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Ann Thorac Surg 1994;57:1027-1029
© 1994 The Society of Thoracic Surgeons
Department of Surgery and Pathology, Misericordia Hospital and University of Manitoba, Winnipeg, Manitoba, Canada
Accepted for publication July 24, 1993.
* Address reprint requests to Dr Urschel, Department of Surgical Oncology, Roswll Park Cancer Institute. Elm and Carlton St, Bulfalo, NY 42630001.
Recessive dysbophic epidermolysis bullosa is an inherited mechanobullous disease characterized by skin blistering and esophageal bullae. In response to minor trauma, a split in the dermis or esophageal subepithelium occurs. Esophageal perforation is a rare complication. Because the esophagus is diseased in these patients, we believe that esophagectomy rather than repair is the treatment of choice for esophageal perforations. We report a patient for whom early primary repair failed and esophagectomy was required.
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