Plummer-Vinson syndrome differential diagnosis: Difference between revisions

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__NOTOC__
__NOTOC__
{{Plummer-Vinson syndrome}}
[[Image:Home_logo1.png|right|250px|link=https://www.wikidoc.org/index.php/Plummer-Vinson_syndrome]]
{{CMG}}; {{AE}}{{Akshun}}
{{CMG}}; {{AE}}{{Akshun}}


==Overview==
==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.
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==  
==Differentiating Plummer-Vinson syndrome from other Diseases==
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="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="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>
{{familytree/start}}
{{familytree | | | | | | | | | | | | | | | | | | | | | | | | A01 | | | |A01=Dysphagia}}
{{familytree | | | | | | | | | | | |,|-|-|-|-|-|-|-|-|-|-|-|-|^|-|-|-|-|-|-|-|-|-|-|-|-|-|-|.| | }}
{{familytree | | | | | | | | | | | B01 | | | | | | | | | | | | | | | | | | | | | | | | | | B02 | |B01=Oropharyngeal dysphagia|B02=Esophageal dysphagia|}}
{{familytree | | | | | | | |,|-|-|-|^|-|-|-|-|-|-|-|-|-|-|.| | | | | | | | | | | | |,|-|-|-|^|-|-|-|-|-|-|-|.| | |}}
{{familytree | | | | | | | C01 | | | | | | | | | | | | | C02 | | | | | | | | | | | C03 | | | | | | | | | | C04 | |C01=Solids only|C02=Solids and Liquids|C03=Solids only|C04=Solids and Liquids|}}
{{familytree | | | | | | | |!| | | | | | | | | | | |,|-|-|^|-|-|.| | | | | | | | | |!| | | | | | | | | | | |!| | | |}}
{{familytree | | | | | | | D01 | | | | | | | | | | D02 | | | | D03 | | | | | | | | D04 | | | | | | | | | | D05 | | |D01=•Zenker's diverticulum<br>•Neoplasm<br>•Webs |D02=Neurogenic|D03=Myogenic|D04=Pain|D05=•Achalasia<br>•Scleroderma<br>•DES|}}
{{familytree | | | | | | | |!| | | | | | | | | | | |!| | | | | |!| | | | | | |,|-|-|^|-|-|-|.| | | | | | | |!| | | |}}
{{familytree | | | | | | | |!| | | | | | | | | | | |!| | | | | E01 | | | | | E02 | | | | | E03 | | | | | | E04 | |E01=•Myasthenia gravis<br>•Connective tissue disorder<br>•Myotonic dystrophy|E02=No|E03=Yes|E04=Heart burn|}}
{{familytree | | | | | | | F01 | | | | | | | | | | F02 | | | | | | | | | | | |!| | | | | | |!| | | | |,|-|-|^|-|-|.| |F01=Barium swallow|F02=Mental status|}}
{{familytree | | | | | | | |!| | | | | | | | |,|-|-|^|-|-|.| | | | | | |,|-|-|^|-|-|.| | | G01 | | | G02 | | | | G03 | |G01=•Pill esophagitis<br>•Caustic injury<br>•Chemotherapy|G02=Yes|G03=No|}}
{{familytree | | |,|-|-|-|-|+|-|-|-|-|.| | | H01 | | | | H02 | | | | | H03 | | | | H04 | | | | | | | |!| | | | | |!| | |H01=Impaired|H02=Normal|H03=Non progressive|H04=Progressive|}}
{{familytree | | I01 | | | I02 | | | I03 | | |!| | | | | |!| | | | | | |!| | | | | |!| | | | | | | | I04 | | | | I05 |  I01=Sac|I02=Webs|I03=Mass|I04=Scleroderma|I05=•Achalasia<br>•DES|}}
{{familytree | | |!| | | | |!| | | | |!| | | J01 | | | | J02 | | | | | J03 | | | | J04 | | | | | | | | | | | | | |!| |J01=Stroke|J02=•ALS<br>•Parkinsonism| J03=•Rings<br>•Webs|J04=•Strictures<br>•Cancer|}}
{{familytree | | K01 | | | K02 | | | K03 | | | | | | | | | | | | | | | |!| | | | | |!| | | | | | | | | | | | | | K04 |K01=Zenker's diverticulum|K02=Plummer-Vinson syndrome|K03=Carcinoma|K04=Chest pain and manometry|}}
{{familytree | | | | | | | | | | | | | | | | | | | | | | | | | | | | | L01 | | | | L02 | | | | | | | | | | | | | |!| |L01=Barium swallow|L02=Weight loss|}}
{{familytree | | | | | | | | | | | | | | | | | | | | | | | | | | |,|-|-|^|-|-|.| | |!| | | | | | | | | | | | | | M01 | |M01=Increase LES pressure|}}
{{familytree | | | | | | | | | | | | | | | | | | | | | | | | | | N01 | | | | N02 | |!| | | | | | | | | | | |,|-|-|^|-|-|.| |N01=Rings|N02=Webs|}}
{{familytree | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | |,|-|-|^|-|-|.| | | | | | | | O01 | | | | O02 | | |O01=Yes|O02=No|}}
{{familytree | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | P01 | | | | P02 | | | | | | | |!| | | | | |!| | P01=Rapid|P02=Slow|}}
{{familytree | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | |!| | | | | |!| | | | | | | | Q01 | | | | Q02 | |Q01=Achalasia|Q02=DES|}}
{{familytree | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | R01 | | | | R02 | | | | | | |R01=Cancer|R02=Strictures/GERD|}}
{{familytree/end}}


<small>
{| class="wikitable"
{| class="wikitable"
! align="center" style="background: #4479BA; color: #FFFFFF; " |Disease
! rowspan="3" align="center" style="background:#4479BA; color: #FFFFFF;" |Disease
! align="center" style="background: #4479BA; color: #FFFFFF; " |Signs & Symptoms
! colspan="8" align="center" style="background:#4479BA; color: #FFFFFF;" |Signs and Symptoms
! align="center" style="background: #4479BA; color: #FFFFFF; " |Findings on barium esophagogram
! rowspan="3" align="center" style="background:#4479BA; color: #FFFFFF;" |Barium esophagogram
! align="center" style="background: #4479BA; color: #FFFFFF; " |Findings on endoscopy
! rowspan="3" align="center" style="background:#4479BA; color: #FFFFFF;" |Endoscopy
! align="center" style="background: #4479BA; color: #FFFFFF; " |Other findings
! rowspan="3" align="center" style="background:#4479BA; color: #FFFFFF;" |Other imaging and laboratory findings
! rowspan="3" align="center" style="background:#4479BA; color: #FFFFFF;" |Gold Standard
|-
|-
|Reflux esophagitis
| rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" |Onset
| colspan="3" align="center" style="background:#4479BA; color: #FFFFFF;" |Dysphagia
| rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" |Weight loss
| rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" |Heartburn
| rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" |Other findings
| rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" |Mental status
|-
| align="center" style="background:#4479BA; color: #FFFFFF;" |Solids
| align="center" style="background:#4479BA; color: #FFFFFF;" |Liquids
| align="center" style="background:#4479BA; color: #FFFFFF;" |Type
|-
| style="padding: 5px 5px; background: #DCDCDC;" align="center" |[[Plummer-Vinson syndrome]]
|
|
* Dysphagia (from peptic stricture)
* Gradual
| +
| -
|Non progressive
| +/-
| -
|
* [[Glossitis]]


* Heartburn
* [[Koilonychia]]


* Hoarseness
|Normal
|
|
* Poor clearance
* Thin projections on the anterior [[esophageal]] wall
* Multiple upper [[Esophageal stricture|esophageal constrictions]]
[[Image:Plummer-vinson-syndrome.jpg|center|200px|thumb|Barium esophagogram (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>)]]
|
* Direct visualization of [[esophageal webs]]
* Superior to [[esophagogram]]
<div style="width:350px">{{#ev:youtube|HFfsTgsB6Pg}}</div>
|
* Videofluoroscopy shows [[mucosal]] and [[submucosal]] foldings


* Free reflux of barium
|
|
* Peptic stricture (advanced cases)
Triad of
* [[Iron deficiency anemia]]
* [[Esophageal webs]]
* [[Glossitis]]
|-
| style="padding: 5px 5px; background: #DCDCDC;" align="center" |[[Esophageal stricture]]
|
|
* Lower esophageal pH studies will demonstrate pathologic gastroesophageal reflux
* Gradual


* A hiatus hernia may be present below the stricture
* Sudden onset
| +
| -
|Progressive
| +/-
| +/-
|
* [[Odynophagia]]
* [[Cough]]


* Manometry shows decreased tone of lower esophageal sphincter
* [[Chest pain]]
|Normal
|
*Sacculations
*Fixed transverse folds
*[[Esophageal]] intramural pseudodiverticula   
[[Image:Benign-oesophageal-stricture.jpg|center|200px|thumb|Case courtesy of Dr Ahmed Abd Rabou, Radiopaedia.org, rID: 23008]]
|
* [[Mucosal]] edema
* Circumferential thickening in [[Gastroesophageal reflux disease|GERD]]
* Pale [[mucosa]] with white [[exudate]] in lymphocytic esophagitis
* [[Swelling]] and [[hemorrhagic]] [[congestion]] in [[caustic]]<nowiki/> ingestion
<div style="width:350px">{{#ev:youtube|vax5E-jMnQ}}</div>
|
* [[Manometry]] may show dysmotility
* [[CT scan]] for staging [[malignant]] [[strictures]]
|
* [[Esophagogram|Barium esophagogram]]
|-
|-
|Esophageal carcinoma
| style="padding: 5px 5px; background: #DCDCDC;" align="center" |[[Diffuse esophageal spasm]]
|
|
* Dysphagia (initially for solids, liquids develops with advanced disease.)
* Sudden
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|Non progressive
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|
* [[Chest pain]]


* Weight loss
|Normal
 
|
* Lymphadenopathy
* Nonperistaltic and nonpropulsive contractions
 
* Corkscrew or rosary bead esophagus
* Cachexia
[[Image:DES radio.png|center|200px|thumb|Barium swallow appearance of DES<br>Source:By Nevit Dilmen [CC BY-SA 3.0 (https://creativecommons.org/licenses/by-sa/3.0)]]
|
* Inconclusive
<div style="width:350px">{{#ev:youtube|2ipA34iMA3c}}</div>
|
*[[Manometry]] shows high-amplitude [[esophageal]] contractions
*[[CT scan]] may show [[hypertrophy]] of esophageal muscles
|
* [[Manometry]]
|-
| style="padding: 5px 5px; background: #DCDCDC;" align="center" |[[Achalasia]]
|
* Gradual
| +
|<nowiki>+</nowiki>
|Non progressive
| +/-
| -
|
* [[Regurgitation]] of undigested food
* [[Chest pain]]
|Normal
|
* "Bird's beak" or "rat tail" appearance
* Dilated esophageal body
* Air fluid level (absent [[peristalsis]])
* Absence of an intragastric air bubble
[[Image:Achalasia-2.jpg|center|200px|thumb|Case courtesy of Dr Mario Umana, Radiopaedia.org, rID: 38071]]
|
|
* Irregular stricture
* Dilated [[esophagus]]
* Pre-stricture dilatation
* Residual food fragments
* Normal [[mucosa]]
<div style="width:350px">{{#ev:youtube|ydLcskQzEjM}}</div>
|
|
* Endoscopy with biopsy is the most accurate test for diagnosis and tumor histology. It may be used to depict:
* Residual pressure of [[Lower esophageal sphincter|LES]] > 10 mmHg
** Esophageal obstruction
* Incomplete relaxation of the [[Lower esophageal sphincter|LES]]
** Staging of disease
* Increased resting tone of [[Lower esophageal sphincter|LES]]
* Aperistalsis
|
|
* CT scan and PET scan of the chest and abdomen is an optional test for staging of the disease
* History of [[dysphagia]] with positive [[endoscopy]] and [[manometry]]
|-
|-
|Systemic sclerosis
| style="padding: 5px 5px; background: #DCDCDC;" align="center" |[[Systemic sclerosis]]
|
* Gradual
| +
|<nowiki>+</nowiki>
|Progressive
| +/-
| +
|
|
* Dysphagia
* [[Muscle pain|Muscle]] and [[Arthralgia|joint pain]]
 
* Muscle and joint pain


* Raynaud's phenomenon
* [[Raynaud's phenomenon]]


* Skin changes (rash, skin thickening)
* [[Skin changes]]
|Normal
|
|
* Dysmotility
* Dysmotility


* Patulous esophagus
* Patulous [[esophagus]]
|
|
* Mucosal damage
* [[Mucosal]] damage


* Peptic stricture (advanced cases)
* [[Peptic]] stricture (advanced cases)
|Positive serology for
|Positive serology for
* Antinuclear antibodies
* [[Antinuclear antibodies]]


* Rheumatoid factor
* [[Rheumatoid factor]]


* Creatine kinase
* [[Creatine kinase]]


* ESR  
* [[ESR]]
|
* [[Skin biopsy]]
|-
|-
|Esophageal spasm
| style="padding: 5px 5px; background: #DCDCDC;" align="center" |[[Zenker's diverticulum]]
|
|
* Chest pain (more prominent)
* Gradual
 
| +
* Dysphagia (intermittent)
|<nowiki>-</nowiki>
|
* Nonperistaltic and nonpropulsive contractions
* Corkscrew or rosary bead esophagus
 
|
|
* Inconclusive
| +/-
| -
|
|
* Manometry shows high-amplitude esophageal contractions
* Food [[regurgitation]]
* CT scan may show show hypertrophy of esophageal muscle wall
|-
|Pseudoachalasia
|Dysphagia


Weight loss
* [[Halitosis]]


Lymphadenopathy
* [[Coughing|Cough]]


Appetite changes
* [[Hoarseness]]
|Normal
|
* Thin projections on [[esophageal]] wall over [[Killian's dehiscence|Killian's triangle]]
[[Image:Zenker-4.jpg|center|200px|thumb| Radiopaedia.org">{{cite web |url=https://radiopaedia.org/cases/zenker-diverticulum |title=Zenker diverticulum &#124; Radiology Case &#124; Radiopaedia.org |format= |work= |accessdate=}}<nowiki></ref></nowiki>]]
|
* Outpouching of posterior [[pharyngeal]] wall


Cachexia
* Exclude the presence of [[Squamous cell carcinoma|SCC]]
 
<div style="width:350px">{{#ev:youtube|FdEruFsNdVA}}</div> 
Older patients
|
 
* [[CT]] & [[MRI]] shows out-pouching over the posterior esophagus in the Killian's triangle
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
* Barium [[Esophagogram|esophagography]]
 
|-
* Temporary patency of LES
| style="padding: 5px 5px; background: #DCDCDC;" align="center" |[[Esophageal carcinoma]]
|
|
* Endoscopy with biopsy is the most accurate test for diagnosis and tumor histology. It may be used to depict:
* Gradual
** Esophageal obstruction
|<nowiki>+</nowiki>
** Staging of disease
|<nowiki>+</nowiki>
 
|Progressive
| +
|<nowiki>+/-</nowiki>
|
|
* Gastroscopic biopsy of gastroesophageal junction and cardia may demonstrate malignancy.
* [[Lymphadenopathy]]


* Findings at endoscopy, barium swallow, and manometry may be indistinguishable from achalasia.
* [[Cachexia]]
|-
|Normal
|Chagas disease
|
|
* Dysphagia
* Irregular [[Strictures|stricture]]
* Toxic megacolon
* Pre-stricture [[dilatation]]
* Myocarditis
[[Image:Oesophageal-squamous-cell-carcinoma-2.jpg|center|200px|thumb|Case courtesy of Dr Bruno Di Muzio, Radiopaedia.org, rID: 4232f]]
* Blepharitis
|
|
* Esophageal dilatation
* [[Esophageal]] obstruction
* Stasis of barium
* Staging of disease
<div style="width:350px">{{#ev:youtube|5ucSlgqGAno}}</div>
|
|
* Dilated esophagus
* [[CT]] and [[PET scan]] is an optional test for staging of the disease
 
* Thickened LES (muscular ring)
|
|
* Giemsa stain will show ''Trypanosoma cruzi''.
* [[Biopsy]]
 
* PCR may be done to determine trypanosome subtype
|-
|-
|Pharyngitis
| style="padding: 5px 5px; background: #DCDCDC;" align="center" |[[Stroke]]
([[Cerebral hemorrhage]])
|
|
* Dysphagia
* Sudden
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|Progressive
|<nowiki>+</nowiki>
|<nowiki>+/-</nowiki>
|
* [[Dysarthria]]


* Fever
* Limb [[weakness]]


* Throat pain
* [[Fatigue]]
|Impaired
|
|
* Normal
* Pooling of [[Contrast medium|contrast]] in the [[pharynx]]
* [[Aspiration]] of [[barium]] [[Contrast medium|contrast]] into the [[airway]]
|
|
* Inconclusive
* Reduced opening of [[upper esophageal sphincter]]
* Reduced [[larynx]] elevation
|
* [[CT]] without [[contrast]] shows acute [[hemorrhage]] as a hyperattenuating [[clot]]
|
|
* Rapid antigen detection test positive for group A streptococccus
* [[CT]] without [[Contrast medium|contrast]]
* Tonsillar hypertrophy may cause severe narrowing of the pharynx
* Physical exam may show:
** Erythema, edema and/or exudates of the pharynx
** Lymphadenopathy
|-
|-
|Esophageal candidiasis
| style="padding: 5px 5px; background: #DCDCDC;" align="center" |Motor disorders
([[Myasthenia gravis]])
|
|
* Dysphagia
* Gradual
 
| +
* Immunocompromised
| +
 
|Progressive
* History of corticosteroid
|<nowiki>+/-</nowiki>
|
|
* [[Ptosis]]
* [[Diplopia]]
* [[Fatigue]]
|Normal
|
* Stasis in [[pharynx]] and pooling in pharyngeal recesses
|
* [[Velopharyngeal insufficiency]]
* Delayed [[swallowing]] function
|
|
* shaggy" appearance (plaques)
* CT may show anterior [[mediastinal]] mass ([[thymoma]])  
 
* Positive tensilon test
* irregular contours in the lower third
|
|
* ulceration 
* Anti–acetylcholine receptor antibody test
 
* plaques and pseudomembranes
* tiny nodules, polypoid folds (advanced cases)
|creamy white or yellowish plaques (thrush) in oropharynx or hypopharynx; may be normal exam
|-
|-
|Stroke
| style="padding: 5px 5px; background: #DCDCDC;" align="center" |[[GERD]]
|progressive Dysphagia; 
|
dysarthria;
* Gradual


limb weakness
* Sudden onset
 
| +
Fatigue
| -
|
|Progressive
* pooling of contrast in the pharynx 
| +/-
* aspiration of the barium contrast into the airway.
| +
|
|
* reduced opening of upper esophageal sphincter
* [[Cough]]
* reduced larynx elevation
|paraplegia, aphasia, dysarthria, vertigo, staggering, diplopia, deafness
|}


{| class="wikitable"
* [[Hoarseness]]
!
|Normal
!Manifestations
!Diagnostic tools
|-
|Achalasia
|
|
* Dyspnea<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>
* Free acid reflux
 
* [[Esophagitis]] with scarring
*[[Dysphagia]] for solids and liquids is the most common feature, being seen in 91 % and 85% of patients respectively<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>
* [[Strictures]]
*[[Regurgitation]] of undigested food occurs in 76-91% of patients<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>
* [[Barrett's oesophagus]]
*[[Cough]] mainly when lying down in 30%<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>
|
|
* Esophagogastroduodenoscopy findings include a dilated esophagus with residual food fragments, normal mucosa and occasionally [[candidiasis]] (due to the prolonged stasis).
* [[Erythema]], erosions and [[ulceration]]
* Barium swallow shows the characteristic bird's beak appearance.
* [[Barrett's esophagus]]
[[Image:Acha.jpg|center|300px|thumb|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.
* Esophageal [[manometry]] may show decreased tone of [[Lower esophageal sphincter|LES]]
* Cough and hoarseness of voice.
* May present with complications such as strictures and dysphagia.<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>
|
|
* Upper GI endoscopy shows the complications such as esophagitis and barret esophagus.
* 24 hour [[esophageal]] pH monitoring
* Esophageal manometry may show decreased tone of the lower esophageal sphincter.
* 24-hour esophageal pH monitoring may be done to confirm the diagnosis.
[[Image:Barretts esophagus.jpg|center|300px|thumb|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 cancer|Esophageal carcinoma]]
| style="padding: 5px 5px; background: #DCDCDC;" align="center" |[[Esophageal web]]
|
|
*[[Dysphagia]]
* Gradual
*[[Odynophagia]]- fluids and soft foods are usually tolerated, while hard or bulky substances (such as bread or meat) cause much more difficulty<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>
| +
*[[Weight loss]]
| +/-
*[[Pain and nociception|Pain]], often of a burning nature, may be severe and worsened by swallowing, and can be spasmodic in character
|Progressive
*[[Nausea]] and [[vomiting]]<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>
| -
| +/-
|
|
* Upper GI endoscopy and esophageal biopsy the gold standard for the diagnosis of esophageal
* Findings of the underlying cause such as [[iron deficiency anemia]] or [[bullous pemphigoid]]
[[Image:Esophageal adenoca.jpg|center|300px|thumb|CC BY-SA 3.0, https://commons.wikimedia.org/w/index.php?curid=2587715]]
|Normal
|-
|[[Esophageal spasm|Corckscrew esophagus]]
|
|
*Retrosternal chest pain that presents with or without food intake.<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>
* Symmetrical narrowing of the [[esophagus]]
*The condition is not progressive and not causing complications.<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>
|
|
* Barium swallow shows the characteristic corckscrew appearance of the esophagus.
* Smooth membrane not encircling the whole [[Lumen (anatomy)|lumen]]
[[Image:Nutcracker-esophagus-004.jpg|center|300px|thumb|Corckscrew esophagus - Case courtesy of Radswiki, Radiopaedia.org, rID: 11680]]
|-
|[[Esophageal stricture]]
|
|
*Patient may present with the symptoms of the underlying GERD.
* Videofluoroscopy shows [[mucosal]] and [[submucosal]] foldings
*Dysphagia and odynophagia.<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>
|
|
* Barium esophagography provides information about the site and the diameter of the stricture before the endoscopic intervention.<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>
* Barium [[esophagogram]]
[[Image:Peptic stricture.png|center|300px|thumb|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:<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>
*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:
* Thin projections on the anterior [[esophageal]] wall.
* Multiple upper ([[cervical]]) [[Esophageal stricture|esophageal constrictions]] consistent with [[esophageal webs]].
 
[[Image:Plummer-vinson-syndrome.jpg|center|200px|thumb|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>)]]
|}
|}
<small>


==References==
==References==

Latest revision as of 21:26, 8 February 2019

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.

Differentiating Plummer-Vinson syndrome from other Diseases

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][3][4][5][6][7][8][9][10][11]

 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Dysphagia
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Oropharyngeal dysphagia
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Esophageal dysphagia
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Solids only
 
 
 
 
 
 
 
 
 
 
 
 
Solids and Liquids
 
 
 
 
 
 
 
 
 
 
Solids only
 
 
 
 
 
 
 
 
 
Solids and Liquids
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
•Zenker's diverticulum
•Neoplasm
•Webs
 
 
 
 
 
 
 
 
 
Neurogenic
 
 
 
Myogenic
 
 
 
 
 
 
 
Pain
 
 
 
 
 
 
 
 
 
•Achalasia
•Scleroderma
•DES
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
•Myasthenia gravis
•Connective tissue disorder
•Myotonic dystrophy
 
 
 
 
No
 
 
 
 
Yes
 
 
 
 
 
Heart burn
 
 
 
 
 
 
 
Barium swallow
 
 
 
 
 
 
 
 
 
Mental status
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
•Pill esophagitis
•Caustic injury
•Chemotherapy
 
 
Yes
 
 
 
No
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Impaired
 
 
 
Normal
 
 
 
 
Non progressive
 
 
 
Progressive
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Sac
 
 
Webs
 
 
Mass
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Scleroderma
 
 
 
•Achalasia
•DES
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Stroke
 
 
 
•ALS
•Parkinsonism
 
 
 
 
•Rings
•Webs
 
 
 
•Strictures
•Cancer
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Zenker's diverticulum
 
 
Plummer-Vinson syndrome
 
 
Carcinoma
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Chest pain and manometry
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Barium swallow
 
 
 
Weight loss
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Increase LES pressure
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Rings
 
 
 
Webs
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Yes
 
 
 
No
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Rapid
 
 
 
Slow
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Achalasia
 
 
 
DES
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Cancer
 
 
 
Strictures/GERD
 
 
 
 
 
 

Disease Signs and Symptoms Barium esophagogram Endoscopy Other imaging and laboratory findings Gold Standard
Onset Dysphagia Weight loss Heartburn Other findings Mental status
Solids Liquids Type
Plummer-Vinson syndrome
  • Gradual
+ - Non progressive +/- - Normal
Barium esophagogram (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>)
{{#ev:youtube|HFfsTgsB6Pg}}

Triad of

Esophageal stricture
  • Gradual
  • Sudden onset
+ - Progressive +/- +/- Normal
  • Sacculations
  • Fixed transverse folds
  • Esophageal intramural pseudodiverticula   
Case courtesy of Dr Ahmed Abd Rabou, Radiopaedia.org, rID: 23008
{{#ev:youtube|vax5E-jMnQ}}
Diffuse esophageal spasm
  • Sudden
+ + Non progressive + + Normal
  • Nonperistaltic and nonpropulsive contractions
  • Corkscrew or rosary bead esophagus
Barium swallow appearance of DES
Source:By Nevit Dilmen [CC BY-SA 3.0 (https://creativecommons.org/licenses/by-sa/3.0)
  • Inconclusive
{{#ev:youtube|2ipA34iMA3c}}
Achalasia
  • Gradual
+ + Non progressive +/- - Normal
  • "Bird's beak" or "rat tail" appearance
  • Dilated esophageal body
  • Air fluid level (absent peristalsis)
  • Absence of an intragastric air bubble
Case courtesy of Dr Mario Umana, Radiopaedia.org, rID: 38071
{{#ev:youtube|ydLcskQzEjM}}
  • Residual pressure of LES > 10 mmHg
  • Incomplete relaxation of the LES
  • Increased resting tone of LES
  • Aperistalsis
Systemic sclerosis
  • Gradual
+ + Progressive +/- + Normal
  • Dysmotility
  • Peptic stricture (advanced cases)
Positive serology for
Zenker's diverticulum
  • Gradual
+ - +/- - Normal
Radiopaedia.org">"Zenker diverticulum | Radiology Case | Radiopaedia.org".</ref>
  • Exclude the presence of SCC
{{#ev:youtube|FdEruFsNdVA}}
 
  • CT & MRI shows out-pouching over the posterior esophagus in the Killian's triangle
Esophageal carcinoma
  • Gradual
+ + Progressive + +/- Normal
Case courtesy of Dr Bruno Di Muzio, Radiopaedia.org, rID: 4232f
{{#ev:youtube|5ucSlgqGAno}}
  • CT and PET scan is an optional test for staging of the disease
Stroke

(Cerebral hemorrhage)

  • Sudden
+ + Progressive + +/- Impaired
Motor disorders

(Myasthenia gravis)

  • Gradual
+ + Progressive +/- Normal
  • Stasis in pharynx and pooling in pharyngeal recesses
  • Anti–acetylcholine receptor antibody test
GERD
  • Gradual
  • Sudden onset
+ - Progressive +/- + Normal
Esophageal web
  • Gradual
+ +/- Progressive - +/- Normal
  • Smooth membrane not encircling the whole lumen

References

  1. Ferri, Fred (2015). Ferri's clinical advisor 2015 : 5 books in 1. Philadelphia, PA: Elsevier/Mosby. ISBN 978-0323083751.
  2. Boeckxstaens GE, Zaninotto G, Richter JE (2013). "Achalasia". Lancet. doi:10.1016/S0140-6736(13)60651-0. PMID 23871090.
  3. 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. 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. Matsuura H (2017). "Diffuse Esophageal Spasm: Corkscrew Esophagus". Am. J. Med. doi:10.1016/j.amjmed.2017.08.041. PMID 28943381.
  6. Lassen JF, Jensen TM (1992). "[Corkscrew esophagus]". Ugeskr. Laeg. (in Danish). 154 (5): 277–80. PMID 1736462.
  7. 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. Shami VM (2014). "Endoscopic management of esophageal strictures". Gastroenterol Hepatol (N Y). 10 (6): 389–91. PMC 4080876. PMID 25013392.
  9. 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. Chisholm M (1974). "The association between webs, iron and post-cricoid carcinoma". Postgrad Med J. 50 (582): 215–9. PMC 2495558. PMID 4449772.
  11. 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.