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Esophageal Pathologies

Esophageal Pathologies

Here we'll learn about esophageal pathologies, which are often characterized by aphagia (inability to swallow), dysphagia (difficulty swallowing), or odynophagia (painful swallowing).

Common diagnostic tools include upper endoscopy, barium swallow (aka, esophagogram), and manometry (a test that measures the pressure in parts of the GI tract).

Inflammatory and infectious disorders

GERD (gastroesophageal reflux disease) is extremely common. It occurs when lower esophageal sphincter tone is reduced. The loss of LES tone allows acid reflux, which damages the lower esophagus.

Common symptoms include esophagitis with heartburn (epigastric pain that radiates to the chest, beware that it can mimic angina) and acid regurgitation, which can create a characteristic bad taste in the mouth. Some patients also have chronic cough and hoarseness from the acid reflux. Many patients are asymptomatic.

Complications: Barrett's esophagus & cancer.

Endoscopy may show erosion, ulcers, and stricture formation.

Risk factors include alcohol use and tobacco smoking.

Treatment typically relies on proton-pump inhibitors. In some cases, Nissen fundoplication is performed to surgically create a new lower esophageal sphincter.

Barrett's esophagus is a complication of GERD; it usually presents 5-10 years after GERD.

Upon examination, we'll see that the normal squamous epithelium of the esophagus is replaced by intestinal epithelium with metaplastic columnar epithelium with goblet cells.

See below for image credits.

Barrett's esophagus predisposes individuals to dysplasia and adenocarcinoma.

Esophageal cancer is characterized by progressive dysphagia and weight loss. The fact that patients first experience difficulty swallowing foods, then also liquids, is an important sign of cancer vs some other conditions.

Squamous cell carcinoma usually occurs in the upper 2/3 of the esophagus; risk factors include strictures and achalasia.

Adenocarcinoma usually occurs in the distal 1/3 of the esophagus; risk factors include GERD and Barrett esophagus. Adenocarcinoma has poor prognosis.

Eosinophilic esophagitis, which is an immune response to dietary allergens; patients often have other atopic disorders (asthma, eczema). Classic endoscopic findings are: stacked circular rings (cirumferential lesions) with muscoal furrowing.

Infectious esophagitis (usually caused by Candida, herpes, or CMV, esp. in immune-compromised patients).

In suspected candida esophagitis, treat with fluconazole. If this fails, proceed to upper endoscopy with biopsy to look for other potential causes. Nystatin swish and swallow is insufficient to treat candida esophagitis.

Pill-induced, when medications stick in the esophagus, or, toxin-induced esophagitis (upon ingestion of corrosive substances).

Motility disorders

These disorders often cause dysphagia.

Esophageal spasms are more common. They are characterized by repetitive and non-propulsive contractions; unlike achalasia, manometry will show normal sphincter responses. Spasms produce angina-like chest pain.

Treatments include sedatives, nitrates, botulinum toxin, and surgery; unfortunately, treatment failure rates are high.

Achalasia is caused by failure of the lower esophageal sphincter to relax due to degeneration in the myenteric plexus (in other words, the LES remains closed).

As a result, patients experience: Regurgitation (with increased risk of aspiration and pulmonary complications). Progressive dysphagia of both foods and liquids, with weight loss due to slower/reduced eating and drinking (although the weight loss may be mild). Notice that this is different from what we see in esophageal cancer, where patients first experience difficulty swallowing solids then difficulty swallowing liquids.

With a barium swallow test, we'll see a characteristic "bird's beak" at the distal end of the esophagus where the constricted LES creates stenosis.

In most U.S. patients, most cases of achalasia are idiopathic; adenocarcinoma of the proximal stomach is the second leading cause.

Be aware that worldwide, achalasia is most often caused by Chagas disease.

Be aware that there are three subtypes of achalasia, which influences their treatment and outcomes.

Treatments for achalasia include surgery, botulinum toxin, and pneumatic dilation.

Structural Disorders

These disorders are typically associated with dysphagia but may be asymptomatic.

Esophageal webs and rings are thin membranes that grow across the esophagus.

We use barium swallow and endoscopy to visualize the membranes.

See below for image credits.

Plummer Vinson syndrome (aka Paterson-Brown-Kelly syndrome) is characterized by the classic triad of dysphagia, iron-deficient anemia, and upper esophageal web with squamous epithelium on both sides.

Patients have increased risk of squamous cell carcinoma of the throat.

Researchers are uncertain why esophageal webs form, but it is thought that iron-deficient anemia causes mucositis and membrane formation (because dysphagia sometimes improves with iron supplementation).

Thus, we look for signs of iron-deficient anemia – weakness, pallor, glossitis (swollen inflamed tongue), koilonychia (spoon nails), and treat with iron supplements or dilation.

Schatzki rings, aka, B rings, appear in the distal esophagus at the squamocolumnar junction.

They are thought to be the result of GERD and are always associated with hiatal hernias. Dilation or ring biopsy can open the esophagus to treat dysphagia.

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Esophageal strictures are abnormal narrowings of the esophageal lumen.

There are many causes, but GERD is the most common cause in adults, and ingestion of corrosive substances is the most common cause in children/adolescents.

Other causes include esophagitis, drugs, malignancies, and radiation.

Treatment includes dilation and addressing the underlying causes (i.e., treating GERD with proton-pump inhibitors).

Perforation allows gastric contents and bacteria to leak into mediastinum; this is rare but often deadly if not surgically repaired within 24 hours.

Patients experience severe chest, neck, and shoulder pain with dysphagia and potentially shock. Notice that the pain pattern resembles that of myocardial infarction.

We also look for Hamman's sign, which is a crunching or rasping sound caused by the heart beating in a pneumomediastinum (aka, mediastinal emphysema - air in the mediastinum).

Perforation is usually iatrogenic (often from endoscopy) but can also be caused by the ingestion of foreign bodies, or by trauma.

Boerhaave syndrome is perforation due to violent vomiting. Recall that Mallory-Weiss tears are tears in the distal esophagus and are often due to vomiting.

Mallory-Weiss tears:

See below for image credits.

Hiatal hernias come in a variety of forms and are often asymptomatic, but may also present with heartburn, regurgitation, chest pain, and dysphagia. The causes are often unknown.

Sliding, aka, Type 1 hernias make up 95% of cases. They occur when the gastroesophageal junction and a portion of the stomach herniate into the thoracic cavity. Sliding hernias can cause GERD and aspiration, and are usually treated with proton-pump inhibitors.

Paraesophageal hernias (types 2-4) are true hernias in which a sac protrudes above the diaphragm. The specific type is determined by the location of the gastroesophageal junction and which segments have herniated; Type 4 involves herniation of the stomach and other organs. Complications of paraesophageal hernias include obstruction, hemorrhage, and strangulation; thus, they are potentially life-threatening and require surgical treatment.

Diverticula are bulges in the esophageal wall that are usually secondary to motility issues; we use barium swallow to diagnose.

Esophageal Diverticula

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Zenker diverticula are the most common type; these form when the upper esophageal sphincter (aka, cricopharyngeal muscle) doesn't relax and intraluminal pressure is increased. They occur in the upper 1/3 of the esophagus, producing dysphagia with regurgitation, halitosis (bad smelling breath), and chronic cough. Note that regurgitated food may be from several days ago.

Traction diverticula (aka, midesophageal diverticula) are secondary lesions caused by external forces. They are usually the result of mediastinal inflammation, often from tuberculosis, that pulls on the esophageal wall and creates a true diverticulum.

Epiphrenic diverticula occur in the distal 1/3 esophagus; these are usually the result of motility disorders.

For references, see full tutorial.

Esophageal Pathologies

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