Esophageal disorders: Pathology review

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Case Study0:00–0:52

A 33-year-old named Ravi came to the clinic because he has difficulty swallowing food and water over the last 3 months. Physical examination shows significant weight loss, of 7-kg or 15-lb, since his last visit 4 months ago.
Esophageal manometry shows incomplete lower esophageal sphincter relaxation in response to swallowing, while barium swallow reveals a dilated esophagus with an area of distal stenosis.
At the same time, a 62-year-old man named Frank comes to the clinic because of bad breath, regurgitation of food overnight, and trouble swallowing food.
He has had these symptoms for several months. He denies fever, chills, nausea, vomiting, or weight loss.
Physical examination shows a mass on the side of the neck. vNow, both Ravi and Frank have some form of the esophageal disorder.

Pathology0:52–1:25

Esophageal disorders can be subdivided into: inflammatory esophageal disorders, or esophagitis, which are characterized by an inflammation of the esophageal lining along with dysphagia, and odynophagia; functional esophageal disorders, which affect the muscles and nerves that control the motility of the esophagus and cause intermittent dysphagia for solids and liquids; and mechanical esophageal disorders, which are characterized by the blockage of the passageway and they typically cause progressive dysphagia for solids.
Inflammatory esophageal disorders, also known as esophagitis, are characterized by an inflammation of the esophageal lining and based on the cause, they are also subdivided into several types.

Esophagitis1:25–4:12

First, there’s reflux esophagitis, which is associated with the reflux of gastric acid from the stomach back into the esophagus.
Alternatively, pill-induced esophagitis, where a medication injures the esophagus thereby causing inflammation and possible upper GI bleeding.
It is associated with medications such as nonsteroidal anti-inflammatory drugs or NSAIDs, bisphosphonates, tetracyclines, iron, and potassium chloride.
In caustic esophagitis, caustic agents, such as strong acids like vinegar or strong bases like detergents, cause esophageal lesions.
The next one is infectious esophagitis, which is most commonly seen in immunocompromised individuals, such as HIV-positive individuals.
The most common causes of HIV associated esophagitis include candida albicans, herpes simplex virus 1, and cytomegalovirus.
A high yield fact to remember is that with candida esophagitis, the upper endoscopy will show patches of adherent, white or grey pseudomembranes on the underlying mucosa.
The histopathology reveals yeast cells and pseudohyphae that invade mucosal cells of the esophagus. Any attempt to remove the pseudomembrane can cause bleeding in the underlying mucosa.
With HSV esophagitis, a high yield fact to remember is that an upper endoscopy will show small vesicles and lesions that look like small punched-out ulcers; while the histopathology reveals eosinophilic intranuclear inclusions in multinuclear squamous cells at the margin of the ulcer.
For your exam, you have to know that these inclusions are called Cowdry type A inclusions. CMV esophagitis features linear ulcers on the upper endoscopy, while histopathology reveals both intranuclear and cytoplasmic inclusions.
Finally, we have eosinophilic esophagitis, also called allergic esophagitis, which is characterized by eosinophils that infiltrated into the lining of the esophagus.
This occurs as a reaction to food allergens and it can lead to dysphagia and food impaction. Eosinophilic esophagitis is most commonly seen in individuals who have other allergies and a high yield fact that’s often used as a clue is that their esophagitis will be unresponsive to GERD therapy.
During an upper endoscopy, eosinophilic esophagitis is characterized by linear furrows and esophageal rings, which are thin mucosal bands that surround the esophagus.
Moving on to functional esophageal conditions, which include achalasia, diffuse esophageal spasm, and sclerodermal esophageal dysmotility.
Achalasia is when there’s impaired esophageal motility and the inability to relax the lower esophageal sphincter. The most common cause of primary achalasia is idiopathic degeneration or damage of postganglionic inhibitory neurons in the myenteric, or Auerbach, plexus of the esophagus.

Achalasia4:12–7:03

There’s also secondary achalasia which is caused by Trypanosoma Cruzi infection that cause Chagas disease, or extraesophageal malignancies.
So normally, the neuron in the myenteric plexus release inhibitory neurotransmitters, such as nitric oxide and vasoactive intestinal peptide, which relax the lower esophageal sphincter.
Eventually, the lack of inhibitory neurotransmitters leads to an increased resting lower esophageal sphincter tone, and this obstruction leads to dilatation of the esophagus.
These individuals present with progressive dysphagia for both solids and liquids, regurgitation of undigested food, aspiration, chest pain, heartburn, and weight loss.
In addition, they have an increased risk for esophageal squamous cell and adenocarcinoma. For diagnosis, remember for your exam that barium swallow in achalasia reveals dilatation of the esophagus above the obstruction and tapering of the lower part of the esophagus near the lower esophageal sphincter.
This is also known as the bird’s beak sign. The gold standard for diagnosis is esophageal manometry, which measures the strength and coordination of the esophageal contractions when a person swallows.
This measurement is done at multiple levels, including the upper, middle, and lower esophagus, as well as the lower esophageal sphincter.
Now, you might be asked to analyze manometry findings on your exam so let’s go over this. Normally when a person swallows, involuntary contractions of the pharyngeal muscles propel the food into the esophagus.
Propulsion of the food bolus is followed by the contraction of the cricopharyngeal muscle which initiates the peristaltic wave of the esophagus.
This contraction is shown as an upward deflection on the upper esophageal manometry. Next, the middle esophageal manometry reflects normal peristalsis of the middle part of the esophagus.
Finally, the lower esophageal manometry suggests the decrease in the lower esophageal sphincter that corresponds to its relaxation and the passing of the bolus into the stomach.
Now for your exam, you have to know that individuals with achalasia have normal findings in the upper part of the esophagus, decrease or absence of peristalsis in the middle part of the esophagus, and high pressure in the lower esophageal sphincter.
For treatment, the obstruction can be corrected with balloon dilation or increased tone can managed with local injection of botulinum toxin.In diffuse esophageal spasm, there’s periodic, non-peristaltic contractions that occur simultaneously with each other.

Diffuse Esophageal Spas7:03–8:20

In contrast to achalasia, diffuse esophageal spasm is associated with a normal lower esophageal sphincter tone since the spasms occur in the walls of the esophagus.
For your exam, you have to know that these disorganized involuntary esophageal contractions can cause intermittent dysphagia for both solids and liquids, and occasional retrosternal chest pain.
It’s important to note that the pain can mimic angina but it’s not associated with physical activity and is not relieved by rest; however this can still resemble unstable angina.
Therefore, every person that is suspected of having diffuse esophageal spasm should undergo a complete cardiac work-up to rule out any cardiac pathology.
If performed at the time of symptoms, barium swallow will show a "corkscrew" or “rosary bead” appearance of the esophagus, but this is not specific to diffuse esophageal spasm.
The gold standard for diagnosis is manometry which shows uncoordinated high-amplitude peristaltic contractions that can appear throughout the esophagus.
Finally, first-line medications in the treatment of diffuse esophageal spasm include nitrates and calcium channel blockers.The next disorder is scleroderma or systemic sclerosis.

Sclerodermal Esophageal 8:20–10:03

This is an autoimmune disease where there is fibrosis of the skin and other organs. These include the esophagus, kidneys, heart, lungs, and muscles.
On the other hand, CREST syndrome, now called limited cutaneous form of systemic sclerosis, is the less severe form of the disease.
CREST stands for: calcinosis, which are calcium deposits in soft tissues, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, which is skin tightening, and telangiectasia, or spider veins near the surface of the skin.
For your exam, you have to know that in sclerodermal esophageal dysmotility, the smooth muscle in the esophagus atrophies and as a result, peristaltic contractions in the mid and distal esophagus become ineffective or totally absent.
In addition, the lower esophageal sphincter tone is lower than normal which can lead to gastroesophageal reflux disease and dysphagia.
So these individuals can also present with Barrett esophagus, esophageal strictures, or aspiration. The barium swallow can appear normal, but an upper endoscopy with biopsy can show evidence of muscle atrophy and fibrosis.
Finally, manometry in these individuals shows reduced esophageal peristalsis in the middle and lower esophagus and decreased pressure in the lower sphincter.Finally, we have mechanical esophageal conditions which include Schatzki’s ring, Plummer-Vinson syndrome, Zenker’s diverticulum, esophageal strictures, and obstruction.
Schatzki’s ring is a thin symmetric mucosal band, which is most commonly located at the gastroesophageal junction. The exact cause is unknown, but it can be associated with chronic acid reflux.

Schatzki’s Ring10:03–10:34

For your exam, you should know that in most cases, individuals have no symptoms, but if the lumen of the esophagus gets too narrowed, individuals can present with intermittent dysphagia for solids.
On a Barium swallow, Schatzki’s ring is seen as a small and regular ridge above the diaphragm that narrows the lumen of the esophagus.The next one is Plummer-Vinson syndrome.

Plummer-Vinson Syndrome10:34–11:31

The high yield concept here is that Plummer-Vinson syndrome is known for causing Dysphagia, Iron deficiency anemia, and Esophageal webs.
So, the unfortunate mnemonic for this disorder is “Plumbers DIE.” These individuals can present with intermittent dysphagia to solids, but also glossitis and fatigue due to iron deficiency anemia.
The symptoms of dysphagia can improve with the correction of iron deficiency anemia if there are no advanced esophageal webs.
With esophageal webs, a thin membrane made of squamous epithelium projects in the lumen of the esophagus, but unlike Schatzki’s ring, an esophageal web is mostly found in the upper third of the esophagus and it doesn’t occupy the entire circumference of the lumen.
On a Barium swallow, esophageal webs appear just like Schatzki’s ring, only higher in the esophagus. Finally, Plummer-Vinson syndrome is associated with an increased risk of squamous cell carcinoma.
The next one is Zenker’s diverticulum, which is also known as a pharyngoesophageal false diverticulum. For your exam, a high yield fact is the underlying cause of this condition is impaired relaxation of the cricopharyngeal muscle.

Zenker’s Diverticulum11:31–12:59

As a result, pharyngeal muscles contract more forcefully to propel the food to the esophagus, but at the same time, they increase the pressure within the distal pharynx.
Ultimately, this leads to herniation of the mucosa and submucosa through an area with muscle weakness, resulting in the formation of a pouch-like structure outside the lumen of the esophagus.
The most common location for Zenker's diverticulum is at the Killian’s triangle, between the thyropharyngeus and cricopharyngeus muscles.
Individuals with cricopharyngeal muscle dysfunction typically complain about difficulty swallowing where food gets stuck at the throat level and this is also described as ‘’high dysphagia’’.
Other symptoms include coughing; choking; but also halitosis, or bad breath, which occurs when food gets trapped in the diverticulum and rots.
Food regurgitation is also a symptom and it can lead to food aspiration and subsequent pneumonia. Zenker’s diverticulum is more common in elderly males which typically present with a palpable mass on the lateral side of the neck.
Finally, the best way to diagnose a Zenker diverticulum is by barium swallow, where the diverticulum fills with contrast medium, while the treatment includes surgical removal of the diverticulum.
Moving on to esophageal strictures which is the narrowing of the esophagus. They can be subdivided into benign or malignant.
Benign esophageal strictures are typically caused by reflux esophagitis, eosinophilic esophagitis, ingestion of caustic agents; and these individuals have symptoms of progressive dysphagia for solids, odynophagia, heartburn, and unintended weight loss.

Esophageal Strictures12:59–14:17

On a barium swallow, the stricture causes the flow of barium to get obstructed so that it looks like a thin string of contrast going through the esophagus.
On the other hand, malignant esophageal strictures are caused by the tumor growing inside the esophagus. Like in benign esophageal stricture, symptoms include progressive dysphagia for solids, odynophagia, heartburn, and unintended weight loss.
In addition, these individuals can have upper gastrointestinal bleeding which can lead to iron-deficiency anemia. On a barium swallow, the stricture again causes the flow of barium to get obstructed, but because tumors are often irregular in shape the narrowing of the lumen will be irregular as well.
Finally, for your exam you have to know that obstruction of the esophagus, such as malignant obstruction, can cause the same presentation and manometric findings as achalasia, and this condition is called pseudoachalasia.
All right, as a quick recap. Inflammatory esophageal disorders, also known as esophagitis, are characterized by an inflammation of the esophageal lining; and they include reflux esophagitis, pill-induced esophagitis, caustic esophagitis, infectious esophagitis, and eosinophilic esophagitis.
Functional esophageal disorders affect the muscles and nerves that control the motility of the esophagus and they typically cause intermittent dysphagia for solids and liquids.
Achalasia is associated with impaired esophageal motility and inability to relax the lower esophageal sphincter and it’s most commonly caused by idiopathic degeneration or damage of postganglionic inhibitory neurons in the myenteric plexus of the esophagus.

Review14:17–16:19

In diffuse esophageal spasm, peristaltic contractions of the esophagus are intermittent and uncoordinated, which leads to ineffective peristalsis of the esophagus.
With sclerodermal esophageal dysmotility, the smooth muscle in the esophagus atrophies and as a result, peristaltic contractions in the mid and distal esophagus becomes ineffective or totally absent.
Mechanical esophageal disorders are characterized by the blockage of the passageway and they typically cause progressive dysphagia for solids.
Schatzki’s ring is a thin, symmetric mucosal band, most commonly located at the gastroesophageal junction and it can be associated with chronic acid reflux.
Plummer-Vinson syndrome is known for causing dysphagia, iron deficiency anemia, and esophageal webs, which are thin asymmetric membranes that don’t occupy the entire circumference of the lumen and appear in the upper third of the esophagus.
With Zenker’s diverticulum, the mucosa and submucosa of the esophagus slide through an area of muscle weakness right above the upper esophageal sphincter and form a pouch-like structure outside the lumen of the esophagus.
Finally, benign esophageal strictures are caused by reflux esophagitis, eosinophilic esophagitis, ingestion of caustic agents; whereas malignant esophageal strictures are caused by the tumor growing inside the esophagus.
Now let’s go back to our case. Ravi came to the clinic because he experienced difficulty swallowing food and water over the last 3 months.
In other words, he has dysphagia to solids and liquids, therefore we can assume it’s a functional esophageal disorder. Esophageal manometry showed an incomplete lower sphincter relaxation, while the barium swallow revealed a dilated esophagus with an area of distal stenosis.
So, this is a classic case of achalasia. On the other hand, Frank came to the clinic because of bad breath, regurgitation of food overnight, and trouble swallowing solids.
Since his problem includes only solids, we can assume he has a mechanical esophageal disorder. The bad breath, food regurgitation and mass on the side of the neck are the key clues here, so in his case, a barium swallow was ordered which confirmed the diagnosis of Zenker’s diverticulum.

Summary16:19–17:12

words. He has dysphagia to solids and liquids.
Therefore we can assume it's a functional esophageal disorder esophageal manometry showed an incomplete lower sphincter relaxation.
While the barium swallow revealed a dilated esophagus with an area of distal stenosis to. This is a classic case of achalasia.
On the other hand, Frank came to the clinic because of bad breath regurgitation of food overnight and trouble. Swallowing solids.
Since his problem includes only solids, we can assume he has a mechanical esophageal disorder. The bad breath food, regurgitation, and mass in the side of his neck, are the key Clues here.
So, in this case, a barium swallow is ordered which confirm the diagnosis of zenker's diverticulum.