Joint Hospital Grand Round - Boerhaave’s Syndrome and Oesophageal Perforation NDH Dr. Samson Tse

Case Presentation (Boerhaave’s Syndrome)  WT Lee  M/69  Good past health except chronic duodenal ulcer detected >10 years ago

Case Presentation  Presented on with repeated vomiting and diarrhoea and epigastric pain radiating to back  No history of foreign body ingestion or trauma  CXR normal and discharged from A&E

Case Presentation  Reattended on with dysphagia, SOB and persistent right sided chest and back pain  Clinical examination – right anterior chest wall tenderness and decreased right sided air entry  CXR – subcutaneous emphysema, pneumomediastinum and RLZ hazziness

Causes of Pneumomediastinum  Pulmonary pathology  Tracheal pathology  Oesophageal pathology  Iatrogenic  Idiopathic

Case Presentation  Water soluble contrast study performed – extraluminal collection of contrast near the gastro-oesophageal junction  CT – pneumomediastinum and pocket of air-fluid level in the lower thorax around the lower thoracic oesophagus

Treatment  Right sided chest drain insertion, blood stained fluid with some debris drained  Drainage and diversion decided - transection of oesophagus, cervical oesophagostomy, gastrostomy and feeding jejunostomy 3 days later  Laparotomy and presternal gastric transposition 3½ months later

Historic Background  Hermann Boerhaave described the clinical presentation, the progress and the autopsy finding of this syndrome in 1724 barogenic perforation, postemetic perforation and spontaneous oesophageal rupture  Boerhaave’s syndrome is synonymous to barogenic perforation, postemetic perforation and spontaneous oesophageal rupture

Clinical Presentation ocationsize time course  Depending on the location and size of the injury and the time course leftdistal third  Almost always on the left side of the distal third oesophagus (~90%)  Most occurs along the longitudinal axis  Mucosal tear often longer than the serosal tear

Clinical Presentation  Pain  Pain occurs in % of cases  Other symptoms including dyspnoea, dysphagia, facial swelling, proptosis, dysphonia, polydipsia, haematemasis, hoarseness and SCM muscle spasm

Clinical Presentation  Signs including an acutely ill patient with fever, subcutaneous or mediastinal emphysema, tachycardia, tachypnea, cyanosis and shock  Hamman’s sign  Hamman’s sign had been reported  Mackler’s classic triad  Mackler’s classic triad of vomiting, chest pain and subcutaneous emphysema is less common than originally thought

Diagnosis  ? History  CXR (AP and lateral), erect AXR  Lateral neck XR  Gastrograffin / Barium contrast study  If gastrograffin negative -> follow by Barium -> will detect 60% of cervical and 90% surgically confirmed perforations – (Bladergroen MR 1986 & Symbar PN 1972 Ann Thor Surg, Kim-Deobald J 1992, Am J GE ) False negative rate of 10-36%

Diagnosis  IV and oral contrast CT scan thorax and abdomen  Endoscopy’s role is highly questionable but has high accuracy for perforation secondary to external injury but not recommended for acute, non-penetrating perforations( Horwitz 1993 & Kim-Deobald 1992 AJGE, Mengoli 1965 Arch Surg )

Diagnosis  Thoracentesis may aid in diagnosis Acidic pH, elevated salivary amylase, purulent foul smelling material, or presence of undigested food are useful finding ( Attar 1990 Ann Thor Surg, Dubost 1979 J Thor Cadiovas, Roufail 1972 GI Endo )

Pathophysiology necrotizing mediastinitis  Mainly due to necrotizing mediastinitis  Hydropneumothorax and localized perioesophageal abscess are common finding  Staphylococcus, Pseudomonas, Streptococcus and Bacteroides  Staphylococcus, Pseudomonas, Streptococcus and Bacteroides usually involved fluid sequestration, sepsis and death  Natural history is fluid sequestration, sepsis and death

Medical Management for Oesophageal Perforation  Principles of medical treatment consists of :- - NPO - parental alimentation - nasogastric suction - board spectrum antibiotics  Good results achieved but only in patients with instrumentation perforation ( Mengoli 1965 Arch Surg, Wesdorp 1984 Gut, Sarr 1982 JTCVS, Michel 1981 Ann Surg )

Medical Management  Criteria for conservative management :- - clinically stable, minimal sepsis - elective instrumental perforation - contained perforation - absence of crepitus, pneumothorax or pneumoperitoneum

Medical Management  Endoprothesis usually reserved for patients with malignant disease and instrumental perforation ( Wesdorp 1984 Gut, Hine 1986 Dig Dis Sci, Nicholson 1995 Clin Rad )  Successful use of endoprothesis in management of Boerhaave’s Syndrome had also been reported ( Chung 2001 Endoscopy, Davies 1999 Ann Thorac Surg )

Surgical Management drainage alone drainage and repair (direct closure, omental; diaphragmatic or fundal patch) drainage and diversion  Surgical techniques include drainage alone, drainage and repair (direct closure, omental; diaphragmatic or fundal patch), and drainage and diversion depending on the location of perforation, time period between perforation and diagnosis and the presence of underlying oesophageal disease

Surgical Management  Open vs minimal invasive technique  Most suitable operation is usually “ tailor made” operation for individual patient

Surgical Management  Criteria for surgical management :- - Boerhaave’s syndrome - clinically unstable with sepsis, shock, and respiratory failure - contaminated mediastinum or pleural space - perforation with retained foreign bodies - perforation in oesophageal disease for which elective surgery is considered - failed medical therapy

Mortality  Overall mortality of oesophageal perforations is 15.5% - 29% (range 0-64%) timing of treatment, location and aetiology of the perforation  Outcome depends on timing of treatment, location and aetiology of the perforation Boerhaave’s syndrome has the highest mortality rate – from 22% - 63% six  Underlying oesophageal disease increases the mortality rate by six times

Conclusion  A diagnostic and therapeutic challenge  High index of suspicion in clinically suspicious cases even if initial investigations are negative  Thoracic site, delayed diagnosis and treatment are the main factors contributing to poor survival  If surgery is performed, a hour window is optimal