Our Team Combines Highly-Developed Diagnostic Skills with State-of-the-Art Imaging and Functional Testing to Diagnose Diseases of the Esophagus.

Our multi-disciplinary team offers an integrated, comprehensive approach and minimally invasive surgery to treat esophageal problems, including:

  • Achalasia
  • Barrett’s esophagus
  • Esophageal cancer
  • Esophageal diverticula
  • Esophageal stricture
  • Esophageal web
  • Esophagitis
  • Gastro-esophageal reflux disease (GERD)
  • Hiatal hernia
  • Zenker’s diverticulum

Types of Benign Esophageal Disease

Esophageal strictures are caused by a problem of the esophagus or compression from the outside. The patient’s symptoms, physical examination, contrast radiographic imaging, endoscopy, and pathology will help us determine the diagnosis.

According to Ferguson, strictures are classified as:
(Evaluation and management of benign esophageal strictures. Ferguson DD. Dis Esophagus. 2005;18(6):359-64.)

  • Simple (not too narrow so the esophagoscope can pass through the narrowing, straight, and short, <2cm)
  • Complex (too narrow so the esophagoscope can pass through the narrowing, angulated, and irregular)

ETIOLOGY

  • Acid from the stomach refluxes to the esophagus to cause scarring in the esophagus.
  • A weak lower esophageal sphincter (LES) which should work as a one way valve to allow the passage of food, but prevent the reflux of acid from the stomach back to the esophagus.
  • Hiatal hernia (the opening in the diaphragm through which the esophagus is too large).

A diverticulum is an outpuching or sac arising from the wall of the esophagus which can contain one or more layers of the wall. Diverticula can be classified based on:

HISTOLOGY

  • Three general types of diverticula exist differentiated by the number of intestinal wall layers involved
    • True DiverticulaContain all layers of the wall
    • False Diverticula (pseudodiverticula)Contain only mucosa and submucosa
    • Intramural Diverticula (pseudodiverticula)Outpouching within the submucosal layer secondary to dilated submucosal excretory ducts

ETIOLOGY

  • Acquired
    • Pulsion Diverticula
      • Secondary to increased intraluminal pressure at a weak point in the wall of the esophagus secondary to either anatomic or functional disturbance. There is a frequent association with esophageal motility disorders such as diffuse esophageal spasm and achalasia.
        • Zenker’s diverticulum
        • Epiphrenic diverticulum
  • TractionDiverticula
    • Secondary to extraluminal traction / pulling on the outside wall of the esophagus secondary to an inflammatory process and fibrosis such as Histoplasmosis or Tuberculosis
      • Midthoracic – Parabronchial location
  • Intramural Diverticula (pseudodiverticula)
    • Esophageal submucosal glands which are in communication with the esophageal lumen become dilated.
    • Associated with inflammation and thickening of the esophagus, but the pathogenesis of this process is uncertain.
    • Possible etiology may be dilation of the submucosal glands secondary to obstruction by inflammation and debris.
    • Stasis and inflammation may also play a role in this disease.
  • Very often there is associated esophageal dysmotility and/or strictures within the body of the esophagus.
  • May be a connection with history of corrosive esophageal injury
  • Congenital
  • Present at birth

ETIOLOGY

  • Originate from the primitive foregut
    • The defect responsible for esophageal cysts is the failure of proper development of the posterior division of the primitive foregut.
  • Esophageal cysts are the second most common benign lesion of the esophagus
  • The cysts are comprised of
    • Acquired epithelial cysts
      • A minority of the cysts
    • Congenital Foregut Cyst
      • Represent majority of cases
      • Lined by squamous, respiratory or columnar epithelium, may contain smooth muscle, cartilage or fat
  • Esophageal duplication cyst
    • A type of congenital foregut cyst
    • Lined by squamous epithelium
    • May contain gastric mucosa
  • Can be attached either to the esophagus or to the tracheobronchial tree.
  • Develop independently from the native esophagus.
  • Usually does not have continuity with the native esophagus.
  • May be associated with other congenital malformations.
    • Vertebral abnormalities
    • Spinal cord abnormalities
    • Tracheoesophageal fistula
    • Esophageal atresia distal to the duplication
    • Other gastrointestinal duplication’s, more often small bowel

CLINICAL

  • Most are diagnosed in childhood, as most children are symptomatic.
  • May present in the first year of life with respiratory compromise secondary to mass effect from the cyst.
  • Cysts may become symptomatic in adulthood:
    • Usually located in the right posterior mediastinum.
    • Chest pain or chest discomfort is most common.
    • Cough, stridor, tachypnea and other respiratory complaints due to compression from mass.
    • Hematemesis can occur if there is gastric epithelium within the cyst.
    • Dysphagia may occur.
    • Cardiac arrhythmias may occur due to compression.
    • Other complications may include:
      • Infarction
      • Rupture
      • Dysplasia and malignant degeneration 60, 61

DIAGNOSIS

  • History and appropriate imaging will usually result in the diagnosis.
  • Imaging:
    • Chest X-Ray:
      • May demonstrate a soft tissue mass or a cystic structure within the mediastinum with possible shift.
    • Barium swallow:
      • May show esophageal compression.
      • May detect tubular esophageal duplication if it has communication with the esophagus.
      • It may miss an esophageal cyst with no communication with the esophageal lumen.
  • Computed Tomography Scan (CT):
    • The study of choice
    • Allows for diagnosis
    • Usually identify a cystic fluid filled mass intimately related to the esophagus.
    • Helps aid in operative preparation, by delineating the anatomy of the mass and its surrounding structures prior to surgical resection.
  • Technetium scan:
    • The addition of a nuclear (technetium) scan may help identify ectopic gastric mucosa within the cyst.
  • Magnetic Resonance Imaging (MRI):
    • May be helpful in diagnosing esophageal cysts.
  • Endoscopy demonstrates extrinsic compression with intact mucosa.
  • Endoscopic ultrasonography reveals a cystic, filled structure in connection with the esophagus.

PROCEDURES

  • Esophagoscopy:
    • Need to rule out intraluminal pathology.
    • If any lesion or abnormality is seen, it should be biopsied to evaluate for possible malignancy.
  • Endoscopic Ultrasound (EUS):
    • Help delineate the intramural or extraesophageal extent of the cyst.
    • Distinguish between solid and cystic masses.

TREATMENT

  • Removal of all discovered cysts is advocated, as most will eventually be symptomatic by adulthood.
  • Definitive treatment involves complete surgical resection of the duplication, even for asymptomatic cysts.
  • Simple cysts may be enucleated
  • Duplications need to be excised.
  • Traditionally a posterolateral thoracotomy was employed for access to the lesion
  • Currently, with the advent of minimally invasive techniques, the use of video-assisted thoracoscopic surgery (VATS) has become the procedure of choice in the approach to these lesions.
  • Transesophageal endoscopic drainage has been described, but this procedure does not address the lining of the cyst and the recurrence rate is high.
Symptoms

Sign and Symptoms

Most patients with Barrett’s esophagus have a history of heartburn and acid regurgitation.

  • Less frequent symptoms include dysphagia
    • Chest pain
    • Hematemesis
    • Melena
    • Cough
    • Wheezing
  • No symptoms specific for Barrett’s
Risk Factors

Causes of Non-Cancerous Strictures of the Esophagus

  • Peptic stricture due to stomach acid
  • Schatzki’s ring
  • Motility disorder of esophagus
  • Autoimmune diseases (scleroderma, lupus)
  • Immunocompromise
  • Collagen vascular disease
  • Crohn’s disease
  • Infectious esophagitis,
  • Hiatal hernia
  • Caustic (swallowing toxic liquid)
  • Congenital (born with it)
  • Caused by medicines
  • Reaction to foreign body reaction
  • Radiation therapy
  • Cancer
  • Unknown

Clinical Features

  • Risk factors for the development of Barrett’s esophagus are:
    • Male sex
    • Smoking history
    • Obesity
    • Caucasian ethnicity
    • Age > 50
    • Greater than 5-year history of reflux symptoms
    • Esophageal motility in Barrett’s
    • Weak lower esophageal sphincter allowing for pathologic reflux to occur.
    • Esophageal peristalsis often impaired, exacerbating the delay in acid clearance from the distal esophagus.
    • Chronic inflammation and fibrosis may lead to esophageal stricture, frequently at the proximal end of the involved segment.
Diagnosis

Benign Esophageal Disease Diagnosis

Diagnosis and cause should be established because treatment depends on etiology. Dysphagia is the most common symptom. Frequently, patients will describe a progression of dysphagia from solids to liquids. The presence of dysphagia should prompt an investigative workup to find the cause behind it.

Suggested by finding abnormal columnar epithelium lining the distal esophagus on endoscopy, but must be confirmed by biopsy showing intestinal metaplasia usually with the hallmark presence of mucin-producing goblet cells.

  • One must remember that shorter segments or even just tongues of columnar epithelium may be associated with adenocarcinoma of the gastro-esophageal junction.
    • Traditionally, the diagnosis of Barrett’s was based on the finding of long segments (≥ 3 cm) of columnar epithelium, but intestinal metaplasia has been identified short segments of columnar epithelium, even in the absence of GERD.

Barrett’s is classified as follows:

  • Long Segment Barrett’s (≥ 3 cm of specialized intestinal metaplasia)
  • Short Segment Barrett’s (≤ 3 cm of specialized intestinal metaplasia)

  • Pharyngeal – Zenker’s
    • Clinical
      • Aspiration pneumonia
      • Halitosis
      • Regurgitation
      • Dysphagia, ranging from globus sensation to obstruction
      • Bleeding
      • Perforation
      • May develop carcinoma within the pouch
    • Physical findings
      • Neck mass if the diverticulum is large
    • Imaging
      • Barium swallow
        • Visualized in the neck / cervical region
        • Lateral views demonstrate the extent of the Zenker’s
      • Computed tomography (CT) scan
        • May show air or fluid filled structure in communication with esophageal lumen
        • Allows for evaluation of surrounding structures in relation to the diverticulum
      • Procedures
        • Esophagogastroduodenoscopy (EGD)
          • Endoscopy is not always necessary in Zenker’s if the diagnosis has already been made by barium swallow.
          • If EGD is to be done, caution is warranted
            • The true esophageal lumen is smaller than the lumen of the Zenker’s
            • To decrease the risk of injury and possible perforation the scope should only be inserted gently and guided in under direct visualization.

  • Midthoracic – Parabronchial
    • Clinical
      • Usually asymptomatic
      • Dysphagia is the most common symptom, but it is often due to an underlying motility disorder rather than the diverticulum itself.
      • Regurgitation
      • Aspiration pneumonia
      • May develop obstruction
      • Bezoar formation has been described
      • May develop carcinoma within the pouch
    • Physical findings
      • Often normal
    • Imaging
      • Barium swallow
        • Visualized in the chest
        • Usually small in size
        • Pouch will usually have a broad base with a wide mouthed pouch
        • Usually located near the tracheal bifurcation
      • Computed tomography (CT) scan may be done,
        • Will show air or fluid filled structure in communication with esophageal lumen
        • Allows for identification of enlarged or calcified mediastinal nodes
        • Allows for evaluation of surrounding mediastinal structures in relation to the diverticulum
      • Procedures
      • Esophagogastroduodenoscopy (EGD)
        • Allows for visual inspection of the esophagus
        • Will help in identifying any erosion or other endoluminal pathology that may be associated with or the cause of the diverticulum.
        • Helpful in evaluation the pouch and ruling out a carcinoma within the diverticulum.
      • Esophageal manometry
        • Helpful in diagnosing an esophageal motility disorder that may be present.
        • Esophageal motility disorder may be the underlying cause of the diverticulum.
        • Manometry is useful in planning for surgical intervention.

  • Epiphrenic
    • Clinical
      • Usually asymptomatic
      • Dysphagia is the most common symptom, but it is often due to an underlying motility disorder, rather than the diverticulum itself.
      • Regurgitation
      • Aspiration pneumonia
      • May develop obstruction
      • Bezoar formation has been described
      • May develop carcinoma within the pouch
    • Physical findings
      • Often normal
    • Imaging:
      • Barium swallow
        • Visualized near the diaphragm
        • Usually a large globular pouch
        • May see abnormal esophageal contractions
      • Computed tomography (CT) scan may be done
        • Will show air or fluid filled structure in communication with esophageal lumen.
        • Allows for evaluation of surrounding structures in relation to the diverticulum.
    • Procedures:
      • Esophagogastroduodenoscopy (EGD)
        • Allows for Visual inspection of the esophagus.
        • Will help in identifying any erosion or other endoluminal pathology that may be associated with or the cause of the diverticulum.
        • Helpful in evaluation the pouch and ruling out a carcinoma within the diverticulum.
      • Esophageal manometry
        • Helpful in diagnosing an esophageal motility disorder that may be present.
          • Esophageal motility disorder may be the underlying cause of the diverticulum.
          • Manometry is useful in planning for surgical intervention.

  • Clinical:
    • Dysphagia is the most common symptom.
    • May have esophageal dysmotility.
    • May be associated with esophageal stricture.
  • Physical findings:
    • Often normal
  • Imaging:
    • Barium swallow
      • May be segmental or diffusely distributed throughout the esophagus.
      • Will see numerous small diverticula lining the esophageal wall.
    • Procedures:
      • Esophagogastroduodenoscopy (EGD)
        • Openings of the small diverticula are usually not visible on endoscopy. Intramural pseudodiverticulosis

Treatment

Benign Esophageal Disease Treatment

The goal of treatment is to relieve the dysphagia. Initial therapy is usually esophageal dilatation, but concomitant implementation of medical therapy is essential to promote healing as well as decrease the chance of recurrence. Esophageal resection may be necessary for patients refractory to the above treatments.

Meeting about benign esophageal disease