Sigurdson E, Stern H S, Houpt J, el-Sharkawy T Y, Huizinga J D
Dis Colon Rectum. 1985 Dec;28(12):962-6. doi: 10.1007/BF02554318.
The Ehlers-Danlos syndrome is a genetically determined disorder of connective tissue which is generally known for its features of fragile, hyperextensible skin, hypermobile joints, and tissue fragility. Less commonly, colorectal complications can occur, including bleeding, prolapse, and diverticulitis. A rare case of colonic perforation associated with Ehlers-Danlos syndrome is presented. Additionally, in vitro electromyographic studies of the colonic tissue were performed which suggested a possible link between abnormal myogenic activity and the colonic perforations. The authors recommend that treatment be either a permanent colostomy or a subtotal colectomy with anastomosis to the rectum for similar cases.
埃勒斯-当洛综合征是一种由基因决定的结缔组织疾病，通常以皮肤脆弱、过度伸展、关节活动过度和组织脆弱为特征。较少见的情况下，会出现结直肠并发症，包括出血、脱垂和憩室炎。本文报告了一例与埃勒斯-当洛综合征相关的结肠穿孔罕见病例。此外，还对结肠组织进行了体外肌电图研究，结果提示肌源性活动异常与结肠穿孔之间可能存在联系。作者建议，对于类似病例，治疗方法可为永久性结肠造口术或直肠吻合的结肠次全切除术。
