Image Diagnosis: Plummer-Vinson Syndrome: An Unusual Cause of Dysphagia

Image Diagnosis: Plummer-Vinson Syndrome: An Unusual Cause of Dysphagia

 

Puneet Chhabra, MD, DM; Hunny Khurana, MD

Perm J 2018;22:18-035 [Full Citation]

https://doi.org/10.7812/TPP/18-035
E-pub: 06/18/2018

Case Presentation

A 50-year-old woman was referred to our gastroenterology clinic for an upper gastrointestinal endoscopy for mechanical dysphagia. She presented with 25 years of dysphagia predominantly to solids without any significant loss of weight or appetite. On examination she had pallor and koilonychia. Blood tests revealed a hemoglobin of 7.8 gm/dL with peripheral blood film and iron studies suggestive of iron-deficiency anemia. She denied any history of blood loss. She had been dewormed with albendazole twice in the preceding 6 months. Her immunoglobulin A tissue transglutaminase serology was negative. Barium swallow revealed a short segment filling defect in the midcervical esophagus with narrowing (Figure 1) and a jet effect of the barium passing distally to the lesion (Figure 2).

Upper gastrointestinal endoscopy showed a postcricoidal web with marked narrowing of the esophagus (Figure 3). A Savary-Gilliard guidewire (Cook Medical, Bloomington, IN) was passed under endoscopic vision through the esophageal narrowing, and the web was fractured using a conventional gastroscope. Successful dilatation was confirmed by the presence of blood at the site of the web and by the gastroscope passing distally to the esophageal narrowing (Figure 4). The rest of the esophagus, stomach, and duodenum appeared normal (normal fold height and number with no grooving or scalloping). No immediate or delayed complications were seen.

We made the diagnosis of Plummer-Vinson syndrome. The patient was started on oral iron supplementation and counseled regarding the need for surveillance endoscopy. At last follow-up six months after diagnosis, she was doing well without any dysphagia.

Image Diagnosis: Plummer-Vinson Syndrome: An Unusual Cause of Dysphagia

Image Diagnosis: Plummer-Vinson Syndrome: An Unusual Cause of Dysphagia

Discussion

Plummer-Vinson syndrome is a rare disorder characterized by the triad of dysphagia, postcricoidal web, and iron-deficiency anemia.1 It is predominantly seen in women aged 40 to 70 years, although it also has been reported in young adolescents and children.2,3 The exact pathophysiology of the disease remains enigmatic, and theories of nutritional deficiencies including iron deficiency, genetic predisposition, and autoimmunity have been described in the literature.4 The majority of patients recover with iron supplementation and conservative management, but a few patients have required esophageal dilatation for relief of dysphagia.5 Various modalities have been described for esophageal dilatation, including dilatation and fracture of the web by the gastroscope itself, bougie dilatation, electro-incisional therapy, argon plasma coagulation, and surgical excision.6 Because Plummer-Vinson syndrome is associated with increased risk of esophageal cancer (squamous cell carcinoma), surveillance endoscopy is recommended although its role and timing is still an issue of debate.5

Disclosure Statement

The author(s) have no conflicts of interest to disclose.

Authocknowledgments

Puneet Chhabra, MD, DM, cared for the patient and contributed critical revision of the manuscript. Hunny Khurana, MD, contributed data acquisition, literature review, and writing the manuscript.

How to Cite this Article

Chhabra P, Khurana H. Image diagnosis: Plummer-Vinson syndrome: An unusual cause of dysphagia. Perm J 2018;22:18-035. DOI: https://doi.org/10.7812/TPP/18-035

References
1.    Novacek G. Plummer-Vinson syndrome. Orphanet J Rare Dis 2006 Sep 15;1:36. DOI: https://doi.org/10.1186/1750-1172-1-36.
    2.    Wynder EL, Hultberg S, Jacobsson F, Bross IJ. Environmental factors in cancer of the upper alimentary tract. A Swedish study with special reference to Plummer-Vinson (Paterson-Kelly) syndrome. Cancer 1957 May-Jun;10(3):470-82. DOI: https://doi.org/10.1002/1097-0142(195705/06)10:3<470::aid-cncr2820100309>3.0.co;2-7.
    3.    Anthony R, Sood S, Strachan DR, Fenwick JD. A case of Plummer-Vinson syndrome in childhood. J Pediatr Surg 1999 Oct;34(10):1570-2. DOI: https://doi.org/10.1016/s0022-3468(99)90134-3.
    4.    Goel A, Bakshi SS, Soni N, Chhavi N. Iron deficiency anemia and Plummer-Vinson syndrome: Current insights. J Blood Med 2017 Oct 19;8:175-84. DOI: https://doi.org/10.2147/jbm.s127801.
    5.    Hoffmann RM, Jaffe PE. Plummer-Vinson syndrome. A case report and literature review. Arch Intern Med 1995 Oct 9;155(18):2008-11. DOI: https://doi.org/10.1001/archinte.155.18.2008.
    6.    Bakari G, Benelbarhdadi I, Bahije L, El Feydi Essaid A. Endoscopic treatment of 135 cases of Plummer-Vinson web: A pilot experience. Gastrointest Endosc 2014 Oct;80(4):738-41.
DOI: https://doi.org/10.1016/j.gie.2014.05.332.

etoc emailClick here to join the eTOC list or text TPJ to 22828. You will receive an Email notice with the Table of Contents of each issue.

The Permanente Journal advances knowledge in scientific research, clinical medicine and innovative health care delivery. It is a peer-reviewed journal of medical science, social science in medicine, and medical humanities.

The Permanente Press

The Permanente Press publishes The Permanente Journal and books related to health care. For information about subscriptions, missing issues, billing, subscription renewal, and back issues, Email: permanente.journal@kp.org.

Circulation

27,000 print readers per quarter, 15,350 eTOC readers, and in 2018, 2 million page views of TPJ articles in PubMed from a broad international readership.

CME

The Kaiser Permanente National CME Program designates this journal-based CME activity for 4 AMA PRA Category 1 Credits. Physicians should claim only the credit commensurate with the extent of their participation in the activity.


ISSN 1552-5767 Copyright © 2019 thepermanentejournal.org.

All Rights Reserved.