Plummer-Vinson Syndrome

Plummer-Vinson Syndrome

Article
Digestive Health
Diseases & Conditions
+3
Contributed byKrish Tangella MD, MBADec 26, 2018

What are the other Names for this Condition? (Also known as/Synonyms)

  • Esophageal Web
  • Kelly-Paterson Syndrome
  • Sideropenic Dysphagia

What is Plummer-Vinson Syndrome? (Definition/Background Information)

  • Plummer-Vinson Syndrome (PVS) is a rare condition that involves swallowing difficulties due to small growths within the esophagus, which block the flow of food to the stomach. These small and benign web-like growths occur in the upper part of the esophagus
  • Along-with the presence of esophageal webs causing partial obstruction of the food-pipe, Plummer-Vinson Syndrome consists of iron-deficiency anemia, inflammation of the tongue (glossitis), and inflammation of the lip (cheilitis)
  • Older men and women with long-term iron deficiency anemia or a family history of the condition may be at an increased risk for developing Plummer-Vinson Syndrome
  • Currently, the cause of Plummer-Vinson Syndrome is unknown. The condition may be diagnosed by a healthcare professional through physical exam and other imaging tests of the esophagus
  • Upon diagnosis, treatment options for Plummer-Vinson Syndrome, such as iron supplements and modifying one’s eating habits, may be suggested. In case of severe obstruction, the esophagus may be mechanically dilated (expanded)
  • The prognosis of Plummer-Vinson Syndrome is generally good with treatment, but complications, such as trauma/tears in the throat during treatment, may worsen the prognosis. Also, individuals with PVS have shown a higher incidence of esophageal cancer

Who gets Plummer-Vinson Syndrome? (Age and Sex Distribution)

  • Plummer-Vinson Syndrome usually presents itself in adults, who are 40-70 years of age. But, the condition has been seen in children including teens
  • Men and women of different racial and ethnic backgrounds can be affected
  • Research has shown that women, especially of Scandinavian descent, are more likely to be affected

What are the Risk Factors for Plummer-Vinson Syndrome? (Predisposing Factors)

Risk factors that may increase the likelihood of developing Plummer-Vinson Syndrome may include:

  • Iron deficiency anemia (IDA), or an underlying condition that may cause iron deficiency in the body
  • A family history of the condition may be a risk factor
  • Post-menopausal women have a higher incidence of the condition

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one's chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Plummer-Vinson Syndrome? (Etiology)

  • The cause of Plummer-Vinson Syndrome is generally unknown
  • Some research scientists believe that it may be to due to genetic factors, nutritional deficiencies, or autoimmunity

What are the Signs and Symptoms of Plummer-Vinson Syndrome?

The signs and symptoms of Plummer-Vinson Syndrome may vary between individuals and may include the following:

  • Difficulty swallowing or choking due to small web-like growths in the esophagus (food-pipe): Initially, swallowing food with difficulty is noticed, following which the symptoms get slowly worse with time
  • Pain during swallowing
  • Over time, difficulty in swallowing may lead to weight loss
  • Weakness and fatigue due to anemia
  • Enlarged spleen (splenomegaly)
  • Signs and symptoms of iron-deficiency such as:
    • Angular cheilitis (inflammatory condition that affects the corners of the mouth)
    • Glossitis (tongue inflammation); burning sensation of tongue
    • Pallor (unhealthy pale appearance)

How is Plummer-Vinson Syndrome Diagnosed?

A healthcare professional can diagnose Plummer-Vinson Syndrome through:

  • A physical examination and analysis of previous medical history
  • Upper GI series: X-rays are used to view the esophagus, stomach, and small intestine, to look for deformities or growths
  • Esophagogastroduodenoscopy (EGD): Examination of the lining of the esophagus, stomach, and beginning parts of the small intestine, with a small camera that is guided to the area of interest
  • Videofluoroscopy: A video swallowing study using a small camera that is guided to the area of interest
  • Blood tests to check for anemia or iron deficiency

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Plummer-Vinson Syndrome?

Complications of Plummer-Vinson Syndrome may include:

  • Injury to the esophagus (tears) from medical devices used to stretch the esophagus during diagnosis and/or treatment
  • Esophageal cancer: Increased risk for squamous cell carcinoma of pharynx/esophagus/mouth (oral cavity)
  • Recurrence of the condition following treatment

How is Plummer-Vinson Syndrome Treated?

The treatment of Plummer-Vinson Syndrome is determined by a healthcare professional and the following may be considered:

  • Encourage the individual to take iron supplements to treat anemia (lack of healthy red blood cells) and the physical signs of iron deficiency
  • Treating the underlying cause of iron deficiency can help symptoms of pain to get better during swallowing
  • Widening and stretching of the web tissue in the esophagus, which is contracting the lumen of the esophagus, during esophagogastroduodenoscopy (the constricted web is dilated)
  • Eating foods slowly, chewing thoroughly, and eating meals in small bites; taking liquids or semi-solid foods

How can Plummer-Vinson Syndrome be Prevented?

Currently, Plummer-Vinson Syndrome is not a preventable condition, as the cause of the condition is still unknown. However, it is important to consider the following:

  • Ensuring that a proper amount of iron is taken through suitable diet, especially in middle-aged and older adults and among post-menopausal women
  • Undertaking periodic medical check-up in case of anemia or other illnesses

What is the Prognosis of Plummer-Vinson Syndrome? (Outcomes/Resolutions)

  • The prognosis of Plummer-Vinson Syndrome is generally good because individuals often respond well to treatment
  • Nevertheless, the prognosis of PVS also depends on the severity of the underlying anemia, the overall health status of the individual, and the development of any complications (such as cancer of the esophagus)

Additional and Relevant Useful Information for Plummer-Vinson Syndrome:

Plummer-Vinson Syndrome incidence rate has declined over the past century due to improvements in diets and health care.

Information on iron deficiency in children can be found at:

http://www.dovemed.com/diseases-conditions/iron-deficiency-anemia-children/

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Krish Tangella MD, MBA

Pathology, Medical Editorial Board, DoveMed Team

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