Plummer-Vinson syndrome

upper esophageal webs, iron deficiency anemia , and glossitis and cheilosis

Esophageal Webs

Endoscopy

• Thin (2-4 mm) ledge-like protrusions, extending < 5 mm into the esophageal lumen
• Usually located in the upper esophagus

Esophageal Webs 

(microscopic)

Fibrovascular connective tissue with overlying squamous epithelium

Esophageal Rings 

• Circumferential and thick
• Found in the distal esophagus, above the gastroesophageal junction

Esophageal Rings 

(microscopic)

• In addition to mucosa, contain submucosa and sometimes hypertrophic muscularis propria.
  • A-____above GE junction, covered by squamous mucosa
  • B-____: at squamocolumnar junction, also covered by squamous mucosa, may have gastric (cardia) mucosa on the underside

Esophageal Stenosis

(endoscopy)

• Narrowing of lumen, may see mucosal damage (dependant on cause)
• Lining usually thin, may be ulcerated

Esophageal Stenosis 

(microscopic)

Esophageal Stenosis

(clinical) 

• Progressive dysphagia, first with solid foods, then progressing to soft foods or liquids.
• Develops slowly
• Patients may subconsciously modify their diet

Achalasia

(clinical pte)

• Progressive dysphagia and weight loss, chest pain, regurgitation
• Treatment: laparoscopic myotomy, pneumatic balloon dilitation (also Botox injection)

Secondary Achalasia 

(etiology)

Primary is usually idiopathic

• Diabetic autonomic neuropathy
• Infiltrative disorders (sarcoidosis, amyloidosis, malignancy)
• Chagas disease (destruction of myenteric plexus)
• Dorsal motor nuclei lesions (polio, surgical ablation

Hiatal Hernia 

(sliding)

GE jxn? 

Above the diaphragm

no surgery unless complications

Paraesophageal Hernia 

Ge jxn normal position 

Stomach herniates along side of stomach

surgery needed --> tissue can incarcerate and strangulate

Types of Esophageal Diverticula

• Zenker diverticulum (pharyngoesophageal diverticulum):
  • Location: immediately above upper esophageal sphincter
  • Can be several cm in diameter

• Traction diverticulum:
  • Location: midesophagus

• Epiphrenic diverticulum:
  • Location: immediately above lower esophageal sphincter

True Diverticulum 

Outpouchings of esophagus containing all 3 layers (mucosa, submucosa, and Muscularis Propia)

Diverticulum 

Clinical Presentation 

Massive amounts of regurgitation

Esophageal Laceration

Mallory-Weiss tears

• Linear tears of the esophageal mucosa (milliliters to centimeters)
• Longitudinally oriented, usually crossing the GE junction

Hematemesis is a presenting sx 

Esophageal Laceration

Esophageal Laceration

(presenting symptoms) 

• Usually presents with hematemesis
• Healing is usually rapid without sequelae without need for intervention

Boerhaave's Syndrome

• Esophageal lacerations extending through esophageal wall, leading to perforation and mediastinitis
• Uncommon and potentially catastrophic, with an emergent presentation.

Esophageal Varices 

(morphology)

• Tortuous, dilated submucosal veins in distal esophagus and proximal stomach with overlying mucosa that may be eroded or inflamed
  • Seen on venogram, but may collapse in surgical specimens and on autopsy
• With rupture, there is massive hemorrhage into the lumen or into the wall; overlying mucosa is may be ulcerated
• Past rupture can show venous thrombosis and inflammation

Presents w/ massive hematemesis 

Esophageal Varices 

Reflux Esophagitis 

(GERD)

microscopic

Reflux Esophagitis (GERD)

endoscopy? 

hyperemic

GERD clinical presentation

• Dysphagia, heartburn
• May have regurgitation of "sour brash" 
• Can be inflammatory erosion, increased pressure with vomiting, or other
• Chronic GERD can cause severe chest pain that may mimic a myocardial infarction
• Complications: esophageal ulceration, hematemesism melena, strictures, Barrett esophagus.

Barret's Esophagus 

(endoscopy)

Tongues or patches of red velvety mucosa extending above the GE junction

Barret's Esophagus 

(microscopic) 

• Intestinal metaplasia, including goblet cells with intestinal-type mucin
  • Intestinal-type goblet cells required for diagnosis (foveolar columnar cells are not sufficient)

Herpes simplex virus (HSV)

• Endoscopic appearance: punched out ulcers
• Microscopic appearance: characteristic nuclear viral inclusions of HSV, degenerating epithelial cells at margin

Cytomegalovirus (CMV)

• Endoscopic appearance: shallow ulcers
• Microscopic appearance: characteristic nuclear & cytoplasmic nuclear inclusions of CMV in capillary endothelial and stromal cells

Candidiasis 

• Endoscopic appearance: adherent grey-white pseudomembranes
• Microscopic appearance: densely matted fungal hyphae & inflammatory cells covering the mucosa

Presents w/ odynophagia (pain on swallowing)

Infectious Esophagitis 

Chemical Esophagitis 

(endoscopy)

mild erythema, to mucosal sloughing, to necrosis

Chemical Esophagitis

(microscopic)

• May see dense infiltrates of neutrophils
• Chemical injury can cause necrosis of esophageal wall (may not have neutrophilic infiltrate).

Chemical Esophagitis 

(clinical presentation)

marked primarily by pain on swallowing, generally self-limited, with a history of esposure to damaging agent, radiation, etc.

Squamous Cell Carcinoma

• Early dysplastic lesions appear as small grey-white, plaque-like thickenings
• May be polypoid or ulcerated and deeply infiltrating with wall thickening and luminal narrowing.

Squamous Cell Carcinoma

(clinical presentation)

• Insidious onset of progressive dysphagia, odynophagia, and signs of obstruction
  • May have a subconscious modification of the diet from solids to softer foods and liquids
• Weight loss and debilitation may develop
• Mid-esophagus 

Adenocarcinoma

(endoscopy)

• Usually located in the distal third of the esophagus
• Early lesions may appear as flat or raised patches on the mucosa
• Later lesions develop into large masses, either polypoid or ulcerated and deeply invasive.

Adenocarcinoma

(morphology)

• Malignant gland-forming cells typical of invasive carcinoma, usually with intestinal-type morphology
  • Mucin production is common
  • Poorly differentiated tumors are less common
• Intestinal metaplasia (Barrett esophagus) is frquently detected in the mucosa adjacent to the tumor

Adenocarcinoma 

(Clinical pte)

Cannot differentiate from SCC of the esophagus until microscopic 

• Progressive dysphagia, odynophagia, and signs of obstruction
  • May have a subconscious modification of the diet from solids to softer foods and liquids
• Weight loss and debilitation may develop

3 hallmark sx of GERD

heartburn

regurgitation

dysphagia

Gold Standard for GERD dx

24-hour ambulatory esophageal pH monitoring

Functional Motility Disorder sx

dysphagia to liquids only 

(not all the case) 

Progressive Anatomical OBSTRUCTION

of esophagus 

progressive dysphagia to solids -->semi-solids -->liquids

Achalasia

Non-relaxing LES (=primary abnl)

non-contractile esophagus on manometry study

Typical bird's beak appearance on barium swallow