Prepapillary vascular loops - A new classification.

Background: Prepapillary vascular loops are a type of congenital vascular anomaly seen on or around the optic disk. Patients with this condition are usually asymptomatic and are detected incidentally on routine fundus examinations. Differential diagnosis for this condition includes neovascularization of the disk and collaterals on the disk. Prepapillary capillary loops are not associated with any systemic condition. They are usually unilateral in presentation, but can rarely be bilateral. Purpose: To discuss the new proposed classification of prepapillary capillary loops. Synopsis: : Prepapillary capillary loops are classified based on their location around the disk, loop characteristics such as elevation, shape, and covering, and presence of vitreoretinal traction. Highlights: The most common vascular loops are arterial in origin and rarely venous in origin. They can sometimes be associated with spontaneous and recurrent vitreous hemorrhage, branch retinal artery or vein occlusion, and subretinal hemorrhage. It is an important differential diagnosis in spontaneous vitreous hemorrhage. Treatment is symptomatic. Video link: : https://youtu.be/gbq_oP7Y2q4.

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.

Racemose hemangioma presenting as a vitreous hemorrhage: case report / Hemangioma racemoso apresentando-se como hemorragia vítrea: relato de caso

ABSTRACT This report describes a case of retinal racemose hemangioma that first presented as a vitreous hemorrhage. The authors present the case of a 47-year-old woman with a sudden 5-day painless visual loss in her left eye. At the first visit, the best-correct visual acuities were 20/20 in the right eye and hand motions in the left eyes. Ultrasonography showed an attached retina and a massive vitreous hemorrhage. Pars plana vitrectomy was performed and a dilatation of large vessels was detected bulging from the optic disc. The best-correct visual acuities on day 30 postoperatively was 20/25 in the left eye. Fundus angiography and spectral-domain optical coherence tomography angiography showed anomalous arteriovenous communications with no intervening capillaries. The diagnosis was racemose hemangioma, an arteriovenous malformation of group 2 retina based on the Archer classification.

RESUMO Este relato descreve um caso de hemangioma racemoso da retina que se apresentou inicialmente como hemorragia vítrea. Os autores apresentam o caso de uma mulher de 47 anos com perda visual súbita e indolor 5 dias antes no olho esquerdo. Na primeira visita, a melhor acuidade visual corrigida foi de 20/20 no olho direito e movimentos das mãos no olho esquerdo. A ultrassonografia mostrou uma retina aderida e uma hemorragia vítrea maciça. Foi realizada vitrectomia pars plana, sendo detectada proliferação de grandes vasos salientes do disco óptico. A acuidade visual no dia 30 de pós-operatório foi de 20/25 no olho esquerdo. A angiografia de retina e a angiotomografia de coerência óptica de domínio espectral mostraram comunicações arteriovenosas anômalas sem capilares intermediários. O diagnóstico foi hemangioma racemoso, uma malformação arteriovenosa da retina do grupo 2 com base na classificação de Archer.

Ambliopia severa na infância relacionada à persistência da vasculatura fetal / Severe childhood amblyopia associated to persistence of fetal vasculature

RESUMO A persistência do vítreo primário hiperplásico, atualmente referida como persistência da vasculatura fetal, é uma anomalia congênita que resulta da não regressão do vítreo vascular primário e do sistema da artéria hialoide durante a embriogênese. Trata-se de uma anomalia unilateral na maioria dos casos, esporádica e comumente não associada a nenhum outro achado sistêmico. Clinicamente, essa condição pode ser classificada em persistência anterior e em persistência posterior da vasculatura fetal. A condição anterior está relacionada ao sistema da artéria ciliar, enquanto a persistência da vasculatura posterior associa-se à artéria hialoide e pode apresentar anormalidades, com desfecho visual desfavorável. A detecção da persistência do vítreo primário hiperplásico é de suma importância, visto que é um diagnóstico diferencial para retinoblastoma. O relato de caso a seguir descreve o acompanhamento ambulatorial em um Serviço de Oftalmologia de uma criança do sexo masculino com persistência da vasculatura fetal unilateral e sem alterações sistêmicas.

ABSTRACT Hyperplastic primary vitreous persistence, currently referred to as fetal vasculature persistence, is a congenital anomaly that results from non-regression of the primary vascular vitreous and hyaloid artery system during embryogenesis. It is a unilateral anomaly in the vast majority of cases, sporadic and commonly not associated with any other systemic finding. Clinically, this condition can be classified into anterior and posterior persistence of fetal vasculature. The anterior condition is related to the ciliary artery system, while the persistence of the posterior vasculature is associated with the hyaloid artery, which may present abnormalities with an unfavorable visual outcome. Detecting persistent hyperplastic primary vitreous is of paramount importance, as it is a differential diagnosis for retinoblastoma. The following case report describes the outpatient follow-up at the ophthalmology service of the Federal University of Triângulo Mineiro (UFTM) of a male child with persistent unilateral fetal vasculature and no systemic changes.

Macrovasos congénitos retinianos: presentación de un caso / Congenital retinal macrovessels: Case presentation

Presentamos un caso de macrovaso congénito retiniano estudiado mediante retinografía, campo visual, angiografía fluoresceínica y tomografía óptica de coherencia macular. El macrovaso atravesaba el meridiano horizontal, nasalmente entre papila y mácula. Además, se realiza una revisión de los macrovasos congénitos retinianos, las alteraciones visuales y estructurales que causan su asociación con otras enfermedades y su diagnóstico diferencial (AU)

We present an uncommon case of a unilateral congenital retinal macrovessel documented with retinography, perimetry, fluorescein angiography and macular optical coherence tomography. In the case presented the macrovessel crossed the horizontal meridian, between macula and optic disk. A literature review has been performed on congenital retinal macrovessels, possible structural and visual alterations they may cause and their association with other pathologies (AU)

Choroidal Vascular Abnormalities by Ultra-widefield Indocyanine Green Angiography in Polypoidal Choroidal Vasculopathy.

Purpose: To evaluate vortex vein engorgement and choroidal vascular hyperpermeability in patients with polypoidal choroidal vasculopathy (PCV) using ultra-widefield indocyanine green angiography (ICGA). Methods: This retrospective case control study included 51 patients with unilateral PCV, 7 patients with bilateral PCV, and 43 age-matched controls. The number of quadrants of vortex vein engorgement was evaluated in the middle phase of ICGA, which was classified as extended engorgement if the dilated choroidal vessels expanded to the macula. The area of choroidal vascular hyperpermeability was quantified stereographically from the late-phase ICGA and correlated with clinical and optical coherence tomography findings. Results: Affected eyes had a larger choroidal hyperpermeability area and a thicker subfoveal choroid than eyes in the control group or fellow eyes (P < 0.001, P < 0.001). More quadrants with extended vortex vein engorgement were observed in affected eyes than in fellow eyes (P < 0.001). Significant differences were observed in the area of choroidal hyperpermeability, Haller layer thickness and greatest linear dimension according to the extended vortex vein engorgement in eyes with PCV (P < 0.001, P = 0.001, and P = 0.001, respectively). The area of choroidal hyperpermeability was significantly correlated with subfoveal choroidal thickness (P < 0.001, Pearson's correlation coefficient = 0.471). Conclusions: Ultra-widefield ICGA results revealed that patients with PCV had vortex vein engorgement and an increased choroidal hyperpermeability area. The results from this study provide substantial information to clarify the pathogenesis and predict the prognosis in the patients with PCV.

Multimodal imaging comparison of perifoveal exudative vascular anomalous complex and resembling lesions.

PURPOSE: Perifoveal exudative vascular anomalous complex (PEVAC) was initially described as an isolated aneurysmal lesion in healthy eyes. Similar aneurysmal abnormalities may occur in association with retinal vascular diseases such as diabetic retinopathy or retinal vein occlusions (PEVAC-resembling). The aim of this study was to compare several imaging characteristics of PEVAC and PEVAC-resembling lesions. METHODS: Ten eyes with a PEVAC and 27 eyes with a PEVAC-resembling lesion were included in this cross-sectional study. They were all imaged with optical coherence tomography (OCT), OCT angiography (OCT-A) and colour fundus photography (CFP). Several clinical, morphological and vascular characteristics were assessed and compared between both PEVAC types. RESULTS: All PEVAC lesions were unilateral, while PEVAC-resembling lesions appeared bilateral in 23% of patients (p > 0.05). Unilateral multifocal PEVAC-resembling lesions were more frequently observed (56%) than unilateral multifocal PEVAC lesions (10%, p < 0.01). Furthermore, 90% of the PEVAC lesions were located within 500 µm from the centre of the fovea, while this was only true for 56% of the PEVAC-resembling lesions (p > 0.05). No notable differences were observed in other studied characteristics. CONCLUSIONS: The clinical, morphological and vascular features of PEVAC and PEVAC-resembling lesions are similar based on multimodal imaging. Given the bilaterality and multifocality seen in PEVAC-resembling lesions, an underlying retinal vascular disease may stimulate the quantity of aneurysmal abnormalities. Due to the similarities with PEVAC-resembling lesions, PEVAC may also be considered a microangiopathy but with an unknown origin.

Widefield Fluorescein Angiography in the Fellow Eyes of Patients with Presumed Unilateral Persistent Fetal Vasculature.

PURPOSE: To examine the retinal vascular findings on widefield fluorescein angiography (FA) in the fellow eyes of patients with unilateral persistent fetal vasculature (PFV). DESIGN: Retrospective case series. PARTICIPANTS: Consecutive patients with unilateral PFV evaluated by a single physician at an academic medical center from February 1, 2011, to November 30, 2018. METHODS: Clinical and demographic information, including age, gender, race, ethnicity, affected eye, subtype, stalk origin, complications on presentation, length of follow-up, and examination findings, was reviewed using the electronic medical record. Fluorescein angiograms of the affected and fellow eyes were reviewed by 2 authors for characteristic retinal vascular abnormalities. Agreement between the authors' scores was analyzed using Cohen's Kappa. MAIN OUTCOME MEASURES: Fluorescein angiography abnormalities, including peripheral vessel avascularity, aberrant circumferential vessels, terminal supernumerary branching, regional capillary dropout, terminal bulbing, abnormal choroidal flush, abnormal vessel straightening, and peripheral vessel leakage or dilation. RESULTS: Inclusion criteria were met by 41 patients. The average age at initial visit was 10.0 months. The average length of follow-up was 36.4 months. Abnormalities on FA were seen in 31 (75.6%) fellow eyes: peripheral vessel avascularity in 27.5 (67.1%), aberrant circumferential vessels in 20 (48.8%), terminal supernumerary branching in 17 (41.5%), regional capillary dropout in 9 (22.0%), terminal bulbing in 6 (14.6%), abnormal choroidal flush in 3.5 (8.5%), and abnormal vessel straightening and peripheral vessel leakage in 2.5 (6.1%). Statistical analysis disclosed an overall observed agreement of 93.4% among the raters (κ = 0.84, P < 0.0001). CONCLUSIONS: Retinal vascular abnormalities seen in patients with unilateral PFV were present in the majority of fellow eyes. This suggests that unilateral PFV may in fact be a bilateral, asymmetric process, but the clinical significance of these subtle findings is not known.

OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY FINDINGS IN A CASE OF CONGENITAL RETINAL MACROVESSEL WITH ANOMALOUS RETINAL ANASTOMOSIS ASSOCIATED WITH CONTRALATERAL MYELINATED NERVE FIBERS AND RETINAL VASCULAR ABNORMALITIES.

PURPOSE: To describe a case of congenital retinal macrovessel complicated by cystoid macular edema associated with contralateral myelinated retinal nerve fibers and retinal vascular abnormalities studied with optical coherence tomography angiography (OCTA). METHODS: Case report. RESULTS: A healthy 25-year-old woman with decreased vision in her right eye was found to have a congenital retinal venous macrovessel in the macula associated with cystoid edema. In the contralateral amblyopic eye, the examination revealed a tuft of myelinated retinal nerve fibers along the superotemporal vascular arcade associated with superficial vascular abnormalities. A complete multi-imaging examination was obtained, including fundus color photography, fluorescein angiography, indocyanine green angiography, optical coherence tomography (OCT), and optical coherence tomography angiography. At 1-week follow-up, the optical coherence tomography displayed spontaneous resolution of the edema that remained stable at consecutive 1-month follow-up. CONCLUSION: Congenital retinal macrovessels can be associated with other ocular developmental anomalies. Vascular complications can occur, leading to macular edema and retinal ischemia. Optical coherence tomography angiography can be useful for the diagnosis and follow-up of this condition.

OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY IN THE DIAGNOSIS AND FOLLOW-UP OF RETINAL ARTERIAL MACROANEURYSMS.

PURPOSE: To describe optical coherence tomography angiography (OCTA) findings in retinal arterial macroaneurysm (RAM) associated with macular edema and to correlate OCTA findings with conventional multimodal imaging. METHODS: The clinical course, conventional multimodal imaging findings including fundus color photography, spectral domain optical coherence tomography (Spectralis; Heidelberg Engineering, Heidelberg, Germany), fluorescein angiography (Heidelberg Engineering), and OCTA (Optovue, Inc, Freemont, CA) findings at baseline and during the follow-up of two eyes (two patients) with symptomatic RAM associated with macular edema were documented. RESULTS: Two eyes of 2 patients, both women, aged 82 and 46 years, which presented with progressive visual decline, were included. On conventional multimodal imaging, exudative RAM with macular edema and lipid exudation were visible in both included eyes. On OCTA, the flow in the two RAM was detected at baseline. Case 1 was treated by focal laser photocoagulation. One month after treatment, fluorescein angiography showed RAM occlusion. Spectral domain optical coherence tomography showed a RAM and retinal thinning and a decreased central foveal thickness, resulting in visual acuity improvement. On OCTA, no flow was detectable in the RAM at 1-month follow-up. Case 2 was not treated at baseline. In this eye, no flow was detected on OCTA at 2-month follow-up. This suggests a spontaneous occlusion, which was confirmed by fluorescein angiography. CONCLUSION: Optical coherence tomography angiography is able to detect the presence or absence of flow signal within RAMs, which may both decrease the need for dye angiography in selected cases with exudative RAM and help in treatment decision making.

Prepapillary vascular loop-a new classification.

BACKGROUND/OBJECTIVES: To analyze the ophthalmic characteristics of congenital prepapillary vascular loop (PVL) and to propose a new morphologic classification dividing the loops into six types. SUBJECTS/METHODS: Collaborative multinational multicentre retrospective study of PVL cases. RESULTS: There was a total of 49 cases (61 eyes), 37 unilateral (75.5%) and 12 bilateral (24.5%), 32 arterial type (65.3%) and 18 venous type (36.7%) (one patient had either kind in each eye). The mean number of loops per eye was 2.7 (range, 1-7). The loops were asymptomatic in 42 cases (85.7%). Other findings included: the presence of cilioretinal artery (14 cases), retinal vascular tortuosity (26 cases), amaurosis fugax (1 case), branch retinal artery occlusion (1 case) and vitreous haemorrhage (3 cases). Six morphologic loop types could be discerned based on elevation (flat vs. elevated), shape (figure of 8 or corkscrew with hyaline sheath), number (multiple or single), location (central or peripheral), lumen size (arterial vs. arteriolar) and presence of vascular tortuosity or vitreous traction. CONCLUSIONS: PVL are usually asymptomatic and can be divided into six morphologic types with different pathogenesis during early embryogenesis.

RETINAL VASCULAR ABNORMALITIES RELATED TO NEUROFIBROMATOSIS TYPE 1: Natural History and Classification by Optical Coherence Tomography Angiography in 473 Patients.

PURPOSE: To analyze and classify neurofibromatosis Type 1 (NF1)-related retinal vascular abnormalities (RVAs), their natural history and correlation with disease severity, in a large cohort of patients. METHODS: This was an observational longitudinal study with prospective enrollment. Four hundred and seventy-three patients affected by NF1 and 150 age-matched healthy subjects were consecutively enrolled. Retinal vascular abnormalities were detected by means of near-infrared reflectance and studied by optical coherence tomography angiography. The superficial vascular plexus and the deep vascular complex (DVC) were quantitatively and qualitatively analyzed. RESULTS: We identified RVAs in 82 of 473 (17%) NF1 patients, but in none of the 150 healthy subjects. A comparison revealed that NF1 patients with RVAs showed a higher number of NF1 diagnostic criteria (4.3 ± 1.5 vs. 3.9 ±1.5, respectively; P = 0.02) than patients without RVAs. Three different RVA types were identified on optical coherence tomography angiography: macrovascular angiomatosis of the sole superficial vascular plexus; macrovascular angiomatosis of the superficial vascular plexus combined with microvascular angiomatosis of the deep vascular complex; and combined macrovascular angiomatosis of both superficial vascular plexus and deep vascular complex. The prospective analysis of optical coherence tomography angiography images showed no significant longitudinal evolution of RVAs (mean follow-up: 3.7 ± 2.8 years). A single patient developed a de novo single RVA, and two RVAs showed detectable changes during follow-up. CONCLUSION: In NF1 patients, RVAs are a characteristic sign that correlates with a more severe systemic disease expression, usually remaining stable during time. Optical coherence tomography angiography allows for the identification of different RVAs subtypes.