Novel Approaches to the Treatment of Idiopathic Intracranial Hypertension.

PURPOSE OF REVIEW: Idiopathic intracranial hypertension (IIH) typically affects women of childbearing age, is associated with recent weight gain, and can result in debilitating headache as well as papilledema that can cause vision loss. There have been advances in the medical and surgical treatment of affected patients with IIH that can improve outcomes and tolerability of therapy. RECENT FINDINGS: Medical treatment with agents that lower intracranial pressure through pathways other than carbonic anhydrase inhibition are being developed, and medically-directed weight loss as well as bariatric surgery now may be considered as primary therapy. New surgical options including venous sinus stenting have shown efficacy even with cases of severe vision loss. Our treatment options for IIH patients are becoming more diverse, and individualized treatment decisions are now possible to address specific components of the patient's disease manifestations and to lead to IIH remission.

Challenges in the use of Venous Sinus Stenting in the Treatment of Idiopathic Intracranial Hypertension and Pulsatile Tinnitus.

Although numerous case series and meta-analyses have shown the efficacy of venous sinus stenting (VSS) in the treatment of idiopathic intracranial hypertension and idiopathic intracranial hypertension-associated pulsatile tinnitus, there remain numerous challenges to be resolved. There is no widespread agreement on candidacy; pressure gradient and failed medical treatment are common indications, but not all clinicians require medical refractoriness as a criterion. Venous manometry, venography, and cerebral angiography are essential tools for patient assessment, but again disagreements exist regarding the best, or most appropriate, diagnostic imaging choice. Challenges with the VSS technique also exist, such as stent choice and deployment. There are considerations regarding postprocedural balloon angioplasty and pharmacologic treatment, but there is insufficient evidence to formalize postoperative decision making. Although complications of VSS are relatively rare, they include in-stent stenosis, hemorrhage, and subdural hematoma, and the learning curve for VSS presents specific challenges in navigating venous anatomy, emphasizing the need for wider availability of high-quality training. Recurrence of symptoms, particularly stent-adjacent stenosis, poses challenges, and although restenting and cerebrospinal fluid-diverting procedures are options, there is a need for clearer criteria for retreatment strategies. Despite these challenges, when comparing VSS with traditional cerebrospinal fluid-diverting procedures, VSS emerges as a favorable option, with strong clinical outcomes, lower complication rates, and cost-effectiveness. Further research is necessary to refine techniques and indications and address specific aspects of VSS to overcome these challenges.

National trends in the endovascular and surgical treatment of idiopathic intracranial hypertension.

BACKGROUND: The pattern of surgical treatments for Idiopathic Intracranial Hypertension (IIH) in the United States is not well-studied, specifically the trend of utilizing endovascular venous stenting (EVS) as an emerging technique. METHODS: In this cross-sectional study, we aimed to explore the national trend of utilizing different procedures for the treatment of IIH including EVS, Optic Nerve Sheath Fenestration (ONSF), and CSF Shunting, with a focus on novel endovascular procedures. Moreover, we explored rates of 90-day readmission and length of hospital stay following different procedures, besides the effects of sociodemographic and clinical parameters on procedure choice. RESULTS: 36,437 IIH patients were identified from records between 2010 and 2018. Those in the EVS group were 29 years old on average, and 93.4 % were female. Large academic hospital setting was independently associated with higher EVS utilization, while other factors were not predictive of procedure choice. The proportion of EVS use in IIH hospitalizations increased significantly from 2010 to 2018 (P < 0.001), while there was a relative decline in the frequency of shunting procedures (P = 0.001), with ONSF utilization remaining stable (P = 0.39). The rate of 90-day readmission and length of hospital stay were considerably lower following EVS compared to other procedures (Ps < 0.001). CONCLUSION: This study presents novel population-level data on national trends in the frequency and outcome of EVS for IIH therapy. EVS was associated with shorter length of hospital stays and fewer readmission rates. In addition, a continuous increase in venous stenting compared to other procedures suggests an increasing role for endovascular therapies in IIH.

Optic nerve sheath fenestration for salvaging acutely threatened vision in Idiopathic Intracranial Hypertension - A two-year completed follow up.

Objectives: To determine the efficacy and safety of Optic Nerve Sheath Fenestration (ONSF) for salvaging acutely threatened vision in patients of Idiopathic Intracranial Hypertension (IIH). Methods: This retrospective, interventional case series study was performed at Institute of Ophthalmology Mayo Hospital Lahore from September 2017 to September 2019. Nine patients diagnosed with Idiopathic Intracranial Hypertension as per Modified Dandy Criteria, underwent medial trans-conjunctival ONSF. Pre-operative and 1st day, 1st week, 1st month, 1st year, and 2nd year postoperative best-corrected logMAR visual acuities (BCVA) were recorded. Mean BCVA were calculated and compared using paired t-test. P-value <0.05 was taken as significant. Results: All nine patients were female with a mean age of 24 years. The average best-corrected pre-operative logMAR visual acuity (BCVA) in the better eye was 0.5 ± 0.28 and in the worse eye was 1.0 ± 0.57. After the worse eye ONSF, at 1st week mean BCVA in better eyes was 0.27 ± 0.32 (p-value=0.001), while it was 0.43 ± 0.63 (p-value=0.006) in the worse eyes. At 2nd year follow-up after optic nerve sheath fenestration mean BCVA in better eyes was 0.30 ± 0.30 (p-value=0.002) and in worse eyes was 0.44 ± 0.63 (p-value=0.007). Four patients (44.4%) had a subconjunctival hemorrhage, two patients (22.2%) had binocular diplopia, one patient (11.1%) pre-septal cellulitis, and one patient (11.1%) had no improvement in vision because of pre-operative secondary optic atrophy. All patients had unilateral fenestration and bilateral improvement, six patients (66.67%) reported improvement in headache and successful tapering of medical therapy. Conclusion: Optic nerve sheath fenestration is effective as well as a safe surgical procedure to salvage acutely threatened vision in patients of Idiopathic Intracranial Hypertension on maximal medical treatment.

A 20-year multicentre retrospective review of optic nerve sheath fenestration outcomes.

Background: Optic nerve sheath fenestration (ONSF) longitudinal outcomes remain unclear and are vital in the assessment of vision failure in patients with raised intracranial pressure (ICP). Furthermore, limited observational data exists regarding its use in other causes of raised ICP. Objective: To determine the efficacy and safety of ONSF for idiopathic intracranial hypertension (IIH), cerebral venous sinus thrombosis (CVST), and other indications. Method: Multicentre study from a tertiary hospital and specialty eye referral hospital in Melbourne, Australia, from July 2000 to December 2020. A total of 116 eyes from 70 patients undergoing ONSF were retrospectively reviewed with patient demographics, surgery indications, visual acuity (VA), visual fields, fundus photos of optic discs, retinal nerve fibre layer (RNFL) thickness, average thickness of optic discs on optical coherence tomography (OCT), and complications recorded. Parametric tests were used to compare the treatment groups pre- and post-operatively. Results: A total of 116 eyes from 70 patients underwent ONSF, which involved 92 eyes with IIH, 9 eyes with CVST, and 15 eyes with other aetiologies ('Other'). Post ONSF, there was a best corrected visual acuity (BCVA) improvement or stabilisation in 84% of patients in all groups, with 50% achieving a BCVA of 6/6 or better at the final follow-up. RNFL, visual fields, and fundus grades all trended towards improvement, with most improvement noted by day 360. Common complications included transient diplopia (n = 29, 25%) and worsening of visual function requiring further cerebrospinal fluid (CSF) diversion procedures (n = 20, 17%). Complications were most significant in the 'Other' group with 1/3 of eyes requiring further CSF diversion procedures. Conclusion: Our data demonstrates effectiveness in the use of ONSF in papilloedema with visual failure due to IIH or CVST and when other CSF diversion procedures or medical therapies have failed.

Efficacy of Optic Nerve Sheath Fenestration in Patients with Increased Intracranial Pressure.

Objectives: To evaluate the effectiveness of optic nerve sheath fenestration (ONSF) on visual functions in patients with increased intracranial pressure (IIP). Materials and Methods: The medical records of 24 eyes of 17 patients who had IIP due to idiopathic intracranial hypertension, cerebral venous sinus thrombosis, or intracranial cyst and underwent ONSF surgery to prevent visual loss were evaluated. Pre- and postoperative visual acuity, optic disc images, and visual field findings were reviewed. Results: The mean age of the patients was 30.4±8.5 years, and 88.2% were female. The patients' mean body mass index was 28.67±6.1 kg/m2. The mean follow-up time was 24±12.1 months (range: 3-44). At postoperative 3 months, the mean best-corrected distance visual acuity had improved in 20 eyes (83.3%) and stabilized in 4 eyes (16.7%) compared to preoperative values. In visual field mean deviation, an improvement was observed in 10 eyes (90.9%), while 1 eye (9.1%) eye remained stable. Optic disc edema decreased in all patients. Conclusion: This study indicates that ONSF has beneficial effects on visual function in patients with rapidly progressive visual loss caused by increased intracranial pressure.

Optic nerve sheath fenestration for visual impairment in cerebral venous diseases.

Objective: Visual impairment is the most common clinical feature of cerebral venous sinus occlusion or cerebral venous thrombosis-induced intracranial hypertension, which can result in optic atrophy, leading to irreversible vision loss, visual field defections, and finally, permanent blindness. Papilledema is a typical early pathophysiological alteration in visual impairment. Optic nerve sheath fenestration (ONSF) has become increasingly accepted as an option to prevent or halt progressive visual loss owing to its low risk and complications. The objective of this study is to review the latest research progress on ONSF for the treatment of visual impairment related to cerebral venous diseases. Methods: Study were searched following PRISMA guidelines based on three electronic databases (Pubmed, Embase and Medline-Ovid). We used the following keywords and variations as keywords to identify studies: "optic nerve sheath fenestration, papilledema, cerebral venous diseases, cerebral venous stenosis, cerebral venous thrombosis, idiopathic intracranial hypertension". The publication date of studies was restricted between 1,872.1.1 and 2,021.12.31. The application of ONSF in papilledema due to cerebral venous diseases is reviewed. Additionally, the common surgical approaches as well as advantages and disadvantages are also described graphically. Results: With the improvement of specific details of the ONSF procedure and surgical instruments, complications of ONSF have reduced and its safety has been significantly improved, although the number of clinically investigated cases in the literature remains low. Conclusion: We recommend that ONSF should be considered as an imperative alternative to reduce or delay the visual morbidity of cerebral venous diseases, although there is yet no consensus on the optimal surgical timing.

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

INTRODUCTION: Progressive cerebral venous sinus thrombosis (CVST)-induced visual loss remains problematic, despite decreasing overall mortality owing to early diagnosis and aggressive treatment. Optic nerve sheath fenestration (ONSF) improves or stabilizes visual function in patients with idiopathic intracranial hypertension; however, its role in CVST awaits elucidation. We evaluated the efficacy and safety of ONSF in resolving CVST-induced visual impairment based on long-term observation. METHODS: This observational study included 18 patients with progressive CVST-induced visual loss, who had undergone ONSF between 2012 and 2021. Patients received maximum medical therapy, including anticoagulants and intracranial pressure (ICP)-lowering medications. The best-corrected visual acuity (BCVA), visual fields (VFs), and optic nerve head were assessed at baseline, at 1 week after ONSF, and over 6 months after ONSF. Activities of daily living (ADL) and National Eye Institute Visual Function Questionnaire-25 (VFQ-25) scores were assessed at final follow-up. RESULTS: Thirty-one ONSF-treated eyes of 18 patients were included. The mean follow-up duration was 35.6 months (range 1 week-8 years). Two patients were lost to follow-up. Before ONSF, all patients were still experiencing progressive visual loss despite receiving adequate anticoagulation and ICP-lowering therapy. Postoperative BCVA remained stable or improved in 25/31 eyes (80.6%) 1 week postoperatively and 17/28 eyes (60.7%) upon final follow-up. All papilledema resolved postoperatively. No complications were reported except for one transient postoperative diplopia. The median ADL score was 100 (range 25-100), and the mean total VFQ-25 score was 40.6 (range 9.5-87.3). CONCLUSION: This was the largest study to describe ONSF's role in CVST based on a long-term follow-up. Considering its efficacy and favorable safety, ONSF can be considered an important adjunctive approach to resolving progressive visual loss of CVST patients, on the basis of anticoagulation and ICP-lowering therapy.

Cerebral venous sinus thrombosis (CVST) is a cerebrovascular disease that generally affects young patients. Medical treatments include anticoagulants, intracranial pressure (ICP)-lowering medications, and repeated lumbar punctures, effectively reducing CVST's mortality rate. However, CVST still carries a potential risk of progressive vision loss. Optic nerve sheath fenestration (ONSF) has been reported to be effective and safe in protecting visual function of patients with idiopathic intracranial hypertension. However, its efficacy and safety have not been evaluated in visual loss caused by CVST. We were the first to evaluate the efficacy and safety of ONSF in CVST-induced progressive visual loss based on long-term follow-ups. Before ONSF, all patients were still experiencing progressive visual loss despite receiving adequate anticoagulation and ICP-lowering therapy. We found ONSF to be 80.6% (1 week postoperatively) and 60.7% (after long-term follow-up of over 6 months) effective in stabilizing and/or improving visual function as well as 100% effective in papilledema resolution. Moreover, ONSF exhibited a favorable safety profile, with an extremely low complication rate of 5.6% despite under perioperative anticoagulation. Although visual impairment in CVST was reported to be uncommon, it often significantly affects quality of life and social value of patients. Thus, visual loss in CVST deserves more attention from neurologists, neurosurgeons, and ophthalmologists. Considering its efficacy and favorable safety, ONSF could be regarded a potentially important adjunctive approach to resolving progressive visual loss in CVST patients, on the basis of anticoagulation and ICP-lowering therapy.Procedural videos available for this article.

Optic nerve sheath fenestration: Current status in France and comparison of 6 different surgical approaches.

PURPOSE: Optic nerve sheath fenestration (ONSF) is a surgical procedure commonly performed in the Anglo-Saxon countries for the treatment of medically refractory idiopathic intracranial hypertension (IIH). We chose to compare 6 different trans-orbital surgical approaches to ONSF. We also desired to determine the number of optic nerve decompression procedures performed in France in 2019 and 2020. METHODS: Four fresh frozen orbits were dissected at the University of Nice anatomy laboratory. We performed the following surgical approaches: (i) eyelid crease, (ii) lid-split, (iii) medial transconjunctival with medial rectus disinsertion, (iv) medial transconjunctival without rectus disinsertion, (v) lateral transconjunctival and (vi) lateral orbitotomy. For each surgical approach, we measured the distance between the incision and the optic nerve dura mater. We also extracted data from the French National PMSI (Programme de Médicalisation des Systèmes d' Information) database from January 2019 through December 2020 to determine the annual number of optic nerve decompression procedures. RESULTS: The lid crease and medial transconjunctival approaches provided the shortest distance to the optic nerve (average 21mm and 24mm, respectively) and the lowest levels of difficulty compared to the other surgical routes. A total of 23 and 45 optic nerve decompressions were performed in France in 2019 and 2020, respectively. Among them, only 2 and 7 procedures, respectively, were performed through a trans-orbital approach. CONCLUSION: Upper lid crease incision and medial transconjunctival approaches are the most direct and easiest surgical routes when performing an ONSF. We found that ONSF was rarely performed in France. We strongly recommend close cooperation between ophthalmologists, neurologists, neurosurgeons and interventional radiologists.

Systematic scoping review of papilledema in vestibular schwannoma without hydrocephalus.

BACKGROUND: Vestibular schwannoma is a common pathology encountered by neurosurgeons worldwide. Often vestibular schwannoma presents with obstructive hydrocephalus. Papilledema is present in 8% of the patients with vestibular schwannoma, primarily due to obstructive hydrocephalus. Hyperproteinorrhachia is believed to be responsible for papilledema in the absence of hydrocephalus in vestibular schwannoma. However, there is a paucity of literature on the mechanism of papilledema in vestibular schwannoma patients with hydrocephalus. OBJECTIVE: The aim of this study was to conduct a scoping review of scientific literature on papilledema in vestibular schwannoma patients without hydrocephalus. METHODS: Design: This was a systematic scoping review and critical appraisal. Literature Search from PubMed was done following PRISMA-ScR (Preferred Reporting Items for Systematic Reviews and Meta-Analyses Extension for Scoping Reviews) and Joanna Briggs Institute guidelines for conducting and reporting scoping reviews. RESULTS: A total of seven studies, including eight patients, were identified for inclusion in the review. The studies were heterogeneous in terms of reporting for various variables. All the included studies were case reports, with the earliest publication in 1954 and the latest publication in 2020. The mean age of the patients in the included studies was 35 years, with a minimum age of 20 years and maximum age of 64 years. Approximately 62.5% were females, and 37.5% were males in the included study. Only three studies have studied cerebrospinal fluid (CSF) proteins levels in these patients. CONCLUSIONS: There is paucity in literature and a lack of evidence to conclusively state hyperproteinorrhachia as an antecedent to the development of papilledema in vestibular schwannoma patients without hydrocephalus. Younger age and female gender are risk factors for developing papilledema in the absence of hydrocephalus in vestibular schwannoma patients. Brainstem compression due to the large size of vestibular schwannoma can still have a patent aqueduct of Sylvius and no obstruction to CSF flow. The development of papilledema in vestibular schwannoma is a complex interplay of multiple factors that must be studied comprehensively for complete understanding.

Visual recovery after unilateral optic nerve sheath fenestration for pseudotumor cerebri syndrome.

PURPOSE: To evaluate the effect of optic nerve sheath fenestration (ONSF) on the rate of visual function improvement in patients with pseudotumor cerebri syndrome (PTCS). METHODS: Retrospective chart review of patients with PTCS who underwent ONSF between 1998 and 2017. Visual function was evaluated by evaluating visual field (VF), mean deviation (MD), retinal nerve fiber layer (RNFL) thickness, papilledema grade, and visual acuity (VA) prior to and after ONSF. RESULTS: Seventeen female patients aged 17 to 36 years underwent unilateral ONSF. Follow-up averaged 40.1 months. VF MD improved steadily in both eyes up to 12 months. Average RNFL thickness improved in the operated eye from 347 ± 166 mm to 92 ± 27 mm (p < .001) and the non-operated eyes from 306 ± 165 mm to 109 ± 46 mm (p < .001). The grade of papilledema improved in the operated eye from 3.3 ± 1.3 to 0.3 ± 0.7 and the non-operated eye from 3.0 ± 1.6 to 0.18 ± 0.4. There was an exponential rate of improvement in papilledema and RNFL thickness, with the greatest improvement occurring within the first 30 days. Average visual acuity remained intact in both eyes before and after surgery. CONCLUSIONS: ONSF in appropriately selected patients leads to rapid improvement in papilledema and a steady recovery in VF.

Dural Venous Sinus Stenting in Idiopathic Intracranial Hypertension: A National Database Study of 541 Patients.

BACKGROUND: Dural venous sinus stenting (VSS) is an effective intervention for patients with idiopathic intracranial hypertension (IIH) refractory to medical treatment. Our goal was to evaluate the efficacy by utilizing a large multi-institutional sample. METHODS: Five hundred forty-one patients >18 years old who underwent VSS within 3 years of IIH diagnosis were queried using Current Procedural Terminology and International Classification of Diseases, Tenth Revision codes from the TriNetX Analytics Network. Patient demographics, baseline symptoms, procedures, and clinical outcomes were evaluated within 1 year postoperatively. Outcomes examined were headache, tinnitus, blindness/low vision, optic nerve sheath fenestration (ONSF), cerebrospinal fluid (CSF) shunt, and use of medications (acetazolamide, methazolamide, furosemide, topiramate, tricyclic antidepressants, and valproate) for IIH. Prestent and poststent data were compared using Fisher exact test, and the odds ratios were computed using the Baptista-Pike method. RESULTS: The mean age at VSS was 36.7 ± 10.6; 92% were female, 65% of patients were Caucasian, 25% were Black/African American, 1% were Asian, and 9% were of other/unknown race. Within the 1-year follow-up, acetazolamide and topiramate use were significantly reduced post-VSS (P < 0.0001∗; odds ratio, 0.45; confidence interval, 0.35-0.57 and P = 0.03∗; odds ratio, 0.71; confidence interval, 0.52-0.95, respectively). Also, headaches, visual disturbance, dizziness/giddiness, and tinnitus significantly improved post-VSS (P < 0.005∗). Finally, the number of CSF shunt procedures and ONSF procedures demonstrated no significant change post-VSS (P > 0.05). CONCLUSIONS: VSS is an effective and safe procedure resulting in significant improvement of headaches, visual impairment, dizziness, and tinnitus, acetazolamide and topiramate usage were lower after VSS in patients with IIH. The paucity of pre-VSS and post-VSS CSF shunt and ONSF procedure data does not provide enough evidence to establish significance.

Current Indications for Management Options in Pseudotumor Cerebri.

There are surgical options available for those patients with idiopathic intracranial hypertension (IIH) who have significant visual threat or visual deterioration despite best medical management or whose visual deterioration is rapid enough to warrant urgent intervention. Optic nerve sheath fenestrations, venous sinus stenting, and cerebrospinal fluid diversion via ventriculoperitoneal and lumboperitoneal shunting are useful adjuncts in the management of this condition. Significant resources are used in the care of patients with IIH. Further understanding of the pathophysiology of IIH will likely direct future treatment options to more targeted therapeutics including surgery for IIH in the future.

Outcomes of optic nerve sheath fenestration from superomedial eyelid crease approach.

PURPOSE: The purpose of this study is to report the safety and outcomes of optic nerve sheath fenestration (ONSF) performed via superomedial eyelid crease approach. METHODS: A retrospective chart review was performed on patients undergoing superomedial eyelid crease ONSF at a single institution between 2014-2019. Data obtained included preoperative visual acuity (VA), mean deviation (MD) on visual field (VF), papilledema grade, intraoperative time, estimated blood loss, intraoperative complications and optic nerve sheath biopsy results. Postoperative month 6 (POM6) data collected included VA, MD, papilledema grade, and lid contour. Outcome measures included overall change in VA, MD, and papilledema grade at POM6. Statistical analysis was performed using STATA version 16 statistical software. Data analysis was done comparing all patients pre- and postoperatively. Additional subgroup analysis was performed on patients undergoing ONSF for IIH vs other indications, and on the contralateral unoperated eye. RESULTS: A total of 31 eyes of 24 patients were identified, four of whom were excluded due to being lost to follow-up. At POM6, improvement was seen in VA (p<0.001) across all groups. There was improvement in POM6 median papilledema grade and MD, but these did not reach statistical significance. Additional subgroup analysis on patients with IIH showed statistically significant improvement in POM 6 VA (p=0.009), papilledema (p=0.009) and MD (p<0.001), as well as VA improvement in the contralateral unoperated eye (p<0.001). CONCLUSIONS: Superomedial eyelid crease approach for ONSF is a safe and effective surgery in IIH and other conditions of excess pressure within the optic nerve sheath.

Endoscopic transconjunctival optic nerve sheath fenestration for progressive idiopathic visual field deficit: a case report.

Intra-orbital optic nerve sheath fenestration (ONSF) is an effective option in patients with progressive vision loss due to idiopathic intracranial hypertension. Most proposed techniques involve surgical trauma and require disinsertion of the medial rectus muscle; thus, less invasive surgical procedures are needed. Here, a feasible and effective technique of endoscopic intra-orbital ONSF through a conjunctival incision is presented, in a patient with a progressively compromised visual field, papilloedema, and distended subarachnoid space around the optic nerves. The retrobulbar segment of the optic nerve was exposed for incision, avoiding manipulation of the lateral orbital rim bones and irritation of the ciliary microvessels and nerves. The patient regained the entire visual field. ONSF was safely and effectively performed endoscopically through a narrow corridor gained by brushing away the orbital fat with minimal traction on the medial rectus muscle. The small postoperative wound was associated with faster and easier convalescence, and less tissue trauma versus conventional open approaches.

Unilateral Optic Nerve Sheath Fenestration in Idiopathic Intracranial Hypertension: A 6-Month Follow-Up Study on Visual Outcome and Prognostic Markers.

Loss of vision is a feared consequence of idiopathic intracranial hypertension (IIH). Optic nerve sheath fenestration (ONSF) may be an effective surgical approach to protect visual function in medically refractory IIH. In this study, we evaluate the impact of unilateral superomedial transconjunctival ONSF on bilateral visual outcome using a comprehensive follow-up program. A retrospective chart review of IIH patients who underwent unilateral ONSF between January 2016 and March 2021 was conducted. Patients fulfilling the revised Friedman criteria for IIH and who had exclusively received ONSF as a surgical treatment were included. Main outcomes were visual acuity (VA); perimetric mean deviation (PMD); papilledema grade; and optic nerve head elevation (maxONHE) 1 week, 2 weeks, and 1, 3, and 6 months after surgery. VA (p < 0.05), PMD (p < 0.05), papilledema grade (p < 0.01), and maxOHNE (p < 0.001) were improved after 6 months on both the operated and non-operated eye. Prolonged surgical delay impedes PMD improvement (r = -0.78, p < 0.01), and an increasing opening pressure initiates a greater ganglion cell loss (r = -0.79, p < 0.01). In this small case series, we demonstrate that unilateral superonasal transconjunctival ONSF is a safe procedure with an effect on both eyes. Optic nerve head elevation and PMD are feasible biomarkers for assessing early treatment efficacy after ONSF.

Optic nerve sheath fenestration in leukemic patients having increased intracranial pressure: a prospective clinical trial.

PURPOSE: This study aimed to assess the safety and efficacy of optic nerve sheath fenestration (ONSF) in leukemic children having increased intracranial pressure with severe uncontrolled visual loss on medical treatment and not eligible for venous sinus stenting. METHODS: In this non-randomized clinical trial, we included patients presenting with severe sight-threatening papilledema due to increased intracranial pressure following leukemia treatment. All included patients were subjected to a complete history taking, assessment of the visual acuity and fundus examination with fundus photography and grading of papilledema using Frisen scale. Patients were evaluated at 2 weeks and 3 months postoperative by recording the best-corrected visual acuity, assessment of the pupillary reaction and fundus examination and fundus photography with grading of papilledema. RESULTS: Among 20 patients included in this study, there was a statistically significant improvement in visual acuity of both eyes in all patients. The mean postoperative visual acuity was 0.28 ± 0.10 two weeks after surgery in the right eye and it was 0.42 ± 0.16 in the left eye. The mean visual acuity in the right eye three months after surgery was 0.78 ± 0.19 and it was 0.87 ± 0.17 in the left eye. Three months postoperatively, papilledema resolved completely in both eyes. There were no recorded ocular complications. CONCLUSION: In this study, ONSF was sight-saving procedure in all leukemic patients with a significant improvement in the visual acuity, stabilization of the visual function and resolving of the papilledema over follow-up period. There were no recorded intraoperative or postoperative complications. Further well-designed studies are needed to assert upon these results.

Extensive Cerebral Venous Sinus Thrombosis Due to Medroxyprogesterone Acetate.

This retrospective patient report describes a unique case of cerebral venous sinus thrombosis arising from hormonal contraceptive use, and the management of said thrombosis and its secondary ophthalmic manifestations. The patient initially presented with significant visual decline, headaches, florid disc oedema, and vessel tortuosity, due to extensive sinus thromboses that were causing increased intracranial pressure. It was determined that the root cause of the thrombosis was the use of injectable medroxyprogesterone acetate, leading to a hypercoagulable state. Optic nerve sheath fenestration was performed for this patient as an early intervention. The patient, though ultimately experiencing some vision loss, was able to recover and retain limited unilateral central vision, despite a protracted hospital course. We hope to propose that optic nerve sheath fenestration, in conjunction with anti-coagulation therapy, may be a consideration for patients with similar presentations on an individualised basis, in order to prevent vision loss from cerebral venous sinus thrombosis. Though studies are limited, we believe early intervention may be what allowed our patient to retain some central vision, and suggest more studies be done into the utility of this procedure for patients with this clinical vignette.

Management of Idiopathic Intracranial Hypertension During the COVID-19 Pandemic.

BACKGROUND: In the current coronavirus disease 2019 (COVID-19) pandemic, health systems are struggling to prioritize care for affected patients; however, physicians globally are also attempting to maintain care for other less-threatening medical conditions that may lead to permanent disabilities if untreated. Idiopathic intracranial hypertension (IIH) is a relatively common condition affecting young females that could lead to permanent blindness if not properly treated. In this article, we provide some insight and recommendations regarding the management of IIH during the pandemic. METHODS: The diagnosis, follow-up, and treatment methods of IIH during the COVID-19 pandemic period are reviewed. COVID-19, as a mimic of IIH, is also discussed. RESULTS: Diagnosis and follow-up of papilledema due to IIH during the COVID-19 pandemic can be facilitated by nonmydriatic fundus photography and optical coherence tomography. COVID-19 may mimic IIH by presenting as cerebral venous sinus thrombosis, papillophlebitis, or meningoencephalitis, so a high index of suspicion is required in these cases. When surgical treatment is indicated, optic nerve sheath fenestration may be the primary procedure of choice during the pandemic period. CONCLUSION: IIH is a serious vision-threatening condition that could lead to permanent blindness and disability at a relatively young age if left untreated. It could be the first presentation of a COVID-19 infection. Certain precautions during the diagnosis and management of this condition could be taken that may allow appropriate care to be delivered to these patients while minimizing the risk of coronavirus infection.

Idiopathic Intracranial Hypertension - Challenges and Pearls.

Idiopathic intracranial hypertension (IIH) is defined as a syndrome of raised intracranial pressure with normal imaging of the brain and cerebrospinal fluid (CSF) composition. There is a rising incidence and prevalence of this disease related to the increased prevalence of obesity. It typically affects women of working age, and headache is the predominant morbidity in over 90%. The disease is also more prevalent in young males. There are many controversies and myths that surround IIH. There are currently few treatment options for IIH, management is typically medical with those experiencing progressive visual loss undergoing surgical procedures. Weight loss and venous sinus stenting are a few therapies directed at the etiology.