Management of Boerhaaves Syndrome Spontaneous Rupture of the

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

Endoscopic treatment Endoscopic clipping • Limited to small clean perforations (<1. 5 cm) and minimal symptoms of infection • Early diagnosis and treatment • Reports of successful clipping of late, mature perforation

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

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