Iglesias J L, Renard T
Department of Surgery, Mail code 9159, The University of Texas, Southwestern Medical Center, 5323 Harry Hines Boulevard, Dallas, Texas 75235-9159, USA.
Pediatr Surg Int. 1998 Oct;13(8):553-5. doi: 10.1007/s003830050401.
Ehlers-Danlos syndrome (EDS) includes a group of connective-tissue disorders characterized by abnormal collagen metabolism. The diverse spectrum of this disease and its complications present a distinct challenge to the surgeon. Patients with this hereditary disorder may have hyperelastic or fragile skin, poor wound healing, hypermobile joints, clotting abnormalities, spontaneous pneumothoraces, recurrent hernias, bowel perforation, and vascular complications. An 8-year-old female with EDS type VII and a newly diagnosed diaphragmatic hernia is presented. Surgical considerations of this syndrome are reviewed.
埃勒斯-当洛综合征（EDS）包括一组以胶原蛋白代谢异常为特征的结缔组织疾病。这种疾病及其并发症的多样性给外科医生带来了独特的挑战。患有这种遗传性疾病的患者可能有皮肤弹性过强或脆弱、伤口愈合不良、关节活动过度、凝血异常、自发性气胸、复发性疝、肠穿孔和血管并发症。本文介绍了一名患有VII型EDS并新诊断为膈疝的8岁女性。本文回顾了该综合征的手术注意事项。
