Management of boerhaave’s syndrome in the intensive care unit

ABSTRACT

Boerhaave’s syndrome (BS), or spontaneous esophageal perforation, is a rare and life-threatening condition. Despite advancements in medical and surgical management, BS carries a high mortality rate, up to 50%, and presents significant diagnostic and therapeutic challenges. Management options include both surgical and nonoperative approaches, with the Pittsburgh score providing a useful tool for predicting outcomes and guiding treatment. Complications such as mediastinitis and sepsis are common. This review explores the demographics, risk factors, clinical presentation, diagnostic modalities, and management strategies for BS. Furthermore, it stresses the importance of early diagnosis and individualized treatment to improve the outcomes in patients with BS.

INTRODUCTION

Boerhaave’s syndrome (BS), also defined as perforation of the esophagus spontaneously, was first reported in 1729 and described by Herman Boerhaave.[1] The rare presentation of BS and lethal outcomes due to tear in the esophagus, under the preview of increased pressure in the esophagus, in the absence of any pertinent cause such as an iatrogenic injury or trauma, makes it a significant health concern in the surgical department to discuss.[2,3] Despite surgical intervention, morbidity and mortality remain high, with mortality reported up to 50%; however, a few case reports have documented successful outcomes with non-operative management (NOM).[4,5]

Age presentation may range from 45 to 70 years, with predominantly male population.[2] Risk factors include excess of alcohol use or any psychiatric disorder.[2] Other risk factors are weight lifting, overeating, seizures, abdominal injury, childbirth among women, and defecation.[6]

Pain in the chest, vomiting, and emphysema at the subcutaneous level are the most common symptoms with which the patient present to the emergency room. The three symptoms have been clubbed together as Mackler’s triad, which is visible in up to 14% of cases.[7] Although these three symptoms are common, the rarity of presentation of these three symptoms (as present in only 14% cases) restricts the diagnostic ability of BS, and it becomes challenging to diagnose it under specific signs and symptoms. Other symptoms are pain in abdomen, uncontrollable vomiting, dyspnea, dysphagia, or systemic signs of sepsis.[8,9]

BS is usually seen as an esophageal perforation in the left side of the lower-third of the esophagus, which is around 2–4 cm distant from the esophageal junction. This area is prone to rupture because the muscle fibers in this area are very thin, and the esophageal wall is weakened to no local protection.[2]

LITERATURE REVIEW

We carried out an extensive review of case reports examining literature from Cochrane Library, MEDLINE, PubMed, and Google Scholar and searched articles from 2012 till date. Only studies published in English were considered. The search strategy incorporated the terms “Boerhaave syndrome” and “spontaneous esophageal perforation.” Titles and abstracts of relevant articles were studied for content specifically related to these conditions.[2,6,10,11,12,13,14,15,16,17,18,19,20,21,22,23,24,25,26,27]

DIAGNOSIS

Diagnostically, imaging is very helpful under which computed tomography (CT) scan has shown sensitivity and specificity of 92% and, in some cases, up to 100% for diagnosing BS as an esophageal tear. An esophagogram has also found its importance for determining the location of leak in the esophagus. In addition, esophageal X-ray and contrast-enhanced CT are also done. Besides allowing for knowing the location and any rupture in esophagus, they also extend their diagnostic ability to know any involvement of pleural layer in the form of hydrothorax, pneumothorax, or pleural effusion or mediastinitis or peritoneal sepsis, and this also allows to rule out and diagnostically differentiate such conditions from BS.[2,28,29,30]

MANAGEMENT

Management of the BS has followed a major shift from open repair to minimal thoracoscopic approach in the surgical management. Besides, certain patients—such as the very elderly, those in severe shock, or individuals with significant comorbidities—who are not suitable candidates for surgery, may be managed conservatively using antibiotics, thoracic drainage, endoluminal drainage, and appropriate nutritional support.[31,32,33]

Surgical management is highly time-sensitive, with mortality rates as low as 36% when performed within 12 hours of diagnosis, rising sharply to approximately 64% when delayed beyond 24 hours. Approaches continue to evolve from open to thoracoscopic approach. Even the risk of leakage goes on increasing from 60 to 80% with a time delay extending from <6 h to more than 24 h. Furthermore, the primary healing rates show a significant fall from 88.9% if the surgery is conducted before 24 h to 0% if the surgery is conducted after 72 h.[33,34,35,36,37]

Technical points to note include extending the muscle rupture to expose the entire mucosa and remove any necrotic muscle tissue, avoiding a tight knot and keeping a margin for appropriate needle pitch, using vascular tissue flaps wherever possible for reinforcing the sutures for providing better healing and decreasing risk of leakage.[37]

Complications that warrant an immediate surgery include mediastinal inflammation, chest infection, and severe sepsis. Debridement and drainage become very important for patients who have severe sepsis. In certain cases, for facilitating postoperative internal nutrition, jejunostomy may be applied.[16]

A careful selection between conservative and surgical approaches is crucial in managing Boerhaave’s syndrome. Each hospital should adopt a practical management algorithm tailored to patient severity to enable effective triage. Key decision-making criteria should include time since diagnosis (particularly whether it is within 24 hours), presence of sepsis, and the extent of perforation or pleural involvement. These factors should guide treatment choice, with strict monitoring, broad-spectrum antibiotic therapy, and the use of endoscopic interventions where appropriate.[16]

Surgical intervention should be reserved for rare cases where medical management is not feasible, even in patients who do not meet the standard criteria for surgery, typically due to poor hemodynamic stability. In such scenarios, the choice between an open and a minimally invasive surgical approach must be made judiciously,[16] since minimally invasive techniques, particularly video-assisted thoracoscopic surgery (VATS), have gained widespread acceptance as they significantly reduce hospital stay, drainage duration, postoperative pain, stress-related morbidity, intraoperative bleeding, and respiratory complications - making it the preferred procedure of surgial approach.[38,39]

The steps include pleural lavage, triangular ligament dissection, debridement of the necrotic tissue, and defect suturing with a T-tube closure. Overall, it provides good recovery and improves the quality of life of patients with minimal postoperative pain and morbidity and better functional scores during the follow-up.[37]

Conservative medical management involves the use of antacids in the form of proton-pump inhibitors, broad-spectrum IV antibiotics, and keeping the patients nil per orally, so that any infection in the form of aerobic and anaerobic bacteria could be covered. During the time, nutritional support is recommended in the form of parenteral administration of fluids.[30]

Pittsburgh score (PS) is recently introduced as well as validated score focusing specifically on cases of esophageal perforation, especially those resulting from spontaneous rupture. This score is significant as it allows for the prediction of outcomes of the patients with BS.[31,32,33]

Schweigert et al.[34] reported an association between higher Perforation Severity (PS) scores and increased mortality, noting that patients with a PS score <2 had a mortality rate below 3%, while those with scores between 3 and 5 exhibited a mortality rate of 7%. Patients classified as high risk (PS >5) demonstrated a higher mortality rate, exceeding 37%. This approach not only demonstrates effective classification but is also helpful in guiding management decisions. For patients with a PS score of <2 and contained leak, NOM may be appropriate. However, for those with a PS score between 3 and 5, it is important to rule out any preexisting esophageal conditions before taking the patients for NOM – making them borderline cases in the management according to PS.[34]

CONCLUSION

BS is a critical surgical emergency with significant mortality. The rarity of the condition, coupled with its nonspecific presentation, often leads to delayed diagnosis, exacerbating the risk of severe complications such as mediastinitis and sepsis. Early recognition through advanced imaging and a thorough understanding of risk factors are essential for improving patient outcomes. The management of BS must be tailored to each patient, considering the extent of esophageal damage, patient stability, and associated comorbidities. Prompt intervention, whether surgical or conservative, is crucial to reducing morbidity and mortality. Continued research and case studies are needed to refine diagnostic criteria and management protocols, ensuring better outcomes for patients with BS.

Conflicts of interest

There are no conflicts of interest.

Funding Statement

Nil.