An atypical presentation of superior ophthalmic vein thrombosis with nonspecific orbital inflammation

Superior ophthalmic vein thrombosis (SOVT) is an uncommon orbital pathology that can present with sudden onset proptosis, conjunctival congestion, eye pain, and visual disturbances. An inflammatory pathology has rarely been reported as a cause of SOVT. A 56-year-old female presented as a case of ocular hypertension with features of raised episcleral venous pressure, and on further imaging, she was found to have SOVT with nonspecific orbital inflammation (NSOI). All blood investigations including serological workup for underlying autoimmune or vasculitic etiology were negative except for a low titer 1+ speckled pattern on ANA-IFA. Presuming it to be a case of SOVT with NSOI, she was started on systemic and topical steroids along with second-line immunosuppressant therapy to which she showed a good response to treatment with a reduction in the orbital swelling and resolution of the left-sided periorbital edema and complete recanalization of the left superior ophthalmic vein on follow-up MRV at 6 months. Orbital inflammation-induced superior ophthalmic vein thrombosis is a rare entity, but it cannot be excluded as an etiology of secondary open-angle glaucoma with raised episcleral venous pressure. The underlying pathogenesis remained concealed until detailed investigative workups were done. A diligent history and clinical evaluation can help in unveiling such rare disorders with atypical presentations and initiate appropriate therapy.