Spontaneous compartment syndrome and endovascular repair of tibioperoneal trunk pseudoaneurysm in Ehlers-Danlos syndrome

Vascular Ehlers-Danlos syndrome is caused by mutations in the COL3A1 (collagen type III alpha-1) gene, resulting in loss of integrity of arteries and hollow organs. Patients are predisposed to dissection, aneurysm, and organ rupture. The median life expectancy is ∼51 years. We have described a unique presentation of spontaneous compartment syndrome, likely secondary to ischemia reperfusion injury, in a 32-year-old man with vascular Ehlers-Danlos syndrome. The compartment syndrome 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.