%0 Journal Article
%T Successful multidisciplinary management of vascular Ehlers-Danlos syndrome.
%A Kanaka S
%A Yamada T
%A Matsuda A
%A Takahashi G
%A Arai M
%A Takiguchi T
%A Tayama H
%A Yoshida H
%J Clin J Gastroenterol
%V 0
%N 0
%D Nov 2021 29
%M 34845584
暂无%R 10.1007/s12328-021-01562-9
%X Vascular Ehlers-Danlos syndrome is a rare connective tissue disease with a high risk of severe complications. Because of these complications, the median life expectancy for patients with vascular Ehlers-Danlos syndrome is estimated at 48 years. However, the optimal management of these complications remains unclear. A 25-year-old man with abdominal pain was transported to our hospital by ambulance. He had undergone Hartmann's operation at 22 years of age for a first-time colonic perforation. At that time, a genetic test revealed germline variants in COL3A1, which encodes type III procollagen; therefore, the patient was diagnosed with vascular Ehlers-Danlos syndrome. When the patient presented to our hospital, we suspected another colonic perforation and thus performed an emergency operation. Open abdominal management, transcatheter arterial embolization, and negative-pressure wound therapy were performed as life-saving measures. Notably, these procedures should initially be avoided in patients with vascular Ehlers-Danlos syndrome because of tissue fragility. Open abdominal management, transcatheter arterial embolization, and negative-pressure wound therapy may be useful for patients with vascular Ehlers-Danlos syndrome who develop panperitonitis and massive intra-abdominal bleeding.