Upper Esophageal Stenosis, Microcytic Anemia, and Plummer-Vinson Syndrome

CASE REPORT

Keywords: Plummer-Vinson syndrome; Paterson-Brown-Kelly syndrome; upper esophageal stenosis; iron deficiency anemia; dysphagia; esophageal web; balloon dilation; lymphocytic esophagitis; esophageal squamous cell carcinoma; endoscopic surveillance

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Clinical History

A 78-year-old White female with a history of chronic iron deficiency anemia (microcytic anemia) and hypertension was referred for upper endoscopy to evaluate longstanding dysphagia. The patient reported progressive difficulty swallowing solids over several months. Laboratory studies confirmed persistent microcytic anemia consistent with chronic iron deficiency. Given the combination of dysphagia and iron deficiency anemia, Plummer-Vinson syndrome was considered in the differential diagnosis prior to endoscopic evaluation.

Endoscopic Findings

Upper endoscopy revealed a proximal esophageal stenosis located in the upper esophagus. The mucosa overlying the stenosis appeared characteristically shiny, and multiple concentric rings were visible, consistent with an esophageal web. These findings, in the clinical context of chronic iron deficiency anemia and dysphagia, were diagnostic of Plummer-Vinson syndrome. No additional mucosal lesions or masses were identified in the esophagus, stomach, or duodenum.

Endoscopic Technique

Through-the-scope balloon dilation was performed to treat the proximal esophageal stenosis. A controlled radial expansion (CRE) balloon was advanced through the working channel of the endoscope and positioned across the stenosis over a guidewire for stability and safety. The balloon was inflated incrementally, starting at 13 mm and progressing to 15 mm, with careful monitoring for mucosal disruption at each step. This graduated approach minimizes the risk of perforation while achieving adequate luminal expansion. For a comprehensive review of esophageal dilation techniques, including the use of the BougieCap device, see our Technical Review: Esophageal Dilation with the BougieCap.

Biopsies of the esophageal mucosa were obtained coincidentally during the dilation procedure. Histopathological examination revealed lymphocytic esophagitis, characterized by increased intraepithelial lymphocytes within the esophageal squamous epithelium. No dysplasia, eosinophilic infiltration, or malignancy was identified. The procedure was completed without complications, and the patient tolerated it well.

Discussion

Plummer-Vinson syndrome (PVS), also known as Paterson-Brown-Kelly syndrome, is an exceedingly rare condition characterized by the classic triad of chronic iron deficiency anemia, dysphagia, and the presence of esophageal webs or rings. The syndrome predominantly affects middle-aged and elderly women and has become increasingly uncommon in developed countries, likely due to improved nutritional status and early treatment of iron deficiency. Patients may also present with glossitis, angular cheilitis, and koilonychia — accessory findings directly attributable to chronic iron deficiency anemia.

The diagnosis of PVS is typically established through barium swallow, which may demonstrate a thin web in the postcricoid region, or through upper endoscopy, which allows both direct visualization and therapeutic intervention. In our patient, the endoscopic appearance of a proximal esophageal stenosis with a shiny mucosal surface and multiple rings, combined with the clinical history of chronic microcytic anemia and dysphagia, confirmed the diagnosis. Esophageal stenosis in the upper esophagus can also arise from other etiologies, including radiation injury; for a related case involving radiation-induced stenosis, see Endoscopic Resection of an Esophageal Pseudodiverticulum in Radiation-Induced Stenosis.

The primary therapeutic interventions for PVS include esophageal dilation to alleviate dysphagia and iron supplementation to correct the underlying deficiency. Through-the-scope balloon dilation, as performed in this case, is safe and effective for disrupting esophageal webs and restoring luminal patency. Repeat dilation sessions may be necessary in some patients, particularly if iron deficiency is not adequately corrected. Another rare esophageal condition that may present with dysphagia and require dilation is esophageal intramural pseudodiverticulosis, which shares some overlapping endoscopic features but has a distinct pathophysiology.

Importantly, PVS is recognized as a premalignant condition. Patients with PVS carry an increased risk of developing esophageal squamous cell carcinoma, as well as hypopharyngeal carcinoma. The exact mechanism linking iron deficiency, esophageal webs, and carcinogenesis remains incompletely understood, but chronic mucosal atrophy and epithelial changes secondary to iron deficiency are thought to play a role. Therefore, lifelong endoscopic surveillance is recommended to facilitate early detection and treatment of potential malignancies.

A notable finding in this case was the histopathological diagnosis of lymphocytic esophagitis in the biopsies obtained during dilation. Lymphocytic esophagitis is a relatively recently described entity characterized by peripapillary lymphocytic infiltration of the esophageal squamous epithelium. It has been associated with various conditions, including Crohn's disease, motility disorders, and immune-mediated processes, but its association with microcytic anemia in the context of Plummer-Vinson syndrome has not been previously described in the literature. Whether lymphocytic esophagitis represents a reactive process secondary to chronic iron deficiency, a coincidental finding, or a pathogenic contributor to web formation in PVS remains to be elucidated. This novel observation warrants further investigation in larger patient cohorts.