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To report associations between disease- and treatment-related variables and rates of recurrence-free survival and ocular survival in eyes treated with ophthalmic artery chemosurgery (OAC) for retinoblastoma. Pre-post study. All eyes treated with OAC for retinoblastoma at Memorial Sloan Kettering Cancer Center between May 2006 and February 2017. This retrospective review included 452 retinoblastoma eyes treated with OAC. The Kaplan-Meier method was used to estimate recurrence-free survival (RFS), progression-free survival (PFS) and ocular survival (OcS), and Cox regression was used to estimate hazard ratios. Eyes treated in the pre-intravitreous chemotherapy era were analyzed separately from eyes treated in the intravitreal era. Recurrence-free survival, ocular survival, associations with risk of recurrence. Disease and treatment characteristics were recorded over a median 23.6 month follow-up. One-year OcS, PFS and RFS were 96% (95% CI 93-99%), 88% (95% CI 88-94%) and 74% (95% CI 67-81%) in the pre-intravitreal era and 96% (95% CI 94-99%), 93% (95% CI 89-96%) and 78% (95% CI 72-83%) in the intravitreal era, respectively. Presence of vitreous seeds was associated with increased risk of recurrence in the pre-intravitreal era but not in the intravitreal era. Longer time interval between OAC sessions was associated with increased risk of recurrence and majority OAC access via the ophthalmic artery was associated with decreased risk of recurrence in both eras. Approximately a quarter of eyes initially treated with ophthalmic artery chemosurgery develop recurrent disease, with the majority of recurrences within the first year following completion of OAC. Despite this, these eyes have a very good chance of salvage. In eyes with vitreous seeds at presentation, intravitreal injections are useful in minimizing future vitreous recurrence. Eyes that receive the majority of drug infusions via non-ophthalmic artery routes or greater interval between OAC are more likely to recur and might warrant closer monitoring.

To report globe salvage rates, patient survival and adverse events of ophthalmic artery chemosurgery (OAC) for International Classification of Retinoblastoma (ICRB) group D retinoblastoma (naive and after prior failures). Single institution retrospective review of all Group D eyes treated with OAC from 5/2006-12/2012. Patients were treated according to our previously-published techniques. Primary outcome was globe retention without need for external beam radiotherapy (EBRT). Demographics, prior treatments, OAC agents used, and adverse events were also recorded. 112 group D eyes (103 patients) that underwent OAC were included (average follow-up was 34 months, range: 2-110 months). 47 eyes were treatment-naïve, 58 eyes received prior treatments elsewhere, and 7 young infants (7 eyes) underwent our published \"bridge therapy\" (single agent intravenous carboplatin) until old enough to undergo OAC. Median number of OAC sessions/eye was 3 (range 1-9). 110/112 eyes received intra-arterial melphalan, but only 31 eyes received melphalan alone. 43 eyes received carboplatin, and 78 eyes received topotecan (never as a single agent). 80/112 eyes received >1 drug over their treatment course, and 39 eyes received all three agents. 24 eyes (16 pretreated, 7 treatment-naïve, 1 bridge) failed treatment and required enucleation during the study period. Enucleation and EBRT were avoided in 88/112 eyes (78.6%; including 40/47 [85.1%] treatment-naïve eyes, 42/58 [72.4%] previously-treated eyes, and 6/7 eyes [85.7%] among bridge patients). By Kaplan-Meier survival analysis, globe salvage rate was 74% at 110 months among all patients, and 85% at 110 months in the treatment-naïve subgroup. Transient grade 3/4 neutropenia was more common in patients receiving OAC bilaterally. No child died of metastatic disease. OAC is effective for curing group D retinoblastoma, achieving rates of globe salvage many times higher than systemic chemotherapy (10-47%), even in eyes that previously failed other treatments. OAC can be performed multiple times, using multiple agents, on one or both eyes of patients.

Intra-arterial chemotherapy (IAC) for retinoblastoma is a minimally invasive and chemotherapeutic approach resulting in eye salvage and vision restoration or preservation. Moreover, IAC has proven to effectively treat advanced retinoblastoma while not compromising patient survival. Our institutional experience with IAC for retinoblastoma has included over 500 patients and over 2400 intra-arterial infusions. Each infusion is completed with the use of a micropuncture for arterial access and microcatheter for infusion, eliminating the need for guide catheters and related complications (video 1). This treatment modality has resulted in >95% ocular survival and reduces enucleation to <5% for this population. In addition to local therapy, including cryotherapy, intravitreal chemotherapy, or laser treatments, by the ophthalmologist, IAC has become an important component of comprehensive multidisciplinary and multimodal therapy for this disease. For what used to require a possibly vision-sacrificing procedure, retinoblastoma treated with IAC minimizes the need for enucleation while maximizing both patient and ocular survival.SP110.1136/neurintsurg-2022-018957.supp1Supplementary data Video 1 media110.1136/neurintsurg-2022-018957.video016305700628112neurintsurg2022018957media1

Abstract BACKGROUND: Neuroform stent-assisted coil embolization facilitates the endovascular treatment of wide-necked intracranial aneurysms. However, the safety and efficacy of its long-term use have not been fully elucidated. OBJECTIVE: To retrospectively examine the long-term results of Neuroform stent usage in conjunction with coil embolization in wide-necked intracranial aneurysms. METHODS: Between November 2002 and December 2010, 79 patients harboring wide-necked intracranial aneurysms were treated with use of the Neuroform stent. The stenting procedure failed in 2 patients. Therefore, 77 patients harboring 79 intracranial aneurysms were included for analysis. Patient and aneurysm characteristics, progression of aneurysm occlusion, and occurrence of complications were analyzed. Follow-up imaging included digital subtraction angiography (DSA) or magnetic resonance angiography (MRA). Kaplan-Meier analysis, as well as univariate analysis were performed to determine the progression of aneurysm occlusion and to examine the predictive factors for complete aneurysm occlusion, respectively. RESULTS: Overall, complete aneurysm occlusion was observed in 42.4% of the cases immediately after treatment and progressed to 96.5% at 7-year follow-up. The mean angiographic follow-up time was 25.8 months (range, 0–84 months). Eleven aneurysms (14%) were re-treated. Sixty-eight patients (88.3%) had favorable clinical outcome with a modified Rankin Scale (mRS) ⩽ 1, 3 patients (3.9%) had an mRS of 2, and 5 patients (6.5%) did not have a clinical follow-up. The mean clinical follow-up time was 45.4 months (range, 3–92 months). One patient (1.3%) died of a procedure-related hemorrhage. CONCLUSION: Neuroform stent-assisted coil embolization of wide-necked intracranial aneurysms prevents hemorrhage and provides a high rate of aneurysm occlusion at long-term follow-up.

To report on the influence of ophthalmic artery chemosurgery (OAC) on enucleation rates, ocular and patient survival from metastasis and impact on practice patterns at Memorial Sloan Kettering for children with advanced intraocular unilateral retinoblastoma. Single-center retrospective review of all unilateral retinoblastoma patients with advanced intraocular retinoblastoma treated at MSKCC between our introduction of OAC (May 2006) and December 2014. End points were ocular survival, patient survival from metastases and enucleation rates. 156 eyes of 156 retinoblastoma patients were included. Primary enucleation rates have progressively decreased from a rate of >95% before OAC to 66.7% in the first year of OAC use to the present rate of 7.4%. The percent of patients receiving OAC has progressively increased from 33.3% in 2006 to 92.6% in 2014. Overall, ocular survival was significantly better in eyes treated with OAC in the years 2010-2014 compared to 2006-2009 (p = 0.023, 92.7% vs 68.0% ocular survival at 48 months). There have been no metastatic deaths in the OAC group but two patients treated with primary enucleation have died of metastatic disease. OAC was introduced in 2006 and its impact on patient management is profound. Enucleation rates have decreased from over 95% to less than 10%. Our ocular survival rate has also significantly and progressively improved since May 2006. Despite treating more advanced eyes rather then enucleating them patient survival has not been compromised (there have been no metastatic deaths in the OAC group). In our institution, enucleation is no longer the most common treatment for advanced unilateral retinoblastoma.

BackgroundThe River stent is the first stent specifically designed for intracranial venous sinuses. We report the 1-year results of the River trial, performed to obtain Humanitarian Device Exemption approval of the River stent in the United States (US).MethodsThe River trial was a prospective, open-label, multicenter, single-arm trial which enrolled 39 subjects at 5 US centers. Eligible patients had clinical diagnosis of idiopathic intracranial hypertension (IIH) with severe headaches or visual field loss and had failed medical therapy. The primary safety endpoint was the 1-year rate of major adverse events compared with cerebrospinal fluid (CSF) shunting using historical controls. The primary benefit endpoint was a composite at 1 year of clinical improvement and absence of venous sinus stenosis. Secondary endpoints included improvement in pulsatile tinnitus, visual symptoms, quality of life (QOL) scores, and medications.ResultsAll procedures were technically successful. There was one serious adverse event, a gastrointestinal hemorrhage observed 2 months after the procedure while the patient was still on dual antiplatelet therapy. The primary safety endpoint was met with a rate of major adverse event of 5.4% versus 51.7% for CSF shunts. The primary benefit endpoint was achieved in 60% of trial participants. Additional improvements were also observed in opening CSF pressure, headaches, papilledema, pulsatile tinnitus, visual symptoms, and QOL scores. Post hoc analysis demonstrated that subjects with minimal or absent papilledema at baseline showed similar improvement compared with subjects with papilledema at baseline, in terms of headaches, pulsatile tinnitus, and QOL.ConclusionsThe River study 1- year results establish safety and suggest efficacy for venous sinus stenting in IIH subjects who have failed medical therapy.

BackgroundIntra-arterial chemotherapy (IAC) is a growing method of therapy for retinoblastoma (Rb). There is an absence of data to support the safety of catheterization with intra-arterial infusion in this pediatric populationObjectiveTo focus on the non-ocular catheter/procedural-related complications that our practice has experienced in order to lay a foundation for practices interested in performing these procedures and hopefully, to help prevent them from occurring.MethodsThis is a retrospective review of the patient population with Rb treated in our center from May 2006 through May 2024. Every procedure performed was reviewed for non-ocular catheterization-related complications. This review included complications of access, the distal vessel (thrombosis, stenosis, and dissection), and non-ocular infarcts.ResultsThere were 2281 vascular access events, and 2681 distal catheterization procedures were performed for IAC infusion on 623 pediatric patients with Rb. Mean age of the population was 18.9 months. There were 31 complications directly related to catheterization: 7 (0.3%) related to femoral artery access and 24 (0.9%) were distal vessel injuries. Two (0.07% of total catheterizations) of the distal vessel injuries were asymptomatic cerebral infarcts diagnosed on follow-up MRI.ConclusionCatheterization with IAC can be performed safely in this young pediatric population. There is a trend for fewer complications when using the smallest catheter system possible for procedures.

Predictions of visual outcomes are useful in clinical and family decisions regarding treatment for retinoblastoma. Very little has been published on the association of post-treatment visual acuity with pre-treatment electroretinography (ERG), which can be performed on infants too young to reliably quantify visual acuity. To report associations of pre-treatment ERG with post-treatment visual acuity in eyes with advanced retinoblastoma treated with ophthalmic artery chemosurgery (OAC). Retrospective case-control study of eyes treated from 2006 through 2017, with mean follow-up of 51 months (range 2.3-150 months). Single large academic center. Group D and E eyes treated with OAC at Memorial Sloan Kettering Cancer Center with recorded visual acuity and ERG (30Hz flicker). Snellen visual acuity (uncorrected) compared to initial 30Hz flicker ERG. This study included 157 Group D and E eyes. Results of the Jonckheere-Terpstra test for trend were statistically significant and indicated that eyes with lower pre-treatment ERG readings tended to have more visual impairment post-treatment. Among eyes with initial ERG 75+ μV, 11 of 32 eyes (34%) had visual acuity 20/40 or better. Among eyes with ERG 0 μV, 44 of 46 (96%) had visual acuity of 20/200 or worse. Eyes with advanced intraocular retinoblastoma treated with OAC can achieve excellent visual acuity, but poor ERG at initial visit is associated with poor visual acuity after treatment in the majority of eyes. Expectations regarding visual potential may influence decisions about treatment.

Report on the 7-year experience with bilateral ophthalmic artery chemosurgery (OAC-Tandem therapy) for bilateral retinoblastoma. Retrospective, single institution study. 120 eyes of 60 children with bilateral retinoblastoma treated since March 2008. Retrospective review of all children treated at Memorial Sloan Kettering with bilateral ophthalmic artery chemosurgery (Melphalan, Carboplatin, Topotecan, Methotrexate) delivered in the same initial session to both naïve and previously treated eyes. Ocular survival, metastatic disease, patient survival from metastases, second cancers, systemic adverse effects, need for transfusion of blood products, electroretinogram before and after treatment. 116 eyes were salvaged (4 eyes were enucleated: 3 because of progressive disease, 1 family choice). Kaplan Meier ocular survival was 99.2% at one year, 96.9% at 2 and 3 years and 94.9% for years 4 through 7. There were no cases of metastatic disease or metastatic deaths with a mean follow-up of 3.01 years. Two children developed second cancers (both pineoblastoma) and one of them died. Transfusion of blood products was required in 3 cases (4 transfusions), 1.9%. Two children developed fever/neutropenia requiring hospitalization (0.95%). ERGs were improved in 21.6% and unchanged after treatment in 52.5% of cases (increase or decrease of less than 25μV). Bilateral ophthalmic artery chemosurgery is a safe and effective technique for managing bilateral retinoblastoma-even when eyes are advanced bilaterally, and if both eyes have progressed after systemic chemotherapy. Ocular survival was excellent (94.9% at 8 years), there were no cases of of metastatic disease and no deaths from metastatic disease, but children remain at risk for second cancers. In 21.6% of cases ERG function improved. Despite using chemotherapy in both eyes in the same session, systemic toxicity was low.

BackgroundEndovascular transvenous embolization of spinal cerebrospinal fluid–venous fistulas (CVFs) has been shown in small studies to be a safe and effective treatment for spontaneous intracranial hypotension (SIH).ObjectiveTo validate the safety and effectiveness of this procedure and to describe catheterization techniques available to safely perform these embolization procedures.ResultsA total of 21 patients with confirmed CVF underwent 23 endovascular embolization procedures; 18 (86%) patients achieved both clinical and radiographic resolution of their SIH. There were no major neurologic complications from any procedure. One patient had postoperative pulmonary embolism and deep vein thrombosis. The most common side effects were prolonged symptoms of rebound intracranial hypertension (29%) and prolonged back pain with radiculopathy (24%). The azygous vein was used for catheterizing the level of interest in 83% of embolization procedures; the lumbar ascending vein was used for catheterization in 17% of the procedures; and direct catheterization of the segmental/foraminal vein at the level of interest was performed in 61% of the procedures. The epidural venous plexus was accessed and traversed to the level of interest in 39% of the procedures.ConclusionEndovascular transvenous embolization of CVF is a safe, well-tolerated, and effective treatment for SIH. Given the variability of the venous anatomy, there are multiple routes of catheterization that can be performed to access the level of interest when performing these procedures.