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Plummer-Vinson Syndrome (PVS)

  • Posted on- Apr 16, 2018
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Plummer-Vinson syndrome (PVS) is a triad of microcytic hypochromic anemia (iron deficiency), atrophic glossitis, and esophageal webs or strictures.


Epidemiology


Plummer-Vinson syndrome (PVS) most commonly affects middle-aged women (this may be related to a propensity for iron deficiency), although it can be occasionally present in a child. The web (containing mucosa and submucosa) occurs at the anterior post-cricoid area of the upper esophagus.


Figures for incidence and prevalence are not available but it is becoming rarer, probably because dietary inadequacy is becoming less common.


A high prevalence of Plummer-Vinson syndrome (PVS) was reported in Sweden in the early 20th century. This reduced after the introduction of iron supplementation in food.


Plummer-Vinson syndrome (PVS) appears to be more common in the Indian subcontinent than in Western countries. Plummer-Vinson syndrome (PVS) is common in men as well as females in the Indian subcontinent.


Risk factors


Iron deficiency - this is related to poor diet or blood loss.


Presentation


The presentation is usually with painless, intermittent dysphagia. It tends to be with solid foods but, if untreated, may progress to soft foods and even liquids.


As with other causes of esophageal strictures, webs and rings, the patient is able to give a good indication of the level of obstruction.


Dysphagia may lead to weight loss. This should be regarded as a sign of danger.


There may also be features of iron-deficiency anemia like lethargy, tiredness and shortness of breath on exertion.


Examination


The features that appear on examination are those associated with iron deficiency. There may be pallor and even tachycardia if anemia is marked. There may be koilonychia (spoon-shaped nails), angular cheilitis and glossitis.


Investigation

  • FBC will show a microcytic, hypochromic anemia. Ferritin is low.
  • The barium swallow may show the web. This may need to be enhanced with videofluoroscopy.
  • A biopsy may be required if malignancy is suspected clinically.


Associated diseases


Various disorders have been known to associate PVS, such as celiac disease, inflammatory bowel disease, pernicious anemia, thyroid disease, Sjögren's syndrome and rheumatoid arthritis.


Treatment

  • Iron replacement can almost invariably be achieved by oral means. Adding vitamin C does not improve absorption significantly. There is rarely any need for parenteral iron. Supplements may be needed long-term because after correction it is important to maintain a normal iron status. Causes of blood loss like menorrhagia may require attention.
  • Endoscopic dilatation or argon plasma coagulation therapy of the esophageal web is occasionally required in cases of persistent dysphagia.


Complications


Post-cricoid carcinoma or carcinoma of the esophagus may develop.


Prognosis and prevention


Correction of iron deficiency will correct the condition if malignancy has not yet occurred. Prevention of iron deficiency prevents the disease.

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