Sneddon Syndrome in Cardiac Surgery: An Anesthetic Challenge Due to the Rare Coexistence of Thrombotic and Hemorrhagic Risk

Background: Sneddon syndrome is a rare non-inflammatory vasculopathy characterized by livedo reticularis and cerebrovascular events1. It is often associated with antiphospholipid antibodies, immune thrombocytopenia, and other autoimmune disorders1. Perioperative management is challenging due to the coexistence of thrombotic and hemorrhagic risks2. Literature on anesthetic management in these patients is limited, making evidence-based clinical decision-making difficult. Case Report: A 44-year-old male with severe aortic valve disease and recurrent syncopal episodes. Medical history includes Sneddon syndrome with hypertension, epilepsy post-stroke, livedo reticularis, immune thrombocytopenia, and positive antiphospholipid antibodies. Chronic treatment includes levetiracetam, aspirin, and azathioprine. Due to complex hemostatic profile, a multidisciplinary team meeting was held. Preoperatively, the patient had a prolonged aPTT (70 seconds) and platelets of 110 x109/L. Optimization included immunoglobulin and corticosteroids. No prophylactic transfusions were administered. In the operating room, baseline ACT was 180 seconds. Heparin (200 mg) was administered without dose reduction as advised by hematology. Aortic valve replacement was successfully performed (CPB time: 85 minutes; cross-clamp time: 54 minutes). After heparin reversal with protamine, a thromboelastogram (Image I) was obtained to guide precise transfusion and avoid tipping the balance toward hypercoagulability. Based on the viscoelastic results, only one unit of fresh frozen plasma was administered with meticulous surgical hemostasis. Postoperative management included close monitoring of bleeding via thoracic drains. The patient was extubated the same day without complications. Platelet count dropped to 70 x109/L within 24 hours, but no transfusions were required due to the absence of active bleeding. On postoperative day 2, he was transferred to the ward, and anticoagulation was initiated with acenocoumarol. However, therapeutic INR levels were not achieved, likely due to the underlying hematologic condition. He was successfully transitioned to low molecular weight heparin, the only evidence-based alternative recommended by current guidelines. Discussion: Sneddon syndrome presents a rare and complex challenge in anesthetic management, particularly in high-risk surgeries such as valve replacement. The coexistence of thrombotic and hemorrhagic risks necessitates a highly individualized approach. In this case, multidisciplinary coordination and the use of viscoelastic testing (thromboelastography)2 were key to achieving precise hemostatic control and minimizing complications. Given the scarce literature available, this case contributes valuable clinical insight into the safe perioperative management of patients with Sneddon syndrome, highlighting the relevance of personalized anticoagulation strategies and dynamic coagulation monitoring in such high-risk settings.