Multimodal evaluation and etiology-oriented management of chronic intracranial venous hypertension

Chronic intracranial venous hypertension (CIVHS) comprises a heterogeneous group of disorders characterized by impaired cerebral venous outflow and symptoms related to elevated intracranial pressure. Although venous sinus stenting is increasingly used for venous sinus stenosis with elevated trans-stenotic pressure gradients, venous sinus stenosis is an imaging phenotype rather than a single diagnosis. We retrospectively reviewed 13 consecutive patients with CIVHS who underwent clinical, imaging, and hemodynamic evaluation, including conventional neuroimaging, MR or CT venography, digital subtraction angiography, venous manometry when indicated, and selective high-resolution MRI. All patients initially presented to ophthalmology and had papilledema. Patients were classified according to presumed mechanisms of venous outflow impairment, and treatment was tailored to the anatomical substrate, hemodynamic findings, and clinical context. Six etiological categories were identified: intrinsic transverse sinus stenosis, extrinsic transverse-sigmoid sinus compression, shunt dependency syndrome, jugular foramen stenosis related to chronic organized thrombus, JAK2 V617F mutation-positive essential thrombocythemia with suspected microvascular venous congestion, and dialysis catheter-related central venous stenosis. An additional chronic cerebral venous sinus thrombosis case was included as an illustrative secondary comparator but was not classified as idiopathic intracranial hypertension. Treatments included venous sinus stenting, shunt-related management, hematologic-directed therapy, anticoagulation, acetazolamide, weight control, and conservative management. Among the 13 cohort patients with available follow-up, symptoms improved or remained clinically stable; Case 14 was lost to follow-up. No stent failure was documented during short-term follow-up, but long-term surveillance data were unavailable. These findings support an etiology-oriented, multimodal approach to chronic intracranial venous hypertension.