A Case of Boerhaave Syndrome


Medical Student Clinical Pearl by Jillian Allan

MD Candidate, Class of 2024

Dalhousie University

Reviewed by Dr. R Goss

Copy Edited by Dr. J Vonkeman

Pdf Download: EMSJ JAllan A Case of Boerhaave Syndrome

 


Case Presentation

44-year-old male presents to the ER with a 5-hour history of retrosternal chest pain and recent onset shortness of breath. He was out drinking the previous night and has been profusely vomiting since 5am.


Differential Diagnosis

A variety of conditions may present in this fashion:

  • GERD/Gastritis/ Esophagitis/Gastric ulcer
  • Pneumothorax
  • Aortic dissection
  • Acute pancreatitis
  • ACS/MI
  • Cannabis hyperemesis syndrome
  • Esophageal rupture

History and Physical

Upon arrival to the ER, he is hemodynamically unstable: tachycardic (125), hypotensive (90/58) and febrile (38.2 C). His O2 sats are 86% on RA. He has no history of gastroesophageal reflux or other relevant medical conditions. He does not use cannabis.

On examination, his abdomen is soft, he is tender in the left upper quadrant and diffusely across his chest wall. Breath sounds are decreased on the leftIn addition, he has bilateral supraclavicular crepitus on palpation and a positive Hamman’s crunch (mediastinal crackling, synchronous with the heartbeat) on auscultation. His neck is becoming increasingly distended, and you have noticed a change in phonation since his arrival.


Etiology 

Boerhaaves syndrome is most commonly caused by profuse vomiting but can also be the result of anything that increases esophageal pressure such as weightlifting, seizures, abdominal trauma, locally invasive cancers/infections, childbirth, or compressed air injuries.7


Pathophysiology 

  • Esophageal perforations are classified into 3 groups:
    • Cervical esophagus: can present with neck tenderness, dysphagia, or dysphonia
    • Thoracic esophagus: presents with severe back pain, pleuritic, chest or epigastric pain, inability to lie supine. Most common area for perforation.
    • Intra-abdominal esophagus: Peritonitis

 

  • Severity of perforation tends to depend on the location of rupture, with intrathoracic esophageal ruptures leading to more devastating outcomes.
    • Intrathoracic rupture results in contamination of the thoracic cavity with gastric contents, which can lead to chemical mediastinitis, infection and mediastinal necrosis.6
    • Barogenic rupture of the cervical esophagus has a more benign course, as the spread of contamination to the mediastinum is slow and attachments of the esophagus to the prevertebral fascia limit the lateral dissemination of esophageal flora.6


Evaluation

  • Diagnosis is established through a computed tomography (CT) scan of the chest or contrast enhanced esophagram. Contrast should be water soluble (gastrografin) to avoid mediastinal contamination with barium contrast.
    • CT: Findings suggestive of esophageal rupture include esophageal wall edema and thickening, peri-esophageal fluid, mediastinal widening, and free air/fluid within the pleural spaces, retroperitoneum, or lesser sac.6
    • Radiography: Plain films may also demonstrate air in the soft tissues of the prevertebral space. Other indications can include pleural effusion, hydropneumothorax, mediastinal widening or subdiaphragmatic air.6 While thoracic and cervical radiography can aid in diagnosis, they cannot exclude or confirm esophageal rupture and should not routinely be performed to diagnose this condition. However, a plain radiograph may be performed, and mediastinal air found incidentally when the diagnosis had not been suspected.
    • Esophagram: Reveals the location and extent of perforation of the esophagus by the extravasation of the contrast medium.6
  • Endoscopy should be performed with caution due to the risk of further esophageal damage.

Case Continued

Laboratory results showed elevated leucocytes at 12.9 x 109/L (normal 4.5-11.5) and an elevated C-reactive protein level but were otherwise unremarkable.

An erect chest radiograph and urgent CT was done, which showed the “V” sign of Naclerio, a V shaped collection of air along the mediastinum and diaphragm, indicating pneumomediastinum (Fig.1a).2 An urgent contrast CT confirmed the radiograph findings, showing pneumomediastinum and left hydropneumothorax (Fig. 1b).2

 

Figure 1. Boerhaave syndrome in a 44-year-old man. (A) Chest radiograph showing Naclerio’s V sign, demonstrating air outlining the mediastinal borders (arrows), indicating pneumomediastinum. (B) Chest CT showing pneumomediastinum and left hydropneumothorax.2

  

Esophageal perforation was confirmed with a contrast esophagram, which showed leakage from the lower esophageal sphincter into the left pleural space.

 

Figure 2. Contrast esophagram showing esophageal rupture at lower esophageal sphincter with leakage into the left pleural space.1


Treatment and Management

  • Mainstay of treatment includes volume resuscitation, broad-spectrum antibiotic coverage, and surgical evaluation.
  • 3 treatments options: conservative, endoscopic, or surgical
    • Conservative: typically reserved for small or contained ruptures.
    • Endoscopic: stent placement to prevent fistula formations or seal esophageal leaks.
    • Surgical: primary esophageal repair via open thoracotomy vs VATS (video-assisted thoracoscopic surgery) with fundic reinforcement- which is the gold standard of treatment if within 24 hours.7

Case Conclusion

The patient underwent an emergency VATS procedure which revealed a small tear in the lower esophagus, which was successfully repaired with sutures and a pleural patch. The patient made an uneventful recovery and was discharged on postoperative day 6.


Summary of Key Points


References

  1. Calvin S.H. Ng, Wilfred L.M. Mui and Anthony P.C. Yim. Barogenic esophageal rupture: Boerhaave Syndrome. CAN J SURG December 01, 2006 49 (6) 438-439;
  2. Chew, Fatt Yang; Yang, Su-Tso. Boerhaave Syndrome. CMAJ 2021 September 27;193:E1499. doi:10.1503/cmaj.202893.
  3. Kassem MM, Wallen JM. Esophageal Perforation And Tears. [Updated 2022 Aug 8]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK532298/
  4. Kukuruza K, Aboeed A. Subcutaneous Emphysema. [Updated 2022 Jul 25]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK542192/
  5. Rawla P, Devasahayam J. Mallory Weiss Syndrome. [Updated 2022 Oct 9]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK538190/
  6. Triadafilopoulos, G. Boerhaave Syndrome: Effort rupture of the esophagus. In: UpToDate, Waltham, MA. (Accessed on October 29th, 2022).
  7. Turner AR, Turner SD. Boerhaave Syndrome. [Updated 2021 Dec 15]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK430808/
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