Introduction

Esophageal cysts are rare congenital anomalies of gastrointestinal tract first described by Blasius in 1711. In 1881, Roth also described esophageal cysts and there are two categories namely simple epithelial lined cysts and esophageal duplication cysts. Esophageal duplication cysts are embryologic duplication of part of the esophageal mucosa and submucosa without epithelial duplication. The prevalence of esophageal duplication cysts is 0.0122% and accounts for 10–15% of duplication cysts in the gastrointestinal tract [1]. Esophageal cysts and duplications usually do not have communication with the lumen and they can be found in the neck, chest, and abdomen. Most of the esophageal duplication cysts (two-thirds) are found in the lower esophagus in the right posteroinferior mediastinum while 1/3 in the upper/middle third of esophagus and sometimes lesions can be found in the intra-abdominal esophagus.

Presentation

67% of esophageal duplication cysts in adults can be presented with symptoms and chest pain is the most common symptom followed by dysphagia. Some other symptoms include epigastric discomfort, vomiting, stridor, cough, bleeding, and hematemesis. Presentation of hematemesis is associated with the presence of gastric epithelium in the cyst. Rarely they can present as malignant transformation. Sometimes esophageal cysts are diagnosed incidentally.

Diagnosis

Diagnosis can be made mainly by computed tomography (CT) and endoscopic ultrasound (EUS). Fluid-filled cystic lesion arising from esophagus in CT scan usually represents the diagnosis of esophageal cysts which can be confirmed by EUS. On EUS examination, the duplication cysts appear as periesophageal homogenous hypoechoic mass with multilayered wall and well-defined margins or sometimes as anechoic cysts due to a considerable amount of central fluid. EUS-guided FNA aspiration is associated with risks of infection as high as 14% and thus EUS FNA/FNAB with appropriate antibiotics cover should be considered only when the diagnosis is in doubt or any suspicious features of malignant transformation. Esophageal cysts/duplication cysts are usually found as submucosal lesions during upper gastrointestinal endoscopy without involving the mucosa. If there is involvement of mucosa then biopsies can be taken during endoscopy.

Indications and Contraindications

Surgical removal is the treatment of choice for symptomatic patients. While asymptomatic patients can be opted for surveillance and follow up, surgical removal can also be considered due to the potential risks of complications including mucosal ulceration, bleeding, perforation, and rarely the malignant transformation. Simple cysts can be enucleated, the duplication cysts are excised. With the advancement in minimally invasive surgeries including endoscopic intervention, the outcomes are quite satisfactory and excellent. As surgical intervention is associated with long-term complications such as heartburn, reflux esophagitis, balancing the risks and benefits of surgical intervention is very important and needed to be carefully considered and counseled in asymptomatic patients without worrisome features. Endoscopic intervention by draining the cyst into the esophageal lumen or submucosal tunneling dissection can be considered for suitable patients including those with high risk for surgery.

Pre-op Assessment

Pre-op assessment is the same for those who need esophageal surgery. Those patients who need transthoracic approach will need their pulmonary function to be evaluated. Patient’s fitness for general anesthesia and surgery will also be assessed preoperatively by anesthesiologists and optimization of comorbidities by respective specialists accordingly. Pre-op optimization of nutrition and rehabilitation with pulmonary physiotherapy will also be helpful for better postoperative outcomes.

OT Setup

Except for the intra-abdominal esophageal cysts which can be approached by laparoscopy most of the esophageal cysts are approached by thoracoscopy.

For laparoscopic approach, monitor for surgeon and scopist is at the eye level on the patient’s left side and the monitor for the assistant on the right side. The energy devices are set up at the patient’s foot, suction and irrigation at the patient’s right near the head (Fig. 1).

Fig. 1
An operation theatre for endoscopy has 3 surgeons, a monitor for showing the internal part, other devices, and lighting equipment for the procedure.

OT setup for laparoscopic approach

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For thoracoscopic approach with left lateral position, monitor for the surgeon and scopist at the eye level in front of the patient (surgeon and scopist positioned at the patient’s back), while the assistant position at the front with the monitor facing him from the patient’s back.

For thoracoscopic approach with semi-prone/prone position, monitor for the surgeon and scopist at the eye level facing the patient’s back (surgeon and scopist position at the patient’s front) while the assistant stands from the patient’s back with monitor at the eye level from patient’s front.

Instrumentations

The following instruments are mostly used in the procedures.

  • 30° telescope with camera system.

  • Short bowel grasper.

  • Mary land grasper.

  • Hook.

  • Energy device (ultrasonic dissector or advanced bipolar dissector).

  • Laparoscopic needle holder.

  • Scissors.

  • Lung retractor.

  • Liver retractor.

Patient’s Position

For laparoscopic approach, patient is put in the supine position with reverse Trendelenburg and right side down position (Fig. 2).

Fig. 2
A photograph of an exposed part of a patient in a supine position illustrates covers, medical instruments, needle like instruments, pipes, and more.

Port positions for laparoscopic procedure

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For thoracoscopic left approach, patient is put in the left lateral position with both ventral and dorsal support with operating table bent at 30° at the level of pelvis to open the rib spaces as well as in the best position if open conversion is needed (Fig. 3).

Fig. 3
A photograph of an exposed part of a patient in a lateral position illustrates blankets, surgeons, and instruments for a thoracoscopic procedure.

Port positions for thoracoscopic procedure in left lateral position

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For thoracoscopic semi-prone or prone approach, the support is put under the abdomen as well as at the level of the shoulder blade in to widen the rib spaces (Fig. 4a).

Fig. 4
Two photographs illustrate; On the left, a patient in a hospital bed in a semi-prone position; On the right, the surgical procedure of a patient.

(a) Patient is prone/semi prone position. (b) Ports positions in semi prone/prone position

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Regardless of the position, it is important to make sure that patient is well supported securely, and all the presser points are supported with soft pads/cushions.

Surgical Technique

Thoracoscopic Procedures for Thoracic Esophageal Cyst

After general anesthesia with one lung ventilation either using double lumen tubes or bronchial blocker, patient is positioned in left lateral position or semi-prone/prone position as per surgeon’s preference. For left lateral position,10 mm camera port is inserted at seventh or eighth intercostal space anterior axillary line. Working ports for surgeons are 1 × 10 × 12 mm port at seventh or eighth intercostal space at posterior axillary line and 1 × 5 mm at third or fourth intercostal space mid axillary line. One assistant port either 5 or 10 mm depending on the availability of the lung retractor is inserted at fifth intercostal place ventral to the anterior axillary line (Fig. 3).

For semi-prone or prone position, 1 × 10 × 12 mm camera port at seventh intercostal space posterior axillary line. 2 × 5 mm surgeon ports one at third or fourth intercostal space posterior axillary line and one just below the tip of the scapula. 1 × 5 mm assistant port at eighth or ninth intercostal space along the scapula line (Fig. 4b).

Laparoscopic Surgery for Intra-abdominal Esophageal Cyst

After general anesthesia, patient is positioned in the supine position, 10 mm camera port is inserted infra umbilicus, the 1 × 10 mm working port is inserted at right hypochondrium along the lateral border of rectus abdominus muscle and just lateral and superior to the camera port. 1 × 5 mm working port is inserted just 3–4 finger breadth above and slightly lateral to the 10 mm working port (Fig. 5) If needed the additional one or two assistant 5 mm ports can be inserted in the left upper abdomen (Fig. 2). The Nathanson liver retractor is inserted from the small 5 mm incision at the epigastrium just below the xiphisternum.

Fig. 5
A photograph illustrates the laparoscopic procedure with three ports on the patient and some medical instruments. A monitor is behind the surgeon.

Reduced Ports(three ports) position for laparoscopic procedure

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Enucleation/Resection of Esophageal Cysts

After ports are inserted, the esophageal cyst is identified and mobilized carefully from mediastinal pleura by dissecting around the cyst using the energy device and hook diathermy. Dissection of the hiatus will be needed for intra-abdominal esophageal cysts. After dissecting the longitudinal and circular fibers of the esophageal wall, the enucleation/resection of the cyst is performed by dissecting the cyst completely off from the mucosa wall without injuring the cyst wall and mucosa. Care must also be taken not to injure the nerves during the dissection. After completion of the enucleation/resection of the cyst, on-table endoscopic examination with air sufflation under water is done to check for any mucosal injury and repair with 3/0 absorbable suture if needed. The dissected muscle fibers and pleura are then closed with 3/0 absorbable sutures to reinforce the defect as well as to prevent the pseudodiverticulum formation. After checking the hemostasis and suctioning of the fluid, the excised cyst is put into the retrieving bag and extracted by enlarging the camera port wound at the end of the surgery. For thoracoscopic procedure, one underwater seal chest tube drain is placed via one of the thoracoscopic port sites. The chest drain is kept for 1 or 2 days for post-op pneumothorax. However, drain is usually not needed for laparoscopic enucleation/resection of abdominal esophageal cyst.

Complications and Management

Even though complications are rare, the possible complications and their prevention/management include.

  • Pneumonia—managed by pre- and post-op chest physiotherapy with incentive spirometry.

  • Air leak and pneumothorax—may need a chest tube.

  • Esophageal injury and leak—adequate drainage of the collection and nasogastric tube (NGT) decompression, application of endoscopic over the scope clip/esophageal stent, or rarely the surgical intervention.

  • Injuries to vagus nerves and/or phrenic nerve—careful dissection during the excision/enucleation is very important to prevent injuries to the nerves.

  • Formation of pseudodiverticulum—it is important to suture the muscle layers to prevent pseudodiverticulum formation.

  • Bleeding—endoscopic hemostasis if intraluminal, or surgical hemostasis if bleeding did not stop spontaneously.

  • Wound infection—less with the minimally invasive approach compared with open approach.

Post-op Care

Postoperatively patients will be monitored in the ward. The chest tube if inserted can be removed in the next 1 or 2 days if there is no pneumothorax. Patient can start oral liquid the next day followed by diet if tolerating well and most of the patients can be discharged on POD (Post-operative Day) 2 or 3. Chest physiotherapy with incentive spirometry and ambulatory physiotherapy can start on POD 1. After discharge, the patient is followed up in outpatient clinic.