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Etiology and Management of Esophageal Perforation & Leaks [SKF 48& 49]

Etiology

  • Esophageal perforation: A serious, life-threatening emergency requiring prompt recognition and treatment.

Common Causes

  1. Iatrogenic Injury (∼60%):
    • Resulting from esophageal instrumentation (e.g., flexible endoscopy).
    • Often recognized immediately with minimal contamination due to empty stomach.
  2. Boerhaave Syndrome (∼15%):
    • Spontaneous perforation due to sudden increase in intraluminal pressure during forceful vomiting.
    • Typically involves the left thoracic or abdominal esophagus.
  3. Other Causes:
    • Foreign body ingestion.
    • Blunt or penetrating trauma.
    • Iatrogenic operative injury.
    • Tumor-related perforation (including necrosis after radiation or chemotherapy).

Presentation

  • Symptoms vary based on:
    • Etiology, size, and location of perforation.
    • Associated esophageal pathology.
    • Time since perforation.
    • Extent of contamination.
    • Patient comorbidities.

Common Symptoms

  • Chest pain (most common).
  • Odynophagia (painful swallowing).
  • Neck or abdominal pain.
  • Dyspnea (shortness of breath).
  • Crepitus (crackling sensation under the skin).
  • Fever and chills.

Progression

  • Within 8 to 24 hours:
    • Development of sepsis: tachycardia, hypotension, altered mental status, respiratory failure.

Clinical Considerations

  • High index of suspicion in patients with:
    • Severe upper GI symptoms after vomiting.
    • Recent esophageal instrumentation.
  • History taking should focus on:
    • Preexisting dysphagia, heartburn, regurgitation.
    • Prior esophageal surgery or known disorders.

Diagnosis

Initial Assessment

  • Upright chest radiograph:
    • Quick, available, inexpensive.
    • May show pleural effusion, pneumothorax, pneumoperitoneum, subcutaneous or mediastinal emphysema, mediastinal widening.
    • Normal X-ray does not rule out perforation.

Contrast Esophagram

  • Water-soluble contrast agent (e.g., Gastrografin) used first.
    • Risk of aspiration pneumonitis if aspirated.
  • Thin barium swallow if initial study is negative.
    • Increases sensitivity.
  • Positioning: Films taken in left and right lateral decubitus positions.
  • False-negative rate: 10% to 38%.

Computed Tomography (CT)

  • Useful for:
    • Detecting pneumomediastinum, pneumoperitoneum, subcutaneous emphysema, mediastinal fluid, pleural effusion, abdominal abscess.
    • Assessing location and size of fluid collections needing drainage.
  • Limitation: Not always sensitive in locating exact perforation site.

Flexible Fiberoptic Upper Endoscopy

  • Advantages:
    • Identifies location and size of mucosal injury.
    • Detects concomitant esophageal pathology (e.g., strictures, malignancies).
  • Considerations:
    • Requires minimal insufflation to reduce risk.
    • Chest tube placement prior if pneumothorax risk exists.
  • Limitations:
    • Invasive, requires sedation.
    • May exacerbate pneumothorax or pneumoperitoneum.

Diagnostic Approach

  • No single study is definitive.
  • Combination of studies often required.
  • Clinical judgment is crucial to:
    • Determine the sequence of studies.
    • Assess patient comorbidities and performance status.
    • Plan for possible therapeutic interventions.
  • Preoperative determination of perforation location and contamination extent is essential.

Treatment

Principles of Initial Management

  • Primary threats: Sepsis and death from leakage of enteric contents.
  • Key Interventions:
    • Timely administration of appropriate systemic antibiotics.
    • Elimination of infection source:
      • Repairing, occluding, diverting, or exteriorizing the leak.
    • Adequate drainage of extraluminal fluid collections.
    • Provision of nutritional support.
  • Initial Steps:
    • Nil per os (NPO): No oral intake.
    • Intravenous fluids to manage hypotension.
    • Broad-spectrum antibiotics covering:
      • Gram-positive, gram-negative, anaerobic bacteria, and fungi.
    • Antifungal therapy for patients with recent proton pump inhibitor use.
  • Early closed tube thoracostomy:
    • Considered to drain large pleural effusions or treat pneumothorax before definitive intervention.

Nonoperative Therapy

Selection Criteria (Altorjay Criteria)

  • Small, contained leak with minimal extraluminal contamination.
  • Contrast drains back into the esophageal lumen on esophagram.
  • Minimal symptoms.
  • Minimal signs of sepsis.
  • Absence of esophageal pathology:
    • No malignancy or distal obstruction (e.g., stricture) that impairs healing.

Management Strategy

  • Intravenous antibiotics.
  • NPO status with close observation.
  • Duration dictated by clinical course.
  • Follow-up imaging or endoscopy to:
    • Confirm resolution of perforation.
    • Determine timing for resuming oral intake.
  • Monitoring for clinical decline:
    • Development of sepsis requires reconsideration of more invasive interventions.

Endoscopic Management

  • Advances in endoscopic technologies have introduced novel techniques for managing esophageal perforations.

Endoscopic Modalities

  • Endoluminal Suturing (OverStitch)
  • Through-the-Scope (TTS) Clips
  • Over-the-Scope Clips (OTSC System)
  • Endoscopic Vacuum Therapy (EVT)
  • Covered Esophageal Stents

These modalities can be used alone or combined, serving as primary therapy or salvage after failed surgical repair.

Esophageal Stents

  • Historical Background:
    • First successful stent placement by Symonds in 1887 using ivory and silver prosthesis.
    • Rigid stents were initially used for palliation in obstructing esophageal neoplasms but were difficult to place and extract.
  • Self-Expanding Metallic Stents (SEMSs):
    • Introduced in the 1990s.
    • Made from alloys like nitinol (nickel-titanium) or elgiloy (cobalt-nickel-chromium).
    • Advantages:
      • Can be placed using flexible upper endoscopy.
      • Require smaller esophageal lumen for insertion.
      • Self-expand to predetermined diameter upon deployment.
  • Fully Covered SEMSs and Hybrid Stents:
    • Allow for later extraction, broadening indications to nonmalignant conditions like strictures and perforations.
    • Fully covered to prevent tissue ingrowth, facilitating easier removal.

Considerations with Stenting

  • Extraluminal Contamination:
    • Must be addressed with appropriate drainage methods:
      • Chest tubes or CT-guided pigtail catheters for small collections.
      • Thoracotomy or Video-Assisted Thoracic Surgery (VATS) for extensive contamination.
  • Perforations Not Amenable to Stenting:
    • High cervical esophagus (stent causes discomfort in pharynx).
    • Spanning gastroesophageal junction (difficult to occlude due to gastric cardia shape).
    • Dilated esophagus in conditions like achalasia.

Complications of Stents

  • Recurrent Leakage
  • Stent Migration
  • Luminal Obstruction
  • Erosion
  • Pain and Bleeding
  • Inextractability
  • Timing of Stent Removal:
    • Requires clinical judgment to balance healing and risk of complications.

Endoscopic Vacuum Therapy (EVT)

  • Mechanism:
    • Applies negative pressure via a nasoesophageal tube-mounted sponge connected to a vacuum device.
    • Custom setup as no commercial system exists.
  • Advantages:
    • Controls extraluminal fluid collections without additional drainage procedures.
    • Applicable in all esophageal regions, including cervical esophagus and gastroesophageal junction.
  • Limitations:
    • Best for small, adjacent fluid collections.
    • Requires regular sponge exchanges and monitoring.

Operative Management

Primary Surgical Repair

  • Gold Standard for managing esophageal perforations.
  • Timing:
    • Traditionally within first 24 hours but can be effective even when delayed.
  • Technique:
    • Two-layer closure:
      • First layer: Mucosa and submucosa sutured.
      • Second layer: Overlying muscle layers closed.
    • Buttressing:
      • Reinforcement with vascularized tissue (e.g., pleura, intercostal muscle, omentum).
    • Myotomy:
      • Extends beyond the defect to expose healthy mucosa for closure.
    • Avoiding Luminal Narrowing:
      • Use a transoral bougie during repair.
    • Drain Placement:
      • Near but not touching the suture line to monitor for leaks.
    • Nutritional Support:
      • Placement of a feeding tube into the stomach or jejunum.
  • Surgical Approaches:
    • Left Thoracotomy: For distal intrathoracic esophagus (via the 7th intercostal space).
    • Right Thoracotomy: For proximal to mid intrathoracic esophagus (via the 4th to 6th intercostal space).
    • Cervical or Abdominal Incisions: For neck or abdominal perforations.

Esophagectomy

  • Indications:
    • When repair or stenting is infeasible or esophagus is unsalvageable (e.g., end-stage achalasia).
  • Approaches:
    • Transthoracic: Allows for removal and drainage of contaminated areas.
    • Transhiatal Resection: Avoids chest incisions and single-lung ventilation.
  • Considerations:
    • Delay Reconstruction if sepsis is present.
    • Cervicothoracic End Esophagostomy:
      • Created to drain oral secretions.
      • Preserve as much proximal esophagus as possible.
    • Esophagostomy Risks:
      • Stricturing or necrosis of the stoma tip may require future intervention.

Esophageal Diversion

  • Alternative to esophagectomy for future reconstruction potential.
  • Techniques:
    • Proximal Diversion:
      • End cervical esophagostomy.
    • Distal Diversion:
      • Division at the gastroesophageal junction using a stapler.
  • Risks:
    • Early Recanalization: May lead to persistent leakage.
    • Pooling of Secretions: Can cause retching or nausea.

Additional Considerations

Location of the Perforation

  • Cervical Perforations:
    • Often well-tolerated due to containment by neck fascia.
    • May resolve with antibiotics alone.
    • Surgical Drainage if condition deteriorates.
  • Intraabdominal Perforations:
    • Less common but may occur with endoscopic interventions.
    • Surgical Approach:
      • Laparotomy or laparoscopy may be necessary.
      • Fundoplication can buttress the repair.

Preexisting Esophageal Pathology

  • Achalasia:
    • Perforation during pneumatic dilation requires addressing the underlying disease.
    • Best Approach:
      • Close the perforation.
      • Perform distal esophageal myotomy (Heller myotomy).
      • Partial fundoplication to prevent reflux and buttress the repair.
  • Esophageal Malignancy:
    • Definitive surgical resection should be considered if the patient is stable and cancer is resectable.

Outcomes

  • High Morbidity and Mortality Historically:
    • Overall mortality rate around 18%.
    • Delays in Treatment significantly worsen outcomes.

Nonoperative Approaches

  • Carefully Selected Patients:
    • Show superior outcomes compared to surgical intervention.
    • Success dependent on strict adherence to selection criteria.

Surgical Treatment

  • Varied Mortality Rates:
    • Primary Repair: ~12% mortality.
    • Esophagectomy: ~17% mortality.
    • Esophageal Exclusion: ~24% mortality.
    • Surgical Drainage Alone: ~36% mortality.

Endoscopic Treatment

  • Esophageal Stenting:
    • 85% success rate in managing leaks.
    • Mortality similar to surgical repair (13%).
    • Complications:
      • Stent migration (29%).
      • Bleeding (2%).
      • Tissue overgrowth (5%).
  • Endoscopic Clipping and EVT:
    • Effective for Small, Early Perforations:
      • TTS clips for defects <10 mm.
      • OTSCs for defects up to 20 mm.
    • EVT Success Rates:
      • Ranging from 70% to 100% in small case series.

Conclusion

  • Esophageal perforation is a life-threatening emergency requiring prompt, appropriate management.
  • Critical Treatment Principles:
    • Close, occlude, exteriorize, or divert the esophageal defect.
    • Drain extraluminal fluid collections.
    • Alleviate distal obstruction.
    • Administer antibiotics.
    • Provide supportive care, including nutrition.
  • Tailored Treatment Plans:
    • Must consider perforation specifics:
      • Size and location.
      • Time since onset.
      • Extent of contamination.
      • Preexisting esophageal disease.
      • Patient's clinical condition.
  • Advancements in Endoscopic Techniques:
    • Fully covered, removable stents, endoscopic clipping, and EVT have expanded non-surgical options.
  • Surgeon's Expertise:
    • Must be proficient in a range of diagnostic and therapeutic modalities.
    • Timely and appropriate intervention is key to successful outcomes.

Note: Managing esophageal perforations demands multidisciplinary collaboration and sound clinical judgment due to the variability in presentation and the risk of rapid deterioration.

Management of Esophageal Perforations and Leaks

Introduction

  • Esophageal perforations are increasingly common.
  • Iatrogenic causes are the most frequent (∼60% of cases), mainly due to the widespread use of endoscopy.
  • Mortality rate remains high: 12% to 50% in some series.
  • Management requires expertise in:
    • Anatomic considerations of the neck, chest, and abdomen.
    • Endoscopic procedures.
    • Esophageal resection and complex reconstruction.
  • Hybrid approaches often involve stenting with a muscle buttress.

Anatomic Considerations

  • The esophagus is a long muscular tube from the pharynx to the gastroesophageal junction.
  • Lacks a serosal layer, making it more prone to leaks and less forgiving with surgical repair.
  • Inner circular and outer longitudinal muscle layers may be weakened by perforation.
  • Perforations can spread infection through the submucosal plane, often underestimated due to muscular covering.

Common Sites of Perforation

  • Pharyngoesophageal junction.
  • Thoracic inlet.
  • Distal esophagus at the gastroesophageal junction.

Etiology of Esophageal Perforations and Leaks

  • Iatrogenic Perforations (∼60%):
    • Mostly related to endoscopy.
    • Diagnostic procedures: ∼0.6% risk.
    • Therapeutic procedures: ∼6% risk.
  • Spontaneous Perforation (15%):
    • Boerhaave Syndrome: Sudden increase in intraesophageal pressure.
    • Associated with vomiting, weight lifting, childbirth, defecation.
  • Trauma (9%):
    • Penetrating Trauma: Gunshot wounds (75%), stab wounds.
    • Blunt Trauma: Rare, mechanisms include chest compression, hyperextension.
  • Foreign Body Ingestion (12%).
  • Malignancy (1%).
  • Infection:
    • Immunocompromised patients.
    • Eosinophilic esophagitis leading to spontaneous perforation.
  • Caustic Ingestion:
    • Acids: Cause coagulative necrosis; less likely to perforate.
    • Alkalis (e.g., lye): Cause liquefactive necrosis; higher risk of perforation.

Patient Presentation

  • General Symptoms:
    • Fever, tachycardia, tachypnea, pain, leukocytosis.
    • Signs of sepsis or shock.
  • Cervical Perforations:
    • Neck pain.
    • Voice changes (nasal sound).
    • Dysphagia.
    • Hemoptysis.
    • Crepitus (subcutaneous emphysema).
  • Thoracic Perforations:
    • Chest or back pain.
    • Dyspnea.
    • Dysphagia.
    • Pleural effusion, pneumomediastinum, pneumothorax.
  • Abdominal Perforations:
    • Abdominal pain and distention.
    • Pneumoperitoneum.
    • Free fluid in the abdomen.

Classic Syndromes

  • Mackler Triad:
    • Vomiting.
    • Chest pain.
    • Subcutaneous emphysema.
  • Anderson Triad:
    • Subcutaneous emphysema.
    • Rapid respirations.
    • Abdominal rigidity.

Evaluation

  • Early identification is crucial; timing correlates with outcome.
  • History and Physical Examination:
    • Check for recent instrumentation, trauma, vomiting, dysphagia.
    • Assess for signs of hemodynamic instability.
  • Imaging Studies:
    • Chest and Abdominal Radiographs:
      • Look for subcutaneous emphysema, pneumomediastinum, pleural effusion.
      • Diagnostic in ∼80% of iatrogenic perforations.
    • Contrast Esophagram (Gold Standard):
      • Use low-osmolar water-soluble contrast (e.g., Omnipaque).
      • Gastrografin has higher false-negative rates and aspiration risk.
      • Barium offers higher accuracy but persists in tissues.
      • False-negative rate of contrast studies is ∼10%.
    • Computed Tomography (CT) Scan:
      • Useful if patient cannot swallow or is intubated.
      • Identifies site of leak and associated collections.
      • Nasogastric tube may be placed for contrast administration.
  • Endoscopy:
    • Diagnostic and therapeutic.
    • Must be performed cautiously by experienced endoscopists.
    • Allows for irrigation and drainage of large perforations.
    • Can sample pleural effusions.

Management

  • Treatment Principles:

    • Drainage of infection.
    • Timely intervention.
    • Prevention of contamination.
    • Restoration of GI continuity.
    • Nutritional support.

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To Repair or Not to Repair

  • Factors to Consider:
    • Size of perforation.
      • Large perforations (>50% circumference or >3 cm) may result in strictures.
    • Viability of tissue.
    • Time of presentation.
      • Delayed presentation (>48 hours) often not suitable for surgical repair.
  • Repair Techniques:
    • Débridement of nonviable tissue.
    • Two-layer closure:
      • Mucosal and muscle layers closed separately.
    • Buttressing with vascularized tissue:
      • Intercostal muscle, sternocleidomastoid, omentum, etc.
  • Alternative Strategies:
    • Hybrid repairs: Débridement with muscle buttress plus stenting.
    • T-tube placement: Externalized fistula drainage.
    • Esophageal diversion: For uncontrolled leaks or severe contamination.

Surgical Approach

  • Cervical Perforations:
    • Left neck incision along sternocleidomastoid.
    • Wide drainage for small, contained perforations.
    • Diversion may be necessary.
  • Thoracic Perforations:
    • Upper two-thirds: Right posterolateral thoracotomy (4th or 5th intercostal space).
    • Lower third: Left posterolateral thoracotomy (6th or 7th intercostal space).
    • Intercostal muscle flaps for buttressing.
  • Abdominal Perforations:
    • Laparoscopy or midline abdominal incision.
    • Focus on débridement and repair or drainage.
  • Adjuncts:
    • Pulmonary decortication to facilitate lung expansion.
    • Chest tubes for drainage.
    • Nutritional support:
      • Jejunostomy or gastrostomy tubes.
    • Broad-spectrum antibiotics:
      • Cover Staphylococcus, Pseudomonas, Streptococcus, Bacteroides.
      • Examples: Piperacillin-tazobactam; adjust based on cultures.

Endoscopic Management of Esophageal Perforations and Leaks

Stent Selection

  • Types of Stents:
    • Self-Expanding Metallic Stents (SEMS):
      • Uncovered, partially covered, or fully covered.
      • Fully covered SEMS prevent tissue ingrowth; removable.
    • Self-Expanding Plastic Stents (SEPS):
      • Made of silicone or other polymers.
      • Fully covered; higher migration rates but removable.

Patient Selection for Endoscopic Management

  • Ideal Candidates:
    • Early diagnosis with minimal contamination.
    • Iatrogenic perforations recognized immediately.
    • No distal obstruction or severe comorbidities.
  • Contraindications:
    • Delayed presentation with extensive contamination.
    • Large perforations not amenable to stenting.

Esophageal Stenting for Perforations and Leaks

  • Benefits:
    • Less procedural morbidity than surgery.
    • Rapid closure of perforation.
    • Early initiation of oral nutrition.
  • Applications:
    • Iatrogenic perforations.
    • Anastomotic leaks post-surgery.
    • Gastric staple line leaks after bariatric surgery.
  • Procedure:
    • Endoscopic placement of stent over the perforation.
    • On-table fluoroscopy to confirm placement and seal.
    • Suturing the stent in place to prevent migration.
  • Adjunctive Measures:
    • Drainage of associated collections (surgical or percutaneous).
    • Nasogastric decompression if necessary.

Post-stenting Considerations

  • Monitoring:
    • Esophagram within 24 hours to check for leaks.
  • Diet:
    • Start with room-temperature liquids, advance to soft solids.
  • Follow-up:
    • Endoscopy within 2 weeks to assess healing.
    • Stent removal if healing is adequate.
    • Continue soft diet for 2 to 6 weeks post-removal.

Complications of Stenting

  • Early Complications:
    • Stent migration (most common).
    • Patient discomfort.
    • Bleeding.
    • Acid reflux.
    • Stent leak.
  • Delayed Complications:
    • Stent migration.
    • Tumor ingrowth (in malignant cases).
    • Injury to surrounding structures (e.g., fistulization).
  • Management of Complications:
    • Repositioning or replacing the stent.
    • Surgical intervention if endoscopic measures fail.
    • Regular monitoring and early extraction to prevent complications.

Stent-Guided Regeneration and Reepithelialization

  • Concept:
    • Stents act as a scaffold to promote tissue regeneration and reepithelialization over large defects.
  • Clinical Evidence:
    • Successful cases of bridging anastomotic disunions with stents.
    • Extracellular matrices and biologic scaffolds being explored for tissue regeneration.
  • Future Directions:
    • Potential to revolutionize esophageal surgery.
    • Ongoing research and clinical trials needed.

Note: Management of esophageal perforations requires multidisciplinary collaboration and sound clinical judgment due to variability in presentation and risk of rapid deterioration. Timely intervention and adherence to treatment principles are key to improving outcomes.