Mackler’s Triad to Suspect Boerhaave’s Syndrome

Archana Nair¹ https://orcid.org/0000-0001-6409-6038, Praveen Aggarwal² https://orcid.org/0000-0002-4611-5458

^1,2Department of Emergency Medicine, All India Institute of Medical Sciences (AIIMS), New Delhi, India

Corresponding Author: Archana Nair, Department of Emergency Medicine, All India Institute of Medical Sciences (AIIMS), New Delhi, India, Phone: +91 8547698676, e-mail: dr.archananair10392@gmail.com

Received: 12 July 2023; Accepted: 02 October 2023; Published on: 02 January 2024

ABSTRACT

Background: A case report of esophageal perforation presenting with the rare symptom triad of vomiting, chest pain, and subcutaneous emphysema (Mackler’s triad) and its diagnostic evaluation and management.

Case description: A 60-year-old patient presented to the emergency department (ED) with a history of two episodes of vomiting, followed by the development of chest pain, abdominal pain, and respiratory distress. On evaluation, the patient was tachypneic, tachycardic, and hypoxic. Subcutaneous emphysema was present in the neck and upper chest. As bedside ultrasound revealed bilateral pneumothorax, chest tubes were inserted on both sides, and the patient was intubated for severe respiratory distress. Computed tomography (CT) chest was suggestive of pneumomediastinum with bilateral pneumothorax. Based on the clinical and radiological evidence, a diagnosis of Boerhaave’s syndrome (BS) was made, and the patient was taken up for upper gastrointestinal endoscopy followed by exploratory laparotomy. Patient had a 3 cm long esophageal perforation in left lateral wall of lower esophagus near gastroesophageal junction. Despite aggressive management, patient succumbed on the seventh postoperative day.

Boerhaave’s syndrome (BS) is a potentially lethal condition that poses a major diagnostic and therapeutic challenge for the emergency physician. Spontaneous esophageal perforation presenting with the classical Mackler’s triad, that is, vomiting, chest pain, and subcutaneous emphysema is rare. A high index of suspicion is needed for prompt diagnosis, which, in turn, paves the way for early and appropriate management.

How to cite this article: Nair A, Aggarwal P. Mackler’s Triad to Suspect Boerhaave’s Syndrome. Indian J Crit Care Case Rep 2024;3(1):21–23.

Source of support: Nil

Conflict of interest: None

Patient consent statement: The author(s) have obtained written informed consent from the patient for publication of the case report details and related images.

Keywords: Boerhaave’s syndrome, Case report, Esophageal rupture, Pneumomediastinum, Pneumothorax

INTRODUCTION

Boerhaave’s syndrome (BS) was first identified by Herman Boerhaave and is named after him. It is a life-threatening surgical emergency where spontaneous esophageal rupture occurs. The incidence is approximately 3.1 per 1,000,000 persons per year.¹ Most often, vertical full-thickness rupture occurs, and the most common site is posterior aspect of the distal esophagus, commonly on the left side, 2–3 cm proximal to the gastroesophageal junction. BS occurs due to a sudden increase in pressure caused by forceful vomiting against a closed glottis.² Clinical features of BS vary with the level of the perforation, degree of perforation, and the time since injury.¹ The classic Mackler’s triad consists of vomiting, chest pain, and subcutaneous emphysema, which are seen in only 5% of cases.³–⁵ BS can cause complications like mediastinitis, empyema, septic shock, multiple organ dysfunction, and even death. Computed tomography (CT) esophagography is the investigation of choice. Surgery is the mainstay of treatment, whereas some prefer a more conservative, endoscopic, or less invasive surgical approach.¹ BS has a mortality rate of >90% without prompt diagnosis and treatment.⁶ Mortality has been reported up to 40% even after appropriate surgical intervention.¹

CASE DESCRIPTION

A 60-year-old gentleman without any prior comorbidities presented to the emergency department (ED) with complaints of two episodes of vomiting about 18 hours back, following which he developed sudden severe chest pain, diffuse abdominal pain, and shortness of breath. On evaluation, the patient was tachypneic, with room air saturation of 76%, and tachycardic. There was swelling over the face, neck, and torso with crepitus on palpation. On further evaluation with point-of-care ultrasound, lung sliding was absent on both sides with a barcode sign suggestive of bilateral pneumothorax for which bilateral chest tubes were placed. Patient was intubated in view of severe respiratory distress after placing bilateral chest tubes. The chest X-ray (CXR) revealed pneumopericardium (Fig. 1) and CT chest. Figure 2 shows pneumomediastinum, pneumopericardium, and subcutaneous emphysema. Lung window (Fig. 3) shows pneumothorax with a chest tube in situ. Combining the history, clinical examination, and radiological investigations, a diagnosis of BS was made. The patient was stabilized in the ED and taken up for upper gastrointestinal endoscopy, which revealed a 3 cm linear esophageal perforation in the lower esophagus in the left lateral wall near gastroesophageal junction with unhealthy mucosa and pus. Patient underwent exploratory laparotomy with cervical esophagostomy and stripping of esophagus, stapling of gastroesophageal junction along with tube gastrostomy, and feeding jejunostomy. Postoperatively, the patient was managed in the intensive care unit, but despite the aggressive management, patient succumbed on postoperative day 7 (Fig. 4).

Fig. 1: Chest X-ray (CXR) showing pneumopericardium

Fig. 2: Computed tomography (CT) chest revealed pneumomediastinum with bilateral pneumothorax, subcutaneous emphysema, and pneumopericardium

Fig. 3: Lung window showing pneumothorax and chest tube in situ

Fig. 4: Ultrasound M-mode image showing barcode sign of pneumothorax

DISCUSSION

Boerhaave’s syndrome (BS) is the effort rupture of esophagus. Clinical features include neck pain, chest pain, dysphagia, respiratory distress, nausea, vomiting, hoarseness, dysphonia, abdominal pain, etc. Up to 30% of patients develop mediastinal emphysema, with crepitus on palpation or the Hamman’s sign, which is a crunching sound heard during auscultation.³ In the abovementioned case, the patient had multiple vomiting episodes followed by chest discomfort and development of subcutaneous emphysema (Mackler’s triad). Patients with mediastinitis may present in septic shock.³ Pneumomediastinum, hydropneumothorax, hemopneumothorax, pleural effusion, and pneumopericardium can occur.⁴

Imaging is the diagnostic modality for BS. CXR can be normal in about 15% of cases.¹ CXR findings include subcutaneous or mediastinal emphysema, mediastinal widening, pneumothorax, pneumopericardium, pleural effusion, and the “Naclerio’s V sign” (a V-shaped air collection, surrounded by a left border of the aorta on one side and diaphragm forming the lower border).¹ The CT chest showed pneumopericardium, pneumomediastinum, and bilateral pneumothorax, which were clues to suspect BS in the ED. Diagnosis was confirmed by on-table upper gastrointestinal endoscopy (UGIE).

Surgery remains the most effective treatment for BS.⁵ Exploratory laparotomy was done in this case, and patient had a linear 3 cm long perforation in lower esophagus in left lateral wall near gastroesophageal junction. The mortality rate depends on the time since rupture and between diagnosis and treatment. If treatment is not started within 24 hours from the onset of symptoms, the mortality rate is 25%; after 24 hours, it is 65%; and after 48 hours, it is 75–89%.⁴

Boerhaave’s syndrome (BS) should be one differential diagnosis in all patients presenting with a history of vomiting, epigastric pain, and shortness of breath. BS is a potentially lethal condition, which is a diagnostic and therapeutic challenge. Diagnostic errors could be due to a lack of awareness of its atypical presentations, incomplete history, and clinical examination. A high index of suspicion is essential for prompt diagnosis. Clinicians should keep this as a differential diagnosis for life-threatening causes of chest pain and should be aware of the varied clinical presentations and the importance of early diagnosis and treatment.⁶

CONCLUSION

Esophageal perforation is a critical diagnosis. Any patient coming with a history of multiple episodes of retching/vomiting followed by epigastric pain or chest pain should be evaluated for esophageal perforation. It may not always have the classical Mackler’s triad. Timely detection and immediate treatment are very important to prevent the high degree of mortality associated with esophageal rupture.

ORCID

Archana Nair https://orcid.org/0000-0001-6409-6038

Praveen Aggarwal https://orcid.org/0000-0002-4611-5458

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