Esophageal perforation in recessive dystrophic epidermolysis bullosa.
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.
Horan
T A
TA
Department of Surgery, Misericordia Hospital, Winnipeg, Manitoba, Canada.
Urschel
J D
JD
MacEachern
N A
NA
Shulman
B
B
Crowson
A N
AN
Magro
C
C
D000328:Adult; D003974:Diatrizoate Meglumine; D016108:Epidermolysis Bullosa Dystrophica / Q000150:complications* / Q000473:pathology; D004939:Esophageal Perforation / Q000000981:diagnostic imaging / Q000209:etiology* / Q000601:surgery*; D016629:Esophagectomy / Q000379:methods*; D004947:Esophagus / Q000293:injuries*; D005500:Follow-Up Studies; D005502:Food / Q000009:adverse effects*; D005547:Foreign Bodies / Q000150:complications*; D006801:Humans; D008297:Male; D011859:Radiography; D012086:Reoperation / Q000379:methods
D003974:Diatrizoate Meglumine