Esophageal Cysts - A Congenital Abnormal Growth

Introduction:

Esophageal cysts are congenital cysts arising from the esophagus, which is a part of the digestive tract. These are often diagnosed in childhood but may also be found in adults. Esophageal cysts can be asymptomatic or present with symptoms such as dysphagia, respiratory distress, and chest pain. Esophageal cysts were first described by Blasius in 1711. The cause of these cysts is not clearly known. Esophageal cysts are rare malformations as their incidence is estimated to be one in 8200 live births. About 80 % of esophageal cysts are diagnosed in childhood. If the esophageal cyst is excised entirely, the recurrence is rare but has been reported, and morbidity is low.

What Are Esophageal Cysts?

Esophageal cysts are rare congenital abnormal growth originating in the esophagus. These cysts are often diagnosed in childhood but are also seen in adults. The etiology of esophageal cysts is clearly not known. The development of esophageal cysts is believed to be a result of the failure of vacuolization and budding of the primitive esophagus between weeks four to eight of gestation. Esophageal cysts are more common on the right lateral aspect of the esophagus. In 1881, Roth divided esophageal cysts into two categories:

• Simple-epithelial lined cysts.

• Esophageal duplication cysts.

Esophageal duplication cysts have a double layer of smooth muscle surrounding them and are either attached or located within the esophageal wall.

What Are the Signs and Symptoms of Esophageal Cysts?

Esophageal cysts are asymptomatic in many children. The signs and symptoms usually seen in adults are:

• Chest pain (fullness or tightness).

• Dysphagia (difficulty swallowing).

• Respiratory distress.

• Failure to thrive.

• Vomiting.

• Hematemesis (vomiting blood) when there is a presence of ectopic gastric epithelium.

What Are the Criteria for the Classification of Esophageal Cysts?

• The esophageal cyst is within the esophageal wall or attached to the wall.

• The lining of the cyst is the squamous, cuboidal, columnar, pseudostratified, or ciliated epithelium.

• The two muscle layers cover the cyst.

How to Diagnose Esophageal Cysts?

The diagnosis of esophageal cysts is made through history, physical examination, specific histopathologic characteristics, and imaging or diagnostic procedures.

• Chest X-Rays - This test uses a small dose of radiation to create black-and-white images of the chest. They reveal a cyst within the mediastinum.

• Barium Swallow Studies - It is an imaging test in which the patient swallows a chalky substance called barium, and it looks distinct on an X-ray. It reveals compression of the esophagus without ulceration.

• CT (Computed Tomography) Scan - This scan reveals a fluid-filled cystic structure arising from the esophagus.

• MRI (Magnetic Resonance Imaging) Scan - This scan also helps in the diagnosis of esophageal cysts by showing further anatomic details.

• Endoscopic Ultrasound - It is a test that uses sound waves to generate images of the gastrointestinal tract and nearby tissues. It is the choice of the diagnostic test to evaluate the esophageal cysts. It reveals a cystic-filled structure originating from the esophagus.

What Is the Differential Diagnosis of Esophageal Cysts?

• Bronchogenic Cysts - These are the congenital abnormal growths of tissue that form in the area of the chest cavity that separates the lungs (mediastinum).

• Lipoma - A lipoma is a slow-growing, round, or oval-shaped fatty lump of tissue that grows between the skin and the underlying muscle layer.

• Neurenteric Cysts - These are the rare benign endodermal growth of the central nervous system, most frequently occurring in the spinal cord.

• Thyroglossal Duct Cyst - A thyroglossal duct cysts are a rare type of cyst or lump filled with fluid that develops in the neck or near the thyroid glands.

• Cervical Duplication Cyst - These are rare congenital anomalies and are causes of upper airway obstruction.

• Lymphangioma - These are benign fluid-filled cysts of the lymphatic system often formed in children in the head and neck region.

• Anterior Meningocele - These are congenital defects characterized as small moist cyst that protrudes through the gap in the anterior aspect of the spine.

• Pericardial Cyst - It is a benign congenital anomaly characterized by abnormal growths in the pericardium, which is the fluid-filled sac surrounding the heart.

• Hemangioma - It is a common bright red birthmark made up of extra blood vessels in the skin.

What Are the Treatment Options for Esophageal Cysts?

• All esophageal cysts should be assessed and eventually resected surgically, except in those situations where the patient's medical conditions do not allow for surgery. Surgical removal of the esophageal cyst is the choice of treatment in most symptomatic cases. Medical therapy has no role in the treatment of esophageal cysts.

• The preferred mode of treatment for simple cysts is enucleation, and for duplications, it is excision. The minimally invasive operative techniques for the removal of esophageal cysts have less morbidity and mortality rates. Endoscopic treatment has proven to be a reasonable and feasible alternative.

• Traditionally, posterolateral thoracoscopic surgery was performed for the removal of the cyst. Currently, video-assisted thoracoscopic surgery (VATS) is used to enucleate cysts and resect duplications. Robotic-assisted thoracic surgery is also used for the surgical removal of the esophageal cyst.

• Another option for the excision of the cyst is endoscopic submucosal tunnel dissection (ESTD), which is less invasive. Laparoscopic resection is a common method for the removal of esophageal cysts that are continuous with the mediastinum and abdominal cavity.

• In cases of esophageal cysts being asymptomatic, there are no clear guidelines on whether the treatment is needed or not. Surgery can be an option to avoid cases of perforation, ulceration, and rare instances of malignancy.

What Are the Complications of Esophageal Cysts?

The overall complication rate of esophageal cysts is very less. The complications that may develop include:

• Heartburn.

• Reflux esophagitis (inflammation of esophageal mucosa that occurs secondary to gastroesophageal reflux disease).

• Rupture of the esophagus.

• Obstruction in the esophagus.

• Hemorrhage.

• Wound infection.

• Pneumonia (an infection causing the inflammation of the air sacs of one or both lungs).

• Malignancy (cancerous abnormal growth of the cells).

• Deep venous thrombosis (blood clot formation in the deep veins of the body, often in the legs).

• Vagus nerve paralysis (paralysis of the tenth cranial nerve known as vagus).

• Esophageal leak (the contents of the esophagus pass into the surrounding area) or pseudodiverticulum (outpouching of the mucosa of the esophagus).

• Persistent air leak.

Conclusion:

Esophageal cysts, after treatment with full surgical enucleation or excision, have a good prognosis. Overall, most patients do well in both the short-term and long-term. Patients and family members should be educated about recognizing the symptoms associated with esophageal cysts. Patients should also be educated about the possible complications if undergoing surgical intervention, as patient education is paramount.