Horan T A, Urschel J D, MacEachern N A, Shulman B, Crowson A N, Magro C
Department of Surgery, Misericordia Hospital, Winnipeg, Manitoba, Canada.
Ann Thorac Surg. 1994 Apr;57(4):1027-9. doi: 10.1016/0003-4975(94)90234-8.
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.
隐性营养不良性大疱性表皮松解症是一种遗传性机械性大疱病，其特征为皮肤水疱和食管大疱。受到轻微创伤后，真皮或食管上皮下会出现裂隙。食管穿孔是一种罕见的并发症。由于这些患者的食管存在病变，我们认为食管切除术而非修复术是食管穿孔的首选治疗方法。我们报告了一例早期一期修复失败而需要进行食管切除术的患者。
