Plummer-Vinson Syndrome

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.

Maleki D, Cameron AJ. Plummer-Vinson syndrome associated with chronic blood loss anemia and large diaphragmatic hernia.

Am J Gastroenterol.

2002;97:190-193.

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.