Spontaneous Esophageal Perforation Due to Eosinophilic Esophagitis in a Teenager

Abstract

Eosinophilic esophagitis (EoE) is a chronic inflammatory disease of the esophagus characterized by eosinophil predominant inflammation on histology. If untreated, EoE can result in esophageal remodeling, fibrosis and strictures. Symptoms in adults are typically obstructive in nature - dysphagia or food impaction. In contrast, children typically present with failure to thrive, feeding difficulty, regurgitation, vomiting, and abdominal pain. While adults have been noted to present with spontaneous perforation of the esophagus, there are a few case reports in the literature describing children with spontaneous perforation of the esophagus due to EoE. We report a teenager with spontaneous micro-perforation of the esophagus due to EoE. A 14-year old Caucasian male with a 2-month history of daily vomiting, early satiety, 20 pound weight loss, and decreased appetite presented to the Emergency Department with worsening vomiting and acute shortness of breath with chest pain. Pertinent history included severe atopic dermatitis, seasonal allergies, hypothyroidism, obesity, ADHD, and autism. CT scan of his chest and abdomen showed thickening of the distal esophagus, extensive pneumomediastinum with extension of air into the neck and axilla but with normal lung parenchyma. Cardiac enzymes and EKG were normal. A water soluble contrast swallow study the next day showed mucosal fold thickening of the distal esophagus without an esophageal leak. He was placed on bowel rest, high-dose proton pump inhibitor (PPI) and antibiotics until resolution of the pneumomediastinum. After 8 weeks of high dose oral PPI therapy, endoscopy showed thickened folds in the distal esophagus. Biopsies revealed 25 eosinophils per high power field in the mid-esophagus and 0-5 eosinophils per HPF in the distal esophagus, consistent with a diagnosis of EoE. Subsequently, he was treated with a budesonide slurry with resolution of his vomiting. Follow-up endoscopy showed reduced eosinophils of 0-5 per HPF in the mid-esophagus. Pneumomediastinum was the presenting symptom of EoE in this teenager due to spontaneous micro-perforation of the esophagus. It is important to consider Eosinophilic Esophagitis in the differential diagnosis of spontaneous esophageal perforation, even in a child.