Spontaneous Esophageal Perforation in a Patient with Mixed Connective Tissue Disease

David Lyman*
Cherry Street Health Services, 100 Cherry Street SE, Grand Rapids, Michigan, 49053, USA

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© David Lyman; Licensee Bentham Open.

open-access license: This is an open access article licensed under the terms of the Creative Commons Attribution Non-Commercial License ( which permits unrestricted, non-commercial use, distribution and reproduction in any medium, provided the work is properly cited.

* Address correspondence to this author at 210 North 2nd St, Kalamazoo, Michigan, 49009, USA; Tel: 269 599 2783; E-mail:


Spontaneous esophageal perforation is a rare and life-threatening disorder. Failure to diagnosis within the first 24-48 hours of presentation portends a poor prognosis. A patient with mixed connective tissue disease (MCTD) on low-dose prednisone and methotrexate presented moribund with chest and shoulder pain, a left hydropneumothorax, progressive respiratory failure and shock. Initial management focussed on presumed community acquired pneumonia (CAP) in a patient on immunosuppressants. Bilateral yeast empyemas were treated and attributed to immunosuppression. On day 26, the patient developed mediastinitis, and the diagnosis of esophageal perforation was first considered. A review of the literature suggests that the diagnosis and management of spontaneous esophageal perforation could have been more timely and the outcome less catastrophic.

Keywords: Esophageal perforation, spontaneous esophageal perforation, mixed connective tissue disease..