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Dr. Andrew Rynne
MD
Dr. Andrew Rynne

Family Physician

Exp 50 years

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What Causes Difficulty In Swallowing?

I've been having problems swallowing. It usually happens several times a day. When it happens I start to panic a bit and I work hard to calm down and eventually I can swallow again. Sometimes it takes 15-20 seconds to swallow. I'm iron deficient and scheduled for an iron infusion on Wed. This scares me a bit and I don't scare easily. I've survived a LOT medically.
Thu, 6 Apr 2017
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Pathologist and Microbiologist 's  Response
Plummer-Vinson syndrome, also known as Paterson-Kelly syndrome, is a rare disorder that is characterized by dysphagia, upper esophageal webs, and iron deficiency anemia. The syndrome is most common among patients aged 40-70 years. Ninety percent of affected patients are female. Patients generally complain of long-standing, intermittent, painless dysphagia. Most commonly, patients first have dysphagia to solids, but, over time, symptoms can progress to involve dysphagia to liquids as well. Progression of the dysphagia can eventually lead to weight loss.
Patients with Plummer-Vinson syndrome are frequently thin and pale. Nail abnormalities are common and koilonychia, spooning of the nails, is typical. The nails are also frequently quite brittle. Other findings on physical examination include angular cheilitis, glossitis, splenomegaly, and edentia.
Because iron deficiency anemia is a part of the syndrome, laboratory findings consistent with iron deficiency anemia are necessary for the diagnosis. The latter includes microcytic anemia with a low iron level, high total iron-binding capacity, and a low ferritin level.
A barium swallow will normally reveal 1 or several esophageal webs. A barium study was not performed on our patient because of the definitive nature of the EGD. However, we have recently encountered another patient with Plummer-Vinson syndrome who had a typical web on barium swallow.

Upper esophageal webs are the classic endoscopic finding in Plummer-Vinson syndrome. These structures, which are smooth, thin, and gray in coloration, are found just below the cricopharyngeus muscle. The web usually attaches to the esophagus anteriorly, and the lumen is generally eccentric. Occasionally, these structural abnormalities can be missed if the endoscope is not passed under direct visualization. The webs frequently can be easily ruptured by the scope. The patient presented in this case did not have a typical web. However, the patient whose barium swallow is shown in Figure 5 had an endoscopy that demonstrated a very classic appearance of a delicate upper esophageal web.

Although esophageal webs are an essential characteristic of Plummer-Vinson syndrome, there have been case reports of patients with all of the classical features of this syndrome, who presented with cervical esophageal strictures instead of webs. In our patient, there was the suggestion that a web high in the esophagus was destroyed with the first passage of the scope. There was an unequivocal short stricture high in the esophagus that had to be dilated. It is possible that NSAIDs were chronically impeded from normal transit and created severe esophageal injury at the level of the web. There have been many reported cases of celiac disease associated with Plummer-Vinson syndrome. Some clinicians recommend random duodenal biopsies in all patients with this disorder.
The precise pathophysiology of Plummer-Vinson syndrome is unclear. It is unlikely that iron deficiency anemia is the sole explanation for the development of the webs, because webs are found in only approximately 10% of patients with iron deficiency anemia. Furthermore, webs that are indistinguishable from those seen in Plummer-Vinson syndrome form in patients in the absence of iron deficiency anemia. However, it is believed that iron deficiency does play a significant role in the development of dysphagia. In animal models of iron deficiency, abnormal muscle fibers in the pharyngeal muscles have been noted and are thought to be responsible for dysphagia. Evidence to support this hypothesis derives from the fact that manometry of patients with Plummer-Vinson syndrome has shown decreased swallowing pressures at the pharyngeal level. Patients treated with iron supplementation alone frequently have rapid improvement of their dysphagia, even if the web is left intact. Other theories have focused on ongoing tissue injury and healing causing mucosal changes. Still other theories have suggested that there may be an autoimmune component to the syndrome, because it appears to be more common among patients with autoimmune conditions, such as rheumatoid arthritis, thyroid disease, and celiac sprue.
Treatment of Plummer-Vinson syndrome has 2 main components. Iron supplementation is the more important of these components. As mentioned above, iron supplementation alone can resolve dysphagia. Additionally, if there is significant obstruction of the lumen by the web, rupture of the membrane may be necessary. The latter can generally be accomplished by standard bougie dilatation. Patients will occasionally require multiple sessions to obtain complete relief of symptoms. It is also important to note that patients with Plummer-Vinson syndrome have an increased risk of developing carcinoma, with an incidence of 3% to 15%. Most clinicians recommend yearly endoscopy to screen these patients.
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What Causes Difficulty In Swallowing?

Plummer-Vinson syndrome, also known as Paterson-Kelly syndrome, is a rare disorder that is characterized by dysphagia, upper esophageal webs, and iron deficiency anemia. The syndrome is most common among patients aged 40-70 years. Ninety percent of affected patients are female. Patients generally complain of long-standing, intermittent, painless dysphagia. Most commonly, patients first have dysphagia to solids, but, over time, symptoms can progress to involve dysphagia to liquids as well. Progression of the dysphagia can eventually lead to weight loss. Patients with Plummer-Vinson syndrome are frequently thin and pale. Nail abnormalities are common and koilonychia, spooning of the nails, is typical. The nails are also frequently quite brittle. Other findings on physical examination include angular cheilitis, glossitis, splenomegaly, and edentia. Because iron deficiency anemia is a part of the syndrome, laboratory findings consistent with iron deficiency anemia are necessary for the diagnosis. The latter includes microcytic anemia with a low iron level, high total iron-binding capacity, and a low ferritin level. A barium swallow will normally reveal 1 or several esophageal webs. A barium study was not performed on our patient because of the definitive nature of the EGD. However, we have recently encountered another patient with Plummer-Vinson syndrome who had a typical web on barium swallow. Upper esophageal webs are the classic endoscopic finding in Plummer-Vinson syndrome. These structures, which are smooth, thin, and gray in coloration, are found just below the cricopharyngeus muscle. The web usually attaches to the esophagus anteriorly, and the lumen is generally eccentric. Occasionally, these structural abnormalities can be missed if the endoscope is not passed under direct visualization. The webs frequently can be easily ruptured by the scope. The patient presented in this case did not have a typical web. However, the patient whose barium swallow is shown in Figure 5 had an endoscopy that demonstrated a very classic appearance of a delicate upper esophageal web. Although esophageal webs are an essential characteristic of Plummer-Vinson syndrome, there have been case reports of patients with all of the classical features of this syndrome, who presented with cervical esophageal strictures instead of webs. In our patient, there was the suggestion that a web high in the esophagus was destroyed with the first passage of the scope. There was an unequivocal short stricture high in the esophagus that had to be dilated. It is possible that NSAIDs were chronically impeded from normal transit and created severe esophageal injury at the level of the web. There have been many reported cases of celiac disease associated with Plummer-Vinson syndrome. Some clinicians recommend random duodenal biopsies in all patients with this disorder. The precise pathophysiology of Plummer-Vinson syndrome is unclear. It is unlikely that iron deficiency anemia is the sole explanation for the development of the webs, because webs are found in only approximately 10% of patients with iron deficiency anemia. Furthermore, webs that are indistinguishable from those seen in Plummer-Vinson syndrome form in patients in the absence of iron deficiency anemia. However, it is believed that iron deficiency does play a significant role in the development of dysphagia. In animal models of iron deficiency, abnormal muscle fibers in the pharyngeal muscles have been noted and are thought to be responsible for dysphagia. Evidence to support this hypothesis derives from the fact that manometry of patients with Plummer-Vinson syndrome has shown decreased swallowing pressures at the pharyngeal level. Patients treated with iron supplementation alone frequently have rapid improvement of their dysphagia, even if the web is left intact. Other theories have focused on ongoing tissue injury and healing causing mucosal changes. Still other theories have suggested that there may be an autoimmune component to the syndrome, because it appears to be more common among patients with autoimmune conditions, such as rheumatoid arthritis, thyroid disease, and celiac sprue. Treatment of Plummer-Vinson syndrome has 2 main components. Iron supplementation is the more important of these components. As mentioned above, iron supplementation alone can resolve dysphagia. Additionally, if there is significant obstruction of the lumen by the web, rupture of the membrane may be necessary. The latter can generally be accomplished by standard bougie dilatation. Patients will occasionally require multiple sessions to obtain complete relief of symptoms. It is also important to note that patients with Plummer-Vinson syndrome have an increased risk of developing carcinoma, with an incidence of 3% to 15%. Most clinicians recommend yearly endoscopy to screen these patients.