Clinical Challenges - October 2016: Boerhaave’s syndrome (spontaneous rupture of the esophagus)

The diagnosis

Gastrografin swallow (Figure C) demonstrated rupture of the distal esophagus, with leakage of gastrografin into the mediastinum (arrow). Upper gastrointestinal endoscopy confirmed rupture of the left posterolateral wall of the distal esophagus consistent with Boerhaave’s syndrome (Figure D), and a self-expanding covered metal stent was placed.

Broad-spectrum antibiotics and nasogastric feeding were commenced, and the left pleural effusion drained with a tube thoracostomy. Unfortunately, despite initial improvement, the patient subsequently deteriorated and died 30 days after admission.

Boerhaave’s is a rare clinical entity defined as spontaneous esophageal rupture, excluding perforations resulting from foreign bodies or iatrogenic instrumentation.

 It was first described by a Dutch physician, Herman Boerhaave, in 1724, about a Dutch Grand Admiral who overate and experienced spontaneous esophageal rupture after vomiting. It results from a sudden rise in intraluminal esophageal pressure from straining or vomiting, with failure of relaxation of the cricopharyngeus muscle. The left posterolateral wall of the esophagus is the most common site of perforation, owing to an anatomic weakness. More recently, an association with eosinophilic esophagitis has been reported, particularly in young men, and this underlying diagnosis should be considered, especially if there is a prior history of dysphagia and allergic manifestations.¹

Mackler’s triad of vomiting, lower chest pain, and subcutaneous emphysema is the classical presentation but is seen in only a minority of cases; thus, diagnostic errors are common.² Importantly, the chest radiograph is almost always abnormal, with pleural effusions or pneumomediastinum often seen.³ Surgical repair is the definitive treatment, but in patients considered unfit for surgery, conservative or endoscopic management is advocated. Mortality remains greater than 30%, and rises sharply if diagnosis is delayed^,2 emphasizing the importance of awareness of this unusual diagnosis.

References

1. Lucendo, A.J., Fringal-Ruiz, A.B., Rodriguez, B. Boerhaave’s syndrome as the primary manifestation of adult eosinophilic esophagitis. (Two case reports and a review of the literature.) Dis Esophagus. 2011 Feb;24:E11-5.

2. Brauer, R.B., Liebermann-Meffert, D., Stein, H.J., et al. Boerhaave’s syndrome: Analysis of the literature and report of 18 new cases. Dis Esophagus. 1997 Jan;10:64-8.

3. Pate, J.W., Walker, W.A., Cole, F.H. Jr, et al. Spontaneous rupture of the esophagus: a 30-year experience. Ann Thorac Surg. 1989 May;47:689-92.

The diagnosis

Gastrografin swallow (Figure C) demonstrated rupture of the distal esophagus, with leakage of gastrografin into the mediastinum (arrow). Upper gastrointestinal endoscopy confirmed rupture of the left posterolateral wall of the distal esophagus consistent with Boerhaave’s syndrome (Figure D), and a self-expanding covered metal stent was placed.

Broad-spectrum antibiotics and nasogastric feeding were commenced, and the left pleural effusion drained with a tube thoracostomy. Unfortunately, despite initial improvement, the patient subsequently deteriorated and died 30 days after admission.

Boerhaave’s is a rare clinical entity defined as spontaneous esophageal rupture, excluding perforations resulting from foreign bodies or iatrogenic instrumentation.

 It was first described by a Dutch physician, Herman Boerhaave, in 1724, about a Dutch Grand Admiral who overate and experienced spontaneous esophageal rupture after vomiting. It results from a sudden rise in intraluminal esophageal pressure from straining or vomiting, with failure of relaxation of the cricopharyngeus muscle. The left posterolateral wall of the esophagus is the most common site of perforation, owing to an anatomic weakness. More recently, an association with eosinophilic esophagitis has been reported, particularly in young men, and this underlying diagnosis should be considered, especially if there is a prior history of dysphagia and allergic manifestations.¹

Mackler’s triad of vomiting, lower chest pain, and subcutaneous emphysema is the classical presentation but is seen in only a minority of cases; thus, diagnostic errors are common.² Importantly, the chest radiograph is almost always abnormal, with pleural effusions or pneumomediastinum often seen.³ Surgical repair is the definitive treatment, but in patients considered unfit for surgery, conservative or endoscopic management is advocated. Mortality remains greater than 30%, and rises sharply if diagnosis is delayed^,2 emphasizing the importance of awareness of this unusual diagnosis.

References

1. Lucendo, A.J., Fringal-Ruiz, A.B., Rodriguez, B. Boerhaave’s syndrome as the primary manifestation of adult eosinophilic esophagitis. (Two case reports and a review of the literature.) Dis Esophagus. 2011 Feb;24:E11-5.

2. Brauer, R.B., Liebermann-Meffert, D., Stein, H.J., et al. Boerhaave’s syndrome: Analysis of the literature and report of 18 new cases. Dis Esophagus. 1997 Jan;10:64-8.

3. Pate, J.W., Walker, W.A., Cole, F.H. Jr, et al. Spontaneous rupture of the esophagus: a 30-year experience. Ann Thorac Surg. 1989 May;47:689-92.

The diagnosis

Gastrografin swallow (Figure C) demonstrated rupture of the distal esophagus, with leakage of gastrografin into the mediastinum (arrow). Upper gastrointestinal endoscopy confirmed rupture of the left posterolateral wall of the distal esophagus consistent with Boerhaave’s syndrome (Figure D), and a self-expanding covered metal stent was placed.

Broad-spectrum antibiotics and nasogastric feeding were commenced, and the left pleural effusion drained with a tube thoracostomy. Unfortunately, despite initial improvement, the patient subsequently deteriorated and died 30 days after admission.

Boerhaave’s is a rare clinical entity defined as spontaneous esophageal rupture, excluding perforations resulting from foreign bodies or iatrogenic instrumentation.

 It was first described by a Dutch physician, Herman Boerhaave, in 1724, about a Dutch Grand Admiral who overate and experienced spontaneous esophageal rupture after vomiting. It results from a sudden rise in intraluminal esophageal pressure from straining or vomiting, with failure of relaxation of the cricopharyngeus muscle. The left posterolateral wall of the esophagus is the most common site of perforation, owing to an anatomic weakness. More recently, an association with eosinophilic esophagitis has been reported, particularly in young men, and this underlying diagnosis should be considered, especially if there is a prior history of dysphagia and allergic manifestations.¹

Mackler’s triad of vomiting, lower chest pain, and subcutaneous emphysema is the classical presentation but is seen in only a minority of cases; thus, diagnostic errors are common.² Importantly, the chest radiograph is almost always abnormal, with pleural effusions or pneumomediastinum often seen.³ Surgical repair is the definitive treatment, but in patients considered unfit for surgery, conservative or endoscopic management is advocated. Mortality remains greater than 30%, and rises sharply if diagnosis is delayed^,2 emphasizing the importance of awareness of this unusual diagnosis.

References

1. Lucendo, A.J., Fringal-Ruiz, A.B., Rodriguez, B. Boerhaave’s syndrome as the primary manifestation of adult eosinophilic esophagitis. (Two case reports and a review of the literature.) Dis Esophagus. 2011 Feb;24:E11-5.

2. Brauer, R.B., Liebermann-Meffert, D., Stein, H.J., et al. Boerhaave’s syndrome: Analysis of the literature and report of 18 new cases. Dis Esophagus. 1997 Jan;10:64-8.

3. Pate, J.W., Walker, W.A., Cole, F.H. Jr, et al. Spontaneous rupture of the esophagus: a 30-year experience. Ann Thorac Surg. 1989 May;47:689-92.