Plummer-Vinson syndrome overview: Difference between revisions

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===Imaging Findings===
===Imaging Findings===
Videofluoroscopy may be helpful in the [[diagnosis]] of Plummer-Vinson syndrome. Videofluoroscopy is done in patients with normal [[barium]] [[esophagogram]] who have a high probability of Plummer-Vinson syndrome. Videofluoroscopy is superior to [[barium]] [[esophagogram]] and has the ability to detect small [[esophageal webs]] resulting from insignificant [[mucosal]] and [[submucosal]] foldings which may otherwise go undiagnosed.


===Other Diagnostic Studies===
===Other Diagnostic Studies===

Revision as of 16:51, 3 November 2017

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

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Overview

The Plummer-Vinson syndrome, also called Paterson-Brown-Kelly syndrome or sideropenic dysphagia is a disorder linked to severe, long-term iron deficiency anemia, which causes swallowing difficulty (dysphagia) due to web-like membranes of tissue growing in the throat (esophageal webs). [1] The disease is named after two Americans, the physician Henry Stanley Plummer, and the surgeon Porter Paisley Vinson. [2][3][4]

It is also sometimes called "Kelly-Paterson syndrome", after Adam Brown-Kelly and Donald Ross Paterson.[2][5][6]

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Plummer-Vinson syndrome overview from Other Diseases

Epidemiology and Demographics

Plummer-Vinson syndrome is a rare disease and the data pertaining to incidence and prevalence is not evidently available. Overall improvement in nutritional status with better medical care has markedly reduced the number of cases of Plummer-Vinson syndrome. However, individuals of any age groups may develop Plummer-Vinson syndrome and it is most commonly seen in the age group of 40-70 years. Plummer-Vinson syndrome usually affects individuals of the caucasian race. Females are commonly affected than males with female to male ratio of 4:1. The majority of Plummer-Vinson syndrome cases are reported in Scandinavian countries or north European countries.

Risk Factors

Screening

Natural History, Complications, and Prognosis

If left untreated, patients of Plummer-Vinson syndrome may progress to develop fatigue, dyspnea on exertion, esophageal strictures, and malignant lesions of the mouth and oral cavity. Common complications of Plummer-Vinson syndrome include hypopharyngeal cancer, esophageal cancer and malignant lesions of oral mucosa. Depending on the extent of Plummer-Vinson syndrome at the time of diagnosis, the prognosis may vary. Prognosis is generally good for patients who receive treatment. Iron replacement therapy and dilatation of esophageal web leads to rapid reversal of symptoms.

Diagnosis

Diagnostic Criteria

History and Symptoms

Physical Examination

Laboratory Findings

Laboratory findings consistent with the diagnosis of Plummer-Vinson syndrome include presence of iron deficiency anemia. Patients suspected of Plummer-Vinson syndrome should be tested with complete blood count (CBC), iron studies, peripheral smear, stool test for occult blood, blood lead levels and bone marrow biopsy for stainable iron.

Imaging Findings

Videofluoroscopy may be helpful in the diagnosis of Plummer-Vinson syndrome. Videofluoroscopy is done in patients with normal barium esophagogram who have a high probability of Plummer-Vinson syndrome. Videofluoroscopy is superior to barium esophagogram and has the ability to detect small esophageal webs resulting from insignificant mucosal and submucosal foldings which may otherwise go undiagnosed.

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Prevention

References

  1. Novacek G (2006). "Plummer-Vinson syndrome". Orphanet J Rare Dis. 1: 36. doi:10.1186/1750-1172-1-36. PMID 16978405.
  2. 2.0 2.1 Template:WhoNamedIt
  3. H. S. Plummer. Diffuse dilatation of the esophagus without anatomic stenosis (cardiospasm). A report of ninety-one cases. Journal of the American Medical Association, Chicago, 1912, 58: 2013-2015.
  4. P. P. Vinson. A case of cardiospasm with dilatation and angulation of the esophagus. Medical Clinics of North America, Philadelphia, PA., 1919, 3: 623-627.
  5. A. B. Kelly. Spasm at the entrance of the esophagus. The Journal of Laryngology, Rhinology, and Otology, London, 1919, 34: 285-289.
  6. D. R. Paterson. A clinical type of dysphagia. The Journal of Laryngology, Rhinology, and Otology, London, 1919, 24: 289-291.