Plummer Vinson Syndrome

What is plummer vinson syndrome?

Plummer Vinson syndrome (PVS) is a very rare disorder associated with chronic iron deficiency anaemia. In such cases patient complains for food deglutition problem which is due to narrowing of esophagus (esophagus web).

 

Oesophageal membranous structure is composed of a thin twisting of tissue; this forms a partial obstacle of the oesophageal lumen and obstructs the passage for food. The reported case has given evidences to prove that the middle aged females are mostly affected. The risk of developing this disorder is also present with children and teenagers. In current time, the number of incidence is very less, but if the symptomatic occurrence will arise then it is necessary to diagnose the proper reason behind this discomfort as these symptoms are similar with squamous cell malignancy of the esophagus and pharynx.

Symptoms of Plummer Vinson Syndrome

The following symptoms associated with plummer vinson syndrome

• Dysphegia (difficulty in swallowing)
• Pain inside the throat while swallowing of solid food
• choking sensation
• Burning sensation inside the throat during swallowing
• Weight loss consequential from an avoidance to eating habit
• Pale skin
• Lethargy
• Pallor inside the oral cavity
• swelling and blistering cracks at the angle of the mouth (Angular cheilitis)
• swelling of the tongue (Glossitis)
• Red, smooth appearance of tongue
• Abnormally thin edges of nails (Koilonychia/ spoon nails)

Causes of Plummer Vinson Syndrome

The exact cause of Plummer Vinson syndrome is yet to be discovered. The possible reasons behind the development of PVS are genetic anomaly and deficiency of nutritional supply. Celiac disease causes mal-absorption of nutrients from diet causing iron deficiency anaemia which results to PVS.  The other possible reason is progression of throat or oesophageal carcinoma. Other associated diseases are internal haemorrhage in case of gastric ulceration, heavy menstrual flow. Autoimmunity causes destruction of haemoglobin can be the cause for PVS.

Diagnosis of Plummer Vinson Syndrome

The measure of iron deficiency anaemia is the first approach to diagnose the syndrome. The laboratory tests help to identify the iron deficiency by estimating the following specifications and result often shows the reduction of the normal levels.

• Haemoglobin value,
• Hematocrit,
• Mean corpuscular volume,
• Serum iron volume and
• Ferritin assessment

Total iron binding capacity is increased which also found in blood test.

Deglutition difficulty is the key symptom of Plummer-Vinson syndrome, the examination is important as the symptoms are also common with other fatal carcinogenic disorders. Tumour growth often restricts the food passage. Other possible reasons for the development of dysphagia are the formation of oesophageal ring, motility dysfunction, gastro-oesophageal reflux abnormality, neurological or musculo-skeleton system disorders. It is important to diagnose the possible differential diagnosis approach to determine the exact reason of dysphegia.

Barium swallow X-ray is usually conducted for testing of oesophageal webs but for confirmatory test video fluoroscopy provides best result. Upper gastrointestinal endoscopy is another approach to diagnose the oesophageal blockade. The test result shows development of a thin, smooth central lumen formation in the oesophagus. There is no history of injury and webs produced at the proximal part of the oesophagus.

In case of cancerous development the webs are different from anaemia and Plummer-Vinson syndrome, and main features are development of number of squamous epithelium and sub-mucosa membrane lining formation.

Treatment of Plummer Vinson Syndrome

Evaluation of proper reason of discomfort is the foremost approach to treat Plummer Vinson syndrome. The symptoms of Plummer Vinson syndrome are similar with squamous cell carcinoma of the pharynx and the oesophagus, so close observation and proper diagnosis is very important.

Proper and efficient treatment of Plummer Vinson syndrome involves two steps as described below:

• Management of iron deficiency
• Correction of oesophageal webs

Management of Iron deficiency

This can be managed by providing iron supplement and dietary maintenance. In severe cases, blood transfusions are also recommended.

Correction of oesophageal webs

For correcting the oesophageal webs, the procedure of intervention is applied for bursting the webs and alteration of narrow food pipe diameter is useful to solve the dysphegia. For altering the diameter, dilators having 17 mm width is useful to correct the passage of food. Generally, single sitting for dilation is enough for correction but for some individual it may require multiple sessions. Balloon burst dilation is also another medical option to correct the problem.

The advanced medical science has invented ‘YAG laser therapy’ and it has been reported that this is a successful technique for rupturing an oesophageal web. This technique is not required in initial stage of Plummer-Vinson syndrome.

Another therapeutic alternative way to correct the diameter of oesophageal narrowing is needle-knife electro-incision.

Open Surgical procedure

Only in case of unmanageable webs which cannot be resolved by above mentioned intervention, then open surgical procedure is recommended, but this approach is rarely used.

References

1. http://www.nlm.nih.gov/medlineplus/ency/article/001158.htm
2. http://emedicine.medscape.com/article/187341-overview
3. http://www.ojrd.com/content/1/1/36#sec5
4. http://patient.info/doctor/paterson-brown-kelly-syndrome
5. http://www.rightdiagnosis.com/p/plummer_vinson_syndrome/symptoms.htm