A case of Plummer Vinson syndrome in an elderly female

Maria Mathew

DNB Gen Medicine, Kauvery Hospital, Hosur, Tamil Nadu

Abstract

Plummer Vinson syndrome is a rare condition characterized by the triad of iron deficiency anemia, dysphagia and esophageal webs, which is less frequently encountered in the elderly population, potentially leading to diagnostic delays. We report a 72-year-old female who presented with severe anemia, dysphagia. On Oesophago Gastro Dudenoscopy she was found to have cricopharyngeal web and underwent endoscopic dilatation. This abstract discusses the clinical presentation of PVS in older adults and emhasizes the role of iron therapy and endoscopic dilatation alleviating symptoms and potentially mitigate the elevated risk of upper aerodigestive tract cancers associated with this syndrome in this vulnerable population.

Introduction

Plummer-Vinson syndrome also known as Paterson Kelly syndrome classically presents as a triad of iron-deficiency anemia, post-cricoid dysphagia, and upper esophageal webs. Data on the incidence and prevalence of Plummer-Vinson syndrome are not widely available due to the rarity of this syndrome. But it’s more commonly found in middle aged women in developing countries. However, it’s rare in elderly group and among males.The malfunctioning of iron-dependent oxidative enzymes produces myasthenic changes in muscles responsible for swallowing, atrophy of the esophageal mucosa, and formation of webs as epithelial complications. Repeated injury to epithelia due to iron deficiency leads to atrophy of mucosa and degradation of pharyngeal muscles, leading to the development of esophageal webs. The post-cricoid region experiences maximum trauma while swallowing the solid bolus, leading to an increased risk of web formation. Here we report a case of PVS in elderly which was treated with blood transfusion, iron supplementation and endoscopic dilatation.

Case Presentation

A 72-year-old female presented with acute gastroenteritis was found to have severe pallor, and she gave a history of difficulty in swallowing for few months and decreased intake of food and generalised tiredness. She had previous history of anemia. There was no history of significant weight loss. No history suggestive of blood loss. On clinical examination she was found to have severe pallor, and ejection systolic murmur. Lab investigation showed Hb:4.6Gm%, and MCV :54.9 .USG abdomen and pelvis was normal .Stool occult blood was negative. She was planned for endoscopy and was found to have cricopharyngeal web and underwent dilatation. Iron Profile study showed Iron 10, Ferritin 12.39, Serum Transferrin 322.99, TIBC 401, Transferrin saturation 2.49%, unsaturated iron binding capacity 391.She was managed with 2 units of PRBC transfusion followed by Iron injection. At discharge her Hb improved to 7.5 and dysphagia was also settled and sent home with iron and folic acid supplements along with nutritional advice.

Discussion

Iron is an important element for normal body homeostasis. It is essential for hemoglobin and myoglobin synthesis and also acts as a cofactor for several enzymes involved in cellular metabolism. The origin of esophageal web is proposed to be related to a reduction in the activity of iron-dependent oxidative enzymes, secondary to iron deficiency. This results in gradual degradation of the pharyngeal muscles and atrophy of the mucosa overlying them, leading to the development of webs. This hypothesis is supported by evidence from a histochemical study of thyropharyngeal, cricopharyngeal and cervical esophageal muscles from rabbits with IDA, which showed mitochondrial damage, with a “moth eaten” appearance, in type I muscle fibers.

Hematological testing is done to ascertain the cause of iron deficiency and the severity of anemia. Complete blood count, peripheral smear, and iron studies (eg, serum iron, ferritin, total iron-binding capacity [TIBC], transferrin saturation) should be carried out to confirm the diagnosis of iron deficiency anemia. Dysphagia can be evaluated by barium swallow, which is easily accessible even in remote areas. However, Fiberoptic endoscopy is the safest and most reliable tool for GI tract examination, OGD an endoscopic examination can be both diagnostic and therapeutic in the same setting. During endoscopy esophageal webs appear as smooth, thin, gray lesions with normal-appearing mucosa and a central or lateral lumen and are most commonly located on the anterior wall of the esophagus.

Patients with Plummer-Vinson syndrome have an excellent outcome, with most symptomatic patients requiring only one esophagogastroduodenoscopy with dilatation for complete relief of symptoms in conjunction with iron replacement therapy. Patients are at an increased risk of developing squamous cell carcinoma of the hypopharynx or upper esophagus, which may be related to chronic iron deficiency. This is believed to cause irreversible mucosal changes leading to malignant degeneration

Although autoimmune, genetic, infectious, and nutritional factors have been proposed, iron deficiency garnered importance as the major etiologic factor as most cases have shown improvement of the dysphagia after iron therapy. Variety of disorders have been known to associate PVS such as celiac disease, inflammatory bowel disease, pernicious anemia, thyroid disease, Sjogren’s disease, and rheumatoid arthritis.

The differential diagnosis includes all other causes of dysphagia especially malignant tumors, benign strictures or esophageal rings like diverticula, motility disorders such as achalasia, spastic motility disorders, scleroderma, diabetes mellitus, gastroesophageal reflux disease, and neuromuscular and skeletal muscle disorders which was ruled out in this case.

Conclusion

This case highlights the importance of considering Plummer-Vinson syndrome in elderly patients presenting with iron deficiency anemia and dysphagia, as timely diagnosis and appropriate management can significantly improve their quality of life and prevent potential complications. Further follow-up is warranted to monitor for recurrence of the esophageal web and to ensure sustained improvement in her iron status.

Reference

Plummer Vinson, Stat pearls, NIH National Library of medicine
Novacek G. Plummer‑Vinson syndrome. Orphanet J Rare Dis 2006; 1:36
Revisting Plummer Vinson Syndrome Case report. Gude D, Bansal DP,Malu A

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A case of Plummer Vinson syndrome in an elderly female