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Esophageal Web

Oesophageal Web

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].

Constitutional Symptom
  • 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]
Intermittent Dysphagia
  • dysphagia If cervical web and iron deficiency consider Plummer-Vinson Syndrome (also achlorhydria, glossitis & stomatitis) If distal esophagus "steak house syndrome" Zenker's Diverticulum Cervical dysphagia, severe halitosis or aspiration Usually seen[fprmed.com]
  • dysphagia and impaction of a bolus of food Treatment Intraluminal balloon dilatation.[medical-dictionary.thefreedictionary.com]
  • It tends to mark the proximal part of a hiatus hernia and usually presents in a patient aged over 50 years whose main complaint is intermittent dysphagia to solid food, spanning months or years, and it is non-progressive.[patient.info]
  • Complications Intermittent dysphagia to solid food is the most common complication. Food impaction, particularly of meat products, is common in patients with lower esophageal rings.[emedicine.com]
Progressive Dysphagia
  • Abstract A 55-year-old woman had chronic, progressive dysphagia. An esophageal web in the cervical esophagus was identified by barium swallow and endoscopy.[ncbi.nlm.nih.gov]
  • 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]
  • Worsening strictures may cause progressive dysphagia - from hard food, such as meat, to sloppy food like porridge, to liquids - so ask about progression of symptoms and the time span.[patient.info]
Deglutition Disorder
  • Key words Plummer-Vinson syndrome Cineradiography Web, cervical esophageal Dilatation, endoscopic Myectomy Deglutition Deglutition disorders This is a preview of subscription content, log in to check access. Preview Unable to display preview.[link.springer.com]
Arthritis
  • Medrano M: Dysphagia in a patient with rheumatoid arthritis and iron deficiency anemia. MedGenMed 2002;4:10.[karger.com]
  • Associated diseases Thyroid disease, rheumatoid arthritis, graft-versus-host disease, Stevens-Johnson syndrome, psoriasis, blistering skin diseases, and pernicious anaemia.[patient.info]
  • Autoimmune theory Autoimmune diseases have been associated with esophageal webs and include thyroid disease, rheumatoid arthritis, graft versus host disease, Stevens-Johnson syndrome, psoriasis, blistering skin diseases, and pernicious anemia.[emedicine.com]
Myopathy
  • Mild Cognitive Impairment 325 Mitral Regurgitation 326 Monoarthritis Acute 327 Mononucleosis 328 Mucormycosis 329 Mumps 330 Muscle Weakness 331 Muscular Dystrophy 332 Mycosis Fungoides 333 Myelodysplastic Syndrome 334 Myocarditis 335 Myoglobinuria 336 Myopathies[books.google.de]
Dermatologic Disorder
  • The literature describes relations between these webs and Plummer-Vinson Syndrome, bullous dermatologic disorders, inlet patch, graft-versus-host disease and celiac disease.[en.wikipedia.org]
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]
Suggestibility
  • Celiac disease was diagnosed on the basis of elevated tissue transglutaminase antibody and suggestive duodenal histology. RESULTS: Twenty one patients were diagnosed to have cervical esophageal web. Eighteen (85.7%) had evidence of iron deficiency.[ncbi.nlm.nih.gov]
  • Eosinophil infiltration suggests a cause from GER, allergic response (food), and idiopathic eosinophil gastroenteritis. The finding of lymphocytes and plasma cells suggests chronic inflammation.[emedicine.com]
  • For this reason, certain authors suggest that videofluoroscopy (also known as the modified barium swallow test) is the optimal method to detect esophageal webs.[symptoma.com]
  • However, there are some conflicting reports on esophageal webs and latent iron deficiency suggesting no correlation between the same [ 5 ].[karger.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].

Suppression
  • It has been speculated that acid suppression may reduce the recurrence of Schatzki rings. Dilation followed by acid suppression treatment reduced the risk of recurrence, even in patients without previous reflux symptoms.[emedicine.com]
  • Treatment usually involves dilation combined with acid suppressive therapy.[gastrointestinalatlas.com]

Treatment

  • STUDY DESIGN: A case report of the treatment of a post-radiation esophageal web. METHODS: A review of the patient's history of treatment and a discussion of reported treatment options.[ncbi.nlm.nih.gov]
  • For thin webs, combined anterograde and retrograde esophagoscopy along with puncture and dilation can be a relatively safe treatment option for selected patients.[ncbi.nlm.nih.gov]
  • This surgical treatment resulted in diminished dysphagia and no recurrence of the web after the surgery.[ncbi.nlm.nih.gov]
  • It confirmed the diagnostic suspicion and allowed treatment by dilatation that resulted in a marked benefit to the patient.[ncbi.nlm.nih.gov]
  • Please consult your own licensed physician regarding diagnosis and treatment of any medical condition! Please see also our disclaimer . This site complies with the HONcode standard for health information: verify here . Database updated 2019-02-19.[diseasesdatabase.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 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]
  • Symptoms Diagnosis Expected Duration Prevention Treatment When To Call a Professional Prognosis Additional Info What Is It? Esophageal rings and webs are folds that block your esophagus either partially or completely.[pernicious-anaemia-society.org]

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]
  • Its etiology remains unknown, but an embryologic origin has been suggested.[ojrd.biomedcentral.com]

Epidemiology

  • 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]
  • 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]
Sex distribution
Age distribution

Pathophysiology

  • 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]
  • 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]
  • 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]
  • Sleisenger and Fordtran's Gastrointestinal and Liver Disease: Pathophysiology/Diagnosis/Management. 10th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 47. Rustgi AK. Neoplasms of the esophagus and stomach. In: Goldman L, Schafer AI, eds.[medlineplus.gov]

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

Article

  1. 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.
  2. Liu JJ, Kahrilas PJ. Pharyngeal and esophageal diverticula, rings, and webs. GI Motility online; 2006.
  3. Gude D, Bansal D, Malu A. Revisiting Plummer Vinson Syndrome. Annals of Medical and Health Sciences Research. 2013;3(1):119-121.
  4. Novacek G. Plummer-Vinson syndrome. Orphanet Journal of Rare Diseases. 2006;1:36.
  5. 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.
  6. 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.

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Last updated: 2018-06-22 11:28