Delayed Onset of Papilledema in Cerebral Venous Sinus Thrombosis (CVST) (P07.257)

Abstract

Objective: To present 2 cases of delayed onset papilledema and visual disturbance in CVST patients presenting initially with only headaches. Background The clinical presentations of CVST are variable, but the most common are headache (70-97%) and isolated intracranial hypertension (18-40%). Although 28-41% of patients have papilledema at presentation, it9s unusual to have delayed intracranial hypertension with papilledema after there9s sinus recanalization. Design/Methods: We report 2 CVST cases of worsening intracranial hypertension with visual symptoms and signs, despite improvement of thromboses on imaging. Results: 33-year-old male body builder with 2 months of worsening headaches had normal neurological exam. Contrast MR venogram showed superior sagittal sinus thrombosis. Investigations revealed myeloproliferative disorder with JAK-2 mutation, and antiphospholipid syndrome. Treated with heparin bridging to warfarin (INR2-3). Two months later, CT venogram showed no thrombus, but he developed visual obscurations, tinnitus, and new onset papilledema, peripheral field defects with normal visual acuity. Ten weeks later, he had worsening constrictive field deficits, with recurrence of thromboses in multiple sinuses. Visual field loss progressed on acetazolamide, so he had ventriculoperitoneal shunting, then aspirin and warfarin (INR3-3.5). Papilledema resolved and visual fields improved at 4months. 14-year-old girl had sudden onset headache, photophobia and vomiting after sneezing, and normal neurological exam. Contrast MR venogram showed right transverse sinus thrombosis. On contraceptive pill (estrogen & progestin), with MTHFR heterozygosity and elevated factor VIII. Received heparin then warfarin. Three weeks later, partial sinus recanalization, but she developed tinnitus, blurred vision and diplopia, with new onset severe papilledema, constrictive field deficits, partial left abducens nerve palsy, but normal visual acuity. Acetazolamide has stabilized papilledema and visual field defects at 10weeks. Conclusions: Clinicians need to remain vigilant in assessing for delayed onset papilledema and visual loss in CVST, even after anticoagulation and sinus recanalization. Follow up with serial quantitative perimetry rather than visual acuity is necessary. Disclosure: Dr. Wang has nothing to disclose. Dr. Matar has nothing to disclose. Dr. Allport has nothing to disclose.