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Medical Science Monitor Basic Research


Optic Nerve Sheath Fenestration for Papilledema Due to Cerebral Venous Sinus Thrombosis Associated with Antiphospholipid Syndrome: A Case Report

Challenging differential diagnosis, Rare disease

Ayman G. Elnahry, Joseph H. Talbet, Iman R. El Mahgoub, Gehad A. Elnahry

Egypt Department of Ophthalmology, Faculty of Medicine, Cairo University, Cairo, Egypt

Am J Case Rep 2021; 22:e930497

DOI: 10.12659/AJCR.930497

Available online: 2021-04-15

Published: 2021-05-24


BACKGROUND: Cerebral venous sinus thrombosis (CVST) is a serious life- and vision-threatening condition that can have a variable presentation according to the site of venous occlusion, including mimicking idiopathic intracranial hypertension. We report on a patient with primary antiphospholipid antibody syndrome (APS) who presented with papilledema due to CVST that was refractory to medical treatment but responded to optic nerve sheath fenestration (ONSF).
CASE REPORT: A 21-year-old man presented with blurred vision of gradual onset and a progressive course for 1 month, accompanied by fever, headache, and confusion. He had a history of lower-limb deep vein thrombosis. Examination revealed decreased vision with bilateral grade IV papilledema. Magnetic resonance venography showed evidence of CVST and laboratory investigations revealed lupus anticoagulant antibodies, antinuclear antibodies, and anti-double stranded DNA antibodies, with hyperhomocysteinemia. The patient did not meet the Systemic Lupus International Collaborating Clinics classification criteria for systemic lupus erythematosus (SLE) nor the new European League Against Rheumatism and American College of Rheumatology SLE classification criteria. He was diagnosed with CVST secondary to APS and hyperhomocysteinemia and treated with acetazolamide, systemic anticoagulation, and vitamins for 1 month, but with no improvement in his ophthalmic condition. He subsequently underwent unilateral ONSF, which resulted in improvement in vision bilaterally that continued throughout a 6-month follow-up period.
CONCLUSIONS: Papilledema associated with CVST can be the first presentation of APS. When performed in a timely manner, ONSF can save useful vision and lead to improvement in vision in patients with papilledema due to CVST that is refractory to medical treatment.

Keywords: Antibodies, Antiphospholipid, Hyperhomocysteinemia, Intracranial Thrombosis, Lupus Vasculitis, Central Nervous System, Magnetic Resonance Imaging, papilledema