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.