Paterson Brown-Kelly Syndrome

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

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.

It tends to be a disease of women in which iron deficiency leads to a post-cricoid web and possible progression to post-cricoid carcinoma.¹

The syndrome most often affects middle-aged women, although it can occasionally present in a child. It is an uncommon condition and much of the literature is case reports of one or a few individuals rather than large series. There is a marked female preponderance.² 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 Plummer-Vinson syndrome and post-cricoid carcinoma.¹

Risk factors

• Iron deficiency - this is related to poor diet or blood loss.²
• Oesophageal webs - these are usually about 2 or 3 mm wide. The web is a smooth extension of normal oesophageal tissue containing just mucosa and submucosa. It can occur anywhere along the oesophagus. In Plummer-Vinson syndrome it classically occurs at the anterior post-cricoid area of the upper oesophagus .⁴ Webs are more frequent in women and this may be related to propensity for iron deficiency. Most upper oesophageal webs are not associated with Plummer-Vinson syndrome.^4,5

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.

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.

• 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.^4,7
• Biopsy may be required if malignancy is suspected clinically.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

Post-cricoid carcinoma or carcinoma of oesophagus may develop.²

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

• 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 who was born in Inverness in 1863 and died in 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 was 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.