Plummer-Vinson syndrome (PVS)


Introduction

Rare syndrome defined by the classic triad of dysphagia, iron-deficiency anemia and oesophageal webs.

  • Rare medical condition and mostly affecting women of 40-70 years
  • Commonly associated with glossitis

History

Plummer-Vinson syndrome is named after Henry Stanley Plummer (1874–1936) and Porter Paisley Vinson (1890–1959) who were physicians on the staff of the Mayo Clinic. In 1912, Plummer reported a series of patients with long-standing iron deficiency anemia, dysphagia and spasm of the upper esophagus without anatomic stenosis, which was described as hysterical. In 1919 Vinson reported another case of ‘angulation’ of the esophagus and attributed the first description of this entity to the earlier report of Plummer. Some years later he published a series of patients with dysphagia who were successfully treated with the passage of bougies; most of the patients were women.

Another term is Paterson-Kelly syndrome, named after Donald Ross Paterson (1863–1939) and Adam Brown-Kelly (1865–1941), both British laryngologists, who published their findings independently in 1919. They were the first to describe the characteristic clinical features of the syndrome. Paterson gave the fullest description but without reference to anemia. He was also the first to draw attention to an association with post-cricoid carcinoma. Brown-Kelly not only described the signs and symptoms of the condition, but also considered anemia.


Clinical features

Classic triad:

  • Dysphagia: Painless and intermittent or progressive over years, limited to solids and sometimes associated with weight loss
  • Iron-deficiency anemia: Characterized by glossitis, angular cheilitis and koilonychia (spoon-shaped finger nails)
  • Esophageal webs

Oesophageal/pharyngeal squamous cell carcinoma

3-15 % of the patients with Plummer-Vinson syndrome, mostly women between 15 and 50 years of age develop cancer

Management

The first step in the management of Plummer-Vinson syndrome is to clarify the cause of iron deficiency in order to exclude active hemorrhage, malignancy or celiac disease.

  • Iron deficiency: Iron supplementation
  • Persistent dysphagia:
    • Endoscopic dilatation
    • Argon plasma coagulation therapy

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