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

CASE REPORT

Álvaro Martínez-Alcalá, MD, Klaus Mönkemüller, MD, PhD, FASGE, FESGE, FJGES, Professor of Medicine, Department of Gastroenterology, Virginia Tech Carilion School of Medicine

Final Diagnosis

Upper esophageal stenosis due to Plummer-Vinson syndrome with concomitant lymphocytic esophagitis, treated with endoscopic balloon dilation.

Patient Demographics

A 78-year-old White female with a history of chronic iron deficiency anemia and hypertension.

Clinical History

The patient presented with chronic dysphagia. The indication for upper endoscopy was evaluation of chronic dysphagia.

Endoscopic Findings

Upper endoscopy revealed a proximal esophageal stenosis. The mucosa of the stenosis appeared shiny, and multiple rings were visible. Biopsies were obtained coincidentally with dilation.

Endoscopic Technique

1. An endoscopic procedure was performed to address the esophageal stenosis.
2. Through-the-scope balloon dilation was utilized.
3. A controlled radial expansion balloon was advanced over a guidewire.
4. The balloon was inflated incrementally from 13 mm to 15 mm.
5. Biopsies of the esophageal mucosa were obtained. Histopathology of the biopsies revealed lymphocytic esophagitis. No complications occurred during the procedure.

Discussion

Plummer-Vinson syndrome, also known as Paterson-Brown-Kelly syndrome, is an exceedingly rare condition characterized by the triad of chronic iron deficiency anemia, dysphagia, and the presence of esophageal webs or rings. Patients may also present with glossitis, angular cheilitis, and koilonychia; these accessory findings are attributable to iron deficiency anemia. The diagnosis of Plummer-Vinson syndrome is typically established via barium swallow or upper endoscopy. The primary therapeutic interventions include esophageal dilation to alleviate dysphagia and iron supplementation to correct the underlying iron deficiency. Plummer-Vinson syndrome is recognized as a premalignant condition with an increased risk of esophageal squamous cell carcinoma; therefore, these patients require regular endoscopic surveillance to facilitate early detection and treatment of potential malignancies. The association of lymphocytic esophagitis with microcytic anemia in the context of Plummer-Vinson syndrome has not been previously described in the literature.

Key Learning Points

1. Plummer-Vinson syndrome is a rare condition presenting with iron deficiency anemia and dysphagia due to esophageal webs or rings.
2. Endoscopic dilation is effective for symptomatic relief of dysphagia in Plummer-Vinson syndrome.
3. Patients with Plummer-Vinson syndrome require lifelong endoscopic surveillance due to the increased risk of esophageal cancer.
4. Lymphocytic esophagitis may be an incidental finding in patients with Plummer-Vinson syndrome and requires further investigation regarding its clinical significance in this context.

References

1. Goel R, Maydonovitch CL, Arora M, et al. Plummer-Vinson syndrome: A rare and forgotten cause of dysphagia. Am J Case Rep. 2017;18:1052-1056.
2. Hoffman RM, Jaffe PE. Plummer-Vinson syndrome. A case report and review of the literature. Arch Intern Med. 1995;155(18):2008-2011.
3. Drossman DA. Esophageal dilation. Endoscopy. 2017;49(12):1201-1202.