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Multiple arterial thromboses due to cystic medial degeneration Erdheim-Gsell: A case report
Philipp Jud1, Thomas Gary, Franz Hafner
1Division of Angiology, Department of Internal Medicine Division of Vascular Surgery, Department of Surgery, Medical University of Graz, Graz, Austria.
Insights
Cystic medial degeneration Erdheim-Gsell is a rare vascular condition that can mimic peripheral artery disease. Patients may experience recurrent arterial thromboses despite standard treatments, highlighting the need for tailored therapeutic strategies.
Area of Science:
- Vascular Pathology
- Cardiovascular Medicine
- Histopathology
Background:
- Cystic medial degeneration Erdheim-Gsell is a rare vascular pathology affecting large vessels, often associated with connective tissue disorders.
- It typically presents as aortic or peripheral artery aneurysms, frequently discovered during acute rupture events.
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.
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