Esophageal Webs And Rings

Article Author:
Sami Ghazaleh
Article Editor:
Krunal Patel
Updated:
5/2/2019 9:56:19 PM
PubMed Link:
Esophageal Webs And Rings

Introduction

The esophagus is a muscular tube for the propulsion of food from the pharynx to the stomach. Histologically, the esophageal wall consists of mucosa, submucosa, and muscularis propria.[1] Esophageal webs and rings are indentations of the esophageal wall that may partially occlude the esophageal lumen. Esophageal webs and rings are usually asymptomatic but can occasionally present with intermittent dysphagia to solids.

Etiology

The etiology of esophageal webs and rings remains a controversial topic. Several conditions have correlated with esophageal webs and rings. Esophageal webs have classically associated with Plummer-Vinson syndrome which is classically a triad of dysphagia, iron-deficiency anemia, and esophageal webs.[2] Other associated conditions include Zenker’s diverticulum, epidermolysis bullosa, pemphigus vulgaris, and bullous pemphigoid.[3][4][5][6] Esophageal rings, on the other hand, are almost always associated with a hiatal hernia.[7] They also correlate with eosinophilic esophagitis.[8]

Epidemiology

Because most webs and rings are asymptomatic, the true prevalence of these lesions is unclear.[9] Esophageal webs and rings are each identified in 5 to 15% of patients undergoing upper endoscopy for dysphagia.[10]

Pathophysiology

The pathophysiology of esophageal webs and rings is poorly understood. Chronic inflammation can irritate the esophageal wall and produce these lesions.[11] They are also present in the pediatric population suggesting a congenital origin.[12] If webs and rings are big enough, they can narrow the esophageal lumen causing dysphagia and food impaction.

Histopathology

Esophageal webs are usually found in the proximal esophagus and have a covering of squamous epithelium.[10] Esophageal rings, on the other hand, are usually located in the distal esophagus and can be covered with squamous epithelium, columnar epithelium, or both depending on their location to the squamocolumnar junction:

  1. The A rings are located proximal to the squamocolumnar junction. They are covered entirely with squamous epithelium. They are uncommon and usually nonpathological.
  2. The B rings or Schatzki rings are located precisely at the squamocolumnar junction. The proximal part is covered with squamous epithelium, and the distal portion is covered with columnar epithelium. They are the most common type of rings and can become symptomatic.
  3. The C rings are located distal to the squamocolumnar junction. They are covered entirely with columnar epithelium. They are the least common, rarely symptomatic, and usually represent diaphragmatic indentation of the esophagus.[13]

History and Physical

Most esophageal webs and rings cause no symptoms. Patients can become symptomatic if an esophageal web or ring protrudes into the lumen enough to cause esophageal narrowing. Patients usually present with intermittent dysphagia to solid food. Some patients may develop food impaction and will present with acute dysphagia and inability to swallow saliva. In the case of Plummer-Vinson syndrome, patients may demonstrate with the triad of dysphagia, esophageal webs, and iron deficiency anemia.[14]

Isolated esophageal webs and rings usually do not have physical exam findings. However, a comprehensive physical exam may point towards an associated condition. Plummer-Vinson syndrome might have the skin manifestations of iron deficiency anemia including koilonychia, glossitis, and cheilosis.[2] A skin examination may reveal bullous lesions consistent with epidermolysis bullosa or pemphigus vulgaris.

Evaluation

Laboratory testing is rarely useful in patients with suspected esophageal webs or rings. It might be reasonable to obtain a complete blood count and iron studies to look for iron deficiency. However, patients with dysphagia need to be evaluated with barium swallow and/or upper endoscopy. Both procedures can help differentiate between the different etiologies of dysphagia.

A barium swallow is particularly useful in delineating the cause of the esophageal lumen obstruction. It can identify esophageal webs, rings, strictures, tumors, and extraesophageal compression. An esophagogastroduodenoscopy (EGD) is usually necessary for the confirmation of the diagnosis. On EGD, esophageal webs appear as thin membranes that do not span the entire circumference of the esophagus.[2] Esophageal rings also appear as thin membranes, but they span the entire circumference of the esophagus.[14] EGD can also identify other causes of dysphagia including stricture, polyps, and tumors. Moreover, it can allow biopsy of the lesions when suspicion of malignancy exists. Conditions associated with esophageal webs and rings may be identified with an EGD including eosinophilic esophagitis, hiatal hernia, and Zenker’s diverticulum.

Treatment / Management

The main initial treatment of symptomatic esophageal webs and rings is endoscopic esophageal dilatation. The goal of treatment is relief of dysphagia and prevention of symptom recurrence. It is common for esophageal webs to rupture during a diagnostic EGD. Esophageal dilatation is still an option on esophageal webs that have partially ruptured.[15] Esophageal rings require biopsy before dilatation to rule out eosinophilic esophagitis. Following dilatation of esophageal rings, patients often require therapy with a proton pump inhibitor (PPI) because most rings are close to the gastroesophageal junction.[16] Esophageal dilatation is generally safe and effective with low risk of complications.[17]

The major problem with dilatation is the high recurrence rate, especially when used for the treatment of esophageal rings. Therapy with a PPI has been shown to decrease the risk of recurrence following dilatation of esophageal rings.[18] Recurrent symptomatic esophageal rings are treatable with repeat esophageal dilation followed by long-term acid suppression with a PPI. Alternative therapies exist for refractory esophageal rings that failed multiple dilatations. These include incisional therapy using electrocautery and laser division.[19][20]

Differential Diagnosis

The differential diagnosis for esophageal webs and rings is extensive and includes other causes of dysphagia. History and evaluation by barium swallow and/or EGD can help identify those causes. Conditions that may mimic webs and rings include achalasia and esophageal strictures. Achalasia presents with progressive dysphagia to solids and liquids. The classic finding on barium swallow is distal dilatation of the esophagus with a “bird-beak” appearance.[21] Esophageal strictures present with progressive dysphagia to solids. They appear longer than webs and rings on barium swallow and have tapered ends.[22]

Prognosis

The prognosis of esophageal webs and rings is excellent because most patients are asymptomatic. Symptomatic webs and rings are treatable with esophageal dilatation which is safe and effective. The primary concern with therapy is the high recurrence rate following dilatation of esophageal rings. The risk of recurrence improves when using a PPI after dilatation.

Complications

Most esophageal webs and rings do not cause symptoms. Dysphagia occurs when webs and rings protrude into the esophageal lumen leading to luminal narrowing. If the luminal narrowing is severe enough, patients may develop food impaction with the inability to swallow food or even saliva.[15]

Deterrence and Patient Education

Patients should receive education about the benign nature of the condition. This training should include information regarding the alarming signs that indicate the evolution of an occult process. These signs include loss of appetite, weight loss, or bloody stools. They should be advised to take their PPI as prescribed to decrease the risk of recurrence following dilatation.

Enhancing Healthcare Team Outcomes

Coordination of care between health care providers (i.e., the primary care provider, nurse practitioner) is essential for health care outcomes and patient safety. Patients who complain of dysphagia may be encountered in the outpatient clinic by a primary care physician. It is important to ask patients about new symptoms that they may be experiencing. When dysphagia is identified, referral to a gastroenterologist is usually necessary for evaluation. Focused history and examination by the primary care physician in addition to proper documentation can enhance care coordination. Evaluation by gastroenterology may reveal esophageal webs and rings that may require treatment with esophageal dilatation. These findings must be communicated back to the primary care physician. Also, the gastroenterologist should thoroughly explain his recommendations including the need for a repeat EGD or the need for a PPI. 


References

[1] Yazaki E,Sifrim D, Anatomy and physiology of the esophageal body. Diseases of the esophagus : official journal of the International Society for Diseases of the Esophagus. 2012 May;     [PubMed PMID: 21385283]
[2] Novacek G, Plummer-Vinson syndrome. Orphanet journal of rare diseases. 2006 Sep 15;     [PubMed PMID: 16978405]
[3] Low DE,Hill LD, Cervical esophageal web associated with Zenker's diverticulum. American journal of surgery. 1988 Jul;     [PubMed PMID: 3134826]
[4] Ergun GA,Lin AN,Dannenberg AJ,Carter DM, Gastrointestinal manifestations of epidermolysis bullosa. A study of 101 patients. Medicine. 1992 May;     [PubMed PMID: 1635437]
[5] Hokama A,Yamamoto Y,Taira K,Nakamura M,Kobashigawa C,Nakamoto M,Hirata T,Kinjo N,Kinjo F,Takahashi K,Fujita J, Esophagitis dissecans superficialis and autoimmune bullous dermatoses: A review. World journal of gastrointestinal endoscopy. 2010 Jul 16;     [PubMed PMID: 21160615]
[6] Foroozan P,Enta T,Winship DH,Trier JS, Loss and regeneration of the esophageal mucosa in pemphigoid. Gastroenterology. 1967 Mar;     [PubMed PMID: 6019973]
[7] Müller M,Gockel I,Hedwig P,Eckardt AJ,Kuhr K,König J,Eckardt VF, Is the Schatzki ring a unique esophageal entity? World journal of gastroenterology. 2011 Jun 21;     [PubMed PMID: 21734791]
[8] Müller M,Eckardt AJ,Fisseler-Eckhoff A,Haas S,Gockel I,Wehrmann T, Endoscopic findings in patients with Schatzki rings: evidence for an association with eosinophilic esophagitis. World journal of gastroenterology. 2012 Dec 21;     [PubMed PMID: 23322994]
[9] Wilcox CM,Alexander LN,Clark WS, Localization of an obstructing esophageal lesion. Is the patient accurate? Digestive diseases and sciences. 1995 Oct;     [PubMed PMID: 7587788]
[10] Kahrilas PJ,Kim HC,Pandolfino JE, Approaches to the diagnosis and grading of hiatal hernia. Best practice     [PubMed PMID: 18656819]
[11] Chen YM,Gelfand DW,Ott DJ,Munitz HA, Natural progression of the lower esophageal mucosal ring. Gastrointestinal radiology. 1987;     [PubMed PMID: 3556982]
[12] Anderson LS,Shackelford GD,Mancilla-Jimenez R,McAlister WH, Cartilaginous esophageal ring: a cause of esophageal stenosis in infants and children. Radiology. 1973 Sep;     [PubMed PMID: 4198827]
[13] Hirano I,Gilliam J,Goyal RK, Clinical and manometric features of the lower esophageal muscular ring. The American journal of gastroenterology. 2000 Jan;     [PubMed PMID: 10638557]
[14] SCHATZKI R, THE LOWER ESOPHAGEAL RING. LONG TERM FOLLOW-UP OF SYMPTOMATIC AND ASYMPTOMATIC RINGS. The American journal of roentgenology, radium therapy, and nuclear medicine. 1963 Oct;     [PubMed PMID: 14068418]
[15] Smith MS, Diagnosis and management of esophageal rings and webs. Gastroenterology     [PubMed PMID: 21437018]
[16] Eckardt VF,Kanzler G,Willems D, Single dilation of symptomatic Schatzki rings. A prospective evaluation of its effectiveness. Digestive diseases and sciences. 1992 Apr;     [PubMed PMID: 1551348]
[17] Mann NS, Single dilation of symptomatic Schatzki ring with a large dilator is safe and effective. The American journal of gastroenterology. 2001 Dec;     [PubMed PMID: 11774974]
[18] Sgouros SN,Vlachogiannakos J,Karamanolis G,Vassiliadis K,Stefanidis G,Bergele C,Papadopoulou E,Avgerinos A,Mantides A, Long-term acid suppressive therapy may prevent the relapse of lower esophageal (Schatzki's) rings: a prospective, randomized, placebo-controlled study. The American journal of gastroenterology. 2005 Sep;     [PubMed PMID: 16128935]
[19] Wills JC,Hilden K,Disario JA,Fang JC, A randomized, prospective trial of electrosurgical incision followed by rabeprazole versus bougie dilation followed by rabeprazole of symptomatic esophageal (Schatzki's) rings. Gastrointestinal endoscopy. 2008 May;     [PubMed PMID: 18313671]
[20] Roy GT,Cohen RC,Williams SJ, Endoscopic laser division of an esophageal web in a child. Journal of pediatric surgery. 1996 Mar;     [PubMed PMID: 8708922]
[21] Reynolds JC,Parkman HP, Achalasia. Gastroenterology clinics of North America. 1989 Jun;     [PubMed PMID: 2668168]
[22] Marks RD,Richter JE, Peptic strictures of the esophagus. The American journal of gastroenterology. 1993 Aug;     [PubMed PMID: 8338082]