Alterations in retrobulbar haemodynamics in thyroid eye disease.

PURPOSE: To study the orbital perfusion parameters of ophthalmic artery (OA) and central retinal artery (CRA) in inactive TED and the changes following surgical decompression. METHODS: Non-randomised clinical trial. 24 inactive moderate-to-severe TED orbits of 24 euthyroid cases underwent surgical decompression and examined again at 3 months. The peak systolic velocity (PSV), end-diastolic velocity (EDV), and resistivity index (RI) of OA and CRA were evaluated using colour doppler imaging and normative database was established using 18 healthy controls. RESULTS: The mean age was 39.38 ± 12.56 years and male: female ratio was 1: 1.18. Intraocular pressure was higher, and CRA-PSV, CRA-RI, OA-PSV, and OA-EDV were lower in TED in comparison to heathy orbits. The CRA-PSV, CRA-EDV, OA-PSV, and OA-EDV negatively correlated with proptosis and duration of thyroid disease. The area under curve of OA-PSV (95% CI:0.964-1.000, p < 0.001) and OA-EDV (95% CI:0.699-0.905, p < 0.001) helped in differentiating TED orbits from HC, and in predicting the severity of disease. Post decompression, CRA-PSV, CRA-EDV, OA-PSV, and OA-EDV improved, with decrease in CRA-RI and OA-RI in both lipogenic and MO. CONCLUSIONS: The orbital perfusion is reduced in inactive TED. The changes in OA flow velocities can help in differentiating inactive TED from healthy orbits and progression of TED. Sequential orbital CDI of OA and CRA can serve as an objective tool for case selection and monitoring response to surgical decompression.

Visualization of Short Posterior Ciliary Artery Insertion Due to Posterior Staphyloma and Myopic Chorioretinal Atrophy.

This case report describes the visualization of a short posterior ciliary artery insertion as a result of posterior staphyloma and chorioretinal atrophy in a patient in their 50s with pathologic myopia.

Contrast Sensitivity Function: A More Sensitive Index for Assessing Protective Effects of the Cilioretinal Artery on Macular Function in High Myopia.

Purpose: To assess the benefits of the cilioretinal artery on macular function in high myopia using the quantitative contrast sensitivity function (qCSF) method. Methods: This cross-sectional study was conducted at the Eye and Ear, Nose, and Throat Hospital of Fudan University. In total, 137 highly myopic patients (with axial length [AL] ≥ 26.00 mm) were enrolled and divided into cilioretinal artery absent and present groups based on their fundus photographs. One eye in each patient was randomly selected. Choroid thickness was measured using macular optical coherence tomography. The best-corrected visual acuity (BCVA) was evaluated by Early Treatment Diabetic Retinopathy Study charts, and the area under the log CSF (AULCSF), CSF acuity, and CS at six spatial frequencies were evaluated with the qCSF method. Results: Although no significant BCVA difference was found between the cilioretinal artery absent (97 patients) and present (40 patients) groups, choroid thickness, AULCSF, CSF acuity, and CSF at low and intermediate spatial frequencies (1-6 cycles per degree) were all significantly higher in the cilioretinal artery present group than in the absent group (all P < 0.05). In addition, eyes with temporal cilioretinal arteries exhibited significantly higher AULCSF, CSF acuity, and CSFs at 3 and 6 cycles per degree (all P < 0.05) than those with a nasal one (all P < 0.05). Multivariate analysis showed that better AULCSF was associated with the presence of cilioretinal artery and the interaction of AL and choroid thickness. Conclusions: The cilioretinal artery may associate with the larger choroid thickness in highly myopic eyes and may play a role in preserving qCSF outcomes, which are more sensitive than chart-based acuity tests.

Received anti-VEGF therapy in a patient with CRAO sparing the CLRA with subretinal fluid: A case report.

RATIONALE: Central retinal artery occlusion (CRAO) sparing the cilioretinal artery (CLRA) with severe subretinal fluid and non-characteristic cherry-red spot is uncommon. In the present case, the patient received an intravitreal injection of anti- vascular endothelial growth factor, which is very rare. PATIENT CONCERNS: A 59-year-old man underwent vitrectomy of the left eye for diabetic retinopathy and vitreous hemorrhage. Six months after the operation, the patient presented with sudden painless visual-acuity decline in his left eye and was diagnosed with CRAO; his best corrected visual acuity was weak light perception. Whole retinal edema was seen on the fundus, and macular gray retinal opacification was present without a characteristic cherry-red spot. Optical coherence tomography revealed subretinal fluid (SRF) in the papillomacular bundle and inner retinal thickening. Fundus fluorescein angiography confirmed that the central retinal artery was not filled at 40 seconds and that the CLRA supplied a part of the macular area. Eight months after the second intravitreal injection of ranibizumab, Optical coherence tomography showed a significant reduction in inner retinal hyperreflectivity and the thickness of the nasal macular retina. The SRF was clearly absorbed, and the visual acuity improved to 1.1 logMAR units. DIAGNOSIS: Atypical CRAO. INTERVENTIONS: The patient was treated with intravitreal injection of anti-VEGF in his left eye. The thickness of the nasal macular retina decreased. OUTCOMES: The SRF was clearly absorbed, and the patient's visual acuity significantly improved. LESSONS: When CRAO occurs in patients with diabetic retinopathy sparing the CLRA, the non-characteristic cherry-red spot may be due to macular inner retinal edema, SRF and other factors. According to the patient's condition, anti-vascular endothelial growth factor can be administered as appropriate to inhibit choroidal neovascularization, reduce SRF in the macular retina, and save some vision.

[Subfoveal posterior ciliary arteries: Multimodal imaging and classification based on choroidal elevation]. / Artères ciliaires postérieures sous-maculaires : imagerie multimodale et classification basée sur l'importance du soulèvement choroïdien.

PURPOSE: To characterize the magnitude and consequences for interpretation of macular elevations associated with short posterior ciliary arteries (SPCA) extending to the fovea. METHODS: Retrospective study of 96 eyes of 138 patients who underwent multimodal imaging (color photographs, SD-OCT with OCT-angiography analysis, fluorescein angiography [FA] and indocyanine green angiography [ICG]) over a two-year period. The authors selected patients with a focal choroidal elevation (FCE) associated with a SPCA extending to the fovea. FCEs were classified according to their magnitude as "Low" (<50µm), "Medium" (50µm to 100µm) and "High" (>100µm). RESULTS: Ten patients aged 80.10±11.82 years had a FCE associated with a macular SPCA. The patients were followed for advanced AMD with neovascularization (2 cases), advanced AMD with geographic atrophy (3 cases), intermediate AMD (1 case) ; one patient was followed for diabetes ; three patients had no identified retinal pathology. The FCE associated with the SPCAs was Low (3 cases), Medium (2 cases), High (3 cases). In 2 cases, the FCE was limited to an unmeasurable choroidal fold. The interpretation associated with these FCEs was not always related to the magnitude of the elevation. CONCLUSION: Our observations show the value of multimodal imaging in the identification of SPCA extending to the fovea, sometimes associated with a macular FCE. In some cases, the signs associated with FCE interfered with the monitoring of the condition for which the patient was being followed. Identifying these FCEs and grading their amplitude is relevant, as they should not be confused with pathological structures.

Combined central retinal vein and cilioretinal artery occlusion in a 25-year-old woman.

We report a case of a 25-year-old woman with sudden and painless diminution in vision and central scotoma in her left eye (LE). She was a smoker and had been taking combined oral contraceptive (COC) pills for 1 year. On admission, the best-corrected visual acuity (BCVA) was 1,5/50 in the LE. Posterior segment examination revealed optic disc edema with flame-shaped retinal hemorrhages, mildly tortuous and dilated retinal veins. Moreover, retinal edema in the peripapillary and perimacular region, foci of hemorrhages and Roth's spots in the posterior pole, as well as pale superior papillomacular bundle were observed. Fundus fluorescein angiography (FFA) confirmed the delayed flow of contrast through the cilioretinal artery in the LE. The clinical picture suggested left central retinal vein (CRVO) with cilioretinal artery occlusion (CLRAO). All laboratory and imaging tests were normal except for homozygous methylenetetrahydrofolate reductase (MTHFR) gene mutation (A1298C genotypes). However, serum homocysteine (Hcy) level was normal. Low molecular weight heparin (LMWH) treatment was administered. Retinal lesions, as well as BCVA improved, but central scotoma remained. Abbreviations: aPTT = activated partial thromboplastin time, BCVA = best-corrected visual acuity, CBC = complete blood count, CLRAO = cilioretinal artery occlusion, COC = combined oral contraceptive, CRA = central retinal artery, CRP = serum C-reactive protein, CRVO = central retinal vein occlusion, CT = computed tomography, CTA = computed tomography angiography, ECG = electrocardiography, ESR = erythrocyte sedimentation rate, FERG = flash electroretinogram, FFA = fundus fluorescein angiography, GCA = ganglion cell analysis, GCL = ganglion cell layer, Hcy = homocysteine, ICGA = indocyanine green angiography, INR = international normalized ratio, IOP = intraocular pressure, IPL = inner plexiform layer, LE = left eye, LMWH = low molecular weight heparin, mfERG = multifocal electroretinogram, MTHFR = methylenetetrahydrofolate reductase, OCT = optical coherence tomography, RE = right eye, VF = visual field.

Ocular blood flow and retinal, choroidal, and retinal nerve fiber layer thickness in children with familial Mediterranean fever with at least five attacks.

PURPOSE: This study aimed to evaluate ocular blood flow and, retinal, choroidal and retinal nerve fiber layer thickness in children with familial Mediterranean fever (FMF) with patients who had at least five attacks. METHODS: Fifty-six patients with FMF and 56 healthy subjects were enrolled in the study. Each participant's choroidal thickness (CT) and retinal thickness (RT) measurements were performed in the foveola and at horizontal nasal and temporal quadrants within 500-µm intervals up to 1.500 µm from the foveola using spectral-domain optical coherence tomography as well as the retinal nerve fiber layer thickness (RNFLT). Disc boundaries were determined manually by identifying seven points on the inner edge of the scleral ring. Following complete ophthalmological evaluation, peak systolic velocity (PSV), end-diastolic velocity (EDV), and resistivity index (RI) values in the central retinal artery (CRA), ophthalmic artery (OA), and nasal posterior ciliary artery (NPCA) were measured using color Doppler ultrasonography (CDU) in the left eyes of the patients diagnosed with FMF and the controls. The results were subsequently compared. RESULTS: No statistically significant differences were observed between the groups in terms of all RNFLT quadrant and RT measurements. All CT measurements were thinner in patients with FMF than in the controls (For Central and Temporal_1500 p = 0.01; For Temporal_500, Nasal 500 and Nasal_1000 p < 0.001; Temporal_1000 p = 0.002) except than the Nasal_1500 point. For nasal_1500 p = 0.121). The PSV and EDV values in the OA, CRA, and NPCA were significantly lower in patients with FMF compared to healthy controls (p < 0.001 for all). In contrast, the RI values of all arteries were significantly higher than in the control group (Regarding OA, CRA and NPCA RI, p = 0.003, p = 0.039, and p = 0.039, respectively). CONCLUSIONS: The results of this study support the idea that chronic inflammation and frequent attacks in FMF may have an effect on decreased ocular blood flow and choroidal thinning.

Retrobulbar blood flow velocity in systemic lupus erythematosus assessed by color Doppler imaging.

OBJECTIVE: To analyze the blood flow in retrobulbar vessels in systemic lupus erythematosus (SLE) by color Doppler imaging (CDI) and to investigate the associations between immunological markers, retinal abnormalities, disease activity, and vascular parameters. METHODS: We examined 30 patients with SLE who were aged 32.1 ± 11.6 years with a disease duration of 68.0 ± 55.6 months and 30 participants in a control group. The peak systolic velocity (PSV), end diastolic velocity (EDV), pulsatility index (PI), and resistive index (RI) of the ophthalmic artery (OA), central retinal artery (CRA), and posterior ciliary arteries (PCAs) were determined. Physical and ophthalmic examinations with assessments of immunological markers were performed. All parameters were compared between SLE patients and normal controls while the correlations among parameters were analyzed. RESULTS: In the SLE group, CDI confirmed blood flow abnormalities in the CRA and PCAs, with significantly lower blood flow velocities and increased RI and PI (p < 0.05). There was a significantly negative correlation between disease duration and EDV in the CRA, PCA, and OA (p = 0.0423, 0.0453, 0.0448). There was also a significant relationship between the SLE Disease Activity Index and the PI of the OA (p = 0.0367). The patients who had received biological agents (Rituximab) had lower EDV in the CRA and PCA (p = 0.0026, 0.028). SLE patients with kidney or CNS involvement had a significant increase in the PI in the OA (p = 0.0287). The PSV and EDV in the CRA were significantly related to creatinine (p = 0.0007 and 0.0418). We also noted a significant decreased EDV in the CRA and an increased RI in the OA among participants positive for anti-dsDNA antibodies (p = 0.0331 and 0.0228). CONCLUSIONS: Retrobulbar circulatory disturbances were detected in SLE patients by CDI. These findings seem likely to affect smaller vessels like the CRA and PCA. However, the presence of measurable changes in the OA is generally indicative of a coexistent nephropathy or central nervous system (CNS) vasculitis. Creatinine, the disease activity index, being positive for anti-dsDNA, and receiving biological agents were associated with measurable changes on the retrobulbar blood flow.

Central retinal artery occlusion with cilioretinal sparing in a patient with Eisenmenger syndrome.

A young boy in his 20s presented with sudden diminution of vision in right eye 2 days ago. Best-corrected visual acuity was hand movement in right eye and 20/20 in left eye. Funduscopy demonstrated diffuse retinal opacification and swelling at the posterior pole with a cherry red spot at the macula. There was sparing of the superonasal macula with a patent cilioretinal artery. OCT showed diffuse inner retinal thickening of the temporal macula. He was diagnosed as central retinal artery occlusion with cilioretinal sparing in right eye. He was a known case of Eisenmenger syndrome secondary to a large congenital ventricular septal defect. Medical history was notable for prominent pulmonary hypertension and shortness of breath on exertion. Physical examination revealed a harsh systolic murmur. Extremities showed digital erythema and severe clubbing. Chest X-ray demonstrated cardiomegaly. Laboratory examination revealed haemoglobin-220 g/L, haematocrit-60%, serum iron-20 µg/dL.

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.

Retinal vascular morphology and blood flow characteristics of patients with various degrees of intracranial internal carotid artery stenosis.

PURPOSE: To explore the retinal vascular morphology and blood flow characteristics of patients with varying degrees of intracranial internal carotid artery (IICA) stenosis. MATERIALS AND METHODS: A total of 280 eligible patients were divided into non-stenosis (n=39), mild stenosis (n=80), moderate stenosis (n=83) and severe stenosis groups (n=49). Hemodynamic indices of the posterior ophthalmic artery (OA), central retinal artery (CRA) and posterior ciliary artery (PCA) in the dark zone of optic nerve were detected using color Doppler ultrasonography. CRA equivalent (CRAE), central retinal vein equivalent (CRVE) and arteriole-to-venule ratio (AVR) within 0.5-1.0 PD from the optic disc rim to the center of the fundus image were measured using IVAN software. The diagnostic values of PCA peak systolic velocity (PSV) and end-diastolic velocity (EDV) for severe IICA stenosis were evaluated by receiver operating characteristic (ROC) curves. RESULTS: The blood flow parameters (PSV and EDV) of the OA, CRA and PCA in the severe stenosis group were the lowest (P<0.05). The PSV and EDV of the OA, CRA and PCA had significant negative correlations with severe IICA stenosis, and PCA PSV was more sensitive in the presence of the same index. The areas under the ROC curves for PCA PSV and EDV were 0.722 and 0.761, respectively, indicating that PCA PSV and EDV had high diagnostic values. CONCLUSIONS: There is no correlation between retinal vascular morphology and IICA stenosis. Severe IICA stenosis has significant negative correlations with ocular the blood flow parameters PSV and EDV, and PCA PSV is most sensitive with the same index, thus being a potential predictive index.

MULTIMODAL IMAGING OF A PRESUMED EMBOLIC CILIORETINAL ARTERY OCCLUSION.

PURPOSE: To describe clinical features including multimodal imaging in a rare case of presumed embolic cilioretinal artery occlusion with long-term follow-up examination. METHODS: A 61-year-old man presented after noticing an acute-onset central scotoma in the left eye during coronary arteriography. Fundus color photography, red-free imaging, near-infrared reflectance, spectral domain optical coherence tomography (OCT), fluorescein angiography, and swept-source OCT angiography were performed. RESULTS: Color and red-free fundus imaging both showed retinal whitening in the territory corresponding to a cilioretinal artery in the left eye. Consistent with an ischemic event, the near-infrared reflectance showed a hyporeflective area in the same location. Spectral domain OCT showed hyperreflectivity and generalized thickening of the inner nuclear layer, ganglion cell layer, and retinal nerve fiber layer. Seven years later, color, red-free, and near-infrared reflectance imaging appeared normal, but the spectral domain OCT showed thinning of all retinal layers in the affected area. Furthermore, swept-source OCT angiography demonstrated markedly reduced capillary flow signal in the superficial and deep capillary plexuses, corresponding to a persistent scotoma described by the patient. CONCLUSION: This case report illustrates hallmark features of a rare presumed embolic cilioretinal artery occlusion and its long-term manifestations with thorough historical and multimodal imaging data. When standard fundus imaging with color, red-free, and near-infrared reflectance appears normal years after a retinal vascular insult, OCT and OCT angiography can corroborate a patient's persistent visual deficit and provide clarity to the diagnosis.