Plummer-Vinson Syndrome/Paterson Kelly Syndrome

In 1912 Plummer described dysphagia and spasm of the upper oesophagus without anatomical stenosis in patients with longstanding iron deficiency. In 1919, Vinson, Paterson and Kelly independently described patients with similar findings. The triad of iron deficiency anaemia, dysphagia and oesophageal webs has been called Plummer-Vinson syndrome or Paterson-Kelly syndrome (depending in the side of Atlantic you reside) after these physicians. It is a rare disease possibly related to iron deficiency and is also known as sideropenic dysphagia.

Etiology and Pathogenesis

Etiology of Plummer –Vinson syndrome is not known, iron deficiency is the most likely cause but malnutrition and genetic predisposition may contribute. Plummer-Vinson syndrome has been associated with coeliac disease, thyroiditis and rheumatoid arthritis raising the possibility of autoimmunity playing a role in Plummer-Vinson syndrome.

The oesophagus of patients with Plummer –Vinson syndrome shows webs at the junction of the hypopharynx and oesophagus, usually extends backwards from the anterior wall. The webs may take a cuff shape and the opening may be reduced to a pinhole. Oesophageal stricture may be seen. Histologically the webs are made of normal oesophageal epithelium, sometimes with chronic inflammation.

Clinical Manifestations

Plummer –Vinson syndrome is a mainly affects women in the fourth to the seventh decade of life but has been described in other age groups. The patients present with gradual onset of dysphagia, initially for solids. Examination reveals evidence of iron deficiency including pallor, koilonachia, glossitis and cheilosis. Splenomegaly and thyroid enlargement may occur.

Diagnosis

The webs are best demonstrated videofluoroscopically by a barum swallow. Endoscopically they appear as 2-3mm smooth grey thin extension of normal mucosa usually with an eccentric lumen. Endoscopy can rupture the webs and the scope should be introduced under direct vision. Laboratory investigations show hypochromic microcytic anaemia, low transferrin saturation and low serum ferritin characteristic of iron deficiency.

Treatment

The iron deficiency should be treated. Dysphagia may be treated by dilatation or endoscopic disruption using a biopsy forceps. The prognosis is good. Patinets with Plummer –Vinson syndrome are predisposed to carcinoma of pharynx and oesophagus and a close follow up of patients is needed.

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