eMedicine Specialties > Thoracic Surgery > Congenital

Esophageal Cysts

Author: Dale K Mueller, MD, Clinical Associate Professor of Surgery, Section Chief, Department of Surgery, University of Illinois College of Medicine; Co-Medical Director, Thoracic Center of Excellence, Vice-Chair, Department of Cardiovascular Medicine and Surgery, OSF St Francis Medical Center; Cardiovascular and Thoracic Surgeon, HeartCare Midwest, SC
Contributor Information and Disclosures

Updated: Jun 11, 2009

Introduction

In 1711, Blasius initially described esophageal cysts as duplications. In 1881, Roth also described these cysts, which can be divided into 2 categories. The term esophageal cysts applies to both categories.

1. Simple epithelial-lined cysts
2. Esophageal duplication, which is an embryologic duplication of a portion of the muscle and submucosa of the esophagus without epithelial duplication

History of the Procedure

The diagnosis and treatment of esophageal cysts is still evolving. Diagnosis is aided by the relatively recent developments of CT scans and endoscopic ultrasonography. Treatment is currently moving from thoracotomy to less-invasive procedures, including video-assisted thoracoscopic surgery (VATS) and endoscopic treatment.^1,2

Problem

Symptoms are caused by compression of surrounding structures. Sixty percent of esophageal cysts occur in the lower third of the esophagus, where difficulty swallowing from compression is the most common symptom; 20% occur in the upper third of the esophagus, where respiratory difficulty from compression of the tracheobronchial tree is the most common symptom; and 20% occur in the middle third of the esophagus, where retrosternal chest pain and difficulty swallowing are the most common symptoms.

Posterior cysts in the lower third of the esophagus can cause cardiac arrhythmias. The larger the cyst, the greater the chance of it causing symptoms.

Frequency

• The true incidence of esophageal cysts is unknown.
• Esophageal cysts are rare.^3,2
• Many patients with esophageal cysts are asymptomatic and never diagnosed.
• No large study has defined the true incidence of esophageal cysts in the United States or internationally.
• Cysts are usually grouped with other benign lesions of the esophagus.
• Cysts comprise up to 20% of benign esophageal lesions.
• Up to 80% of cysts are diagnosed in childhood.

Etiology

Esophageal cysts develop from aberrant elements of the esophageal wall. Simple cysts are duplication of the epithelium, whereas true esophageal duplications are duplications of the submucosa and the muscle wall without duplication of the epithelium. Maldevelopment of the posterior division of the primitive foregut is the embryologic defect responsible for esophageal cysts.

The lining of the cyst can vary and can include squamous columnar, cuboidal, pseudostratified, ciliated, and gastric mucosae. Hemorrhage can be the presenting symptom if gastric mucosa is present in the cyst. The other types of mucosa are not specifically associated with particular symptoms.

Pathophysiology

• Embryologic duplication of specific elements of the esophageal wall causes cysts.
  • Cysts are duplication of the epithelium.
  • True duplications are duplication of the muscle and submucosa.

Presentation

• Many children with esophageal cysts are asymptomatic.
• Most cysts are diagnosed during childhood.²
• Most adults (67%) with cysts are symptomatic.
  • Chest pain (tightness or fullness) is the most common presentation.¹
  • Dysphagia may also occur.¹
  • Hematemesis can occur if gastric epithelium is present in the cyst.
• Most esophageal cysts develop in the right posteroinferior mediastinum.
• Although rare, malignant degeneration can occur.⁴

Indications

• All esophageal cysts should be evaluated and, eventually, resected.
• Radiographs guided by history and physical examination findings usually confirm the diagnosis.
  
  
  • Plain chest radiographs can reveal a cyst within the mediastinum.
  • Barium swallow studies reveal compression of the esophagus without ulceration.
  • CT scan reveals a fluid-filled cystic structure originating from the esophagus.
  • Endoscopy demonstrates extrinsic compression with intact mucosa.
  • Endoscopic ultrasonography reveals a cystic, filled structure in connection with the esophagus.
• MRI scans can also help diagnose esophageal cysts.
• Nearly 75% of patients with esophageal cysts eventually become symptomatic; therefore, cysts should be resected when they are diagnosed.

Relevant Anatomy

• Embryologically, the upper gastrointestinal tract develops from the posterior division of the primitive foregut.
  • During the fourth week of gestation, the primitive foregut develops an anterior diverticulum, which becomes the respiratory bud.
  • Meanwhile, the posterior division develops into the esophagus and upper gastrointestinal tract.
  • The tracheoesophageal septum separates the primitive esophagus from the primitive trachea.
  • As the esophagus continues to develop, the epithelium eventually obliterates the lumen and later recannulizes.
• As expected based on the common embryologic origin, bronchogenic and esophageal cysts can occur together.
  • Esophageal cysts develop from aberrant elements of the esophageal wall.
  • Simple cysts are duplication of the epithelium, whereas true esophageal duplications are duplications of the submucosa and the muscle wall without duplication of the epithelium.
• Esophageal cysts and duplications do not usually communicate with the lumen of the esophagus.
• Esophageal cysts usually occur in the neck, chest, and abdomen.

Contraindications

All cysts should be resected unless the patient's other medical ailments prohibit operation.

• Search for CME/CE on This Topic »

• Esophageal Leiomyoma (Gastroenterology)