Esophageal duplications cysts are rare congenital anomalies of the foregut. They are derived from the posterior part of the primitive foregut and may contain gastric, intestinal mucosal, or neural tissue. There are 3 broad categories of foregut malformations: bronchogenic cysts, enteric cysts, and tubular esophageal duplications.
Cysts comprise up to 20% of benign esophageal lesions and up to 80% of the cysts are diagnosed during childhood. Esophageal duplications cysts have a male predominance (2:1), and the most common location is on the right side of the lower third of the esophagus. Because it is a developmental anomaly, enteric cysts may be associated with other foregut or vertebral anomalies.
Esophageal duplication cysts are round, well-circumscribed lesions that are filled with fluid. Simple cysts comprise of a duplication of the epithelium, while true cysts involve duplication of the submucosa and the muscle wall.
Enteric cysts present early in life and cause symptoms from compression of surrounding structures. Secretions accumulate within the cyst and increase the size, thus causing pressure symptoms such as dysphagia, epigastric discomfort, pain, neck enlargement or respiratory difficulties.
The patient may also present with complications of the cyst due to the gastric mucosa. These complications include hemorrhage, perforation, ulceration or infection.
On the other hand, many patients are asymptomatic and may never be diagnosed.
The differential diagnosis may include bronchogenic cyst, neuroenteric cyst, cystic lymphangioma, anterior meningocele, or mature cystic teratoma.
Chest films may be helpful in showing the position of the cyst, or may reveal tracheal compression or deviation. It may also show other congenital associations, such as vertebral anomalies.
Ultrasound is useful, especially because the mass is cystic. The cysts may be adjacent to the esophagus and displace it. The mass does not usually communicate with the lesion.
The CT scan is the best imaging tool to evaluate the cyst because it can reveal the location, extent and the relationship to adjacent structures. It is useful before treatment because it may also detect complications. On CT, the cyst will appear smooth and will not enhance with contrast.
Barium exam may show displacement of the esophagus and trachea by either an intramural or extramural mass. Endoscopy will show extrinsic compression of an intact mucosa. A Meckel scan can also be utilized if the duplication contains ectopic gastric mucosa.
Treatment and Prognosis
Surgery is recommended, even for patients without symptoms because of the high probability of developing symptoms and complications in the future. These complications may be infection, hemorrhage or malignant degeneration. Surgical excision is the standard treatment, and traditionally the resection is done via thoracotomy. However, less invasive approaches such as, video-assisted thoracoscopy, should be the first-line approach to these patients. The outcome of surgery for these patients is favorable.
Jane West, Medical Student, Creighton University School of Medicine, Omaha, NE
Phillip Silberberg, Pediatric radiologist, Omaha Children’s Hospital, Creighton University and UNMC, Omaha, Nebraska
Dr. Paul Babyn, Hospital for Sick Children, Toronto, Ontario, Canada
Brandi Reeve, Medical Student, University of Nebraska Medical Center, Omaha, NE