Retinal Manifestations Research Articles

Association of Retinal Vascular Manifestation and Obstructive Sleep Apnea (OSA): A Narrative Review.

Obstructive sleep apnea (OSA) is characterized by frequent episodes of partial or complete obstruction of the airway during sleep causing repeated episodes of apnea. OSA is more prevalent in middle-aged and older adults. OSA is associated with numerous ocular manifestations, including retinal manifestations. Literature highlighted the clear association between OSA and numerous ocular conditions including glaucoma and papilledema. This comprehensive and narrative review aims to summarize up-to-date clinical research concerning the association of OSA and vascular conditions that affect the retina. OSA is associated with the central serous chorioretinopathy (CSC), retinal vein occlusion (RVO), hypertensive retinopathy (HTRP) and development of diabetic retinopathy (DR). Sympathetic activation, hypoxia, and hormonal dysregulation all lead to serious retinal vascular conditions that will worsen OSA patients’ quality of life. It is important to refer patients with newly diagnosed OSA to an ophthalmology clinic for the appropriate test.

Bilateral Post Fever Retinitis With Retinal Vascular Occlusions Following Severe Acute Respiratory Syndrome Corona Virus (SARS-CoV2) Infection

ABSTRACT Introduction As the world fights the pandemic caused by the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), several reports of retinal manifestations of the disease are emerging. Post fever retinitis is a well reported ocular inflammatory reaction to multiple invasive agents, be it virus, bacteria or other microbial agent. Purpose We report an interesting case of bilateral multifocal retinitis with multiple vascular occlusions in a patient, three weeks after a febrile illness. Results Investigations to ascertain the cause of fever resulted in identifying ephemeral seropositivity for several different Ribonucleic acid (RNA) viruses. The retinitis was managed with systemic steroids, doxycycline and anticoagulants with improvement in vision. Conclusion High and persistent seropositive response against (SARS-CoV-2) helped us to narrow it as the causative agent but the cross reactivity of SARS-CoV-2 with other viruses can be misleading and needs careful interpretation.

Multimodal imaging of bilateral ischemic retinal vasculopathy associated with Berger\u2019s IgA nephropathy: case report

BackgroundBerger’s IgA nephropathy (IgAN) is the most common primary glomerulonephritis. However, some rare cases of retinal manifestations have been described, with only two cases of retinal vasculopathy reported in the literature.Here we report an uncommon case of bilateral ischemic retinal vasculopathy associated with Berger IgAN, evaluated with complete multimodal imaging including ultra-wide field (UWF) imaging and swept source optical coherence tomography angiography (SS-OCTA).Case presentationA 51-year-old woman with a history of Berger’s IgA nephropathy complained of visual impairment in both eyes. Fundus examination showed bilateral peripapillary arterial attenuation and perivascular sheathing, associated to perifoveal telangiectatic lesions. There was a central scotoma in the perimetry of the right eye and peripheral visual field defect in the left eye. Full-field electroretinogram revealed significantly reduced oscillatory potentials. Spectral domain optical coherence tomography showed multiple focal areas of thinning of the inner retina, indicating long-lasting vascular occlusion lesions. UWF fluorescein angiography showed the presence of bilateral vasculitis, diffuse capillary leakage, macular ischemia and telangiectasia. SS-OCTA better highlighted the macular ischemia and vascular anomalies layer-by-layer.ConclusionsRetinal vasculopathy is a very rare condition observed in IgA nephropathy. To our knowledge, this is the first report of complete multimodal functional and structural imaging. UWF imaging was very useful for accurate and comprehensive disease assessment, and OCTA was able to assess posterior pole vascular lesions.

Retinal manifestations in patients following COVID-19 infection: A consecutive case series.

Purpose:To describe retinal manifestations seen in patients associated with COVID-19 infection at a multi-specialty tertiary care hospital in Southern India.Methods:In this retrospective chart review, all consecutive cases presenting to the Retina-Uveitis service from May 2020 to January 2021 with retinal manifestations associated with COVID-19 infection or its sequelae or as a result of treatment given for COVID-19 were included.Results:Of the 7 patients, 3 were female, and 4 were male. Four patients had onset of symptoms during the active phase of COVID-19 infection. Four had bilateral and three had unilateral involvement. The manifestations ranged from mild to vision threatening. Vision threatening manifestations included infections: endogenous endophthalmitis, candida retinitis and tubercular choroidal abscess and bilateral pre-foveal hemorrhages. Milder manifestations included paracentral acute middle maculopathy, central serous chorio-retinopathy and voriconazole induced visual symptoms. Final visual acuity was 6/36 or better in the four severe cases and 6/9 or better in the mild cases.Conclusion:This study highlights the retinal manifestations associated with COVID-19 infection and its sequelae. As these patients presented with an association with COVID-19 (either during or after recovery), ophthalmologists should be vigilant and screen for such entities in case of complaints of visual symptoms or in the presence of systemic sepsis. The outcomes can be good with prompt and aggressive management.

Ocular Leishmaniasis - A systematic review.

The incidence of leishmaniasis is reported to be up to 1 million per year. To date, there has been no comprehensive review describing the diversity of clinical presentations of ocular leishmaniasis (OL) and its treatment. This systematic review aims to address this knowledge gap and provide a summary of the clinical presentation, natural course, and treatment options for OL. Our study identified a total of 57 published articles as describing cases of OL involving: adnexa (n = 26), orbit (n = 1), retina (n = 7), uvea (n = 18) and cornea (n = 6). Though well described and easily treated, palpebral leishmaniasis is often misdiagnosed and may lead to chronic issues if untreated. The retinal manifestations of Leishmaniasis consist of self-resolving hemorrhages secondary to thrombocytopenia. Two main uveitis etiologies have been identified: uveitis in the context of active Leishmanial infection (associated with immunosuppression) and uveitis occurring as an immune reconstitution syndrome. Corneal involvement in most geographic areas generally follows an aggressive course, most often ending in corneal perforation if left untreated. In the Americas, a chronic indolent interstitial keratitis may also occur. Topical steroids are of little use in keratitis (systemic antileishmanials being the cornerstone of treatment). However, these are essential in cases of uveitis, with or without concomitant systemic antileishmanial therapy. In conclusion, though ocular involvement in Leishmaniasis is rare, severe sight-threatening consequences follow if left untreated. Early diagnosis, enthusiastic follow-up and aggressive treatment are essential for good outcomes.

Retinal manifestations of COVID-19

Retinal manifestations of COVID-19 - IJCEO- Print ISSN No: - 2395-1443 Online ISSN No:- 2395-1451 Article DOI No:- 10.18231/j.ijceo.2021.038, Indian Journal of Clinical and Experimental Ophthalmology-Indian J Clin Exp Ophthalmol

Role of Retinal Amyloid-β in Neurodegenerative Diseases: Overlapping Mechanisms and Emerging Clinical Applications.

Amyloid-β (Aβ) accumulations have been identified in the retina for neurodegeneration-associated disorders like Alzheimer’s disease (AD), glaucoma, and age-related macular degeneration (AMD). Elevated retinal Aβ levels were associated with progressive retinal neurodegeneration, elevated cerebral Aβ accumulation, and increased disease severity with a decline in cognition and vision. Retinal Aβ accumulation and its pathological effects were demonstrated to occur prior to irreversible neurodegeneration, which highlights its potential in early disease detection and intervention. Using the retina as a model of the brain, recent studies have focused on characterizing retinal Aβ to determine its applicability for population-based screening of AD, which warrants a further understanding of how Aβ manifests between these disorders. While current treatments directly targeting Aβ accumulations have had limited results, continued exploration of Aβ-associated pathological pathways may yield new therapeutic targets for preserving cognition and vision. Here, we provide a review on the role of retinal Aβ manifestations in these distinct neurodegeneration-associated disorders. We also discuss the recent applications of retinal Aβ for AD screening and current clinical trial outcomes for Aβ-associated treatment approaches. Lastly, we explore potential future therapeutic targets based on overlapping mechanisms of pathophysiology in AD, glaucoma, and AMD.

Commentary: Retinal manifestations in patients following COVID-19 infection: A consecutive case series.

Coronavirus disease-2019 (COVID-19) pandemic has created a global health challenge owing to widespread mortality and morbidity. Alterations of the immune system, activation of coagulation pathways, and direct cytopathic effect of the virus are purported as the possible mechanisms that are responsible for the pathology of COVID-19. In addition to these, side effects of drugs and complications related to prolonged hospitalization and intensive care all combine to cause systemic manifestations of this virus, including ocular complications.[12] Multiple ophthalmic manifestations related to COVID-19 have been reported. Initially, when the pandemic started, ocular surface involvement was reported with self-limiting conjunctivitis being the earliest reported ocular manifestation.[3] Subsequently, a plethora of retinal manifestations have been reported in these patients, namely cotton wool spots, flame-shaped and dot-blot hemorrhages, retinal vascular occlusions, acute macular neuroretinopathy (AMN), paracentral acute middle maculopathy (PAMM), fungal retinitis, acute retinal necrosis, and serpiginous choroiditis. Neuro-ophthalmic manifestations like optic neuritis, papillo-phlebitis, extra-ocular muscle palsies, neurogenic ptosis, intracranial hypertension, cerebrovascular accident with vision loss, and orbital manifestations like mucormycosis and histiocytic orbital mass have also been reported in patients with COVID-19.[145] In the current study, authors have enumerated varied retinal manifestations following COVID-19. Patients with candida retinitis, tubercular choroidal abscess, and central serous chorioretinopathy (CSC) have been reported for the first time in this cohort.[6] Coagulopathy and endothelial dysfunctions are known associations of COVID-19 infection. In the eye, these manifest as cotton wool spots, central retinal vein occlusion, central retinal artery occlusion, AMN, and PAMM.[57] As reported in the current article, the aforementioned entities may occur during the active phase or even after recovery from COVID-19. Presence of cotton wool spots has been shown to be a potential biomarker in identifying underlying vascular and/or neurological comorbidity in COVID-19 patients.[7] Mean retinal artery diameter and the mean retinal vein diameter (MVD), measured using the Automated Retinal Image analyzer, have been shown to be higher in COVID-19 patients compared to healthy cohorts. MVD was negatively correlated with the time from onset of symptoms and positively correlated with disease severity.[8] Herein lies the role of an ophthalmologist and ocular examination in COVID-19 patients. Fundus examination is a simple, easy to perform and non-invasive. It has been shown to predict systemic endothelial dysfunction and triggering of the inflammatory cascade in subclinical and asymptomatic patients, thereby allowing earlier institution of appropriate therapy and potentially improving long-term morbidity and mortality.

Retina as a Model to Study In Vivo Transmission of α-Synuclein in the A53T Mouse Model of Parkinson's Disease.

Parkinson's disease is a neurodegenerative disorder characterized by accumulation of misfolded α-synuclein within the central nervous system (CNS). Retinal manifestations have been widely described as a prodromal symptom; however, we have a limited understanding of the retinal pathology associated with Parkinson's disease. The strong similarities between the retina and the brain and the accessibility of the retina has potentiated studies to investigate retinal pathology in an effort to identify biomarkers for early detection, as well as for monitoring the progression of disease and efficacy of therapies as they become available. Here, we discuss a study conducted using a transgenic mouse model of Parkinson's disease (TgM83, expressing human α-synuclein containing the familial PD-associated A53T mutation) to demonstrate the effect of the A53T α-synuclein mutation on the retina. Additionally, we show that "seeding" with brain homogenates from clinically ill TgM83 mice accelerates the accumulation of retinal α-synuclein. The work described in this chapter provides insight into retinal changes associated with Parkinson's disease and identifies retinal indicators of Parkinson's disease pathogenesis that could serve as potential biomarkers for early detection.

Retinal manifestations of COVID-19 disease - A review of available information

Ocular signs and symptoms have been reported in COVID-19 patients, and there is a surge of information about corneal, uveal, retinal, and neuro-ophthalmological involvement in COVID-19 infection. This review attempts to determine from various researches published during the time of the pandemic, the retinal manifestations of COVID-19 infection, and its significance and correlation to severity of systemic disease. An extensive search strategy was employed using keywords “novel corona virus”, “COVID-19”, “SARS CoV2,” or “retina,” either singly or variably combined, to retrieve the articles of retinal manifestations of COVID-19 published from January 2020 till date. Retinal manifestations are ubiquitous and include microangiopathy, occlusive vascular conditions, alterations in vascular caliber, and capillary density, among others. They seem to correlate with disease severity and reflect on similar changes in other parts of the body as well. Newer studies are bringing out more and more evidence of hitherto unknown retinal manifestations of COVID-19 disease.

Ocular manifestations of COVID-19 infection: A review of available information

Ocular signs and symptoms have been reported in COVID-19 patients, and there is increasing recognition of conjunctivitis as a prodromal symptom of this disease. There is also a surge of information about corneal, uveal, retinal, and neuroophthalmological involvement in COVID-19 infection. This review attempts to determine from various researches published during the time of the pandemic, the various ocular presentations of COVID-19 infection, its significance, correlation to the severity of systemic disease, and its importance as a mode of transmission of the disease. An extensive search strategy was employed to retrieve articles of ocular manifestations of COVID-19 published from January 2020 to date. Conjunctivitis was found to be the most common ocular manifestation, and viral RNA was noted in conjunctiva and tears, posing a risk for ophthalmologists. Other less commonly seen findings in COVID-19 infections include keratoconjunctivitis, blepharitis, retinal changes, and ophthalmoparesis. However, recent studies show increasing reports of retinal and neuroophthalmological manifestations of the COVID-19 infection.

Post corona virus Disease-19 (COVID-19): Hyper inflammatory syndrome-associated bilateral anterior uveitis and multifocal serous retinopathy secondary to steroids

Coronavirus disease 2019 (COVID-19) pandemic can cause ocular manifestations which include conjunctivitis to retinal manifestations. A 41-year-old Asian Indian male presented with unilateral blurring, glare, and pain in the left eye (LE), 22 days after COVID-19 infection. He had been administered intravenous steroids followed by oral steroids. Eye evaluation showed corrected distance visual acuity of 20/20 in both the eyes (BEs). Anterior chamber showed inflammation in the LE, a day later in the right eye. Fundus examination of the BE showed retinal pigment epithelial changes with serous elevation suggestive of multifocal serous chorioretinopathy. A month later, he had improvement in his symptoms with no cells in BE with topical steroids with resolution of serous retinopathy. Bilateral anterior uveitis possibly represents an immune-mediated trigger of his clinically quiescent psoriasis causing first-ever uveitis episode, possibly due to COVID-19. Serous chorioretinopathy could be secondary to steroids following treatment of COVID-19-related sequelae.

Chapter 6 - The role of optical coherence tomography in the diagnosis of afferent visual pathway problems: A neuroophthalmic perspective

Optical coherence tomography (OCT) is a noninvasive ocular imaging technique that has become a standard tool in neuroophthalmic practice. Specifically, OCT captures retinal manifestations of neuroaxonal injury caused by lesions along anterior and posterior regions of the afferent visual pathway, in patients presenting with vision loss. More recently, the advent of OCT angiography (OCTA) has enabled evaluation of the choroidal and retinal microvasculature, thus informing our understanding regarding vascular mechanisms associated with optic nerve and retinal injuries. Much of our longitudinal experience with OCT in the field of neuroophthalmology has been acquired from the study of optic neuritis (ON) caused by inflammatory disorders of the central nervous system (CNS). Over the past two decades, OCT has emerged as a surrogate endpoint for CNS neuroaxonal injury in multiple sclerosis (MS) research trials. On a more pragmatic level, OCT is used in the clinical arena to diagnose ON associated with: MS, neuromyelitis optica spectrum disorder (NMOSD), and myelin oligodendrocyte glycoprotein (MOG) antibody associated disease (MOGAD). Subsequent advancements in swept-source (SS) and enhanced depth imaging (EDI) have established OCT as the new "gold standard" in the diagnosis of optic disc drusen. Recent studies have highlighted pathognomonic OCT features that distinguish cases of true papilledema from pseudopapilledema, in patients presenting with undifferentiated optic disc elevation. Preoperative OCT measures of neuroaxonal integrity have shown prognostic value in predicting postoperative visual outcomes for patients with compressive anterior visual pathway lesions. Finally, OCT is indispensable in differentiating optic neuropathies from retinal diseases in patients with visual loss and a nondiagnostic fundus examination. An in-depth discussion regarding the technical aspects of OCT is beyond the scope of this chapter. Instead, we wish to highlight the value OCT brings to the diagnosis and management of common neuroophthalmic conditions, with emphasis on optic neuropathies and retinal disorders.

Malignant Hypertension

Abstract:
 Case review follows a 49-year-old patient with malignant hypertension with retinopathy and resultant visual decline.
 Case Report: Severely uncontrolled hypertension manifested as malignant hypertensive retinopathy. The patient presented with substantial retinal and visual changes and was monitored over 6 months. While the patient experienced marked improvement in visual acuity, significant retinal damage occurred and the patient experienced persistent qualitative vision and visual field loss.
 Discussion: Observed fundus changes are consistent with advanced risk for morbidity and mortality associated with hypertension. Management of systemic disease is a critical step in reversing retinal manifestations and minimizing the risk for permanent vision loss. The criticality of systemic treatment also applies to lowed risk for systemic vascular events that may compromise quality of life. This case demonstrates that improvement in Snellen acuity does not always parallel functional visual improvement. Eye care providers managing these cases should consider both systemic risks and visual outcome.

Retinal Spheroids and Axon Pathology Identified in Amyotrophic Lateral Sclerosis.

PurposeTo determine whether patients with amyotrophic lateral sclerosis (ALS) show retinal axon pathology.MethodsPostmortem eyes from 10 patients with ALS were sectioned and compared with 10 age-matched controls. Retinal sections were evaluated with periodic acid Schiff and phosphorylated (P-NF) and nonphosphorylated (NP-NF) forms of neurofilament with SMI 31 and 32 antibodies. Spheroids identified in the retinal nerve fiber layer were counted and their overall density was calculated in central, peripheral, and peripapillary regions. P-NF intensity was quantified. Morphometric features of ALS cases were compared with age-matched controls using the exact Wilcoxon matched-pairs signed-rank test.ResultsDistinct periodic acid Schiff–positive round profiles were identified in the retinal nerve fiber layer of patients with ALS and were most commonly observed in the peripapillary and peripheral retina. The density of periodic acid Schiff–positive spheroids was significantly greater in patients with ALS compared with controls (P = 0.027), with increased density in the peripapillary region (P = 0.047). Spheroids positive for P-NF and NP-NF were detected. P-NF–positive spheroid density was significantly increased in patients with ALS (P = 0.004), while the density of NP-NF spheroids did not differ significantly between ALS and control groups (P > 0.05). P-NF immunoreactivity in the retinal nerve fiber layer was significantly greater in patients with ALS than in controls (P = 0.002).ConclusionsRetinal spheroids and axon pathology discovered in patients with ALS, similar to hallmark findings in spinal cord motor neurons, point to disrupted axon transport as a shared pathogenesis. Retinal manifestations detected in ALS suggest a novel biomarker detectable by noninvasive retinal imaging to help to diagnose and monitor ALS disease.

Systemic Disease and Ocular Comorbidity Analysis of Geographically Isolated Federally Recognized American Indian Tribes of the Intermountain West.

Background: The American Indian Navajo and Goshute peoples are underserved patient populations residing in the Four Corners area of the United States and Ibupah, Utah, respectively. Methods: We conducted a cross-sectional study of epidemiological factors and lipid biomarkers that may be associated with type II diabetes, hypertension and retinal manifestations in tribal and non-tribal members in the study areas (n = 146 participants). We performed multivariate analyses to determine which, if any, risk factors were unique at the tribal level. Fundus photos and epidemiological data through standardized questionnaires were collected. Blood samples were collected to analyze lipid biomarkers. Univariate analyses were conducted and statistically significant factors at p < 0.10 were entered into a multivariate regression. Results: Of 51 participants for whom phenotyping was available, from the Four Corners region, 31 had type II diabetes (DM), 26 had hypertension and 6 had diabetic retinopathy (DR). Of the 64 participants from Ibupah with phenotyping available, 20 had diabetes, 19 had hypertension and 6 had DR. Navajo participants were less likely to have any type of retinopathy as compared to Goshute participants (odds ratio (OR) = 0.059; 95% confidence interval (CI) = 0.016–0.223; p < 0.001). Associations were found between diabetes and hypertension in both populations. Older age was associated with hypertension in the Four Corners, and the Navajo that reside there on the reservation, but not within the Goshute and Ibupah populations. Combining both the Ibupah, Utah and Four Corners study populations, being American Indian (p = 0.022), residing in the Four Corners (p = 0.027) and having hypertension (p < 0.001) increased the risk of DM. DM (p < 0.001) and age (p = 0.002) were significantly associated with hypertension in both populations examined. When retinopathy was evaluated for both populations combined, hypertension (p = 0.037) and living in Ibupah (p < 0.001) were associated with greater risk of retinopathy. When combining both American Indian populations from the Four Corners and Ibupah, those with hypertension were more likely to have DM (p < 0.001). No lipid biomarkers were found to be significantly associated with any disease state. Conclusions: We found different comorbid factors with retinal disease outcome between the two tribes that reside within the Intermountain West. This is indicated by the association of tribe and with the type of retinopathy outcome when we combined the populations of American Indians. Overall, the Navajo peoples and the Four Corners had a higher prevalence of chronic disease that included diabetes and hypertension than the Goshutes and Ibupah. To the best of our knowledge, this is the first study to conduct an analysis for disease outcomes exclusively including the Navajo and Goshute tribe of the Intermountain West.

Retinal involvement and ocular findings in COVID-19 pneumonia patients

Changes in immune and coagulation systems and possible viral spread through the blood–brain barrier have been described in SARS-CoV-2 infection. In this study, we evaluated the possible retinal involvement and ocular findings in severe COVID-19 pneumonia patients. A cross-sectional study was conducted on 46 patients affected by severe COVID-19 who were hospitalized in one intensive care unit (ICU) and in two infectious disease wards, including bedside eye screening, corneal sensitivity assessment and retinography. A total of 43 SARS-CoV-2-positive pneumonia patients affected with COVID-19 pneumonia were included, including 25 males and 18 females, with a median age of 70 years [IQR 59–78]. Except for one patient with unilateral posterior chorioretinitis of opportunistic origin, of whom aqueous tap was negative for SARS-CoV-2, no further retinal manifestation related to COVID-19 infection was found in our cohort. We found 3 patients (7%) with bilateral conjunctivitis in whom PCR analysis on conjunctival swabs provided negative results for SARS-CoV-2. No alterations in corneal sensitivity were found. We demonstrated the absence of retinal involvement in SARS-CoV-2 pneumonia patients. Ophthalmologic evaluation in COVID-19, particularly in patients hospitalized in an ICU setting, may be useful to reveal systemic co-infections by opportunistic pathogens.

Retinal manifestations of the neurocutaneous disorders.

The neurocutaneous disorders are a genetically and phenotypically diverse group of congenital syndromes characterized by cutaneous, ocular, and central nervous system manifestations. This review provides an overview of the clinical features and retinal findings in selected neurocutaneous disorders. Advances in genetics and diagnostic retinal and neuroimaging allow for the recognition of retinal features of common neurocutaneous syndromes and for improved characterization of rarer entities based on previously underdiagnosed or unrecognized retinal findings. Better characterization of the neurocutaneous disorders allows for earlier recognition and the potential for expeditious vision-saving and life-saving treatment.

Retinal vascular manifestations of obstructive sleep apnea.

The aim of this article is to summarize up-to-date research on the effects of obstructive sleep apnea (OSA) on retinal vascular conditions. OSA is associated with the development of diabetic retinopathy, retinal vein occlusion, and central serous chorioretinopathy. The severity of OSA and biomarkers such as the apnea-hypopnea index (AHI) correlate with the severity of retinal disease. Dysregulation of circadian locomotor output cycles kaput (CLOCK) genes that govern circadian rhythm is associated with development of proliferative retinal disease. OSA and retinal vascular disease have a high cost burden on the healthcare system. OSA creates systemic changes and hypoxic conditions that may incite or exacerbate retinal vascular diseases. Retinal changes may be the first clinical manifestation of otherwise undiagnosed OSA, so it is important to refer patients with new-onset retinal vascular disease for appropriate sleep testing.

Early management of sight threatening retinopathy in incontinentia pigmenti

BackgroundEarly blindness secondary to incurable retinal detachment is one of the main complications of incontinentia pigmenti (IP). The efficiency of ophthalmological management for preventing such evolution has not been proven.The objective of this retrospective study was to report a screening and treatment strategy of the vascular retinopathy in newborns and infants with IP.ResultsAll files of patients diagnosed with IP within the two first months of life in a single tertiary referral center, between 2010 and 2015, were retrospectively included. The minimum follow-up duration was three years.Patients had undergone systematic indirect ophthalmoscopy examination, looking for signs of peripheric retinal vasculopathy, according to a standardized schedule: at diagnosis, at age 1, 2, 3, 6, 9, 12, 18 and 24 months, and then once a year. Urgent laser therapy was performed under anesthesia in case of signs of retinal ischemia.Nineteen children files (17 girls) were studied. Median age at IP diagnosis was 1 day [0–44]; median age at first retinal evaluation was 25 days. Retinal manifestations occurred in 7 patients (n = 10/38 eyes, 26.3%); they were diagnosed at median age 19 days [3–59]. These patients underwent one or two ablative session per eye (mean 1.7, median 2), under general anaesthesia. No retinal detachment or fold occurred during the follow-up (median 6 years [3–9.8]).ConclusionOcular screening should be performed in all cases of IP as soon as possible after diagnosis. A strict ophthalmological monitoring and prophylactic treatment of retinal vasculopathy can efficiently prevent the early blinding complications of the disease.