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Plummer-Vinson Syndrome with Improvement After Iron Repletion

Valerian Mwenda1, Jackson Njuguna2, Ancent Nzioka2.

  1. Department of Internal Medicine and Endoscopy, Consolata Hospital Nkubu

  2. Department of Pathology, School of Medicine, Kenyatta University.

Correspondence to: Dr. Valerian Mwenda P.O Box 205-60200 Meru, Kenya.

Email: valmwenda@gmail.com

Abstract

Plummer-Vinson Syndrome (PVS) is a rare disorder characterized by upper esophageal webs and iron deficiency anemia and is even more uncommon among people of African descent. We report a case of this syndrome in a middle-aged black woman, which improved on iron-repletion therapy alone. The patient presented with long-standing dysphagia and symptomatic anemia. Gastrointestinal endoscopy revealed an upper esophageal web arising from the anterior wall. She was started on iron therapy daily and after 2 months both the dysphagia and anemia had improved significantly. Therefore, though this is a relatively rare condition in Africa, it still warrants being part of the differential diagnosis for dysphagia especially in the setting of anemia.

 

Keywords: Plummer-Vinson Syndrome, Anemia, Dysphagia, Endoscopy

Ann Afr Surg. 2016;13(2): 81-3.

DOI: http://dx.doi.org/10.4314/aas.v13i2.11

Introduction

Plummer-Vinson syndrome (synonyms: Paterson-Kelly Syndrome, Paterson-Brown Kelly syndrome, Sideropenic dysphagia), is defined by the classic triad of dysphagia, iron deficiency anemia and esophageal webs (1). During the first half of the 20th century the syndrome was common among middle-aged Caucasian women (2), but the prevalence has fallen over the years. The fall in prevalence has largely been attributed to improvement in nutrition in areas where it used to be common, although in Africa where prevalence of iron deficiency and malnutrition is still high, the syndrome is rare (1). The syndrome has also been described in children and adolescents (3, 4, 5). We present a case of a middle aged woman of African descent with classic symptoms of the syndrome and who showed a very remarkable improvement in the dysphagia after two months of iron repletion therapy. The case report is presented to remind clinicians of the existence of this rare disorder and the diagnostic approach, especially differentiating it with other causes of dysphagia.

 

Case Presentation

A 34 year old woman was referred to our health facility with history of dysphagia to solids over the preceding three years and easy fatigability and intermittent dizziness over the preceding seven years. She could swallow liquids and very thin semi-solids, and reported gradual loss in weight. There was no cough or odynophagia. Her menses were regular and normal in amount. There was no history of melena stool or hematochezia. Her last delivery was 10 years prior to presentation. There was no familial history of similar symptoms or cancer. She had received short course iron supplementation in other hospitals with intermittent relief of the symptoms. In the referring hospital, upper intestinal endoscopy had been attempted but failed to successfully intubate the upper esophagus. On examination the patient was not in distress, had conjuctival pallor and tachycardia, and was moderately wasted. A full blood count showed a red cell count of 4.33×1012/ L( reference; 4.10-5.90 ×1012/L), hemoglobin level of 6.5g/dl , hematocrit of 23.5% (reference range 38-46%), mean corpuscular volume of 59 fl ( reference range, 80-100 fl) and mean corpuscular hemoglobin of 17.9 pg ( reference range; 28-32 pg).The platelets, white cell count and differential counts were all within the reference ranges, as were the liver and renal function tests. While adequately anaesthetized, upper gastrointestinal endoscopy was done. A web was visualized on the upper esophagus, approximately 2 cm below the upper esophageal sphincter; it had a smooth appearance and a narrowed, eccentric lumen was visible. The endoscope was maneuvered slowly across the narrowed lumen, in the process tearing part of the web. The web was originating from the anterior wall of the esophagus as shown in figure 1. The rest of the esophagus was normal. The stomach mucosa and wall were very transparent, with the mucosal blood vessels vey prominent, as depicted in figure 2. The first and second parts of the duodenum were normal. Samples were obtained from the web and