Novel Insights Into Gyrate Atrophy of the Choroid and Retina (GACR): A Cohort Study.

Gyrate atrophy of the choroid and retina (GACR, OMIM #258870) is a rare inherited metabolic disorder characterized by progressive chorioretinal degeneration and hyperornithinemia. Current therapeutic modalities potentially slow disease progression but are not successful in preventing blindness. To allow for trial development, increased knowledge of the clinical phenotype and current therapeutic outcomes is required.

Longitudinal changes of macular neurodegenerative and vascular abnormalities on OCTA in sickle cell disease.

Purpose: To evaluate the presence and progression of maculopathy in patients with sickle cell disease (SCD) using Optical Coherence Tomography (OCT) and OCT-Angiography (OCTA), and to identify clinical/laboratory risk factors for progression during follow-up.

Methods: Complete ophthalmic examination, including fundoscopy and macular SD-OCT/OCTA scans, was performed in consecutive SCD-patients (HbSS/HbSβ0/HbSβ+/HbSC genotype) during baseline and follow-up visits. SCR stage was based on fundoscopy instead of the Goldberg classification, since fluorescein angiography was not routinely used.

Perspectives and Update on the Global Shortage of Verteporfin (Visudyne).

An ongoing global shortage of verteporfin (Visudyne) limits the treatment possibilities for several chorioretinal diseases, including central serous chorioretinopathy, choroidal hemangioma, and polypoidal choroidal vasculopathy. Verteporfin is required to perform photodynamic therapy in these ocular diseases. Therefore, the current situation has a substantial impact on eye care worldwide.

Choroidal hyperpermeability patterns correlate with disease severity in central serous chorioretinopathy: CERTAIN study report 2.

Purpose: Choroidal vascular hyperpermeability (CVH) on indocyanine green angiography (ICGA) is a hallmark feature of central serous chorioretinopathy (CSC). We identified three distinct CVH phenotypes in CSC: uni-focal indistinct signs of choroidal hyperpermeability (uni-FISH) with one focal area of CVH, multiple areas of focal CVH (multi-FISH), and diffuse hyperpermeability covering most of the posterior pole (DISH). This report investigates the distribution of these phenotypes and their association with signs of disease chronicity.

Central serous chorioretinopathy: An evidence-based treatment guideline.

Central serous chorioretinopathy (CSC) is a relatively common disease that causes vision loss due to macular subretinal fluid leakage and it is often associated with reduced vision-related quality of life. In CSC, the leakage of subretinal fluid through defects in the retinal pigment epithelial layer's outer blood-retina barrier appears to occur secondary to choroidal abnormalities and dysfunction. The treatment of CSC is currently the subject of controversy, although recent data obtained from several large randomized controlled trials provide a wealth of new information that can be used to establish a treatment algorithm.

Efficacy of Carbonic Anhydrase Inhibitors on Cystoid Fluid Collections and Visual Acuity in Patients with X-Linked Retinoschisis.

Purpose: To date, there is no standard treatment regimen for carbonic anhydrase inhibitors (CAIs) in X-linked retinoschisis (XLRS) patients. This retrospective study aims to evaluate the efficacy of CAIs on visual acuity and cystoid fluid collections (CFC) in XRLS patients in Dutch and Belgian tertiary referral centers.

Design: Retrospective cohort study.

NOn-Pachychoroid PEripapillary Schisis (NOPPES) of the Retina: A New Phenotype and its Differential Diagnosis.

Purpose: The presence of peripapillary intraretinal fluid (IRF) has a broad differential diagnosis, including several types of neovascular and pachychoroid-related diseases. However, the clinician may encounter cases without signs of neovascular or pachychoroid disease, or any other previously described diagnosis. For these patients, we propose the term NOn-Pachychoroid PEripapillary Schisis (NOPPES) of the retina, and we discuss the differential diagnosis.

Choroidal Vascular Changes on Ultrawidefield Indocyanine Green Angiography in Central Serous Chorioretinopathy: CERTAIN Study Report 1.

Purpose: Choroidal venous overload was recently suggested to be a pathogenetic factor in central serous chorioretinopathy (CSC). Manifestations of venous overload on ultrawidefield indocyanine green angiography (UWF ICGA) include asymmetric arterial choroidal filling (AACF), enlarged choroidal vessels ("pachyvessels"), and asymmetric venous drainage (AVD) leading to choroidal intervortex venous anastomoses (CVAs) accompanied by choroidal vascular hyperpermeability (CVH). The purpose of the current study is to assess the presence of these signs of venous overload in a large cohort of CSC patients.

SINGLE-SESSION BILATERAL REDUCED-SETTINGS PHOTODYNAMIC THERAPY FOR BILATERAL CHRONIC CENTRAL SEROUS CHORIORETINOPATHY.

Purpose: We performed a multicenter, retrospective study on patients with bilateral chronic central serous chorioretinopathy (cCSC) who received single-session bilateral reduced-settings photodynamic therapy (ssbPDT) and assessed anatomical (resolution of subretinal fluid [SRF]) and functional (best-corrected visual acuity [BCVA]) outcomes and safety.

Methods: Patients who underwent ssbPDT between 01/01/2011 and 30/09/2022 were included. The resolution of SRF at first, second, and final follow-up was assessed on optical coherence tomography (OCT), and BCVA measurements were collected at these visits.

INCREASING EVIDENCE FOR THE SAFETY OF FOVEA-INVOLVING HALF-DOSE PHOTODYNAMIC THERAPY FOR CHRONIC CENTRAL SEROUS CHORIORETINOPATHY.

Purpose: A retrospective study was performed with data from the prospective randomized controlled trials, PLACE and SPECTRA, assessing the risk of foveal atrophy and the likelihood of structural and functional improvement on optical coherence tomography, after foveal half-dose photodynamic therapy in chronic central serous chorioretinopathy.

Methods: A total of 57 chronic central serous chorioretinopathy patients received a single half-dose photodynamic therapy with a treatment spot that included the fovea. Optical coherence tomography scans and fundus autofluorescence images were analyzed for structural improvement and possible atrophy development, at baseline and at several visits after treatment.

European Prevalence of Polypoidal Choroidal Vasculopathy: A Systematic Review, Meta-Analysis, and Forecasting Study.

The purpose of this study was to provide an estimate of the number of current and future patients with polypoidal choroidal vasculopathy (PCV) in Europe. We systematically searched 11 literature databases on 18 May 2022 for studies on the prevalence of PCV among a consecutive and representative group of patients with suspected neovascular age-related macular degeneration (AMD). Prevalence of PCV in patients with suspected neovascular AMD was summarized and included in a prevalence meta-analysis.

Crossover to Half-Dose Photodynamic Therapy or Eplerenone in Chronic Central Serous Chorioretinopathy Patients.

Purpose: To compare the efficacy and safety of crossover treatment to half-dose photodynamic therapy (PDT) and eplerenone treatment after the failure of primary treatment in patients with chronic central serous chorioretinopathy (cCSC).

Design: Multicenter crossover clinical trial.

Subjects: At 3 months after the baseline visit of the SPECTRA (Half-Dose Photodynamic Therapy Versus Eplerenone: Treatment Trial for Chronic Central Serous Chorioretinopathy) randomized controlled trial, either half-dose PDT or eplerenone treatment was evaluated for each patient, and patients who still demonstrated subretinal fluid (SRF) were included in the current study, the SPECS (Central Serous Chorioretinopathy Treated with Half-Dose PDT or Eplerenone Crossover Study) trial.

Clinical impact of the worldwide shortage of verteporfin (Visudyne®) on ophthalmic care.

Introduction: Since July 2021, a worldwide shortage of verteporfin (Visudyne®) occurred: an essential medicine required for photodynamic therapy (PDT). PDT with verteporfin has a broad range of indications in ophthalmology, including chronic central serous chorioretinopathy, polypoidal choroidal vasculopathy and choroidal haemangioma. For these disorders, PDT is either the first-choice treatment or regarded as a major treatment option.

Half-Dose Photodynamic Therapy Versus Eplerenone in Chronic Central Serous Chorioretinopathy (SPECTRA): A Randomized Controlled Trial.

Purpose: To compare the efficacy and safety between half-dose photodynamic therapy (PDT) and eplerenone therapy for treating chronic central serous chorioretinopathy (cCSC).

Design: This was a multicenter, open-label, randomized controlled trial.

Methods: This investigator-initiated trial was conducted in 3 academic medical centers in the Netherlands.

Incidence of redetachment 6 months after scleral buckling surgery.

Purpose: The preoperative and intraoperative clinical variables associated with redetachment and/or a poor visual outcome following scleral buckling (SB) surgery for rhegmatogenous retinal detachment (RRD) have mainly been studied after a short follow-up. This study aimed to analyse long-term effects by following patients for at least 6 months.

Methods: In a retrospective survey we evaluated the data of 436 eyes that underwent SB surgery.

Two autocrine pathways to regulate cyclic GMP synthesis in cultured human retinal pigment epithelial cells.

Aim: To investigate the role of two separate enzymatic pathways [soluble (sGC) vs. particulate (pGC) guanylyl cyclase] in the synthesis of cyclic GMP (cGMP) in cultured human retinal pigment epithelial (RPE) cells.

Methods: cGMP accumulation was evaluated by quantitative analysis of cGMP immunoreactivity.

Cyclic GMP in the pig vitreous and retina after experimental retinal detachment.

Purpose: Earlier studies have revealed a decreased level of cGMP in vitreous fluid obtained from patients with a retinal detachment. To further investigate this phenomenon, we developed an experimental retinal detachment model in pigs.

Methods: Experimental unilateral retinal detachments were induced in pig eyes by subretinal injection of 0.

Balance of vascular endothelial growth factor and pigment epithelial growth factor prior to development of proliferative vitreoretinopathy.

Background: Pigment epithelium-derived factor (PEDF) and vascular endothelial growth factor (VEGF) are imbalanced in eyes with proliferative diabetic retinopathy or proliferative vitreoretinopathy (PVR). It is not known whether such an imbalance is already present in early PVR stages. We therefore analyzed VEGF and PEDF concentrations in subretinal fluids prior to PVR development.

Scleral buckling surgery after macula-off retinal detachment: worse visual outcome after more than 6 days.

Purpose: To determine the effect of duration of macular detachment (DMD) on visual acuity (VA) in patients with macula-off rhegmatogenous retinal detachment (RD).

Design: Retrospective observational case series.

Participants: Two hundred two consecutive patients (202 eyes) with primary uncomplicated macula-off RD, preoperative VA of 10/100 or worse, a precise history of when macular function was lost, successful reattachment surgery, and a minimal follow-up of 3 months.