S1994 Spontaneous Intramural Esophageal Rupture: A Rare Complication of Eosinophilic Esophagitis

Abstract

INTRODUCTION: Perforation of esophageal mucosa is often associated with Mallory Weiss tears and Boerhaave syndrome. However, spontaneous intramural esophageal rupture (SIER) or incomplete esophageal dissection (IED) are possible variants of Boerhaave syndrome only described in case reports, and often not in the initial differential for acute onset of chest pain, dysphagia and hematemesis. We present a case of spontaneous partial tear of the esophagus likely as a result of untreated eosinophilic esophagitis (EoE). CASE DESCRIPTION/METHODS: A 50-year-old male without significant medical history presented to the hospital with sudden epigastric pain, dysphagia, and multiple episodes of hematemesis, not preceded by retching. Labs were notable for stable hemoglobin 16 g/dL. He underwent an EGD which revealed chronic eosinophilic esophagitis, a benign esophageal stricture, and a large linear blood clot of unknown etiology (Figure 1). CT chest and abdomen/pelvis were significant for mild esophageal wall thickening and reactive lymphadenopathy without evidence of esophageal perforation. On repeat EGD three days later, a medium-sized dissection flap was identified as the gastroscope was advanced into a false tract (Figure 2). The wall of this lumen appeared to have granulation tissue and raw areas consistent with chronic inflammatory changes, though there were no signs of full perforation. To confirm, a CT chest with oral gastrografin showed no free air or contrast extravasation into the mediastinum, only eccentric foci of air in the distal esophagus. Fluoroscopy esophagram revealed distal esophageal stenosis with adjacent well-contained extravasation, without evidence of mediastinal leakage (Figure 3), further corroborating a submucosal tear. The patient was placed on antibiotics and was made NPO for seven days with total parenteral nutrition. He then gradually advanced to clear liquids followed by esophageal soft solids. Repeat endoscopy was performed one month later and revealed healing of the submucosal tear as well as linear furrows consistent with EoE. DISCUSSION: EoE results in chronic inflammatory changes and mucosal friability that often lead to food impaction or esophageal strictures, but rarely result in esophageal tear or perforation. This case emphasizes the need for broadening the differential in a patient presenting with epigastric pain, dysphagia and hematemesis. Clinicians must have a high suspicion to detect rare cases of spontaneous intramural esophageal rupture or incomplete dissection.Figure 1.: Initial endoscopy revealed a longitudinal clot of unclear etiology.Figure 2.: Repeat endoscopy showed a medium-sized dissection flap. The small arrows point to the true lumen of the esophagus, while the larger arrow indicates the false lumen.Figure 3.: Fluoroscopy esophagram revealed a well-contained extravasation into a separate lumen, consistent with a submucosal tear.