|Year : 2018 | Volume
| Issue : 1 | Page : 30-32
P Gopalakrishna1, M Rajendra Prasad1, PT Joyes2, MS Sridhar1
1 Department of General Medicine, Meenakshi Medical College, Hospital and Research Institute, Kanchipuram, Tamil Nadu, India
2 Department of Medical Gastroenterology, Meenakshi Medical College, Hospital and Research Institute, Kanchipuram, Tamil Nadu, India
|Date of Web Publication||8-Jan-2019|
M S Sridhar
Department of General Medicine, Meenakshi Medical College, Hospital and Research Institute, Enathur, Kanchipuram, Tamil Nadu
Source of Support: None, Conflict of Interest: None
The clinical findings of a 36-year-old female who presented to Medicine out-patient service with the triad of dysphagia, iron deficiency anaemia, splenomegaly and oesophageal web are reported. Clinical pathology of anaemia is diagnostic of iron deficiency anaemia. Dysphagia was treated with endoscopic dilation of the web and was prescribed iron therapy. This relatively rare condition is reported as the physician should be alert to its possibility because of its association with increased risk of hypopharyngeal carcinoma.
Keywords: Dysphagia, iron-deficiency anaemia, oesophageal web
|How to cite this article:|
Gopalakrishna P, Prasad M R, Joyes P T, Sridhar M S. Plummer–Vinson syndrome. J Clin Sci Res 2018;7:30-2
| Introduction|| |
Plummer–Vinson syndrome or Paterson–Kelly syndrome which is a well-known clinical entity was not considered a strictly defined condition as late as in 1975. This condition was defined as simple, painless, permanent stricture in the cervical oesophagus, not associated with inflammation, swelling or ulceration, which causes obstructive dysphagia. The classic triad of dysphagia, iron-deficiency anaemia and oesophageal webs describes Plummer–Vinson syndrome. Although it is a relatively rare condition, the physician should be alert to its possibility because of its association with increased risk of hypopharyngeal carcinoma.
| Case Report|| |
A 35-year-old female presented herself with gradual onset of difficulty in swallowing, mainly for solids, of 6-month duration. She complained of generalised weakness and tiredness of 2-month duration. There was no history of pain during swallowing, weight loss or abdominal pain. She attained menarche at 13 years of age and has regular menstrual cycle of 30-day duration with flow for 3 days.
The patient was moderately built and nourished, and the patient's height was 1.55 m, weight was 44 kg and body mass index was 18.31 kg/m. There was pallor of palpebral conjunctivae and nail bed. Koilonychias was absent; there was no icterus, cyanosis, lymphadenopathy or pedal oedema. Her pulse was 84/min, blood pressure was 120/70 mmHg, respiratory rate was 16/min and oral temperature was 36.9°C. Systemic examination was normal except for the fact that the spleen was palpable.
Her laboratory results were as follows: haemoglobin 6.8 g/dL, packed cell volume 23%, red blood cells 2.42 million/mm, mean corpuscular volume 51fl, mean corpuscular volume 15 pg, mean corpuscular haemoglobin concentration 31%, total leucocyte count 5600/mm, differential count showed neutrophils 65%, lymphocytes 32%, eosinophils 1%, monocytes 2%, platelets 283,000/mm3, erythrocyte sedimentation rate 8 mm at the end of 1st h, random blood glucose 77 mg/dL and serum ferritin <1.0 ng/mL. Peripheral smear showed microcytic hypochromic anaemia.
Ultrasonography of the abdomen was normal, and splenic size was 10.8 cm and 11.5 cm on two different occasions.
She was suspected to be having oesophageal growth for which she underwent upper gastrointestinal random endoscopy which revealed a typical circumferential membranous cricopharyngeal web [Figure 1] which was treated by endoscopic dilatation [Figure 2]. She improved symptomatically. She was transfused one unit of packed red blood cells for anaemia correction. She was prescribed iron supplementation and discharged.
| Discussion|| |
Plummer–Vinson syndrome is easy to diagnose because of easy availability of endoscopy even in smaller centres. The dysphagia and iron-deficiency anaemia can be identified without any difficulty, and the web is detected by endoscopy. The oesophageal web is usually formed during long-standing iron deficiency, and since iron-deficiency anaemia is common among women, this syndrome is said to be much more common in women. Onset is said to be very gradual. There is danger of mistaking it for 'globus hystericus'. The incidence and prevalence of the condition are largely unknown due to uncertain level of ascertainment.
A study by Lundholm was quoted by Larson-Gunnar et al., wherein 19% of persons with primary hypochromic anaemia were found to be having Plummer–Vinson syndrome. Among 353 patients with iron-deficiency anaemia, this condition was found in 1.7%. Based on endoscopy studies on 15,000 participants, the relative frequency of this condition was 1/1000; cervical oesophageal mucous diaphragms were found in 25/100,000 and women-to-men ratio was 3:1. Among those who had webs in hypopharynx and oesophagus, there was a higher number of persons with iron-deficiency anaemia. A review of patients with Plummer–Vinson syndrome in English literature was quoted to highlight female predominance and the mean haemoglobin was 8.2 g/dL. As a part of clinical picture, enlargement of spleen and thyroid has been described. In the patient described in this report, the spleen was clinically palpable; however, by ultrasonography, it measured 10.8 cm and 11.5 cm on two different occasions, and the haemoglobin level was 6.8 g/L. While correction of iron deficiency did not change the structure, there is an opinion that correction of anaemia with iron supplementation is, in fact, beneficial., Recommended treatment was surgery or by dilatation, and early recurrence was expected. At present, iron supplementation coupled with mechanical dilatation appears to be the definitive treatment for the condition. In addition, endoscopic surveillance is suggested for detecting recurrence as well as post-cricoid carcinoma at an early stage.
A high degree of suspicion for Plummer–Vinson syndrome in all patients with iron-deficiency anaemia with dysphagia would allow recognition of the rare disorder and enable endoscopic surveillance which is recommended for early detection and treatment of carcinoma hypopharynx. The correction of anaemia by iron supplementation and health education would be beneficial.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
We sincerely thank Professor and Head, Department of Medical Gastroenterology, Professor and Head, Department of Medicine and the Dean, Meenakshi Medical College, Hospital and Research Institute, Enathur, Kanchipuram, for their help and permission to publish this case.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]