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Plummer-Vinson Syndrome Affecting Esophagus

mucosal web in plummer-vinson syndromeWhat Is Plummer-Vinson Syndrome Affecting the Esophagus?

[dropcap]P[/dropcap]lummer-Vinson syndrome is a manifestation of severe, long-term, iron deficiency anemia that is characterized by post-cricoid esophageal webs and dysphagia.

Q: What are esophageal webs?

A: Esophageal webs are one or more thin horizontal membranes consisting of squamous epithelium (cells that line the surface of the esophagus) and submucosa. They usually protrude from the anterior (front) wall, extending laterally across the inside esophagus but not to the posterior (rear) wall, which means that they rarely encircle the lumen.1

Dysphagia, or difficulty swallowing, from these webs is commonly painless and intermittent or progressive and may cause obstruction.

Webs can be detected by barium swallow X-ray, but the best way for demonstration is videofluoroscopy and by upper gastrointestinal endoscopy. They appear smooth, thin, and gray with eccentric or central lumen space. The webs typically occur in the upper part of the esophagus and may be missed and accidentally ruptured unless the endoscope is introduced under direct visualization.1

Iron deficiency is believed to decrease the contraction amplitude or force of the esophageal muscle resulting in motility impairment. Slower transit times have been recorded at the proximal and middle parts of the esophagus of Plummer-Vinson syndrome patients compared to healthy volunteers.2 Transit time is how fast ingested food and fluids travel through the esophagus.

Gude et al, report that iron replacement does not necessarily reverse the dysphagia in all the cases of Plummer-Vinson syndrome and that close monitoring of the web is mandated to watch for malignant change.  In fact, 3 to 15 per cent of the patients with Plummer-Vinson syndrome, mostly women between 15 and 50 years of age, have been reported to develop esophageal or pharyngeal cancer.2

What Is Plummer-Vinson Syndrome Affecting the Esophagus In Celiac Disease and/or Gluten Sensitivity?

Sources:
  1. Novacek G. Plummer-Vinson syndrome. Orphanet J Rare Dis. 2006; 1: 36. Published online 2006 September 15. doi:  10.1186/1750-1172-1-36. [↩] [↩]
  2. Gude D, Bansal DP, and Malu A. Revisiting Plummer Vinson Syndrome. Annals of Medical and Health Sciences Research. 2013 Jan-Mar;3(1):119-121. [↩] [↩]

Gastroesophageal Reflux Disease (GERD) 

GERD Gluten Celiac Disease
Arrows Show Abnormal Movement of Gastric Acid in Gastroesophageal Reflux Disease.

What Is Gastroesophageal Reflux Disease (GERD)?

[dropcap]G[/dropcap]ERD is an upper digestive disorder that is characterized by a decrease in lower esophageal sphincter pressure (LES,) which allows the abnormal reflux or backflow of stomach contents into the esophagus. It is also called erosive esophagitis or reflux esophagitis and is the most common disorder of the esophagus.

The esophagus is a muscular tube that transports swallowed substances to the stomach. It begins at the cricoid cartilage (Adam’s apple) as a continuation of the pharynx and ends at the lower esophageal sphincter (LES).

The lower esophageal sphincter is located at the junction of the esophagus and the stomach. It functions like a circular band to tighten after food is ingested in order to prevent its going back up the esophagus.

Q: How does reflux damage the esophagus?

A: Damage to the lining of the esophagus is induced by the caustic, chemical action of acid and pepsin in gastric juice and, in severe cases, also bile salts, that back upwards from the stomach through an impaired LES. Gastric acid combined with pepsin or bile salts seems to be more harmful to the esophageal epithelial layers than gastric acid alone.1

Pepsin is normally produced by the stomach to dissolve protein in swallowed food. Unfortunately, when the esophagus is inflamed, pepsin will act on it to break down the protein in its sore wall. These sores are called erosions.

Importantly, refluxate to the esophagus in patients with acid suppression therapy is different from those in patients without. Higher levels of secondary bile acids are detected in patients with acid suppression therapy. Even if acid suppression is successful, weakly acidic reflux with bile acids can damage the esophagus.1

Damage starts at the luminal surface (inside where food passes through) of the squamous epithelium (tough surface cells) and progresses through the underlying layers into the submucosa.

One of the primary functions of the esophageal epithelium is to protect the underlying tissue from mechanical and chemical damage by acting as a barrier. The epithelial layers of the distal esophagus need to withstand reflux from the stomach and its contents. When the epithelium fails to protect the underlying tissue from this damage, it leads to erosions, esophagitis, and may lead to Barrett’s esophagus.1

Barrett’s esophagus and esophageal small cell cancer are severe complications of GERD that can be fatal.

GERD can result from too much, or more commonly, too little stomach acid.

What Is Gastroesophageal Reflux Disease (GERD) In Celiac Disease and/or Gluten Sensitivity?

Sources:
  1. Chen X, Oshima T, Tomita T, Fukui H, Watari J, Matsumoto T, Miwa H. Acidic bile salts modulate the squamous epithelial barrier function by modulating tight junction proteins. Am J Physiol Gastrointest Liver Physiol. 2011 Aug;301(2):G203-9. doi: 10.1152/ajpgi.00096.2011. Epub 2011 May 26. [↩] [↩] [↩]

Glycogenic Acanthosis (Esophageal Plaques)

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View of Esophagus Showing Distinctive Plaques In Glycogenic Acanthosis.

What Is Glycogenic Acanthosis?

Glycogenic acanthosis is a benign thickening of the esophageal squamous epithelium (surface cell lining)  characterized by the presence of numerous, uniformly grey-white plaques made up of large squamous cells filled with glycogen.

The wax-like plaques in glycogenic acanthosis are usually 2-10 mm in diameter and may be confluent round elevations involving the entire esophageal surface.1

in radiographs (side-by-side) showing appearances of esphagus with glycogenic acanthosis. Courtesy The Radiology Assistant.com
Two radiographs (side-by-side) showing appearances of different esophagus with glycogenic acanthosis. Courtesy Radiology Assistant.nl

On x-ray  views of the well-distended esophagus, the plaques appear as a finely nodular or cobblestone mucosal pattern.

The findings are not associated with mucosal ulcerations, luminal narrowing, or mobility disturbance, although some patients may have coexistent hiatal hernia and gastroesophageal reflux.2

What Is Glycogenic Acanthosis In Celiac Disease and/or Gluten Sensitivity?

Sources:
  1. Suoglu OD, Emiroglu HH, Sokucu S, Cantez S, Cevikbas U, Saner G. Celiac disease and glycogenic acanthosis: a new association? Acta Paediatrica. Apr 2004;93(4):568-70. [↩]
  2. Ghahremani GG, Rushovich AM. Glycogenic acanthosis of the esophagus: radiographic and pathologic features. Gastrointest Radiol. 1984;9(2):93-8. [↩]

Cancer, Esophageal Small Cell

esophageal small cell carcinomaWhat Is Esophageal Small Cell Cancer (Carcinoma)?

[dropcap]E[/dropcap]sophageal small cell carcinoma is a rare and aggressive malignancy arising in the esophagus with a poor prognosis.

Q: Where does the cancer occur in the esophagus?

A: Esophageal cancer can occur anywhere in the esophagus.

The esophagus is a muscular tube that transports swallowed substances to the stomach. It begins at the cricoid cartilage (Adam’s apple) as a continuation of the pharynx and ends at the lower esophageal sphincter (LES).

The LES is a circular muscle surrounding the junction of the esophagus and stomach. The LES opens to allow swallowed food and liquids to enter the stomach and closes to prevent their travelling back into the esophagus.

Dysphagia (difficulty swallowing) occurs when the inside diameter of the esophagus constricts to less than 14 millimeters. First there is difficulty swallowing solid food then liquids and saliva due to progressive growth of the cancer.

What Is Esophageal Small Cell Carcinoma In Celiac Disease and/or Gluten Sensitivity?

Esophageal Motor Abnormalities – Food Sticks In Esophagus

Beaded Barium Swallow Shows Spasms of Espophagus. Courtesy Quizlet.com
Beaded Barium Swallow Shows Spasms of Espophagus. Courtesy Quizlet.com

What Are Esophageal Motor Abnormalities?

[dropcap]E[/dropcap]sophageal motor abnormalities are disorders of the esophagus involving altered muscular motility characterized by impaired esophageal peristalsis, or the progressive wave-like movement that occurs automatically when food or fluids are swallowed.

Q: What is altered motility?

A: Altered motility describes the lack of coordinated muscular movements or weak muscular movement needed to rapidly propel food and fluids through the highly muscular esophagus from the throat to the stomach.

The esophagus is a tube-like organ that begins at the cricoid cartilage (Adam’s apple) as a continuation of the pharynx and ends at the lower esophageal sphincter (LES).

The LES is a very strong, circular muscle surrounding the junction of the esophagus and stomach. The LES opens to allow swallowed food and liquids to enter the stomach and closes to prevent their travelling back into the esophagus.

Failure of the lower esophageal sphincter to remain tightly closed after swallowing allows stomach contents to reenter the esophagus giving symptoms of heartburn.

On the other hand, failure of the LES to relax traps the contents of the esophagus so that it cannot quickly empty into the stomach as it should. Because the esophagus is not designed to hold food/fluids, the abnormal expansion results in pain, anxiety and possibly vomiting and choking.  If the contents are aspirated into the windpipe upon choking, there will be coughing with attempts to clear it.

What Are Esophageal Motor Abnormalities In Celiac Disease and/or Gluten Sensitivity?

Heartburn

Heartburn Due to Acidic Stomach Contents Re-entering the Esophagus. Courtesy kaiserpermanente.org
Heartburn Due to Acidic Stomach Contents Re-entering the Esophagus.

What Is Heartburn?

[dropcap]H[/dropcap]eartburn is a functional upper digestive symptom of esophagitis that is characterized by a burning sensation behind the breastbone.

Q: Why does esophagitis cause heartburn?

A: Esophagitis is inflammation of the mucosal lining of the esophagus. The most common cause of esophagitis is the re-entering of stomach contents into the esophagus because the lower esophageal sphincter (LES) does not close tightly after food or fluids enter the stomach from the esophagus and/or the LES does not remain tightly closed.

The LES is located at the junction of the esophagus and the stomach. It functions like a circular band to tighten after food is ingested in order to prevent its going back up the esophagus when powerful stomach muscles churn ingested food.

Damage to the lining of the esophagus (esophagitis) results upon contact with hydrochloric acid and pepsin in gastric juice. Hydrochloric acid, produced by the stomach to dissolve food, inflames the esophagus, which is the opposite, or alkaline. Pepsin, produced by the stomach to dissolve protein in swallowed food, acts on the inflamed esophagus to break down the protein in its sore wall.

Heartburn can be caused by other disorders such as low stomach acid, obesity, and smoking. Relaxation of the LES can also result from dietary items such as chocolate, mint, caffeine, and alcoholic drinks.

Who is Affected in the General Population? Heartburn affects an estimated 42% of the US population.1

What Is Heartburn In Celiac Disease and/or Gluten Sensitivity?

Sources:
  1. http://informahealthcare.com/doi/abs/10.1185/03007990903553812. [↩]

Cancer Of The Esophagus 

A real photomicrograph of carcinoma of the esophagus. Panorama of 6 photos of a slide at 40x through the microscope. Some areas may appear blurry due to shallow DOF.
A real photomicrograph of carcinoma of the esophagus. Panorama of 6 photos of a slide at 40x through the microscope. Some areas may appear blurry due to shallow DOF.

What Is Cancer Of The Esophagus?

[dropcap]C[/dropcap]ancer of the esophagus is a malignancy arising in the stratified squamous cell lining of any part of the esophagus and having a poor prognosis.

This tumor first invades the deeper layers of the esophagus which includes the layer beneath the mucosa (submucosa) and muscle before it may invade structures close to it including the aorta and trachea. Later, it may spread to organs further away such as the liver, lungs and bones.

Q: What is the stratified squamous cell lining of the esophagus?

A: Stratified squamous cells are flat epithelial cells that are composed of several layers, called the epithelium. This type of epithelium forms the surface mucosa that lines the inside of the esophagus.

The esophagus itself is a muscular tube that transports swallowed substances to the stomach. It begins at the cricoid cartilage (Adam’s apple) as a continuation of the pharynx in the throat and ends at the lower esophageal sphincter (LES).

The LES is a circular muscle surrounding the junction of the esophagus and stomach. The LES opens to allow swallowed food and liquids to enter the stomach and closes to prevent their travelling back into the esophagus.

Who is Affected in the General Population?

Esophageal cancer is the fifth leading cause of death in men from cancer worldwide.1

What Is Cancer Of The Esophagus In Celiac Disease and/or Gluten Sensitivity?

Sources:
  1. Feldman M, Friedman LS, Brandt LJ. Sleisenger & Fordtran’s Gastrointestinal and Liver Disease. ed 9. Philadelphia: Saunders; 2010. pp. 745–767. [↩]

Dysphagia (Difficulty Swallowing)

What Is Dysphagia? [dropcap]D[/dropcap]ysphagia is a functional upper digestive disorder characterized by difficulty in swallowing or food getting stuck and swallowing that takes longer than 10 seconds to complete. Q: What determines the symptoms? A:…