IDIOPATHIC EOSINOPHILIC ESOPHAGITIS (EE): AN UNUSUAL PRESENTATION

W Al-Hashash, DS Mulder, S Mayrand

McGill University Health Center, McGill University, Montreal, Quebec

EE is a rare condition, which is defined by the presence of eosinophils within the esophageal wall.
HISTORY AND FINDINGS: A 62 year-old male patient was referred for the evaluation of severe retching and, minimal dysphagia to solids and liquids, with several episodes of food bolus impaction of 6-months duration. Several medications (histamine H2blockers, proton pump inhibitors, long-acting nitrates, domperidone) were tried with no benefits. His past medical history was remarkable for a right middle lobe resection for a benign bronchial leiomyoma and long-standing insulin-dependent diabetes. There was no history of atopy.
INVESTIGATIONS: A complete blood count was normal with no eosinophilia. An upper endoscopy revealed a perfectly normal mucosa and several esophageal biopsies were normal. A barium swallow showed decreased peristaltic activity in the thoracic esophagus as well as a smooth narrowing of the inferior third of the esophageal body. An esophageal nuclear transit study only revealed a very mild delay in emptying. The esophageal manometry suggested diffuse esophageal spasm with a normal lower esophageal sphincter. A chest CT scan showed a concentric thickening of the esophageal wall from the thoracic inlet to the gastroesophageal junction. An endoscopic ultrasound (EUS) confirmed these findings and a fine needle aspiration only revealed the presence of benign smooth muscle cells.
TREATMENT: In the absence of a clear diagnosis, the patient underwent a left thoracotomy, a full-thickness biopsy and an extended myotomy of the esophagus. The per-operative biopsy revealed a marked intramural eosinophilic infiltrate without parasite or granuloma. Post-operatively the patient did very well and he remains asymptomatic one year after.
CONCLUSIONS: This case has several unique aspects, including the patient's age and presentation, the absence of any history of allergy and eosinophilia, and the absence of any eosinophil in the initial esophageal mucosal biopsy and fine needle aspirate (EUS). To our knowledge, this is only the second report of an intramural EE successfully treated with an esophageal myotomy. This may represent an alternative to the standard treatment using corticosteroids, especially in the older diabetic patients.