Overview
Definition:
Thoracoscopic enucleation of an esophageal leiomyoma is a minimally invasive surgical technique used to remove benign smooth muscle tumors of the esophagus, performed via a thoracoscopic approach
Leiomyomas are the most common benign esophageal tumors, arising from the muscularis propria.
Epidemiology:
Esophageal leiomyomas represent approximately 70% of all benign esophageal neoplasms, with an incidence of about 0.01%
They are most common in individuals aged 20-40 years, with a slight female predominance
Most occur in the distal esophagus (within 10 cm of the gastroesophageal junction).
Clinical Significance:
While often asymptomatic, large or symptomatic leiomyomas can cause dysphagia, odynophagia, chest pain, regurgitation, and weight loss
Early and accurate diagnosis is crucial to differentiate from malignant lesions and to manage symptoms effectively
Thoracoscopic enucleation offers the benefits of minimally invasive surgery, including reduced postoperative pain, shorter hospital stays, and faster recovery.
Clinical Presentation
Symptoms:
Dysphagia, typically for solids
Substernal chest pain
Regurgitation of food
Heartburn or reflux symptoms
Weight loss in advanced cases
Hoarseness of voice due to recurrent laryngeal nerve compression (rare).
Signs:
Often unremarkable on physical examination unless the tumor is very large
Palpable abdominal mass (extremely rare)
Vital signs usually normal.
Diagnostic Criteria:
Diagnosis is based on characteristic findings from barium esophagography, esophagoscopy, and typically confirmed by endoscopic ultrasound (EUS) or CT scan
EUS is invaluable for tumor size, location, depth, and assessment of intramural origin, helping to differentiate from other masses
Definitive diagnosis is often histological post-excision.
Diagnostic Approach
History Taking:
Detailed history of dysphagia onset, progression, and character
Associated symptoms like chest pain, regurgitation, or weight loss
Past medical and surgical history
Family history of GI malignancies
Red flags include rapid onset of symptoms, significant weight loss, or hematemesis, which might suggest malignancy or other urgent conditions.
Physical Examination:
General examination for signs of malnutrition or anemia
Abdominal examination to rule out other causes of dysphagia or masses
Cardiopulmonary examination to assess baseline function for thoracic surgery
Examination of the neck and chest for any palpable masses or lymphadenopathy.
Investigations:
Barium esophagography: smooth, intraluminal filling defect
Esophagoscopy: intrinsic submucosal lesion, often with normal overlying mucosa
biopsy can confirm submucosal origin but not definitively leiomyoma
Endoscopic Ultrasound (EUS): delineates tumor size, echogenicity (typically hypoechoic), origin from muscularis propria, and relationship to adjacent structures
essential for surgical planning
CT scan: evaluates tumor size, location, and relationship to surrounding mediastinal structures, useful for large tumors or suspected extraluminal extension
MRI: provides better soft tissue contrast than CT for large tumors
Blood tests: CBC, LFTs, RFTs, coagulation profile to assess general health and surgical fitness.
Differential Diagnosis:
Gastrointestinal stromal tumor (GIST) of the esophagus: larger, often more heterogenous on imaging, and may show increased vascularity
biopsy required for definitive diagnosis
Leiomyosarcoma: malignant counterpart, aggressive behavior
Esophageal duplication cyst
Benign strictures
Achalasia
Extrinsic compression of the esophagus
Schwannoma
Fibrovascular polyp.
Management
Initial Management:
Observation for asymptomatic, small leiomyomas
Dietary modifications (soft diet) for mild dysphagia
Medical management of reflux symptoms if present.
Medical Management:
Primarily symptomatic management for reflux
No specific medical therapy for leiomyomas themselves
Proton pump inhibitors (PPIs) for associated GERD.
Surgical Management:
Indications for surgery include symptomatic lesions (dysphagia, chest pain, regurgitation), rapidly growing tumors, or tumors larger than 5 cm
Thoracoscopic enucleation is the preferred approach for distal and mid-esophageal leiomyomas
Procedure Steps: 1
Patient positioning: lateral decubitus position
2
Port placement: typically 3-4 ports in the chest
3
Dissection: identification and mobilization of the esophagus
4
Tumor identification: precise localization of the leiomyoma
5
Incision: small mucosal incision over the tumor
6
Enucleation: meticulous dissection of the tumor from the muscularis propria using blunt and sharp dissection, often facilitated by endoscopic instruments and energy devices
Care is taken to preserve the integrity of the muscularis propria and mucosa
7
Mucosal closure: watertight closure of the mucosal defect with sutures
8
Drain placement and port site closure
Laparoscopic approach may be used for very distal tumors near the GE junction.
Supportive Care:
Postoperative pain management with analgesics
Nasogastric tube decompression if placed
Gradual reintroduction of diet, starting with clear liquids
Nutritional support if oral intake is delayed
Monitoring for complications like bleeding, leakage, or infection
Chest physiotherapy.
Complications
Early Complications:
Bleeding from the dissection site
Esophageal leak or perforation at the suture line
Pneumothorax
Surgical site infection
Injury to adjacent structures (e.g., vagus nerve, aorta)
Postoperative pneumonia
Atelectasis.
Late Complications:
Stricture formation at the site of enucleation
Recurrence of leiomyoma (rare if completely excised)
Persistent dysphagia
Gastroesophageal reflux disease (GERD).
Prevention Strategies:
Meticulous surgical technique to ensure complete tumor removal and watertight mucosal closure
Careful handling of tissues to minimize trauma
Adequate drainage
Prophylactic antibiotics
Chest physiotherapy
Early mobilization
Careful postoperative diet progression.
Prognosis
Factors Affecting Prognosis:
Complete tumor excision
Size and location of the leiomyoma
Presence of symptoms prior to surgery
Surgeon's experience with thoracoscopic procedures.
Outcomes:
Excellent outcomes are expected with complete enucleation of benign leiomyomas
Symptom relief is usually significant and rapid
Thoracoscopic approach leads to faster recovery and lower morbidity compared to open surgery.
Follow Up:
Regular follow-up is recommended, particularly with imaging (EUS or CT) at 6-12 months postoperatively to ensure no recurrence
Symptomatic patients should be monitored for persistent dysphagia or reflux
Long-term follow-up is generally not required for completely excised benign leiomyomas.
Key Points
Exam Focus:
Thoracoscopic enucleation is the gold standard for symptomatic esophageal leiomyomas
EUS is crucial for preoperative assessment
Differentiate leiomyoma from GIST and leiomyosarcoma
Key surgical steps involve meticulous dissection and watertight mucosal closure.
Clinical Pearls:
Achieving complete enucleation without damaging the mucosa is paramount
Intraoperative visualization with high-definition cameras and good lighting is essential
Consider a laparoscopic approach for very distal tumors near the GE junction to avoid a thoracotomy.
Common Mistakes:
Incomplete tumor excision leading to recurrence
Inadequate mucosal closure causing leakage or stricture
Injury to surrounding vital structures
Failure to consider malignancy in atypical cases
Overly aggressive dissection leading to esophageal wall injury.