Alaa Tayyib, Deepika C Parameswarappa, Peter J Kertes, Rajeev Muni, Anupreet Tumber, Gregory Costain, Alex Schramm, Heather MacDonald, Regan Klatt, Ajoy Vincent, Elise Héon
{"title":"Insights into the effects of subretinal voretigene neparvovec-rzyl in RPE65-associated Leber congenital amaurosis: an 18-Month report.","authors":"Alaa Tayyib, Deepika C Parameswarappa, Peter J Kertes, Rajeev Muni, Anupreet Tumber, Gregory Costain, Alex Schramm, Heather MacDonald, Regan Klatt, Ajoy Vincent, Elise Héon","doi":"10.1016/j.jcjo.2024.12.006","DOIUrl":"https://doi.org/10.1016/j.jcjo.2024.12.006","url":null,"abstract":"<p><strong>Objective: </strong>Assess safety and effectiveness of subretinal gene replacement therapy at 18 months post treatment.</p><p><strong>Design: </strong>Retrospective, longitudinal study conducted at the Hospital for Sick Children in Toronto, Canada.</p><p><strong>Participants: </strong>Patients with bi-allelic RPE65 variants, early onset retinal degeneration, and residual viable retina who underwent voretigene neparvovec r-zyl gene replacement therapy.</p><p><strong>Methods: </strong>Data collected included demographic information, molecular genetic results, and comprehensive ocular assessment results from preoperataive and postoperative visits up to 18 months. To assess the treatment's efficacy, postoperative best corrected visual acuity, full-field stimulus test (FST), visual field (VF) area, optical coherence tomography, and global satisfaction were compared to preoperative findings.</p><p><strong>Results: </strong>The procedures were safe with no complications. There was no significant improvement in visual acuity. Three eyes showed a slight reduction in VF area. All showed a reduction in ellipsoid zone thickness and area, but the outer nuclear layer thickness and area were stable. All had a significant improvement in retinal sensitivity, as per FST, allowing better navigation in a dim environment. All 3 patients reported being \"very satisfied\".</p><p><strong>Conclusion: </strong>Following a safe gene replacement therapy, the 3 first Canadian cases had an improvement in retinal sensitivity as per FST, and our patients described their experience as positively life changing.</p>","PeriodicalId":9606,"journal":{"name":"Canadian journal of ophthalmology. Journal canadien d'ophtalmologie","volume":" ","pages":""},"PeriodicalIF":3.3,"publicationDate":"2025-01-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143000563","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Raphael Lechtenboehmer, Matthias M Mauschitz, Frank G Holz, Robert P Finger, Maximilian W M Wintergerst
{"title":"A case of VEXAS syndrome with therapy refractive macular involvement.","authors":"Raphael Lechtenboehmer, Matthias M Mauschitz, Frank G Holz, Robert P Finger, Maximilian W M Wintergerst","doi":"10.1016/j.jcjo.2025.01.002","DOIUrl":"10.1016/j.jcjo.2025.01.002","url":null,"abstract":"","PeriodicalId":9606,"journal":{"name":"Canadian journal of ophthalmology. Journal canadien d'ophtalmologie","volume":" ","pages":""},"PeriodicalIF":3.3,"publicationDate":"2025-01-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142982875","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Deepika C Parameswarappa, Kirk A J Stephenson, Mark Seamone, Cynthia X Qian, Rajeev H Muni, Peter J Kertes, Ajoy Vincent, Elise Héon
{"title":"\"Blindness\" is not a contraindication for voretigene neparvovec-rzyl treatment: a review of 9 cases.","authors":"Deepika C Parameswarappa, Kirk A J Stephenson, Mark Seamone, Cynthia X Qian, Rajeev H Muni, Peter J Kertes, Ajoy Vincent, Elise Héon","doi":"10.1016/j.jcjo.2024.12.013","DOIUrl":"10.1016/j.jcjo.2024.12.013","url":null,"abstract":"<p><strong>Objective: </strong>Biallelic RPE65 pathogenic variants may cause Leber congenital amaurosis (LCA). Voretigene neparvovec-rzyl (VN, Luxturna) is the only approved subretinal gene therapy that demonstrated benefit and safety. The eligibility criteria are vague and variable between centres. This is the first comprehensive outcome report of RPE65-LCA patients with World Health Organization blindness criteria vision treated with VN.</p><p><strong>Design: </strong>Multicentre retrospective case series.</p><p><strong>Participants: </strong>Patients meeting the treatment criteria for VN who had best-corrected visual acuity (BCVA) <20/400 or visual field (VF)III4e isopter <10°.</p><p><strong>Methods: </strong>Patients were followed for a mean of 11.1 ± 4.7 months. Age, sex, BCVA, central retinal thickness (CRT), retinal atrophy, VF, full-field stimulus testing (FST), and subjective impressions were assessed.</p><p><strong>Results: </strong>Nine patients met the inclusion criteria (mean: BCVA 1.89 LogMAR, range: 1.4 - 2.7 LogMAR, mean age: 28.7-years-old, range: 17-59 years). Though VF area did not improve, FST improved in patients with better baseline FST (-8.83 dB vs -0.56 dB; p = 0.010), and better VFV4e (7245 vs 341<sup>o2</sup>; p < 0.001) and III4e (596.1 vs 24.8<sup>o2</sup>; p = 0.011) area. VA improved in younger (20 vs 32 years; p = 0.011) patients with thinner CRT<sup>1mm</sup> (155 vs 193 µm; p = 0.038). VFV4e loss occurred in older (38 vs 19 years; p = 0.001) patients with worse baseline V4e area (1728 vs 8159<sup>o2</sup>; p < 0.001). Subjective improvement in dim light navigation skills occurred in younger patients (20.3 vs 45.3 years; p < 0.001).</p><p><strong>Conclusions: </strong>Blindness is not a contraindication to VN treatment for RPE65-LCA. Superior results correlated with greater baseline FST but not with CRT<sup>1mm</sup>, provided that measurable outer retinal structures persist.</p>","PeriodicalId":9606,"journal":{"name":"Canadian journal of ophthalmology. Journal canadien d'ophtalmologie","volume":" ","pages":""},"PeriodicalIF":3.3,"publicationDate":"2025-01-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142944911","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Michael T Kryshtalskyj, Jeremy Rau, Emi Sanders, Helen Chung, Andrew C Crichton, Chryssa McAlister, Alex M Ragan
{"title":"A national benchmarking survey for quality improvement and safety in ophthalmology.","authors":"Michael T Kryshtalskyj, Jeremy Rau, Emi Sanders, Helen Chung, Andrew C Crichton, Chryssa McAlister, Alex M Ragan","doi":"10.1016/j.jcjo.2024.11.013","DOIUrl":"10.1016/j.jcjo.2024.11.013","url":null,"abstract":"","PeriodicalId":9606,"journal":{"name":"Canadian journal of ophthalmology. Journal canadien d'ophtalmologie","volume":" ","pages":""},"PeriodicalIF":3.3,"publicationDate":"2025-01-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142876244","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Sunil Ruparelia, Rodolfo Bonatti, Jeremy A Murphy, Marcelo T Nicolela, Brennan D Eadie, Balwantray C Chauhan, Oksana M Dyachok, Lesya M Shuba
{"title":"Twenty four-hour intraocular pressure fluctuation in treated glaucoma patients: a pilot study.","authors":"Sunil Ruparelia, Rodolfo Bonatti, Jeremy A Murphy, Marcelo T Nicolela, Brennan D Eadie, Balwantray C Chauhan, Oksana M Dyachok, Lesya M Shuba","doi":"10.1016/j.jcjo.2024.11.010","DOIUrl":"10.1016/j.jcjo.2024.11.010","url":null,"abstract":"<p><strong>Objective: </strong>To conduct a pilot study to evaluate and compare the 24-hour habitual intraocular pressure (IOP) and ocular perfusion pressure (OPP) fluctuation in glaucoma patients treated with medical therapy, selective laser trabeculoplasty (SLT) or trabeculectomy.</p><p><strong>Design: </strong>Pilot study.</p><p><strong>Participants: </strong>Criteria for inclusion were patients aged 18 years or older with well-controlled IOP with either maximum tolerated medical therapy, previous SLT, or previous trabeculectomy.</p><p><strong>Methods: </strong>Recruited patients were admitted to the sleep lab for 24-hour serial habitual IOP and blood pressure measurements. IOP and OPP fluctuation among the 3 treatment groups were compared.</p><p><strong>Results: </strong>Thirty three (33) eyes from 33 patients were recruited in this study, including 11 patients in the medical therapy group, 11 patients in the SLT group, and 11 patients in the trabeculectomy group. The medical therapy group was found to have significantly higher 24-hour IOP fluctuation (8.3 ± 1.6 mmHg) than the SLT (3.5 ± 1.9 mmHg) and trabeculectomy (4.3 ± 1.3 mmHg) groups (P < 0.001). Mean 24-hour OPP fluctuation was also significantly higher in the medical therapy group (18.5 ± 4.0 mmHg) than the SLT (11.9 ± 7.3 mmHg) and trabeculectomy (14.1 ± 3.9 mmHg) groups (P < 0.05). No difference in IOP or OPP fluctuation was found between SLT and trabeculectomy groups (P > 0.05).</p><p><strong>Conclusions: </strong>Both SLT and trabeculectomy may be more effective in reducing 24-hour IOP and OPP fluctuation than medical therapy alone. IOP and OPP fluctuation was comparable between SLT and trabeculectomy cohorts. Future studies are warranted to investigate this further.</p>","PeriodicalId":9606,"journal":{"name":"Canadian journal of ophthalmology. Journal canadien d'ophtalmologie","volume":" ","pages":""},"PeriodicalIF":3.3,"publicationDate":"2025-01-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142884983","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Mobin Abdi, Erica Darian-Smith, Gilad Litvin, David S Rootman, Clara C Chan
{"title":"Dual-organism fungal keratitis in a novel keratoprosthesis device.","authors":"Mobin Abdi, Erica Darian-Smith, Gilad Litvin, David S Rootman, Clara C Chan","doi":"10.1016/j.jcjo.2024.11.014","DOIUrl":"10.1016/j.jcjo.2024.11.014","url":null,"abstract":"","PeriodicalId":9606,"journal":{"name":"Canadian journal of ophthalmology. Journal canadien d'ophtalmologie","volume":" ","pages":""},"PeriodicalIF":3.3,"publicationDate":"2025-01-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142876247","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Prem A H Nichani, Alonso Gutierrez Guerinoni, David S Rootman, Clara C Chan
{"title":"Bilateral corneal perforation secondary to immunotherapy for metastatic melanoma.","authors":"Prem A H Nichani, Alonso Gutierrez Guerinoni, David S Rootman, Clara C Chan","doi":"10.1016/j.jcjo.2024.11.011","DOIUrl":"10.1016/j.jcjo.2024.11.011","url":null,"abstract":"","PeriodicalId":9606,"journal":{"name":"Canadian journal of ophthalmology. Journal canadien d'ophtalmologie","volume":" ","pages":""},"PeriodicalIF":3.3,"publicationDate":"2025-01-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142871584","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Oded Rock, Tiran Golani, Sari David, Mor Vered, Rasha Mosleh, Sharon Armarnik, Tamara Wygnanski-Jaffe
{"title":"Improvement of stereopsis following strabismus surgery in adults: a retrospective analysis.","authors":"Oded Rock, Tiran Golani, Sari David, Mor Vered, Rasha Mosleh, Sharon Armarnik, Tamara Wygnanski-Jaffe","doi":"10.1016/j.jcjo.2024.12.007","DOIUrl":"https://doi.org/10.1016/j.jcjo.2024.12.007","url":null,"abstract":"<p><strong>Objective: </strong>Adults who undergo strabismus surgery, in addition to cosmesis, could benefit from improved stereopsis. This improvement is associated with the performance of motor skill tasks in young adults; they reduce the risk of tripping or falling during everyday locomotion and improve reading efficiency. This study aimed to assess stereopsis level after strabismus surgery in adults who underwent strabismus surgery for any reason.</p><p><strong>Methods: </strong>Retrospective observational study of adult patients who underwent strabismus surgery at our institution between 2010 and 2022.</p><p><strong>Results: </strong>One hundred and thirty-two patients were included. Postoperatively, 48% had a stereo acuity better than 100 seconds of arc, and only 21.5% had no stereopsis. The most significant factor influencing improved stereopsis after surgery was the angle of strabismus postoperatively (p = 0.018). There was a statistically significant correlation between fusion at a distance and the stereo acuity (p = 0.0337). There was no statistically significant correlation between the stereo acuity level and the strabismus angle of deviation, the strabismus direction (vertical, horizontal, or combined), or the type (esotropia or exotropia) before or after the surgery. Although not statistically significant (p = 0.6579), the underlying cause categories were correlated with the stereo level. Patients with neurological causes demonstrated higher levels of stereopsis, followed by TED, whereas patients with childhood strabismus have the lowest probability of regaining stereopsis after surgery.</p><p><strong>Conclusions: </strong>Although long-standing strabismus reduces the potential for binocularity, surgery may be beneficial and improve stereopsis regardless of the cause of strabismus.</p>","PeriodicalId":9606,"journal":{"name":"Canadian journal of ophthalmology. Journal canadien d'ophtalmologie","volume":" ","pages":""},"PeriodicalIF":3.3,"publicationDate":"2025-01-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143000604","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Fatma F Shakarchi, Ahmed F Shakarchi, Mohamed K Soliman, Abdallah A Ellabban, Ahmed B Sallam
{"title":"Subsequent intraocular lens surgery in eyes with combined versus sequential phacovitrectomy.","authors":"Fatma F Shakarchi, Ahmed F Shakarchi, Mohamed K Soliman, Abdallah A Ellabban, Ahmed B Sallam","doi":"10.1016/j.jcjo.2024.11.012","DOIUrl":"10.1016/j.jcjo.2024.11.012","url":null,"abstract":"<p><strong>Objective: </strong>To determine the risk factors for subsequent intraocular lens (IOL) surgery among eyes undergoing either combined or sequential phacovitrectomy.</p><p><strong>Design: </strong>Retrospective cohort study.</p><p><strong>Participants: </strong>Adult patients undergoing phacoemulsification at 8 United Kingdom National Health Service clinical centers between July 2003 and March 2015. Patients undergoing surgery for a dropped nucleus fragment, glaucoma, or iris problems were excluded.</p><p><strong>Methods: </strong>The eyes were categorized into 3 groups: with previous vitrectomy, undergoing combined phacovitrectomy, and with both previous vitrectomy and current combined phacovitrectomy. These groups were compared based on preoperative ocular and systemic factors, intraoperative findings, and the subsequent need for a second IOL surgery. The Poisson model was used to calculate adjusted relative risks (aRR).</p><p><strong>Results: </strong>5,215 eyes were included: 2,124 with previous vitrectomy, 2,512 with combined phacovitrectomy, and 579 eyes with previous vitrectomy and currently combined phacovitrectomy. The risk of subsequent IOL surgery was 0.6% (reference group) in eyes with previous vitrectomy, 1.6% (aRR 2.6, CI: 1.4-5.1) in eyes with current combined phacovitrectomy, and 3.3% (aRR 3.8, CI: 1.7-8.3) in eyes with previous plus current combined phacovitrectomy. Other significant risk factors were age (aRR 0.98/year), pseudoexfoliation (aRR 6.76, CI: 2-28), zonular dialysis (aRR 10.6, CI: 4.8-24), scleral buckle in the current surgery (aRR 8.05, CI: 4-14), and the use of silicone oil (aRR 3.6, CI: 1.6-8.4) compared to no tamponade.</p><p><strong>Conclusions: </strong>Combined phacovitrectomy was associated with a higher risk of IOL complications than sequential surgery. This information is useful for planning surgery in patients requiring both cataract and retinal surgeries.</p>","PeriodicalId":9606,"journal":{"name":"Canadian journal of ophthalmology. Journal canadien d'ophtalmologie","volume":" ","pages":""},"PeriodicalIF":3.3,"publicationDate":"2025-01-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142892249","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Haowen Li, Jingting Luo, Zhaoxun Feng, David Maberley, Yang Li, Wenbin Wei, Yueming Liu
{"title":"Uveal metastasis: clinical characteristics, treatment, and prognostic factors in a cohort of 161 patients in China.","authors":"Haowen Li, Jingting Luo, Zhaoxun Feng, David Maberley, Yang Li, Wenbin Wei, Yueming Liu","doi":"10.1016/j.jcjo.2024.12.002","DOIUrl":"https://doi.org/10.1016/j.jcjo.2024.12.002","url":null,"abstract":"<p><strong>Objective: </strong>To investigate the clinical and prognostic features of uveal metastasis in a Chinese population and compare these features across different primary cancers.</p><p><strong>Design: </strong>Retrospective cross-sectional study.</p><p><strong>Participants: </strong>161 patients with uveal metastasis at Beijing Tongren Hospital.</p><p><strong>Methods: </strong>Clinical characteristics of the primary tumour and uveal metastasis, treatments and outcomes were reviewed. Tumor regression was assessed using B-scan ultrasonography to measure changes in tumor height.</p><p><strong>Results: </strong>Among 161 patients, 185 eyes had uveal metastasis. Lung cancer was the most common primary tumour (49.4%), followed by breast cancer (22.4%), kidney cancer (4.3%). Uveal metastasis was the initial cancer manifestation in 39 patients (24.2%). Unilateral involvement was predominant (86.3%), with the choroid being the most common site (91%). Compared with lung cancer and other cancers, breast carcinoma patients developed uveal metastasis at a younger age (p < 0.001), had a longer interval to uveal metastasis diagnosis (0.67, 4.19, and 6.27 years, p < 0.0001), were prone to be bilateral (11.1%, 9.1%, and 27.8%; p < 0.05) and exhibited lower tumour height (4.47 ± 2.49 mm and 4.88 ± 3.01 mm, 3.09 ± 2.46 mm; p < 0.01). Local treatments (133 eyes) included plaque radiotherapy (PRT, brachytherapy), transpupillary thermotherapy (TTT), anti-VEGF and enucleation. The tumour regression correlated with increased tumor height (r = 0.5699; p < 0.05) in eyes treated with PRT (n = 15). Despite effective local tumour control, the 1-year, 3-year, and 5-year survival rates were 72.61%, 31.82%, and 19.84%, respectively (n = 103). Among the 54 deceased patients, 27 (50%) died within one year following the diagnosis of uveal metastasis. The mean survival was 18.84 months. Extraocular metastasis correlated with shorter survival (p < 0.05).</p><p><strong>Conclusion: </strong>This study provides a comprehensive analysis of uveal metastasis in Chinese patients, highlighting the distinct characteristics from various primaries. Although survival rates remain uncertain, local therapies were effective at achieving ocular tumor control, which aligns with the expectations for patients battling metastatic cancers.</p>","PeriodicalId":9606,"journal":{"name":"Canadian journal of ophthalmology. Journal canadien d'ophtalmologie","volume":" ","pages":""},"PeriodicalIF":3.3,"publicationDate":"2025-01-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142982878","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}