Oesophageal perforation was discussed in Question 3 from the first paper of 2011 and Question 16 from the second paper of 2016. Specifically, Boerhaave's syndrome is the oesophageal perforation which one sustaines in the course of vomiting. The classical case, I suppose, is that of gross gluttony followed by forced vomiting, as was the case which claimed the life of Baron von Wassenaer, Grand Admiral of the Dutch Fleet. All other forms of non-vomit-induced oesophageal perforation appear to be non-eponymous.

Clinical features of oesophageal perforation

In one retrospective audit, the following features were commonly noted:

  • Subcutaneous emphysema in 69.4%
  • Pain in 66.7%
  • Dyspnea in 38.9%
  • Fever in 33.3%

That's right, subcutaneous emphysema was one of the presenting complaints in over 1/3rd of patients. The pain is usualy retrosternal or pleuritic.

Diagnostic tests

  • CXR looking for pneumomediastinum
    • Occasionally, a pleural effusion (composed of undigested food and stomach juice) may be present.
    • Apparently, about 15% of patients have a confusingly normal CXR.
  • CT chest with contrast
    • This will demonstrate subtle pneumomediastinum, and may show discontinuities in the wall of the oesophagus, but can be confounded by the motion artifact from cardiac pulsation.
  • Gastrograffin swallow (not barium) - this is the "contrast oesophagogram" described in the LITFL summary - the gold standard for imaging the perforated oesophagus
  • Intercostal catheter to drain pleural effusion and analyse it, looking for acidity (suggestive of gastric contents) and food particles.
    • How acidic should it be? Well. Gastric content can have a pH as low as 1.5. The pleural fluid, no matter how infected, should never have a pH lower than 7.0. The presence of gastric contents should lower it below 6.0.
    • Also, a gastric content-based pleural effusion should have an elevated amylase level.

Management options

Back in my day, the only options were surgical. Any sort of conservative management has come on the menu only since TPN became available.

Thus, the options of definitive treatment are surgical, endoscopic and conservative.

Surgical options:

  • Primary repair: patch-over for the perforation.
  • Exclusion and oesophageal diversion (i.e. the oesophagus opens into a pouch attached to your neck, and a re-anastomosis is carried out once the sepsis settles down).
  • Resection and reconstruction which is delayed, as it is essentially an Ivor Lewis procedure and therefore would be disastrous in the setting of a big stinky empyema.
  • Drainage +/- decortication of the pleural effusion is usually something that can wait, and only needs to be urgent in those patients whose ventilation is severely affected (i.e. if you cannot extubate them safely with an undrained effusion ).

Endoscopic options:

  • Endoluminal stenting can be used in situations where there is no overwhelming mediastinal sepsis. Small or incomplete perforations are best managed eitehr conservatively or with endoscopic stenting.
  • This option is particularly good in situations where the perforation is actually due to a malignancy rather than just vomiting.
  • Complications might include stent migration, and worsening perforation (i.e. the endoscope can actually complete an incomplete perforation).

Conservative options

  • TPN: they won't be eating for a while
  • Broad-spectrum antibiotics - it is controversial as to whether or not the patient should be offered antifungal drugs, because the upper GI tract is usually colonised with some Candida species. Biancari et al (2013)
  • Proton pump inhibitors to encourage the process of oesophageal repair
  • Eventually, these people end up having surgery - but it is delayed until the empyema or mediastinal abscess are well-circumscribed.
  • The outcome is surprisingly good in selected patient groups- Hasan et al (2005) saw a survival rate of 84% in patients with an average age of 59; of the dead, all died of something other than mediastinitis.

Idiosyncratic issues of ICU management

There are a few little details about the perforated oesophagus which must be considered:

  • No positive pressure without intubation: even high flow nasal prongs can blow gas into the mediastinum.
  • Thus, no bag-mask ventilation with intubation. All of these patients should be getting an RSI-like induction.
  • TPN should probably start early because it is unlikely the patient will be fed within 7 days of their perforation.

Outcomes in the elderly

Presumably to reduce the incidence of horrendoplasty, Biancari et al published on the "Treatment of esophageal perforation in octogenarians" in 2014. Surprisingly, this small scale (33 patient) observational study did not reveal much of a mortality difference between surgical, conservative and endoscopic approaches. The mortality at 30 days was approximately 40%. These were not randomised patients, suggesting that those (11 of them) who were operated on were probably reasonably fit in  order to be even considered.

Tracheo-oesophageal fistula

This hideous and fortunately extremely rare complication was the topic of Question 23 from the second paper of 2019, which discussed the "principles and priorities in the management" of a patient who, after intubation, has a massive air leak through a hole in their left main bronchus which communicates directly with their oesophago-gastric anastomosis. It is extremely unlikely that any intensivist (let alone an end-stage trainee) have ever come directly in contact with such a beast, and so it is rather surprising and encouraging that the pass rate for that SAQ was 43.9%.  

In terms of published recommendations, one could do no better than the article  by Shamji & Inculet (2018), which is unfortnately paywalled. As much of the content of this article as possible has been integrated into the management options below.

Management priorities:

  1. Stabilise the patient (control ABCD)
  2. Organise definitive management, which will be surgical, bronchoscopic or endoscopic
  3. Prepare the family for the worse

As usual, the management can be divided into "supportive" and "specific".

Supportive management:

  • Airway management:
    • Intubate the patient with a right-sided  double lumen tube
    • Ideally do this in theatre, with an upper GI and thoracic surgen present. The risk here is that potentially, you will place the tube in such a violently stupid way that the bronchio-oesophageal tear is extended. The  consequence may be massive pneumomediastinum and an unventilatable patient with rapidly falling blood pressure due to greater vessel compression. 
  • Ventilator management
    • With a single lumen tube:
      • Account for large leak: use a pressure-control mode
    • With a double lumen tube:
      • Use differential lung ventilation
      • Ventilate the "good" lung with normal one-lung ventilation
      • Ventilate the "bad" lung with some modest  PEEP (~ 5-8) and zero inspiratory pressure (i.e. CPAP)
  • Sedation and analgesia
    • ​​​​With a double lumen tube, ideally the patient should be paralysed and sedated to prevent tube dislodgement and to facilitate differential lung ventilation
    • With a single lumen tube, spontaneous respiratory activity should be encouraged and sedation should be minimised
  • Gastrointestinal/nutritional management:
    • If this is feasible,place an NGT endoscopically and allow it to vent freely or place it on low wall suction
    • Most likely, this is not feasible, and the whole anastomotic site should be left well alone  
    • Feed the patient parenterally 
    • Use liberal PPI  therapy (perhaps an infusion of pantoprazole)
  • Management of infection
    • One can almost guarrantee that some infectious process will take place, 
      • in the lung (aspirtation)
      • in the chest cavity (collection)
      • in the abdominal cavity
    • Thus, empiric antibiotics should probably be commenced, and these should ideally be broad enough to cover upper GI anaerobes. You can always stop them again in a couple of days, when the antimicrobial stewardship people start mocking you openly in the corridors.
  • Management of family expectations
    • The outcome for these scenarios is almost uniformly bad, particularrly wherre a malignancy is involved

Specific management resembles the oesophageal perforation options mentioned above, with the exception of all the bronchoscopic stuff:

  • Bronchoscopic repair
    • Bronchial stent
  • Gastroscopic repair options
    • self-expanding esophageal stainless steel-covered metal stents (SEM)
  • Surgical repair options
    • Resection and diversion (i.e. oesophagus is externalised in the neck; one essentially disconnects the upper GI tract from the lower)
    • Resection and re-anastomosis (the latter is risky)
    • Conservative management (i.e. after having a look and the placement of a NG tube, the patient returns to ICU for conservative and palliative management)

References

Curci, JOSEPH J., and MARC J. Horman. "Boerhaave's syndrome: The importance of early diagnosis and treatment." Annals of surgery 183.4 (1976): 401.

Teh, Elaine, et al. "Boerhaave's syndrome: a review of management and outcome." Interactive cardiovascular and thoracic surgery 6.5 (2007): 640-643.

Eroǧlu, Atilla, et al. "Esophageal perforation: the importance of early diagnosis and primary repair." Diseases of the Esophagus 17.1 (2004): 91-94.

Patton, Anthony S., et al. "Reevaluation of the Boerhaave syndrome: A review of fourteen cases." The American Journal of Surgery 137.4 (1979): 560-565.

Movsas, S. "Spontaneous Rupture of the Oesophagus Is Conservative Treatment Ever Justified?." Thorax 21.2 (1966): 111-114.

Bolooki, H. O. O. S. A. N. G., et al. "Spontaneous rupture of the esophagus: Boerhaave's syndrome." Annals of surgery 174.2 (1971): 319.

Curci, JOSEPH J., and MARC J. Horman. "Boerhaave's syndrome: The importance of early diagnosis and treatment." Annals of surgery 183.4 (1976): 401.

Teh, Elaine, et al. "Boerhaave's syndrome: a review of management and outcome." Interactive cardiovascular and thoracic surgery 6.5 (2007): 640-643.

Eroǧlu, Atilla, et al. "Esophageal perforation: the importance of early diagnosis and primary repair." Diseases of the Esophagus 17.1 (2004): 91-94.

Bhatia, Pankaj, et al. "Current concepts in the management of esophageal perforations: a twenty-seven year Canadian experience." The Annals of thoracic surgery 92.1 (2011): 209-215.

Spapen, J., et al. "Boerhaave's Syndrome: Still a Diagnostic and Therapeutic Challenge in the 21st Century." Case reports in critical care 2013 (2013).

Biancari, Fausto, et al. "Current treatment and outcome of esophageal perforations in adults: systematic review and meta-analysis of 75 studies." World journal of surgery 37.5 (2013): 1051-1059.

Hasan, Shafqat, Ali NA Jilaihawi, and Dhruva Prakash. "Conservative management of iatrogenic oesophageal perforations—a viable option." European journal of cardio-thoracic surgery 28.1 (2005): 7-10.

Biancari, F., et al. "Treatment of esophageal perforation in octogenarians: a multicenter study." Diseases of the Esophagus 27.8 (2014): 715-718.

Spalding, Alanson R., Donald P. Burney, and Robert E. Richie. "Acquired benign bronchoesophageal fistulas in the adult." The Annals of thoracic surgery 28.4 (1979): 378-383.

Kalmár, Katalin, et al. "Non-malignant tracheo-gastric fistula following esophagectomy for cancer." European journal of cardio-thoracic surgery 18.3 (2000): 363-365.

Lolley, David M., et al. "Management of malignant esophagorespiratory fistula.The Annals of thoracic surgery 25.6 (1978): 516-520.

Shamji, Farid M., and Richard Inculet. "Management of malignant tracheoesophageal fistula." Thoracic surgery clinics 28.3 (2018): 393-402.