Perforation, Fistula, and Caustic Injury


Esophageal Perforation

  1. Boerhaave Syndrome
    1. Etiology
      • spontaneous perforation of the esophagus that results from a sudden increase in intraesophageal pressure combined with negative intrathoracic pressure
      • most commonly results from severe retching or vomiting
      • most patients have a normal underlying esophagus

    2. Pathogenesis
      • usually involves a longitudinal tear of the posterolateral aspect of the distal esophagus
      • mediastinum becomes contaminated with gastric contents, resulting in a chemical mediastinitis
      • left untreated, bacterial infection and mediastinal necrosis will supervene
      • rupture of the mediastinal pleura contaminates the pleural cavity

    3. Clinical Manifestations
      • classic presentation is severe retrosternal chest pain following an episode of vomiting or retching
      • within several hours of perforation, patients develop tachycardia, fever, dyspnea, and hypotension
      • some patients will have crepitus of the chest wall

    4. Diagnosis
      1. Chest X-ray
        • suggestive findings include a left pleural effusion, mediastinal air, or subcutaneous emphysema

        CXR Showing Mediastinal Air and a Left Pleual Effusion
      2. Esophagram
        • should be done initially with gastrograffin, but if this study is negative and suspicion is high, then a barium esophagram can be performed
        • accurately demonstrates the site and extent of perforation in 90% of cases

        Esophagram Showing Free Perforation
      3. CT Scan
        • useful study when an esophagram cannot be done (uncooperative or unstable patient)
        • suggestive findings include esophageal wall thickening and edema, periesophageal fluid, or mediastinal air
        • does not accurately localize the site of perforation

        CT Scan showing Mediastinal Air and Esophageal Wall Thickening
    5. Initial Management
      • all patients will require fluid resuscitation and broad-spectrum antibiotics in preparation for definitive management

    6. Definitive Management
      1. Endoscopic Stenting
        • in cases of spontaneous perforation, endoscopic stenting should be limited to patients who are unlikely to tolerate surgery because of significant comorbidities
        • additional measures include drainage of fluid collections, PPIs, TPN
        • clinical deterioration will require surgery

      2. Primary Surgical Repair
        • the distal esophagus is best approached through a left thoracotomy at the 7th or 8th intercostal space
        • debride all necrotic tissue
        • incise the muscle to expose the entire extent of the mucosal injury
        • close the defect in 2 layers
        • an intercostal muscle flap or pleural flap can be used to buttress the repair
        • the repair and mediastinum are widely drained with 2 chest tubes
        • consider a jejunostomy tube in malnourished patients

        Intercostal Muscle Flap and Pleural Flap
        1. Damage Control Procedures
          • for delayed presentations of perforation > 24 hours, mediastinal inflammation or necrosis may preclude safe primary repair
          • cervical esophagostomy, gastrostomy tube, wide chest tube drainage, and a feeding jejunostomy should control drainage above and below the perforation and allow for postop alimentation
          • another option would be to place a large t-tube into the perforation and widely drain the mediastinum

          Esophageal T-tube Placement for Damage Control
  2. Iatrogenic Esophageal Perforation
    1. Etiology
      • most esophageal perforations occur after therapeutic upper endoscopy: pneumatic dilation for strictures or achalasia, stent placement, sclerotherapy for varices
      • iatrogenic perforations may occur in the neck, thorax, or abdomen

    2. Clinical manifestations
      • need a high index of suspicion to make an early diagnosis
      • cervical perforations may present with a neck ache, swelling, or crepitation
      • abdominal perforations may present with an acute abdomen
      • thoracic perforations may present like spontaneous perforations
      • some patients will have tachypnea, tachycardia, low-grade fever, but no other overt signs of perforation

    3. Diagnosis
      • esophagram with water-soluble contrast usually reveals the perforation
      • chest CT may show mediastinal air and fluid at the site of perforation
      • abdominal CT may show free intraabdominal air

    4. Management
      1. Resuscitation
        • large-bore IVs, aggressive fluid resuscitation, ICU monitoring
        • broad-spectrum antibiotics covering oral flora, including fungi
        • NG-tube should be carefully placed just above the site of perforation

      2. Cervical Perforation
        • explored through a left neck incision along the sternocleidomastoid muscle
        • the middle thyroid vein and omohyoid muscle should be divided to improve exposure
        • retroesophageal space is entered bluntly along the prevertebral fascia
        • dissection is carried down to the posterior mediastinum to drain all collections
        • if the perforation is identified, it can be closed with absorbable suture and drained
        • if the perforation is not visualized, simple drainage results in healing of the defect

      3. Thoracic Perforation
        • for stable patients with contained perforations, endoscopic stent placement and drainage of fluid collections is effective
        • patients with iatrogenic free perforations will require a surgical approach similar to spontaneous perforations
        • right thoracotomy via the fifth interspace is the best approach for perforations of the upper two thirds of the esophagus
        • left thoracotomy via the seventh interspace is the best approach for distal perforations

      4. Abdominal Perforation
        • can be approached via an upper midline incision
        • to expose the hiatus, the triangular ligament must be divided and the left lobe of the liver retracted laterally
        • repair can be buttressed with omentum
        • fundus of the stomach as a Nissen or Dor fundoplication may also be used as a buttress, depending upon the patient’s history of reflux and dysphagia
        • place a jejunostomy tube for postop alimentation

  3. Unusual Perforations
    1. Perforation and Malignancy
      • for a contained perforation, an esophageal stent and chest tube drainage is indicated, leaving the cancer resection for another day
      • for a free perforation with minimal contamination, resection with primary anastomosis is ideal
      • for extensive contamination, resection, cervical esophagostomy, and gastrostomy tube for feeding is appropriate

    2. Perforation with Achalasia
      • if the perforation is above the LES, elevated esophageal pressure will impede healing of the repair
      • myotomy must be done at the time of the repair and performed on the side of the esophagus opposite the perforation

    3. Perforation after Nissen Fundoplication
      • fever, tachycardia, elevated WBC count, upper abdominal pain several days after a Nissen is worrisome for an esophageal perforation
      • will be diagnosed by CT scan or contrast study
      • management is emergent laparotomy, takedown of the wrap, suture repair of the perforation over a bougie, rewrap of the Nissen

    4. Anastomotic Leak
      • most feared complication of a thoracic esophagectomy
      • most critical variable is the viability of the gastric conduit
      • if the stomach is viable, then a stent and chest tube drainage is a reasonable approach
      • if the stomach is gangrenous, then it must be resected and an esophagostomy performed

Tracheoesophageal Fistulas (TEFs) in Adults

  1. Etiology
    • epithelialized tract between the esophagus and trachea
    • in adults, most TEFs result from lung or esophageal cancer
    • other causes include prolonged mechanical ventilation and caustic ingestion

  2. Symptoms
    • persistent coughing with meals
    • production of bile-stained mucus
    • frequent aspiration, respiratory infections, fever

  3. Diagnosis
    • made by endoscopy, bronchoscopy, barium esophagram
    • size of the fistula and location with respect to the carina, vocal cords, UES, LES should be noted

    Esophagram Showing a Tracheoesophageal Fistula
  4. Treatment
    1. Preventing Further Lung Contamination
      • NPO
      • HOB elevated to 45 degrees
      • frequent pulmonary suctioning
      • feeding tube placement
      • IV antibiotics, PPIs

    2. Malignant TEFs
      • treated palliatively since life expectancy is minimal
      • for lesions in the proximal to mid esophagus, combined tracheal and esophageal stenting should be done since an esophageal stent alone can cause airway compromise
      • more distal esophageal fistulas usually just require an esophageal stent since airway compromise is less likely in this location

      Combine Tracheal and Esophageal Stents for a Malignant TEF
    3. Nonmalignant TEFs
      • use of biologic glues or endoscopic clips has been used for small tracts with some success
      • larger tracts will require surgical closure
      • fistula tract is exposed and divided through a cervical or thoracic incision
      • the esophagus and trachea are primarily repaired
      • segmental resection of the trachea may reduce the incidence of tracheal stenosis
      • intercostal or strap muscle flap must be placed between the trachea and esophagus
      • quick extubation should be the goal since positive pressure mechanical ventilation may lead to wound dehiscence and fistula recurrence

      Primary Repair of a TEF

Caustic Injury in Adults

  1. Etiology
    • in children ingestion is by accident, and is usually of low volume
    • in teenagers and adults, ingestion is usually deliberate (suicide attempt)
    • alkali ingestion is more serious and results in significant destruction of the esophagus

    1. Alkali Ingestion
      • most common agents are drain cleaners, other household cleaning products, and disc batteries
      • does not cause immediate symptoms such as a burning sensation in the mouth
      • dissolves tissues by liquefaction necrosis
      • phase 1: acute necrotic phase lasts 1 – 4 days
      • phase 2: ulceration and granulation phase follows and lasts 3 – 12 days (esophagus is weakest during this phase)
      • phase 3: scar tissue formation leads to stricture formation

    2. Acid Ingestion
      • hard to ingest large quantities because acids cause an immediate burning sensation in the mouth
      • coagulative necrosis causes eschar formation that limits tissue penetration
      • overall, acid ingestion is less severe than alkali (lye) ingestion

  2. Symptoms
    • acute symptoms include oral and substernal pain, hypersalivation, odynophagia, dysphagia, hematemesis, vomiting, fever
    • hoarseness, stridor, dyspnea indicates respiratory injury, and are usually worse with acid ingestion
    • back and chest pain may indicate mediastinal perforation
    • abdominal pain may indicate abdominal perforation

  3. Diagnosis
    • inspect the lips, mouth, palate, pharynx, larynx
    • auscultate the lungs to assess degree of airway injury
    • palpate the abdomen for evidence of perforation

    1. Endoscopy
      • used to identify the grade of the injury and guide treatment (similar to burns)
      • should only be done in patients with no emergency indication for surgery
      • first degree: mucosal hyperemia, edema
      • second degree: exudates, ulceration, pseudomembrane formation
      • third degree: mucosal sloughing, deep ulcerations, hemorrhage, luminal obstruction, charring
      • fourth degree: perforation

      Endoscopic Caustic Injury Grades
  4. Treatment
    1. Acute Phase
      1. Neutralization
        • many authors recommend against this because it can worsen the injury
        • other experts suggest neutralization can be attempted if the patient presents within an hour of ingestion
        • alkalis (lye) are neutralized with half-strength vinegar or citrus juice
        • acids are neutralized with milk, egg whites, or antacids
        • emetics (cause retching) or sodium bicarbonate (releases heat) should be avoided because they increase the chance of perforation
        • no evidence that corticosteroids are beneficial in adults

      2. No Evidence of Injury
        • if the physical exam is negative, the patient is asymptomatic, and the ingestion volume was low, then oral nutrition can be started once the patient can swallow saliva painlessly
        • endoscopy is not necessary
        • most patients can be safely discharged from the ER

      3. First-Degree Injury
        • 48 hours of observation indicated
        • acid suppression with PPIs
        • oral nutrition can be resumed once patient can swallow saliva painlessly
        • will need outpatient monitoring for stricture formation (most strictures will have developed by 8 months)

      4. Second- and Third-Degree Injuries
        1. Resuscitation
          • massive fluid shifts, renal failure, sepsis can occur rapidly
          • aggressive fluid resuscitation in the ICU
          • IV antibiotics
          • proton pump inhibitor
          • aerosolized steroids can reduce airway obstruction
          • fiberoptic intubation may be required
          • use of steroids to reduce stricture formation is controversial – may mask symptoms or peritonitis

        2. Esophagus Management
          • more than one acceptable approach
          • if the stomach and esophagus are viable, then a jejunostomy tube for feeding and an esophageal stent can be placed, with oral intake resuming when the patient can swallow painlessly
          • laparoscopy, thoracoscopy may be necessary to fully evaluate the viability of the esophagus and stomach
          • if the viability of the esophagus and stomach are questionable, then a second look procedure should be performed in 36 hours
          • if full-thickness necrosis or perforation is present, then all the necrotic stomach and esophagus must be resected and an end-cervical esophagostomy is performed, along with a feeding j-tube
          • if necrosis is limited to the stomach, then a total gastrectomy can be performed with preservation of the esophagus
          • esophagectomy with preservation of the stomach is rarely possible or indicated

          Esophagostomy (Spit Fistula)
          Cervical Esophagostomy

    2. Chronic Phase
      1. Strictures
        • some authors recommend esophageal stenting and prophylactic bougienage to prevent stricture formation, although there are only case series to support this practice
        • scheduled esophagrams and endoscopy are used to screen for stricture formation
        • any stricture, even if asymptomatic, is treated with frequent bougie dilation
        • esophageal resection will be necessary for strictures that do not respond to dilation

      2. Reconstruction
        • delayed until 6 – 12 months
        • if the damaged esophagus has been left in situ, it must be resected (1000X risk of esophageal cancer in patients with a caustic injury)
        • gastric conduit is preferred if available
        • other possible conduits include a colon interposition or jejunal interposition
        • site of anastomosis is dependent on the extent of injury (chest versus neck)

        Jejunal Interposition Pedicle Graft
        Pedicled Jejunal Interposition Graft

      3. Screening for Esophageal Cancer
        • up to 30% of patients with caustic injuries develop esophageal squamous cell cancer, with a mean latency period of 41 years
        • overall, these cancers have a better prognosis than other esophageal squamous cell cancers
        • recommended screening protocol is endoscopy every 2 – 3 years beginning 10 – 20 years after the caustic ingestion

      4. Other Rare Complications
        • upper GI bleeding occurs in 3% of patients, usually 2 – 4 weeks after ingestion
        • tracheoesophageal fistulas occur in 3% of patients
        • aortoenteric fistulas are vary rare but are associated with a high mortality rate







References

  1. Sabiston, 19th ed., pgs 1040 – 1047
  2. Cameron, 11th ed., pgs 64 – 68
  3. UpToDate. Boerhaave Syndrome: Effort Rupture of the Esophagus. Triadafilopoulos md, George. Feb 21, 2018. Pgs 1 – 21
  4. UpToDate. Surgical Management of Esophageal Perforation. Raymond MD, Daniel. May 30, 2018. Pgs 1 – 47
  5. UpToDate. UpToDate. Tracheo- and Broncho-Esophageal Fistula in Adults. Majid MD, Adnan. May 15, 2018. Pgs 1 – 24
  6. UpToDate. Caustic Esophageal Injury in Adults. Triadafilopoulos MD, George. Jan 09, 2019. Pgs 1 – 31