Plummer-Vinson Syndrome

September 15, 2006

A 44-year-old African American woman presented to the emergency department with constipation of 4 days' duration. She also complained of a sticking sensation in her throat over the past year and dysphagia to solids but not liquids. Over the past 2 years, she had an unintentional 40-lb weight loss, which she attributed to decreased appetite.

A 44-year-old African American woman presented to the emergency department with constipation of 4 days' duration. She also complained of a sticking sensation in her throat over the past year and dysphagia to solids but not liquids. Over the past 2 years, she had an unintentional 40-lb weight loss, which she attributed to decreased appetite.

Two months earlier, the patient began to have shortness of breath on exertion; she had to rest after walking a few blocks and became dizzy when she rose quickly from a sitting position. She denied chest pain, orthopnea, and paroxysmal nocturnal dyspnea. Her menstrual history was significant for menorrhagia of 1 year's duration.

The patient had pale conjunctivae, bilateral angular cheilitis (A), glossitis (B), and bilateral spoon-shaped nails (koilonychia) (C). The white blood cell count was 7300/µL; hemoglobin level, 5.7 g/dL; platelet count, 555,000/µL; mean corpuscular volume, 55 fL; red blood cell distribution width, 22%; and ferritin, 6 ng/mL.

A pelvic ultrasonogram showed an enlarged uterus and multiple fibroids; the largest lesion was 5 cm. An upper GI barium study disclosed a cervical web on the anterior part of the esophagus (D). Upper endoscopy revealed thickening of the esophageal mucosa and a web, which was ruptured with the scope.

Mayada Issa, MD, Linda Green, MD, Jalil Ahari, MD, and Kalai Ayyanar, MD, of Prince George's Hospital Center, Cheverly, Md, diagnosed Plummer-Vinson syndrome (PVS) based on the following findings:

•History of painless dysphagia and the finding of an esophageal web.

•Presence of glossitis, angular cheilitis, and koilonychia.

•Iron deficiency anemia caused by the long-standing menorrhagia secondary to fibroids.

PVS occurs most frequently in women aged 30 to 70 years.1 The syndrome is characterized by a triad of dysphagia, an esophageal web, and iron deficiency anemia.2 It has been associated with other causes of iron deficiency, such as celiac disease3 and a large diaphragmatic hernia that causes chronic blood loss.1

Iron repletion often ameliorates symptoms; however, some patients may require esophageal dilatation or bougienage.1,4 Because PVS is associated with an increased incidence of postcricoid carcinoma, patients with the syndrome require yearly endoscopic surveillance.4,5

This patient was transfused with 2 units of packed red blood cells followed by intravenous iron therapy. Her dysphagia and constipation resolved within a few days, and she was discharged. She was advised to take an oral iron supplement daily and to follow up with her gynecologist. *

References:

REFERENCES:

1.

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Am J Gastroenterol.

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2.

Lopez Rodriguez MJ, Robledo Andres P, Amarilla Jimenez A, et al. Sideropenic dysphagia in an adolescent.

J Pediatr Gastroenterol Nutr.

2002;34:87-90.

3.

Dickey W, McConnell B. Celiac disease presenting as the Paterson-Brown Kelly (Plummer-Vinson) syndrome.

Am J Gastroenterol

. 1999;94:527-529.

4.

Hoffman RM, Jaffe PE. Plummer-Vinson syndrome. A case report and literature review.

Arch Intern Med.

1995;155:2008-2011.

5.

Godino J, Wong PW. A triad of troubling findings. Plummer-Vinson syndrome.

Postgrad Med.

2000;108: 109-110.