4 
Benign Disease of the Esophagus
Neil R. Floch
ESOPHAGEAL RINGS AND WEBS
Esophageal rings and webs are growths of tissue that partially obstruct
the lumen of the esophagus. The prevalence of esophageal rings and
webs has not been determined, as most patients are asymptomatic.
Most lesions are found incidentally at the time of a radiologic study
or endoscopy in 18%. No consensus exists as to the cause, location, or
significance of the rings.
Rings are categorized as “A” and “B,” formerly known as Shatzki
rings (Fig. 4.1). They are most common distally but may occur along
the entire length of the esophagus. The structure described as an “A”
ring is believed to be a normal smooth muscle contraction of the distal
esophagus located at the gastroesophageal junction (GEJ). “A” rings are
rare and found mostly in children at the time of a barium swallow or
esophagogastroduodenoscopy (EGD). Symptomatic patients present
with intermittent dysphagia to solid food.
In 1953, Schatzki first reported a circumferential stricture, or ring,
at the GEJ known as a “B” ring. It is composed of the connective tissue
and the muscularis mucosa at the squamocolumnar junction and is
less than 0.5 cm in length. It has also been attributed to the impression
of the diaphragm at the LES. Barium esophagrams performed for
symptom of dysphagia demonstrate esophageal “B” rings in 6% to 14%
of patients. They are associated with hiatal hernias in 97% patients. Of
patients with a Schatzki “B” ring, 65% have reflux, 50% erosive esopha-
gitis, and 25% have a nonspecific dysmotility disorder. It is believed
that over time, a ring may progress to form a stricture.
The differential diagnosis of a ring includes a congenital web, gas-
troesophageal reflux disease, or carcinoma-induced strictures. Eosino-
philic esophagitis and reflux may play a role in the development of a
Schatzki or “B” ring. Some evidence indicates that a ring may have a
protective effect from Barrett esophagus. A Schatzki or “B” ring may
be a rare cause of swallow syncope.
An esophageal web is less than 2 mm in diameter and encroaches on
the esophageal lumen. It is covered with squamous epithelium and is
most commonly found at the cricoid location of the cervical esophagus.
The cause of esophageal webs is unknown but they have been thought
to be due to chronic GERD. Evidence of reflux as an origin suggests
that webs may progress to strictures if reflux is untreated. Congenital
development has also been suggested. Webs may be associated with a
Zenker diverticulum, iron-deficiency anemia, and chronic graft-versus-
host disease following bone marrow transplantation. Webs may also
be an extracutaneous manifestation in 14% to 33% of patients with
epidermolysis bullosa, bullous pemphigoid, and pemphigus vulgaris.
Clinical Picture
Most patients are asymptomatic, but many have symptoms of dysphagia
to solids such as meat, bread, and hard vegetables. Symptoms may be
intermittent and patients may modify their eating behavior by increased
26
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chewing and the avoidance of certain foods. The severity of the problems
depends on the degree of the narrowing. An esophageal lumen of less
than 39 Fr or 1.3 cm is likely to result in symptoms of dysphagia. Analysis
of the data by Schatzki indicates that decreasing the ring’s diameter by
1 mm results in a 46% increase in the incidence of dysphagia. Patients
may present with complete obstruction from a pill or a food at the site
of a ring or web.
Diagnosis
A Schatzki ring is diagnosed by barium esophagram, which reveals two
protrusions of less than 0.3 mm located several centimeters above the
diaphragm. The protrusions resemble pencil tips at the GEJ. Muscular
rings found on barium esophagram may be transient; they are 0.5 cm
wide, multiple, symmetric, indentations. Swallowing a marshmallow bolus
results in impaction in 75% of patients during a barium esophagram but
this can increase the diagnostic yield from 17% to 100%. Manometry
usually reveals high-amplitude contractions. Upper endoscopy is less
sensitive in detecting rings and webs and may reveal a web as a smooth,
noncircumferential membrane when the esophagus is fully distended.
The Shatzki ring is almost always associated with a sliding hiatal hernia.
A biopsy performed at endoscopy may reveal a submucosa with basal
cell hyperplasia, hyperkeratosis, and eosinophils.
Treatment and Management
Patients should chew food thoroughly to prevent impaction. Esopha-
goscopy with bolus extraction is the simplest measure to relieve obstruc-
tion. Glucagon administration may reduce spasm and allow an obstructed
object to pass. Both “A” rings and webs may be torn during upper
endoscopy, whereas most other rings are amenable to dilatation. If
dilatation is needed, a 50-Fr bougie or 18- to 20-mm balloon dilator
may be used. One dilatation is usually all that is needed. Esophageal
rings should be biopsied to eliminate the diagnosis of eosinophilic
esophagitis, defined by greater than 18 eosinophils per high-power field.
Esophageal rings associated with GERD warrant a 6-week treatment
with proton-pump inhibitor (PPI) medication. Symptoms recur at 1,
2, and 5 years in 32%, 65%, and 89% of patients, respectively. Repeat
dilatation and chronic PPI therapy may be indicated. The ring may be
incised if repeat dilatations are ineffective. Dilatation after incision is
performed if there is further failure.
PLUMMER-VINSON SYNDROME
This disease was named after two Americans, physician Henry Stanley
Plummer and surgeon Porter Paisley Vinson. It usually occurs in eden-
tulous, premenopausal, married women and rarely in men (Fig. 4.2).
Plummer-Vinson syndrome (PVS) develops over months to years,
manifests in the fourth to fifth decades of life, and is more common
in Scandinavian countries than in the United States. It had been
 CHAPTER 4  Benign Disease of the Esophagus 27

Barium retained in vestibule and hernial

sac; distal tubular esophagus and inferior
esophageal sphincter region contracted;
lower esophageal ring indicated by notches
Tubular
esophagus

Location of
inferior
esophageal
sphincter

Vestibule

Lower esophageal
ring
Sliding hernia
Peritoneum
Tubular esophagus
Diaphragm
Location of
Phreno- inferior esophageal
esophageal sphincter
ligament
Vestibule
Lower esophageal
ring
Sliding hernia
Peritoneum

Phrenoesophageal ligament

Lower esophageal
ring (lower arrow);
Diaphragm
also faint ring at level
of inferior esophageal
sphincter
(upper arrow)

Fig. 4.1  Schatzki Esophageal Ring Formation.

attributed to iron-deficiency anemia in northern countries in the past. Clinical Picture

It has also been found in children and adolescents. PVS is a premalignant
process, as it is a risk factor for the development of squamous cell
carcinoma of the esophagus and hypopharynx.
The hallmark finding of PVS is a weblike structure that originates
on the posterior wall of the cervical esophagus between the hypophar-
ynx, 1 to 2 cm below the cricopharynx. The web is thick at its base and
becomes thinner as it protrudes inward, where it has the consistency
of paper. The cause of the web is unknown, but genetic factors and
nutritional deficiencies may play a role.
There is a triad of iron-deficiency anemia, dysphagia, and a cer-
vical esophageal web, although weakness is also one of the most
common characteristic symptoms. Dysphagia of solids occurs fre-
quently but dysphagia of liquids is rare. Odynophagia may also be
present. Oral symptoms are common, and patients complain of glos-
sitis or burning of the tongue and oral mucosa. Possible atrophy of
lingual papillae produces a visually smooth and shiny glossal dorsum.
Patients may have stomatitis with painful cracks in the angles of a
dry mouth.

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28 SECTION I Esophagus

Plummer–Vinson syndrome

Hypochromic anemia

Glossitis

Esophageal
(after filtration) web

Achlorhydria

Barium study showing

esophageal web

Simple esophageal web without other Web: Esophagoscopic view

manifestations of Plummer–Vinson syndrome

Fig. 4.2  Plummer-Vinson Syndrome.

Atrophic mucosa may involve the esophagus and the hypopharynx.
Patients with PVS may also have achlorhydria, brittle fingernails (which
may indicate vitamin deficiency), and splenomegaly (33% of patients).
Anemia may result in hemoglobin levels that are 50% of normal values.
Other findings include: splenomegaly and an enlarged thyroid.
Diagnosis
Barium esophagraphy reveals a fibrous web under the cricopharyngeus
muscle seen as a filling defect below the level of the cricoid cartilage
in the esophagus. The web may involve the entire circumference of the
esophagus and is thought to be the cause of dysphagia. Serum tests

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 CHAPTER 4  Benign Disease of the Esophagus
may reveal hypochromic microcytic anemia, consistent with iron-
deficiency anemia. Biopsy of mucosa should demonstrate epithelial
atrophy and submucosal chronic inflammation as well as possible epi-
thelial atypia or dysplasia.
Treatment and Management
Treatment of PVS is primarily aimed at correcting the iron-deficiency
anemia. Patients should receive iron supplementation as well as foods
high in iron content, which may lead to resolution of dysphagia prior
to resolution of the anemia. With treatment, symptoms such as dys-
phagia, as well as oral and tongue pain, usually resolve. Iron supple-
mentation usually resolves the anemia.
Dilatation of the esophageal web may be necessary when significant
obstruction of the esophageal lumen is present. Only a small amount
of pressure ruptures a web, so introducing an endoscope is usually
therapeutic because it reestablishes a normal passage through the esopha-
gus. Annual surveillance with upper endoscopy for esophageal squamous
cell carcinoma has not been shown to improve patient outcomes.
Course and Prognosis
Iron replacement therapy reverses anemia, and strictures are almost
always dilated successfully. Unfortunately malignant lesions of the oral
mucosa, hypopharynx, and esophagus may be observed in as many as
100% of patients with PVS on long-term follow-up.
ADDITIONAL RESOURCES
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2002.
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29
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