume=6 |issue=5 |pages=112–20 |year=2014 |pmid=24834141 |pmc=4021327 |doi=10.4251/wjgo.v6.i5.112 |url=}}</ref><ref name="pmid28943381">{{cite journal |vauthors=Matsuura H |title=Diffuse Esophageal Spasm: Corkscrew Esophagus |journal=Am. J. Med. |volume= |issue= |pages= |year=2017 |pmid=28943381 |doi=10.1016/j.amjmed.2017.08.041 |url=}}</ref><ref name="pmid1736462">{{cite journal |vauthors=Lassen JF, Jensen TM |title=[Corkscrew esophagus] |language=Danish |journal=Ugeskr. Laeg. |volume=154 |issue=5 |pages=277–80 |year=1992 |pmid=1736462 |doi= |url=}}</ref><ref name="pmid17227515">{{cite journal |vauthors=Ruigómez A, García Rodríguez LA, Wallander MA, Johansson S, Eklund S |title=Esophageal stricture: incidence, treatment patterns, and recurrence rate |journal=Am. J. Gastroenterol. |volume=101 |issue=12 |pages=2685–92 |year=2006 |pmid=17227515 |doi=10.1111/j.1572-0241.2006.00828.x |url=}}</ref><ref name="pmid25013392">{{cite journal |vauthors=Shami VM |title=Endoscopic management of esophageal strictures |journal=Gastroenterol Hepatol (N Y) |volume=10 |issue=6 |pages=389–91 |year=2014 |pmid=25013392 |pmc=4080876 |doi= |url=}}</ref><ref name="pmid11753173">{{cite journal |vauthors=López Rodríguez MJ, Robledo Andrés P, Amarilla Jiménez A, Roncero Maíllo M, López Lafuente A, Arroyo Carrera I |title=Sideropenic dysphagia in an adolescent |journal=J. Pediatr. Gastroenterol. Nutr. |volume=34 |issue=1 |pages=87–90 |year=2002 |pmid=11753173 |doi= |url=}}</ref><ref name="pmid4449772">{{cite journal |vauthors=Chisholm M |title=The association between webs, iron and post-cricoid carcinoma |journal=Postgrad Med J |volume=50 |issue=582 |pages=215–9 |year=1974 |pmid=4449772 |pmc=2495558 |doi= |url=}}</ref><ref name="pmid1192404">{{cite journal |vauthors=Larsson LG, Sandström A, Westling P |title=Relationship of Plummer-Vinson disease to cancer of the upper alimentary tract in Sweden |journal=Cancer Res. |volume=35 |issue=11 Pt. 2 |pages=3308–16 |year=1975 |pmid=1192404 |doi= |url=}}</ref>


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Revision as of 18:31, 6 November 2017

Plummer-Vinson syndrome Microchapters

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

Overview

Plummer-Vinson syndrome must be differentiated from other diseases that cause dysphagia such as reflux esophagitis, esophageal carcinoma, systemic sclerosis, esophageal spasm, pseudoachalasia, stroke, esophageal candidiasis and Chagas disease.

Differential Diagnosis

Plummer-Vinson syndrome must be differentiated from other diseases that cause dysphagia such as reflux esophagitis, esophageal carcinoma, systemic sclerosis, esophageal spasm, pseudoachalasia, stroke, esophageal candidiasis and Chagas disease.[1][2][2][2][3][4][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)
  • 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
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
Esophageal spasm
  • Chest pain (more prominent)
  • Dysphagia (intermittent)
  • Nonperistaltic and nonpropulsive contractions
  • Corkscrew or rosary bead esophagus
  • Inconclusive
  • Manometry shows high-amplitude esophageal contractions
  • CT scan may show show hypertrophy of esophageal muscle wall
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.

Plummer-Vinson syndrome must be differentiated from other causes of dysphagia, odynophagia and food regurgitation such as GERD, esophageal adenocarcinoma and esophageal stricture.

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 character
  • Nausea 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
Corckscrew esophagus
  • Retrosternal chest pain that presents with or without food intake.[5]
  • The condition is not progressive and not causing complications.[6]
  • Barium swallow shows the characteristic corckscrew appearance of the esophagus.
Corckscrew esophagus - Case courtesy of Radswiki, Radiopaedia.org, rID: 11680
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

  1. ↑ Ferri, Fred (2015). Ferri's clinical advisor 2015 : 5 books in 1. Philadelphia, PA: Elsevier/Mosby. ISBN 978-0323083751.
  2. ↑ 2.0 2.1 2.2 2.3 2.4 2.5 Boeckxstaens GE, Zaninotto G, Richter JE (2013). "Achalasia". Lancet. doi:10.1016/S0140-6736(13)60651-0. PMID 23871090.
  3. ↑ 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. ↑ 4.0 4.1 4.2 4.3 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.
  5. ↑ 5.0 5.1 Matsuura H (2017). "Diffuse Esophageal Spasm: Corkscrew Esophagus". Am. J. Med. doi:10.1016/j.amjmed.2017.08.041. PMID 28943381.
  6. ↑ 6.0 6.1 Lassen JF, Jensen TM (1992). "[Corkscrew esophagus]". Ugeskr. Laeg. (in Danish). 154 (5): 277–80. PMID 1736462.
  7. ↑ 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. ↑ 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. ↑ 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. ↑ 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. ↑ 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.
  12. ↑ Ferri, Fred (2015). Ferri's clinical advisor 2015 : 5 books in 1. Philadelphia, PA: Elsevier/Mosby. ISBN 978-0323083751.
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