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.

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.

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.

Efficacy and Safety of Gonioscopy-Assisted Transluminal Trabeculotomy for Primary Congenital Glaucoma.

PRCIS: Gonioscopy-assisted transluminal trabeculotomy (GATT) provided effective intraocular pressure (IOP) control in primary congenital glaucoma (PCG). Also, approximately two third of patients did not need antiglaucoma medication at an average follow-up of 1 year after surgery. PURPOSE: The purpose of this study was to assess the safety and efficacy of GATT surgery in eyes with PCG. MATERIALS AND METHODS: This study is a retrospective review of patients who underwent GATT surgery for PCG. Outcome measures were changes in IOP and number of medications at all time points (1, 3, 6, 9, 12, 18, 24, and 36 mo after surgery), and success rates. Success was defined as IOP<21 mm Hg with at least a 30% reduction from the baseline, complete if without medications, or qualified if with or without medications. Cumulative success probabilities were analyzed using the Kaplan-Meier survival analyses. RESULTS: Twenty-two eyes of 14 patients diagnosed with PCG were enrolled in this study. The mean IOP reduction was 13.1 mm Hg (57.7%) with a mean decrease of 2 glaucoma medications at the final follow-up. All mean IOP readings during postoperative follow-up were significantly lower than baseline ( P <0.05 for all). Cumulative probability of qualified success was 95.5% and the cumulative probability of complete success was 66.7%. CONCLUSION: GATT was safe and successfully lowered IOP in patients with PCG with the advantage of avoiding conjunctival and scleral incisions.

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.

Outcomes of Prolene Gonioscopy Assisted Transluminal Trabeculotomy in Primary Open Angle Glaucoma and Pseudoexfoliation Glaucoma: A Comparative Study.

PRCIS: Gonioscopy-assisted transluminal trabeculotomy (GATT) provides greater intraocular pressure (IOP) reduction in pseudoexfoliative glaucoma (PXG) than in primary open angle glaucoma (POAG) in the first year of surgery; however, the difference between groups equalizes in the long term. PURPOSE: To compare outcomes of GATT in eyes with POAG and PXG. METHODS: Single-center, retrospective, comparative case-series. A total of 202 eyes (91 eyes of POAG; 111 eyes of PXG) were included. GATT was performed as a standalone procedure or in combination with cataract extraction. Outcome measures were change in IOP and number of medications at all time points (1, 3, 6, 9, 12, 18, 24, and 36 mo after surgery), success rate (IOP reduction ≥20% from baseline or IOP between 6 and 21 mmHg, without further glaucoma surgery), and complication rate. Cumulative success probabilities were compared using Kaplan-Meier survival analyses. RESULTS: The mean IOP decreased by 8.8 mmHg (34.4%) in the POAG group with a mean decrease of 2 glaucoma medications at final visit. In the PXG group, the mean IOP decreased by 12.8 mm Hg (44.6%) on 2.3 fewer medications. Mean IOP reduction was significantly higher in PXG than POAG at all time points up to 2-year visit ( P <0.05 for all), after which the difference was not significant. Cumulative success probability during the first year was significantly higher in PXG (97.6%) than in POAG (86.8%) ( P =0.01); no significant difference was found at 2-year ( P =0.07) and 3-year visits ( P =0.24). CONCLUSION: GATT was safe and effectively reduced the IOP and medication burden in patients with POAG and PXG. In the first year after GATT, a significantly higher success rate was noted in PXG compared with POAG; however, in subsequent years, the success rate was similar at ~75%.

Pseudo-hyaloidal Stalk in Anterior Persistent Fetal Vasculature: A Report of Two Cases.

Persistent fetal vasculature (PFV) syndrome is characterized by abnormal regression of the fetal hyaloid system and may occur in various forms. In this report, two atypical cases associated with posterior capsular defect and ectopic lens material located along Cloquet's canal are discussed. Ultrasonography of these patients presenting with bilateral total cataracts revealed a hyaloidal stalk extending from the optic nerve head to the retrolental area. During lensectomy, it was observed that lens particles were moving anteriorly from the central mid-vitreous to the aspiration port and that the posterior capsule was developmentally defective. There was no pathological vascular remnant, rather the lens material partially filled Cloquet's canal through the opening in the posterior capsule and created a pseudo-stalk appearance on the preoperative ultrasonography. We aim to discuss possible mechanisms underlying these cases, which may help to improve our understanding of the PFV spectrum.

Optical coherence tomography angiography markers associated with treatment response in neovascular age-related macular degeneration.

PURPOSE: To investigate whether qualitative and quantitative features of choroidal neovascular membranes are associated with anti-vascular endothelial growth factor treatment response in patients with neovascular age-related macular degeneration. METHODS: A total of 41 eyes were included in this cross-sectional observational study and divided into "good responders" and "poor responders" based on the long-term functional changes, frequency of recurrent choroidal neovascular activity, and injection need. Enface optical coherence tomography angiography images were obtained and qualitative features of choroidal neovascular membranes were described based on vessel network density. Then, quantitative measurements including vessel area, vessel length, junction density, and lacunarity were calculated using validated software (ImageJ and AngioTool). Chi-square tests, t-test, or Mann-Whitney U tests were used for group comparisons, and intraclass correlation coefficients were calculated for reliability assessment of quantitative analyses. RESULTS: Twenty-two eyes treated by a median of 9 (3-24) anti-vascular endothelial growth factor injections at a median follow-up of 41 (12-89) months were categorized as good responders, 19 treated by a median of 26 (11-46) injections at a median follow-up of 44.5 (12-84) months as poor responders. Good responder group mostly had loose network (45.5%), and poor responders mostly had dense network (47.7%). The difference was not statistically significant (p = 0.25). The size of the lesion was 2.7 times greater at baseline (p = 0.04) and 1.7 times greater at final examination in the poor responder group (p = 0.04). Lacunarity index, showing lesion heterogeneity, was higher in good responders (p = 0.018) than poor responders. Other quantitative vascular features did not significantly differ between the groups (p > 0.05). CONCLUSION: Long-term remodeling of chronically treated choroidal neovascular may be non-invasively and reproducibly investigated using optical coherence tomography angiography. Quantitative analysis and lacunarity index, in particular, may be used as a measure of vessel maturation and guide treatment strategies in neovascular age-related macular degeneration.

Relation of anatomy with function following the surgical treatment of idiopathic epiretinal membrane: a multicenter retrospective study.

PURPOSE: To investigate the prognostic factors associated with functional and anatomical outcomes and to assess the longitudinal course of visual acuity and retinal morphology after vitreoretinal surgery for idiopathic epiretinal membrane (ERM). METHODS: This multicenter, retrospective study included a total of 634 eyes who underwent surgery for idiopathic ERM in 22 academic centers nationwide in Turkey. Data on best-corrected visual acuity (BCVA) and optical coherence tomography features (central foveal thickness (CFT), ERM and foveal contour morphology, ellipsoid zone (EZ) integrity) were collected and compared at baseline, 6-month, 12-month, and 24-month follow-ups. Prognostic factors for functional (having ≥ 20/25 Snellen BCVA) and anatomical (having normal/shallow foveal contour) recoveries after surgery were investigated by means of multivariate regression analyses. A cutoff value of preoperative BCVA optimizing functional recovery was calculated using receiver operating characteristic curve analysis. RESULTS: At a median follow-up of 24 months, 37.4% of the eyes achieved ≥ 20/25 BCVA and 54% regained normal or shallow foveal contour. Functional recovery was more likely in eyes with better baseline BCVA and intact EZ (R2 = 0.356, p < 0.001). The cutoff baseline BCVA value for good visual prognosis was 0.35 logarithm of the minimum angle of resolution (Snellen 20/44) (sensitivity 60%, specificity 85%, p < 0.001). Anatomical recovery was negatively associated with advanced age, higher baseline CFT, foveal herniation-type ERM morphology, and internal limiting membrane (ILM) peeling (R2 = 0.225, p < 0.001). The negative effect of ILM peeling on anatomical recovery was not significant after the first postoperative year (p = 0.05). Mean BCVA values and foveal morphology progressively improved at each visit. Cases with convex baseline foveal contour continued to change towards normal foveal depression over 24 months of follow-up, which took longer than the eyes with shallow/flat contoured cases. One-third of eyes with severe baseline EZ defects showed recovery at follow-up and achieved significantly greater visual acuity gains than the remaining eyes with persistent defects (p < 0.001). CONCLUSIONS: Functional and anatomical restoration of the eyes appears to be a slow process after ERM surgery. This process may take much longer in eyes with worse foveal morphology at baseline. Although photoreceptor disruption may be reversible in some eyes, full functional recovery is unlikely when it persists.

Anterior elongation of the retina in persistent fetal vasculature: emphasis on retinal complications.

PURPOSE: To identify the characteristics of peripheral retinal anomalies associated with persistent fetal vasculature (PFV) and evaluate the complications and outcomes. METHODS: Retrospective, noncomparative case series of patients with PFV who underwent surgery. Type of PFV, presence of peripheral retinal anomalies, extent of the retrolental membrane, complications, and functional and anatomical results were evaluated. RESULTS: Twenty-nine eyes of 28 patients were enrolled in the study: 14 eyes (48.3%) with anterior PFV, 10 eyes (34.5%) with posterior PFV, and 5 eyes (17.2%) with combined PFV. The retina was found to elongate anteriorly as finger-like projections beyond the ora serrata, incorporating into retrolental fibrovascular tissue in 81.8% of the anterior PFV cases. Cases with more extensive retrolental tissue had a higher risk of retinal complications (p = 0.009) and anterior segment complications (p = 0.026) than those with localized disease. Leaving the peripheral part of the fibrovascular tissue in place led to complications by later contraction. A total of 35.7% of the anterior PFV cases had 20/200 or better vision at the final follow-up versus 6.7% of the cases with posterior involvement. Retinal attachment was achieved in 12 eyes (80%) with posterior involvement. Four eyes (13.7%) resulted in total retinal detachment at final follow-up. CONCLUSIONS: The high incidence of peripheral retinal anomalies that are found in anterior PFV patients and their relation to retinal complications warrant careful examination of the ora serrata-ciliary body area. Limbal approach may be preferred as a safer method in cases with an extensive fibrovascular membrane obscuring the view. Leaving the peripheral part of the fibrovascular tissue may result in severe postoperative complications, which should be avoided.