Elton C, Marshall R E, Hibbert J, Cameron R, Mason R C
Oesophageal Unit, Department of Surgery, Guy's Hospital, London, UK.
Dis Esophagus. 2000;13(2):175-7. doi: 10.1046/j.1442-2050.2000.00109.x.
Epidermolysis bullosa comprises a group of rare heritable disorders, characterized by blistering of skin and other epithelial lined structures following minor trauma. In the oesophagus, trauma from food boluses leads to bullae, ulceration and scarring, with formation of strictures. Oesophageal strictures are usually managed with balloon dilatation. We describe a case of a 19-year-old woman whose oesophageal stricture did not respond to balloon dilatation. She underwent a substernal colon interposition between the pharynx and stomach. The surgery included cervical, thoracic and abdominal approaches, with involvement of three specialist surgeons. Three months after surgery, the patient reported to be swallowing with little difficulty. Because of the high risk of morbidity and mortality associated with this surgery, we recommended that the patient should be managed in a specialist centre, with availability of intensive care facilities and the possibility of providing a multidisciplinary approach during surgery.
大疱性表皮松解症是一组罕见的遗传性疾病，其特征是在轻微创伤后皮肤和其他上皮内衬结构出现水疱。在食管中，食物团块造成的创伤会导致大疱、溃疡和瘢痕形成，并伴有狭窄。食管狭窄通常采用球囊扩张术治疗。我们描述了一名19岁女性的病例，其食管狭窄对球囊扩张术无反应。她接受了咽胃间胸骨后结肠间置术。该手术包括颈部、胸部和腹部入路，由三位专科外科医生参与。术后三个月，患者报告吞咽困难轻微。由于该手术相关的高发病率和死亡率风险，我们建议患者应在具备重症监护设施且手术期间能够提供多学科治疗方法的专科中心接受治疗。
