Multimodal imaging of congenital peri-papillary arterial loops presenting with vitreous hemorrage in a young male: A case report.

INTRODUCTION: Congenital arterial peripapillary loops are rare entities and very few cases are described in literature. CASE DESCRIPTION: A 25-year-old Asian man presented a diffuse vitreous hemorrhage in his Left Eye (LE). OCT-A revealed the presence of bilateral vascular loops at the optic nerve head. Fluorescein angiography (FA) confirmed the vascular abnormality in both eyes, with arterial filling in early phases and no dye leakage. At twenty days of follow up, the vitreous hemorrhage in the LE completely reabsorbed and BCVA improved from 20/63 to 20/20. CONCLUSION: Congenital peripapillary loops should be considered in the differential diagnosis of vitreous hemorrhage, especially in young patients with no history of ocular/head trauma. Multimodal imaging is highly recommended to properly manage the patients, avoiding unnecessary therapeutic choices.

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.

Ultrawide Field Imaging of Progressive Retinal Arteriovenous Malformation in a Pediatric Patient with Wyburn-Mason Syndrome.

Wyburn-Mason syndrome (WMS) is a rare congenital disease that presents with unilateral arteriovenous malformation (AVM) in the visual pathway, midbrain, and/or skin. We report a case of a 5-year-old girl with a history of cerebral and orbital AVM who presented with left exotropia and was found to have group 3 retinal AVM consistent with WMS. Here, we use ultrawide field imaging to show the progression of retinal AVM and peripheral nonperfusion areas for a period of 1 year in a pediatric patient with WMS. [Ophthalmic Surg Lasers Imaging Retina. 2022;53:46-48.].

Prevalence of Cilioretinal Arteries: A systematic review and a prospective cross-sectional observational study.

PURPOSE: To review studies focusing on cilioretinal arteries (CLRA) in order to assess the overall prevalence and establish the prevalence of CLRA in a Hungarian Caucasian population. METHODS #1: Systematic literature review of published studies with at least 100 participants. METHODS #2: Non-mydriatic digital colour photographs were taken of 1000 consecutively enrolled healthy Caucasian young adult volunteers. Images were graded by two trained independent observers. Number and location of identified cilioretinal arteries were recorded and statistically analysed. RESULTS #1: Prevalence of CLRA ranges from 6.9% to 49.5%. Detection with fluorescein angiography yields the highest values followed by fundus photography and ophthalmoscopy. Unilateral presence of CLRA is between 70.30% and 93.65%, and temporal location is between 80.77% and 100%. RESULTS #2: We found at least one CLRA in 36.5% of the participants and in 22.75% of all the examined eyes. Cilioretinal arteries (CLRA) were unilateral in 75.34% and bilateral in 24.66%. Of all the identified CLRA, 96.16% were originating from the temporal rim of the optic disc. We identified at least one temporal CLRA supplying the macula in 28% of the participants and 16.95% of the examined eyes. CONCLUSION: Prevalence of CLRA varies depending on identification method. Unilateral presence is unequivocally more frequent similarly to temporal location. From a risk of bias standpoint, high-quality studies are rare. Our data on the distribution pattern of CLRA are similar to that in the international literature. Based on our findings, we assume that slightly more than one-third of the Hungarian Caucasian population has a CLRA.

Arteriovenous crossing in retinal vessels of mice, rats, and pigs.

Purpose: Retinal vein occlusions (RVOs) are a common disease, but there are no animal models for spontaneous RVO formation. The critical sites of predilection, especially for branch RVO (BRVO), are the arteriovenous crossing sites in the inner retina. To gain more insight into possible animal models, the anatomic structure of retinal arteriovenous crossings was investigated in mice, rats, and pigs and compared to the human situation. Methods: Retinal flat mounts and paraffin sections of eyes from mice, rats, pigs, and humans were stained with GS lectin, Masson's trichrome, or immunohistochemistry for ACTA2 and GFAP. Serial sections of arteriovenous crossing sites were investigated. Results: Mice usually do not show retinal arteriovenous crossings. Rats have a mean of 2.8±1.4 crossings per eye at a mean distance from the optic nerve head of 2.79±0.53 mm, though the diameters of the crossing vessels are small. The situation in pigs is similar to that in humans, with many arteriovenous crossings of vessels and with similar diameters as found in humans. A mean of 28.4±3.5 crossings per retina was found, and 23% of these were arterial overcrossings. Serial paraffin sections showed that the tunica media of the artery touched that of the vein, but they did not fuse. Conclusions: While the retinal arteriovenous crossings of mice and rats are absent or comprised of rather thin vessels, those in the porcine retina are similar to adult humans. Therefore, the porcine retinal vascular bed may serve as a model to assess early steps in the formation of RVOs.

Unilateral Situs Inversus of the Optic Disc in a Patient With Retinal Arteriolar Tortuosity.

Familial retinal arteriolar tortuosity (fRAT) is a rare autosomal dominant disorder that is characterized by tortuosity of the second- and third-order arterioles around the posterior pole of retina. Most cases with fRAT lack associated systemic vascular abnormality. Situs inversus, a congenital anomaly, features emergence of retinal vessels from the optic nerve head in a nasal direction, usually occurs bilaterally. In this case report, the authors describe a unique case of fRAT with unilateral situs inversus. [Ophthalmic Surg Lasers Imaging Retina. 2020;51:533-535.].

Retinal Arteriovenous Malformation Occlusion and Optic Nerve Drusen: Casuality or Causality?

Multimodal imaging of an impending retinal vein occlusion in an arteriovenous malformation associated with optic nerve drusen (OND) in a 16-year-old girl affected by Wyburn-Mason Syndrome. The authors seek to determine whether the association between the two entities has had an additive role in the acute retinal vascular event. [Ophthalmic Surg Lasers Imaging Retina. 2020;51:418-419.].

Bilateral and multiple sub-internal limiting membrane hemorrhages in a familial retinal arteriolar tortuosity patient by Valsalva-like mechanism: an observational case report.

BACKGROUND: Bilateral and multiple Valsalva-related sub-internal limiting membrane (ILM) hemorrhages in a familial retinal arteriolar tortuosity (FRAT) patient is rare, and we treated this patient by both observation and Neodymium yttrium aluminum garnet (Nd: YAG) laser membranotomy methods. CASE PRESENTATION: A 13-year-old female student presented with sudden visual loss and central scotoma in both eyes after running 800 m at the school gym. The examination revealed six sub-ILM hemorrhages with the biggest hemorrhage measuring approximately 1.5-disc diameters (DD) in the right eye and two sub-ILM hemorrhages with the biggest one measuring 5.5 DD in the left eye. The patient was diagnosed as having Valsalva retinopathy associated with FRAT. Nd: YAG laser membranotomy was performed at the biggest hemorrhages and the rest hemorrhages were treated with observation in both eyes. The visual acuity recovered to 20/16 in the right eye and 20/20 in the left eye. Epiretinal membrane (ERM) formation was observed in the left eye. CONCLUSIONS: Nd: YAG laser could be considered for treating premacular hemorrhage in FRAT patient especially when a quick vision recovery was needed. This is the first reported case of a FRAT patient suffering from bilateral and multiple Valsalva-related sub-ILM hemorrhages which were treated by both observation and Nd: YAG laser treatment.

Detection of Occult Arteriovenous Malformation With Annular Array Ultrasonography.

Retinal vascular tortuosity may occur in a wide range of ocular disorders. When retinal vascular tortuosity involves both arteries and veins, and presents unilaterally and without hemorrhage, a diagnosis of Wyburn Mason syndrome (WMS) should be considered due to the potential morbidity and mortality associated with cerebral involvement. Magnetic resonance imaging (MRI) and MRI angiography (MRA) are important tools for identifying cerebral arteriovenous malformations (AVMs), but these imaging modalities have limited spatial resolution to detect very small vascular lesions. Annular array contact ocular ultrasound is a new imaging modality capable of detecting small intraorbital AVMs. [Ophthalmic Surg Lasers Imaging Retina. 2020;51:239-243.].

MACROANEURYSMS ASSOCIATED WITH CONGENITAL RETINAL MACROVESSELS.

PURPOSE: Congenital retinal macrovessels are large aberrant retinal blood vessels that cross the horizontal raphe and can traverse the central macula. Using multimodal imaging and optical coherence tomography angiography, we describe 2 cases of congenital retinal macrovessel associated with macroaneurysms. METHODS: Two patients presented for evaluation and were found to have congenital retinal macrovessels associated with macroaneurysms. Color photography, optical coherence tomography, fundus autofluorescence fluorescein angiography, and optical coherence tomography angiography were performed and used to establish the diagnosis and monitor resolution at follow-up visits. RESULTS: The first patient presented with central vision loss in the right eye and was noted to have a ruptured macroaneurysm and scattered microaneurysms along the course of a venous macrovessel. After 3 months of observation, the patient's vision improved. The second patient presented for evaluation of a cataract in her left eye and was incidentally found to have an arterial macrovessel in her right eye with an associated macroaneurysm. Both cases demonstrated an intricate capillary network in the central macula best visualized on optical coherence tomography angiography. CONCLUSION: Macroaneurysms can occur on both arterial and venous macrovessels. After rupture of these lesions, hemorrhage and exudation can resolve with observation alone. Macrovessels can also present with microaneurysms. Optical coherence tomography angiography can effectively image the complex capillary network associated with these vascular anomalies.

Dilated and tortuous retinal vessels as a sign of Cantu syndrome.

When encountering patients with markedly dilated and tortuous retinal vessels, Wyburn-Mason syndrome (WMS) or racemous angiomatosis (phacomatosis) is commonly thought of as the archetypal entity that can produce these findings. We describe a patient with Cantu syndrome with phenotypical findings identical to those seen in patients with WMS and want to highlight this as another entity that can present with tortuous and dilated retinal vessels.

The Oxygen Saturation in Vascular Abnormalities Depends on the Extent of Arteriovenous Shunting in Diabetic Retinopathy.

Purpose: Diabetic retinopathy is characterized by disturbances in retinal blood flow mediated by capillary occlusion, intraretinal microvascular abnormalities (IRMAs), neovascularizations, and omega loops and reduplications. It is likely that the study of oxygen saturation in these abnormalities can provide knowledge about their role in the development of diabetic retinopathy. Methods: The oxygen saturation in IRMA vessels and venous loops and reduplications were studied in 40 diabetic patients with severe nonproliferative or proliferative diabetic retinopathy. The saturation values in the studied vascular abnormalities were compared to those of the larger retinal arterioles and venules. Results: There was a similar oxygen saturation (mean ± SD) in IRMAs observed to connect arterioles with venules (78.6% ± 11.8%, n = 22) and IRMAs connecting venules with venules (79.2% ± 9.0%, n = 12; P > 0.999). The saturation in IRMAs was significantly lower (P < 0.0002) than in arterioles (97.4% ± 5.2%, n = 40) and significantly higher (P < 0.0001) than the saturation in omega loops and reduplications (54.2% ± 19.3%, n = 6), which in turn showed no significant difference from the saturation in the venules (61.8% ± 6.8%, n = 40, P = 0.4). Conclusions: The findings suggest that the oxygen saturation in vascular abnormalities in diabetic retinopathy depends on the extent of arteriovenous (A-V) shunting, with venous saturation due to no A-V shunting in venous loops and reduplications, and intermediate oxygen saturation due to moderate shunting in IRMAs. This may precede the development of neovascularizations with arterial oxygen saturation due to high A-V shunting.

[Spontaneous visual recovery following a central retinal artery occlusion in a patient with a cilioretinal artery]. / Szemfenéki arteria centralis retinae érelzáródás miatt kialakult hirtelen látásromlás spontán restitúciója cilioretinalis artéria jelenlétében.

Retinal arterial occlusion causes acute, painless vision loss, and it requires immediate emergency care. There are two separate arterial systems (retinal and ciliary) in the retina, and in most cases only the central retinal artery and its branches supply blood to the inner retinal layers. Cilioretinal artery is an anatomical variant, which can also supply blood to the macula from the ciliary arterial system, and in the case of a retinal arterial occlusion, the cilioretinal artery could save central vision. We report a case of a 67-year-old woman who suffered a central retinal arterial occlusion while having a patent cilioretinal artery and she had a complete recovery of her central visual acuity. A series of fundus photography and optical coherence tomography images are presented that were taken during follow-up. The patient's complaints started one week before she presented in our department therefore acute therapy was not given. However, during the course of the follow-up her status gradually improved, and she finally regained 1,0 (20/20) visual acuity. In the presence of a cilioretinal artery following a central retinal arterial occlusion, there is a chance of visual acuity preservation. Orv Hetil. 2019; 160(29): 1146-1152.