Posterior Polar Annular Choroidal Dystrophy: Genetic Insights and Differential Diagnosis in Inherited Retinal Diseases.

Posterior polar annular choroidal dystrophy (PPACD) is a rare ocular disorder and presents as symmetric degeneration of the retinal pigment epithelium (RPE) and the underlying choriocapillaris, encircling the retinal vascular arcades and optic disc. This condition distinctively preserves the foveal region, optic disc, and the outermost regions of the retina. Despite its distinct clinical presentation, due to the infrequency of its occurrence and the limited number of reported cases, the pathophysiology, and the genetic foundations of PPACD are still largely uncharted. This review aims to bridge this knowledge gap by investigating potential genetic contributors to PPACD, assessing current findings, and identifying genes that warrant further study. Emphasis is also placed on the crucial role of multimodal imaging in diagnosing PPACD, highlighting its importance in understanding disease pathophysiology. By analyzing existing case reports and drawing comparisons with similar retinal disorders, this paper endeavors to delineate the possible genetic correlations in PPACD, providing a foundation for future genetic research and the development of targeted diagnostic strategies.

Ocular endothelial dysfunction in pediatric inflammatory bowel disease.

OBJECTIVES: To assess ocular microvasculature changes using optical coherence tomography angiography (OCTA) in pediatric patients with inflammatory bowel disease (IBD). METHODS: Patients (aged 6-18 years) with IBD were recruited between September 2021 and May 2023. All eligible participants underwent comprehensive clinical assessment and laboratory investigation. Patients with functional gastrointestinal disorders served as the controls. This study assessed specific IBD phenotypes, disease duration, clinical and endoscopic activity indices, laboratory markers, and medication histories. OCTA was utilized to evaluate ocular microvasculature changes in both groups. RESULTS: A total of 63 children (mean age 12.9 ± 3.3 years) were enrolled, comprising 38 in the IBD group (16 ulcerative colitis, 22 Crohn's disease, and 25 in the control group). Most patients in the IBD group were in remission or had mild-to-moderate disease activity at enrollment. Analysis of the OCTA results revealed significant differences in the choroidal luminal area and total choroidal area between the IBD and control groups. CONCLUSIONS: The study identified distinct ocular microvasculature changes in pediatric IBD patients through OCTA, suggestive of potential systemic endothelial dysfunction. These findings underscore the utility of OCTA in evaluating microvascular alterations associated with pediatric IBD, offering insights into potential systemic complications linked to inflammation in IBD patients.

Parkinson's Disease: What Can Retinal Imaging Tell Us?

Parkinson's disease (PD) is a neurodegenerative disorder characterized by motor symptoms such as tremors, rigidity, and bradykinesia. While the diagnosis of PD primarily relies on clinical assessments and neurological examination, there has been growing interest in exploring non-invasive imaging techniques to aid in early detection and monitoring of the disease. In recent years, retinal imaging has emerged as a promising tool for studying PD due to the close anatomical and functional similarities between the retina and the brain. Retinal imaging methods, such as spectral domain optical coherence tomography and optical coherence tomography angiography, enable non-intrusive visualization and measurement of retinal structures and blood vessels. These techniques hold the promise of capturing alterations in retinal structure and function that could potentially mirror the underlying pathological mechanisms in PD. This review article aims to provide an overview of the current understanding of retinal changes in PD and the potential utility of retinal imaging as a diagnostic and monitoring tool.

Exploring Current Molecular Targets in the Treatment of Neovascular Age-Related Macular Degeneration toward the Perspective of Long-Term Agents.

Long-lasting anti-vascular endothelial growth factor (anti-VEGF) agents have become an option to reduce treatment frequency, with ongoing research exploring optimal responses and safety profiles. This review delves into molecular targets, pharmacological aspects, and strategies for achieving effective and enduring disease control in neovascular age-related macular degeneration (AMD). The molecular pathways involved in macular neovascularization, including angiogenesis and arteriogenesis, are explored. VEGF, PlGF, Ang-1, and Ang-2 play crucial roles in regulating angiogenesis, influencing vessel growth, maturation, and stability. The complex interplay of these factors, along with growth factors like TGFß and bFGF, contributes to the pathogenesis of neovascular membranes. Current anti-VEGF therapies, including bevacizumab, ranibizumab, aflibercept, brolucizumab, and faricimab, are discussed with a focus on their pharmacokinetics and clinical applications. Strategies to achieve sustained disease control in AMD involve smaller molecules, increased drug dosages, and novel formulations. This narrative review provides a comprehensive overview of the molecular targets and pharmacological aspects of neovascular AMD treatment.

An update on ophthalmological perspectives in oculodermal melanocytosis (Nevus of Ota).

PURPOSE: To provide a review of the literature on oculodermal melanocytosis (ODM) with a focus on the diagnostic and therapeutic implications of multimodal imaging techniques in the management of ophthalmic complications. METHODS: The authors carried out a literature search on PubMed, Medline, and Scopus of English language articles published on ODM through August 2021. This review presents traditional and novel diagnostic methods in the diagnosis and follow-up of patients with particular emphasis on addressing the role of imaging in the management of the ophthalmic complications of the condition towards improving current practice patterns. RESULTS: ODM is a rare, prevalently unilateral, congenital condition that presents with brown or blue/gray flat asymptomatic lesions of the skin, mucosae, episclera/sclera, and uvea localized within the territory of distribution of the ophthalmic and mandibular branches of the trigeminal nerve. Glaucoma and predisposition to uveal melanoma are the main ophthalmic complications. Diagnosis and management are through comprehensive opthalmological examination and traditional imaging methods such as ultrasonography and fluorescein/indocyanine green angiography as pigmentation of the fundus can conceal subtle retinal and choroidal alterations. Anterior segment optical coherence tomography and ultrasound biomicroscopy are used to evaluate the anterior segment and the ciliary body in the presence of glaucoma or melanoma of the anterior uveal tract. Fundus autofluorescence and retinal pigment epithelium (RPE) alterations are of aid in the differential diagnosis between choroidal nevi and melanoma. Enhanced depth imaging spectral domain optical coherence tomography offers outstanding in vivo evaluation of the dimensions and details of tumors or nevi and surrounding choroidal tissues and small choroidal melanomas may show distortions of the retinal and sub-retinal profile, presence of intra and sub-retinal fluid, abnormalities of the RPE, and compression of the choriocapillaris. CONCLUSIONS: Novel multimodal imaging techniques are significant in the diagnosis and management of the ophthalmic complications of ODM. Fundus autofluorescence and enhanced depth spectral domain optical coherence tomography have adjunctive value in the detection of early-stage melanoma and differential diagnosis between nevi and melanoma. Awareness of current and emerging imaging techniques can propagate improved standardized definition and assessment of the complications of ODM.

Oxidative stress and optical coherence tomography angiography evaluation of choriocapillaris and retinal vessel density in children with obstructive sleep apnea.

OBJECTIVES: To investigate the vascular networks of the retina and choroid using optical coherence tomography angiography (OCTA) to identify early biomarkers of obstructive sleep apnea (OSA) severity and to evaluate correlations with blood levels of oxidative stress. STUDY DESIGN: Patients with OSA were diagnosed based on video-polysomnography (PSG) and blood samples were collected to evaluate oxidative stress markers: total antioxidant status (TAS), biological antioxidant potential (BAP) test, Diacron reactive oxygen metabolites (d-ROMs) test. The eyes of children with OSA were evaluated and compared with eyes of healthy age-matched children. OCTA imaging was carried out to evaluate the choroidal and retinal vascular network density indices. RESULTS: A total of 31 children with OSA were recruited and compared with 10 healthy children. Choriocapillaris flow area decreased (p = 0.006) and superficial capillary plexus vessel density increased (p=0.01) with increasing severity of OSA. Children with OSA showed significant differences in TAS and d-ROMs test when compared to normal pediatric values (p<0.05). In calculating the correlations between PSG, oxidative stress, and OCTA variables, there was a negative correlation between choriocapillaris flow area and apnea hypopnea index (AHI) (p = 0.02, r2 -0.5) and between choriocapillaris flow area and the d-ROMs test (p 0.03; r2 0.5). CONCLUSIONS: The severity of OSA was associated with the choroidal and retinal capillary vascular networks. The correlation of the choriocapillaris flow area with AHI and the d-ROMs test indicates the connection of the choroidal microvasculature with the number of obstructive apnea and hypopnea events and oxidative stress.

Retinal and Choriocapillaris Vascular Changes in Early Alzheimer Disease Patients Using Optical Coherence Tomography Angiography.

BACKGROUND: Alzheimer disease (AD) is a neurodegenerative disorder characterized by ß-amyloid accumulation in the brain. A simple and reliable biomarker for AD that is not invasive is urgently needed, particularly in the preclinical and early stages of the disease. The retina shares with the brain, the same embryologic origins and it is affected by similar vascular changes. The aim of this study was to analyze the characteristics of the retinal and choriocapillaris vascular structure through optical coherence tomography-angiography (OCTA) evaluation in patients with early AD. METHODS: Eighteen patients with early AD (study group) and 18 healthy age-matched subjects (control group) were enrolled in the study. All patients underwent full neurologic and ophthalmologic examination, and OCTA scans. RESULTS: We found a significant reduction in flow area of choriocapillaris in the study group compared with the control group (P-value: 0.006), suggesting an impairment of choriocapillaris circulation in patients with early AD. CONCLUSION: OCTA provides accumulative evidence on the microvasculature changes of the retina and choriocapillaris in patients with AD. Further studies and improved OCTA software are necessary to better evaluate the role of vascular changes shown with OCTA as potential biomarkers in early disease.

Choroidal Neovascular Membranes in Retinal and Choroidal Tumors: Origins, Mechanisms, and Effects.

Choroidal neovascularizations are historically associated with exudative macular degeneration, nonetheless, they have been observed in nevus, melanoma, osteoma, and hemangioma involving the choroid and retina. This review aimed to elucidate the possible origins of neovascular membranes by examining in vivo and in vitro models compared to real clinical cases. Among the several potential mechanisms examined, particular attention was paid to histologic alterations and molecular cascades. Physical or biochemical resistance to vascular invasion from the choroid offered by Bruch's membrane, the role of fibroblast growth factor 2 and vascular endothelial growth factor, resident or recruited stem-like/progenitor cells, and other angiogenic promoters were taken into account. Even if the exact mechanisms are still partially obscure, experimental models are progressively enhancing our understanding of neovascularization etiology. Choroidal neovascularization (CNV) over melanoma, osteoma, and other tumors is not rare and is not contraindicative of malignancy as previously believed. In addition, CNV may represent a late complication of either benign or malignant choroidal tumors, stressing the importance of a long follow-up.

The Role of Diabetic Choroidopathy in the Pathogenesis and Progression of Diabetic Retinopathy.

Diabetic choroidopathy was first described on histopathological specimens of diabetic eyes. This alteration was characterized by the accumulation of PAS-positive material within the intracapillary stroma. Inflammation and polymorphonuclear neutrophils (PMNs) activation are crucial elements in choriocapillaris impairment. The evidence of diabetic choroidopathy in vivo was confirmed with multimodal imaging, which provides key quantitative and qualitative features to characterize the choroidal involvement. The choroid can be virtually affected in each vascular layer, from Haller's layer to the choriocapillaris. However, the damage on the outer retina and photoreceptor cells is essentially driven by a choriocapillaris deficiency, which can be assessed through optical coherence tomography angiography (OCTA). The identification of characteristic features of diabetic choroidopathy can be significant for understanding the potential pathogenic and prognostic implications in diabetic retinopathy.

The Role of Alpha-Synuclein Deposits in Parkinson's Disease: A Focus on the Human Retina.

Parkinson's disease (PD) is a neurodegenerative condition characterized by the progressive deterioration of dopaminergic neurons in the central and peripheral autonomous system and the intraneuronal cytoplasmic accumulation of misfolded α-synuclein. The clinical features are the classic triad of tremor, rigidity, and bradykinesia and a set of non-motor symptoms, including visual deficits. The latter seems to arise years before the onset of motor symptoms and reflects the course of brain disease. The retina, by virtue of its similarity to brain tissue, is an excellent site for the analysis of the known histopathological changes of PD that occur in the brain. Numerous studies conducted on animal and human models of PD have shown the presence of α-synuclein in retinal tissue. Spectral-domain optical coherence tomography (SD-OCT) could be a technique that enables the study of these retinal alterations in vivo. The objective of this review is to describe recent evidence on the accumulation of native or modified α-synuclein in the human retina of patients with PD and its effects on the retinal tissue evaluated through SD-OCT.

The Role of the Choroid in Stargardt Disease.

Stargardt disease is the commonest juvenile macular dystrophy. It is caused by genetic mutations in the ABCA4 gene. Diagnosis is not always straightforward, and various phenocopies exist. Late-onset disease can be misdiagnosed with age-related macular disease. A correct diagnosis is particularly critical because of emergent gene therapies. Stargardt disease is known to affect retinal pigment epithelium and photoreceptors. Many studies have also highlighted the importance of the choroid in the diagnosis, pathophysiology, and progression of the disease. The choroid is in an integral relationship with the retinal pigment epithelium and photoreceptors, and its possible involvement during the disease should be considered. The purpose of this review is to analyze the current diagnostic tools for choroidal evaluation and the extrapolation of useful data for ophthalmologists and researchers studying the disease.

Choroidal Vasculature Changes in Age-Related Macular Degeneration: From a Molecular to a Clinical Perspective.

The contribution of choroidal vasculature to the pathogenesis of age-related macular degeneration (AMD) has been long debated. The present narrative review aims to discuss the primary molecular and choroidal structural changes occurring with aging and AMD with a brief overview of the principal multimodal imaging modalities and techniques that enable the optimal in vivo visualization of choroidal modifications. The molecular aspects that target the choroid in AMD mainly involve human leukocyte antigen (HLA) expression, complement dysregulation, leukocyte interaction at Bruch's membrane, and mast cell infiltration of the choroid. A mechanistic link between high-risk genetic loci for AMD and mast cell recruitment has also been recently demonstrated. Recent advances in multimodal imaging allow more detailed visualization of choroidal structure, identifying alterations that may expand our comprehension of aging and AMD development.

An update on the ophthalmic features in hereditary haemorrhagic telangiectasia (Rendu-Osler-Weber syndrome).

Hereditary haemorrhagic telangiectasia (HHT) or Osler-Rendu-Weber syndrome is a rare autosomal dominant disease, characterised by systemic angiodysplasia. Dysfunction of the signalling pathway of ß transforming growth factor is the main cause of HHT principally owing to mutations of the genes encoding for endoglin (ENG) and activin A receptor type II-like 1 (ACVRL1). Clinical manifestations can range from mucocutaneous telangiectasia to organ arterio-venous malformations and recurrent epistaxis. The early clinical manifestations may sometimes be subtle, and diagnosis may be delayed. The main ophthalmic manifestations historically reported in HHT are haemorrhagic epiphora, and conjunctival telangiectasia present in 45-65% of cases, however, imaging with wide-field fluorescein angiography has recently shown peripheral retinal telangiectasia in 83% of patients. Optimal management of HHT requires both understanding of the clinical presentations and detection of early signs of disease. Advances in imaging methods in ophthalmology such as wide-field fluorescein angiography, spectral domain optical coherence tomography, and near infrared reflectance promise further insight into the ophthalmic signs of HHT towards improved diagnosis and early management of possible severe complications.

Overview of the retina and imaging in patients with severe acute respiratory syndrome coronavirus 2.

INTRODUCTION: The role of the human eye in severe acute respiratory syndrome coronavirus 2 (SARS-COV-2) is still under investigation. The pathophysiology of the ocular findings is arduous when dealing with critically ill Covid-19 patients with comorbidities. Multiorgan involvement and the effects of inflammation, infection and systemic treatment on the retina are complex, and comparison of studies is difficult. Most studies in human patients have investigated the anterior segment, whereas few reports deal with the posterior segment of the eye. The present review aims to evaluate the retinal manifestations and imaging features in COVID-19 patients. METHODS: Studies on the retinal manifestations and retinal imaging in COVID-19 patients published through June 2021 were reviewed. We included cross-sectional and case-control studies, case series, case reports and correspondence in the analysis. RESULTS: Flame-shaped hemorrhages, cotton wool spots, augmented diameter and tortuosity of retinal vessels were found on funduscopic examination. Peripapillary, macular retinal nerve fiber layer and ganglion cell layer thickness alterations were reported on spectral domain optical coherence tomography. Reduced vessel density of the superficial and deep retinal capillary plexus on optical coherence tomography angiography was reported. CONCLUSIONS: Retinal complications may arise in COVID-19 patients. Although no consensus on presentation is currently available, retinal funduscopy and imaging has shown neuronal and vascular alterations. Systemic neurological complications and microangiopathy are associated with SARS-COV-2; thus, as the retina has a neuronal and vascular component, funduscopy and retinal imaging on COVID-19 patients can provide further insight to SARS-COV-2 disease and the follow-up of patients.

Neurocognitive Assessment and Retinal Thickness Alterations in Alzheimer Disease: Is There a Correlation?

BACKGROUND: The relation of retinal thickness to neuropsychological indexes of cognitive impairment in patients with Alzheimer disease (AD) remains an area of investigation. The scope of this investigation was to compare volume and thickness changes of neuronal retinal layers in subjects with AD with those of age-matched healthy controls and to estimate the relation between cognitive functioning evaluated by neuropsychological assessment and thickness changes of the retina. METHODS: This was a prospective single-site study where we evaluated 25 subjects with probable AD matched for age, sex, and education to 17 healthy control subjects (HC). All participants underwent a full medical evaluation, neuropsychological assessment, and optical coherence tomography (OCT) to evaluate the peripapillary retinal nerve fiber layer (pRNFL) thickness, ganglion cell complex (GCC) thickness, and macular volume. RESULTS: The pRNFL thickness of AD patients showed a significant overall reduction compared with healthy controls (P = <0.0001). Furthermore, pRNFL was reduced in each retinal quadrant, particularly the inferior, nasal, and superior quadrants. GCC thickness and macular volume were reduced in AD patients in comparison with HC (P = 0.004; P = 0.001). Of particular interest was the correlation between OCT findings and neuropsychological assessment; we did not find a significant association of retinal thinning with worse MMSE score, but reduction of macular volume was associated with worse constructional praxis performance. Impairment of semantic-lexical and processing speed was associated with attenuation of macular GCC thickness. CONCLUSIONS: OCT can show early thickness changes in AD patients with subtle memory disturbances. These results suggest that correlations between retinal thinning and cognitive performance warrant further investigation.

Deep topical anesthesia with ropivacaine-soaked sponge for phacoemulsification.

PURPOSE: To assess safety and efficacy of deep topical anesthesia with ropivacaine-soaked sponge compared with topical anesthesia with oxybuprocaine in patients undergoing phacoemulsification. METHODS: This was a retrospective study where records of patients operated for cataract were evaluated. Patients using a visual analogue scale scored pain during surgery, and the surgeon on a questionnaire recorded ease of operation. Medical records were evaluated for patients who received topical anesthesia with multiple administrations of oxybuprocaine 0.4% or those who received deep topical anesthesia with a polyvinyl acetal sponge impregnated with ropivacaine 0.75% and positioned under the eyelid 30 min before surgery. RESULTS: A total of one hundred patient records, equally divided in patients receiving deep topical anesthesia or topical anesthesia, were included. The visual analogue scale scores among the groups were statistically significant for a lower pain score in patients who received deep topical anesthesia with ropivacaine-soaked sponges (p = 0.0069). The average surgeon score was significantly higher for the deep topical anesthesia group indicating favorable ease of surgery (p = 0.0341). Six patients had major complications during surgery. No additional anesthesia was necessary to manage the complications in four patients in the deep anesthesia group, whereas propofol was used for the induction and maintenance of anesthesia in two patients in the topical anesthesia group. CONCLUSIONS: Deep topical anesthesia with ropivacaine-soaked sponges performed as well as topical oxybuprocaine regarding safety and efficacy. It provided a lower patient pain score, favorable surgeon satisfaction, and long-lasting anesthesia.

OPTICAL COHERENCE TOMOGRAPHY EVIDENCE ON THE CORRELATION OF CHOROIDAL THICKNESS AND AGE WITH VASCULARIZED RETINAL LAYERS IN NORMAL EYES.

PURPOSE: To correlate choroidal thickness (CT) and age with vascularized retinal layer and outer retinal layer thickness in normal eyes. METHODS: This was a prospective, cross-sectional study. Complete ophthalmological examination, biometry, and enhanced depth imaging spectral domain optical coherence tomography were performed. Choroidal and individual retinal layer thickness measurements were obtained. Thickness maps for all layers were evaluated using the 1 mm, 3 mm, and 6 mm early treatment diabetic retinopathy study (ETDRS) macular grid areas. RESULTS: One hundred and twenty eyes were included. Choroidal thickness correlated negatively with age in all ETDRS areas. The ganglion cell layer (GCL) in the 1 mm; the GCL and inner plexiform layer (IPL) in the 3 mm and 6 mm; and the GCL, IPL, and inner nuclear layer in the 6 mm areas correlated negatively with age and positively with CT. Retinal nerve fiber layer thickness in the 6 mm area correlated negatively with age. The retinal pigment epithelium-photoreceptor layer in all areas correlated negatively with age and positively with CT. CONCLUSION: In normal subjects, vascularized retinal layer thicknesses and outer retinal layer thickness correlate positively with CT and negatively with age. The role of neuronal versus vascular components should be considered when evaluating individual retinal layer thicknesses.

SPECTRAL DOMAIN OPTICAL COHERENCE TOMOGRAPHY EVIDENCE OF RETINAL NERVE FIBER LAYER AND GANGLION CELL LOSS IN ADULT PATIENTS WITH NEUROFIBROMATOSIS TYPE 1.

PURPOSE: To evaluate peripapillary retinal nerve fiber layer, macular retinal nerve fiber layer, and ganglion cell layer-inner plexiform layer thickness and analyze their correlations in adult patients with neurofibromatosis Type 1 (NF1) and disease-free controls. METHODS: This cross-sectional study was performed at the Azienda Policlinico Umberto I, University of Rome "La Sapienza." All participants underwent complete ophthalmologic examination. Spectral domain optical coherence tomography was used to evaluate peripapillary retinal nerve fiber layer and obtain retinal segmentation measurements to assess macular retinal nerve fiber layer and ganglion cell layer-inner plexiform layer at 1,000 µm nasal, temporal, superior, and inferior to the fovea. RESULTS: Thirty-four eyes of 17 patients with NF1 (mean age, 42.2 ± 14.3 years) and 34 eyes of 17 disease-free control subjects (mean age, 41.4 ± 12.2 years) were included. All participants had best-corrected visual acuity of 20/20. The mean thickness of peripapillary retinal nerve fiber layer, macular retinal nerve fiber layer, and ganglion cell layer-inner plexiform layer was lower in patients with NF1 with respect to controls (P = 0.003, P = 0.022, P < 0.001, respectively). Regression analysis showed a significant correlation (P < 0.001) between mean ganglion cell layer-inner plexiform layer thickness and mean peripapillary retinal nerve fiber layer thickness in patients with NF1. CONCLUSION: Retinal nerve fiber layer and ganglion cell loss correlate well with each other in adult patients with NF1 in comparison with a healthy control population.

Spectral domain optical coherence tomography and B-scan ultrasonography in the evaluation of retinal tears in acute, incomplete posterior vitreous detachment.

BACKGROUND: The purpose of this study was to evaluate the extension and traction effects of posterior vitreous detachment (PVD) complicated with retinal tears using spectral domain optical coherence tomography (OCT) and B-scan ultrasonography. METHODS: Complete ophthalmological examination, B-scan ultrasonography and spectral domain OCT were performed in patients with acute PVD and retinal tears. Vitreous detachment was classified as complete or incomplete, based on extent of posterior pole or peripheral vitreous detachment. Retinal tear location and persistent traction on the retinal flap was evaluated with B-scan ultrasonography and OCT. Categorical data were evaluated with Fisher's exact test. Statistical significance was considered as P < 0.05. RESULTS: Twenty-six eyes of 25 patients were assessed. Four eyes (15 %) presented complete PVD with detachment at the posterior pole and periphery. 22 eyes (85 %) presented incomplete PVD with detachment in the periphery. Twenty eyes presented retinal tears in the superior quadrants with respect to only 6 in the inferior quadrants (p = 0.006). There was a higher incidence of retinal tears in the pre with respect to post-equatorial areas (19 vs 7 eyes, p = 0.019). B-scan ultrasonography and OCT revealed persistent traction on the retinal tear flap in 19 and 15 eyes, respectively. CONCLUSIONS: In acute PVD, retinal tears are prevalently associated with peripheral vitreous detachment. The impact of complete or incomplete PVD can be of clinical value when evaluating patients with retinal tears.

Intravitreal anti-vascular endothelial growth factor for retinal angiomatous proliferation in treatment-naive eyes: long-term functional and anatomical results using a modified PrONTO-style regimen.

PURPOSE: To evaluate long-term outcome of intravitreal anti-vascular endothelial growth factor monotherapy in retinal angiomatous proliferation. METHODS: Twenty-one treatment-naive eyes were included in this prospective, interventional case series. Treatment was three monthly injections of bevacizumab and/or ranibizumab with a modified PrONTO-style regimen. Best-corrected visual acuity (BCVA) was evaluated. The influence of baseline BCVA and pretreatment pigment epithelial detachment on BCVA outcome or retreatment were assessed by Pearson correlation analysis. RESULTS: Results were evaluated at 2 years and 3 years for 21 and 13 eyes, respectively. Mean baseline BCVA improved significantly from 44.5 (± 11.0) (20/32) to 51.1 (± 9.7) (20/24) and 50.8 (± 10.4) letters (20/24) at 2 and 3 years, respectively (P = 0.02 and P = 0.049). Pigment epithelial detachment correlated negatively with BCVA outcome (r = -0.65, P = 0.002 and r = -0.67, P = 0.01 at 2 years and 3 years, respectively) and was significantly associated with retreatment (r = 0.62, P = 0.003 and r = 0.87, P < 0.0001 at 2 years and 3 years, respectively). Complete occlusion of the lesion was obtained in 71% and 69% of eyes at 2 years and 3 years, respectively, with a mean of 9.4 injections at 3 years. CONCLUSION: Intravitreal anti-vascular endothelial growth factor monotherapy was a valid option for retinal angiomatous proliferation. Stable or improved visual acuity was obtained in 95% and 100% of eyes at 2 years and 3 years, respectively.