A 42-year-old female presented to the Otolaryngology Department with a two year history of progressive dysphagia to solids. She did not have any comorbidities yet complained of fatigue and shortness of breath on exertion. When questioned about her menstrual cycle, the patient was unaware that she was having menorrhagia for several years. Her physical examination was normal with the exception of her skin pallor and platynychia (Figure 1). Hematological investigation revealed a hemoglobin level of 6.5 g/dL, hematocrit of 24%, mean corpuscular volume of 58.5 fL, a red cell distribution width of 19.1%, and a serum ferritin level of 9 ng/mL. Barium esophagogram demonstrated an esophageal web within the proximal esophagus at the level of the C6 vertebral body (Figure 2). A diagnosis of Plummer–Vinson syndrome was made and the patient was referred to gastroenterologist for therapeutic intervention.

Upper gastrointestinal endoscopy showed a focal, tight upper esophageal web (Figure 3). Serial dilation was performed using 11, 13, and 15 mm Savary-Giliard dilators over a guidewire. Adequate dilation was successfully achieved despite moderate resistance with the last two dilators. Post-dilation inspection showed a superficial tear with no signs of bleeding or perforation (Figure 4). The patient had an uneventful post-procedural course and her dysphagia was relieved immediately. She was discharge with oral hematinics.

The patient was evaluated six weeks post-procedure at the outpatient clinic. She had no swallowing complaints and histopathological analysis of the duodenal biopsy specimens revealed no evidence of celiac disease. She was counseled on the risk of post-cricoid carcinoma and the need for long-term surveillance of the upper gastrointestinal tract. Gynecological consultation was requested to investigate the patient’s menorrhagia. A bulky fibroid uterus was discovered during the assessment, for which the patient eventually had a total hysterectomy.

Plummer–Vinson syndrome (PVS) or sideropenic dysphagia is an uncommon entity characterized by dysphagia, iron deficiency anemia, and the presence of an esophageal web [1]. The frequency of reported cases has decreased which may possibly be due to the overall improvement in nutrition and health care [2]. The syndrome occurs more commonly in middle-aged women, approximately 9 times more frequently than men [2],[3]. Although the exact etiopathogenesis of this entity is unclear, several hypotheses have been made, the most popular of which is based on iron-dependent oxidative enzyme deficiency [2],[4]. These iron-dependent oxidative enzymes are crucial for maintaining the epithelial integrity of the upper alimentary canal. As a result, deficiencies of these enzymes result in gradual degradation of pharyngeal musculature, leading to mucosal atrophy and web formation [4],[5]. However, studies have shown that PVS is not naturally endemic in areas of iron deficiency anemia, in fact, only 10% of patients with iron deficiency anemia develop PVS. This suggests that other factors like genetic, environmental, and autoimmune may be involved [2],[6].

Holistic management of the patient with PVS includes investigating and treating the etiology of the chronic iron deficiency anemia, correcting the anemia, and resolving the symptom of dysphagia. Common causes of iron deficiency anemia include gastrointestinal bleeding, nutritional deficiency due to poor intake or malabsorption, gastrointestinal parasites, menorrhagia, and menometrorrhagia [7].

Iron supplementation and mechanical dilation are the mainstay of treating the dysphagia of PVS [8]. Correction of anemia via oral or intravenous route may be sufficient to eradicate the web and resolve dysphagia in patients who are not considerably obstructed. Patients who have failed conservative hematinic management and others who are inherently obstructed like the index case will require endoscopic therapeutic intervention [3]. Plummer–Vinson syndrome is considered a premalignant condition and as such, long-term follow-up of the post-cricoid region is mandatory [1],[3],[9].

Plummer–Vinson syndrome is a rare condition that is defined by the triad of dysphagia, chronic iron deficiency anemia, and the presence of an esophageal web. Management of PVS includes identifying and correcting the underlying cause of the iron deficiency anemia, treating the anemia with hematinics and mechanically resolving the dysphagia as required. Long-term follow-up of these patients are necessary as PVS is considered a premalignant condition for gastrointestinal malignancies.