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Original Articles: Gastroenterology

Plummer-Vinson Syndrome in Children

Butori, Mathilde*; Mahmoudi, Sana*; Dugelay-Ecochard, Emmanuelle*; Belarbi, Nadia; Bellaïche, Marc*; Hugot, Jean-Pierre*; Viala, Jérôme*

Author Information
Journal of Pediatric Gastroenterology and Nutrition: November 2015 - Volume 61 - Issue 5 - p 547-552
doi: 10.1097/MPG.0000000000000842
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What Is Known

  • Plummer-Vinson syndrome is a rare cause of dysphagia in children because of an upper esophageal web.
  • In adults, Plummer-Vinson syndrome may be associated with iron deficiency or diverse inflammatory disorders.
  • In adults, iron supplementation is frequently sufficient to control dysphagia.
  • What Is New
  • All of the reported pediatric cases of Plummer-Vinson syndrome were correlated to iron deficiency with no immunological pathology.
  • Barium swallow with profile incidence is necessary to diagnose Plummer-Vinson syndrome.
  • Two-thirds of the children needed an endoscopic dilation of the esophageal web.

Upper cervical dysphagia is a rare symptom in children, which requires etiological explorations. Several causes may lead to dysphagia, including congenital stenosis of the esophagus, eosinophilic esophagitis, esophageal peptic stenosis, achalasia, hypertrophy of the cricopharyngeal muscle (1,2), and Plummer-Vinson syndrome (PVS).

PVS, previously called Kelly-Paterson syndrome, is a rare disorder mostly reported in middle-aged women, which associates an iron-deficiency anemia and a proximal dysphagia because of an acquired stenosing web of the esophagus. The clinical picture was first described by Plummer (3) and Vinson (4) in 1912 and 1919, respectively. The esophageal web is a thin mucosal membrane, composed of normal squamous epithelium, which is localized just below the cricopharyngeal muscle and is usually asymmetrically attached to the anterior esophageal wall (5). When biopsies are performed, no inflammatory infiltrate is found (6).

In adults, PVS can be efficiently treated by iron supplementation in most patients, and endoscopic dilation is rarely required to resolve dysphagia. We reported our pediatric cases of PVS, who needed an endoscopic dilation despite a prolonged iron supplementation. Because PVS is exceptional in children, we performed a systematic review of the published pediatric PVS cases.


We retrospectively reported every case of PVS occurring in our tertiary pediatric center between 2000 and 2014. In the medical charts, we collected clinical presentation, radiological and endoscopic features of the lesion, treatment, and evolution of the disease.

We also performed a systematic computerized literature review from January 1960 through September 2014 by using PubMed and EMBASE databases. Articles were selected by using a combination of MeSH terms and text words related to Plummer-Vinson, Kelly-Paterson, Kelly-Brown-Paterson, esophageal web, sideropenic dysphagia, and children. We included all of the studies in French and English reporting at least 1 pediatric case.

This study obtained the agreement of the local committee of ethics (No. 2015/169). All of the families and the Commission Nationale de l’Informatique et des Libertés were informed.


Patient 1

A 5-year-old girl was referred for a progressively increasing dysphagia to solid food that started 18 months before. Since 15 months of age, she experienced a hypochromic microcytic anemia because of iron deficiency (hemoglobin [Hb] 7.91 g/dL, mean corpuscular volume [MCV] 45 μm3, and low level of ferritin). Oral iron supplementation permitted a partial improvement of hematological parameters (Hb 8.7 g/dL, MCV 50 μm3, reticulocytes 90,300/mm3), but the level of serum iron remained low with a transferrin saturation coefficient (TSC) of 3% and normal transferrin level. She was born of consanguineous parents and originated from Guinea. Her father had chronic anemia. Her 10-year-old sister was treated for a refractory microcytic hypochromic anemia by intravenous iron supplementation because of the inefficiency of oral treatment.

Clinical examination revealed pallor, cheilitis, glossitis, and koilonychia. Barium swallow showed an indentation on the anterior esophageal wall (Fig. 1A). Upper endoscopy showed a narrowing web of the postcricoidal esophagus (Fig. 1B). Initial hematological results showed a persistent iron-deficiency anemia in spite of the daily oral iron supplement (Hb 8 g/dL, MCV 50.3 μm3, mean corpuscular hemoglobin concentration [MCHC] 28.8 g/dL, reticulocytes 49,770/mm3, serum iron 1.4 μmol/L, and TSC 2.2%). Stool antigen and serological tests for Helicobacter pylori were positive, and the infection was efficiently treated using a triple therapy for 15 days. Antitransglutaminase antibodies were negative. Oral and intravenous iron supplementations did not treat the dysphagia. Thus, 3 endoscopic sessions of hydrostatic dilation (8, 9, and 10 mm) were performed at 5-week intervals (Fig. 1C). At the end, a moderate stenosis persisted but dysphagia disappeared (Fig. 1D). No clinical relapse occurred with a 10-year follow-up.

Radiological and endoscopic presentations of Plummer-Vinson in 3 new children. In the first patient (A–D), a web of the proximal esophagus (A and B) induced solid dysphagia, which was resistant to iron treatment. Multiple endoscopic dilations (C) were required to reduce stenosis (D). In the second patient, the initial esophageal indentation (E) was improved after prolonged iron supplementation with no dilation (F). Lateral (E–G) and frontal (A and H) incidences are important to diagnose the esophageal web with barium swallow.

Patient 2

A 3-year-old girl experienced a dysphagia to solid food associated with dyspnea. She was born prematurely at week 36 of gestation, with a Fallot tetralogy, complicated by a laryngeal paralysis that required tracheotomy and right adenoidectomy. She also experienced gastroesophageal reflux and hypochromic microcytic anemia related to an iron deficiency (Hb 9.6 g/dL, MCV 67.8 μm3, mean corpuscular Hb 21.3 pg per cell, MCHC 31.5 g/dL). An oropharyngeal fibroscopy was normal. The first upper barium swallow showed a stenosis of the proximal esophagus (Fig. 1E).

Because PVS was suspected, iron was given orally. After normalization of the iron status (ferritin >20 μg/L), she was able to eat mixed food, and food impaction was exceptional. After 1 year of follow-up, a less narrow stenosis persisted (Fig. 1F). Parents refused endoscopic dilation. Dysphagia persisted a further 10 months. Finally, we performed an endoscopy that was normal, and the patient was asymptomatic for the next 6.7 years. No dilation was performed.

Patient 3

An 11-year-old boy experienced a progressive dysphagia to solids for 5 years, with frequent food impactions. He had a history of ventricular septal defect and asthma. He experienced a poor nutritional status with an isolated iron deficiency. An upper barium swallow showed a proximal indentation of the anterior esophageal wall (Fig. 1G and H). The endoscopy showed a cervical esophageal web. Oral supplementation enabled the iron deficit to be corrected (ferritin 23.3 μg/L).

After 3 months of treatment, the dysphagia persisted in part. A new upper barium swallow showed a persistent but less narrowed stenosis. The persistence of the esophageal web was confirmed by endoscopy, and a hydrostatic dilation was performed. After the dilation, the residual stenosis did not allow the endoscope to go through, but dysphagia disappeared 3 months later, and the parents discontinued the follow-up after 2 years with no symptoms.

Review of the Literature

A total of 10 publications reporting 14 pediatric cases met the inclusion criteria (Table 1) (6–15). Excepting 2 previous articles and our own study, all of the other publications were single-case reports. Symptoms of esophageal obstruction (ie, dysphagia or food impaction) were present in all of the cases. The mean dysphagia duration before PVS diagnosis was 12 months (3–60 months).

Clinical, radiological, endoscopic presentations, and treatments of 17 reported PVSs in children

All of the patients were treated for an iron deficiency. Among 15 precisely known treatments, parenteral iron supplementation was required in 3 patients (20%). In 3 other patients, the severity of anemia required a transfusion of red blood cells. The cause of the iron deficiency remained undefined in all of the patients.

Because upper endoscopy was not available in children before 1970, PVS diagnosis was confirmed by endoscopy in 12 of 14 patients (86%). None of the endoscopies were normal. Despite systematic iron supplementation, endoscopic dilation was necessary in 9 of 12 (75%) patients. In 1 patient, the dysphagia disappeared after an endoscopic biopsy of the esophageal web (6). All but 1 dilation were efficient to resolve dysphagia. The follow-up durations were reported in 14 patients with a median of 13.5 months (3–130 months). Neither recurrence of dysphagia nor cancer was reported in this pediatric population.


PVS is defined by a progressive dysphagia, which is because of a single or multiple thin web(s) in the proximal part of the esophagus associated with an iron deficiency. This syndrome is a rare but probably an underrated etiology of dysphagia in children.

PVS is predominantly a disease of middle-aged adults and is very rare in childhood. In children, most cases were reported in adolescence with 10 of 17 children who were >10 years of age. The youngest child reported to date was 20 months old and had experienced dysphagia for 1 year (15). No esophageal web was observed, however, in this very young patient with a normal barium swallow and an endoscopic exploration refused by the parents. PVS diagnosis was thus established only by the rapid reversal of dysphagia after 1 week of iron supplementation. Although most of patients with PVS (89%) were women in adult literature (16), pediatric cases showed a balanced sex ratio.

The dysphagia especially concerns solid foods and is painless, intermittent, or progressive in most cases (9,17). Frequent adaptation of the lifestyle often leads to a prolonged delay before diagnosis. At maximum, an adult diagnosed at the age of 38 years had experienced a dysphagia since childhood (18). In our pediatric review, the mean duration of dysphagia was 12 months before diagnosis. Some PVS patients could be considered as hysterical patients (3). Conversely, the presence of an esophageal web is not systematically symptomatic because only 11% of adult patients reported a dysphagia among 55 patients with a proved proximal esophageal web (19).

Sometimes the symptoms of iron deficiency may dominate the clinical picture with pallor, fatigue, tachycardia, abnormal fingernails, cheilitis, papillary atrophy and fissures of the tongue, glossitis, or even spleen enlargement (5,20). Laboratory examinations usually confirm the diagnosis of anemia related to iron deficiency.

The pathogenesis of the esophageal lesion remains unclear. The near-constant association with severe and prolonged iron deficiency and the regression of symptoms after iron supplementation, however, argue for a causal relation (21,22). Indeed, there seems to be a temporal relation between iron deficiency and PVS because the anemia precedes dysphagia (23), and PVS frequency is reduced when the prevalences of iron deficiency and malnutrition decrease (24). For this reason, an iron deficiency needs to be identified, looking for an inadequate dietary intake, a hampered absorption or an occult bleeding. In adults, the most frequently reported causes of iron deficiency during PVS are celiac disease, increased menstrual blood loss, hiatus hernia, and gastrointestinal bleeding of unknown origin (21,22).

Whatever the reason of iron deficiency, it likely induces a dysfunction of iron-dependent enzymes within epithelial cells, an oxidative stress, and subsequently DNA damages that have been implicated in epithelial changes of the esophageal mucosa (5). These ongoing tissue injuries and healing could cause permanent esophageal mucosal changes leading to web formation (21). Moreover, a decreased contraction amplitude of the esophageal muscle has been reported in iron deficiency with or without esophageal web (23), and transit times of proximal and median esophagus are slower in PVS patients (14). A deficit in pyridoxine was also involved in esophageal dysmotility during PVS (25).

In children, iron deficiency was diagnosed in all of the 17 reported PVS patients. In adults, iron deficiency is not systematically associated with esophageal web in PVS, whereas dysphagia is always required for diagnosis (26). An immunological process involving autoantibodies directed against the esophagus has been suggested (23). Celiac disease, systemic lupus erythematosus, thyroid disease, rheumatoid arthritis, and Sjögren syndrome have been reported in association with PVS (16,27–31). Consequently, PVS diagnosis should prompt a search for immunological causes. It is unclear whether these autoimmune disorders, however, could cause PVS through autoantibody production itself or because of the inflammatory process and iron chelation. Other factors such as a genetic predisposition have been suggested but not proved to play a role in the pathogenesis of PVS. In children, no case of PVS was associated with dysimmunity excepted in a milk allergy in a 20-month-old boy (7). In the latest patient, the esophageal aspect was not explored because of the stenosis.

The radiological findings are most often typical: barium swallow is of great diagnostic value, showing a postcricoid esophageal web that can be single or multiple (5,32,33). Lateral pictures are especially important because of the frequent anterior location of the esophageal web (Fig. 1E–G). In severe cases, it can form an annular stricture. The barium swallow, however, could be normal (17).

Webs are also detectable by upper endoscopy. They appear smooth, thin, gray, with a centric or lateral lumen at the anterior esophageal wall. The endoscopic examination needs to be performed carefully because the web can be missed or accidentally torn by the endoscope, especially after a blind crossing of the upper esophageal sphincter. The endoscopy is necessary to perform biopsies, searching for mucosal changes or dysplasia in adults. In children, esophageal biopsies are important to eliminate an eosinophilic esophagitis.

The mainstay of PVS treatment is iron supplementation. In many adult cases, it is sufficient to reduce the dysphagia (21,22,34). Relapses of dysphagia were reported when iron deficiency recurred (35,36). In the case of long-standing dysphagia, refractory iron deficiency, or stricturing webs, several authors have proposed a rupture or mechanical dilation by bougienage (37,38) or a hydrostatic balloon (39). Argon plasma coagulation therapy of esophageal webs has also been successfully used (40). Surgical disruption or excision of the web was rarely necessary (41).

In the pediatric population, 93% of PVS patients needed an upper endoscopy and 64% of PVS patients needed an endoscopic dilation suggesting that iron supplementation has a lower efficiency than in adults. In most patients, a single dilation procedure resolved the symptoms. As in adults (35), multiple dilations were rarely necessary in children with a single case in this review.

PVS is associated with an increased risk of squamous cell carcinoma of pharynx and proximal esophagus (32,36,42–44). In adults, 3% to 15% of the patients developed a pharyngeal or esophageal cancer, mostly in women between 15 and 50 years of age (43,44). The physiopathology of this association is not exactly known; premalignant mucosal changes resulting from iron deficiency are involved (33). In Sweden, the previous high incidence of upper digestive tract cancer in women was correlated to the high prevalence of PVS. More recently, the decreasing of hypopharyngeal cancer was linked to the reducing incidence of PVS (34). Although there is actually no guideline for cancer follow-up in patients with PVS, some authors propose an annual or biannual routine endoscopic surveillance in adults (33,34,45). Noteworthy, no studies of the risk of developing an esophageal cancer after pediatric PVS has been carried out, but 1 patient experiencing dysphagia since childhood developed a squamous cell carcinoma of the pharynx 10 years later (18).

In conclusion, the diagnosis of PVS is exceptional in children, but it should be raised in all children with dysphagia associated with iron-deficiency anemia. The barium swallow with lateral incidences is important to visualize the web of the anterior proximal esophagus. Contrary to adults, iron supplementation is rarely sufficient in children, and an endoscopic dilation is often necessary. Screening for esophageal cancer should be considered in adults after several years of evolution.


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dilation; dysphagia; endoscopy; iron deficiency; Kelly-Paterson syndrome; Plummer-Vinson syndrome

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