1. Spontaneous rupture of the oesophagus Boerhaave syndrome
Dr Kylie JY Szeto
Tuen Mun Hospital
15 Dec 2018

2. Outline
Case presentation
Background
Investigation
Management

3. Our patient
71/M
History of perforated DU with patch repair 2001, severe AS with AVR on warfarin
Admitted in 4/2018 for epigastric discomfort after food intake
4 episodes of vomiting

4. BP 117/78 P 105 Afebrile
Neck soft, no surgical emphysema
Abdomen soft, only mild epigastric tenderness, midline scar+
PR: empty
CXR: no free gas
AXR: no dilated bowels

5. However patient gradually deteriorated
Shock, fast AF and desaturation
Raised WCC to 22
Repeated CXR showed left hydropneumothorax
Left pleural drain inserted

6. CT thorax Left hydropneumothorax with loculations
No obvious oesophageal defect but Boerhaave syndrome cannot be excluded given the clinical context

7. OGD
Oesophageal perforation suspected although CT findings inconclusive
Perforation encountered at 2cm above OGJ at left side
Contrast extravasation+

8. Background
Defined as spontaneous rupture of the oesophagus after forceful emesis
First described by Herman Boerhaave in 1724
Transmural rupture
Rare condition accountable for 15% of oesophageal perforation
Almost always fatal in the past
High mortality even now (35-40%)9

9. Pathophysiology
Secondary to raised intra-abdominal pressure and failed relaxation of cricopharyngeus
Longitudinal perforation
90% occurs at the left postero-lateral aspect of the distal intra-thoracic oesophagus, ~3cm above oesophago- gastric junction2

10. Presentation Symptoms are often atypical
Prior overindulgence of food or alcohol
Mackler's triad
vomiting, chest pain, subcutaneous emphysema
only presents in 15% of patients3
Esophageal perforation: continuing challenge to treatment
RV Romero, KL Goh - Gastrointestinal Intervention, 2013

11. Investigations
CXR
classical findings include pneumomediastinum, left pneumothorax and left pleural effusion
Naclerio V sign
air outlining left lower lateral mediastinal border and medial left hemidiaphragm

12. Investigations Water-soluble contrast study
shows location and extent of perforation
sensitivity depends on size and location of the perforation
false negative can be up to 10%4

13. Investigations CT
sensitive in detecting intra-peritoneal gas or mediastinal gas
assess extent of contamination
Endoscopy
reserved for patients with unclear diagnosis on imaging
enables lesion characterisation and shows viability of oesophageal mucosa
? turns concealed perforation into free perforation

14. Early diagnosis and treatment are crucial for successful outcome in oesophageal perforation
Mortality rate with a delayed diagnosis was 40% compared to 6.2% who were diagnosed in <24 hrs (p = 0.047)5

15. Management principles
Broad spectrum antibiotics
Drainage
Debridement of necrotic tissue
Restore GI tract integrity and continuity
Maintain nutrition

16. Drainage Chest drain insertion
VATS or thoracotomy for pulmonary decortication
to facilitate lung expansion by removing debris and exudates

17. Management Non-operative Conservative Operative Endoscopic treatment
Primary closure +/- reinforcement
Exclusion and diversion
Oesophagectomy

18. Primary closure +/- reinforcement
The choice of treatment when there is no underlying oesophageal pathology
Early presentation (within 24 hours)
Small defect
Longitudinal myotomy superior and inferior to the perforation to expose the entire extent of mucosal injury

19. Reinforcement can reduce rate of post-operative leakage8
54.5% vs 7.1% (p=0.021)
no significant difference in death or need of revision
no difference in hospital stay
small sample size

20. Options for reinforcement
Vascularised pedicle flap
Intercostal muscle flap is a common option
Other options include pleura, diaphragm, omentum, fundoplication etc

21. Oesophagectomy For patients with underlying oesophageal pathologies
Emergency oesophagectomy carries high mortality rate reaching up to 67%10

22. Exclusion and diversion
When primary repair is not feasible
late presentation
poor tissue condition
Or when patient is haemodynamically unstable
Followed by reconstruction of the oesophagus later
Aim to divert oesophageal contents in order to control and drain extraluminal contamination

23. Exclusion and diversion
Proximal diversion with a cervical oesophagostomy
Staple transection at oesophago-gastric junction
Feeding with gastrostomy
Possible incomplete diversion with continuous soiling

24. Conservative management
Controversial
NPO for at least 7 days
Intravenous broad spectrum antibiotics
ICU care
Drainage of fluid collections
Nutritional support
Only in highly selected patients with contained leak and minimal sepsis
Any deterioration should be followed by an immediate change of treatment plan towards surgery

25. Endoscopic treatment
Stenting (SEMS/SEPS), clipping, endoscopic vacuum therapy
Weak evidence
Most are case series with small sample size and not specific to Boerhaave syndrome
May be appropriate for patients with significant co-morbidities who cannot tolerate extensive surgery

26. ? A systematic review shows success rate of endoscopic stenting up to 81%7
only 26 out of 340 cases are Boerhaave syndrome

27. A retrospective comparative study shows no advantage in endoscopic stenting8 in Boerhaave syndrome
pulmonary decortication included as surgical re-intervention as well

28. Summary ? Confirm diagnosis of Boerhaave syndrome
Resuscitation and control sepsis
Early presentation/stable
Healthy oesophagus
Primary repair +/- reinforcement
Diseased oesophagus
Oesophagectomy
Free perforation
Late presentation/unstable
Exclusion and diversion
Delayed reconstruction
Contained perforation
Conservative management
ABC
Antibiotics, PPI
Chest drain for drainage
high mortality*
Endoscopic treatment
More research needed ?

29. Back to our patient
Open trans-abdominal repair of oesophagus with fundoplication
Findings
2cm oesophageal perforation over left postero-lateral aspect, 2cm above OGJ
VATS for decortication of left lung

30. Bring home message
Prompt recognition of this potential lethal condition is crucial to ensure appropriate treatment
High index of suspicion is important
Management plan has to be tailored for every patient

31. Reference
Brinster CJ, Singhal S, Lee L, et al. Evolving options in the management of esophageal perforation. Ann Thorac Surg 2004; 77:1475.
de Schipper JP, Pull ter Gunne AF, Oostvogel HJ et-al. Spontaneous rupture of the oesophagus: Boerhaave's syndrome in Literature review and treatment algorithm. Dig Surg. 2009;26 (1): 1-6. doi: /
Woo, Kar-mun C.; Schneider, Jeffrey I. (2009). "High-Risk Chief Complaints I: Chest Pain—The Big Three". Emergency Medicine Clinics of North America. 27 (4): 685–712, x. doi: /j.emc
Bladergroen MR, Lowe JE, Postlethwait RW. Diagnosis and recommended management of esophageal perforation and rupture. Ann Thorac Surg 1986; 42:235.
Shaker H, Elsayed H, Whittle I et-al. The influence of the 'golden 24-h rule' on the prognosis of oesophageal perforation in the modern era. Eur J Cardiothorac Surg ;38 (2): doi: /j.ejcts
Sulpice L, Dileon S, Rayar M, Badic B, Boudjema K, Bail J, et al. Conservative surgical management of Boerhaave's syndrome: Experience of two tertiary referral centers. International Journal of Surgery 2013;11(1):64-67
Dasari BV, Neely D, Kennedy A, Spence G, Rice P, Mackle E, et al. The role of esophageal stents in the management of esophageal anastomotic leaks and benign esophageal perforations. Ann Surg 2014 May;259(5):
Schweigert M, Beattie R, Solymosi N, Booth K, Dubecz A, Muir A, et al. Endoscopic stent insertion versus primary operative management for spontaneous rupture of the esophagus (Boerhaave syndrome): an international study comparing the outcome. Am Surg 2013;79(6):
Turner AR, Turner SD. Boerhaave syndrome. StatPearls [Internet] Jan
Evaluation of urgent esophagectomy in esophageal perforation. José Luis Braga de AQUINO, José Gonzaga Teixeira de CAMARGO, Gustavo Nardini CECCHINO, Douglas Alexandre Rizzanti PEREIRA, Caroline Agnelli BENTO, Vânia Aparecida LEANDRO-MERHI. Arq Bras Cir Dig Oct-Dec; 27(4): 247–250. doi: /S

32. Local experience
Total 11 cases of Boerhaave syndrome in TMH from 2004 – 2013

33. DDx for pneumomediastinum Trauma ARDS Pneumonitis
Necrotising mediastinitis
DDx for unilateral pleural effusion
Malignancy
Infection
Haemorrhage
Iatrogenic
DDx for loculated unilateral pleural effusion
Tuberculosis
Haemorrhage
Empyema

34. Endoscopic vacuum therapy