Septic shock revealing boerhaave's syndrome a case report

Abstract

Introduction and importance

Boerhaave's syndrome, recognized as spontaneous esophageal rupture, is an uncommon and perilous medical condition marked by the spontaneous tearing of the esophagus. This paper highlights the importance of an early diagnosis and its correlation to better outcomes for a rare pathology with high mortality.

Clinical presentation

A 67-year-old female presenting with unexplored vomiting and hypertension, presented to the ER with a septic shock. The patient's clinical deterioration prompted emergency exploration, revealing a dilated esophagus with a 3-cm perforation. Despite surgical intervention, including suturing with a T-tube and esophageal exclusion, the patient succumbed to multiorgan failure.

Clinical discussion

Boerhaave's syndrome, triggered by forceful vomiting, presents diverse clinical manifestations, making accurate diagnosis challenging. The characteristic triad of vomiting, pain, and subcutaneous emphysema is observed in a minority of cases, often overshadowed by acute respiratory distress. Diagnostic modalities include chest X-rays, contrast esophagography, and computed tomography, aiding in visualizing contrast leakage and confirming the diagnosis.

The choice of surgical technique, ranging from esophageal suturing to esophagectomy, depends on the duration between rupture and surgery initiation. In this case, a bipolar esophageal exclusion was performed due to the patient's critical condition.

Conclusion

Boerhaave's syndrome demands consideration in patients presenting with thoracic pain and vomiting, particularly in those with a pathological esophagus. Early diagnosis and surgical intervention remain pivotal in improving outcomes. Identification of hydro-pneumothorax in radiographic studies should prompt consideration of spontaneous esophageal rupture, highlighting the need for heightened clinical suspicion in nonspecific clinical scenarios.

Highlights

• •BOERHAAVE'S SYNDROME, A SPONTANEOUS ESOPHAGEAL RUPTURE, IS A RARE AND LIFE-THREATENING CONDITION REQUIRING PROMPT DIAGNOSIS AND INTERVENTION.
• •CLINICAL MANIFESTATIONS VARY, WITH THE CHARACTERISTIC TRIAD OF VOMITING, PAIN, AND SUBCUTANEOUS EMPHYSEMA OBSERVED IN A MINORITY OF CASES, OFTEN OVERSHADOWED BY ACUTE RESPIRATORY DISTRESS.
• •EARLY DIAGNOSIS AND SURGICAL INTERVENTION ARE CRUCIAL FOR IMPROVING OUTCOMES, AND THE IDENTIFICATION OF HYDRO-PNEUMOTHORAX IN RADIOGRAPHIC STUDIES SHOULD PROMPT CONSIDERATION OF SPONTANEOUS ESOPHAGEAL RUPTURE.
• •THE CHOICE OF SURGICAL TECHNIQUE DEPENDS ON THE DURATION BETWEEN RUPTURE AND SURGERY INITIATION, WITH OPTIONS RANGING FROM ESOPHAGEAL SUTURING TO ESOPHAGECTOMY.
• •BOERHAAVE'S SYNDROME SHOULD BE CONSIDERED IN PATIENTS WITH THORACIC PAIN AND VOMITING, ESPECIALLY IN THOSE WITH A PATHOLOGICAL ESOPHAGUS.

1. Introduction

Boerhaave's Syndrome, also known as spontaneous esophageal rupture, is a rare and life-threatening condition that arises from increased intraesophageal pressure and weakened esophageal walls. Early diagnosis is essential to prevent complications like mediastinitis and Septic shock. This paper highlights the importance of timely medical intervention and its correlation to better outcomes for a rare pathology with high mortality [1,2].

This case report has been reported in line with the SCARE Criteria [3].

2. Case report

A 67-year-old female patient with a history of unexplored vomiting and hypertension was admitted to the emergency department in a state of septic shock. Upon examination, the patient presented with a fever of 38.7 °C, a heart rate of 110 bpm, and a blood pressure of 90/50, requiring administration of norepinephrine due to hypotension. Subcutaneous emphysema was also noted. White blood cell count of 8400, CRP level of 243, and hemoglobin level of 7.2 g/dL. Arterial blood gas analysis revealed metabolic acidosis with a lactate level of 8.

The patient was admitted to surgical intensive care and placed on broad-spectrum antibiotic therapy. A central venous catheter was inserted to promptly initiate catecholamine, along with standard intravenous infusion to correct electrolyte imbalances caused by vomiting.

An emergency chest X-ray revealed an air-fluid level in the right lung. Hydro-pneumothorax (Fig. 1) was suspected, and a thoracic drainage was performed, immediately retrieving 700 cc of gastric stasis fluid. Considering the patient's history of vomiting, shock, subcutaneous emphysema on examination, and the presence of digestive fluid after thoracic drainage, esophageal perforation was strongly suspected.

Fig. 1.

Chest X-ray revealing an air-fluid level in the right lung.

After stabilization computed tomography scan confirmed a dilated esophagus with a 3-cm perforation on the right lateral wall. Contrast leakage was observed at this site during opacification (Fig. 2). Emergency surgery via right lateral posterior thoracotomy revealed purulent pleural effusion mixed with undigested food (Fig. 3). The esophagus was dilated with a 3-cm perforation on the right lateral aspect located in the midsection of the esophagus, below the azygos vein arch. (Fig. 4), which was sutured using a T-tube (Fig. 5), exclusion of the abdominal esophagus was performed with a TA-55 stapler, followed by Witzel jejunostomy, abdominal decompression gastrostomy, and cervical esophagostomy. The procedure was done by MD-PHD in a University Hospital Center but with limited expertise in esophageal pathology.

Fig. 2.

Computed tomography scan showing contrast leakage.

Fig. 3.

Purulent pleural effusion mixed with undigested food.

Fig. 4.

Perforation on the right lateral aspect of the esophagus.

Fig. 5.

Perforation reparation using a T-tube.

The microbiological sample concluded the presence of Candida albicans. The patient remained intubated in the postoperative period, requiring controlled assisted ventilation. The noradrenaline tapering was done gradually, but a low dose was still necessary to maintain a good mean arterial pressure. The patient was kept on antibiotic therapy with ceftriaxone and metronidazole, along with fluconazole for Candida albicans. Unfortunately, the patient rapidly shifted to multiple-organ system failure (MOSF) and eventually succumbed.

3. Discussion

Boerhaave's syndrome is triggered by vigorous vomiting, causing a sudden increase in intraluminal pressure and resultant esophageal barotrauma. The clinical presentation of Boerhaave's syndrome is diverse, posing challenges for accurate diagnosis. The characteristic triad (comprising vomiting, pain, and subcutaneous emphysema) is observed in merely 20 % of cases [4]. In the majority of instances, the clinical focus shifts rapidly to signs of acute respiratory distress, indicative of pleural involvement. For instance, our patient presented to the ER with difficulty breathing and rapidly shifted to tachycardia hypotension with fever associated with vomiting and subcutaneous emphysema, inducing septic shock.

In the context of an emergency, a combination of patient history, clinical examination, electrocardiogram, and non-invasive supplementary tests is instrumental in excluding alternative diagnoses for thoracic pain these differential diagnoses may vary and include conditions associated with shock, ultimately aiding in the identification of mediastinitis. Notably, frontal chest X-rays reveal abnormalities in nearly 91 % of cases [4], indicating mediastinal enlargement, unilateral or bilateral pleural effusion, pneumothorax, or hydropneumothorax. Confirmation of the diagnosis involves water-soluble contrast esophagography, which illustrates contrast leakage into the pleura or mediastinum [5]. Emergency thoracic computed tomography with water-soluble contrast opacification also plays a crucial role [4,6], providing a means to visualize contrast extravasation through the esophageal breach. However, digestive endoscopy is contraindicated due to its unreliability and potentially traumatic nature [7]. Confirming these statements, an emergency chest X-ray revealed an air-fluid level in the right lung. A thoracic drainage was then performed due to the patient's condition, immediately retrieving 700 cc of gastric stasis fluid. Considering the high probability of esophageal perforation, a computed tomography scan confirmed a perforation on the right lateral wall. Contrast leakage was observed at this site during opacification.

Boerhaave's syndrome primarily affects men (constituting 80 % of cases) aged between 50 and 70, commonly characterized by obesity, hearty eating habits, and frequent chronic alcoholism [8]. Surprisingly our patient was a 67-year-old woman who didn't have any of these factors aside from age.

Many of these patients often present with a pre-existing pathological esophagus, which seems to act as a predisposing factor for Boerhaave's syndrome, including conditions like peptic esophagitis, gastroduodenal ulcer, gastroesophageal reflux, and hiatal hernia [9]. The coexistence of esophagitis and hiatal hernia may triple the susceptibility of the esophageal wall [5]. Recurrent episodes of vomiting can result in Mallory-Weiss-type mucosal ulceration. This particular syndrome, characterized by a partial longitudinal rupture of the esophageal mucosa, could potentially signify an early stage of Boerhaave's syndrome [10]. The pre-existence of a hiatal hernia emerges as a predisposing factor for Mallory-Weiss syndrome and seems to influence the location of this lesion [[11], [12], [13]]. In our case we suspected that the patient had achalasia since the CT scan findings confirmed a dilated esophagus, we reported a recent case of spontaneous perforation in a patient with achalasia [14] that had some similarity with our case.

Therefore what differentiates the two cases is that our patient didn't have a known history of achalasia but the CT scan and operative finding showed a large esophagus suggesting that the patient potentially had achalasia but wasn't aware of this condition.

The prognosis of Boerhaave's syndrome is significantly influenced by early surgical intervention, and the choice of surgical techniques (such as suturing the esophageal breach, bipolar esophageal exclusion, esophagectomy, percutaneous or thoracotomy drainage) depends on the duration between rupture and the initiation of surgery [6,7]. In our case unfortunately the patient was already in septic shock when routed to the operating room thus we decided to perform a bipolar esophageal exclusion and to aid the healing of perforation we sutured with a T tube for a directed fistulation.

4. Conclusion

Boerhaave's syndrome is a rare and extremely serious condition, the diagnosis of which remains challenging, early surgical intervention is a crucial determinant of prognosis.

The medical treatment of septic shock and visceral failure is considered an essential adjunct to any surgical procedure.

Therefore, consideration of Boerhaave's syndrome is warranted in the presence of any thoracic pain associated with vomiting, especially in patients with a pathological esophagus.

As well the identification or discovery of hydro-pneumothorax (via unprepared radiography or computed tomography) during a clinical scenario that may sometimes be nonspecific should prompt consideration of the diagnosis of spontaneous esophageal rupture.

Our cases highlighted a rare case of Boerehave's syndrome in a patient with achalasia, a pathology rarely associated with this condition. The postoperative course was complicated due to septic shock upon admission, resulting in a high mortality rate, making early diagnosis and surgical intervention in this pathology mandatory.

Consent

Written informed consent was obtained from the patients for publication of this case series and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.

Ethical approval

Our institutions “la Rabta Hospital” and “School of Medicine of Tunis” require no ethical approval for case series. It is required for studies on human participants. This is just a case series with written patient approval.

Funding

No funding.

Author contribution

Amine Sebai: conceptualization, data curation, redaction, project manager.

Elaifia Rany: conceptualization, data curation, redaction, project manager.

Souhaib Atri: conceptualization, redaction.

Mariem ben brahim: resources, visualization.

Haddad Anis: resources, visualization.

Montassar Kacem: supervision, validation, visualization.

Guarantor

Elaifia rany.

Research registration number

Not applicable.

Conflict of interest statement

All authors declare that they have no conflicts of interest.