Sharp recanalization of extensive multilevel central venous occlusions in a young patient with familial Mediterranean fever: A case report

Chronic multilevel central venous occlusion (CVO) in young adults is uncommon and presents major challenges in establishing reliable vascular access. Patients with recurrent thrombosis develop extensive collateral formation and progressive loss of venous access. Although familial Mediterranean fever (FMF) is primarily an autoinflammatory disease, emerging evidence suggests an increased risk of thromboembolic events mediated by chronic endothelial inflammation and dysfunction. Endovascular recanalization using conventional and sharp techniques is well described for complex long-segment occlusions; reports specifically describing this approach in a young patient with FMF and recurrent venous thromboembolism, however, remain limited. We report a 23-year-old woman with type 1 diabetes mellitus, hypothyroidism, epilepsy, asthma, and FMF (MEFV E148Q variant), with 3 prior ischemic strokes and approximately 30 venous thromboembolic events despite anticoagulation, in whom an extended inherited thrombophilia workup, including whole-exome sequencing and JAK2 testing, was negative. She presented with chest and abdominal pain, palpitations, dizziness, and limb swelling. Imaging revealed extensive multilevel CVO involving the axillary, subclavian, brachiocephalic, internal jugular, and external iliac veins with prominent collateral formation, and repeated attempts at peripheral and central venous access had failed. Endovascular recanalization was performed under sedation. Conventional wire-and-catheter technique crossed most occlusions; sharp recanalization with a 0.018-inch guidewire was required for the left subclavian vein. Subsequently, sequential balloon angioplasty and stent placement were followed by bilateral placement of 5-French dual-lumen peripherally inserted central catheters (PICCs). Finally, postprocedure venography demonstrated satisfactory recanalization with no major complications. Combined conventional and sharp recanalization restored central venous patency and enabled venous access in extensive multilevel CVO. Both PICC lines remained functional without complication over 7 months of follow up. However, the patient required escalating anticoagulation with an unexplained subtherapeutic anti-Xa level, and CT at 4.5 months showed the subclavian stent in situ without acute pulmonary embolism but persistent chronic left brachiocephalic stenosis with extensive collaterals. This case illustrates both the technical feasibility of endovascular recanalization in this setting and the durability challenge posed by an ongoing, incompletely controlled inflammatory and thrombotic state.