1. Management of Boerhaave's Syndrome
Spontaneous Rupture of the Esophagus
Joint Hospital Surgical Grand Round
21 April 2012
Dr Lee Wang Fai Frank
Princess Margaret Hospital

2. Boerhaave's Syndrome Spontaneous rupture of the esophagus
Rare condition with high mortality rate
Originally described in 1724 by Dutch physician Hermann Boerhaave
Classical symptoms: Forceful vomiting followed by pain, dyspnoea, shock

3. Pathophysiology
Barogenic rupture caused by rapid rise in intraluminal pressure in the distal esophagus
90% at the left lateral position of lower third of esophagus due to anatomic weakness at that point

4. Pathophysiology
Esophageal and gastric contents sucked out through the perforation into mediastinum by negative intrathoracic pressure
Chemical burn by gastric juice
Super-imposed necrotizing infection due to digestive enzymes and oral bacteria
Rapid tissue destruction and severe sepsis

5. Diagnosis Mackler's triad (<14% of patients) Common misdiagnosis
Vomiting (~80%)
Lower chest pain
Subcutaneous emphysema (~25%)
Common misdiagnosis
PPU
Myocardial infarction
Pneumonia
Pulmonary embolism
Aortic dissection
Pancreatitis

6. Diagnosis Diagnosis is commonly delayed
CXR: left pleural effusion, pneumomediastinum
Contrast esophagiogram
CT scan
Upper endoscopy

7. Management principles
Resuscitation and stabilization
Elimination of infection
Prevent further spoilage from the perforation
Control of extraluminal contamination
Appropriate broad-spectrum antibiotics coverage
Enteric access for nutritional support
Restoration of gastrointestinal continuity

8. Therapeutic options Conservative treatment
Surgical treatment ("Gold standard")
Primary closure +/- reinforcement
Drainage
Exclusion and diversion
Esophagectomy
Endoscopic treatment
Esophageal stenting
Endoclip application

9. Factors to consider Location of perforation
Degree of tissue destruction
Degree of contamination and sepsis
Time interval from injury
Presence of underlying esophageal disorder
Patient’s general condition and comorbidities

10. Conservative treatment
In patients present late, with contained perforation
Patient selection
Criteria by Cameron (1970)
Minimal clinical sepsis
Disruption contained in mediastinum
Drainage of the cavity back into esophagus

11. Conservative treatment
Principles:
Restriction of oral intake
Parenteral antibiotics
Gastric acid suppresion
Fluid resuscitation
+/- Percutaneous drainage of abscess
+/- Nasogastric tube insertion

12. Surgical treatment Transthoracic primary repair “Gold standard”
Best result for patients present within 24 hours
Tension-free apposition of healthy mucosal and submucosal tissue
+/- Reinforcement with autologous tissue, e.g. intercostal muscle, pleural or omental flap
Thoracotomy vs VATS

13. Surgical treatment Drainage
When direct repair is thought to have high chance of leakage
Drainage alone
+/- T-tube
Convert into controlled fistula

14. Surgical treatment Exclusion and diversion
Repair may be impossible in some patients, who present late with sepsis, heavy mediastinal contamination and devitalized esophageal tissue
Exclusion of the esophagus
Ligation of the cardia
Prevent reflux of gastric content
Diversion of oral secretions
Cervical esophagostomy
Require a second operation for restoration of gastrointestinal continuity
Esophagus is preserved for later reconstruction

15. Surgical treatment Esophagectomy
When there is heavy mediastinal contamination and necrotized esophageal tissue beyond salvage, or when underlying esophageal pathology is suspected
Transthoracic / transhiatal esophagectomy
Closure of cardia
Formation of cervical esophagostomy
Delayed reconstruction

16. Surgical treatment Additional procedures to consider
Decompressing gastrostomy
Drainage of gastric content
Feeding jejunostomy
Facilitate early enteric feeding
Fundoplication
Prevention of reflux

17. Endoscopic stenting Endoscopic stenting Effective seal of perforation
Self-expanding metallic stent (SEMS)
Fully covered vs Partially covered
Self-expanding plastic stent (SEPS)
Effective seal of perforation

18. Endoscopic stenting High reported success rate (~85%)
Mean time of stent placement: 6-8 weeks
Time delay between rupture and treatment remains most critical prognostic factor
Require concurrent adequate drainage of fluid collection in mediastinum / pleural cavity
Patient selection remains a topic of continued study; no guideline available currently

19. Endoscopic stenting Complications
Stent migration (25%)
More common in fully covered stent
Tissue in-growth and over-growth
Increased difficulty in removal of stent
More common in partially covered stent
No significant differences in efficacy between different types of stents

20. Endoscopic treatment Endoscopic clipping
Limited to small clean perforations (<1.5cm) and minimal symptoms of infection
Early diagnosis and treatment
Reports of successful clipping of late, mature perforation

21. Outcome Reported mortality varies in the literature (8-60%)
Mortality remains high and seemingly unchanged in recent 20 years
Delayed treatment is associated with higher mortality and complication rate

22. Conclusion Multiple treatment options and operative strategies
Limited evidence in the literature on best treatment
Rare disease
Retrospective case series, case reports, expert opinions
Reporting bias
Treatment should be individualized
Early recognition and prompt treatment are needed to maximize survival