Esophageal webs are defined as extensions of normal esophageal tissue and may develop at any point along the esophagus. They are primarily encountered in patients suffering from Plummer-Vinson syndrome, in which iron deficiency is the presumed cause. Patients may be asymptomatic or can develop dysphagia. The diagnosis is confirmed by both invasive and noninvasive imaging studies.
Presentation
An esophageal web, described as a thin membranous extension of the esophageal mucosa and submucosa, is assumed to be present in about 10% of the population and is asymptomatic in virtually all cases [1] [2]. Its potential protrusion into the lumen of the esophagus (most commonly in the proximal portion) and compromise of food passage, however, can occur in certain disorders, such as Plummer-Vinson syndrome, where iron deficiency, the principal pathophysiological event, is assumed to promote esophageal web development [1] [3] [4] [5]. Moreover, various studies have associated esophageal webs with bullous pemphigoid, pemphigus vulgaris, epidermolysis bullosa, gastroesophageal reflux disease (GERD), glossitis, and desquamative esophagitis in graft-versus-host disease (GVHD) [2]. Regardless of the etiology, the principal symptom that could be triggered by an esophageal web is dysphagia, which may be initially painless and intermittent [2] [3]. More severe symptoms, on the other hand, include nasopharyngeal reflux, aspiration (and consequent pneumonia) and even spontaneous perforation in the setting of significant obstruction [2]. Among adults, a minor but notable predilection toward women has been observed [2] [6]. If Plummer-Vinson syndrome is the underlying cause of esophageal webs, accompanying signs are brittle hair, thinning of nails, glossodynia, angular cheilitis, and koilonychia, as well as constitutional symptoms due to anemia - fatigue, pallor, tachycardia and weakness [3] [4].
Entire Body System
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Weight Gain
In the last 2 years, he developed progressive dysphagia, anorexia, vomiting, and poor weight gain. An esophagoscopy and barium esophagogram revealed an esophageal web in the distal third of the esophagus. [ncbi.nlm.nih.gov]
Gastrointestinal
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Dysphagia
The web was easily ruptured upon endoscopy with subsequent bouginage being performed twice leading to the complete relief of dysphagia. [ncbi.nlm.nih.gov]
Iron deficiency clearly can precede dysphagia. [emedicine.com]
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Abdominal Mass
Other worrying features include rapidly progressive symptoms, weight loss, low haemoglobin and an abdominal mass [ 4 ]. [patient.info]
Skin
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Brittle Hair
If Plummer-Vinson syndrome is the underlying cause of esophageal webs, accompanying signs are brittle hair, thinning of nails, glossodynia, angular cheilitis, and koilonychia, as well as constitutional symptoms due to anemia - fatigue, pallor, tachycardia [symptoma.com]
Workup
The differential diagnosis of dysphagia is rather broad, making a detailed patient history and a complete physical examination necessary to narrow the list of possible diseases. Patients should be asked about the onset and progression of symptoms, whereas additional signs may be of critical importance to suspect Plummer-Vinson syndrome or other known associated diseases in which esophageal webs are a constitutive feature. Nevertheless, the cornerstone of diagnosing esophageal webs is the implementation of one of the three imaging studies - the barium swallow test, videofluoroscopy, or upper endoscopy [4]. Barium swallow test is somewhat safer, as the web may be mechanically injured and ruptured during insertion of the endoscope, thus the diagnosis can be missed [4]. For this reason, certain authors suggest that videofluoroscopy (also known as the modified barium swallow test) is the optimal method to detect esophageal webs [2] [4]. Smooth and thin protrusions of the central lumen, often originating from the anterior wall and spreading to the lateral aspects of the esophagus, are typical findings [4]. Whenever esophageal webs are detected, a complete blood count (CBC) and further tests to confirm iron-deficiency anemia (serum iron levels, ferritin, and total iron binding capacity, or TIBC) should be performed, in order to obtain valid clinical suspicion toward Plummer-Vinson syndrome [6].
Treatment
To present a technique for the treatment of complete esophageal stenosis in the post-radiation patient that may be applied to selected patients with obstructing stenoses. A case report of the treatment of a post-radiation esophageal web. [ncbi.nlm.nih.gov]
In case of severe obstruction, the esophagus may be mechanically dilated (expanded) The prognosis of Plummer-Vinson Syndrome is generally good with treatment, but complications, such as trauma/tears in the throat during treatment, may worsen the prognosis [dovemed.com]
Prognosis
In case of severe obstruction, the esophagus may be mechanically dilated (expanded) The prognosis of Plummer-Vinson Syndrome is generally good with treatment, but complications, such as trauma/tears in the throat during treatment, may worsen the prognosis [dovemed.com]
Prognosis of Plummer-Vinson Syndrome/Paterson-Kelly Syndrome/Sideropenic Dysphagia/Esophageal Web. Prognosis is good after prompt treatment. [drthindhomeopathy.com]
[…] distal esophagus web) 7 external beam radiation Fluoroscopy: barium swallow may be demonstrated on high-volume barium oesophagrams when the esophagus is fully distended 4 a "jet effect" of contrast passing distal to the web may be seen 6 Treatment and prognosis [radiopaedia.org]
Prognosis The prognosis of esophageal webs is generally good especially with treatment of the underlying cause. [wikidoc.org]
Prognosis The prognosis in patients with mild symptoms is excellent, because most respond to dietary modifications and change in eating habits. Patients with refractory dysphagia usually respond to mechanical esophageal dilation. [emedicine.com]
Etiology
Certain conditions have both an underlying etiology and multiple body system manifestations due to the underlying etiology. [icd10coded.com]
This patient's unique findings may provide further insight into the etiology of upper esophageal webs or rings (Paterson-Kelly syndrome). [ncbi.nlm.nih.gov]
Regardless of the etiology, the principal symptom that could be triggered by an esophageal web is dysphagia, which may be initially painless and intermittent. [symptoma.com]
Etiology The cause of esophageal rings and webs remains controversial and can be classified as congenital or acquired. [emedicine.com]
Epidemiology
This review attempts to provide a practical update on esophageal rings and webs and to discuss their definition, epidemiology, pathophysiology, clinical presentation, differential diagnosis, diagnosis, treatment, and follow-up care. [emedicine.com]
Molecular Epidemiology of Hereditary Epidermolysis Bullosa in a Middle Eastern Population. Journal of Investigative Dermatology (2006) 126, 777–781. Stewart MI,Woodley DT, Briggaman RA. [journalijar.com]
Epidemiology Prevalence Webs are diagnosed in 5-15% of patients doing barium esophagogram for diagnosing the cause of dysphagia. Congenital esophageal webs are estimated to be 1 in 25,000 to 1 in 50,000 live births. [wikidoc.org]
Eslick GD, Talley NJ (2008) Dysphagia: epidemiology, risk factors and impact on quality of life--a population-based study. Aliment Pharmacol Ther 27: 971-979. [archivesofmedicine.com]
Pathophysiology
Its potential protrusion into the lumen of the esophagus (most commonly in the proximal portion) and compromise of food passage, however, can occur in certain disorders, such as Plummer-Vinson syndrome, where iron deficiency, the principal pathophysiological [symptoma.com]
Esophageal Web & Zenker's Diverticulum Pathophysiology A web is mucosal structure occur anywhere along the esophagus; reflux esophagitis may predispose A hypopharyngeal diverticulum, associated w/ dysfunctioning upper esophageal sphincter Diagnosis Esophageal [fprmed.com]
Pathophysiology The pathophysiology of esophageal rings and webs is controversial. Several theories have been proposed for the formation of esophageal webs and rings. [emedicine.com]
Pathophysiology Lower Esophageal Sphincter (LES) constriction Associated with Hiatal Hernia in nearly all cases III. Symptoms Dysphagia for solids only IV. Diagnosis Barium swallow Endoscopy (EGD) V. [fpnotebook.com]
Prevention
Stretching of strictures usually is combined with maximum treatment for acid reflux in order to prevent further scarring and narrowing of the esophagus. Permanent treatment of strictures requires surgery. Medical Author: Jay Marks, M.D. [medicinenet.com]
[…] individuals with PVS have shown a higher incidence of esophageal cancer Please find comprehensive information on Plummer-Vinson Syndrome regarding definition, distribution, risk factors, causes, signs & symptoms, diagnosis, complications, treatment, prevention [dovemed.com]
Centers for Disease Control and Prevention Intersex (Medical Encyclopedia) [ Read More ] Esophagus Disorders The esophagus is the muscular tube that carries food and liquids from your mouth to the stomach. [icdlist.com]
Gastrointestinal endoscopy and barium-swallow esophagography detected a web that prevented passage of the endoscope into the upper portion of the esophagus. [karger.com]
To prevent aspiration, barium studies are contraindicated in patients with suspected food impaction. Spontaneous esophageal perforations have been reported for both esophageal webs and rings. [emedicine.com]
References
- Tahara T, Shibata T, Okubo M, et al. A Case of Plummer-Vinson Syndrome Showing Rapid Improvement of Dysphagia and Esophageal Web after Two Weeks of Iron Therapy Case Rep Gastroenterol. 2014;8(2):211-215.
- Liu JJ, Kahrilas PJ. Pharyngeal and esophageal diverticula, rings, and webs. GI Motility online; 2006.
- Gude D, Bansal D, Malu A. Revisiting Plummer Vinson Syndrome. Annals of Medical and Health Sciences Research. 2013;3(1):119-121.
- Novacek G. Plummer-Vinson syndrome. Orphanet Journal of Rare Diseases. 2006;1:36.
- Terui K, Saito T, Mitsunaga T, Nakata M, Yoshida H. Endoscopic management for congenital esophageal stenosis: A systematic review. World J Gastrointest Endosc. 2015;7(3):183-191.
- Longo DL, Fauci AS, Kasper DL, Hauser SL, Jameson J, Loscalzo J. eds. Harrison's Principles of Internal Medicine, 18e. New York, NY: McGraw-Hill; 2012.