Hosaka Akihiro, Miyata Tetsuro, Shigematsu Hiroshi, Deguchi Jun-o, Kimura Hideo, Nagawa Hirokazu, Sato Osamu, Sakimoto Takehiko, Mochizuki Tomoyuki
Division of Vascular Surgery, Department of Surgery, Graduate School of Medicine, University of Tokyo, Japan.
J Gastrointest Surg. 2006 Apr;10(4):583-5. doi: 10.1016/j.gassur.2005.07.018.
The vascular type of Ehlers-Danlos syndrome is a genetic disorder of connective tissue and is frequently associated with catastrophic arterial complications. Its surgical treatment is extremely difficult because of the fragility of vessels. This article describes three patients with vascular type of Ehlers-Danlos syndrome who developed mesenteric hemorrhage due to spontaneous arterial rupture. The clinical and molecular characteristics of the disease are briefly reviewed.