THE RAP STUDY, REPORT 5: REDISCOVERING MACULAR NEOVASCULARIZATION TYPE 3: Multimodal Imaging of Fellow Eyes over 24 months.

PURPOSE: To explore the condition of fellow eyes of patients with macular neovascularization Type 3 (MNV3) and to verify whether the retinal-choroidal anastomosis (RCA) develops equally in all MNV types. METHODS: The contralateral eyes of 94 patients with MNV3, 96 patients with MNV1, and 96 patients with MNV2 were included. Multimodal imaging was performed. The MNV3 stage including the development of fibrosis and RCA over 24 months was determined. RESULTS: In the contralateral eyes of patients of the solitary (one lesion) MNV3 group, 32 eyes (42.1%) showed early/intermediate age-related macular degeneration, 25 eyes (33%) showed MNV3, and 11 eyes (14.5%) experienced fibrosis, of which 4 eyes (5.2%) had a RCA, 7 eyes (9.2%) had atrophy after resolved MNV3, and 1 eye (1.3%) developed MNV1. In the multifocal (more than one lesion) MNV3 group, 2 eyes (11.1%) showed early/intermediate age-related macular degeneration, 9 eyes (50%) showed 15 MNV3 lesions, and 4 eyes (22.2%) showed fibrosis, of which 2 eyes (11.1%) manifested with a RCA and 3 eyes (16.7%) showed atrophy after resolved MNV3. The number of eyes with a RCA accounted for 40% of all eyes with fibrosis. The count of simultaneous bilateral multifocal MNV3 was 5 (55.6%). In the MNV1 and MNV2 groups, no eye developed a RCA. The incidence of RCAs in the scarred eyes in MNV3 was significantly higher (P < 0.0001). CONCLUSION: Retinal-choroidal anastomosis is an exclusive clinical feature of MNV3. The development of the multifocal MNV3 is usually bilateral and simultaneous. The occurrence of fibrosis in MNV3 has decreased dramatically after the introduction of the antiangiogenic therapy.

Choroidal Structural Changes in Sympathetic Ophthalmia on Swept-Source Optical Coherence Tomography.

Purpose: To analyze choroidal angioarchitecture in sympathetic ophthalmia (SO) using swept-source optical coherence tomography (SS-OCT) images.Methods: Case-control study of six patients with SO. Qualitative changes and quantitative parameters, including choroidal thickness (CT) and choroidal vascularity index (CVI), were analyzed.Results: Qualitative findings in the acute phase of SO on SS-OCT included retinal serous detachment with hyperreflective septa, choroidal folds, alterations in angioarchitecture with loss of vascular lacunae, and Dalen-Fuchs nodules. There was significantly higher CT in SO (284.05 ± 24.12 µm) compared to healthy controls (229.57 ± 46.67 µm, p = 0.04) and also increased CVI in SO (62.06 ± 2.07% vs 56.79 ± 3.15%, p = 0.006).Conclusions: CVI was significantly increased in SO, representing a novel noninvasive biomarker of disease activity. SS-OCT provides a useful qualitative and quantitative parameter, which can be potentially explored in the diagnosis and monitoring of patients with SO.

Paracentral acute middle maculopathy associated with hypoperfusion of the cilioretinal artery and impending central retinal vein occlusion.

BACKGROUND: Paracentral acute middle maculopathy is defined as ischemia of the deep retinal layers. We report an unusual case of paracentral acute middle maculopathy associated with hypoperfusion of the cilioretinal artery and impending central retinal vein occlusion in a young male with no previous comorbidities. CASE REPORT: The patient was a 22-year-old male complaining about a sudden loss of vision in his right eye upon awakening. Fundus examination showed optic disk edema, and increased tortuosity of the retinal veins and a few retinal hemorrhages. Swept-source optical coherence tomography found a hyperreflective band that was more pronounced at the level of the inner nuclear layer of the retina. These findings led us to a diagnosis of paracentral acute middle maculopathy associated with hypoperfusion of the cilioretinal artery. CONCLUSION: The finding of paracentral acute middle maculopathy on optical coherence tomography demands a proper investigation of its cause, because it has an intimate association with vascular diseases and is not an isolated entity. In our case, we could not identify the etiology of the unilateral event in this young male, although dehydration due to alcohol consumption and subsequent hypotension might have played a role in this ischemic event.

Evaluation of the choroidal features in pachychoroid spectrum diseases by optical coherence tomography and optical coherence tomography angiography.

PURPOSE: To evaluate choroidal area, stroma/lumen ratio, choriocapillaris vessel density, and choriocapillaris flow area in eyes with central serous chorioretinopathy, uncomplicated pachychoroid, and pachychoroid pigment epitheliopathy using enhanced depth imaging-optical coherence tomography and optical coherence tomography angiography. MATERIALS AND METHODS: This retrospective study analyzed enhanced depth imaging-optical coherence tomography and optical coherence tomography angiography scans of 142 eyes of 92 patients with central serous chorioretinopathy, uncomplicated pachychoroid, and pachychoroid pigment epitheliopathy. The choroidal area and stroma/lumen ratio were measured by binarization of enhanced depth imaging-optical coherence tomography images. Choriocapillaris vessel density and choriocapillaris flow area were measured at the choriocapillaris level by manual segmentation of optical coherence tomography angiography scans. RESULTS: The mean stroma/lumen ratio results were 0.361, 0.345, and 0.354 in central serous chorioretinopathy, uncomplicated pachychoroid, and pachychoroid pigment epitheliopathy groups, respectively (p > 0.05). The mean whole image choriocapillaris vessel density in uncomplicated pachychoroid group was higher compared with central serous chorioretinopathy and pachychoroid pigment epitheliopathy groups (p < 0.0001). The mean foveal, parafoveal, and perifoveal choriocapillaris vessel densities were lower in central serous chorioretinopathy group than in uncomplicated pachychoroid group (p < 0.0001). The mean choriocapillaris flow area was lower in central serous chorioretinopathy group than in uncomplicated pachychoroid and pachychoroid pigment epitheliopathy groups (p < 0.0001 and p = 0.01, respectively). CONCLUSION: Our findings suggest that both choroidal vessels and stroma are equally involved in central serous chorioretinopathy, uncomplicated pachychoroid, and pachychoroid pigment epitheliopathy. The choriocapillaris segment seems to be more affected in central serous chorioretinopathy compared to uncomplicated pachychoroid and pachychoroid pigment epitheliopathy. However, the reduced optical coherence tomography angiography signal in central serous chorioretinopathy group could be due to shadowing artifact or choriocapillaris hypoperfusion and further studies with higher quality imaging tools are needed.

RAP study, report 1: novel subtype of macular neovascularisation type III, cilioretinal MNV3.

PURPOSE: To report on patients with macular neovascularisation type III (MNV3) arising from cilioretinal arteries (CRAs) (cilioretinal macular neovascularisation type III (cMNV3)). METHODS: We reviewed baseline examinations of patients with neovascular age-related macular degeneration using multimodal imaging. We determined the type and distribution of MNV lesions in each cMNV3 case, the range of distances from the fovea, existence of exudative maculopathy, intraretinal haemorrhage and other morphological characteristics. 50 consecutive eyes with usual MNV3 without CRA were included as a control group. RESULTS: 102 eyes of 102 patients were identified with MNV3 lesions. Among these, we found 12 eyes (12%) with cMNV3, 84 eyes (82%) with usual MNV3 without CRA and 6 eyes (6%) with usual MNV3 with CRA. Ten cases of cMNV3 had one lesion, and two cases had two lesions. The lesions were distributed equally between the superior and inferior halves of the macula, whereas in the nasal and temporal halves, there were 8 (57%) and 6 (43%) lesions, respectively. All cMNV3 lesions were located between 500 and 1500 µm from the central fovea except one, which was located between 1500 and 3000 µm. None of the lesions had macular neovascularisation type I (MNV1) or macular neovascularisation type II (MNV2) elsewhere in both groups. Exudative maculopathy and intraretinal haemorrhage were found in seven (88%) and five (63%) of the eight pure cMNV3 cases, respectively. CONCLUSION: cMNV3 can be solitary or multiple, isolated or accompanied with usual MNV3 lesions, but not with concurrent MNV1 or MNV2. It is frequently associated with extensive exudative maculopathy, intraretinal haemorrhage and subretinal fluid.

Glaucoma-like Parapapillary Choroidal Microvasculature Dropout in Patients with Compressive Optic Neuropathy.

PURPOSE: To characterize peripapillary choroidal microvasculature dropout (MvD) in patients with compressive optic neuropathy (CON) as compared with those with open-angle glaucoma (OAG) using OCT angiography (OCTA). DESIGN: Cross-sectional, observational study. PARTICIPANTS: Eighty-eight eyes of 44 patients with CON; 88 eyes of 88 patients with OAG matched by age, spherical error, and OCT-determined retinal nerve fiber layer thickness (RNFLT); and 88 eyes of 44 control participants matched by age and spherical error. METHODS: Peripapillary microvasculature was evaluated, and peripapillary vessel density was measured in en face images segmented into inner-retinal and choroidal layers using swept-source OCTA. An MvD was defined as a focal sectoral capillary dropout with no visible microvascular network in the choroidal layer. MAIN OUTCOME MEASURES: Comparative characteristics of MvD in eyes with CON and OAG. RESULTS: Microvasculature dropout was observed in 30 eyes (34.1%) of 22 patients (50.0%) with CON, and in 48 eyes of 48 patients (54.5%) with OAG (P = 0.011). All MvDs in the CON group were located in the temporal parapapillary sector, whereas MvDs in the OAG group were located in the temporal-inferior (n = 36) and temporal-superior (n = 4) sectors. At their locations, MvDs in the CON group were accompanied by significant reductions in retinal vessel density and RNFLT, but this was not observed in the OAG group. The presence of MvD was associated significantly with female gender (P = 0.020) and thinner global retinal nerve fiber layer (P = 0.006) in the CON group, but not in the OAG group. CONCLUSIONS: OCT angiography of the peripapillary area showed retinal and choroidal microvasculature impairment in patients with both CON and OAG. However, the features and associated characteristics of MvD differed between these groups, suggesting that the pathogenesis of peripapillary microvascular impairment may be diverse.

Cilioretinal Arteries and Macular Vasculature in Highly Myopic Eyes: An OCT Angiography-Based Study.

PURPOSE: To determine the association between the presence of cilioretinal arteries and the macular vasculature in highly myopic eyes using OCT angiography (OCTA). DESIGN: Retrospective, observational case series. PARTICIPANTS: Four hundred eighty-one highly myopic eyes of 481 patients. METHODS: Fundus photographs were reviewed to determine the presence of a cilioretinal artery and its distribution, based on whether its path or visible branches reached the region within 500 µm of the foveal center. The macular vasculature was analyzed in OCTA images, including the vessel density (VD), fractal dimension (FD), and foveal avascular zone (FAZ). The associations between the presence of a cilioretinal artery and its distribution and between the macular vasculature and visual acuity were evaluated. MAIN OUTCOME MEASURES: Cilioretinal arteries, macular vasculature, and their associations. RESULTS: Of the eyes included, 17.05% (82/481) had a cilioretinal artery. Based on the OCTA analysis, the eyes with cilioretinal arteries showed significantly higher VD and FD in both superficial and deep capillary plexuses and smaller FAZ than those without (all P < 0.001). However, these differences were not found in the subgroup of eyes with an axial length of more than 30 mm. Eyes with cilioretinal arteries that reached the central foveal area showed significantly higher VD and FD in both capillary plexuses and smaller FAZ than those that did not (all P < 0.05). Better best-corrected visual acuity was identified in the eyes with cilioretinal arteries than in those without (0.09 ± 0.14 logarithm of the minimum angle of resolution [logMAR] vs. 0.21 ± 0.27 logMAR, respectively; P < 0.001). In particular, eyes with cilioretinal arteries that reached the central foveal area had better visual acuity than those without (0.05 ± 0.06 logMAR vs. 0.16 ± 0.20logMAR, respectively; P = 0.005). CONCLUSIONS: This OCTA-based study suggested that cilioretinal arteries in highly myopic eyes potentially may improve the macular vasculature and influence visual function.

Choroidal Microvasculature Dropout is Associated with Generalized Choroidal Vessel Loss within the ß-Parapapillary Atrophy in Glaucoma.

PURPOSE: To determine whether eyes with open-angle glaucoma (OAG) and a localized choroidal microvasculature dropout (MvD) are associated with a greater degree of generalized choroidal vascular insufficiency within the ß-parapapillary atrophy (ß-PPA) than OAG eyes without MvD. DESIGN: Retrospective cross-sectional study. METHODS: This study included 100 OAG eyes with visual field (VF) loss confined to a single hemifield (50 with and 50 without MvD, matched for age [≤10 years ols], axial length [≤1 mm], and VF severity [≤1 dB]), as well as 50 healthy eyes. Using optical coherence tomography angiography, parapapillary choroidal vessel density (pCVD) was measured on en-face images of choroidal maps within the entire ß-PPA after excluding the MvD area and hemi-sectors of the ß-PPA. pCVDs were compared among the 3 groups. The relationships between pCVD outcomes and various clinical variables were assessed. Logistical regression analyses were performed to determine the clinical factors associated with the presence of MvD in eyes with OAG. RESULTS: pCVDs corresponding to the VF-intact hemi-sectors and the entire ß-PPA, excluding the MvD area, were significantly lower in eyes with MvD than in matched sectors of eyes without MvD. Multivariate linear regression analysis showed that the presence of MvD and greater MvD angular extent were independently associated with lower global pCVD in OAG eyes (all P < .05). Logistic regression analyses showed that lower pCVD was the only factor significantly associated with the presence of MvD. CONCLUSIONS: Localized MvD was a strong predictor of generalized pCVD loss within the ß-PPA in OAG eyes.

Choriocapillaris dropout in early age-related macular degeneration.

Loss of choriocapillaris (CC) in advanced age-related macular degeneration (AMD) is well documented but changes in early AMD have not been quantified. Postmortem eyes from donors with clinically documented early AMD were examined in choroidal whole mounts to determine the area, pattern, and severity of CC loss. Choroids from postmortem human eyes without AMD (n = 7; mean age = 86.1) and from eyes with a Grade 2 clinical classification of early AMD (n = 7; mean age = 87) were immunolabeled with Ulex europaeus agglutinin (UEA) lectin-FITC to stain blood vessels. Whole mounts were imaged using confocal microscopy and image analysis was performed to determine the area of vascular changes and density of vasculature (percent vascular area, %VA). All areas evaluated had a complete RPE monolayer upon gross examination. In age-matched control eyes, the CC had broad lumens and a homogenous pattern of freely interconnecting capillaries. The mean %VA ± standard deviation in submacula of control subjects was 78.1 ± 3.25%. In eyes with early AMD, there was a significant decrease in mean %VA to 60.1 ± 10.4% (p < 0.0001). The paramacular %VA was not significantly different in eyes with or without AMD. The area of submacular choroid affected by CC dropout was 0.04 ± 0.09 mm2 in control eyes. In eyes with early AMD, the mean area affected by CC dropout was significantly increased (10.4 ± 6.1 mm2; p < 0.001). In some cases, incipient neovascular buds were observed at the border of regions with CC dropout in early AMD choroids. In conclusion, UEA lectin-labeled choroidal whole mounts from donors with clinically documented early AMD has provided a unique opportunity to examine regional changes in vascular pathology associated with choriocapillaris. The study demonstrated attenuation of submacular CC in early AMD subjects but no vascular pathology was observed outside the submacular region. While the affected area in some eyes was quite extensive histologically, these changes may not be detectable clinically using standard in vivo imaging.

Exploring choroidal angioarchitecture in health and disease using choroidal vascularity index.

The choroid is one of the most vascularized structures of the human body and plays an irreplaceable role in nourishing photoreceptors. As such, choroidal dysfunction is implicated in a multitude of ocular diseases. Studying the choroid can lead to a better understanding of disease pathogenesis, progression and discovery of novel management strategies. However, current research has produced inconsistent findings, partly due to the physical inaccessibility of the choroid and the lack of reliable biomarkers. With the advancements in optical coherence tomography technology, our group has developed a novel quantitative imaging biomarker known as the choroidal vascularity index (CVI), defined as the ratio of vascular area to the total choroidal area. CVI is a potential tool in establishing early diagnoses, monitoring disease progression and prognosticating patients. CVI has been reported in existing literature as a robust marker in numerous retinal and choroidal diseases. In this review, we will discuss the current role of CVI with reference to existing literature, and make postulations about its potential and future applications.

Angiographic features of polypoidal choroidal vasculopathy using indocyanine green angiography and optical coherence tomography angiography: A comparative study.

BACKGROUND: To compare the vascular lesion size using optical coherence tomography angiography and indocyanine green angiography in eyes with polypoidal choroidal vasculopathy. METHODS: Treatment-naïve cases (46 eyes of 44 patients) with polypoidal choroidal vasculopathy were retrospectively analyzed. The comparison of mean area of branching vascular network and polyp detection rate was done between indocyanine green angiography and optical coherence tomography angiography and correlated with various optical coherence tomography features. RESULTS: The mean age of the study patients was 62.33 ± 10.74 years. The mean branching vascular network size was 7.47 ± 5.74 and 7.51 ± 5.69 mm² in indocyanine green angiography and optical coherence tomography angiography, respectively, with an excellent correlation (r = 0.997). Optical coherence tomography angiography overestimated (mean ± SD: 0.28 ± 0.19 mm²) and underestimated branching vascular network area (0.36 ± 0.33 mm²) in 23 eyes each as compared to indocyanine green angiography. However, the difference in branching vascular network size was not statistically significant (p = 0.53). Indocyanine green angiography and optical coherence tomography angiography could identify polyps in 43 of 46 (93.48%) and 32 of 46 (69.57%) patients, respectively. CONCLUSION: Branching vascular network size measurements with indocyanine green angiography and optical coherence tomography angiography were comparable and showed significant correlation, albeit the polyp identification rate was lower with optical coherence tomography angiography. Optical coherence tomography angiography may serve as a useful substitute to indocyanine green angiography in measurements of branching vascular network for photodynamic therapy and follow-up of polypoidal choroidal vasculopathy eyes.

Isolated cilioretinal artery occlusion secondary to perinuclear antineutrophil cytoplasmic antibody vasculitis.

INTRODUCTION: Antineutrophil cytoplasm antibody-associated vasculitides encompass a diverse spectrum of autoimmune diseases characterized by necrotizing small vessel vasculitis. Ocular manifestations may be the presenting findings of antineutrophil cytoplasm antibody-associated vasculitides. METHODS: Single, retrospective case study. RESULTS: We report the rare case of a 55-year-old woman with a cilioretinal artery occlusion as the presenting feature of perinuclear antineutrophil cytoplasm antibody-associated microscopic polyangiitis. CONCLUSIONS: Although rare, antineutrophil cytoplasm antibody-related vasculitis should be considered in any retinal vascular occlusion, particularly in the setting of patients with new vague headaches and a paucity of vasculopathic risk factors.

Development of visual acuity under hyperbaric oxygen treatment (HBO) in non arteritic retinal branch artery occlusion.

PURPOSE: Nonperfusion of retinal tissue due to arterial occlusion leads inevitably to mostly irreversible retinal damage. Until today no evidence-based treatment exists. Inhalation of 100% oxygen at high atmospheric pressure causes an increased solubility of oxygen in the blood that helps the retinal tissue to survive through diffusion in case of an artery occlusion till vascular recanalization occurs. Hence the purpose of this study is to compare the visual outcome in patients with retinal branch artery obstruction treated with hyperbaric oxygen versus patients treated with hemodilution only. METHODS: Non-randomized, monocentric, retrospective study. Patients with diagnosis of non-arteritic retinal branch artery occlusion (BRAO) treated with hyperbaric oxygen therapy between 1997 and 2017. Exclusion criteria were central retinal artery occlusion, presence of a cilioretinal artery and arteritic cases. The control group was matched based on visual acuity (VA) at admission, age, and delay between symptoms and beginning of clinical care. RESULTS: The control group and the matching oxygen group contained 14 patients each. Initial VA in the matched HBO group was 0.18 ± 0.19 and 0.23 ± 0.19 in the control group (p = 0.57). Final VA at discharge was 0.69 ± 0.29 in the matched oxygen group and 0.32 ± 0.23 in the control group (p = 0.0009). HBO-treated patients had a significant visual increase compared with the control group. The most common comorbidities were arterial hypertension and vascular sclerosis. CONCLUSION: HBO treatment appears to have a beneficial effect on visual outcome in patients with retinal branch artery occlusion. HBO treatment could be a rescue therapy at an early stage of BRAO, especially to bridge the time of a potential reperfusion. However, further, prospective, randomized clinical trials are required to verify this assumption.

Evaluation of Peripapillary Choroidal Microvasculature to Detect Glaucomatous Damage in Eyes With High Myopia.

PRECIS: Parapapillary choroidal microvasculature dropout (MvD), as observed by optical coherence tomography (OCT) angiography, was useful to detect glaucomatous damage in highly myopic eyes with unreliable OCT results due to segmentation errors. PURPOSE: The purpose of this study was to evaluate the usefulness of optical coherence tomography angiography (OCTA) imaging of the peripapillary choroidal microvasculature in detecting glaucomatous damage in highly myopic eyes, in cases where evaluating the thickness of the retinal nerve fiber layer (RNFL) is unreliable due to OCT segmentation errors. MATERIALS AND METHODS: Forty-five highly myopic eyes with primary open-angle glaucoma (POAG) with an axial length >26.5 mm, and 15 age-matched and axial length-matched 15 control eyes were included in this cross-sectional observational study. All participants had a segmentation error in OCT circumpapillary RNFL scanning. The peripapillary choroidal microvasculature was evaluated on en-face images obtained using swept-source OCTA. MvD was defined as a focal sectoral capillary dropout with no visible microvascular network identified in the choroidal layer. The topographic correlation between the MvD and a hemifield visual field (VF) defect was assessed using κ statistics. The MvD size was assessed by measuring both its area and circumferential extent. RESULTS: Choroidal MvD was observed in 44 of the 45 (97.8%) POAG eyes with high myopia, while none of the control eyes showed a choroidal MvD. There was an excellent topographic relationship between the choroidal MvD and the hemifield VF defect (κ=0.863, P<0.001). The area (R=0.2619, P=0.0006) and circumferential extent (R=0.3088, P=0.0002) of the MvD have significantly associated with the VF mean deviation. CONCLUSIONS: Choroidal MvDs were observed in most of the highly myopic POAG eyes and were topographically correlated with the location of glaucomatous VF defects despite unreliable OCT RNFL thickness measurements. Using OCTA to investigate the choroidal microvasculature may facilitate diagnoses of glaucoma in highly myopic eyes.

Non-arteritic anterior ischemic optic neuropathy with Cilioretinal artery occlusion: a case report.

BACKGROUND: To describe a peculiar case of concurrent non-arteritic anterior ischemic optic neuropathy (NAION) and cilioretinal arteries occlusion (CLRAO) without other causative agents which responded well to intravenous and intravitreal injection of corticosteroids. CASE PRESENTATION: A 41-year-old woman presented with painless vision loss in the right eye for 1 week. Fundus examinations showed marked disc swelling, flame-shaped hemorrhage over the superior nerve fiber area, and well-demarcated retinal ischemia superior to the fovea in the right eye. Under the impression of NAION with branch retinal artery occlusion, the patient was treated with intravenous and intravitreal injection of corticosteroids. Two months later, as the disc swelling and retinal ischemia resolved, we found that the occluded artery was the cilioretinal artery and not the ordinary branch retinal artery. CONCLUSIONS: CLRAO may be concomitant with the setting of NAION, the physicians should be aware that CLRAO may be misinterpreted as BRAO owing to profound disc edema during the early stages of the disease.

Anterior Optic Nerve Head Perfusion is Dependent on Adjacent Parapapillary Choroidal perfusion.

Recent studies reported that parapapillary microvascular dropout (MvD) was significantly associated with glaucoma and glaucoma progression. To understand the clinical relevance/importance of MvD, it is essential to know the exact vascular anatomy of optic nerve head (ONH). Although it is known that parapapillary choroid and the deep ONH structure including prelaminar tissue are both supplied by branches of short posterior ciliary artery, it remains controversial whether parapapillary choroid provides a major contribution to the prelaminar tissue perfusion. This study investigated perfusion within and around the ONH using indocyanine green angiography. Thirty-three eyes from 33 patients with primary open-angle glaucoma and 10 eyes from 10 normal subjects were included. The temporal sequence of dye appearance in various tissues was analyzed. We also sought the microvessels directly responsible for blood supply to the prelaminar tissue. The perfusion of the prelaminar tissue, which occurred in a sectoral fashion, was dependent on the dye appearance in the adjacent parapapillary choroid. In addition, microvessels crossing over the optic disc margin from the parapapillary choroid to the ONH were found. The findings suggest that the centripetal flow from the parapapillary choroid is an important source of prelaminar tissue perfusion.

New Observations regarding the Assessment of Cilioretinal Arteries Coexistence with Optic Disc Pit.

Purpose: To investigate the coexistence of cilioretinal arteries (CRAs) with optic disc pit (ODP), and to delineate the characteristics of CRAs related to their number, location of their emergence and their association with the size of ODP. Methods: 47 patients (49 eyes) with ODP were diagnosed and followed-up between 1997 and 2017, using slit-lamp biomicroscopy, color fundus photographs, fluorescein angiography and indocyanine green angiography. The presence of CRAs was recorded in association with the size of the ODP, along with their number and location of emergence. The fellow normal eyes of patients were also analyzed. Results: 42 out of 49 eyes with ODP (85.7%) presented CRAs. In 35 out of 42 eyes (83.3%) CRAs emerged from the pit, either from bottom or from its margin. In 7.1% of cases, CRAs were emerged outside the ODP, while in 9.6% of cases, the type of CRA emergence could be characterized as mixed. The number of CRAs, that ranged from 1 to 4, was positively associated with ODP size. In the fellow normal eyes, CRAs was found in 22.2% of cases, difference which was significant compared to patients with ODP. Conclusion: Based on the high percentage of CRAs coexistence with ODP and the excessive frequency of their emergence from ODP (83.3%), it is supported that ODP as a developmental disorder could go along with further anatomic peculiarities, that also include the presence of multiple CRAs.

Evaluation of Parapapillary Choroidal Microvasculature Dropout and Progressive Retinal Nerve Fiber Layer Thinning in Patients With Glaucoma.

Importance: Parapapillary choroidal microvasculature dropout (MvD) is considered one of pathophysiological manifestations of glaucomatous damage. Objective: To evaluate the longitudinal change in the parapapillary choroidal MvD in patients with primary open-angle glaucoma (POAG), and to determine whether this change is associated with progressive retinal nerve fiber layer (RNFL) thinning. Design, Setting, and Participants: This prospective observational case series was conducted at a tertiary referral center in Korea and included 68 patients with POAG who exhibited parapapillary choroidal MvD in en face optical coherence tomography (OCT) angiography (OCTA) images who were enrolled from the ongoing Investigating Glaucoma Progression Study from January 1, 2016, through July 31, 2018. The mean (SD) follow-up period was 2.5 (0.2) years and observers were masked to the clinical characteristics of the participants for measurements. The OCTA images were obtained twice at an interval of at least 2 years, during which the RNFL thickness was measured at least 4 times in serial OCT examinations. Microvasculature dropout was defined as a focal sectoral capillary dropout with no visible microvascular network identified in the choroidal layer. The MvD area was measured in the OCTA images obtained at the baseline and the final follow-up. The significance of changes in the MvD area was defined using the 95% Bland-Altman limits of agreement. The rate of RNFL thinning was determined by linear regression of the serial OCT RNFL thickness measurements. Main Outcomes and Measures: The association between the change in the MvD area and the rate of RNFL thinning. Results: Of 68 Korean participants, 37 (54.5%) were women, and the mean (SD) age was 54.3 (13.1) years. Among 68 eyes, 22 (32.4%) showed increases in the MvD area during the follow-up. Faster global RNFL thinning was associated with a larger baseline parapapillary atrophy ß-zone (ß = -0.55; 95% CI, -0.96 to -0.14; P = .01), disc hemorrhage detection during the follow-up period (ß = -0.75; 95% CI, -1.67 to -0.34; P = .001), and a larger increase in the MvD area (ß = -4.74; 95% CI, -7.72 to -1.75; P = .002). The MvD area was not associated with the rate of RNFL thinning at baseline or the final follow-up. Conclusions and Relevance: Microvasculature dropout enlargement was associated with progressive RNFL thinning in POAG.