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

Abstract

Patient: Male, 21-year-old Final Diagnosis: Antiphospholipid antibody syndrome • cerebral venous sinus thrombosis Symptoms: Blurring of vision • confusion • fever • headache Medication: — Clinical Procedure: Laboratory • magnetic resonance imaging Specialty: Neurology • Ophthalmology Objective: Rare disease 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 hyper-tension. 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.

Optic Nerve Sheath Fenestration for Progressive Visual Loss in Cerebral Venous Sinus Thrombosis: A Long-Term Retrospective Observational Study

“…Noteworthily, among eight eyes with an ''NLP'' status before ONSF, four improved to ''LP,'' ''HM,'' or ''CF'' status soon after ONSF (patient NO. 7,15,16), although another four were still in ''NLP'' status, potentially attributable to irreversible optic nerve damage. This indicates that ONSF plays a positive role in relieving the pressure around the optic nerve and in resolving severe visual loss in CVST patients.…”

“…Notably, among 8 eyes with an ''NLP'' status before ONSF, 4 improved to ''LP,'' ''HM,'' or ''CF'' status (patient NO. 7,15,16) in the first postoperative week, although another 4 were still in NLP.…”

“…It is unclear whether ONSF could help improve visual impairment in CVST. Concerns about surgical risks of intraoperative bleeding from anticoagulation greatly limit the use of ONSF in CVST, as most patients have been receiving long-term anticoagulation therapy since diagnosis, so few sporadic cases with relatively short-term follow-ups describing ONSF utility in CVST have been reported [ 3 , 14 , 15 ]. Therefore, an evaluation of ONSF’s efficacy and safety for CVST based on long-term observations is of paramount important, while anticoagulants are used simultaneously.…”

Cerebral Venous Thrombosis in a 17-Year-Old Female Patient: A Case Report

“…Patients with SLE and APS may present CVT; however, it is an unusual clinical manifestation [ 5 , 11 - 13 ]. Signs and symptoms rely upon the location of the thrombus; clinical manifestations include headaches reported in 90% of patients, focal deficit in 40%, altered mental status in 20%, nausea and vomiting in 38.1%, and seizures in 40% [ 6 , 14 ]. The diagnosis could be challenging and requires clinical suspicion confirmed by images showing complete or partially obstructed cerebral veins [ 4 , 12 , 14 , 15 ].…”

“…The most frequent sites involved with thrombosis are the superior sagittal sinus and the transverse sinus [ 5 ]. CVT management includes anticoagulation and treatment of the underlying cause when established [ 6 ]. The majority of patients present favorable outcomes with 80% of them recovering completely [ 3 ].…”