What is Plummer Vinson Syndrome?
This is a syndrome occurring with chronic, long term “Iron deficiency anemia”. Individuals with this syndrome have problems swallowing because of esophageal webs – thin, small tissue growths that block partially the esophagus. It is defined in the literature as having a triad of “iron deficiency anemia”, esophageal webs, and dysphagia. Currently this syndrome is extremely rare but it is significant to be able to recognize it as it isolates a patient group that has an enlarged risk of “squamous cell carcinoma” of the esophagus and pharynx.
It is also referred to as PVS.
Plummer Vinson Syndrome Symptoms
The list of symptoms and signs mentioned in various literature for PVS include the below listed symptoms:
- Pain in the throat during swallowing
- Sensation of stuck food in larynx
- Sensation of burning when swallowing
- Inside of mouth is pale
- Hypochromic anemia
- Difficulty swallowing
- Sideropenic anemia
- Mucosal webs in esophagus
- Smooth tongue
- Painful tongue
- Red tongue
Plummer Vinson Syndrome Causes
The cause of PVS is unknown. Genetic factors as well as a lack of some nutrients – nutritional deficiencies – might play a role. This is a rare disorder that may be linked to cancers of the throat and esophagus. PVS is most common in women especially those in middle age. The peak age is believed to be over 50 years old.
Plummer Vinson Syndrome Diagnosis
The diagnosis of PVS is made on the confirmation of” iron-deficiency” anemia as well as one (1) or even more esophageal webs in a patient with postcricoid difficulty in swallowing. Webs in the esophagus are identified by barium X-ray but the preeminent way for finding them is the videofluoroscopy. These webs are also demonstrable by upper GI endoscopy. These webs appear thin, smooth and gray with central or eccentric lumen. They typically occur in the proximal portion of the esophagus and often can be missed or ruptured accidentally unless the endoscope is presented under direct visualization. These esophageal webs, which also can happen in the absence of anemia and PVS, are categorized by one or more thin horizontal membranes that consist of squamour epithelium and submucosa and they usually stick out from the anterior wall, spreading laterally but not to the posterior wall, which means they rarely surround the lumen.
Lab test normally expose “iron-deficiency” anemia with values of hemoglobin, hematocrit, serum iron and ferritin decreased and increased total of iron binding capacity. Other lab abnormalities are normally not described.
As dysphagia is a major symptom of PVS, the differential diagnosis comprises all other reasons of dysphagia basically malignant tumors, esophageal rings, or benign strictures. Other reasons for dysphagia are diverticulat, motility disorders such as achalasia, scleroderma, disorders of spastic motility, diabetes mellitus, GI reflux disease and skeletal muscle and neuromuscular conditions.
Plummer Vinson Syndrome Treatment
The first step in the management of PVS is to make clear the cause for the iron deficiency so as to exclude any active bleeding, malignancy or celiac disease.
PVS can easily be treated and effectively with iron supplements and mechanical dilation. Iron supplements unaccompanied can take care of dysphagia in many patients. But, in cases of substantial obstruction of the esophageal lumen by esophageal web and continued dysphagia in spite of iron supplements, rupture and dilator of the web needs to be done. After employment of a guide-wire, dilators with a diameter of up to 17 mm should be used. Normally only a single dilation is sufficient to get rid of dysphagia, but in some cases multiple sessions might be needed. Furthermore successful balloon dilation has been defined in the literature. Since the PVS is linked to enlarged risk of squamous cell carcinoma of the esophagus and pharynx, the patients need to be closely followed. An exploration of upper GI endoscopy is recommended every year, even if the usefulness of this endorsement has not been clearly confirmed.