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The Annals of Thoracic Surgery, Vol 57, 1027-1029, Copyright © 1994 by The Society of Thoracic Surgeons

ARTICLES

Esophageal perforation in recessive dystrophic epidermolysis bullosa

TA Horan, JD Urschel, NA MacEachern, B Shulman, AN Crowson and C Magro
Department of Surgery, Misericordia Hospital, Winnipeg, Manitoba, Canada.

Recessive dystrophic 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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Copyright © 1994 by The Society of Thoracic Surgeons.