Esophageal perforation in recessive dystrophic epidermolysis bullosa.

TitleEsophageal perforation in recessive dystrophic epidermolysis bullosa.
Publication TypeJournal Article
Year of Publication1994
AuthorsHoran TA, Urschel JD, MacEachern NA, Shulman B, Crowson AN, Magro C
JournalAnn Thorac Surg
Date Published1994 Apr
KeywordsAdult, Diatrizoate Meglumine, Epidermolysis Bullosa Dystrophica, Esophageal Perforation, Esophagectomy, Esophagus, Follow-Up Studies, Food, Foreign Bodies, Humans, Male, Radiography, Reoperation

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.

Alternate JournalAnn Thorac Surg
PubMed ID8166505
Related Faculty: 
Cynthia M. Magro, M.D.

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