First Case Reported with Infrainguinal Vascular Disease Manifestation from Cogans Syndrome
michael sywak, MD1, Linda Harris, MD2.
1Buffalo, getzville, NY, USA, 2Buffalo, Buffalo, NY, USA.
DEMOGRAPHICS: 20-year-old Caucasian male college student HISTORY: The patient developed bilateral deafness, tinnitus and uveitis over the previous 2 years. He was diagnosed with unspecified autoimmune disease, and was on corticosteroid eye drops and methotrexate. He developed right leg pain with walking approximately 5 months prior and eventually underwent duplex after being evaluated by a different rheumatologist. He was referred to vascular. However, prior to his appointment developed numbness and tingling, presenting to the emergency room. Physical exam revealed a palpable left femoral pulse, non-palpable right femoral pulse, with no pulses in the right leg. The right foot was cool with no sensory or motor deficits. Doppler signals were present. Lower extremity arterial studies were again obtained. Right ABI was 0.54 with TBI of 0.0. Duplex showed diffuse thickening of the arterial wall and occlusion in affected vessels. Erythrocyte sedimentation rate was 78 (down from 120 at rheumatology visit), C-reactive protein 48 with a white blood cell count of 12.8. CT angiogram of the torso with runoff in the lower extremities was obtained, demonstrating occlusion of the external iliac, common femoral, and superficial femoral arteries. PLAN: The patient was diagnosed with Cogan Syndrome by the vascular team, and after consultation with rheumatology, placed on additional immunosuppression allowing for normalization of inflammatory markers. The patient underwent an open right common iliac to common femoral onto profunda femoris bypass with Dacron graft, after endarterectomy of the fibrotic occlusion in the femoral artery. Stent grafting of right superficial femoral to P1 popliteal segment was also performed with Viabahn after removal of the fibrotic core from the proximal SFA. The patient recovered and was discharged on immunosuppressants. Follow up ABI was 1.0. DISCUSSION: This is the first case reported with infrainguinal vascular disease manifestation from Cogans Syndrome. Cogans is a rare autoimmune disease with deafness, blindness and vascular involvement, either aneurysms or occlusive disease, in young patients. Our case highlighted the use of a hybrid approach for revascularization with open repair of the iliofemoral segment and endovascular repair of the superficial femoral segment.
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