Synonyms: Plummer-Vinson syndrome, PVS, Paterson-Brown Kelly syndrome, Waldenström-Kjellberg syndrome, sideropenic dysphagia, sideropenic nasopharyngopathy.

Introduction

This is a condition where iron deficiency is associated with a post-cricoid oesophageal web. A description of this syndrome was published by Henry Plummer in Chicago in 1911 and also by Porter Vinson in Philadelphia in 1919. Donald Ross Paterson and Adam Brown-Kelly published independently of each other in London in 1919. Hence British doctors call it the Paterson Brown-Kelly syndrome, although increasingly known as the Plummer-Vinson syndrome on both sides of the Atlantic.

Epidemiology

The syndrome most often affects middle-aged women (this may be related to a propensity for iron deficiency), although it can occasionally present in a child.¹ The web (containing mucosa and submucosa) occurs at the anterior post-cricoid area of the upper oesophagus.² It is an uncommon condition and much of the literature is case reports of one or a few individuals rather than large series. Figures for incidence and prevalence are not available but it is becoming rarer, probably because dietary inadequacy is becoming less common.³ In northern Sweden, where the risk in women has been traditionally very high, correction of deficiency has had a beneficial effect on both Paterson Brown-Kelly syndrome and risk of post-cricoid carcinoma.⁴ Most upper oesophageal webs are not associated iron deficiency, i.e. with Plummer-Vinson syndrome.^2,5

Risk factors

• Iron deficiency - this is related to poor diet or blood loss.¹

History¹

The presentation is usually with painless, intermittent dysphagia. It tends to be with solid foods but, if untreated, may progress to soft foods and even liquids. As with other causes of oesophageal strictures, webs and rings, the patient is able to give a good indication of the level of obstruction. In this condition the patient indicates the upper oesophagus. Dysphagia may lead to weight loss. This should be regarded as a sign of danger.⁶

There may also be features of iron deficiency anaemia like lethargy, tiredness and shortness of breath on exertion.

Examination¹

The features that appear on examination are those associated with iron deficiency. There may be pallor and even tachycardia if anaemia is marked. There may be koilonychia (spoon shaped nails), angular cheilitis and glossitis.

Investigation

• Full blood count will show a microcytic, hypochromic anaemia. Ferritin is low.
• Barium swallow may show the web. This may need to be enhanced with videofluoroscopy.^2,7
• Biopsy may be required if malignancy is suspected clinically.⁷

Treatment^1,7

• Iron replacement can almost invariably be achieved be oral means. Adding vitamin C does not improve absorption significantly.⁸ There is rarely any need for parenteral iron. Supplements may be needed long-term because after correction it is important to maintain a normal iron status. Causes of blood loss like menorrhagia may require attention.
• Dilation using an endoscope, a dilating instrument (e.g. Savary dilator), or balloon dilatation is occasionally required in cases of persistent dysphagia.^9,10

Complications

Post-cricoid carcinoma or carcinoma of the oesophagus may develop.¹

Prognosis and prevention

Correction of iron deficiency will correct the condition if malignancy has not yet occurred. Prevention of iron deficiency prevents the disease.⁷

Historical notes

• Henry Plummer was an American internist - born 1874 and died 1937. He was a professor at the Medical School of Minnesota. He published: Diffuse dilatation of the esophagus without anatomic stenosis (cardiospasm). A report of ninety-one cases. Journal of the American Medical Association, Chicago, 1912, 58: 2013-2015.
• Porter Paisley Vinson was an American surgeon at the Mayo Clinic - born 1890 and died 1959. He published: A case of cardiospasm with dilatation and angulation of the esophagus. Medical Clinics of North America, Philadelphia, PA., 1919, 3: 623-627.
• Donald Ross Paterson was an otolaryngologist - born in Inverness 1863 and died 1939. He worked in Cardiff and published: A clinical type of dysphagia. The Journal of Laryngology, Rhinology and Otology, London, 1919, 24: 289-291.
• Adam Brown-Kelly - born 1865 and died 1941. He graduated from Glasgow but worked as an ENT surgeon in London, Berlin and Vienna. He published: Spasm at the entrance of the esophagus. The Journal of Laryngology, Rhinology and Otology, London, 1919, 34: 285-289.

Document references

1. Novacek G; Plummer-Vinson syndrome. Orphanet J Rare Dis. 2006 Sep 15;1:36. [abstract]
2. Zervos X et al; Esophageal Webs and Rings, eMedicine, Nov 2008
3. Chen TS, Chen PS; Rise and fall of the Plummer-Vinson syndrome. J Gastroenterol Hepatol. 1994 Nov-Dec;9(6):654-8. [abstract]
4. Larsson LG, Sandstrom A, Westling P; Relationship of Plummer-Vinson disease to cancer of the upper alimentary tract in Sweden. Cancer Res. 1975 Nov;35(11 Pt. 2):3308-16. [abstract]
5. Nosher JL, Campbel WL, Seaman WB; The clinical significance of cervical esophageal and hypopharyngeal webs. Radiology. 1975 Oct;117(1):45-7. [abstract]
6. Jones R, Latinovic R, Charlton J, et al; Alarm symptoms in early diagnosis of cancer in primary care: cohort study using General Practice Research Database. BMJ. 2007 May 19;334(7602):1040. Epub 2007 May 10. [abstract]
7. Wong Kee Song L; Plummer Vinson Syndrome; eMedicine.(2008).
8. Teucher B, Olivares M, Cori H; Enhancers of iron absorption: ascorbic acid and other organic acids. Int J Vitam Nutr Res. 2004 Nov;74(6):403-19. [abstract]
9. Demirci F, Savas MC, Kepkep N, et al; Plummer-Vinson syndrome and dilation therapy: a report of two cases. Turk J Gastroenterol. 2005 Dec;16(4):224-7. [abstract]
10. Yasawy MI; Treatment of Plummer-Vinson syndrome with Savary-Gilliard dilatation. Saudi Med J. 2004 Apr;25(4):524-6. [abstract]

Internet and further reading

• Plummer-Vinson Syndrome; whonamedit.com 2007