Clinical and Demographic Characteristics of Treatment Requiring Retinopathy of Prematurity in Big Premature Infants in Turkiye: Report No. 1 (BIG-ROP Study).

INTRODUCTION: The aim of the study was to analyse the clinical and demographic features of infants with gestational age (GA) of 32-37 weeks and birth weight (BW) of >1,500 g who developed treatment requiring retinopathy of prematurity (ROP). METHODS: Data on the infants with a GA of 32-37 weeks and BW >1,500 g who developed treatment requiring ROP (TR-ROP) were collected retrospectively from the 33 ROP centres in Turkiye. GA, BW, type of hospital, neonatal intensive care units (NICUs) level, presence of an ophthalmologist and neonatologist in the same hospital, length of stay in NICU, duration of oxygen therapy, comorbidities, type of ROP, and timing for TR-ROP development were analysed. RESULTS: A total of366 infants were included in the study. Mean GA and BW were 33 ± 1 weeks and 1,896 ± 316 g, respectively. Duration of hospitalization was 3-4 weeks in 46.8% of them. The first ROP examination was performed at postnatal 4-5 weeks in 80.3% of infants, which was significantly later in level 2 and lower NICUs and non-university clinics. At the first ROP examination, any stage of ROP was detected in 90.9% and TR-ROP was detected in 15.3% of the infants. The mean postnatal week of TR-ROP development was 6.16 ± 2.04. CONCLUSION: Routine ROP screening thresholds need to be expanded in hospitals with suboptimal NICU conditions considering the development of TR-ROP in more mature and heavier preterm infants. The first ROP examination should be earlier than the fourth postnatal week.

Overlapping clinical features of persistent fetal vasculature and combined hamartoma of the retina and retinal pigment epithelium.

PURPOSE: To investigate cases exhibiting overlapping features of persistent fetal vasculature (PFV) and combined hamartoma of the retina and retinal pigment epithelium (CHRRPE) and to explore potential associations between these developmental ocular anomalies. METHODS: This retrospective, descriptive case series included 9 eyes of 8 patients aged 0-7 years with shared clinical features of PFV and CHRRPE. Diagnoses were established through clinical examination and intraoperative findings. RESULTS: All eyes exhibited elevated pigmented retinal thickening, increased vascular tortuosity, and preretinal fibrotic/gliotic changes or epiretinal membranes. Macular involvement was observed in 56% of cases; peripapillary involvement, in 44%. Four eyes showed hyaloid stalklike fibrotic remnants extending from the lesion to the posterior lens surface, suggestive of PFV component; the other 5 harbored isolated CHRRPE. One patient presented with PFV in one eye and CHRPPE in the other. CONCLUSIONS: This study reveals significant clinical overlap between PFV and CHRRPE, with some cases displaying features typically associated with the other condition. The presence of both diagnoses in the same patient further suggests a potential association between these entities. Further research, including molecular studies, is needed to explore this potential connection and deepen our understanding of ocular development.

Subfoveal neurosensory detachment flattening and observe (SNF-Ob) approach for the management of Ci-DMO - a multicentric study.

PURPOSE: To understand subfoveal neurosensory detachment flattening and observe (SNF-Ob) strategy and its relationship with visual acuity in the management of centre-involved diabetic macular oedema (Ci-DMO). METHODS: This was a multicentric retrospective observational study. We reviewed data of 188 eyes of 130 patients who presented with Ci-DMO with subfoveal neurosensory detachment (NSD) and treated with intravitreal anti-vascular endothelial growth factor (anti-VEGF) agents or steroids. The primary outcome was best corrected visual acuity (BCVA) measured at the time of the first subfoveal neurosensory detachment flattening (SNF) and at the end of follow-up. RESULTS: Eyes that achieved 20/50 (LogMAR = 0.40) or better at first SNF had mean LogMAR BCVA 0.38 ± 0.21, 0.24 ± 0.11 and 0.21 ± 0.15 at baseline, at the time of first SNF, and at the end of the last follow-up respectively. Mean LogMAR BCVA significantly improved from baseline to first SNF (p < 0.0001; 95% CI 0.115-0.183) and at the end of the last follow-up (p < 0.0001; 95% CI 0.126-0.213) with a change of Early Treatment Diabetic Retinopathy Study (ETDRS) 10 letters. There was no significant difference in improvement in BCVA from the first SNF and at the end of the last follow-up (p = 0.0781; 95% CI -0.002 to 0.046). CONCLUSIONS: Eyes presenting with Ci-DMO and subfoveal NSD are unlikely to improve at SNF with BCVA > 20/50 (LogMAR = 0.40). Further evidence is needed before the combination of good BCVA and SNF may be considered as endpoint of pharmacological therapy for DMO.

Surgical Outcomes of Rhegmatogenous Retinal Detachment Associated with Regressed Retinopathy of Prematurity.

Objectives: To evaluate the characteristics and surgical outcomes of late-onset rhegmatogenous retinal detachment (RRD) associated with regressed retinopathy of prematurity (ROP) and the status of fellow eyes. Materials and Methods: Retrospective review of consecutive cases undergoing surgery for regressed ROP-related RRD and the fellow eyes between 2012-2022. Demographic data, fundus findings, retinal detachment characteristics, surgical procedures, and anatomic and functional outcomes were analyzed. Anatomic success was defined as retinal attachment after silicone oil removal at final follow-up. Results: Fifteen eyes of 14 patients with a history of regressed ROP underwent surgical repair for RRD at a mean age of 12 (range, 3-26) years. Primary surgical intervention yielded a 53% failure rate overall. This rate was 33% for scleral buckling (SB), 100% for pars plana vitrectomy (PPV), and 40% for combined SB-PPV surgery. Eyes with posterior cicatricial changes and/or proliferative vitreoretinopathy (PVR) demonstrated a higher tendency for recurrence. The final anatomic success rate was 73% after a mean number of 2.3 (range, 1-5) surgeries. The chances of restoring useful vision diminished with repeated surgery despite the improvement in anatomic success. In the fellow eyes, peripheral retinal pathologies were universally observed, with posterior cicatricial changes noted in 33%. Conclusion: The study reveals a significant initial failure rate in surgical treatment of cases with late-onset RRD associated with regressed ROP, particularly in eyes with posterior cicatricial changes or PVR, suggesting the need for a combined surgical approach as an initial strategy in such high-risk cases. The consistent presence of retinal abnormalities in fellow eyes calls for proactive monitoring and potential prophylactic intervention.

Current clinical practice and needs assessment in inherited eye diseases from the perspective of ophthalmologists.

BACKGROUND: The clinical approach to inherited eye diseases has evolved due to advances in genetic testing methods and treatment opportunities. However, no data are available on the current practices of ophthalmologists in countries, such as Turkey, with higher rates of consanguinity and inherited eye diseases. The aim of this study was to evaluate the current practices, knowledge, and needs of ophthalmologists in Turkey regarding inherited eye diseases. METHODS: A 29-item self-administered survey with a branching algorithm was developed through Google Forms. The survey link was sent to 2983 ophthalmologists in Turkey. The survey assessed respondents' occupational characteristics, current practices, knowledge about available diagnostic and therapeutic options, and opinions on improving continuing education and healthcare services. RESULTS: Responses from 414 ophthalmologists (20.8%) were analyzed. The responses suggested that ophthalmologists mainly collaborate with medical geneticists in respect of inherited eye diseases. The majority of ophthalmologists reported a lack of knowledge about genetic diagnostic tests, and approximately 90% of the ophthalmologists thought training after residency was inadequate for inherited eye diseases. CONCLUSION: This is the most extensive survey exploring ophthalmologists' practice patterns and needs in a setting without specialists or specialized centers in ophthalmic genetics. The results emphasize the need for continued education on updated approaches to inherited eye diseases.

Reappearance of excentric persistent fetal vasculature stalk following laser treatment in a patient with type 1 retinopathy of prematurity.

PURPOSE: In this report, we aim to present an unusual reappearance of hyaloidal artery remnant with atypical localization during the follow-up of an infant who underwent indirect laser photocoagulation for type 1 ROP. METHODS: Retrospective case report. RESULTS: We report a case of reappearance of an eccentrically located hyaloidal stalk in the macular area during the follow-up period, 2 weeks after laser photocoagulation for type 1 ROP subsequently progressed to cause foveal distortion, which is successfully removed with a lens-sparing vitrectomy. CONCLUSIONS: To the best of our knowledge, there is no similar case in the literature. In the presence of fibrovascular proliferation extending into the vitreous, especially in premature infants, it should be kept in mind that this may be a reappearance of PFV and it may not always be located on the optic disc.

Persistent Fetal Vasculature: Current Insights and Future Directions.

BACKGROUND AND PURPOSE: Persistent fetal vasculature (PFV) is a complex congenital ocular condition, characterized by the incomplete regression of the embryonic hyaloid system. It encompasses a spectrum of abnormalities, affecting various ocular structures and presenting a range of fetal hyaloid remnants. Despite its long-standing recognition, the full extent of PFV's manifestations continues to evolve, unveiling novel findings, primarily driven by advancements in clinical experience and imaging techniques. This review focuses on the evolution of PFV management, emphasizing the disease heterogeneity and the consequent challenges in diagnosis and treatment. METHODS: We present a comprehensive guide on PFV based on the current evidence, detailing its recognition, associated anatomical variations, the intricacies of surgical indications and techniques, and postoperative expectations. CONCLUSION: The progress over the last decade in innovative instruments and surgical techniques has not only enhanced functional and anatomical outcomes but also enriched our understanding of PFV. However, continued exploration and research remain pivotal for future breakthroughs in more effectively understanding and managing this complex ocular anomaly.

Surgical Approaches to Serous Retinal Detachment With Retina-Lens Touch in Eyes With Nanophthalmos.

Purpose:To describe the visual outcomes and problems that resulted from surgical treatment of nanophthalmic complete retinal detachment (RD) with retina-lens contact. Methods: A multicenter retrospective case series with deep sclerectomy as a treatment was performed. Results: Five cases had extensive deep sclerectomies, 3 with intended drainage of subretinal fluid (SRF). The RD resolved 1 week postoperatively in 4 cases and within 1 month in 1 case. The visual acuity improved from light perception to a median of 20/100. Three cases had longstanding lens touch beyond 1 year and improved VA to 20/100, 20/150, and hand motions, respectively. Complications included focal lens dialysis in 2 cases (passive drainage of SRF) and lens or intraocular lens dislocation in 1 case each (active drainage of SRF). Ultrasound biomicroscopy and anterior optical coherence tomography showed a very narrow angle and short zonules. Conclusions: Deep sclerectomy results in good anatomic and functional improvements in advanced cases of nanophthalmos exudative detachment, which is often considered to be incurable.

Evaluation of Central and Peripheral Retinal Vascular Changes in the Fellow Eyes of Patients with Unilateral Retinal Vein Occlusions

Objectives: To evaluate the subtle peripheral retinal and macular vascular changes in the fellow eyes of patients with unilateral retinal vein occlusion (RVO). Materials and Methods: This retrospective study included 53 patients with unilateral RVO and 44 age-matched controls. The frequency of peripheral retinal vascular pathologies in both eyes was evaluated using high quality ultra-wide field fluorescein angiography (UWFFA). Macular vascular density, flow area, and foveal avascular zone measurements from optical coherence tomography angiography (OCTA) were analyzed together with laser flare photometry values in patients and controls. Results: Peripheral retinal vascular pathologies were detected on UWFFA in the fellow eyes of 36 (67.9%) patients. No significant central vascular pathologies were detected on OCTA and there was no significant difference in OCTA parameters between the fellow eyes and the controls. Flare values did not differ significantly between the control and the fellow eyes. Conclusion: Two thirds of the fellow eyes of unilateral RVO patients had subtle peripheral retinal vascular changes, while there was no significant microvascular change detected with OCTA in the macula. This suggests that vascular changes caused by systemic vascular disorders probably first start in the peripheral retina of the fellow eyes of patients with RVO.

Neurofibromatosis Type 1 Vasculopathy Presenting as Branch Retinal Vein Occlusion: Case Report and Review of the Literature

Systemic vascular occlusive disease associated with neurofibromatosis type 1 (NF1) has been reported in the aortic, cerebral, renal, celiac, and mesenteric vessels and is referred to as NF1 vasculopathy. Although retinal vascular involvement in patients with NF1 usually manifests as retinal capillary hemangiomatosis, a few cases of NF1 with retinal vascular occlusive disease have also been described. Here, we report a 2-year-old girl with NF1 who presented with branch retinal vein occlusion and peripheral retinal ischemia secondary to NF1. This case demonstrates that NF1-related retinal occlusive vasculopathy may occur in very young patients and that detailed fundus examination with fluorescein angiography is necessary in all patients with NF1.

Surgical Treatment of Bullous Exudative Retinal Detachment Secondary to Atypical Bilateral Central Serous Chorioretinopathy

This study aimed to report the diagnostic process, treatment, and follow-up of a patient with bullous exudative retinal detachment (RD) associated with an atypical variant of bilateral central serous chorioretinopathy (CSCR). A 28-year-old woman was referred to our clinic for total bullous RD in the right eye with a vision level of light perception only. She had been previously diagnosed with idiopathic uveal effusion syndrome and treated with systemic corticosteroid therapy with no response, and was referred to us for scleral window surgery. Four-quadrant scleral window surgery with external drainage of the subretinal fluid was performed, resulting in a transient partial attachment of the retina. RD started to progress again within 3 weeks, which prompted comprehensive imaging together with more advanced systemic workup for systemic lupus erythematosus and other rheumatological and immunological diseases. Systemic corticosteroid therapy was initiated during this period but did not stop the progression and was discontinued after a short time. Fluorescein angiography and indocyanine green angiography revealed multifocal choroidal leakage foci and large choroidal vessels without any intraocular inflammation findings and led to the diagnosis of atypical CSCR. Pars plana vitrectomy (PPV), internal drainage of the subretinal fluid, endolaser to the focal leakage areas, and intravitreal aflibercept injection were performed. Visual acuity increased to 0.8 within 8 months after the surgery with no recurrence. Bullous exudative RD is a very rare and atypical form of CSCR, and a favorable outcome can be obtained with PPV and surgical drainage of subretinal fluid followed by laser photocoagulation.

Peripheral and central retinal vascular changes in asymptomatic family members of patients with familial exudative vitreoretinopathy.

PURPOSE: To evaluate the peripheral vascular changes and effects of these on macular microvasculature in asymptomatic family members of familial exudative vitreoretinopathy (FEVR) patients. METHODS: This is a retrospective study including 61 eyes of asymptomatic family members of FEVR patients. Retinal abnormalities were assessed via ultra-widefield fluorescein angiography (UWF-FA) and optical coherence tomography angiography (OCTA). The eyes were grouped into 3: the first group comprised of eyes with normal findings on UWF-FA; the second group comprised of eyes with abnormal findings on UWF-FA but without any retinal ischemia; and the third group involved eyes with retinal ischemia or neovascularization. RESULTS: Best corrected visual acuity (BCVA) was 20/20 in all eyes. Forty eyes (65.6%) had abnormalities on UWF-FA. The most common feature was peripheral vascular looping, increased tortuosity, and anastomosis (63.9%). ODM/ODD ratio was higher in group 3 compared to groups 1 and 2. Deep foveal VD was lower in group 1 compared to groups 2 and 3. The mean FAZ area and perimeter were smaller in groups 2 and 3 compared to group 1. CONCLUSION: Even asymptomatic family members of FEVR patients may have significant peripheral retinal vascular abnormalities which may be associated with smaller optic disc, macular ectopia, and macular microvascular changes.

Strabismus in Retinopathy of Prematurity: Risk Factors and the Effect of Macular Ectopia.

Objectives: This study aimed to examine factors associated with strabismus in patients with retinopathy of prematurity (ROP) and the relationship between strabismus and macular ectopia. Materials and Methods: Patients with ROP were divided into three groups: Group 1, patients with spontaneous regression (n=45); Group 2, patients who received laser treatment (n=70); and Group 3, patients who underwent surgical treatment (n=91). Rates of anisometropia, amblyopia, nystagmus, macular ectopia, and retinal pathologies were evaluated and their impacts on strabismus development were determined. Disc-to-fovea distance (DFD) was measured from color fundus photographs and the correlation of macular ectopia with severity of strabismus was evaluated. Results: A total of 206 patients were included. Rates of anisometropia, amblyopia, nystagmus, macular ectopia, retinal pathologies causing vision loss, and strabismus were higher in Group 3 (p=0.0001) and correlated with higher stages of ROP (p=0.0001). Macular ectopia (p=0.005), retinal pathologies (p=0.005), and amblyopia (p=0.012) had the strongest impact on strabismus development in ROP patients. DFD and strabismus severity were not significantly correlated (p=0.364). Mean visual acuity (VA) was significantly higher in orthophoric patients compared to those with esotropia and exotropia (p=0.027). Esotropic patients had lower VA compared to patients with exotropia, but this finding was not statistically significant (p=0.729). Conclusion: Presence of macular ectopia, retinal pathologies, and amblyopia were the most strongly correlated risk factors for strabismus development in ROP patients. DFD was not associated with severity of strabismus. Exotropia was mostly related to higher DFD and a possible relationship between esotropia and lower VA was observed.

Retinotomies and retinectomies: A review of indications, techniques, results, and complications.

Retinotomy refers to "cutting" or "incising" the retina, whereas retinectomy denotes "excising" the retina. Retinotomies and retinectomies aid in tackling traction and retinal shortening that persist following membrane dissection and scleral buckling. We performed a literature search using Google Scholar and PubMed, followed by a review of the references procured. All relevant literature was studied in detail and summarized. We discuss the indications of retinotomies and retinectomies for relaxing retinal stiffness, accessing the subretinal space for choroidal neovascular membrane, hemorrhage and abscess clearance, drainage retinotomies to allow retinal flattening, radial retinotomies to release circumferential traction, harvesting free retinal grafts, and prophylactic chorioretinectomies in trauma.

Clinical Characteristics of Patients with Intraocular Lens Calcification after Pars Plana Vitrectomy.

AIM: To determine the clinical risk factors that may increase the occurrence of intraocular lens (IOL) calcification in patients who had undergone pars plana vitrectomy (PPV). METHODS: The medical records of 14 patients who underwent IOL explantation due to clinically significant IOL opacification after PPV were reviewed. The date of primary cataract surgery, technique and implanted IOL characteristics; the time, cause and technique of PPV; tamponade used; additional surgeries; the time of IOL calcification and explantation; and IOL explantation technique were investigated. RESULTS: PPV had been performed as a combined procedure with cataract surgery in eight eyes and solely in six pseudophakic eyes. The IOL material was hydrophilic in six eyes, hydrophilic with a hydrophobic surface in seven eyes and undetermined in one eye. The endotamponades used during primary PPV were C2F6 in eight eyes, C3F8 in one eye, air in two eyes and silicone oil in three eyes. Two of three eyes underwent subsequent silicone oil removal and gas tamponade exchange. Gas in the anterior chamber was detected in six eyes after PPV or silicone oil removal. The mean interval between PPV and IOL opacification was 20.5 ± 18.6 months. The mean BCVA in logMAR was 0.43 ± 0.42 after PPV, which significantly decreased to 0.67 ± 0.68 before IOL explantation for IOL opacification (p = 0.007) and increased to 0.48 ± 0.59 after the IOL exchange (p = 0.015). CONCLUSIONS: PPV with endotamponades in pseudophakic eyes, particularly gas, seems to increase the risk for secondary IOL calcification, especially in hydrophilic IOLs. IOL exchange seems to solve this problem when clinically significant vision loss occurs.

Severe anterior persistent fetal vasculature: the role of anterior retinal elongation on prognosis.

PURPOSE: This study aims to investigate surgical outcomes of eyes with severe anterior persistent fetal vasculature (PFV) and the role of associated anatomical anomalies on prognosis. METHODS: This is a retrospective, comparative case series of 32 eyes of 31 patients who underwent vitreoretinal surgery for severe anterior PFV, defined as fibrovascular tissue totally covering the back of cataractous lens. Based on the degree of anterior retinal elongations, cases were classified as follows: group 1, eyes with well-developed pars plana and minor/no abnormalities (n = 11, 34%); group 2, eyes with partially developed pars plana and broad-based elongations (n = 9, 28%); and group 3, eyes with no visible pars plana and fibrovascular membrane having 360° continuity with peripheral retina (n = 12, 38%). Complications and functional and anatomical outcomes were investigated. RESULTS: The median surgical age was 2 (1-12) months. The median follow-up was 26 (6-120) months. Seventy-three percent in group 1 achieved finger counting or better vision with a single surgery and without any pupillary/retinal complication. Groups 2 and 3 required 2.1 ± 0.9 and 2.6 ± 1.2 surgeries on average. Pupillary obliteration and RD occurred in 33% and 22% in group 2 and 58% and 67% in group 3. Retina remained attached after silicone oil removal in 89% of group 2 and 25% of group 3. Phthisis developed in 50% in group 3. CONCLUSION: Peripheral retinal anomalies are common in severe anterior PFV and have a major impact on prognosis. Prognosis is favorable in cases with mild-to-moderate anomalies with appropriate management of possible retinal tears. In eyes with 360° retinal elongations, severe fibrous proliferation and eventual eye loss are common.

Flap-Related Complications Following Temporal Inverted Internal Limiting Membrane Flap for Macular Hole Repair.

Here we report three cases of flap-related complications following temporal inverted internal limiting membrane (ILM) flap technique for the repair of macular holes (MH). The first case showed a flap closure pattern in which the MH completely closed at 2 months spontaneously. The second case showed early anatomical and functional improvement provided by an immediate closure of the MH but developed flap contracture and nasally located epiretinal membrane (ERM) at postoperative 18 months. There was no functional deterioration, thus no further intervention was required. In the third case, early postoperative flap dislocation was observed and an additional surgery to reposition the flap was needed. The flap closure pattern observed with inverted ILM flap techniques may represent the ongoing healing process of large MHs and may be related to delayed spontaneous anatomical closure. ILM flap contracture and ERM formation may be a harmless long-term complication. Dislocation of the ILM flap is an unexpected early postoperative complication that may necessitate a second surgery for flap repositioning.

Avascular Peripheral Retina in Infants.

Avascular peripheral retina in an infant is a common characteristic of numerous pediatric retinal vascular disorders and often presents a diagnostic challenge to the clinician. In this review, key features of each disease in the differential diagnosis, from retinopathy of prematurity, familial exudative vitreoretinopathy, Coats disease, incontinentia pigmenti, Norrie disease, and persistent fetal vasculature, to other rare hematologic conditions and telomere disorders, will be discussed by expert ophthalmologists in the field.

Biosimilars for retinal diseases: United States-Europe awareness survey (Bio-USER - survey).

PURPOSE: To assess the awareness of biosimilar intravitreal anti-VEGF agents among retina specialists practicing in the United States (US) and Europe. METHODS: A 16-question online survey was created in English and distributed between Dec 01, 2021 and Jan 31, 2022. A total of 112 respondents (retinal physicians) from the US and Europe participated. RESULTS: The majority of the physicians (56.3%) were familiar with anti-VEGF biosimilars. A significant number of physicians needed more information (18.75%) and real world data (25%) before switching to a biosimilar. About one half of the physicians were concerned about biosimilar safety (50%), efficacy (58.9 %), immunogenicity (50%), and their efficacy with extrapolated indications (67.8 %). Retinal physicians from the US were less inclined to shift from off-label bevacizumab to biosimilar ranibizumab or on-label bevacizumab (if approved) compared to physicians from Europe (p=0.0001). Furthermore, physicians from the US were more concerned about biosimilar safety (p=0.0371) and efficacy compared to Europe (p= 0.0078). CONCLUSIONS: The Bio-USER survey revealed that while the majority of retinal physicians need additional information regarding the safety, efficacy and immunogenicity when making clinical decisions regarding their use. Retinal physicians from US are more comfortable in continuing to use off-label bevacizumab compared to physicians from Europe.