Spontaneous Compartment Syndrome and Endovascular Repair of Tibio-peroneal Trunk Pseudoaneurysm in Ehlers-Danlos Syndrome

Abstract: Vascular Ehlers-Danlos (vEDS) syndrome is caused by mutations in the type III alpha-1 gene (COL3A1), resulting in loss of integrity of arteries and hollow organs. Patients are predisposed to dissections, aneurysms and organ rupture. Median life expectancy is approximately 51 years. We describe a unique presentation of spontaneous compartment syndrome, likely secondary to ischemia reperfusion injury, in a thirty-two year old male with vEDS. This was treated with four-compartment fasciotomy and subsequent evaluation demonstrated a pseudoaneurysm of the tibioperoneal trunk. Endovascular intervention and stent graft deployment guided by intravascular ultrasound successfully excluded the pseudoaneurysm with three vessel run-off preserved.