​PATTERSON BROWN-KELLY SYNDROME: OESOPHAGEAL DILATATION​

by Monzur Ahmed

A 25 year old female gave a 6 months history of high dysphagia and choking. The symptoms were worse with solids. She had lost one stone in weight. 3 weeks ago, an urgent (2 week wait) OGD was performed by one of our Nurse Endoscopists. This showed an impassable high oesophageal stricture (not biopsied). A subsequent barium swallow showed a short narrowing at the junction of the hypopharynx and oesophagus. Routine blood tests revealed iron deficiency anaemia (Hb 8.0 g/d, MCV 66). On direct questioning, the patient admitted to longstanding menorrhagia. There was no PR bleeding or haematuria and she was not a vegetarian. She was referred for a further OGD with a view to dilatation (see video…) ​

In 1906 D. R. Paterson described, in The British Medical Journal, the condition of inflammation, accompanied by spasm and stenosis, of the lower pharynx [1]. Paterson pointed out that this may be a cause for dysphagia, and in 1919, discussing dysphagia in women, he noted that cheilosis, glossitis, pharyngitis, and postcricoid carcinoma could be a sequel [2]. Anaemia was not mentioned in either of Paterson's papers, but, also in 1919, A. Brown-Kelly added this feature to the account given by Paterson [3], and thus the British eponym Paterson Brown-Kelly (PB-K) syndrome was introduced. In the United States, Mayo clinic physician, H. S. Plummer described (though not published) a series of patients with long-standing iron deficiency anaemia, whom he said tended to develop hysterical dysphagia! It was not until 1922 that P. P. Vinson, his pupil also at the Mayo clinic, published a full account which emphasised that the spasm was secondary to anaemia [4]. Neither Plummer nor Vinson mentioned postcricoid carcinoma as a complication. Should the condition be called Patterson Brown-Kelly (PB-K) syndrome or Plummer Vinson (PV) syndrome? In an assessment of the merits of the various contenders for priority, F. C. Ormerod considers that Brown-Kelly and Paterson have the edge on their rivals [5]. J. Waldenstrom emphasized that a low level of iron in the plasma (sideropenia) rather than anaemia was a basic feature of the syndrome [6]. He described a woman with postcricoid dysphagia, but who was not anaemic, whose dysphagia was cured by iron. Other manifestations of iron deficiency, such as soreness of the tongue and fissures at the corners of the mouth, which were present in this patient, were also cured by the iron therapy. Waldenstrom suggested that the formation of a postcricoid "web" (which is shown by a barium swallow) and the dysphagia were due to iron deficiency, and subsequently this association has been recognized frequently. Nowadays, PB-K syndrome is said to be characterized by the classic triad of dysphagia, iron-deficiency anemia and esophageal web. However, even after a century since the first case reports, the aetiology of PB-K syndrome remains largely unknown. Although genetic predispositions and several other mechanism have been postulated, the evidence remains weak although iron deficiency appears to consistently play an important role. This is partly due to studies which have reported an improvement in dysphagia with iron supplementation whereas iron deficiency is suspected to cause mucositis leading to web formation. As patients with PB-K syndrome may also suffer from malnutrition, deficiency of vitamin B has also been suggested as a cause although the evidence is weak and inconclusive. Other disorders reported to be associated with PB-K syndrome include celiac disease, Crohn's disease, rheumatoid arthritis and thyroid disease raising the possibility immune dysregulation may be involved in its pathogenesis although this remains to be proven [7-9]. Patients with PB-K syndrome have an excellent outcome with most symptomatic patients requiring only one OGD with dilatation for complete relief of symptoms in conjunction with iron replacement therapy. Patients are at an increased risk of developing squamous cell carcinoma of hypopharynx or upper oesophagus which may be related to chronic iron deficiency. This is believed to to cause irreversible mucosal changes leading to malignant degeneration [10].

REFERENCES
1. Paterson, D. R., Brit. Med. J., 1906, 2: 353.
2. Paterson D.R., J. Laryng., 1919, 34: 289.
3. Kelly, A. Brown, J. Laryng., 1919, 34: 285.
4.Vinson, P. P., Minn. Med., 1922, 5: 107.
5. Ormerod, F. C., J. Laryng., 1966, 80: 894.
6. Waldenstrom, J., Acta med. scand., Supplement, 1938, 90: 380.
7. Hefaiedh R, Boutreaa Y, Ouakaa-Kchaou A et al. Plummer Vinson syndrome association with coeliac disease. Arab J Gastroenterol. 2013;14(4):183-5.
8. Medrano M. Dysphagia in a patient with rheumatoid arthritis and iron deficiency anemia. MedGenMed. 2002; 28;4(3):10.
9.Park JM, Kim KO, Park CS, Jang BI. A case of plummer-vinson syndrome associated with Crohn's disease. Korean J Gastroenterol. 2014; 63(4):244-7.
​ 10. Watts JM. The importance of the Plummer-Vinson syndrome in the aetiology of carcinoma of the upper gastrointestinal tract. Postgrad Med J. 196;37:523-33.