Plummer -Vinson Syndrome: Diagnosis by Barium Esophagogram Studies May Still Be Important in the Shortage of Endoscopic Sources

Background: Plummer-Vinson syndrome (PVS), association of iron deficiency with dysphagia due to proximal esophageal web, is still a relevant cause of dysphagia in Western societies as well as developing countries. This prospective study aims to emphasize PVS and iron deficiency prevalence in patients with dysphagia, evaluate utility of barium esophagograms in diagnosis of esophageal webs and assess efficacy of endoscopic treatment using a valid dysphagia scaling system. Methods: Patients presenting with esophageal dysphagia were graded by Dysphagia Outcome and Severity Scale (DOSS) and 2809 adults underwent barium esophagogram studies prior to endoscopic examination. Esophageal stenosis ratios, prestenotic dilatation and diameter of web openings were measured from esophagograms. Blood samples were obtained for anemia work-up. Patients were reassessed by DOSS after endoscopic dilation therapy. Results: Ninety-nine out of 2809 esophagograms (3.5%) depicted esophageal webs all of which were endoscopically confirmed. PVS cases were predominantly premenopausal women. Mean esophageal stenosis ratio was 40.1%. There was a weak inverse correlation between prestenotic dilatation and diameter of web opening (rho=- 0.350, p=0.001). There were no associations between hematologic parameters and fluoroscopic measurements. There was no correlation between pretreatment DOSS scores and stenosis ratios (rho=-0.221, p=0.127). All patients did benefit from endoscopic dilation and iron supplementation. Conclusion: We could not find any correlation between hematologic data and fluoroscopic measurements. All patients benefited from endoscopic dilation therapy with very low complication ratio. Although current trends favor endoscopy as the initial test for dysphagia, barium studies may still remain as an initial step in the diagnostic algorithm of dysphagia, especially in the shortage of endoscopic unit sources. Esophagogram


Introduction
and lower iron deficiency prevalence, there has been only anecdotal reports from populations in which PVS had been originally described. But PVS is still prevalent in developing countries affecting both genders, all age groups, and ethnicities. [3] Recognition of PVS is important because, apart from being a more prevalent cause of treatable dysphagia in certain populations, it represents a precancerous condition with increased risk of squamous cell carcinoma of pharynx and esophagus. PVS may also be a remote manifestation of concomitant, but undetected, pathologic processes. [4,5] This study presents a large series of PVS verified by barium esophagogram and treated with esophagoscopic intervention. Epidemiologic, clinical, and biochemical aspects of PVS are reviewed and diagnostic algorithm is reassessed based on work-up results reported from within a larger series of dysphagia cases.

Materials and Methods
This prospective study was conducted between June 2008 and September 2011 as a collaboration among radiology, otorhinolaryngology, and gastroenterology clinics of a district-level referral hospital for rural health centers serving a population of 1.1 million people. In that area, only one gastroenterology doctor was serving at that time. So, there was a shortage for reaching endoscopic unit services. Patients presenting with dysphagia were evaluated in gastroenterology and otorhinolaryngology clinics by detailed history of symptoms and thorough physical examination.
Patients with symptoms suggesting esophageal dysphagia, i.e., difficulty in transporting ingested material down the esophagus as opposed to oropharyngeal dysphagia which is the inability to initiate swallowing and failure of bolus transfer from the mouth to the esophagus, were further assessed by grading of symptoms using Dysphagia Outcome and Severity Scale (DOSS). [6] This test, a simple and practical 7-point scale for rating functional severity of dysphagia, is based on level of independence, nutrition type and diet level and has proven to have favorable inter and intra-rater reliability (Table 1). [7] A total of 2809 adult patients were referred for barium esophagogram studies to provide a roadmap prior to upper endoscopy.
Studies were performed using standard and modified barium swallow and esophagography protocols with single-contrast examinations incorporating a full column of thin-barium suspension, i.e., 40 -80% w/v, to provide optimal luminal distension and enable motility assessment. [8][9][10] Barium studies were examined by two radiologists experienced in performing and interpreting barium esophagograms. Radiographic diagnosis of proximal esophageal web, defined as a very short segment of acute narrowing in the barium column at cervical levels, was made by consensus of the two radiologists.
Following measurements were recorded: a. Narrowest width of the barium column at the level of the web, b. Maximum width of the barium column at the prestenotic dilated segment, and c. Diameter of normal-looking esophagus distal to the web. Ratio of prestenotic dilatation to poststenotic normal esophagus, and stenosis ratio were derived from the measurements  b.
[C] Measurements on barium esophagograms (a: narrowest width of barium column, b: maximum width of the barium column at prestenotic dilated segment, and c: diameter of normal-looking esophagus distally).  B. Endoscopy emonstrating a circumferential esophageal web just below the upper esophageal sphincter.
C. Endoscopic view during insertion of a metallic guidewire through the web opening prior to bougie dilation.

Results
Ninety-nine out of 2809 esophagograms (i.e.,3.5%) depicted esophageal webs all of which were later confirmed endoscopically by direct visualization as smooth, thin, grayish membranes with eccentric or central openings. Ninety-one out of 99 cases were female (92%) and seventy-five percent of female patients were premenopausal. Patient ages ranged 18 to 84 years with a mean of 46.7±14.1 years. Age distribution among genders were statistically insignificant. Anemia work-up revealed sideropenia, hence confirming diagnosis of PVS (Table 2). Esophageal stenosis ratios ranged from 6% to 97% (mean 40.1 ± 23.2 percent). Ratio of prestenotic dilatation to poststenotic normal esophagus varied between 1.04 and 3.31 (mean 1.51 ± 0.41). There was no significant correlation between the two derived ratios (rho=0.198, p=0.071).
There was a weak, albeit statistically significant, correlation between width of prestenotic dilatation and diameter of web opening (rho= -0.350, p=0.001). On the other hand, there were no statistically significant associations between hematologic parameters and fluoroscopic measurements or derived ratios    It is highly probable that anemia states had been previously noticed in patients, and treatments such as dietary or parenteral iron supplementation, and even blood transfusions, were attempted before dysphagia became a major symptom. This, we believe, may arthritis -may be left undiagnosed. [3,19] Underdiagnosis of PVS is not of trivial importance because it is considered not only as a precancerous lesion itself, but also as a sign of malignancy elsewhere. [11,15,20] It has been proposed that barium swallow was more cost effective than upper endoscopy for diagnosis of abnormal esophageal motility, and in contrast, initial endoscopy with therapeutic intent was less costly for patients suspected with history suggesting benign obstruction. [ populations. Genetic underlying factors must also be further evaluated to reach universal conclusions. [3,5,11] In conclusion, Plummer -Vinson syndrome must be regarded as a sign reflecting potentially serious problems in both the individual's health and the public healthcare system, and not as an ancient and extinct disease. Despite the decline in referrals for barium swallow studies due to current trends favoring endoscopy, interdisciplinary cooperation is still the key to reducing underdiagnosis of PVS.
Barium studies are good in the detection of upper esophageal webs, and they provide an accurate roadmap for endoscopic intervention which substantially eases the process for both the patient and the endoscopist. Thus, barium studies are still indispensable and still may remain as an initial step in diagnostic algorithm of dysphagia, especially in the shortage of endoscopy unit sources.

Conflict of Interest Statement
All authors certify that they have no affiliations with or involvement in any organization or entity with any financial interest (such as honoraria; educational grants; participation in speakers' bureaus; membership, employment, consultancies, stock ownership, or other equity interest; and expert testimony or patentlicensing arrangements), or non-financial interest (such as personal or professional relationships, affiliations, knowledge or beliefs) in the subject matter or materials discussed in this manuscript.