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.

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.

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.

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.

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.

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.

COMBINED HAMARTOMA OF THE RETINA AND RETINAL PIGMENT EPITHELIUM AT PEDIATRIC AGE: Surgical Versus Conservative Approach.

PURPOSE: To report outcomes of pediatric patients with combined hamartoma of the retina and the retina pigment epithelium followed up conservatively or after pars plana vitrectomy. METHODS: This retrospective multicenter study included 62 eyes of 59 pediatric patients with combined hamartoma of the retina and the retina pigment epithelium from 13 different international centers with an average age of 7.7 ± 4.7 (0.3-17) years at the time of the diagnosis and having undergone pars plana vitrectomy or followed conservatively. At baseline and each visit, visual acuity values, optical coherence tomography for features and central foveal thickness, and tumor location were noted. Lesions were called as Zone 1, if it involves the macular and peripapillary areas, and the others were called as Zone 2 lesions. RESULTS: Twenty-one eyes of 20 patients in the intervention group and 41 eyes of 39 patients in the conservative group were followed for a mean of 36.2 ± 40.4 (6-182) months. Best-corrected visual acuity improved in 11 (68.8%) of 16 eyes in the intervention group and 4 (12.9%) of 31 eyes in the conservative group ( P < 0.001). The mean central foveal thickness decreased from 602.0 ± 164.9 µ m to 451.2 ± 184.3 µ m in the intervention group, while it increased from 709.5 ± 344.2 µ m to 791.0 ± 452.1 µ m in Zone 1 eyes of the conservative group. Posterior location of tumor, irregular configuration of the foveal contour and ellipsoid Zone defect in optical coherence tomography, subretinal exudate and prominent vascular tortuosity were associated with poor visual acuity. CONCLUSION: Vitreoretinal surgery is safe and effective in improving vision and reducing retinal distortion in Zone 1 combined hamartoma of the retina and the retina pigment epithelium in children.

Surgical outcomes of posterior persistent fetal vasculature syndrome: cases with tent-shaped and closed funnel-shaped retinal detachment.

BACKGROUND/OBJECTIVES: To determine the role of vitreoretinal surgery (VRS) for two different forms of posterior persistent fetal vasculature syndrome (PFVS); with tent-shaped tractional retinal detachment (TRD) and closed funnel-shaped TRD. SUBJECTS/METHODS: Retrospective, single surgeon, consecutive case series of 52 eyes of 44 patients with posterior PFVS who underwent VRS. Cases were divided into "tent-shaped TRD" and "funnel-shaped TRD" groups based on the preoperative TRD configuration. Associated anomalies, functional and anatomical outcomes were evaluated. The cosmetic appearance was defined as poor if there was phthisis bulbi, gross buphthalmos, or corneal opacification; acceptable if there was apparent leukocoria; and excellent if none were noted at the last follow-up. RESULTS: Thirty eyes of 29 patients presented with tent-shaped TRD; 70% of which obtained counting fingers or better vision and 90% showed significant reversal of tenting achieving retinal reattachment. The cosmetic appearance was excellent in 87%. Two eyes (7%) became phthisic. Twenty-two eyes of 15 patients presented with funnel-shaped TRD and leukocoria; 45% achieved LP vision and 70% of patients with bilateral pathology had LP in at least one eye. The cosmetic appearance was acceptable to excellent in 73%. Three eyes (14%) became phthisic, one (5%) of which required enucleation. The median follow-up time was 16 (6-71) months. CONCLUSIONS: VRS often provides functional vision and anatomy in posterior PFVS with tent-shaped TRD morphology. In the funnel-shaped TRD morphology, where no treatment has historically been recommended, surgery may be considered with an aim of restoring light perception and globe preservation, particularly in bilateral cases.

Bilateral acute Iris transillumination after COVID-19 pneumonia.

PURPOSE: To report the diagnosis, treatment and follow-up of a case of bilateral acute iris transillumination (BAIT) developed after COVID-19 pneumonia. METHOD: Case report. CASE: A 66-year-old male patient with COVID-19 pneumonia received systemic treatments of favipiravir, prednisolone, moxifloxacin and piperacillin-tazobactam during hospitalization. The patient applied to our clinic with the complaint of blurred vision 20 days after the diagnosis of COVID-19. The best corrected visual acuity (BCVA) was 0.3 in the right eye and 0.5 in the left eye. In the anterior segment examination; ciliary injection, intense pigment dispersion in the anterior chamber, pigment deposits on the lens and iris, 2-3 + cells in the anterior chamber, posterior synechia, and 360 degrees diffuse iris transillumination were observed in both eyes. The pupillary response to light was weak. Bilateral fundus examination were normal. In the anterior chamber sample; HSV, VZV, CMV and Toxoplasma PCR were negative. Bilateral acute iris transillumination (BAIT) diagnosed in the patient and topical 0.1% dexamethasone and topical 1% cyclopentolate were started. In the follow-up, visual acuity increased 1.0 in both eyes, there were no cells in the anterior chamber, and the pigment dispersion was still continuing despite a decrease. CONCLUSION: BAIT, which can usually be seen after upper respiratory tract infections, can also be seen after covid 19 pneumonia and be kept in mind as a possible eye involvement in patients with COVID-19 infection.

Expert recommendations for the management of diabetic macular edema with intravitreal dexamethasone implant: A Turkish Delphi study.

PURPOSE: To provide consensus on the clinical use of intravitreal dexamethasone (DEX) implant for the treatment of diabetic macular edema (DME) in Turkey. METHODS: A panel of 22 retina experts prepared 77 statements of recommendation, and 80 retinal specialists practicing in Turkey were chosen to vote either in support or against each one. A Delphi-based method was used through which the ophthalmologists were able to view all of the results anonymously after two rounds and modify their subsequent answers. The survey was conducted via a mini website, and statements without consensus were resent to the specialists with the latest vote results a week later. RESULTS: A total of 72 ophthalmologists answered the first and second round questions. After the first stage, consensus was achieved on 55 of the statements, leaving 22 without agreement. After the second stage, consensus was reached on 11 of the remaining statements. Strong consensus was achieved on statements regarding the etiopathogenesis of DME and the first-line indications and safety of the DEX implant procedure. The panel recommended the use of DEX implant for patients with an arterial thromboembolic event in the last three months and also agreed that pro re nata DEX implant treatment not only provides better outcomes for DME patients but also reduces the treatment burden for those who could not receive an adequate number of anti-vascular endothelial growth factor (VEGF) injections. CONCLUSION: This study provides clinical consensus and recommendations about the use of DEX implant in the clinical practice of DME management in Turkey.

Late sequelae of retinopathy of prematurity in adolescence and adulthood.

This article provides information about late complications of retinopathy of prematurity (ROP), especially seen in adolescence and adulthood. The majority of ROP patients recover without complications and treatment, but severe ROP cases should be properly treated. Both prematurity itself and the treatment of ROP cause some changes in the anterior (refractive changes, cataract, and glaucoma) and posterior segments (tractional, rhegmatogenous and exudative retinal detachment, vitreous hemorrhage, etc.,) of the eye and predispose to significant lifelong complications. Awareness of these late complications can minimize severe vision loss with proper follow-up and appropriate treatment. Therefore, life-long ophthalmological follow-up is mandatory in all prematures with the diagnosis of ROP.