Esophageal Cysts 

Updated: Jan 29, 2020
Author: Dale K Mueller, MD; Chief Editor: Jeffrey C Milliken, MD 

Overview

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:

  • Simple epithelial-lined cysts
  • 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. 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.[2, 3]

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 of it causing symptoms.

Epidemiology

Esophageal cysts are rare,[4, 3]  but their true 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 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.

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.

 

Presentation

History and Physical Examination

Most esophageal cysts are diagnosed during childhood.[3]  Many children with these cysts are asymptomatic.

Most adults (67%) with esophageal cysts are symptomatic. Chest pain (tightness or fullness) is the most common presentation.[2]  Dysphagia may also occur.[2, 5]  Hematemesis can occur if gastric epithelium is present in the cyst.

Most esophageal cysts develop in the right posteroinferior mediastinum.

Malignant degeneration can occur, albeit rarely.[6]

 

Workup

Laboratory Studies

Laboratory studies do not specifically aid in the diagnosis of esophageal cysts. Laboratory evaluation should be guided by the patient's other medical problems.

Imaging Studies

Radiography guided by history and physical examination findings usually confirms the diagnosis. Plain chest radiographs can reveal a cyst within the mediastinum. Barium swallow studies reveal compression of the esophagus without ulceration.

Computed tomography (CT) reveals a fluid-filled cystic structure originating from the esophagus. CT is the radiologic modality of choice to aid in diagnosis and operative preparation (see the image below). Chest radiographic findings that suggest a mass in the mediastinum should be followed by a CT scan. Magnetic resonance imaging (MRI) can also help diagnose esophageal cysts.

CT scan of esophageal cyst demonstrated by the whi CT scan of esophageal cyst demonstrated by the white line.

Procedures

Endoscopy demonstrates extrinsic compression with intact mucosa. Esophagoscopy should be performed to rule out an intrinsic component, which should be biopsied to exclude malignancy. Endoscopic ultrasonography (EUS) reveals a cystic filled structure in connection with the esophagus. If EUS is available, then esophagoscopy should be performed to further delineate the extent of the cyst.

 

Treatment

Approach Considerations

Medical therapy has no role in the management of esophageal cysts. All esophageal cysts should be evaluated and, eventually, resected, except in those situations where the patient's other medical ailments prohibit operation. Given that nearly 75% of patients with esophageal cysts eventually become symptomatic, cysts should be resected when they are diagnosed.

The future of the treatment of esophageal cysts lies in the advancement of minimally invasive operative techniques, which will lessen morbidity and mortality. Endoscopic treatment has been reported as a feasible and reasonable alternative.[7, 8] Robotic-assisted thoracic surgery has also been used for resection of an esophageal cyst.[9]

Surgical Therapy

Simple cysts are enucleated, whereas duplications are excised.[10]  Previously, a posterolateral thoracotomy was required to remove the cyst or the duplication; however, video-assisted thoracoscopic surgery (VATS) is currently used to enucleate cysts and resect duplications, and it is the procedure of choice.[11, 2, 12, 13]

Also described in the literature is endoscopic treatment of esophageal duplications, which, essentially, creates a lumen from the cyst into the esophageal lumen.[3, 7]  Laparoscopic excision of intra-abdominal esophageal cysts has been described as well.[14, 15, 16, 17]

Preparation for surgery

Preoperative workup is based on the following two points:

  • A thorough history and a careful physical examination are important for elucidating comorbid conditions that can be addressed before the operation, thus decreasing morbidity
  • A thorough radiologic workup demonstrates the anatomy of the cyst and assists in the planning of the operation

Operative details

Before induction of anesthesia, an epidural catheter is placed for pain control. Alternatively, an ON-Q pain pump (VQ OrthoCare, Irvine, CA) can be placed at the time of operative intervention. Anesthesia is administered, and a double-lumen endotracheal tube is placed. The patient is then placed in the full lateral position.

A non-rib-spreading thoracotomy is performed (3-6 cm), with an additional Thoracoport (Tyco Healthcare, Mansfield, MA) used for visualization. Esophageal muscle fibers are carefully separated to expose the cyst. Blunt dissection is then performed to enucleate the cyst. If a duplication is present, it is excised in a similar manner. During dissection, it is important to preserve both vagus nerves and the phrenic nerves.

After the lesion is removed, the muscle layers are reapproximated, thus preventing pseudodiverticula formation. Simultaneous esophagoscopy to illuminate the esophagus assists in visualization of the mucosa. Placement of a chest tube (or tubes) follows, and the incisions are closed in standard fashion.

Alternatives to VATS include robotic-assisted thoracoscopic surgery[9] and posterolateral thoracotomy.

Postoperative Care

Most patients do well after surgical therapy, with minimal morbidity. Aggressive pulmonary toilet and early mobilization prevent pulmonary complications. Adequate analgesia is essential to patient cooperation with pulmonary toilet.

If the mucosa was not violated, the patient can be started on liquids within 1-2 days of the operation. If the mucosa was violated, then placement of a drain assists in determining the presence of a leak. Esophagography can be performed to assess esophageal integrity.

Complications

The overall complication rate is very low. Most complications are inherent to the thoracotomy or to VATS. Complications that may develop include the following[18] :

A small study by Benedict et al suggested that after resection of an esophageal duplication cyst, closing the esophageal muscle layer rather than leaving it open may reduce complications and the need for repeat operations.[19]

Long-Term Monitoring

Patients require close follow-up care. Pseudodiverticulum can develop if the muscle is not reapproximated. Complications of vagal injury develop if these nerves are not preserved. Recurrence is rare, especially if the entire cyst was excised.