British Journal of Ophthalmology最新文献

{"title":"Two-year outcome of phacogoniotomy for advanced primary angle-closure glaucoma with cataracts: a multicentre study","authors":"Yunhe Song, Lin Xie, Xiaomin Zhu, Sujie Fan, Aiguo Lv, Guangxian Tang, Hengli Zhang, Li Tang, Yao Zhang, Lan Lu, Meichun Xiao, Huiping Yuan, Wulian Song, Minwen Zhou, Xiaohuan Zhao, Zhixuan Wang, Fengbin Lin, Xiaohong Liang, Yuying Peng, Yu Chen, Zhenyu Wang, Ling Jin, Ki Ho Park, Keith Barton, Weirong Chen, Ningli Wang, Ying Han, Dennis Lam, Robert Weinreb, Tin Aung, Clement C Tham, Xiulan Zhang","doi":"10.1136/bjo-2024-325375","DOIUrl":"https://doi.org/10.1136/bjo-2024-325375","url":null,"abstract":"Purpose To report the 2-year effective and safety profile of integrated phacoemulsification with intraocular lens implantation (PEI), goniosynechialysis (GSL) and 120° goniotomy (GT) in advanced primary angle-closure glaucoma (PACG) and cataracts. Design Multicentre prospective study. Methods 201 eyes of 196 patients who received combined PEI+GSL+ GT were assessed at baseline and re-evaluated at days 1, 7, and at 1, 3, 6, 12 and 24 months postsurgery. The intraocular pressure (IOP), the number of topical hypotensive medications, surgical complications and achievements of surgery success were also evaluated. Results All participants were followed up for a 2-year period. The average IOP declined from 33.0±10.7 mm Hg to 13.6±2.9 mm Hg, and the number of topical hypotensive medication decreased from an average of 2.4 types to 0.4 at month 24. For the safety profile, the major complications were hyphema (n=14, 7.0%), IOP spike (n=9, 4.9%) and transit corneal oedema (n=23, 11.4%). The complete success was achieved in 72.6% of the eyes (146 out of 201), and the qualified success rate was 91.5% (184 out of 201). Only one eye required tube shunt surgery while no one developed severe vision-threatening complications. The best-corrected visual acuity increased from 0.80±1.08 to 0.54±0.78 LogMAR. Conclusions PEI+GSL+GT is shown to be effective and safe in treating advanced PACG with cataract over a 2-year follow-up period. The combined surgery may be considered as an alternative for patients with advanced PACG. Data are available on reasonable request.","PeriodicalId":9313,"journal":{"name":"British Journal of Ophthalmology","volume":"87 1 1","pages":""},"PeriodicalIF":4.1,"publicationDate":"2025-03-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143570428","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"At a glance","authors":"Frank Larkin","doi":"10.1136/bjo-2025-327321","DOIUrl":"https://doi.org/10.1136/bjo-2025-327321","url":null,"abstract":"The cornea layers are closely integrated, both structurally and in the expression of basement membrane and adhesion proteins, at the periphery, where they blend with the trabecular meshwork beams forming a transition zone. For correction of pre-surgery astigatism in cataract patients, toric intraocular lens implantation provides (i) less postoperative refractive cylinder and (ii) better uncorrected distance visual acuity than femtosecond laser-assisted astigmatic keratotomy. This is a further category of postoperative outcome in which use of femtosecond laser has not been found to be advantageous compared to alternative interventions. The authors report a non-linear relationship between pressure, maximum flow velocity and the extent of angle closure. Pressure started to elevate when the extent of angle closure was >180°, and increased dramatically when >270°. Two and three quadrants …","PeriodicalId":9313,"journal":{"name":"British Journal of Ophthalmology","volume":"4 1","pages":""},"PeriodicalIF":4.1,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143477470","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Cataract surgery in the extremely small eye: morphology, comorbidities and outcomes in 300 eyes","authors":"Maximilian Hammer, Lilly Teich, Maximilian Friedrich, Emanuel Reitemeyer, Leoni Britz, Ramin Khoramnia, Timur Mert Yildirim, Gerd U Auffarth","doi":"10.1136/bjo-2024-326998","DOIUrl":"https://doi.org/10.1136/bjo-2024-326998","url":null,"abstract":"Background/aims Data on morphological characteristics and outcomes of extremely short eyes undergoing cataract surgery is sparse. Thus, an in-depth characterisation of eyes implanted with a high-power intraocular lens (IOL) (>30 dioptres) was performed. Methods In this retrospective cohort study from January 2009 to October 2023, 300 eyes of 191 patients undergoing cataract surgery with extremely short axial length requiring the implantation of a high power IOL>30D were included. Eyes were categorised into the morphologies of nanophthalmos (N), relative anterior microphthalmos (RAM) and high or low/moderate hyperopia (HH and MH). Comorbidities, intraoperative and postoperative complications, preoperative and postoperative refraction and visual outcomes were investigated. Results Mean preoperative spherical equivalent (SE) was +6±2.85 D. The mean axial length was 20.68±0.92 mm. 19.3%, 45.3%, 22.7% and 12.7% of the studied eyes were categorised as MH, HH, N and RAM, respectively. Amblyopia (14.7%), previous strabismus surgery (7.3%), glaucoma (12.7%) and previous iridotomy (9.4%) were prevalent. Postoperative SE was −0.42±1.56 D. Preoperative Corrected Distance Visual Acuity (CDVA) and postoperative Uncorrected Distance Visual Acuity (UDVA) were not significantly different (0.34±0.39 Logarithm of the Minimum Angle of Resolution (logMAR) vs 0.47±0.38 logMAR, respectively, p=0.47), postoperative CDVA was slightly improved (0.28±0.31 logMAR, p=0.02). The narrow anterior chamber angle was significantly alleviated, posterior capsule rupture rates (3%) were within previously reported ranges. Conclusions Lens surgery is safe, improves the anterior chamber situation but is rather imprecise in extremely short eyes. Of all subtypes, nanophthalmic eyes showed compromised outcomes. Postoperative CDVA is only slightly improved to preoperative CDVA, while postoperative UDVA closely resembles preoperative CDVA. Surgery thus provides spectacle independence leading to good patient satisfaction. Data are available upon reasonable request. The datasets generated and analysed during the current study are not publicly available, but are available from the corresponding author upon reasonable request.","PeriodicalId":9313,"journal":{"name":"British Journal of Ophthalmology","volume":"49 1","pages":""},"PeriodicalIF":4.1,"publicationDate":"2025-02-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143517918","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Disorganisation of inner retinal layers in diabetic macular oedema: is it time to standardise this OCT biomarker?","authors":"Luca Danieli, Giulia Midena, Luisa Frizziero, Edoardo Midena","doi":"10.1136/bjo-2024-326843","DOIUrl":"https://doi.org/10.1136/bjo-2024-326843","url":null,"abstract":"Disorganisation of inner retinal layers (DRIL) is an optical coherence tomography (OCT) biomarker in patients with diabetic macular oedema. Different ways of quantification of DRIL have been proposed, without final agreement. We found a significant difference in DRIL length if measured with different techniques. Our data highlight that DRIL needs to be measured in a standardised way to provide comparable clinical results. Whereas an international consensus is needed, DRIL measurement as mean of five central OCT scans spaced 120 µm seems better than a single scan and comparable to seven scans, which needs a longer assessment time.","PeriodicalId":9313,"journal":{"name":"British Journal of Ophthalmology","volume":"69 1","pages":""},"PeriodicalIF":4.1,"publicationDate":"2025-02-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143517915","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Retinal detachment incidence in refractive lens exchange versus cataract surgery: uncommon versus rare – systematic review and meta-analysis","authors":"Maria Laura Passaro, Deniz Kilic, Gianni Virgili, Vito Romano, Ersilia Lucenteforte, Burkhard Dick, Suphi Taneri","doi":"10.1136/bjo-2024-326592","DOIUrl":"https://doi.org/10.1136/bjo-2024-326592","url":null,"abstract":"Aims Refractive lens exchange (RLE) is becoming a popular refractive solution for patients ineligible for corneal surgeries or phakic intraocular lenses. However, concerns regarding retinal detachment (RD) risk persist. Methods A systematic literature review and meta-analysis was performed in Medline and Scopus. The final search was updated on 30 March 2024. Results Of 2415 full-text screening reports, 40 studies met inclusion criteria, encompassing 8 592 380 eyes. This included 20 264 eyes that underwent RLE and 8 572 116 eyes that underwent cataract surgery. In the RLE surgery subgroup, the RD incidence for follow-up <12 months was 0.00016% (0.00000%; 0.00034%), for follow-up between 12 and 24 months was 0.0000% (0.0000%; 0.03102%), and for follow-up >24 months was 0.02312% (0.00984%; 0.03640%) (p<0.01). In the cataract surgery subgroup, the incidence for follow-up >24 months was 0.01244% (0.00655%; 0.01832%), and for missing follow-up was 0.00429% (0.00221%; 0.00637%). Conclusions We found that the incidence of RD after RLE was approximately double that observed after cataract surgery with at least 24 months (median 4 years) follow-up. Thus, RD may be classified as rare complication after cataract surgery (1/1000) and an uncommon complication after RLE (1/500). It must be noted that the heterogeneity of the subgroups after RLE was low. While some limitations, such as the lack of long-term follow-up in certain studies and incomplete reporting of factors like axial length or PVD status, may slightly influence the interpretation, these findings provide valuable insights. Acknowledging the inherent uncertainty in interpreting these results, additional studies are recommended. PROSPERO registration number CRD42023431420. Data are available on reasonable request.","PeriodicalId":9313,"journal":{"name":"British Journal of Ophthalmology","volume":"30 1","pages":""},"PeriodicalIF":4.1,"publicationDate":"2025-02-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143517916","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Peripapillary atrophy in patients with birdshot chorioretinitis","authors":"Thomas Ferreira de Moura, Aurélie Enjalbert, Dominique Monnet, Souhila Kecili, Linda Imikerene, Jordan Loeliger, Jennifer E Thorne, Antoine P Brézin","doi":"10.1136/bjo-2024-326440","DOIUrl":"https://doi.org/10.1136/bjo-2024-326440","url":null,"abstract":"Aims This study aims to analyse peripapillary atrophy (PPA), its frequency, extent and relation with measures of visual function in patients with birdshot chorioretinitis (BSCR). Methods Patients of the single-centre prospective COhort of patients with BIRDshot chorioretinitis (ClinicalTrials.gov Identifier: [NCT05153057][1]) were included. The areas of the optic nerve head (ONH) and PPA were measured on red–green and autofluorescence fundus images acquired by ultrawidefield retinal photography. The main outcome measure was the frequency of PPA. Correlations between the PPA/ONH ratio and disease duration, age, best corrected visual acuity (BCVA) and visual field results (mean deviation (MD) and the pattern SD (PSD)—Humphrey perimeter 30–2) were evaluated using the Spearman coefficient. Results A total of 752 eyes from 384 patients (mean age 62.4±12.1 years) were included. The median disease duration was 13.0 (7.0–19.0) years. PPA was observed in 717 (95.4%) eyes. The mean PPA/ONH ratio was 2.9±2.2 on red–green images, 3.1±2.2 on autofluorescence, and these measures were highly correlated (R=0.95, p<0.0001). The ratio of PPA/ONH increased with the duration of the disease (R=0.57, p<0.0001) from 1.6±0.6 in the 0–5 years group to 5.2±2.9 after 25 years and with age (R=0.51, p<0.0001). PPA was also associated with a worse BCVA (R=−0.42, p<0.0001) and worse visual field results: MD (R=−0.41, p<0.0001), PSD (R=0.42, p<0.0001). A strong intereye symmetry of the PPA/ONH ratio was observed (R=0.91, p<0.0001), which was greater than that of BCVA (R=0.58, p<0.0001), MD (R=0.76, p<0.0001) or PSD (R=0.70, p<0.0001). Conclusions Almost all patients with BSCR exhibit PPA and its extent increases over time. PPA is associated with poorer vision. All data relevant to the study are included in the article or uploaded as supplementary information. [1]: /lookup/external-ref?link_type=CLINTRIALGOV&access_num=NCT05153057&atom=%2Fbjophthalmol%2Fearly%2F2025%2F02%2F26%2Fbjo-2024-326440.atom","PeriodicalId":9313,"journal":{"name":"British Journal of Ophthalmology","volume":"28 1","pages":""},"PeriodicalIF":4.1,"publicationDate":"2025-02-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143506821","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Timing of nodal metastasis in patients with eyelid sebaceous carcinoma and implications for surveillance and survival","authors":"Tracy Lu, Casey Anthony, Xinyang Jiang, Jing Ning, Ryan Goepfert, Bita Esmaeli","doi":"10.1136/bjo-2024-326923","DOIUrl":"https://doi.org/10.1136/bjo-2024-326923","url":null,"abstract":"Background/aims Sebaceous carcinoma (SC) is a rare cancer with periocular predilection. Studies focused on patterns and timing of nodal metastasis are scarce. Our aim is to report patterns of regional nodal metastasis in patients with eyelid SC, including results of sentinel lymph node biopsy (SLNB). Methods All consecutive patients with eyelid SC treated during 1999–2023 were retrospectively reviewed. All subjects had standard of care surgery for eyelid SC and regional lymph node evaluation using physical examination and ultrasonography (US) at baseline and during the surveillance period. 38 patients with negative findings on US had SLNB to rule out microscopic metastasis. The frequency and timing of lymph node metastasis, the SLNB positivity rate, management of nodal metastasis, overall and disease-specific survival were analysed. Results A total of 138 patients were included. 30 patients (22%) had lymph node metastasis: 15 patients were diagnosed at the time of initial diagnosis of eyelid SC; 18 patients developed nodal metastasis during the follow-up period, at a median of 12 months (range, 1–132). Of 38 patients, 5 (13%) had a positive SLNB. Management of nodal metastasis entailed surgical resection of the involved nodal basins in 27 patients; 20 had adjuvant radiotherapy and 7 had adjuvant concurrent chemoradiation. In patients with nodal metastasis, the 2-year and 5-year disease-specific survival rates were 87.5% and 75.4%, respectively. Conclusions One-half of the patients with eyelid SC with nodal metastasis had negative findings at initial presentation but developed nodal metastasis during follow-up, highlighting the importance of continued lymph node surveillance. All data relevant to the study are included in the article or uploaded as supplementary information.","PeriodicalId":9313,"journal":{"name":"British Journal of Ophthalmology","volume":"17 1","pages":""},"PeriodicalIF":4.1,"publicationDate":"2025-02-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143495371","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Relationship of posterior peripheral corneal layers and the trabecular meshwork: an immunohistological and anatomical study.","authors":"Burçin Kepez Yildiz, Rui Freitas, Perla Filippini, Fatima Habroosh, Dalia G Said, Harminder Singh Dua, Prity Sahay","doi":"10.1136/bjo-2023-324844","DOIUrl":"10.1136/bjo-2023-324844","url":null,"abstract":"<p><strong>Background/aim: </strong>With the popularity of endothelial keratoplasty (EK) procedures, Descemet membrane (DM) EK and pre-Descemet EK, considerable work has been done on understanding the posterior corneal anatomy. Most of the information available relates to the central cornea. We evaluated the peripheral cornea to explore the immunohistological and anatomical relationship between the pre-Descemet layer (PDL), DM and trabecular meshwork (TM).</p><p><strong>Methods: </strong>Six donor human sclerocorneal discs were studied. PDL, DM and TM were examined by light microscopy, transmission electron microscopy (TEM) and immunohistology. The DM was peeled from the centre to the limit of its peripheral attachment, to reach the transition zone (TZ) between TM and peripheral cornea. Ten-micron sections were stained with antibodies against collagens 1, 2, 3, 4, 5, 6, 12, elastin, myocilin, wnt-1, aquaporin, tenascin C, laminin and integrin alpha 3.</p><p><strong>Results: </strong>Collagens 2, 3, 4, laminin and myocilin were predominantly seen in the TZ between TM and peripheral cornea. Wnt-1, integrin alpha 3 and tenascin C were highly concentrated in TM. Collagen 1 was present predominantly in the corneal stroma. On TEM; DM was thinner with a denser banded structure spread throughout its thickness in the periphery compared with the central cornea where it presents as the distinct anterior banded layer.</p><p><strong>Conclusion: </strong>The TZ between DM, PDL and TM shows a unique histological structure at the periphery. The collagen and elastin fibres of the TM are continuous with the PDL. The structures are firmly attached to each other. These findings provide structural information that is relevant to the preparation of DMEK donor tissue.</p>","PeriodicalId":9313,"journal":{"name":"British Journal of Ophthalmology","volume":" ","pages":"316-323"},"PeriodicalIF":3.7,"publicationDate":"2025-02-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139989366","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Prognostic significance of melanogenesis pathway and its association with the ultrastructural characterisation of melanosomes in uveal melanoma.","authors":"Jayanti Jha, Mithalesh Kumar Singh, Lata Singh, Neelam Pushker, Rachna Meel, Neiwete Lomi, Sameer Bakhshi, Tapas Chandra Nag, Kunzang Chosdol, Seema Sen, Seema Kashyap","doi":"10.1136/bjo-2023-323181","DOIUrl":"10.1136/bjo-2023-323181","url":null,"abstract":"<p><strong>Background: </strong>Pigmentation could be a relevant prognostic factor in uveal melanoma (UM) development. Microphthalmia-associated transcription factor (MITF) regulates melanin synthesis by activating tyrosinase-related protein 2 (TYRP2) and silver protein (SILV) that induce the melanogenesis pathway. Although their oncogenic potential has been observed in various malignancies but has not been investigated in UM Asian population. Our aim is to study the ultrastructure of melanosomes and the prognostic significance of pigmentation markers such as TYRP2, MITF and SILV in UM.</p><p><strong>Methods: </strong>Transmission electron microscopy was performed to compare the ultrastructure of melanosomes in the normal choroid and UM cases. Immunoexpression of TYRP2, SILV and MITF was analysed in 82 UM samples. The mRNA expression level of all genes was measured in 70 UM cases. A statistical correlation was performed to determine the prognostic significance of all markers.</p><p><strong>Results: </strong>Premelanosomes and mature melanosomes undergoing dedifferentiation were observed in high-pigmented UM cases as compared with low-pigmented UM cases. Seventy per cent of UM cases showed high SILV expression while TYRP2 and MITF expression was present in 58% and 56% of cases, respectively. At the mRNA level, upregulation of TYRP2, SILV and MITF markers was seen in around 50% of UM cases, which was statistically significant with high pigmentation. Reduced metastatic-free survival was statistically significant with the MITF protein expression.</p><p><strong>Conclusion: </strong>Our results demonstrated that ultrastructural changes in melanosomes and high expression of TYRP2, MITF and SILV could dysregulate the melanogenesis pathway and might be responsible for the aggressive behaviour of UM.</p>","PeriodicalId":9313,"journal":{"name":"British Journal of Ophthalmology","volume":" ","pages":"416-425"},"PeriodicalIF":3.7,"publicationDate":"2025-02-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41107876","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Long-term natural history of ellipsoid zone width in <i>USH2A</i>-retinopathy.","authors":"Michael Heyang, Joshua L Warren, Paulina Ocieczek, Jacque L Duncan, Mariya Moosajee, Lucian V Del Priore, Liangbo Linus Shen","doi":"10.1136/bjo-2024-325323","DOIUrl":"10.1136/bjo-2024-325323","url":null,"abstract":"<p><strong>Aims: </strong>To investigate the long-term natural history of ellipsoid zone (EZ) width in <i>USH2A</i>-retinopathy.</p><p><strong>Methods: </strong>EZ width measurements from optical coherence tomography were retrospectively obtained from 110 eyes of 55 participants with molecularly confirmed biallelic <i>USH2A</i>-retinopathy. We used a hierarchical Bayesian method to construct and compare different mathematical models describing the long-term decline of EZ width.</p><p><strong>Results: </strong>Compared with linear and quadratic models, exponential decline best represented the long-term loss of EZ width based on the deviance information criterion score. Log-transformed EZ width declined linearly over 30 years of inferred disease duration (median: 0.063 (IQR: 0.040-0.086) log (µm)/year). Compared with the raw EZ width decline rate, the log-transformed EZ width decline rate required 48% fewer patients to achieve an identically powered 1-year trial (38 vs 73 participants). Log EZ width decline rate was uncoupled from baseline EZ width (Spearman ρ=-0.18, p=0.06) and age (ρ=-0.10, p=0.31). Eyes with Usher syndrome exhibited earlier median onset ages of macular EZ width loss (18.8 (IQR: 13.1-24.7) vs 28.1 (IQR: 18.5-35.8) years, p<0.001) but comparable log EZ width decline rates (0.060 (IQR: 0.035-0.100) vs 0.065 (IQR: 0.050-0.079) log (µm)/year; p=0.42).</p><p><strong>Conclusions: </strong>EZ width follows an exponential decline in <i>USH2A</i>-retinopathy. Compared with raw EZ width decline rate, log-transformed EZ width decline rate may be a superior endpoint for clinical trials. Syndromic eyes exhibit an earlier onset of macular EZ width loss but progress at comparable rates to non-syndromic eyes.</p>","PeriodicalId":9313,"journal":{"name":"British Journal of Ophthalmology","volume":" ","pages":"383-390"},"PeriodicalIF":3.7,"publicationDate":"2025-02-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11866300/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141892912","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}