GI Endoscopy · 2 min read

Plummer-Vinson Syndrome with Celiac Disease and Esophageal Varices

Iron-deficiency anemia, proximal esophageal webs that blocked a standard endoscope, biopsy-proven celiac disease, and varices that improved without banding.

Figure 1: Endoscopic views of the esophageal webs and duodenal mucosa.

Experienced teaching points

Clinical Pearls

  1. Plummer-Vinson belongs on the differential when dysphagia meets iron-deficiency anemia, especially with proximal esophageal webs.
  2. Celiac disease can be the iron-loss driver. Check tTG and biopsy the duodenum even when the presenting problem is a web.
  3. Endoscopic dilation is effective for symptomatic webs that block a standard endoscope.
  4. When webs, varices, and celiac sit in one case, look for the shared systemic thread before treating each finding in isolation.

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Patient: 48-year-old woman with one month of dysphagia and no weight loss
Indication: Dysphagia with iron-deficiency anemia and a markedly elevated tissue transglutaminase
Final diagnosis: Plummer-Vinson syndrome associated with celiac disease, treated with endoscopic dilation and medical management of esophageal varices
Proximal esophageal lumen with a circular opening ahead of the scope
Figure 1A: Proximal esophageal lumen with a circular opening ahead of the scope.
Tight concentric esophageal web
Figure 1B: Tight concentric esophageal web. A standard endoscope could not pass.
Balloon dilation across the esophageal web
Figure 1C: Balloon dilation to traverse the webs.
Duodenal mucosa
Figure 1D: Duodenal mucosa. Biopsies showed total mucosal atrophy.
Low-power duodenal biopsy with villous flattening
Figure 2A: Low-power duodenal biopsy with villous flattening and packed crypts, consistent with total mucosal atrophy.
Higher-power duodenal mucosa with intraepithelial lymphocytes
Figure 2B: Higher-power view with intraepithelial lymphocytes and a hypercellular lamina propria.

Clinical History

A 48-year-old woman presented with one month of dysphagia and no weight loss. Physical examination was unremarkable. Hemoglobin was 8.9 g/dL, MCV 78, transferrin low, and iron 18. Tissue transglutaminase was 10 times the upper limit of normal.

Endoscopic Findings

  1. Esophageal webs that could not be traversed with a standard endoscope.
  2. Esophageal varices.
  3. Duodenal biopsies showed total mucosal atrophy.

Endoscopic Technique

  1. A standard endoscope could not pass the webs.
  2. Careful endoscopic dilation was performed to traverse them.
  3. Variceal banding was not possible because the webs blocked access.
  4. At one-month follow-up the varices were smaller and banding was not required.

Discussion

This is classic Plummer-Vinson: esophageal webs plus iron-deficiency anemia. The authors propose that chronic iron deficiency, driven or worsened by celiac disease, produced the webs. That matches the usual pathophysiology of Plummer-Vinson. The celiac-plus-PVS pairing is still thinly described.

Tight webs blocked a standard endoscope, so dilation came first. Varices were seen but could not be banded through the webs. Duodenal biopsies showed total mucosal atrophy, matching a tTG ten times the upper limit of normal. One month later the varices had shrunk on medical therapy and banding was unnecessary.

Treat the web, then keep looking. Iron deficiency is the link, and celiac can be the source. The varices improved medically. A direct causal tie from PVS or celiac to the varices is not clear from this case, so do not force one. When several GI findings land in the same patient, hunt for the shared driver.

References

  1. Hoffman RM, Jaffe PE. Plummer-Vinson syndrome. A case report and review of the literature. Arch Intern Med. 1995;155(18):2008-2011.
  2. Dickey W, Cullen G. The aetiology of iron deficiency in patients with celiac disease. Scand J Gastroenterol. 1999;34(12):1203-1206.
  3. Genta RM, Gist TL, Hammer RA. Esophageal webs: an unusual cause of dysphagia. Am J Gastroenterol. 1980;73(4):307-310.

About the authors

Jorge Pacheco

Jorge Pacheco, MD, MSc

Gastroenterologist

Division of Gastroenterology and Endoscopy, Hospital Roosevelt, Guatemala

Jorge Pacheco, MD, MSc, is a gastroenterologist in the Division of Gastroenterology and Endoscopy at Hospital Roosevelt in Guatemala.

More articles by Jorge

Ninoska Melendez

Ninoska Melendez, MD

Internist

Division of Gastroenterology and Endoscopy, Hospital Roosevelt, Guatemala

Ninoska Melendez, MD, is an internist in the Division of Gastroenterology and Endoscopy at Hospital Roosevelt in Guatemala.

More articles by Ninoska

Luis Quevedo

Luis Quevedo, MD

Chief, Gastroenterology Service

Division of Gastroenterology and Endoscopy, Hospital Roosevelt, Guatemala

Luis Quevedo, MD, is a gastroenterologist and chief of the gastroenterology service at Hospital Roosevelt in Guatemala.

More articles by Luis

Abel Sanchez

Abel Sanchez, MD

Gastroenterologist

Division of Gastroenterology and Endoscopy, Hospital Roosevelt, Guatemala

Abel Sanchez, MD, is a gastroenterologist in the Division of Gastroenterology and Endoscopy at Hospital Roosevelt in Guatemala and coordinates the gastroenterology and digestive endoscopy master's program at Universidad de San Carlos de Guatemala.

More articles by Abel

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