Abstract
Dysphagia is defined as having difficulty in swallowing which may affect any part of the swallowing pathway from the mouth to stomach. Oesophageal webs are rare cause of dysphagia. It is found to occur in association with iron deficiency anaemia and dysphagia. Classical features of Plummer Vinson Syndrome include dysphagia, iron deficiency anaemia, oesophageal webs, koilonychia, angular stomatitis. Predominantly affects females past 40 years. Barium swallow and esophagoscopy shows web in the post cricoid region. About 10% of cases with this syndrome will develop post cricoid carcinoma. Treatment is to correct anaemia by oral and parenteral iron. To estimate the occurrence of anaemia and cricoid webs in patients with dysphagia attending ENT OPD in a tertiary care centre. It is a six-month observational study with 186 patients. Patients with dysphagia was further evaluated for anaemia and cricoid webs. A detailed clinical history along with blood investigations and barium swallow was done. In our study out of 186 patients with dysphagia 176 have decreased haemoglobin with decrease in serum iron and serum ferritin levels. Out of 176 only 6 patients have cricoid webs and it is commonly seen in females above 35 years. Plummer–Vinson syndrome is predominantly seen in middle aged women and present with symptoms of iron deficiency anaemia and dysphagia. Iron supplementation alone is helpful in majority of patients. PVS is a precancerous condition with high malignant potential, hence early diagnosis and regular follow up is necessary.
Supplementary Information
The online version contains supplementary material available at 10.1007/s12070-025-05363-1.
Keywords: Plummer vinson syndrome (PVS), Iron deficiency anaemia (IDA), Barium swallow (BS), Oesophageal web (OW), Haemoglobin (HB)
Introduction
Plummer-Vinson syndrome is defined by the classic triad of dysphagia, iron-deficiency anaemia and oesophageal webs [1]. Even though the syndrome is very rare nowadays, its recognition is important because it identifies a group of patients at increased risk of squamous cell carcinoma of the pharynx and the oesophagus [2].
The most used name is Plummer-Vinson syndrome, named after Henry Stanley Plummer (1874–1936) and Porter Paisley Vinson (1890–1959) who were physicians on the staff of the Mayo Clinic [3]. In 1912, Plummer reported a series of patients with long-standing iron deficiency anaemia, dysphagia and spasm of the upper oesophagus without anatomic stenosis, which was described as hysterical. In 1919 Vinson reported another case of ‘angulation’ of the oesophagus and attributed the first description of this entity to the earlier report of Plummer [4].
The clinical features of PVS include dysphagia which is initially to solids and then to liquids, lump in the throat and painful mouth ulcers [5]. On general examination there was pallor, koilonychia and splenomegaly present. Oral cavity examination showed angular stomatitis, glossitis, mouth ulcers. Then examination of hypopharynx and larynx showed pooling of saliva in pyriform fossa in some patients.
Review of Literature
| S.No | Authors | Place | Population | Results |
|---|---|---|---|---|
| 1. | Mallikarjun Patil, Renuka Malipatel, Harshad Devarbhavi | Bangalore | All adults with dysphagia, anemia and postcricoid web or those with iron deficiency anemia and post cricoids web. | Overall, 153 patients exhibited esophageal web, of which 132 (86.27%) patients had concomitant PVS and 21 (13.7%) patients did not. The mean age was 43.50 years (range 16–76) and 113 (85.6%) were women. Single session of Savary–Gilliard bougie dilation was successful in 90.7% of patients in relieving dysphagia and 9.3% developed recurrence, requiring repeated dilations. Four patients had concomitant squamous cell carcinoma of esophagus along with PVS and two developed upper gastrointestinal malignancy during follow-up [6]. |
| 2. | Vikas Sinha, Bela Prajapati, Ajay George, Devang Gupta. | Ahamadabad | 25 confirmed cases of Plummer-Vinson syndrome | PV syndrome has the highest incidence in females and in the fifth decade of life. Grade I dysphagia with soreness of mouth is the commonest presenting complaint. Major clinical signs of iron deficiency anemia like pale bald tongue, angular chelitis, pallor etc. are found in almost all cases [1]. |
| 3. | Saqib Munir, *Danyal Rashid, Aurangzeb | Rawalpindi | 54 cases of dysphagia 32 male and 22 female, from outpatient department of CMH Rawalpindi. | 31.45% were cases of foreign body esophagus, 16.66% were case of Carcinoma esophagus, 9.25% were of leiomyoma esophagus, 11.11% were of Carcinoma pyriform fossa hypopharynx, 7.40% were of peptic stricture esophagus, 3.7% were of globus pharyngeus, 3.7% were of Plummer Vinsons disease, 1.85% of Zenker’s Diverticulum. All the esophageal pathologies have equal sex involvement. 14.81% were of post cricoid carcinoma female predominance [7]. |
| 4. | A. Goel,1 C. P. Lakshmi,1 S. S. Bakshi,2 N. Soni,3 S. Koshy1 | Puducherry | Dysphagia which was found to be due to esophageal web | PVS is commonly seen in the southern part of India. Web in PVS can safely be dilated with a single session of CRE balloon dilatation. In majority, balloon dilatation effectively alleviates dysphagia and response is sustained during intermediate duration of follow up. |
Materials and methods
Transverse lucent thin membranous band arsing from anterior wall of Pharynx at C4 – C5 space
Hypopharyngeal web causing luminal narrowing and proximal holdup of barium at the level of C4 cervical vertebra
Over a period of 6 months, all the patients with dysphagia attending the department of Otorhinolaryngology were examined and investigated for the presence of anaemia. Informed and written consent was obtained from all the patients after discussing the procedure in detail and Ethical committee approval was also obtained.
Diagnostic Criteria
Patients with dysphagia for more than 2 weeks, Age > 18 years, both females and males were included in this study, with Serum iron < 37 micrograms/dl and serum ferritin < 9.3 mg/dl and presence of cricoid webs in barium swallow. In this study, peripheral smear was not included and diagnostic criteria for IDA in this has been explained above.
Patients with age < 18 years, pregnant females and dysphagia due to known neurogenic causes, malignancy of oropharynx, hypopharynx, oesophagus and foreign body were excluded from this study. In this study, patients were enrolled based on this inclusion and exclusion criteria. Then a detailed history with clinical examination, and blood investigations was done to rule out anaemia. Serum iron and serum ferritin levels were also measured. Barium swallow was also done to rule out post cricoid webs.
Results
Among 186 patients, 183 were females and 3 were males. The gender distribution in study population was given in (Table 1), with increased female preponderance. The mean age was 45.32 years with standard deviation 7.24. The age distribution in study population is given in (Table 2). Of 186 patients with dysphagia 176 have decreased haemoglobin with decrease in serum iron and serum ferritin levels. Out of 176 only 6 patients have cricoid webs and it is commonly seen in females above 35 years and it is given in (Table 3). The comparison with HB versus cricoid web positive was given in Table 4 with a p value of < 0.0040*. The comparison with haemoglobin versus serum iron and serum ferritin was given in Tables 5 and 6 with p value of 0.3302 and 0.3928 respectively. Comparison of HB with Cricoid webs show a p value of < 0.0040. There is a Significant positive correlation between SR Ferritin and HB (r -value = 6742) P-value < 0.001* and it is given in Fig. 1. There is a Significant Positive correlation between SR Iron and HB. (r -value = 0.5527) P-value < 0.001* which is given Fig. 2. Our study demonstrates that (3.22%) of post-cricoid webs are associated with PVS. Barium swallow was done which showed cricoid webs in 6 patients. In our study 98.39% of patients were females which is similar to other studies. In our study majority had grade 1 and grade 2 dysphagia which responded well to iron supplementation. Treatment protocol for PVS includes Iron supplementation which can resolve dysphagia in many of the patients, upper endoscopy can widen the web in the oesophagus to allow normal swallowing and this can be done with bougie, ballon and endoscopes. Regular follow up is necessary as there is increased risk of squamous cell carcinoma of pharynx and oesophagus.
Table 1.
Descriptive analysis of gender in study population (N=186)
| Sex | Frequency | Percentage |
|---|---|---|
| Male | 3 | 1.61% |
| Female | 183 | 98.39% |
| Total | 186 | 100 |
Table 2.
Descriptive analysis of age distribution in study population (N=186)
| Age distribution | Frequency | Percentage |
|---|---|---|
| 26 to 34 | 4 | 2.15 |
| 35 to 44 | 88 | 47.31 |
| 45 to 54 | 76 | 40.86 |
| 55 to 64 | 18 | 9.68 |
| Total | 186 | 100 |
| Mean(SD) | 45.32 (7.24) | |
| Median | 45 | |
| Range | 26 to 64 | |
Table 3.
Comparison with age vs cricoid web positive in study population (N = 186)
| Age | Cricoid Web+ | P-Value | ||
|---|---|---|---|---|
| No | Yes | Total | ||
| 26 to 34 | 4 | 0 | 4 | 0.833 |
| 2.22 | 0 | 2.15 | ||
| 35 to 44 | 85 | 3 | 88 | |
| 47.22 | 50 | 47.31 | ||
| 45 to 54 | 73 | 3 | 76 | |
| 40.56 | 50 | 40.86 | ||
| 55 to 64 | 18 | 0 | 18 | |
| 10 | 0 | 9.68 | ||
| Total | 180 | 6 | 186 | |
| 100 | 100 | 100 | ||
Table 4.
Comparison with HB vs cricoid web positive in study population (N = 186)
| Web | Mean(SD) | Range | P-Value |
|---|---|---|---|
| Yes | 5.46(0.59) | 4.8 to 6.3 | < 0.0040* |
| No | 7.61(1.79) | 4.4 to 14 |
Table 5.
Comparison with SR iron vs cricoid web positive in study population (N = 186)
| Web | Mean(SD) | Range | P-Value |
|---|---|---|---|
| Yes | 17(4.90) | 11.1 to 22 | 0.3302 |
| No | 21.08(10.18) | 5.9 to 58 |
Table 6.
Comparison with SR ferritin vs cricoid web positive in study population (N = 186)
| Web | Mean(SD) | Range | P-Value |
|---|---|---|---|
| Yes | 6.41(1.77) | 4 to 9 | 0.3928 |
| No | 8.43(5.74) | 3.14 to 39 |
Fig. 1.
Scatter plot for SR ferritin and HB in study population (N = 186). There is a Significant positive correlation between SR Ferrtin and HB.(r -value = 6742) P-value < 0.001*
Fig. 2.
Scatter plot for SR Iron and HB in study population (N = 186). There is a Significant Positive correlation between SR Iron and HB. (r -value = 0.5527) P-value < 0.001*
Discussion
Plummer-Vinson syndrome (PVS) is a rare condition characterized by the classic triad of dysphagia, iron-deficiency anaemia, and oesophageal webbing [8, 9]. Plummer-Vinson syndrome is more common in middle-aged women and is associated with an increased risk of developing squamous cell carcinoma of the pharynx and proximal oesophagus. It is known as Paterson-Brown-Kelly syndrome. This name was given after two British laryngologists, Donald Ross Paterson (1863–1939) and Adam Brown-Kelly (1865–1941), who published their findings in 1919 [10, 11]. This syndrome was named Plummer-Vinson syndrome after Henry Stanley Plummer (1874–1936) and Porter Paisley Vinson (1890–1959), who noted cases of iron deficiency and dysphagia in the presence of suspected spasm of the upper oesophagus or abnormal angulation of the oesophagus [12] Plummer Vinson syndrome can occur in people with chronic iron deficiency anaemia, though it is rare, but there is still a possibility that long standing cases of anaemia may develop cricoid webs [13, 14].
Etiopathogenesis
Most commonly iron deficiency anaemia causing mucositis which further develops into webs, Vitamin B deficiency, autoimmune conditions like rheumatoid arthritis, pernicious anaemia and celiac disease [15, 16]. It is characterized by thinning of mucosa of upper digestive tract including the pharynx and oesophagus and is associated with superficial pharyngeal oesophagitis. Disappearance of rete pegs from mucosa as a result of thinning [17, 18]. Submucosal fibrosis and atrophy of mucosa. Narrowing and web formation at the region of cricopharynx [19]. Most patients with PVS were initially asymptomatic and later presents with the triad of dysphagia, iron deficiency anaemia and oesophageal webs.
Dysphagia in PVS is divided into two groups, grade I (occasional dysphagia on taking solids) or grade II (able to swallow only semi-solid diet) and other clinical findings can be glossitis and angular cheilitis. Complete blood count, peripheral smear and iron studies like serum iron, ferritin, should be done to confirm the diagnosis of iron deficiency anaemia [20]. Radiographical investigation like Barium swallow should be carried out to rule out oesophageal webs.
Treatment of PVS includes iron supplementation to correct anaemia. Most of the patient respond well to iron supplementation alone. Patients with dysphagia caused by more advanced disease may require endoscopic dilation of web [21].
Based on the review of literature
| Author | Sample Size | Predominant Gender | Incidence Of Web | Dilation Performed or Medical Management | Success Rate |
|---|---|---|---|---|---|
| Mallikarjun Patil, Renuka Malipatel, Harshad Devarbhavi | 153 | women | 86.27% (132 patients) | dilation performed with savary gilliard bougie | 90.7% patients relieved of dysphagia. |
| Vikas Sinha, Bela Prajapati, Ajay George, Devang Gupta | 25 | women | 15 cases | Iron and B complex supplementation alone given. Dilation not performed. | Symptoms improved 15 cases was under follow up. |
| Saqib Munir, *Danyal Rashid, Aurangzeb | 54 | women | 3.7% (2 cases) | dilation not performed | was under regular follow up. |
Conclusion
Even though PVS is rare, it is still seen in middle aged females with low socio-economic status. Most of the patients responded well to iron supplementation alone.
For patients with Plummer Vinson Syndrome, iron supplementation alone can improve swallowing problems. In our institution, after confirming iron deficiency anaemia oral or parenteral iron will be given at long term basis to maintain normal iron levels, followed by mechanical dilatation of webs if symptoms persists. The prognosis was good. Most of the patients were free of dysphagia during follow up visits. This study clearly explains the need for regular follow up and endoscopic evaluation to rule out malignancies.
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Declarations
Ethical approval
The authors declare that the procedures to this work acted in accordance with the ethical standards. Patients included in this study gave their informed consent for the study. This study comply with ethical standards.
Conflict of interest
No conflict of interest.
Footnotes
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