The Turkish Journal of Gastroenterology
2005, Volume 16, No 4, Page(s) 224-227
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|Plummer-Vinson syndrome and dilation therapy:
A report of two cases
|Fikret DEMİRCİ1, M. Cemil SAVAŞ1, Necip KEPKEP2, Vahap OKAN3, Mehmet YILMAZ4,
Mehmet BÜYÜKBERBER1, M. Taner GÜLŞEN1
|Division of 1Gastroenterology and 4Hematology, Department of 3Internal Medicine, 2Obstetrics and Gynecology, Gaziantep
University, School and Hospital of Medicine, Gaziantep
|Keywords: Plummer-Vinson syndrome, dysphagia, upper
esophageal web, dilation therapy.
Plummer-Vinson syndrome is known as the association of postcricoid
dysphagia, upper esophageal web, and iron deficiency
anemia. Although correction of iron deficiency may result in resolution
of dysphagia and sometimes disappearance of the
webs, dilation therapy is usually necessary to remove webs and
relieve dysphagia. We report two cases of Plummer-Vinson
syndrome. Both patients presented with significant and longstanding
dysphagia, sideropenia, glossitis and koilonychia.
Our two patients had occasional choking and aspiration episodes
at eating and endoscope did not pass through at the level of
the upper esophagus. Patients’ esophagograms revealed the presence
of webs in part of the post-cricoid region. Both patients
were treated with esophageal bougienage or balloon dilation,
and iron supplementation. The patients were examined periodically
for two years after the initial treatment and found to be in
good general condition.
Plummer-Vinson syndrome is characterized by
cervical dysphagia, iron deficiency anemia, and
upper esophageal web or webs. While this syndrome
is known as Plummer-Vinson syndrome in the
United States, it is known as Paterson-Brown
Kelly syndrome in the United Kingdom. It has been
known since the beginning of the 20th century.
Plummer (1) established the syndrome for the first
time in 1912 and has published 21 cases with diffuse
dilation of the esophagus and spasm of the
upper esophagus without anatomic stenosis. Then
Vinson (2), Plummer’s pupil, published another
case with angulation of the esophagus. Paterson
and Kelly (3, 4) described for the first time the
characteristic clinical signs of the syndrome: anemia,
dysphagia, glossitis, cheilitis, iron deficiency,
Herein we present two patients with significant
and long-standing dysphagia and sideropenia.
Both patients were treated with dilation therapy
and iron supplementation.
A 47–year-old woman presented with intermittent
and long-standing (five years) symptom of dysphagia.
She had a history of five births and two abortions. The patient informed us that she sometimes
took iron salts because of anemia of iron deficiency.
The clinical examination showed signs of
iron deficiency such as cheilitis and spoon-shaped
Hematological tests showed the presence of an
iron deficiency anemia (serum Fe 40 μg/dl, ferritin
50 ng/ml, Hb 9.6 g/dl, Ht 29%). The examination of
peripheral blood smear revealed hypochromia and
aniso-poikilocytosis. Tests for other causes of chronic
anemia were negative. The patient’s esophagogram
revealed the presence of a web in the subcricoid
region (Figure 1) and the endoscope did not
pass through at the level of the web. The patient
was treated with oral ferrous agent, 150 mg daily
(p.o.) for three weeks, in addition to esophageal dilation
with Eder-Pustov dilators. After dilation,
the endoscope passed through the esophagus easily,
but there were remnants of the web (Figure
2). The remaining esophagus, stomach and duodenum
were normal. The patient was given iron salt
for four months until the hematological tests normalized.The remnant webs disappeared in the follow-
up upper endoscopic examination. Two years
later the patient was found to be well and she continues
to be healthy.
A 45-year-old woman presented with progressive
dysphagia and repeated episodes of menorrhagia
over the preceding three-year period. The patient
reported that she sometimes experienced choking
and aspiration at eating. The clinical examination
revealed signs of iron deficiency anemia, such as
glossitis and spoon-shaped nails. Ultrasonographic
examination of the lower abdomen showed normal
uterus and the diagnostic curettage was also
Esophagogram showed a ring in part of the upper
esophagus. The esophagoscopy confirmed the
presence of a web in the sub-cricoid region and the
instrument did not pass through at the level of the
web (Figure 3).
Hematological tests showed signs of iron deficiency
anemia including hypochromia, microcytosis
(serum Fe: 47 g/dl, serum ferritin: 52 ng/ml, Ht
33%, Hb 10.8 g/dl, MCHC 28%). The examination
of peripheral blood smear revealed the presence of
hypochromia, poikilocytosis and target cells. The
patient was investigated for other causes of chronic
anemia, and all results were negative.
The patient was treated with oral ferrous agent,
150 mg daily (p.o.) for three weeks, in addition to
esophageal dilation with a balloon. Balloon dilation
was successful under scopic control. After balloon
dilation, endoscope passed through the esophagus
easily (Figure 4) and the remainder of the esophagus, stomach and duodenum were normal.
She continued the intake of iron until her hematocrit
and ferritin levels were normalized, which
occurred after six months. The patient was found
to be in good general condition two years after diagnosis.
Figure 1. Photographic image of a web
Figure 2. Endoscopic image of web
remnants after bougienage
dilation (arrow, case 1)
Figure 3. Endoscopic image of
esophageal web (case 2)
Figure 4. Appearance of upper esophagus
after balloon dilation
The classical triad of the Plummer-Vinson or Paterson-
Kelly syndrome consists of upper esophageal
web or webs, dysphagia and sideropenic anemia.
In the past, this syndrome was more common
among the Scandinavian population, especially in
rural areas of Sweden, but it is extremely rare today.
The decline in reports and incidence of the syndrome may be related to the improvement in
nutritional status and better treatment of iron deficiency
(5). In Turkey, however, many Plummer-Vinson
syndrome cases are still being reported (6-8).
Cervical dysphagia is the rarest type of dysphagia.
The most common benign cause of cervical type of
dysphagia is upper esophageal web(s). These webs
may be found in approximately 5-15% of patients
with dysphagia. Webs are fragile membranes and
respond well to esophageal dilation therapy (9).
In our cases, dysphagia was the main symptom
that led both patients to seek medical help and dilation
Plummer-Vinson syndrome affects mainly white
women, in the fourth to seventh decade of life, but
some cases in children and adolescents have been
reported (10, 11). Prevalence data are not reliable
because many studies done on patients with
dysphagia have not included the hematological parameters
The pathogenesis of the syndrome remains unclear,
but possible etiopathogenetic mechanisms include
iron deficiency, genetic predisposition or autoimmune
disorder. It is reported that iron deficiency
leads to the reduction of iron-dependent oxidative
enzymes, which results in gradual degradation
of muscles of the pharynx. As a result, mucosal
atrophy leads to development of webs (9). It
was reported that in a patient with Plummer-Vinson
syndrome, iron deficiency caused esophageal
motility decrease; new motility studies showed
normal amplitude of contraction after iron therapy
(13). In another patient with Plummer-Vinson
syndrome, iron therapy alone resolved the patient’s
symptom, and the patient’s esophagogram
showed 30% benign upper esophagal stricture, reduced
from 90% before therapy (14). But population
studies do not confirm the etiologic association
between iron deficiency and web formation. Furthermore,
this theory does not explain the presence
of these webs exclusively in the upper part of
the esophagus (9, 15). Finally, the precise role of
iron deficiency in Plummer-Vinson syndrome has
yet to be defined and remains a subject of debate.
Genetic transmission has gained little acceptance
to date. But some families and relatives have been
reported as having this syndrome. Furthermore,
Plummer-Vinson syndrome and sideropenic
dysphagia are rare among blacks (9, 16).
Plummer-Vinson syndrome may be accompanied
by pernicious anemia, thyroiditis or celiac disease.
Celiac disease is a recognized cause of chronic iron
deficiency and should be considered as an etiological
factor in sideropenic dysphagia (17-20).
The most important symptom of this syndrome is
dysphagia, which is limited to solid foods and is
generally intermittent. Patients may also complain
about choking and aspiration episodes (21). The
symptoms of sideropenic anemia such as weakness,
pallor, fatigue and tachycardia may dominate
the clinical picture. The clinical signs of sideropenic
anemia including angular cheilitis, glossitis
and koilonychia may also be seen on physical examination.
The treatment of Plummer-Vinson syndrome is
iron supplementation, and iron therapy may be
necessary even though hematologic parameters
are normal in the presence of a web formation (13,
14). The patients who have choking and aspiration
episodes need dilation therapy together with
iron supplementation. Endoscopic dilation is
simple and a chosen procedure in treatment of the
syndrome and cervical web of the esophagus (6, 7).
Our two patients had choking and aspiration episodes
and the endoscope did not pass through at
the level of the web.
It is important that this syndrome be differentiated
from other causes of dysphagia, e.g. malignant
tumors, strictures, esophageal burns, heterotopic
gastric mucosa or blistering skin disease.
Plummer-Vinson syndrome is known to be associated
with upper alimentary tract cancer and surveillance
endoscopy is recommended (22, 23). Celiac
disease, large diaphragmatic hernia, gastric
cancer, Sjogren’s syndrome, and pernicious anemia
may cause Plummer-Vinson syndrome. It is
proposed that these diseases may cause iron deficiency
and then result in upper esophageal webs
(17-20, 24, 25).
In our patients, treatments were achieved by dilation
and iron therapy. Although bougienage dilation
was preferred in previous reports (6, 7), we experienced
that balloon dilation was efficient and
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