Dear editor,
Boerhaave syndrome, firstly described by Hermann Boerhaave in 1724, is a rare and life-threatening condition characterized by spontaneous transmural tear of the oesophagus.[1] The early diagnosis of Boerhaave syndrome is extremely important since a delay in the diagnosis can increase the mortality rate.[2] Here, we reported a Boerhaave syndrome patient combined with tension hydropneumothorax.
CASE
A 48-year-old man was brought to our emergency department (ED) complaining of epigastric pain, vomiting, dark-colored stools for one day, and worsening chest pain for nine hours. He previously had been well.
His symptoms began one day prior to the arrival in ED with nausea followed by several episodes of forceful emesis, then progressed to black-colored stools and intermittently vomiting black-colored material. About nine hours prior to the arrival in ED, the patient developed persistent lower thoracic and upper abdominal pain associated with dizziness, fatigue, and dyspnea. Social history was remarkable for 1,000 mL daily intake of alcoholic spirits for the past seven years. Physical examination revealed a middle-aged male in obvious painful distress, afebrile, tachycardia (168 beats per minute) with normal blood pressure (BP, 109/47 mmHg [1 mmHg=0.133 kPa]) and rapid respiratory rate (35 breaths per minute), and pulse oximetry cannot be measured.
Five minutes after arrival to our ED, he vomited once again, followed by a sudden loss of consciousness with no palpable pulses. Cardiopulmonary resuscitation (CPR) was started immediately. After 13 minutes of resuscitation, including mask ventilation for three minutes, endotracheal intubation, and positive pressure ventilation, the patient was successfully resuscitated (heart rate 156 beats per minute, oxygen saturation [SpO2] 91% on 100% fraction of inspired oxygen [FiO2], and BP 87/56 mmHg).
On examination, the patient had both midrange neck swelling and subcutaneous crepitation. His abdomen was diffusely tender with normoactive bowel sounds. Arterial blood gas was as follows: pH 7.02, partial pressure of carbon dioxide (PCO2) 54 mmHg, partial pressure of oxygen (PO2) 53 mmHg, and serum lactic acid 12.6 mg/dL. The white blood cell count was 6.37×109/L. The red blood cell was normal (117 g/L). Potential kidney injury was noted by an elevated serum creatinine (2.6 mg/dL) and blood urea nitrogen (66 mg/dL). The levels of glucose (8.8 mmol/L) and potassium (5.1 mmol/L) were slightly elevated. Mallory-Weiss syndrome or aortic dissection was initially suspected, so an aortic-enhanced computed tomography (CT) was performed. The image indicated subcutaneous emphysema, mediastinal emphysema, bilateral pleural cavity-free air, and large left-sided hydropneumothorax with the air around the esophagus (Figure 1). These findings strongly suggested spontaneous esophageal perforation.
Figure 1.
Figure 1.
The aortic-enhanced computed tomography performed on the patient.
During resuscitation, 520 mL of black-colored fluid was extracted via a nasogastric tube. The left-sided emergent closed thoracic drainage with a 32-F chest tube was then performed, which produced about 650 mL of thick and black-colored fluid. The patient received intravenous hydration, large doses of vasopressors, and broad-spectrum antibiotics. Emergent surgery was considered, but given the patient's condition and high surgical risk, his son refused further interventions. The patient died due to acute circulatory failure within 24 hours after ED arrival.
DISCUSSION
In practice, the “H's and T's” are potentially reversible causes of cardiac arrest. Timely correction of a reversible cause of cardiac arrest may lead to a good prognosis.[3] Cardiac arrest in this case may attribute to several different factors. Firstly, it is reasonable that acidosis from shock and hypoxia causes cardiac arrest. Secondly, tension pneumothorax, a complication of spontaneous esophageal rupture, can also cause cardiac arrest. It is likely that our patient suffered cardiac arrest due to tension hydropneumothorax. In addition, the possibility of cardiac arrest caused by circulatory shock should also be considered.
Boerhaave syndrome is usually associated with the excessive indulgence of food or alcohol. Our patient initially had dark-colored vomitus with a past medical history of alcoholism, which may suggest spontaneous esophageal rupture. Prolonged alcohol use can lead to severe vomiting. The study showed that lower esophageal tears might involve a spectrum of disease from Mallory-Weiss syndrome to full-thickness perforation.[4] Autopsy examinations revealed that a Mallory-Weiss tear caused by barotrauma from severe vomiting or air insufflation during endoscopy might evolve into a full-thickness rupture.[5]
A delay in diagnosis and treatment of Boerhaave syndrome is associated with significantly higher morbidity and mortality. CT abnormalities in Boerhaave syndrome include extraluminal air, peri-esophageal fluid, esophageal thickening, and extraluminal contrast. These CT findings can be the first clue to the diagnosis of esophageal perforation.[6] Contrast CT is considered the ‘‘golden standard’’ for diagnosing spontaneous esophageal rupture in suspected patients.[7] Traditionally, an esophagram performed with a water-soluble contrast agent can be helpful for the diagnosis of Boerhaave syndrome. Suzuki et al[8] reported a case of Boerhaave syndrome that was confirmed via thoracic drainage, which revealed bloody fluid and food residue in the drainage bag. Therefore, thoracic drainage can be used as a strategy for diagnosis and treatment in some cases.
The reported mortality rate of esophagus perforation is estimated to be 35%-40%. A favorable prognosis is associated with early definitive surgical treatment within 12 hours of rupture. If the intervention is delayed above 24 hours, the mortality rate (even with surgical intervention) rises to over 50% and reaches nearly 90% after 48 hours. If left untreated, the mortality rate is close to 100%.[8,9] Endoscopic stent insertion provides a promising approach for some Boerhaave syndrome patients, but it is associated with a high rate of treatment failure and further surgical intervention.[10] Prompt surgical repair and drainage can play an important role in management.[11]
This case presented with Mackler's triad, tension hydropneumothorax and cardiac arrest, and was confirmed to have Boerhaave syndrome on contrast CT and through thoracic drainage within 12 hours of ED arrival. Even though initial resuscitation efforts were successful, he still died of acute circulatory failure. While paying attention to the possibility of Mallory-Weiss syndrome, aortic dissection, or gastrointestinal bleeding, hydropneumothorax should alert providers to the possibility of Boerhaave syndrome. Thoracic drainage is a useful measure to confirm Boerhaave syndrome rapidly and can relieve pressure inside the thoracic cavity. The lack of prompt treatment can significantly increase morbidity and mortality despite a timely diagnosis.
Funding: None.
Ethical approval: The study was approved by the Ethics Committee of the hospital.
Conflicts of interest: The authors declare that there are no conflicts of interest regarding the publication of this paper.
Contributors: CTW and HJ contributed equally to this work. All authors contributed substantially to the writing and revision of this manuscript and approved of its contents.
Reference
Honoring the Admiral: Boerhaave-van Wassenaer’s syndrome
PMID:16722990
[Cited within: 1]
Dr. Herman Boerhaave (1668-1738) first described esophageal rupture and the subsequent mediastinal sepsis based upon his careful clinical and autopsy findings and hundreds of references have since been written about Boerhaave's syndrome. Several fine historical accounts of this brilliant scientist have been published over the years and he has received appropriate credit for his valuable contributions. But what about that unfortunate propositus that Dr. Boerhaave attended to, performed necropsy upon, and subsequently received acclaim with? Medical history pays inadequate regard to the Baron Jan Gerrit van Wassenaer heer van Rosenberg, Prefect of Rhineland and Grand Admiral of the Dutch Fleet. This figure was a nobleman and war hero at the peak of the Dutch Golden Age who played his role in steering the course of European history. Without this nobleman's heroic contemporaneous account, Boerhaave's celebrated impact on medical science would never have been realized. Therefore, we offer an overdue recitation of Admiral van Wassenaer's biography. Based on found precedent we propose that spontaneous rupture of the esophagus be henceforth referred to as the 'Boerhaave-van Wassenaer's syndrome'.
Evolving options in the management of esophageal perforation
PMID:15063302
[Cited within: 1]
Esophageal perforation remains a devastating event that is difficult to diagnose and manage. The majority of injuries are iatrogenic and the increasing use of endoscopic procedures can be expected to lead to an even higher incidence of esophageal perforation in coming years. Accurate diagnosis and effective treatment depend on early recognition of clinical features and accurate interpretation of diagnostic imaging. Outcome is determined by the cause and location of the injury, the presence of concomitant esophageal disease, and the interval between perforation and initiation of therapy. The overall mortality associated with esophageal perforation can approach 20%, and delay in treatment of more than 24 hours after perforation can result in a doubling of mortality. Surgical primary repair, with or without reinforcement, is the most successful treatment option in the management of esophageal perforation and reduces mortality by 50% to 70% compared with other interventional therapies.
European Resuscitation Council Guidelines for Resuscitation 2015: Section 4. Cardiac arrest in special circumstances
DOI:10.1016/j.resuscitation.2015.07.017 URL [Cited within: 1]
An unusual presentation of Boerhaave Syndrome: a case report
DOI:10.4076/1757-1626-2-8000 URL [Cited within: 1]
Transition of a Mallory-Weiss syndrome to a Boerhaave syndrome confirmed by anamnestic, necroscopic, and autopsy data: a case report
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The life-saving emergency thoracic endovascular aorta repair management on suspected aortoesophageal foreign body injury
DOI:10.5847/wjem.j.1920-8642.2020.03.004 URL [Cited within: 1]
Diagnostic performance of CT esophagography in patients with suspected esophageal rupture
DOI:10.1007/s10140-014-1222-4
PMID:24748526
[Cited within: 1]
Esophageal rupture is a surgical catastrophe. The gold standard for diagnosing is iodine, water-soluble contrast medium esophagography. CT esophagography has shown promising results. This study aimed to assess the diagnostic performance of CT esophagography in patients with a suspicion of esophageal rupture. This prospective study assessed the performance of a diagnostic test and was approved by local IRB committee. Patients who presented with a clinical suspicion of esophageal rupture were included. CT esophagography findings were described by the emergency radiologist. Clinical outcomes (presence or absence of esophageal rupture) were reported by surgeons. The operative characteristics were calculated. A final predictive scale for rupture was built. A total of 64 patients were recruited (age 26.5 years, 90 % male, 82 % trauma). Sensitivity, specificity, and positive and negative likelihood ratios (LRs) were 77.7 % (95 % confidence interval (CI) 45-100), 94.3 % (87.2-100), 14 (9.81-19.9), and 0.24 (0.05-1.22), respectively. The final model for predicting rupture included five variables: age (odds ratio (OR) 1.03; 95 % CI, 0.95-1.11; p=0.04), leakage of contrast media into the mediastinum or pleural space (OR 10.0; 95 % CI, 0.64-156.9; p=0.10), extraluminal air or fluid collections (OR 43.1; 95 % CI, 1.52-1217.3; p=0.027), esophageal wall thickening (OR 10.1; 95 % CI, 0.50-202.8; p=0.12), and left pneumothorax or pleural effusion (OR 6.5; 95 % CI, 0.31-132.7; p=0.2). The overall agreement was 0.40 (95 % CI, 0.09-0.72) for the predictive model. The model sensitivity was 50.0 %, and the specificity was 98.4 %. CT esophagography shows a good diagnostic performance in patients with a suspected esophageal rupture.
A case of rapid diagnosis of Boerhaave syndrome by thoracic drainage
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The influence of the “golden 24-h rule” on the prognosis of oesophageal perforation in the modern era
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Endoscopic stent insertion versus primary operative management for spontaneous rupture of the esophagus (Boerhaave syndrome): an international study comparing the outcome
PMID:23711276
[Cited within: 1]
Spontaneous rupture of the esophagus (Boerhaave syndrome) is an extremely rare, life-threatening condition. Traditionally surgery was the treatment of choice. Endoscopic stent insertion offers a promising alternative. The aim of this study was to compare the results of primary surgical therapy with endoscopic stenting. A British and a German high-volume center for esophageal surgery participated in this retrospective study. At the British center, operative therapy (primary repair or surgical drainage) was routinely carried out. Endoscopic stent insertion was the primary treatment option at the German center. Only patients with nonmalignant, spontaneous rupture of the esophagus (Boerhaave syndrome) were included. Demographic characteristics, comorbidity, clinical course, and outcome were analyzed. The study comprises 38 patients with a median age of 60 years. Time between rupture and treatment was less than 24 hours in 22 patients. Overall mortality was four of 38. Diagnosis greater than 24 hours was associated with higher risk for fatal outcome (odds ratio [OR], 4.64; 95% confidence interval [CI], 0.33 to 265.79). The surgery (S) and the endoscopic stent group (E) included 20 and 13 cases, respectively. Esophagectomy was unavoidable in three cases and two were managed conservatively. There were no significant differences in age, time to diagnosis less than 24 hours, intensive care unit days, hospital stay, sepsis, renal failure, slow respiratory weaning, or presence of comorbidity between the two groups. In 11 of 13 in the stent group, operative intervention (video-assisted thoracic surgery, thoracotomy, mediastinotomy) was eventually mandatory and three of 13 even required repeated surgery. The rate of reoperation in the surgery group was six of 20. Mortality was two of 13 (E) versus one of 20 (S). The odds for fatal outcome were 3.3 times higher in the stent group than in the surgery group (OR, 3.32; 95% CI, 0.15 to 213.98). Management of Boerhaave syndrome by means of endoscopic stent insertion offers no advantage regarding morbidity, intensive care unit or hospital stay, and is associated with frequent treatment failure eventually requiring surgical intervention. Furthermore, endoscopic stenting shows a higher risk for fatal outcome than primary surgical therapy.
The surgical management of spontaneous esophageal perforation (Boerhaave’s syndrome) 20 years of experience
DOI:10.5582/bst.2016.01009
PMID:27052150
[Cited within: 1]
Spontaneous esophageal perforation (Boerhaave's syndrome) is an uncommon and challenging condition with significant morbidity and mortality. Surgical treatment is indicated in the large majority of cases and different procedures have been described in this respect. We present the results of a mono-institutional evaluation of the management of spontaneous esophageal perforation over a 20-year period. The charts of 25 patients with spontaneous esophageal perforation treated at the Surgical Department of the University Hospital of Lausanne were retrospectively studied. In the 25 patients, 24 patients were surgically treated and one was managed with conservative treatment. Primary buttressed esophageal repair was performed in 23 cases. Nine postoperative complications were recorded, and the overall mortality was 32%. Despite prompt treatment postoperative morbidity and mortality are still relevant. Early diagnosis and definitive surgical management are the keys for successful outcome in the management of spontaneous esophageal perforation. Primary suture with buttressing should be considered as the procedure of choice. Conservative approach may be applied in very selected cases.
