AUTHOR AND EDITOR INFORMATION

Section 1 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

INTRODUCTION

Section 2 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Background

Rings and webs are the most common structural abnormalities in the esophagus. The terminology, pathogenesis, and treatment of these esophageal lesions remain controversial. The terms rings and webs often are used interchangeably in the literature. The pathogenesis and treatment of esophageal rings and webs are evolving. Most of these structural lesions are asymptomatic but can cause dysphagia. 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.

An esophageal ring is defined as a concentric, smooth, thin (3-5 mm) extension of normal esophageal tissue consisting of 3 anatomic layers of mucosa, submucosa, and muscle. An esophageal ring can be found anywhere along the esophagus, but it usually is found in the distal esophagus. Three types of esophageal rings exist, and they are classified alphabetically as A, B, and C (see Media file 1).

The A ring is uncommon and represents a muscular ring several centimeters proximal to the squamocolumnar junction (SQJ). This muscular ring is not a fixed structure and only may be demonstrated in some barium swallows. It is located approximately 1.5 cm proximal to the SQJ, and it is rarely symptomatic. Some debate surrounds the existence of the A ring as a separate entity. Hirano et al described a unique manometric pattern of peristaltic, high-amplitude, long-duration, and multiple peaked esophageal contractions in 3 men with A rings.

The most famous and common ring in the esophagus is the B ring or Schatzki ring (see Media files 2-3). By definition, the Schatzki ring is a web because it is composed of only mucosa and submucosa. Typically, the Schatzki ring is located at the SQJ, and it marks the proximal margin of a hiatal hernia. Autopsy examinations support that the free margin of the Schatzki ring marks the SQJ. Because of the difficulty in accurately locating the SQJ, lower esophageal sphincter, and lower esophageal ring during swallowing, the exact anatomic relationship between the esophageal ring and the SQJ remains controversial.

Some authors believe the Schatzki ring is a protective barrier against gastroesophageal reflux (GER), while others, such as Winters et al, question the protective nature of rings. A common complication of Schatzki ring is meat impaction (see Media file 4). Esophageal rings usually exist as a single lesion but can be multiple (see Media file 5). Several names have been coined for when multiple rings are found in the esophagus, including multiple esophageal rings or webs, congenital esophageal stenosis, ringed esophagus, corrugated esophagus, and feline esophagus.

In 1944, Templeton first described the esophageal ring as a weblike membranous ring in the lower esophagus. Prior to 1953, all webs were found in infants and children and were believed to be congenital. In 1953, the term ring became popularized because of its association with dysphagia. During this time, Ingelfinger, Kramer, Schatzki, and Gary separately reported a group of adults older than 50 years with x-ray findings of a ringlike narrowing of the distal esophagus associated with intermittent dysphagia particularly occurring after swallowing solid foods. Poorly chewed meat can impact at a distal esophageal ring, leading to the term "steak-house syndrome."

Ingelfinger and Kramer reported 6 cases of esophageal rings, and examination of 1 of these rings following esophageal resection revealed a circle of hypertrophied muscle underlying normal esophageal mucosa. Schatzki and Gary reported 5 cases with interesting findings, all of which were associated with a hiatal hernia on x-ray film. Partial excision of one of these rings exhibited involvement of mucosa and submucosa only. In 1968, subsequent analysis of 64 pathologic cases of lower esophageal rings by Miller and Wichern showed that none of the rings had muscle wall involvement except for the 1 case from Ingelfinger and Kramer.

The C ring is a rare anatomic finding on radiographic studies referring to the indentation caused by the diaphragmatic crura. It is rarely symptomatic.

An esophageal web is a thin (2-3 mm), eccentric, smooth extension of normal esophageal tissue consisting of mucosa and submucosa that can occur anywhere along the length of the esophagus but typically is located in the anterior postcricoid area of the proximal esophagus (see Media files 6-7).

Pathophysiology

The pathophysiology of esophageal rings and webs is controversial. Several theories have been proposed for the formation of esophageal webs and rings. These include etiologies related to congenital origin, iron deficiency, development, inflammation, and autoimmunity.

Congenital theory

Rings and webs may represent a remnant of embryologic development in which the esophagus fails to recanalize completely. In children, fragments of cartilage in esophageal ring-like structures similar to trachea have been described. Fonkalsrud and Anderson reported ciliated pulmonary epithelium and bronchial remnants in resected specimens; however, the fact that most patients with symptoms present when they are older than 40 years suggests that rings and webs alone do not produce any symptoms.

Recent publications in pediatric radiology have suggested that Schatzki rings are more common in children than once thought. The majority of children are asymptomatic, and, thus, they are probably far less detected than indicated. These findings might support the theory that the pathophysiology of the rings contains a congenital factor.

Iron deficiency

The link between iron deficiency and esophageal webs remains controversial. Evidence for this theory is limited by the uncertainty in duration of iron deficiency necessary for web formation. Another challenge to this theory is that most esophageal webs are asymptomatic. In rabbits that were iron deficient, histologic examination of esophageal muscle revealed muscle fiber abnormalities. This histologic finding suggests that myasthenic changes in esophageal muscles may lead to dysphagia.

In patients with postcricoid webs and Plummer-Vinson syndrome (PVS), histologic findings of hyperplasia degeneration of mucosa have been reported and attributed to iron deficiency. Okamura hypothesized that mucosal degeneration can lead to a cascade of decrease in swallowing, restriction of esophageal wall dilation, repetitive tissue injury and healing, and, eventually, permanent esophageal mucosal changes such as a web. Chisholm and associates observed 72 patients with postcricoid webs for 15 years and found iron deficiency in 90%. However, an epidemiologic study by Elwood et al failed to show a correlation between iron deficiency and cervical esophageal webs.

Other nonsupportive studies include the equal likelihood of esophageal webs in patients who are iron deficient and in controls, lack of anemia in most patients with esophageal webs, and resolution of dysphagia but not webs following iron therapy. Further, Chisholm reported only a 10% prevalence of esophageal webs in patients who were iron deficient. Studies are needed to determine if the duration or severity of iron deficient anemia influences web formation with or without dysphagia.

Developmental theory

Esophageal rings have been postulated to occur during development when a pleat of mucosa is formed by infolding of redundant esophageal mucosa due to shortening of the esophagus. The cause for the repeated plication is unknown.

Inflammation theory

Inflammatory cells can be found in biopsy specimens of esophageal rings and webs. These inflammatory cells are more common in distal esophageal lesions than in proximal esophageal lesions. The presence of neutrophils suggests an acute inflammatory response to a variety of insults including GER, medications, caustic ingestion, radiation, and trauma. Eosinophil infiltration suggests a cause from GER, allergic response (food), and idiopathic eosinophil gastroenteritis. The finding of lymphocytes and plasma cells suggests chronic inflammation.

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. Patients with PVS have elevated thyroid cytoplasmic autoimmune antibodies of unknown significance. No other autoimmune antibodies have been associated with esophageal webs. More studies are needed to support this casual association.

Frequency

United States

The true prevalence of esophageal rings and webs is unknown because most of these lesions are asymptomatic. Lower esophageal rings are found in 6-14% of routine barium radiographs for various reasons. In patients with dysphagia, esophageal webs can be found in 5-15% by barium radiography. For patients with multiple esophageal rings and webs or congenital esophageal stenosis, esophageal webs and rings are estimated to occur in 1 in 25,000 to 1 in 50,000 live births.

International

The international prevalence is unknown.

Mortality/Morbidity

Most patients with esophageal rings and webs are asymptomatic. Schatzki reported a direct correlation between the luminal diameter of an esophageal ring and patients' symptoms. Almost all patients with an esophageal lumen less than 13 mm have dysphagia. Patients with esophageal lumen from 13-20 mm may or may not have dysphagia, and if the luminal diameter is greater than 20 mm, dysphagia is rare. Spontaneous perforation of the esophagus is rare, but it has been reported. No reports on mortality exist.

Race

To date, no studies compare racial prevalence; however, esophageal rings and webs appear to predominantly affect white individuals. Wong et al reported 13 patients with multiple esophageal rings, and all were Caucasians. Larger studies are needed to support this observation.

Sex

Esophageal rings do not demonstrate a sex preference, but esophageal webs are found mostly in female patients. The reason for this difference is unknown, but population studies suggest iron deficiency, particularly in menstruating females, may be a cause. However, for unknown reasons, multiple esophageal rings are found predominantly in young males.

Age

Rings and webs have been identified in all age cohorts. Patients do not typically become symptomatic until after the age of 40 years. Dysphagia is a very common occurrence in the elderly. Oropharyngeal and dental problems may exacerbate symptomatic rings in the elderly, as their ability for mastication and swallowing are deteriorated.

CLINICAL

Section 3 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

History

Most patients with rings and webs of the esophagus have no symptoms. Instead, esophageal webs and rings are detected incidentally on upper GI series and/or upper endoscopy.

• The hallmark symptom of esophageal rings and webs is dysphagia. Dysphagia to solid food usually is greater than dysphagia to liquid food. If liquid dysphagia is the predominant symptom, suspect a motility disorder such as achalasia, nutcracker esophagus, or diffuse esophageal spasm.
• Suspect the presence of a symptomatic Schatzki ring in a patient older than 50 years with a chief complaint of intermittent dysphagia to solid food spanning months or years. Other supporting history for Schatzki rings includes heartburn and a hiatal hernia.
• The anatomic location of the rings, webs, or narrowing structures causing dysphagia may be inferred from the patient's history. The clinician should ask the patient to locate the area of dysphagia along the sternum.
• • If the dysphagia is located below the sternum, then the obstruction site is likely in the mid-to-lower esophagus, and an excellent correlation exists between the patient's location and the anatomic obstruction site on barium study or endoscopy.
  • If the patient's area of dysphagia is located at or above the sternal notch, the obstruction site cannot be determined with any accuracy.
• Alarming symptoms that are not consistent with esophageal rings are progressive dysphagia, weight loss, and anemia. These symptoms suggest a mechanical, fixed obstruction such as a stricture or malignancy, making an early diagnosis mandatory.

Physical

Although most patients with esophageal rings and webs do not have any physical findings, the skin, mucous membrane, and nails may suggest an etiology.

• In PVS, physical findings include koilonychia (spoon nails), cheilosis, and glossitis.
• Rare skin diseases, such as epidermolysis dystrophica and benign mucous membrane pemphigoid, present with skin bullous lesions.
• Acute caustic ingestion of lye or alkaline solution can lead to oropharyngeal injury manifested as ulcerations, exudates, edema, and erythema.

Causes

The cause of esophageal rings and webs remains controversial and can be classified as congenital or acquired. Acquired causes of lower esophageal rings include gastroesophageal reflux disease (GERD), caustic ingestion, pill-induced inflammation, and mediastinal radiation; an association also exists with some skin diseases. Acquired causes for esophageal webs include PVS, iron deficiency anemia, celiac sprue, inlet patch, graft versus host disease, and skin diseases.

• Lower esophageal rings
• • GERD has been studied as a cause of lower esophageal rings. Symptomatic esophageal rings typically present when an individual is older than 40 years, suggesting that chronic injury from GERD may be involved in the pathogenesis. Although lower esophageal rings are thinner structures than peptic strictures and have no surrounding inflammation, they may be part of the spectrum in a GERD-related injury.
  • • In a small retrospective study using endoscopy and barium radiography, Chen et al demonstrated a progression of normal mucosa or lower esophageal ring to peptic stricture in some patients.
    • In another study using endoscopy or ambulatory pH monitoring, Marshall et al found GER in 13 of 20 (65%) consecutive patients with symptomatic lower esophageal rings. Most Schatzki rings are associated with a hiatal hernia, suggesting that GERD may be involved in their pathogenesis.
    • With regard to esophageal motility, Chen et al found no relationship between lower esophageal rings and esophageal dysmotility or lower esophageal sphincter hypotension. Evidence exists that acid suppression prevents recurrent symptoms in patients with peptic strictures. Similar studies are needed to demonstrate the effects of acid suppression on symptomatic lower esophageal rings. However, a study by Ott and associates using ambulatory pH monitoring found no difference in abnormal distal esophageal acid exposure in patients with hiatal hernia and lower esophageal rings compared to those with hiatal hernia alone.
  • Patients with evidence of Schatzki rings commonly present with gastric acid symptomatology. 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. Sgouros et al observed 44 consecutive patients who underwent dilation of Schatzki rings. Esophageal manometry and pH studies revealed that 14 had objective evidence of GERD, all of whom were treated with omeprazole. The remaining patients were randomly assigned to omeprazole or placebo. There were no recurrences of Schatzki rings in the group with documented GERD during a mean follow-up of 43 months. Recurrence rates were also significantly lower in the group without objective evidence of GERD who were randomized to omeprazole.
  • Smith et al evaluated 336 patients with peptic esophageal strictures that were randomized to omeprazole (20 mg daily) or ranitidine (150 mg twice daily) for 1 year after esophageal dilation to 12-18 mm. Subsequent endoscopy and dilation was performed when clinically indicated. The omeprazole-treated patients required significantly fewer repeated dilation sessions (30% vs 46%) and had improved dysphagia scores compared to the ranitidine-treated group.
  • Ingestion of alkaline or acidic agents can cause caustic injury to the esophagus. Severe injuries have occurred from ingestion of alkaline agents such as lye, sodium and potassium hydroxides in oven cleaners, washing detergents, Clinitest tablets, cosmetics, soaps, and button batteries. Ingestion of caustic agents can lead to esophageal stricture. Milder injuries have occurred from ingestion of alkaline agents such as sodium carbonate, ammonium hydroxide, and bleaches (sodium, calcium hypochlorite, hydrogen peroxide). Toilet bowel cleaners (sulfuric, hydrochloric), antirust compounds (hydrochloric, oxalic), battery fluids (sulfuric), and slate cleaners (hydrochloric) can cause acid injuries. Esophageal strictures from caustic injury develop in 15-38% of cases and occur as early as 2 weeks after caustic exposure.
  • Uncommon causes of lower esophageal rings include pill-induced esophagitis, rare skin disorders such as epidermolysis bullosa dystrophica, benign mucous membrane pemphigoid, and mediastinal radiation.
• Upper esophageal webs
• • The association between iron deficiency and esophageal webs is controversial. Chisholm and Jacobs supported this association in 2 case series of 72 and 63 patients. However, a careful epidemiologic study by Elwood failed to show a correlation between iron deficiency and cervical esophageal webs. Less controversy is found between iron deficiency and dysphagia without webs. Iron deficiency clearly can precede dysphagia. Chisholm and Bredenkamp et al noted resolution of dysphagia but not webs after iron supplementation.
  • PVS or Paterson-Brown-Kelly syndrome is characterized by postcricoid or upper esophageal webs eccentrically attached to the anterior wall of the esophagus and iron deficiency anemia. Other associated features of PVS are koilonychia, cheilosis, and glossitis. Webs are believed to arise in iron deficiency states. Pharyngeal and cervical esophageal cancers have been associated with PVS. Periodic screening for esophageal cancer in patients with PVS is recommended because of its malignant potential.
  • Celiac disease or gluten-sensitive enteropathy is characterized by small intestinal malabsorption. Histologic examination reveals a flat mucosal surface with complete absence of normal intestinal villi. Because iron is absorbed predominantly in the proximal small intestine, iron absorption is impaired in celiac disease. Dickey and McConnell described 2 patients with PVS and chronic iron deficiency anemia who were found to have celiac disease by histology. Dickey and McConnell hypothesized that iron deficiency from celiac disease is the primary cause of upper esophageal webs and PVS.
  • Heterotropic gastric mucosa can occur throughout the esophagus, and it is termed an inlet patch if it occurs in the proximal esophagus. The typical location of an inlet patch is usually right below the cricopharyngeal muscle at approximately 20-25 cm from the incisors. Inlet patch has been observed in 2.8% and 3.5% of consecutive endoscopies. On biopsy, corpus-fundic or antral-type mucosa is observed, sometimes containing parietal cells capable of acid secretion. Inlet patches are usually incidental findings, and most cases are believed to be clinically insignificant. Rarely, an esophageal ring and web can be found at the distal margin of the inlet patch, presumably from exposure to acid secretion (see Media file 8).
  • Upper esophageal webs have been reported in patients with chronic graft versus host disease after bone marrow transplantation. The mechanism is believed to be the accretion of desquamated esophageal epithelium. Caution is advised when performing endoscopy in patients with esophageal webs and graft versus host disease because of an increased risk of perforation.
  • Several skin diseases have been reported in association with esophageal webs including mucous membrane pemphigoid (cicatricial pemphigoid), epidermolysis bullosa, Stevens-Johnson syndrome, and psoriasis. An autoimmune process is believed to be the cause of these associations.
  • Other esophageal disorders have been reported to be associated with esophageal webs including Zenker diverticulum (see Media file 7) and esophageal duplication cyst. The pathogenesis for these associations is unknown.
• In patients with multiple esophageal rings, acquired causes include GER and eosinophilic esophagitis.
• • Wong and associates reported abnormal distal esophageal acid exposure in 7 of 9 (77%) patients by ambulatory pH monitoring.
  • Siafakas et al reported a case of multiple esophageal rings in an 8-year-old boy with histologic eosinophilic esophagitis who responded to an elimination diet and in whom antireflux treatment failed.

DIFFERENTIALS

Section 4 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Other Problems to be Considered

Toxicity, Caustic Ingestions
Peptic stricture
Esophageal tumor
Extrinsic compression
Neuroma
Congenital cartilaginous remnants
Feline esophagus
Aberrant vascular structures
Dysphagia lusoria
Mediastinal irradiation
Pill-induced esophagitis and ulcers
Epidermolysis bullosa dystrophica
Benign mucous membrane pemphigoid

WORKUP

Section 5 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Lab Studies

• Laboratory testing is not necessary in most patients. For the diagnosis of celiac sprue, iron deficiency anemia, and PVS, the following tests are necessary:
• • CBC count
  • Iron panel
  • Ferritin
  • Antigliadin antibodies
  • Antiendomysial antibodies

Imaging Studies

• Barium swallow
• • This is the diagnostic test of choice and should be the initial test in patients with dysphagia. Barium swallow allows detection of intraluminal obstruction. In addition to detecting esophageal rings and webs, it is useful in excluding other diagnoses, including peptic strictures, pill-induced esophagitis and/or strictures, tumors, intramural tumors (primarily leiomyomas), and extraesophageal compression. Infections related to candidiasis, cytomegalovirus, herpes simplex virus, and idiopathic ulcers sometimes may be suspected following barium studies.
  • Barium studies are relatively safe, with low radiation exposure, and relatively inexpensive. Most clinicians believe that the barium swallow is more sensitive than endoscopy in detecting rings and webs. A presbyesophagus (tortuous) or a hypermobile esophagus can prevent detection of a small ring or web by endoscopy. In addition, a barium swallow provides a "roadmap" for the endoscopist.
  • Reflux of barium provides additional information on the competency of the lower esophageal sphincter and the presence of GER. Finally, the administration of a barium pill at the end of the examination is recommended because the finding of obstruction to a 13-mm pill indicates a high-grade obstruction and the need for esophageal dilation.

Procedures

• Perform upper endoscopy (esophagogastroduodenoscopy) in patients with symptomatic esophageal rings and webs.
• • This procedure is diagnostic and enables therapeutic intervention.
  • It is probably less sensitive than barium radiography when diagnosing esophageal rings and webs. In fact, the physician occasionally can miss the diagnosis with endoscopy because of the fragility of esophageal webs, hypermotility of the esophagus, the presence of excessive fluid and secretion in the esophagus, presbyesophagus (tortuosity), or very small rings and webs.
  • Endoscopy allows biopsy of lesions such as strictures, polyps, or masses when any question of malignancy exists.
  • Routine biopsy of rings and webs is not necessary.

Histologic Findings

Histologic findings of esophageal rings include normal squamous epithelium, basal cell hyperplasia, hyperkeratosis, proliferative connective tissues, plasma cells, and eosinophilic and lymphocytic infiltration typical of chronic inflammation.

Histologic findings of esophageal webs are usually normal squamous epithelia with occasional chronic inflammatory cells in the subepithelial tissue.

TREATMENT

Section 6 of 11  

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• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Medical Care

• Lower esophageal rings and webs
• • Most esophageal rings are found incidentally, are asymptomatic, and do not require treatment.
  • Instruct patients with mild symptoms to modify their diet and eating habits by eating soft food, cutting solid food into smaller pieces, and eating slowly.
  • Before patients take oral medications, warn patients of the hazard of pills lodging in their esophagus; hence, they should cut large sized pills into smaller pieces prior to ingestion when possible. In addition, instruct patients to drink at least 8 ounces of liquid and to avoid laying supine for at least 30 minutes after taking their oral medications. These precautionary measures may prevent pill-induced esophagitis even in patients without esophageal disorders.
  • If these conservative measures are not adequate in preventing dysphagia, esophageal dilation with mechanical bougie is indicated.
  • Patients with eosinophilic esophagitis and evidence of proximal strictures and multiple mucosal rings may benefit from fluticasone propionate 220 µg/puff, twice daily without spacer.
• Esophageal dilation
• • Two types of mechanical bougies are used for esophageal dilation, Savary dilator and Maloney (mercury filled) dilator. Both types of bougies are graded in millimeters (mm) and French (1F = 3 mm). Both types of dilators are equally effective and safe. Perform an initial endoscopy prior to esophageal dilation to confirm the diagnosis when using Maloney dilators. With Savary dilators, an endoscopy is a part of each dilation procedure.
  • The goal of using mechanical bougies is to disrupt the rings rather than stretching them. In most cases, passage of one large bougie is adequate to disrupt the ring. Despite a lack of conclusive evidence, passing a single large bougie is believed to be more effective than serial progressive dilation of esophageal rings.
  • Fluoroscopic visualization rarely is needed for either procedure, but it is recommended if the lumen distal ring cannot be visualized.
  • A persistent ring after esophageal dilation as shown in postdilation barium study does not predict failure of therapy. In fact, in a prospective study by Eckardt et al, 33 consecutive patients with symptomatic esophageal rings experienced relief of their dysphagia after passage of a single Maloney bougie (46-58F), regardless of ring rupture. However, repeat dilation is safe and effective.
  • Unlike the lower esophageal rings, patients with multiple esophageal rings follow a set of different therapeutic rules for esophageal dilation. This recommendation is based on the author's cumulative experience with this rare condition. The esophageal lumen in patients with multiple esophageal rings is typically much narrower than in patients with lower esophageal rings. Medical therapy alone is usually unsuccessful. The treatment of choice is mechanical dilation. Unlike lower esophageal rings, multiple esophageal rings are tighter, and dilation should be performed very slowly using the smallest size dilator that encounters moderate resistance on initial passage into the esophageal lumen. Initially, only one dilator should be used, with serial dilations reserved for later sessions. Starting with a 20-30F dilator is not uncommon. Transient chest pain from mucosal tear is common after dilation in this population.
  • Patients with multiple rings may be presenting with eosinophilic esophagitis. Dilation in these patients should be performed with care, as deep mucosal tears and esophageal perforations may occur.
  • For esophageal rings refractory to esophageal dilation, therapeutic success using neodymium:yttrium-aluminum-garnet (Nd:YAG) laser therapy has been reported. In a study of 14 patients by Hubert et al, Nd:YAG laser incision of lower esophageal rings provided good symptomatic relief.
  • Like distal esophageal rings, most esophageal webs are asymptomatic and do not require treatment. Mild symptoms often can be treated with diet modification and lifestyle changes (see Patient Education). If these conservative measures are unsuccessful, esophageal dilation with mechanical bougie is the next step in treatment. Esophageal dilation with endoscope, bougie, and an esophageal balloon is effective in disrupting esophageal webs, resulting in long-term relief.
  • Like esophageal rings, postdilation barium study may reveal a persistent esophageal web despite symptom relief. Successful treatment of an esophageal web using Nd:YAG laser has been reported, but this treatment rarely is required. In patients with associated disorders, such as iron deficiency, inflammatory diseases, or chronic graft versus host disease, treating the underlying disorders is warranted.
  • New technology in endoscopic dilation has recently been studied by Jones et al in a group of 26 patients who presented with dysphagia.
  • • The InScope Optical Dilator that allows actual visualization during dilation was used on these patients. Seventeen patients had evidence of peptic stricture, and 9 with Schatzki ring were dilated. Eighteen of these 26 patients reported either significant or complete resolution of the dysphagia at week 3 postdilation.
    • The dilations were performed by 2 operators that related the experience similar to the use of bougies with respect to intubation and tactile response. The significant benefit reported was increased visualization during dilation.
    • The authors concluded that larger scale trials should be undertaken to validate the theory of direct visualization as an added benefit to esophageal dilation. Patients with eosinophilic esophagitis may benefit the most by this method as dilation of multiple rings may be aborted if excessive tear is seen.

Surgical Care

Esophageal rings and webs rarely need surgical therapy.

• Endoscopic sphincterotomy: Endoscopic electrocautery incision using a papillotome catheter was reported to be successful in alleviating symptoms associated with refractory lower esophageal rings in 2 studies involving 7 and 17 patients.
• • In the first study, 7 patients were observed for as long as 36 months with only 1 patient requiring a second treatment at 6 months and 1 patient developing chest pain after treatment. Whether the single patient that required a second treatment developed any symptoms is not clear. The patient continued to have persistent dysphagia from the ring but no new symptoms, unlike the patient who developed new-onset chest pain, which is likely a complication from the treatment.
  • In the second study, 17 patients had mean follow-up care of 14 months, with 3 patients requiring a second treatment and 1 patient having bleeding.

Consultations

Refer patients with symptomatic esophageal rings or webs to a gastroenterologist.

Diet

Patients with mild symptoms from esophageal rings or webs should modify their diet and eating habits.

• Soft food, such as pasta, vegetables, and carbohydrates, is less likely than meat to become lodged in the esophagus.
• Advise patients to eat slowly, chew thoroughly, and cut large chunks of food into smaller pieces.

Activity

Modification of physical activities is not necessary.

MEDICATION

Section 7 of 11  

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• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Therapy targets underlying causes of esophageal rings and webs. Antireflux therapy is indicated if GERD is associated with esophageal rings or webs. Evidence of GERD includes classic symptoms of pyrosis (heartburn worsens after meals or when lying flat) and extraesophageal symptoms (chronic cough, globus sensation, hoarseness, asthmalike symptoms refractory to bronchospasm therapy). Other evidence for GERD includes esophagitis, peptic strictures, and abnormal acid exposure on esophageal pH monitoring. Iron therapy is indicated for PVS and iron deficiency anemia.

Drug Category: Proton pump inhibitors

Decreases gastric acid secretion by inhibiting the parietal cell H⁺/K⁺ ATP pump.

Drug Category: Minerals

Used to treat iron deficiency anemia.

FOLLOW-UP

Section 8 of 11  

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• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Further Inpatient Care

• Esophageal rings and webs usually are managed in the outpatient setting.

Further Outpatient Care

• Patients with recurrent symptoms from esophageal rings and webs require repeat esophageal dilation. Repeat esophageal dilation is safe and can relieve symptoms long-term.

In/Out Patient Meds

• Histamine type 2 (H2)-receptor antagonists may be used for mild-to-moderate GERD symptoms. These include cimetidine, famotidine, and ranitidine.
• For severe GERD symptoms, proton pump inhibitors (eg, omeprazole, lansoprazole, rabeprazole, pantoprazole) are recommended.

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. Signs of esophageal obstruction are dysphagia and an inability to swallow secretion. This is a medical emergency, and prompt endoscopy with removal of the obstructed food bolus is warranted. Intravenous glucagon is not an effective therapy. 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. The subgroup of patients who may be at risk of this rare complication is unclear.
• Esophageal rings may progress to a stricture, possibly due to underlying GERD. Aggressive GERD management may be needed.
• For unknown reasons, patients with PVS are at a higher risk of esophageal carcinoma.
• Celiac disease may present as PVS. Test for antigliadin and antiendomysial antibodies. Duodenal biopsy is recommended in patients with PVS.

Prognosis

• 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.
• Patients with recurrent dysphagia after dilation usually respond to repeat dilation. Surgery rarely is needed.

Patient Education

• Following are 3 phases of treatment for patients with symptomatic esophageal rings or webs and chronic GERD:
• • Phase 1 - Lifestyle modification; weight reduction to ideal body weight; raise the head of bed by 6 inches; stop tobacco and alcohol use; avoid eating less than 2 hours prior to bedtime; limit intake of peppermint, spearmint, spicy food, tomato products, caffeine, and greasy food
  • Phase 2 - Medical treatment with either an H2-receptor antagonist or a proton pump inhibitor
  • Phase 3 - Fundoplication surgery
• Instruct patients with dysphagia to change their diet and eating habits. A liquid diet is safe. Patients should eat slowly, chew their food carefully, avoid alcohol, and cut food into smaller pieces.
• For excellent patient education resources, visit eMedicine's Heartburn/GERD/Reflux Center; Procedures Center; Blood and Lymphatic System Center; Esophagus, Stomach, and Intestine Center; and Cancer and Tumors Center. Also, see eMedicine's patient education articles Reflux Disease (GERD), Gastrointestinal Endoscopy, Anemia, Celiac Sprue, and Cancer of the Esophagus.

MISCELLANEOUS

Section 9 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Medical/Legal Pitfalls

• All patients with dysphagia should receive an endoscopy to help establish the diagnosis. Barium radiography may not distinguish a benign from a malignant intraluminal abnormality of the esophagus. Endoscopy allows biopsy of lesions when any doubt exists.
• Although no guidelines have been established for patients with PVS, screening upper endoscopy every 1-2 years for postcricoid malignancy is prudent.

MULTIMEDIA

Section 10 of 11  

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• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Media file 1:  Diagram of A, B (Schatzki), and C rings in the distal esophagus. The B ring marks the proximal border of a hiatal hernia. V=vestibule and HH=hiatal hernia.
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Media file 2:  Schatzki ring with a distal hiatal hernia.
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Media file 3:  Schatzki ring on barium swallow.
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Media file 4:  Meat (chicken) impaction within a Schatzki ring.
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Media file 5:  Multiple esophageal rings found throughout the entire esophagus.
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Media file 6:  An upper esophageal web (arrow) in a patient with Plummer-Vinson syndrome.
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Media file 7:  Upper gastrointestinal series showing upper esophageal webs (small arrows) in a patient with Plummer-Vinson syndrome. Incidental finding of a small Zenker diverticulum (large arrow).
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Media type:  X-RAY

Media file 8:  Postcricoid esophageal web and an inlet patch (arrows outlining the border).
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Media type:  Photo

REFERENCES

Section 11 of 11

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

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Article Last Updated: Jun 27, 2006