Plummer Vinson Syndrome

 Author: Louis-Michel Wong Kee Song, MD; Chief Editor: Julian Katz, MD

Updated: Jun 4, 2013

Background

The association of postcricoid dysphagia, upper esophageal webs, and iron deficiency
anemia is known as Plummer-Vinson syndrome (PVS) in the United States and Paterson-
Brown Kelly syndrome in the United Kingdom.[1, 2, 3, 4, 5] The term sideropenic dysphagia
has also been used, since the syndrome can occur with iron deficiency (sideropenia), but
it is not associated with anemia.[6]

Pathophysiology

The pathogenesis of PVS remains speculative, and the existence of the syndrome has
been challenged. Postulated etiopathogenic mechanisms include iron and nutritional
deficiencies, genetic predisposition, and autoimmune factors, amongst others.[7, 8]

The prevalent iron deficiency theory remains controversial. Older reports have implicated
iron deficiency in the pathogenesis of esophageal webs and dysphagia in predisposed
individuals. The depletion of iron-dependent oxidative enzymes may produce myasthenic
changes in muscles involved in the swallowing mechanism, atrophy of the esophageal
mucosa, and formation of webs as epithelial complications.

The improvement in dysphagia after iron therapy provides evidence for an association
between iron deficiency and postcricoid dysphagia.[2] Anecdotal reports have also been
made of patients with PVS exhibiting impaired esophageal motility (with dysphagia) that
recovers following iron therapy.[9] Moreover, the decline in PVS seems to parallel an
improvement in nutritional status, including iron supplementation.

However, population-based studies have shown no relationship between postcricoid

dysphagia and anemia or sideropenia.[6] Other studies have demonstrated that patients
with webs are as likely to be iron deficient as controls, and webs are often found in
patients without iron deficiency or dysphagia. Lastly, the iron deficiency theory does not
explain the predilection of webs for the upper esophagus and the rarity of the syndrome
in populations in which chronic iron deficiency is endemic (eg, eastern and central
Africa).[2]

PVS has also been viewed as an autoimmune phenomenon. The syndrome has been
associated with autoimmune conditions, such as rheumatoid arthritis, pernicious anemia,
celiac disease, and thyroiditis.[10, 7] In one study, a significantly higher proportion of
patients with PVS had thyroid cytoplasmic autoimmune antibodies compared to controls
with iron deficiency. The autoimmune theory, however, has gained little acceptance to
date.

A complicated inlet patch (heterotopic gastric mucosa) has also been implicated in the
pathogenesis of PVS.[11, 12] An ulcerated inlet patch in the upper esophagus can cause
stricturing (weblike formation) and bleeding (with subsequent iron deficiency).[13]
However, most studies with biopsy or autopsy specimens have not demonstrated the
presence of gastric metaplasia in the samples.

Epidemiology
Frequency

United States

Reliable prevalence data on PVS are lacking. The syndrome is now a rarity, and its
decline has been attributed to better nutrition and health care. Webs may be found in 5-
15% of selected patients presenting with dysphagia, but most of these patients do not
have PVS.

International

In the first half of the 20th century, PVS was a relatively common finding, particularly
among middle-aged Scandinavian women. The rapid fall in prevalence of the syndrome
in the latter part of the 20th century has paralleled an improvement in nutritional status,
including widespread addition of iron to flour.[14]

Mortality/Morbidity

Morbidity issues primarily relate to diet modification and repeat esophageal dilations
(with a small risk of perforation) in patients with PVS who have recurrent dysphagia.
Updated mortality data are unavailable for this rare syndrome, but, presumably, mortality
is low.

Race

PVS has mainly been described in whites.

Sex

PVS is more frequently observed in women. In earlier studies from Scandinavia, up to

90% of patients were women.
Age

The typical age range at diagnosis is 40-70 years. A handful of cases have been reported
in children.[15]

History

Dysphagia, if present, is typically intermittent and limited to solids.[16] It is usually felt in

the throat.

Choking spells and aspiration may occur because of the proximal location of the web.

Weakness, fatigue, and dyspnea are secondary to iron deficiency anemia.

Weight loss is uncommon.

Physical

Manifestations of iron deficiency (with or without anemia) may be evident, including the
following:

 Angular cheilitis
 Glossitis[17]
 Koilonychia (spoon nails)
 Pallor

Splenomegaly, edentia, and enlarged nodular thyroid glands are also described in a few
patients with PVS.

Causes

The cause of PVS is unclear. Proposed etiopathogenic mechanisms include iron and
nutritional deficiencies, genetic predisposition, and autoimmunity.[7]

Seek the underlying cause of iron deficiency anemia (eg, gastrointestinal blood loss,
celiac sprue).[18]

Proceed to Differential Diagnoses

Differential Diagnoses
 Esophageal Cancer
 Esophageal Webs and Rings
 Hypopharyngeal Cancer
 Iron Deficiency Anemia
Laboratory Studies

Perform CBCs, peripheral blood smears, and iron studies (eg, serum iron, total iron-
binding capacity [TIBC], ferritin, saturation percentage) to confirm iron deficiency, with
or without hypochromic microcytic anemia.

Imaging Studies

Barium esophagram and videofluoroscopy are the most sensitive methods and diagnostic
tests of choice to detect esophageal webs.

Conventional barium swallow may detect the web, which characteristically appears as a
thin projection off the postcricoid, anterior esophageal wall. The web is best visualized
on lateral view (see following image). On occasion, multiple webs can be observed.

Postcricoid web. Courtesy of Alan Cameron, MD.

If conventional barium swallow findings are negative or equivocal and a high index of
suspicion remains, a video swallow study can be performed. The latter can distinguish
subtle true webs from weblike formations (false webs) due to insignificant mucosal
foldings and submucosal phenomena.

Other Tests

Order specific tests, as necessary, for evaluation of the etiology of iron deficiency.

Procedures

Esophagogastroduodenoscopy may be used. A thin web can be easily ruptured and can go
unnoticed during passage of the endoscope into the upper esophagus. If webbing is
suspected, the endoscope should be advanced carefully under direct vision through the
upper esophageal sphincter. The web typically appears as a thin mucosal membrane
covered by normal squamous epithelium. Most webs are located along the anterior
esophageal wall in the shape of a crescent (see following images), but a few are
concentric.
 Upper esophageal web.
Upper esophageal web.

Carefully examine the upper esophagus for presence of an inlet patch. The differential
diagnosis at endoscopy should also include inflammatory or infectious stricture,
postcricoid cancer, or extrinsic compression from a submucosal venous plexus.

Potential causes of iron deficiency anemia may be found, including Cameron erosions
within a large diaphragmatic hernia or angioectasias.[18] It is also prudent to obtain small
bowel biopsies at the time of endoscopy to exclude villous atrophy from celiac sprue as
the cause of iron deficiency anemia.[10]

Histologic Findings

The web is composed of a thin layer of normal squamous mucosa and submucosa.
Sometimes, chronic inflammatory cells may be observed in the submucosa.

Proceed to Treatment & Management

Medical Care

Treat iron deficiency and its underlying cause.

 Iron replacement is necessary to correct the anemia, if present, and to resolve

most of the physical signs of iron deficiency. The necessity for continued iron
treatment is doubtful other than for anemia correction.[19]
 Dysphagia may improve with iron replacement alone, particularly in patients
whose webs are not substantially obstructive. Dysphagia caused by more
advanced webs is unlikely to respond to iron replacement alone and, thus, is
managed with mechanical dilation.
 Address the cause of the iron deficiency (eg, celiac sprue, bleeding angiectasias).

Treat dysphagia and the web.

  Aside from iron replacement, diet modification may be sufficient in mildly
symptomatic patients (see Diet). Those with significant and long-standing
dysphagia usually require mechanical dilation. The web can often be disrupted
during simple passage of the endoscope into the esophagus. Otherwise, passage of
a bougie (eg, Savary dilator) is quite effective. In most cases, passage of a single
large dilator is adequate and is thought to be more effective than serial
progressive dilations.
 Fluoroscopic guidance is usually not required unless a tight web precludes further
passage of the endoscope. The proximal location of the webs in PVS makes
endoscopic balloon dilation difficult, but it has been performed successfully by
radiology under fluoroscopic guidance.[20, 21]
 ND:YAG laser therapy has also been reported as a successful means of disrupting
an esophageal web.[22] This modality is rarely required.
 Needle-knife electroincision has been described as a therapeutic alternative to
dilation.[23]

Surgical Care

Surgery is rarely needed and is reserved for patients whose webs are recalcitrant to
dilation or associated with Zenker diverticulum.

Consultations

Gastroenterologists - For management of dysphagia requiring dilation

Diet

Advise patients to eat slowly and chew thoroughly. Solid foods should be prepared and
cut in small pieces, especially meats.

Activity

Unrestricted

Proceed to Medication

Read more about Plummer-Vinson Syndrome on Medscape

Related Reference Topics

Medication Summary

The goals of pharmacotherapy are to reduce morbidity and to prevent complications.

Iron preparations
Class Summary

Iron replacement is an important component of the treatment of PVS.[19]

View full drug information

Ferrous sulfate (Feosol)

A nutritionally essential inorganic substance used to treat iron deficiency anemia

associated with PVS.

Proceed to Follow-up

Read more about Plummer-Vinson Syndrome on Medscape

Related Reference Topics
 Malignant Tumors of the Mobile Tongue
 Hypopharyngeal Cancer

 Dermatologic Manifestations of Gastrointestinal Disease

Further Inpatient Care

PVS is usually managed on an outpatient basis.

Further Outpatient Care

Perform follow-up CBCs and iron studies in 3 months, after initiation of iron
replacement, to document resolution of sideropenia and anemia.

Provide follow-up care for patients whose treatment has been initiated for the specific
cause, if any, of the iron deficiency anemia.

Repeat esophageal dilations may be required in patients with recurrent dysphagia.

Inpatient & Outpatient Medications

Ferrous preparations are administered to correct the deficiency in iron (with or without
anemia).
Complications

Although reports are inconsistent, patients with PVS seem to be at an increased risk for
hypopharyngeal and esophageal cancers. A high prevalence of hypopharyngeal cancers in
Swedish women in the 1930s and 1940s was attributed to PVS.[24]

The reported frequency of postcricoid carcinoma associated with PVS varies (4-16% in
older studies) and remains a matter of debate.[25]

Prognosis

Prognosis is generally good, unless PVS is complicated by hypopharyngeal or esophageal

carcinoma.[26, 27] Patients with PVS usually respond well to iron therapy, diet modification,
and, if necessary, esophageal dilation.

Patient Education

Instruct patients with PVS on dietary modification and eating habits.

Educate patients with PVS about the potential association of PVS and hypopharyngeal
and esophageal cancers to ensure appropriate follow-up care.

For excellent patient education resources, visit eMedicineHealth's Digestive Disorder

Center and Thyroid and Metabolism Center. Also, see eMedicineHealth's patient
education articles Rheumatoid Arthritis, Anemia, Celiac Sprue, and Thyroid Problems.