Management of Boerhaave's Syndrome

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

3. Pathophysiology • Barogenic rupture caused by rapid rise in intraluminal pressure in the distal esophagus • 90% at the leftlateral position of lowerthird 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 destructionand severe sepsis

5. Diagnosis • Mackler's triad (<14% of patients) • 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 • 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