Successful multidisciplinary management of vascular Ehlers–Danlos syndrome

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.