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.

OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY CAN CATEGORIZE DIFFERENT SUBGROUPS OF CHOROIDAL NEOVASCULARIZATION SECONDARY TO AGE-RELATED MACULAR DEGENERATION.

PURPOSE: Choroidal neovascularization (CNV) is a common complication of patients affected by age-related macular degeneration, showing a highly variable visual outcome. The main aim of the study was, at baseline, to perform a quantitative optical coherence tomography angiography assessment of CNV secondary to age-related macular degeneration and to assess posttreatment outcomes. METHODS: Seventy-eight naïve age-related macular degeneration-related CNV patients (39 men, mean age 78 ± 8 years) were recruited and underwent complete ophthalmologic evaluation and multimodal imaging. Several OCT and optical coherence tomography angiography parameters were collected, including vessel tortuosity and vessel dispersion (VDisp), measured for each segmented CNV. All patients underwent anti-vascular endothelial growth factor PRN treatment. Vessel tortuosity and VDisp values of CNVs were tested at baseline to establish a cutoff able to distinguish clinically different patient subgroups. RESULTS: Mean best-corrected visual acuity was 0.49 ± 0.57 (20/62) at baseline, improving to 0.31 ± 0.29 (20/41) at the 1-year follow-up (P < 0.01), with a mean number of 6.4 ± 1.9 injections. Our cohort included the following CNV types: occult (45 eyes; 58%), classic (14 eyes; 18%), and mixed (19 eyes; 24%). Observing optical coherence tomography angiography parameters, classic, mixed, and occult CNV revealed significantly different values of VDisp, with classic forms showing the highest values and the occult CNVs showing the lowest (P < 0.01); mixed forms displayed intermediate VDisp values. The ROC analysis revealed that a CNV vessel tortuosity cut-off of 8.40, calculated at baseline, enabled two patient subgroups differing significantly in visual outcomes after anti-vascular endothelial growth factor treatment to be distinguished. CONCLUSION: A baseline quantitative optical coherence tomography angiography-based parameter could provide information regarding both clinical and functional outcomes after anti-vascular endothelial growth factor treatment in age-related macular degeneration-related CNV.

MULTIMODAL IMAGING OF CHOROIDAL AND OPTIC DISK VESSELS NEAR OPTIC DISK PITS.

PURPOSE: To describe modern multimodal imaging of the choroidal and optic disk vessels in optic disk pits. METHODS: Case reports of four patients with optic disk pit who underwent multimodal imaging of the optic pit and surrounding structures. Patients included in this article were found to have optic disk pits and subsequently underwent multimodal imaging. RESULTS: Cilioretinal arteries were present in two of the four cases (50%). SPECTRALIS optical coherence tomography showed intraretinal and subretinal fluid in all cases. Small vessels in the choroid and in the disk around the pit were also present in all cases through optical coherence tomographic angiography. Confocal fluorescein angiographic imaging in the first case showed leakage from the vessels adjacent to the optic disk pit. CONCLUSION: Modern multimodal imaging shows that there are anomalous vessels in and around an optic pit. Whether these vessels affect the development of optic pit, maculopathy needs to be further evaluated.

[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.

CENTRAL RETINAL ARTERY OCCLUSION WITH DOUBLE CILIORETINAL ARTERY SPARING.

PURPOSE: To report a patient with central retinal artery occlusion with double cilioretinal artery sparing. METHODS: Retrospective case report. RESULTS: A 45-year-old woman presented with sudden vision loss in her left eye for 10 days. Her best-corrected visual acuity was counting fingers at 2 meters in the left eye and 20/20 in the right eye. Funduscopy showed retinal opacity of posterior fundus, most marked in the macular region, and two small areas of the normal retina; one temporal to the optic disk and the other superior to the optic disk corresponding to the patent cilioretinal retinal arteries in the left eye; the right eye was normal. Fundus fluorescein angiography showed rapid filling of the two cilioretinal vessels and delayed filling of the central retinal artery at initial and last visits. Spectral domain optical coherence tomography findings disclosed diffuse thickening of the neurosensory retina in the perifoveolar area except two small areas at the first visit. After 6 months, spectral domain optical coherence tomography revealed diffuse thinning of the neurosensory retina except two small areas of the retina fed from two patent cilioretinal arteries. CONCLUSION: In this report, we documented double cilioretinal artery sparing in a patient with central retinal artery occlusion. It seems that a second cilioretinal artery can be present in some patients with central retinal artery occlusion.

Central retinal artery originating from the temporal short posterior ciliary artery associated with intraorbital external-to-internal carotid arterial anastomoses.

Although variations in the peripapillary division pattern of the central retinal artery (CRA) are common, variations in the origin of this artery are seldom reported in the literature. Herein, the authors report a variant of the CRA that originated from the temporal short posterior ciliary artery. Two intraorbital anastomoses were also noted between the internal and external carotid arterial systems: one through the lacrimal artery-middle meningeal artery to the ophthalmic artery and the other through supraorbital artery-recurrent meningeal artery to the ophthalmic artery. A brief review of CRA variations and potential clinical significances of the observed variant are discussed.

Ocular manifestations of Fabry disease within in a single kindred.

BACKGROUND: Fabry disease is an X-linked lysosomal storage disorder that causes progressive complications within the kidneys, brain, and heart. Ocular manifestations of this disease are often present at a very young age, thereby facilitating early diagnosis, before the signs and symptoms of renal disease, stroke, or hypertrophic cardiomyopathy. Early diagnosis by the eye care provider may eventually reduce the morbidity and mortality of this disease through the institution of therapy before the development of sclerotic end organ damage. This study evaluated 23 Fabry-affected members of a single cohort for the presence of ocular signs of Fabry disease. METHODS: Twenty-three patients of a single family were seen on a single day. Patients were given comprehensive ophthalmic examinations and completed a health and lifestyle questionnaire. RESULTS: Eight hemizygous men (mean age, 32.3 years) and 15 heterozygous women (mean age, 26.9 years) from a single family of 43 known Fabry patients were evaluated. Corneal verticillata was present in all patients. Additional findings in the male patients included anterior capsule opacity (25% total) and Fabry cataract (12.5%). Thinning of the retinal nerve fiber layer was observed in one man whose medical history was significant for stroke. Conjunctival and/or retinal vessel tortuosity was present in the majority of patients (62.5% and 75% of hemizygotes, respectively; 40% and 13.3% heterozygotes, respectively). Additional findings in the women included anterior capsule opacity. The majority of patients (87.5% hemizygotes, 60% heterozygotes) felt Fabry disease had an impact on their quality of life. CONCLUSIONS: All evaluated patients who had Fabry disease had corneal verticillata, which generally does not affect vision and is readily recognizable by slit lamp examination. Greater than 60% showed conjunctival and/or retinal vessel tortuosity. The eye care provider can play a crucial role in the early recognition of ocular manifestations of Fabry disease and decrease both the time to accurate diagnosis and the delay in the institution of disease-modifying therapy.

Amniotic membrane grafting in the management of conjunctival vascular malformations.

PURPOSE: Vascular malformations of the conjunctiva are symptomatically distressing and surgically challenging. Because their expansive nature necessitates broad conjunctival sacrifice in some cases, epithelial reconstitution may be the best way to avoid symblepharon formation. Amniotic membrane grafts have been useful for conjunctival reconstruction after excision of squamous cell carcinoma and melanoma but have not been used after excision of extensive conjunctival vascular malformations. The authors report the use of amniotic membrane grafts in the management of large conjunctival vascular malformations. METHODS: The authors reviewed the clinical records and photographs of 4 patients with combined orbital and conjunctival vascular malformations. The operative details including amniotic membrane grafting and postoperative results were included. The average follow-up period was 9 months. RESULTS: Four patients underwent a total of 6 resections; 2 patients required reoperation. Three of these lesions were lymphatic and 1 was venous. Dehydrated amniotic membrane was initially used in 2 patients who were subsequently grafted with cryopreserved amniotic membrane. Postoperative trauma in 1 case and staged surgery in the other necessitated these second surgeries. Cryopreserved amniotic grafting was successful in all 4 patients with no signs of graft dislocation, rejection, or tumor overgrowth after an average postoperative period of 9 months. CONCLUSIONS: Large conjunctival vascular malformations, when extensively resected, leave large, denuded epibulbar and palpebral surfaces that would likely produce severe symblepharon. Conversely, incomplete resection can lead to early recurrent growth. Commercially available amniotic membrane grafts now provide a method for reconstructing these defects, allowing more extensive resection of the tumor.

OCT and ultrasound biomicroscopic findings in iris arteriovenous malformation.

Iris arteriovenous malformation, also labeled as iris racemose hemangioma, is a rare condition in which abnormal vessels are present on the iris surface. Slit-lamp findings in addition to anterior segment fluorescein angiographic features of such lesions have been described in the past. The authors report anterior segment optical coherence tomography and ultrasound biomicroscopic features in two patients with iris arteriovenous malformation.

Retinal angiomatous proliferation with a cilioretinal artery anastomosis: an unusual presentation.

PURPOSE: To report an unusual phenotype of retinal angiomatous proliferation (RAP) in age-related macular degeneration (AMD) and its short-term response to laser photocoagulation. METHODS: Case report. RESULTS: An 85-year-old woman was found to have an unusual RAP with a major feeder vessel originating from a cilioretinal artery and associated with a cilioretinal-retinal anastomosis (C-RRA). Diffuse cystoid macular edema (CME), intraretinal hard exudates in a circinate pattern, and a fibrovascular pigment epithelial detachment (PED) were present. Laser photocoagulation was performed and led to occlusion of the cilioretinal feeder vessel and angiomatous lesion, with less CME. The other (retinal) arm of the C-RRA became more engorged and a new cilioretinal feeder developed, and both were associated with intraretinal hemorrhages. Visual acuity was stable and the PED persisted throughout follow-up. CONCLUSIONS: We report an unusual phenotype of RAP that is associated with a major cilioretinal feeder vessel and comment on the possible effect of such an association on the response to laser treatment.

[Isolated and combined occlusion of the cilioretinal artery and the central retinal vein]. / Izolovaná a kombinovaná oklúzia cilioretinálnej artérie a centrálnej vény sietnice.

Cilioretinal artery occlusion is rare pathological condition because of infrequent occurrence of the cilioretinal artery in the human retina. It presents as an isolated entity, or as a combined cilioretinal artery--central retinal vascular, mostly venous occlusion. Authors present the course, angiographic features, prognosis, risk factors and final outcome of the isolated cilioretinal artery occlusion and combined cilioretinal artery--central retinal vein occlusion. Occlusion of the cilioretinal artery is believed to result from obstruction of the central retinal vein, as a primary process, in the case of combined cilioretinal artery--central retinal vein occlusion.

Decompensation of a congenital retinal macrovessel with arteriovenous communications induced by repetitive rollercoaster rides.

PURPOSE: To describe a congenital retinal venous macrovessel that communicates with a cilioretinal artery and a retinal artery, and to report how this vascular anomaly decompensated as a result of repetitive rollercoaster rides. METHODS: Case report with serial fundus photography and fluorescein angiography. RESULTS: After a short period of intensive rollercoaster rides, a 19-year-old woman complained of reduced vision in one eye. Funduscopy and fluorescein angiography revealed a venous congenital retinal macrovessel with arteriovenous communications, and retinal exudation was visible at the termination of the anomalous vessel. Exudation resolved, and acuity recovered after a period of avoidance of rollercoaster rides. CONCLUSION: This case represents the first report of a retinal artery and a cilioretinal artery communicating with a congenital retinal macrovessel, and it suggests that such patients are at increased risk of retinal vascular decompensation if involved in activities associated with changes in g-forces, such as bungee jumping or rollercoaster rides.

Optical coherence tomography (OCT) in idiopathic polypoidal choroidal vasculopathy (IPCV).

PURPOSE: Idiopathic polypoidal choroidal vasculopathy (IPCV) is a distinct clinical entity characterized by an edematous maculopathy and typical choroidal vascular change. The purpose of this study is to evaluate the use of optical coherence tomography (OCT) for diagnosing of this disease. METHODS: 4 patients affected by IPVC (mean age 71-84 years) underwent biomicroscopy with a three mirror conctact lens, fluorescein angiography (FA), indocyanine green angiography (ICGA) and OCT (Humphrey Zeiss) scan. RESULTS: in all 4 cases OCT tomograms acquired at the location of the typical choroidal abnormalities demonstrated by ICGA, revealed a characteristic hyper-reflectivity in the choroidal layers. CONCLUSION: ICGA is essential to diagnose IPVC, but OCT may be able to identify characteristic reflectivity patterns.