Boerhaave's syndrome as an initial presentation of eosinophilic esophagitis: a case series

Abstract

Background: Prior studies report esophageal rupture following endoscopy or bolus impaction in eosinophilic esophagitis (EoE). The purpose of this study is to add new information to available evidence defining the clinical spectrum of spontaneous rupture (Boerhaave's syndrome) associated with vomiting in EoE.

Methods: A retrospective search of inpatient and outpatient records was conducted from January 2001 to January 2011. A faculty member in pathology blindly reviewed all esophageal biopsy specimens. EoE was defined as 15 or more eosinophils in at least 2 high-power fields (hpfs) or 25 or more eosinophils in any single HPF.

Results: In ten years, 447 patients were identified with a diagnosis of EoE. Of these, four patients presented with Boerhaave's syndrome in the setting of EoE. None of the patients had an established diagnosis of EoE prior to presentation. All cases presented with a triad of vomiting, chest pain and pneumomediastinum. In two patients, water-soluble contrast extravasation prompted surgical intervention (50%). Full thickness surgical specimen provides a unique opportunity to show eosinophils in the muscularis propria. Intraepithelial eosinophil infiltration was seen on all mucosal biopsies (>25/hpf) with significant improvement after steroid (topical or systemic) treatment.

Conclusions: Spontaneous esophageal rupture is a rare (4/447, less than 1%) but critical presentation of EoE manifesting with vomiting, chest pain and pneumomediastinum. Surgery is required if extravasation is seen with water-soluble contrast. We suggest that EoE may be a transmural disease in some patients, thus making the esophageal wall susceptible to spontaneous rupture with vomiting (Boerhaave's syndrome).

Keywords: Boerhaave’s syndrome; eosinophilic esophagitis; esophageal rupture.

Figure 1

Patient 2: (A) CT scan. Mediastinal air and extravasated contrast are present below the level of the carina compatible with rupture of the distal esophagus. Air extends into the gastrohepatic space. (B) Water-soluble contrast esophagram. Contrast leaks behind the stomach as well and from the right aspect of the esophagus consistent with perforation. Patient 3: (C) CT scan. Pneumomediastinum is present as well as air dissecting into the pericardium, but no frank defect is visualized in the trachea or esophagus. (D) Barium esophagram following initial evaluation with water-soluble contrast esophagram. There is no evidence of esophageal leak and no contrast extravasation

Figure 2

(A) Transmural esophageal biopsy at time of presentation of Patient 2 demonstrating eosinophilic infiltration into the muscularis propria. Approximately 40 eosinophils per high power field (EOS/ HPF). (H&E stain, 10× magnification). Insert. Evidence of eosinophils infiltrating the muscularis propria of the esophagus. (H&E stain, 40× magnification). (B) Esophageal mucosa after treating Patient 2 with steroids showing no evidence of eosinophils. (H&E stain, 40× magnification)