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Thoracic Surgery > Congenital
Esophageal Cysts
Article Last Updated: Sep 17, 2007
AUTHOR AND EDITOR INFORMATION
Section 1 of 12  
Author: Dale K Mueller, MD, Associate Professor of Surgery, Section Chief, Department of Surgery, University of Illinois at Peoria; Co-Medical Director, Thoracic Center of Excellence, Vice-Chair, Department of Cardiovascular Medicine and Surgery, OSF St Francis Medical Center
Dale K Mueller is a member of the following medical societies: American College of Chest Physicians, American College of Surgeons, American Medical Association, Chicago Medical Society, Illinois State Medical Society, and Society of Thoracic Surgeons
Coauthor(s):
Christian Birkedal, MD, Clinical Associate Professor of Surgery, Florida State University College of Medicine; Medical Director, Wound Treatment Center, Capital Regional Medical Center and Tallahassee Memorial Hospital;
J Thomas Williams, MD, Associate Program Director, Consulting Surgeon, Department of Surgery, Division of Thoracic Surgery, Baptist Health System Princeton Medical Center
Editors: Jeffrey C Milliken, MD, Chief, Division of Cardiothoracic Surgery, University of California at Irvine Medical Center; Clinical Professor, Department of Surgery, University of California at Irvine School of Medicine; Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine; Daniel S Schwartz, MD, FACS, Clinical Assistant Professor of Cardiothoracic Surgery, New York University School of Medicine; Consulting Staff, Department of Surgery, Division of Thoracic Surgery, North Shore University Hospital/Long Island Jewish Medical Center; Alex J Mechaber, MD, FACP, Assistant Dean for Medical Curriculum, Associate Professor of Medicine, Division of General Internal Medicine, University of Miami Miller School of Medicine; Mary C Mancini, MD, PhD, Director of Cardiothoracic Transplantation, Professor, Department of Surgery, Louisiana State University Health Sciences Center
Author and Editor Disclosure
Synonyms and related keywords:
esophageal cyst, cyst resection, esophageal duplication, thoracotomy, thoracoscopy, esophagoscopy, pseudodiverticula
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.
- 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
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.
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.
- 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.
Clinical
- Many children with esophageal cysts are asymptomatic.
- Most cysts are diagnosed during childhood.
- Most adults (67%) with cysts are symptomatic.
- Chest pain (tightness or fullness) is the most common presentation.
- Dysphagia may also occur.
- 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.
- 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.
- 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.
All cysts should be resected unless the patient's other medical ailments prohibit operation.
Lab 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
- CT scan is the radiologic modality of choice to aid in diagnosis and operative preparation (see Image 1).
- Chest radiographic findings that suggest a mass in the mediastinum should be followed by a CT scan.
- See Indications.
Diagnostic Procedures
- Esophagoscopy should be performed to rule out an intrinsic component, which should be biopsied to exclude malignancy.
- If endoscopic ultrasonography is available, then esophagoscopy should be performed to further delineate the extent of the cyst.
- See Indications.
Medical Therapy
Medical therapy has no role in the management of esophageal cysts.
Surgical Therapy
- Simple cysts are enucleated, whereas duplications are excised.
- 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. Also described in the literature is endoscopic treatment of esophageal duplications, which, essentially, create a lumen from the cyst into the esophageal lumen.
Preoperative Details
Preoperative workup should focus on 2 facets. - A thorough history and physical examination is important to elucidate comorbid conditions that can be addressed prior to the operation, thus decreasing morbidity.
- A thorough radiological workup demonstrates the anatomy of the cyst and assists in the planning of the operation.
Intraoperative Details
- Prior to induction of anesthesia, an epidural catheter is placed for pain control. Alternatively, an ON-Q pain pump (VQ OrthoCare, Irvine, Calif) 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, Mass) used for visualization.
- Esophageal muscle fibers are carefully separated to expose the cyst.
- Blunt dissection is then used to enucleate the cyst.
- If a duplication is present, it is excised in a similar manner.
- During dissection, 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.
- Chest tube(s) is placed, and the incisions are closed in standard fashion.
- An alternative to VATS includes a posterolateral thoracotomy with the addition of the above steps.
Postoperative Details
- Most patients do well, with minimal morbidity.
- Aggressive pulmonary toilet and early mobilization prevents 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. An esophagram can be used to assess esophageal integrity.
Follow-up
- 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.
- If the entire cyst is excised, recurrence is rare.
- The morbidity rate is low.
- Overall, most patients do well in both the short and long term.
The future of the treatment of esophageal cysts lies in the advancement of minimally invasive operative techniques, which will lessen morbidity and mortality rates. Endoscopic treatment has been reported as a feasible and reasonable alternative. Recently, robotic-assisted thoracic surgery has also been used for resection of an esophageal cyst.
| Media file 1:
CT scan of esophageal cyst demonstrated by the white line. |
 |  View Full Size Image | |
Media type: CT
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Esophageal Cysts excerpt Article Last Updated: Sep 17, 2007
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