Multiple arterial thromboses due to cystic medial degeneration Erdheim-Gsell: A case report

Abstract

Rationale: Cystic medial degeneration Erdheim-Gsell is a vascular pathology mainly of the large vessels, which is mostly associated with Marfan syndrome or Ehlers-Danlos syndrome. The clinical findings of this entity are aneurysms of the aorta or large peripheral arteries which usually present in an acute setting due to rupture of an aneurysm.

Patient concerns: We present a case of a 43-year-old Caucasian male with histologically proven cystic medial degeneration of the lower limb vessels mimicking peripheral artery occlusive disease. Despite antiplatelet and anticoagulant treatment, the patient suffered multiple vascular stenosis and occlusions.

Diagnoses: Multiple arterial stenoses and thromboses leading to peripheral artery occlusive disease caused by cystic medial degeneration Erdheim-Gsell.

Interventions: Multiple surgical and endovascular interventions including bypass graft and intra-arterial thrombolysis as well as oral antiplatelet and anticoagulant therapy.

Outcome: Despite dual antiplatelet therapy, anticoagulant therapy with rivaroxaban and multiple surgical and endovascular interventions, the patient developed recurrent arterial thromboses. The patient did not suffer further thrombotic events since clopidogrel and phenprocoumon were administered.

Lessons: Clinical presentation of cystic medial degeneration Erdheim-Gsell mimicking peripheral artery occlusive disease is very unusual. Due to the fragile vessel wall, patients with cystic medial degeneration might have a higher risk to develop arterial thromboses, even under antiplatelet therapy or anticoagulant treatment.

Figure 1

Digital subtraction angiography of the right popliteal artery. (A) Arrow points toward a high-grade stenosis. (B) Right popliteal artery after angioplasty and stenting.

Figure 2

Magnetic resonance angiography of the aorta, iliacal, and leg arteries. The white arrows point to the occluded bypass graft of the right leg. Red arrows point to stenoses of the right popliteal artery and tibioperoneal trunk. There are no aortal or iliacal aneurysms.

Figure 3

Color-coded duplex ultrasonography of the bypass graft's aneurysm with a size of 1.4 × 1.35 cm.

Figure 4

(A) Digital subtraction angiography of the right superficial femoral artery before and (B) after intra-arterial thrombolysis. Arrow points toward the occluded bypass graft.