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

Abstract

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.

Keywords: Compartment syndrome; Infrageniculate pseudoaneurysm; Intravascular ultrasound; Tibioperoneal trunk pseudoaneurysm; Vascular Ehlers-Danlos syndrome.

Fig 1

A, Medial incision of left lower extremity four compartment fasciotomy displaying diffusely edematous muscle intraoperatively. B, Lateral incision of left lower extremity four compartment fasciotomy displaying diffusely edematous muscle intraoperatively.

Fig 2

A, Axial view of computed tomography angiogram of bilateral lower extremities showing left external iliac artery (EIA) aneurysm and focal dissection and mid-distal right EIA dissecting aneurysm. B, Sagittal view of computed tomography angiogram of left lower extremity displaying left common iliac artery and EIA aneurysm and distal focal EIA dissection.

Fig 3

A, Axial view of computed tomography angiogram of left lower extremity showing dissection of the tibioperoneal trunk (TPT) with pseudoaneurysm. B, Axial view of computed tomography angiogram of left lower extremity showing dissection of the TBT with pseudoaneurysm. C, Sagittal view of computed tomography angiogram of left lower extremity displaying dissection of the TPT with pseudoaneurysm.

Fig 4

A, Transverse view of arterial duplex ultrasound of left lower limb showing a 4.28-cm × 1.75-cm pseudoaneurysm of the left distal tibioperoneal trunk (TPT). B, Longitudinal view of arterial duplex ultrasound of left lower limb displaying pseudoaneurysm (arrow) of left distal TPT.

Fig 5

A, Left lower extremity angiogram displaying left tibioperoneal trunk (TPT) pseudoaneurysm before intervention. B, Left lower extremity angiogram displaying left TPT pseudoaneurysm after exclusion with stent graft deployment.