History

Fact Explanation
Difficulty in swallowing (dysphagia) Occurs due to long term iron deficiency anemia leading to formation of esophageal webs. [1], [2]. These esophageal webs are thin mucosal folds (covered with squamous epithelium) protruding into the lumen of proximal esophagus. Dysphagia resulted due to esophageal webs is usually painless and intermittent or progressive. [1]. Patients usually complain dysphagia to solid but with the time it can progress into dysphagia for liquids as well. [2]
Weight loss Dysphagia occurs in Plummer Vinson syndrome (PVS) is painless thus leading to progressive weight loss. As the progressive dysphagia is painless patient tolerate it for long before presenting to the clinician causing malnourishment and weight loss. [2]
Weakness Symptom that occurs secondary to iron deficiency anemia.[1],[2].
Fatigue Also occurs due to iron deficiency anemia.[2],[3].
Pallor Common secondary manifestation of iron deficiency anemia (IDA). [1]. Patients with mild IDA are most likely asymptomatic.Symptoms appear when the anemia becomes more severe.[4].
Exertional dyspnea Non specific manifestation of severe anemia.[4].
References
  1. GUDE D, BANSAL D, MALU A. Revisiting Plummer Vinson Syndrome Ann Med Health Sci Res [online] 2013, 3(1):119-121 [viewed 29 July 2014] Available from: doi:10.4103/2141-9248.109476
  2. NAIK SUDHIR M, MC SHIVAKUMAR, APPAJI MOHAN K, RAVISHANKARA S, NAIK SARIKA S, DE SOUZA CHRIS. A Case of Plummer-Vinson Syndrome Esophageal Web Dysphagia treated by Dilatation with Cuffed Endotracheal Tube. IJHNS [online] 2011 September, 2:161-165 [viewed 29 July 2014] Available from: doi:10.5005/jp-journals-10001-1076
  3. NOVACEK G. Plummer-Vinson syndrome Orphanet J Rare Dis [online] :36 [viewed 29 July 2014] Available from: doi:10.1186/1750-1172-1-36
  4. ZHU AMY, KANESHIRO MARC, KAUNITZ JONATHAN D.. Evaluation and Treatment of Iron Deficiency Anemia: A Gastroenterological Perspective. Dig Dis Sci [online] December, 55(3):548-559 [viewed 30 July 2014] Available from: doi:10.1007/s10620-009-1108-6

Examination

Fact Explanation
Esophageal webs Iron deficiency causes reduction of iron dependent oxidative enzymes. It leads to degeneration of muscles of pharynx, leading to mucosal atrophy and development of webs.[1] The high cellular turnover rate in the epithelium of the upper digestive tract makes it vulnerable to iron deficiency due to deficiency of the iron dependent enzymes.[2] And also iron deficiency decreases the contraction amplitude of the esophageal muscles, leading to motility impairment.The webs limited to proximal and middle part of the esophagus can explained,because transient time in upper esophagus slower in affected individuals than the normal individuals.[1].
Glossitis Manifestation of IDA.[3]. Iron deficiency causing atrophy of upper GI mucosa is the reason for glossitis.[4].
Angular cheilitis Occurs due to IDA.[3]. Cracks or fissure at the corners of the mouth along with painful tongue can be seen.[1].
Smooth tongue Tissue iron plays an important role in the proliferation of epithelial cells.Smooth tongue is a physical sign of tissue iron deficiency.[5].
Koilonychia Spoon-shaped fingernails.Also a secondary manifestation of anemia.[3]. Those patients have nails that are brittle which break easily.[1].
Tachycardia Resulting from anemia.Plummer-Vinson syndrome (PVS) is defined by the classic triad of dysphagia, iron-deficiency anemia and esophageal webs.So the features of iron deficiency anemia commonly seen in patients with PVS.[3].
References
  1. NAIK SUDHIR M, MC SHIVAKUMAR, APPAJI MOHAN K, RAVISHANKARA S, NAIK SARIKA S, DE SOUZA CHRIS. A Case of Plummer-Vinson Syndrome Esophageal Web Dysphagia treated by Dilatation with Cuffed Endotracheal Tube. IJHNS [online] 2011 September, 2:161-165 [viewed 29 July 2014] Available from: doi:10.5005/jp-journals-10001-1076
  2. GUDE D, BANSAL D, MALU A. Revisiting Plummer Vinson Syndrome Ann Med Health Sci Res [online] 2013, 3(1):119-121 [viewed 29 July 2014] Available from: doi:10.4103/2141-9248.109476
  3. NOVACEK G. Plummer-Vinson syndrome Orphanet J Rare Dis [online] :36 [viewed 29 July 2014] Available from: doi:10.1186/1750-1172-1-36
  4. BAYRAKTAR ULAS D. Treatment of iron deficiency anemia associated with gastrointestinal tract diseases. WJG [online] 2010 December [viewed 30 July 2014] Available from: doi:10.3748/wjg.v16.i22.2720
  5. TAHARA T, SHIBATA T, OKUBO M, YOSHIOKA D, ISHIZUKA T, SUMI K, KAWAMURA T, NAGASAKA M, NAKAGAWA Y, NAKAMURA M, ARISAWA T, OHMIYA N, HIRATA I. A Case of Plummer-Vinson Syndrome Showing Rapid Improvement of Dysphagia and Esophageal Web after Two Weeks of Iron Therapy Case Rep Gastroenterol [online] , 8(2):211-215 [viewed 30 July 2014] Available from: doi:10.1159/000364820

Differential Diagnoses

Fact Explanation
Malignant tumors of the esophagus Causes dysphagia.[1]. Some may also have weight loss, odynophagia, retrosternal pain,dyspnea and hoarseness.The physical examination is usually unremarkable. People who are having distal metastasis may present with Lymphadenopathy, hepatomegaly, and a pleural effusion.[2]. However PVS itself is a premalignant condition and it has a risk of developing in to squamous cell carcinoma of the postcricoid pharynx and upper esophagus.[1].
Benign esophageal strictures Dysphagia is a presenting symptom. [1]. Benign esophageal strictures are sequelae of deep esophageal injuries.These injuries may be due to peptic ulcer disease, radiation therapy, esophageal webs, surgery or a corrosive injury.[3]. Complications include malnutrition, aspiration and pain.[3].
Achalasia cardia Gastric motility disorder. present with dysphagia.[1]. The primary presenting symptom is dysphagia for both solids and liquids that is constant rather than intermittent. It is often accompanied by regurgitation of undigested food and saliva minutes or even hours after the meal. Regurgitation can occur at night. So patients try to compensate it by elevating their head or even sitting upright to sleep. Some Patients also experience chest pain or heartburn.[4].
Gastroesophageal reflux disease (GERD) Also causes dysphagia.[1]. However typical symptoms are heartburn and regurgitation.Gastroesophageal reflux is the most common cause of heartburn. Several extraesophageal manifestations of the disease are well recognized.Those include laryngitis, cough,spectrum of injury includes esophagitis and strictures.[5].
References
  1. NOVACEK G. Plummer-Vinson syndrome Orphanet J Rare Dis [online] :36 [viewed 29 July 2014] Available from: doi:10.1186/1750-1172-1-36
  2. ENZINGER PETER C., MAYER ROBERT J.. Esophageal Cancer. N Engl J Med [online] 2003 December, 349(23):2241-2252 [viewed 29 July 2014] Available from: doi:10.1056/NEJMra035010
  3. KIM JH, SHIN JH, SONG HY. Benign Strictures of the Esophagus and Gastric Outlet: Interventional Management Korean J Radiol [online] 2010, 11(5):497-506 [viewed 29 July 2014] Available from: doi:10.3348/kjr.2010.11.5.497
  4. PANDOLFINO JOHN E., KAHRILAS PETER J.. Presentation, Diagnosis, and Management of Achalasia. Clinical Gastroenterology and Hepatology [online] 2013 August, 11(8):887-897 [viewed 31 July 2014] Available from: doi:10.1016/j.cgh.2013.01.032
  5. KAHRILAS PETER J.. Gastroesophageal Reflux Disease. N Engl J Med [online] 2008 October, 359(16):1700-1707 [viewed 31 July 2014] Available from: doi:10.1056/NEJMcp0804684

Investigations - for Diagnosis

Fact Explanation
Full blood count/Complete blood count Reveal iron deficiency anemia with decreased values of hemoglobin, hematocrit, and mean corpuscular volume. [1],[2].
Serum iron studies Reveal iron deficiency anemia with decrease level of iron, ferritin and and increased total iron binding capacity.[1],[2].
Barium swallow Esophageal webs can be detected by barium swallow X-ray. Barium-swallow studies and fluoroscopic evaluation suggest the diagnosis and also degree of stenosis.[1],[2].
Videofluoroscopy The best way for demonstration of esophageal webs than the barium swallow. Webs are also detectable by upper gastrointestinal endoscopy.Those webs appear smooth, thin, and gray with eccentric or central lumen.These esophageal webs typically occur in the proximal part of the esophagus. They may be missed or accidentally ruptured unless the endoscope is introduced under direct visualization.[2]
References
  1. GUDE D, BANSAL D, MALU A. Revisiting Plummer Vinson Syndrome Ann Med Health Sci Res [online] 2013, 3(1):119-121 [viewed 29 July 2014] Available from: doi:10.4103/2141-9248.109476
  2. NOVACEK G. Plummer-Vinson syndrome Orphanet J Rare Dis [online] :36 [viewed 29 July 2014] Available from: doi:10.1186/1750-1172-1-36

Investigations - Followup

Fact Explanation
Surveillance upper gastrointestinal endoscopy Recommended every year, because PVS is associated with an increase risk of squamous cell carcinoma of the pharynx and esophagus. So the patient should be followed up closely. [1].
References
  1. NOVACEK G. Plummer-Vinson syndrome Orphanet J Rare Dis [online] :36 [viewed 29 July 2014] Available from: doi:10.1186/1750-1172-1-36

Management - General Measures

Fact Explanation
Iron replacement Iron supplementation alone can resolve dysphagia in many patients with PVS.[1]. Usually both anemia and dysphagia effectively treated with iron therapy.Iron therapy is recommended even if the hemoglobin percentage is normal in the presence of esophageal web formation. [2]. And the others,iron replacement is recommended at least until normalization of the hematocrit and ferritin levels.[1].
References
  1. NOVACEK G. Plummer-Vinson syndrome Orphanet J Rare Dis [online] :36 [viewed 29 July 2014] Available from: doi:10.1186/1750-1172-1-36
  2. NAIK SUDHIR M, MC SHIVAKUMAR, APPAJI MOHAN K, RAVISHANKARA S, NAIK SARIKA S, DE SOUZA CHRIS. A Case of Plummer-Vinson Syndrome Esophageal Web Dysphagia treated by Dilatation with Cuffed Endotracheal Tube. IJHNS [online] 2011 September, 2:161-165 [viewed 29 July 2014] Available from: doi:10.5005/jp-journals-10001-1076

Management - Specific Treatments

Fact Explanation
Endoscopic dilatation In case of significant obstruction of the esophageal lumen by esophageal web and persistent dysphagia despite iron supplementation have to go for an alternative procedure like rupture and dilation of the web. After endoscopic placement of a guidewire, dilators with a diameter of up to 17 mm can be used.Usually only one dilation is enough to relieve dysphagia. But in some cases several attempts should be taken. [1]
Argon plasma coagulation therapy To treat esophageal webs. However it has been tried with reasonable success.The prognosis is mostly good at short-term followups.[2].
References
  1. NOVACEK G. Plummer-Vinson syndrome Orphanet J Rare Dis [online] :36 [viewed 29 July 2014] Available from: doi:10.1186/1750-1172-1-36
  2. GUDE D, BANSAL D, MALU A. Revisiting Plummer Vinson Syndrome Ann Med Health Sci Res [online] 2013, 3(1):119-121 [viewed 30 July 2014] Available from: doi:10.4103/2141-9248.109476