Introduction

  • Gastroesophageal submucosal tumors are a heterogeneous group of tumors comprising of leiomyomas, gastrointestinal stroma tumors (GIST), or neurogenic tumors such as schwannoma or neurofibroma. Other possible differentials include congenital causes such as duplication cysts.

  • Leiomyomas account for two-thirds of benign tumors in the esophagus and stomach. One-third of these tumors are located in the gastroesophageal junction.

  • Leiomyomas are benign mesenchymal tumors and arise in the smooth muscle cells.

  • Symptomatically, gastroesophageal submucosal tumors may result in dysphagia, vague chest discomfort, reflux, occasional regurgitation, or bleeding. In most other instances, asymptomatic tumors may be diagnosed following screening gastroscopy or incidentally as mediastinal masses or abdominal masses on CT scan.

  • Operative technique for gastroesophageal include laparoscopic approach with or without fundoplication, laparoscopic transgastric approach, transthoracic approach, and combined laparoscopic and endoscopic approach.

Indications

  • Patient factors

  • Patients fit enough to tolerate general anesthesia and undergo laparoscopy; patients who require resection of the submucosal tumor via a transthoracic approach may require single lung ventilation during the procedure.

  • Disease factors

    • Symptomatic patients.

    • Giant leiomyoma measuring greater than 10 cm.

    • Submucosal lesions measuring greater than 2 cm, where histology is not available.

    • Benign submucosal tumors demonstrating an increase in size on follow-up imaging (> 1 cm/year).

    • Indeterminate histology on histology after endoscopic ultrasound (EUS) guided fine needle biopsy (FNA) has been performed.

Contraindications

  • Patient factors

    • Patients with severe comorbidities (American Society of Anesthesiologists (ASA) score IV and V).

    • Patients who are unable to tolerate laparoscopy (relative contraindication consider open surgery).

  • Disease factors

  • Small submucosal tumors measuring lesser than 2 cm.

Pre-op Assessment

  • Upper gastrointestinal gastroscopy:

    • The size and location of the lesion is recorded.

    • The relationship of the lesion to the gastroesophageal junction and lower esophageal sphincter is assessed in order to decide the operative technique.

    • In the case of leiomyoma, rounded, mobile lesions with normal overlying mucosa are observed.

  • Barium contrast swallow study is an alternative where gastroscopy is not available.

  • Endoscopic Ultrasound to assess-which layer the tumor arises from,the ultrasonic features of the lesion,-any acoustic shadowingassess the presence of any mediastinal lymph nodes.EUS—FNA can be used to biopsy the lesion.

  • CT thorax/abdomen: assessment of the lesion in relation to the gastroesophageal junction, assess for metastases in the case of malignant submucosal tumors.Leiomyomas are classically intramural and solitary on CT scan with smooth outlines and are multilobulated. The presence of calcifications is pathognomonic for leiomyomas.

OT Setup

Operation Room set up and patient positioning

  1. 1.

    The patient is placed in supine position in the reverse Trendelenburg position. Bilateral arms can be placed on arm boards if IV access is required, or they can be tucked in bilaterally (Fig. 1).

  2. 2.

    A footboard is placed, and the bilateral ankles are padded with gel pads. Crepe bandages are applied to secure the feet to the footboard as well as over bilateral knees to secure the patient.

  3. 3.

    Mechanical deep vein thrombosis prophylaxis in the form of calf compressors or TED stockings should be instituted.

  4. 4.

    The operating surgeon stands on the right of the patient and the first assistant opposite the operating surgeon. The laparoscopy stack is placed to the left of the patient at the level of the shoulder of the patient.

Fig. 1
An illustration of a patient lying down on the operating table with his stomach exposed. The surgeon and another person are on the left of the patient, on the right are the first and second assist, and above the head of the patient is a person in front of a machine.

OT set up and patient position

Surgical Technique

  • Essential Steps in Synthesis.

    • Safe entry and pneumoperitoneum creation.

    • Port placement.

    • Liver retraction for adequate visualization of the hiatus.

    • Hiatal dissection and mobilization of the distal esophagus.

    • Excision of the GEJ SMT.

    • Crural repair.

    • Fundoplication.

  • Description of the technique.

    • Abdominal access for pneumoperitoneum.

      • Pneumoperitoneum is established using the surgeon’s preferred method for abdominal entry. Options include the use of a Veress needle at the Palmar’s point, open Hasson technique, or the use of optical entry.

    • We perform optical entry using a 12 mm disposable optical entry port. Port Placement (Fig. 2).

      • The initial trocar site for pneumoperitoneum is made 1/3 the distance up between the umbilicus and the xiphoid process, about three finger breaths to the left of the midline, this corresponds to the left mid clavicular line.

      • A 12 mm port is placed to the right of the umbilicus (surgeon’s right hand) and another 5 mm port (surgeon’s left hand) is placed in the right subcostal area. Triangulation provided by these two working ports should allow the operating surgeon good access to the fundus of the stomach.

      • A 5 mm port is placed in the left subcostal area as the assistant port to aid retraction if necessary.

    • Liver retraction.

      • Liver retraction can be provided either with a Nathanson retractor/a snake retractor placed in the epigastric area or the use of OR bowel forceps to retract the liver for visualization of the hiatus.

    • Hiatal dissection and mobilization of distal esophagus.

      • Entry in the lesser sac: The lesser omentum is divided and dissection taken to the level of the right crus. Any hepatic branches of the vagi or accessory left hepatic artery should be preserved as much as possible.

      • Division of the phrenoesophageal membrane: The phrenoesophageal membrane is divided from the right crus toward the left crus.

      • Blunt dissection of the hiatus and mobilization of the esophagus: The areolar tissue between the esophagus and the diaphragmatic crus can easily be mobilized by blunt dissection or using an energy device. When dissecting the hiatus, care must be taken to avoid injury to the abdominal aorta which lies just posterior to the esophagus.

      • Creation of retroesophageal window (only required if performing posterior fundoplication or lesion is located posteriorly): Using a suction device or a grasper, a posterior esophageal window is created by bluntly dissecting the posterior adventitia tissue. Care must be taken not to injure the posterior vagus. Once the window is created, a nylon tap helps sling the esophagus and used for retraction by the assistant to facilitate exposure and dissection.

    • Excision of the GEJ SMT.

      • For submucosal tumors that are away from the GEJ along the lesser curve, wedge resection can be performed provided narrowing the GEJ can be avoided.

      • Prevention of stenosis: A orogastric Bougie inserted into the stomach helps to prevent stenosis at the gastroesophageal junction.

      • Excision of the tumor (Fig. 3):Once isolated, excision of the tumor can then be performed with a laparoscopic linear cutter stapler. When performing tumor excision using the stapler technique, a 30 mm stapler can be utilized for the first fire as it is of a smaller profile and offers better maneuverability. Subsequent stapler fires may be performed with 45 mm or 60 mm staplers.

      • In cases where a full-thickness excision of the lesion is performed with an energy device, the resultant defect is closed in a single layer using an absorbable 3/0 barbed suture run continuously.

      • Leak test: an optional leak test can then be performed using air insufflation with gastroscopy or injection of methylene blue via the orogastric bougie.

      • Where the tumor encroaches upon GEJ and the lower esophageal sphincter (LES), local excision may not be possible due to high likelihood of stenosis and injury to LES under such circumstance proximal gastrectomy should be considered.

    • Crural repair and reconstruction of the phrenoesophageal ligament.

      • Posterior crural repair: simple interrupted or figure of eight sutures using 2/0 nonabsorbable sutures like ethibond are used to approximate the left and right crus.

      • The esophagus is fixed to the central tendon of the diaphragm with absorbable suture.

    • Fundoplication.

      • In patients with GEJ SMT, excision of the lesion will result in disruption of the lower esophageal sphincter fibers thereby predisposing to reflux postoperatively. Fundoplication is performed to reduce the incidence and severity of reflux post GEJ lesion excision. A wrap commensurate to the site of excision may offer the additional advantage of mitigating the effects of leakage from the repair of the GEJ excision site.

      • Please refer to the chapter on gastroesophageal reflux disease for details on fundoplication.

    • Conclusion

      • Careful hemostasis.

      • Removal of the esophageal sling if used.

      • Removal of all laparoscopic ports under vision and evacuation of pneumoperitoneum.

      • Typically, surgical drain placement is not required.

Fig. 2
An illustration of a patient's exposed stomach. There are five points depicted that indicate port placement.

Port placement

Fig. 3
A photograph of surgically removing a tumor with a laparoscopic linear cutter stapler.

Stapling of the lesser curve submucosal tumor

Complications and Management

  • Postoperative hemorrhage.

    • Hemostasis must be observed intraoperatively and bleeding of the stapler line secured with either diathermy or suture ligation prior to ending the procedure.

    • Postoperative bleeding is usually reactive in nature and is either from cut edge of the omentum or the stapler line. A trial of conservative therapy may be attempted in hemodynamically stable patients with packed cell transfusion as necessary. Reexploration may be required if there is evidence of ongoing hemorrhage, hemodynamic instability, or low hemoglobin counts despite transfusion.

  • Leakage from the repair site.

    • Perforation and leakage resulting from the stapler line or repair site may happen infrequently (1–3%).

    • In most cases, expedient reexploration is necessary. Surgical principles include fashioning of the defect, repairing the defect, abdominal washout, and placement of drains. In cases not amenable to repair, a proximal gastrectomy and distal esophagectomy may be required.

  • Postoperative dysphagia: managed initially with conservative therapy with a liquid diet. Consider endoscopy and balloon dilatation or revision surgery if symptoms are severe and persistent.

  • Gas Bloat syndrome: conservative management and routine counseling to avoid excessive aerophagia.

Post-op Care

  • Regular and adequate analgesia is prescribed. In our unit, we typically prescribe intravenous paracetamol 1 g q.d.s. Breakthrough pain is managed with opiates on PRN basis. Aggressive prophylaxis against postoperative nausea and vomiting.

  • The patient is started on proton pump inhibitor therapy. PPI therapy can be discontinued after 4 weeks if the patient has symptoms of reflux or dyspepsia following surgery.

  • The patient is started on clear liquids on the day of surgery and liquid diet on postoperative day 1 and minced diet on postoperative day 2.

  • Patients who are clinically well and able to tolerate diet on postoperative day 2 can be safely discharged.

  • The patients were reviewed in the outpatient clinic 2 weeks and 1 month after surgery.