scholarly journals NF1 optic pathway glioma: analyzing risk factors for visual outcome and indications to treat

2020 ◽  
Author(s):  
Amedeo A Azizi ◽  
David A Walker ◽  
Jo-Fen Liu ◽  
Astrid Sehested ◽  
Timothy Jaspan ◽  
...  
Keyword(s):  
Risk Factors ◽  
Optic Atrophy ◽  
Vision Loss ◽  
Multivariable Analysis ◽  
Visual Outcome ◽  
Optic Pathway Glioma ◽  
Optic Pathway ◽  
Visual Outcomes ◽  
Treatment Indications ◽  
Tumor Involvement

Abstract Background The aim of the project was to identify risk factors associated with visual progression and treatment indications in pediatric patients with neurofibromatosis type 1 associated optic pathway glioma (NF1-OPG). Methods A multidisciplinary expert group consisting of ophthalmologists, pediatric neuro-oncologists, neurofibromatosis specialists, and neuro-radiologists involved in therapy trials assembled a cohort of children with NF1-OPG from 6 European countries with complete clinical, imaging, and visual outcome datasets. Using methods developed during a consensus workshop, visual and imaging data were reviewed by the expert team and analyzed to identify associations between factors at diagnosis with visual and imaging outcomes. Results Eighty-three patients (37 males, 46 females, mean age 5.1 ± 2.6 y; 1–13.1 y) registered in the European treatment trial SIOP LGG-2004 (recruited 2004–2012) were included. They were either observed or treated (at diagnosis/after follow-up). In multivariable analysis, factors present at diagnosis associated with adverse visual outcomes included: multiple visual signs and symptoms (adjusted odds ratio [adjOR]: 8.33; 95% CI: 1.9–36.45), abnormal visual behavior (adjOR: 4.15; 95% CI: 1.20–14.34), new onset of visual symptoms (adjOR: 4.04; 95% CI: 1.26–12.95), and optic atrophy (adjOR: 3.73; 95% CI: 1.13–12.53). Squint, posterior visual pathway tumor involvement, and bilateral pathway tumor involvement showed borderline significance. Treatment appeared to reduce tumor size but improved vision in only 10/45 treated patients. Children with visual deterioration after primary observation are more likely to improve with treatment than children treated at diagnosis. Conclusions The analysis identified the importance of symptomatology, optic atrophy, and history of vision loss as predictive factors for poor visual outcomes in children with NF1-OPG.

PRO score: predictive scoring system for visual outcomes after rhegmatogenous retinal detachment repair

2021 ◽  
pp. bjophthalmol-2021-320440
Author(s):  
Louis Z Cai ◽  
Jeffrey Lin ◽  
Matthew R Starr ◽  
Anthony Obeid ◽  
Edwin H Ryan ◽  
...  
Keyword(s):  
Risk Factors ◽  
Retinal Detachment ◽  
Scoring System ◽  
Rhegmatogenous Retinal Detachment ◽  
Vision Loss ◽  
Visual Outcome ◽  
Visual Outcomes ◽  
Poor Visual Outcome ◽  
History Of ◽  
Endothelial Growth Factor

Background/aimsTo compare risk factors for poor visual outcomes in patients undergoing primary rhegmatogenous retinal detachment (RRD) repair and to develop a scoring system.MethodsAnalysis of the Primary Retinal detachment Outcomes (PRO) study, a multicentre interventional cohort of consecutive primary RRD surgeries performed in 2015. The main outcome measure was a poor visual outcome (Snellen VA ≤20/200).ResultsA total of 1178 cases were included. The mean preoperative and postoperative logMARs were 1.1±1.1 (20/250) and 0.5±0.7 (20/63), respectively. Multivariable logistic regression identified preoperative risk factors predictive of poor visual outcomes (≤20/200), including proliferative vitreoretinopathy (PVR) (OR 1.26; 95% CI 1.13 to 1.40), history of antivascular endothelial growth factor (VEGF) injections (1.38; 1.11 to 1.71), >1-week vision loss (1.17; 1.08 to 1.27), ocular comorbidities (1.18; 1.00 to 1.38), poor presenting VA (1.06 per initial logMAR unit; 1.02 to 1.10) and age >70 (1.13; 1.04 to 1.23). The data were split into training (75%) and validation (25%) and a scoring system was developed and validated. The risk for poor visual outcomes was 8% with a total score of 0, 17% with 1, 29% with 2, 47% with 3, and 71% with 4 or higher.ConclusionsIndependent risk factors were compared for poor visual outcomes after RRD surgery, which included PVR, anti-VEGF injections, vision loss >1 week, ocular comorbidities, presenting VA and older age. The PRO score was developed to provide a scoring system that may be useful in clinical practice.

NCOG-22. RETROSPECTIVE ANALYSIS OF VISUAL OUTCOMES AFTER BEVACIZUMAB-BASED THERAPY IN OPTIC PATHWAY GLIOMA

2021 ◽  
Vol 23 (Supplement_6) ◽  
pp. vi156-vi156
Author(s):  
Benjamin Siegel ◽  
Tobey MacDonald ◽  
David Wolf
Keyword(s):  
Radiographic Progression ◽  
Primary Treatment ◽  
Tumor Burden ◽  
Optic Pathway Glioma ◽  
Optic Pathway ◽  
Visual Outcomes ◽  
Treatment Indications ◽  
Before And After ◽  
Stable Vision ◽  
Clinically Significant

Abstract BACKGROUND Patients with optic pathway glioma (OPG) are vulnerable to debilitating visual impairment. Consequently, vision stabilization is a primary treatment goal. Bevacizumab has demonstrated promising effects on radiographic tumor burden, but less is known about its impact on vision. Our objective was to characterize visual outcomes associated with bevacizumab-based therapy (BBT) in OPG. METHODS This is a single-institution, retrospective review of patients treated with BBT for OPG from 2011 to 2020. Ophthalmologic and radiographic data were abstracted before and after treatment. Clinically significant visual acuity (VA) impairment was defined as logMAR > 0.5 and change in VA was defined as change from baseline of logMAR ≥ 0.2. RESULTS Sixteen patients (13 sporadic OPG, 3 NF1-associated OPG) with evaluable vision outcomes were identified. Treatment indications were radiographic progression (N=15) and vision deterioration (N=4). Prior to BBT, 15 (94%) had failed at least one chemotherapy regimen. BBT regimens included bevacizumab/irinotecan (N=12), bevacizumab monotherapy (N=3) and bevacizumab/vinblastine (N=1). Nine patients (56%) had baseline VA impairment. Thirteen patients (81%) had stable or improved vision after BBT, including 8 of 9 with baseline VA impairment and all 4 patients with vision deterioration as a treatment indication. Eleven patients (69%) had radiographic progression following BBT (Median time-to-progression 66 weeks, IQR 27 weeks), 9 of whom had stable vision at time of progression. There were no associations between VA and age at treatment, NF1-status, histology, or BBT regimen. CONCLUSIONS BBT was associated with favorable visual outcomes for most patients with OPG in this modest retrospective cohort. Consistent with prior research, radiographic and ophthalmologic outcomes were discordant; a majority of patients experienced progressive disease despite stable vision. Next steps include (1) assessing visual field and optical coherence tomography outcomes in the same cohort and (2) comparing outcomes for BBT with other common therapies including carboplatin/vincristine and vinblastine.

scholarly journals Optic Pathway Glioma in Type 1 Neurofibromatosis: Review of Its Pathogenesis, Diagnostic Assessment, and Treatment Recommendations

Cancers ◽  
2019 ◽  
Vol 11 (11) ◽  
pp. 1790 ◽  
Author(s):  
Matteo Cassina ◽  
Luisa Frizziero ◽  
Enrico Opocher ◽  
Raffaele Parrozzani ◽  
Ugo Sorrentino ◽  
...  
Keyword(s):  
Clinical Management ◽  
Visual Outcome ◽  
Genetically Engineered ◽  
Optic Pathway Glioma ◽  
Anatomical Site ◽  
Optic Pathway ◽  
Central Nervous System Tumor ◽  
Comprehensive Overview ◽  
Type 1 Neurofibromatosis

Type 1 neurofibromatosis (NF1) is a dominantly inherited condition predisposing to tumor development. Optic pathway glioma (OPG) is the most frequent central nervous system tumor in children with NF1, affecting approximately 15–20% of patients. The lack of well-established prognostic markers and the wide clinical variability with respect to tumor progression and visual outcome make the clinical management of these tumors challenging, with significant differences among distinct centers. We reviewed published articles on OPG diagnostic protocol, follow-up and treatment in NF1. Cohorts of NF1 children with OPG reported in the literature and patients prospectively collected in our center were analyzed with regard to clinical data, tumor anatomical site, diagnostic workflow, treatment and outcome. In addition, we discussed the recent findings on the pathophysiology of OPG development in NF1. This review provides a comprehensive overview about the clinical management of NF1-associated OPG, focusing on the most recent advances from preclinical studies with genetically engineered models and the ongoing clinical trials.

scholarly journals Visual outcome of a cohort of children with neurofibromatosis type 1 and optic pathway glioma followed by a pediatric neuro-oncology program

2007 ◽  
Vol 9 (4) ◽  
pp. 430-437 ◽  
Author(s):  
Paola Dalla Via ◽  
Enrico Opocher ◽  
Maria Luisa Pinello ◽  
Milena Calderone ◽  
Elisabetta Viscardi ◽  
...  
Keyword(s):  
Neurofibromatosis Type 1 ◽  
Visual Outcome ◽  
Neurofibromatosis Type ◽  
Optic Pathway Glioma ◽  
Optic Pathway

Visual Outcomes After Radiation Therapy for Optic Pathway Glioma

2019 ◽  
Vol 105 (1) ◽  
pp. E631
Author(s):  
S. Quesada ◽  
K. Coca ◽  
M. Hoehn ◽  
I. Qaddoumi ◽  
T.E. Merchant ◽  
...  
Keyword(s):  
Radiation Therapy ◽  
Optic Pathway Glioma ◽  
Optic Pathway ◽  
Visual Outcomes

Visual Outcome in Ocular Sarcoidosis: Retrospective Evaluation of Risk Factors

2011 ◽  
Vol 21 (6) ◽  
pp. 802-810 ◽  
Author(s):  
Elisabetta Miserocchi ◽  
Giulio Modorati ◽  
Federico Di Matteo ◽  
Laura Galli ◽  
Paolo Rama ◽  
...  
Keyword(s):  
Risk Factors ◽  
Visual Acuity ◽  
Macular Edema ◽  
Cystoid Macular Edema ◽  
Multivariable Analysis ◽  
Visual Outcome ◽  
Ocular Sarcoidosis ◽  
Poor Visual Outcome ◽  
Posterior Synechia

Purpose. To analyze risk factors associated with poor visual outcome in patients with ocular sarcoidosis. Methods. In this retrospective study, charts of 44 patients with uveitis and biopsy-proven sarcoidosis were reviewed. Ocular parameters evaluated were as follows: location, type of uveitis, visual acuity, presence of posterior synechia, iris nodules, vitritis, snowballs, chorioretinal lesions, retinal vasculitis, papillitis, macular edema, cataract, and glaucoma. Final visual acuity of the worst-seeing eye at last follow-up was the outcome considered in univariable and multivariable analyses. Visual acuity of the worst-seeing eye was stratified into 2 categories according to the threshold 20/50 (≤20/50 and >20/50). Results. A total of 44 patients with bilateral uveitis were studied. The majority of patients presented with panuveitis (52%), granulomatous type (61%), posterior synechia (62%). The most frequent vision-threatening complications were cystoid macular edema (56%) and cataract (56%). The median best-corrected visual acuity in the worst-seeing eye at presentation and at end of follow-up was respectively 0.4 (interquartile range [IQR] 0.26–0.80) and 0.63 (IQR 0.36–1.00). At univariable analysis, the presence of iris nodules (p=0.049), cystoid macular edema (p=0.007), and cataract (p=0.007) were clinically significant conditions for a visual outcome of 20/50 or worse in the worst-seeing eye. In multivariable analysis, cystoid macular edema (p=0.034) was the only statistically significant predictor associated with unfavorable visual outcome. Conclusions. In this study, we attempted to find risk factors related to poor visual outcome in patients with ocular sarcoidosis. The results suggest that only the presence of cystoid macular edema was significantly associated with worst visual outcome.

scholarly journals Intraocular Foreign Bodies: Clinical Characteristics and Factors Affecting Visual Outcome

2021 ◽  
Vol 2021 ◽  
pp. 1-9
Author(s):  
Yanyan Liang ◽  
Shuang Liang ◽  
Xiaoli Liu ◽  
Danyan Liu ◽  
Jialiang Duan
Keyword(s):  
Risk Factors ◽  
Study Design ◽  
Clinical Characteristics ◽  
Foreign Bodies ◽  
Visual Outcome ◽  
Factors Affecting ◽  
Visual Outcomes ◽  
Entrance Wound ◽  
Wound Length ◽  
Capsule Rupture

Objective. To investigate the clinical characteristics and factors affecting visual outcome in patients with intraocular foreign bodies (IOFBs) and determine the risk factors for the development of endophthalmitis. Study Design. A retrospective case-series study design was adopted. Subjects. In total, 242 patients (242 eyes) who were hospitalized and underwent surgical treatment for IOFB at the Second Hospital of Hebei Medical University between January 1, 2008, and December 31, 2019, were included. Methods. The demographic data, cause of injury, characteristics of IOFBs, postinjury ocular manifestations, and surgical details of the subjects were collected, and the factors affecting visual outcome and endophthalmitis development were analyzed. Results. The most common cause of IOFBs was the propulsion of foreign bodies into the eye due to hammering (149 cases, 61.57%), followed by foreign body penetration (57 cases, 23.55%). Most of the subjects were young adult men who sustained injuries in the work environment. Poorer visual outcomes were found in subjects with initial presenting symptoms visual acuity (PVA) < 0.1, largest IOFB diameter ≥ 3 mm, IOFBs located in the posterior segment, wound length > 5 mm, entrance wound length larger than the largest IOFB diameter, concomitant retinal detachment, concomitant vitreous hemorrhage, concomitant endophthalmitis, and concomitant proliferative vitreoretinopathy (PVR). Factors related to the development of endophthalmitis included lens capsule rupture, time of stage 1 repair surgery ≥ 24 h after trauma, removal of IOFBs ≥ 24 h after trauma, and nonadministration of intravitreal antibiotic injection. Conclusion. Among patients with IOFBs, initial PVA < 0.1, entrance wound length larger than the largest IOFB diameter, concomitant endophthalmitis, and concomitant PVR were risk factors for poor visual outcomes. Lens capsule rupture was a risk factor for endophthalmitis development, and the administration of intravitreal antibiotic injection was a protective factor against endophthalmitis development.

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