Ophthalmology. Retina最新文献

Objective: To refine retinal peripherin-2 (PRPH2)-associated retinal degeneration (PARD) phenotypes using multimodal imaging.

Design: Retrospective review of clinical records and multimodal imaging.

Subjects: Patients who visited the inherited retinal degeneration (IRD) clinic at 2 tertiary referral eye centers with molecularly confirmed IRD due to PRPH2 variants.

Methods: Retinal imaging was reviewed using ultrawidefield (UWF) pseudocolor, UWF fundus autofluorescence, and spectral-domain OCT. Phenotypes were identified in the macular or peripheral region. A combined phenotype was considered if any phenotypes were present in both macular and peripheral regions. Mixed phenotypes in the macula or peripheral retina were considered if there were 2 distinct phenotypes identified in the same eye. The presence or absence of atrophy in the macular or peripheral area was also noted.

Main outcome measure: Grading of multimodal imaging by phenotype and atrophy.

Results: A total of 144 eyes of 72 patients were included in this study. The majority of the eyes had combined macular and peripheral phenotypes (89/144, 61.8%), whereas 44 (30.6%) eyes had isolated macular findings, and 11 (7.6%) had isolated peripheral findings. Twenty-five eyes were classified with mixed macular phenotypes, whereas fundus flavimaculatus dystrophy type was the most common combined macular and peripheral phenotype (54/144, 37.5%): n = 10 with macular dystrophy and macular flavimaculatus dystrophy (MFD), and n = 15 with butterfly pattern dystrophy and MFD. Nearly half of the eyes (71/144, 49.3%) were identified to have concomitant outer retinal atrophy. Fundus flavimaculatus type dystrophy was also associated with the highest proportion of concomitant atrophy (57/71, 80.3%).

Conclusions: Peripherin-2-associated retinal degeneration demonstrates a wide array of phenotypes using multimodal imaging. We report that combinations of classically described phenotypes were often seen. Additionally, macular and peripheral atrophy were often associated with PARD phenotypes. Refinement of PARD phenotypes using newer multimodal imaging techniques will likely assist diagnosis and future clinical trials.

Financial disclosure(s): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.

Objective: To describe multimodal imaging of peculiar bilateral globular subretinal deposits and acquired serous retinal detachment in patients with systemic immunoglobulin light chain deposition.

Design: A retrospective observational case series.

Participants: We examined 6 eyes in 3 patients (1 with multiple myeloma, 1 with membranous nephropathy, and 1 with immunoglobulin A nephropathy) at the Eye and ENT Hospital of Fudan University. The patients presented with peculiar globular subretinal deposits along the retinal pigment epithelium (RPE)‒Bruch's membrane complex and acquired serous retinal detachment.

Methods: Fundus appearance was documented with multimodal imaging, which included fundus photography, fundus autofluorescence, spectral domain OCT, swept-source OCT (SS-OCT), en face OCT, and SS-OCT angiography. Additional evaluations included serum protein electrophoreses, positron emission tomography computed tomography, and renal and bone biopsies to assess the primary diseases.

Main outcome measures: Multimodal imaging, course, and prognosis of bilateral RPE immunoglobulin light chain deposition in patients with systemic immunoglobulin light chain deposition.

Results: Bilateral, multiple, speckled, or patchy RPE changes in the posterior fundus that corresponded to striking multifocal hyperautofluorescence on fundus autofluorescence and lumpy, globular hyperreflective deposits along the RPE‒Bruch's membrane complex were identified as characteristic features of bilateral RPE light chain deposition. These features may be accompanied by dense light chain deposits in the choriocapillaris and choroid vessels, diffuse choroidal thickening, and "angiographically silent" serous retinal detachment in patients with systemic immunoglobulin light chain deposition.

Conclusions: We have documented the characteristic features, clinical course, and prognosis of bilateral RPE immunoglobulin light chain deposition in patients with systemic immunoglobulin light chain deposition. Appropriate evaluations, including serum protein electrophoresis and hematologic consultation, are recommended to manage patients with this fundus abnormality.

Financial disclosure(s): The authors have no proprietary or commercial interest in any materials discussed in this article.

Objective: This study investigated the outcomes of premature infants diagnosed with retinopathy of prematurity (ROP) that were treated with intravitreal bevacizumab (IVB), as well as the need for further treatment after injection.

Design: Retrospective case series.

Participants: Seventy-three premature infants born between 2016 and 2020 at a large county hospital and children's hospital.

Methods: Chart review was performed and patient demographics, neonatal intensive care unit (NICU) course, ROP exams, and treatment were collected.

Main outcome measures: Rates of recurrent ROP disease, complete vascularization, persistent avascular retina (PAR), as well as rates of secondary and tertiary laser photocoagulation, IVB, or pars plana vitrectomy.

Results: Infants included in this study were born at a median gestational age (GA) of 24.6 weeks (range, 23.0-30.1) and a median birth weight of 670 g (range, 370-1080). Patients received their IVB injection at a median postmenstrual age (PMA) of 36.4 weeks (range, 16.0-87.9). Five patients died during their NICU course and did not have long-term follow-up. Of the remaining patients, 24 (33%) experienced complete vascularization after 1 injection; 13 (18%) experienced regression followed by disease recurrence necessitating additional interventions; 5 (7%) had persistent disease and did not experience any regression requiring laser treatment or a second IVB injection; and 26 (36%) experienced regression with PAR. Patients with persistent or recurrent ROP had a significantly lower GA than other patients in the study (P < 0.05).

Conclusions: For one-third of premature patients, 1 IVB injection was sufficient for ROP regression and complete vascularization of the retina. The remaining patients required some form of additional intervention after their injection, with a majority receiving laser for PAR. One-fifth of patients experienced disease recurrence up to 58 weeks PMA. Future studies should be performed on PAR prevalence and presentations to explore how long patients with PAR should be monitored.

Financial disclosure(s): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.

Objective: To evaluate the impact of total duration of intraretinal fluid (IRF) exposure on visual acuity and vision-related quality of life in patients with neovascular age-related macular degeneration (nAMD).

Design: A post hoc analysis of integrated data from the VIEW 1 and VIEW 2 trials.

Participants: Patients with nAMD.

Methods: Patients receiving intravitreal ranibizumab 0.5 mg every 4 weeks (Rq4) or intravitreal aflibercept injection (IAI) 2 mg every 4 weeks (2q4) or 2 mg every 8 weeks (2q8) were evaluated. Data were pooled across treatment groups, and the duration of IRF exposure was assessed by quartiles (first quartile [Q1]: ≤2 weeks; second quartile [Q2]: 3 to ≤8 weeks; third quartile [Q3]: 9 to ≤18 weeks; fourth quartile [Q4]: >18 weeks). Changes from baseline in visual acuity and vision-related quality of life were evaluated by quartiles of duration of IRF exposure in the pooled treatment group and each treatment group.

Main outcome measures: Mean changes from baseline in best-corrected visual acuity (BCVA) and improvement in vision-related quality of life as assessed via National Eye Institute Visual Function Questionnaire-25 (VFQ-25) composite and subscale scores at week 52.

Results: A total of 1804 patients were evaluated. In the pooled analysis, mean BCVA gains from baseline at week 52 for Q1, Q2, Q3, and Q4 were +10.9, +10.1, +9.4, and +6.6 letters, respectively (Least squares mean difference Q4-Q1 [95% confidence interval]; -4.3 [-6.1, -2.5]). Mean changes from baseline to week 52 in VFQ-25 composite score for Q1, Q2, Q3, and Q4 were +5.9, +6.6, +6.1, and +4.0 points, respectively (-1.9 [-3.6, -0.2]). A similar trend was observed across VFQ-25 subscales, with patients in Q4 generally experiencing less improvement versus Q1. When BCVA gains were assessed by quartiles of duration of IRF exposure within each treatment group, mean BCVA gains for Q1, Q2, Q3, and Q4 were +11.0, +11.1, +9.3, and +5.8 letters for Rq4 (nominal P < 0.05; Q4 vs. Q1); +10.7, +9.7, +9.2, and +7.7 letters for IAI 2q4; and +11.3, +11.2, +8.6, and +6.3 letters for IAI 2q8 (nominal P < 0.05; Q4 vs. Q1), respectively.

Conclusions: Increasing duration of IRF exposure was associated with lower visual gains and less improvement in vision-related quality of life in patients with nAMD.

Financial disclosure(s): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.