Practice Essentials
In 1711, Blasius initially described esophageal cysts as duplications. In 1881, Roth also described these cysts, which can be divided into the following two categories:
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Simple epithelial-lined cysts
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Esophageal duplication, which is an embryologic duplication of a portion of the muscle and submucosa of the esophagus without epithelial duplication
The term esophageal cysts applies to both categories. [1]
The diagnosis and treatment of esophageal cysts are still evolving. [2] Diagnosis is aided by the use of computed tomography (CT) and endoscopic ultrasonography (EUS). Treatment is currently moving from thoracotomy to less invasive procedures, including video-assisted thoracoscopic surgery (VATS) and endoscopic techniques. [3, 4, 5]
Anatomy
Embryologically, the upper gastrointestinal (GI) tract develops from the posterior division of the primitive foregut. During week 4 of gestation, the primitive foregut develops an anterior diverticulum, which becomes the respiratory bud. Meanwhile, the posterior division develops into the esophagus and upper GI 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 would be expected from 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.
Pathophysiology
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.
Symptoms are caused by compression of surrounding structures. Approximately 60% of esophageal cysts occur in the lower third of the esophagus, where difficulty with 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 with 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 that it will cause symptoms. [6]
Epidemiology
Esophageal cysts are rare, [7, 8] but their precise incidence is unknown. No large study has defined the true frequency of esophageal cysts in the United States or internationally. Cysts are usually grouped with other benign lesions of the esophagus, and they may account for as many as 20% of such lesions.
Many patients with esophageal cysts are asymptomatic and are never diagnosed. As many as 80% of these cysts are diagnosed in childhood; clinical descriptions of esophageal cysts in adults are comparatively uncommon. [7]
Prognosis
If the entire cyst is excised, recurrence is rare. Morbidity is low. Overall, most patients do well, both in the short term and in the long term.
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CT scan of esophageal cyst demonstrated by the white line.
