Esophageal duplication cysts are a type of congenital foregut duplication cyst.
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Epidemiology
Less common compared to other foregut duplication cysts. There may be an increased male predilection 5.
Clinical presentation
Esophageal duplication cysts typically present in childhood, can occur anywhere along the esophagus, and are usually asymptomatic. The majority (about 90%) do not communicate with the oesopageal lumen. More than half occur in the lower third of the esophagus 4, where they can cause dysphagia due to compression. There have also been reports of lower esophageal duplication cysts causing cardiac arrhythmias. Esophageal cysts occuring in the upper two thirds of the esophagus can cause upper respiratory symptoms (e.g. cough, wheezing, stridor) and resrosternal chest pain.
Pathology
They are a congenital malformation of the posterior primitive foregut and result from an aberration of the posterior division of the embryonic foregut at 3-4 weeks gestation. They comprise two outer muscle layers and an inner epithelial lining which is variable and can include stratified squamous, simple columnar, pseudostratified columnar, cuboidal, and ciliated epithelium; there are even reports of intermixed heterotopic gastric or pancreatic mucosa.
Associations
Radiographic features
Plain radiograph
Well-defined soft tissue density in close association with the esophagus.
Fluoroscopy
On barium swallow, the cyst may cause extrinsic compression of the esophagus.
CT
Well-defined structure with internal fluid density along the esophagus. Its wall may be slightly thicker than a bronchogenic cyst.
MRI
T1: low to intermediate signal intensity
T2: high signal intensity
Treatment and prognosis
Surgical resection is the mainstay of management 8. Prognosis tends to be very good as recurrence is rare 8.
Complications
Recognized complications include carcinoma arising within the cyst 5. If gastric mucosa is present, peptic ulceration may occur. Very occasionally they may perforate, hemorrhage or erode into adjacent structures.
Differential diagnosis
For uncomplicated cysts consider:
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congenital cysts and malformations in the region
cervical lymphadenopathy
pancreatic pseudocyst: for retroperitoneal bronchogenic cysts or for pancreatic pseudocysts that extend intrathoracically through the aortic or esophageal hiatus
For complicated cysts (e.g. with hemorrhage/necrosis), the differential can be broader and can include:
abscess(es)
enlarged lymph nodes (especially centrally necrotic)
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pulmonary masses
hematoma
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