Infrared images help to identify retinal emboli in hemodynamic retinal artery occlusion.

To report a case of stroke with internal carotid artery (ICA) occlusion and retinal artery occlusion (RAO) with multiple emboli identified by infrared images. The patient presented with acute blurred vision and slurred speech since woke up in the morning. Carotid Doppler ultrasonography showed severe left ICA stenosis with the decreased flow. Computed tomography angiography demonstrated left ICA critical stenosis with compensatory flows from the right ICA. There was coexistent RAO with multiple emboli, silver-wiring, and segmentation of the retinal artery. More hyperreflective emboli were uncovered by infrared images of spectral-domain optical coherence tomography. Embolism may accompany with the compensatory flow for ICA occlusion. RAO patients should have thorough carotid evaluations, especially those with multiple retinal emboli.

Bilateral retinal artery occlusions with carotid artery occlusions-ocular and cerebral hemodynamic changes.

PURPOSE: To introduce a case of bilateral retinal artery occlusions with carotid occlusions to achieve a fuller understanding of hemodynamic flow changes and the origin of emboli. OBSERVATIONS: A 58-year-old male presented with binocular vision loss. Fundus examination revealed bilateral retinal whitening with multiple emboli. Cherry red spot was surrounded and shaped by white edematous ischemic retina. FAG showed retinal refilling but not to the macula and choroidal background. Carotid arteriography and color Doppler images demonstrated bilateral carotid occlusions. CT angiography showed compensatory flows perfused from vertebral arteries for the brain. Orbital color Doppler images revealed bilateral reversed ophthalmic flows indicating another compensatory flow arising from external carotid/ophthalmic collaterals to both eyes. CONCLUSIONS AND IMPORTANCE: Reversed ophthalmic flow indicates the presence of external carotid/ophthalmic collaterals as the source of ocular blood supply and the origin of emboli. In this case, all the embolic, hemodynamic, and serotonin mechanisms may be responsible for the pathogenesis. RAO with multiple emboli is an important sign warning critical flow changes of carotid occlusions.

Retinal arterial occlusion with multiple retinal emboli and carotid artery occlusion disease. Haemodynamic changes and pathways of embolism.

OBJECTIVE: To introduce a special subgroup, retinal artery occlusion (RAO) with multiple emboli, which is highly associated with ipsilateral carotid artery occlusion disease (CAOD). METHODS AND ANALYSIS: This is a cohort study. Cases of RAO with multiple retinal emboli were consecutively enrolled. All patients underwent at least one of the carotid/cerebral evaluations: carotid arteriography, orbital/carotid colour Doppler ultrasonography and CT angiography to demonstrate haemodynamic changes and to discuss possible mechanisms and pathways of the emboli. RESULTS: Among 208 RAO eyes, 12 eyes (5.7%) in 11 patients had multiple emboli were recruited in this study. Eleven eyes (91.6%) had ipsilateral carotid plaques and atherosclerosis with high-grade stenosis; among them, five were total carotid occlusion. Haemodynamic changes were found in nine patients with RAO (81.8%) with carotid stenosis 60% or greater. Most compensatory intracranial circulations were re-established via the circle of Willi with antegrade ophthalmic flows, but the direction of ophthalmic flow reversed in three eyes indicating the recruitment of external collaterals. Two cases underwent carotid stent successfully. CONCLUSION: RAOs with multiple emboli are rare but highly associated with severe CAOD with haemodynamic flow changes, warning critical condition in carotid/cerebral circulations. Either direct embolism from the carotid or cardiac lesions or indirect embolism via the collateral pathways is the mechanism of pathogenesis. Immediate action should start to manage these patients to prevent further deterioration.

Peripheral Retinal Vascular Patterns in Patients with Rhegmatogenous Retinal Detachment in Taiwan.

This is an observational study of fluorescein angiography (FA) in consecutive patients with rhegmatogenous retinal detachment (RRD) in Changhua Christian Hospital to investigate the peripheral retinal vascular patterns in those patients. All patients had their age, sex, axial length (AXL), and refraction status (RF) recorded. According to the findings in FA of the peripheral retina, the eyes were divided into 4 groups: in group 1, there was a ramified pattern of peripheral retinal vasculature with gradual tapering; in group 2, there was an abrupt ending of peripheral vasculature with peripheral non-perfusion; in group 3, there was a curving route of peripheral vasculature forming vascular arcades or anastomosis; and in group 4, the same as in group 3, but with one or more wedge-shaped avascular notches. Comparisons of age, sex, AXL, and RF, association of breaks with lattice degeneration and retinal non-perfusion, surgical procedures utilized, and mean numbers of operations were made among the four groups. Of the 73 eyes studied, there were 13 eyes (17.8%) in group 1, 3 eyes (4.1%) in group 2, 40 eyes (54.8%) in group 3 and 17 eyes (23.3%) in group 4. Significant differences in age, AXL and RF, and association of retinal breaks to non-perfusion were noted among the four groups. Patients in group 1 had older ages, while younger ages were noted in groups 3 and 4. Eyes in group 1 had the shortest average AXL and were least myopic in contrast to the eyes in groups 3 and 4. Association of retinal breaks and retinal non-perfusion was significantly higher in groups 2, 3 and 4 than in group 1. In conclusion, peripheral vascular anomalies are common in cases with RRD. Patients with peripheral non-perfusion tend to be younger, with longer axial length and have the breaks associated with retinal non-perfusion.

Progressive outer retinal necrosis associated with occlusive vasculitis in acquired immunodeficiency syndrome.

A 45-year-old man, a case of acquired immunodeficiency syndrome, received a highly active antiretroviral therapy at the outpatient service for 4 years without regular follow-up. He experienced progressively blurred vision for 6 months and a cutaneous zoster on his back 3 months ago. He was diagnosed with progressive outer retinal necrosis by polymerase chain reaction-restriction fragment length polymorphism using an aqueous humor sample, which revealed an existence of varicella zoster virus. He was given a combination of systemic, intravitreal antiviral and a highly active antiretroviral therapy. Occlusive vasculitis, an unusual finding for progressive outer retinal necrosis, developed in both eyes 1 week after the secondary intravitreal injection. Unfortunately, his vision deteriorated to no light perception in both eyes within 2 weeks. Progressive outer retinal necrosis is characterized clinically as showing minimal or no inflammation in the aqueous and vitreous humors, absence of retinal vasculitis, and patches of yellowish spots located deep in the retina. Physicians should pay attention to this rare case of progressive outer retinal necrosis associated occlusive vasculitis with very poor prognosis in spite of aggressive treatment.

Multilayered optic disc hemorrhages in adolescents.

PURPOSE: To describe the clinical characteristics of multilayered optic disc hemorrhages, which are defined as subretinal, superficial retinal, and subhyaloid or vitreous hemorrhages in adolescents. METHODS: Case records of adolescents with acute multilayered optic disc hemorrhages were identified and evaluated retrospectively from 1994 to 2012. The appearance of the hemorrhages including the size, location, extension, and disc anatomy was recorded. Fluorescein angiography, visual field examination, and spectral-domain optical coherence tomography were performed in select cases. RESULTS: Nine boys and 7 girls were included, with a mean age of 15.0 ± 2.6 years. No precipitating factor, such as involuntary Valsalva maneuver, was identified in the majority of patients (93.75%). All eyes were myopic with an average refraction of -4.64 ± 1.88 diopters. Fifteen (93.75%) of the affected optic discs were crowded and tilted with small cups. Peripapillary subretinal hemorrhages were all crescent in shape and located at the nasal disc. Eight (50%) eyes had marked subretinal blood extension exceeding 1 disc diameter away from the disc edge. Superficial flame hemorrhages were predominantly located in the superotemporal part of the disc. Spectral-domain optical coherence tomography of the disc showed vitreopapillary traction and obvious subretinal hemorrhage with increased thickness. All multilayered optic disc hemorrhages resolved spontaneously. CONCLUSIONS: Multilayered optic disc hemorrhages in adolescents more commonly affect myopic eyes with crowded and tilted discs. The visual outcome is excellent.

Clinical manifestations of central retinal artery occlusion in eyes of proliferative diabetic retinopathy with previous vitrectomy and panretinal photocoagulation.

PURPOSE: To report the clinical characteristics of central retinal artery occlusion in eyes of proliferative diabetic retinopathy with previous vitrectomy and panretinal photocoagulation. METHODS: Retrospective case series. RESULTS: Twelve eyes in 12 patients (4 women and 8 men) with a mean age of 55.3 ± 6.2 years of age were included in this study. All patients had successful previous surgery for complications of proliferative diabetic retinopathy and complete panretinal photocoagulation performed. All patients had sudden visual deterioration and fluorescein angiography confirmed central retinal artery occlusion. Prominent cherry-red spot was noted in only two eyes. The other eyes either had less prominent or incomplete, or invisible cherry-red spots. Sequential optical coherence tomography in 7 of the 12 eyes showed increased optical reflectivity at inner retinal layer in 6 of the 7 eyes. Rubeosis iridis developed later in six eyes and neovascular glaucoma in four eyes. Vision worsening was noted in five eyes, improved in three eyes, and stable in four eyes after an averaged follow-up of 4 months. CONCLUSION: Less typical cherry-red spots, higher possibility of rubeosis despite previous complete panretinal photocoagulation, are the characteristic features of central retinal artery occlusion in patients with proliferative diabetic retinopathy with previous vitrectomy.

Ocular coherence tomographic and clinical characteristics in patients of punctuate inner choroidopathy associated with zonal outer retinopathy.

PURPOSE: To describe the findings of optical coherence tomography and clinical characteristics in patients of zonal outer retinopathy associated with punctuate inner choroidopathy. METHOD: Review of consecutive cases on fundus photographs, spectral domain ocular coherence tomography, fluorescein angiography, indocyanine green angiography, visual field, and electrophysiological studies of patients with punctate inner choroidopathy and associated zonal outer retinopathy. RESULTS: This study involves 4 patients suffering visual field defect far beyond the area corresponding to punctate inner choroidopathy lesions. Findings in optical coherence tomography include attenuated signals of photoreceptor inner/outer segment areas corresponding to visual field defect, and increased choroidal thickness. After treatment with immunosuppressive agents, improvements are noted in all 4 patients. CONCLUSION: Optical coherence tomography is helpful in the diagnosis of patients suffering zonal ocular outer retinopathy associated with punctate inner choroidopathy. All those patients responded well to immunosuppressive agents.

Nonparaneoplastic autoimmune retinopathy presenting with peripheral retinal vasoocclusion: case report.

PURPOSE: To report a case of nonparaneoplastic autoimmune retinopathy presenting with severe peripheral retinal vasoocclusion. METHODS: Interventional case report. CASE REPORT: A 44-year-old woman complained of blurred vision and photopsia in both eyes. She has no history of cancer or autoimmune disease. Best-corrected visual acuity was 20/40 in the left eye and 20/30 in the right eye. Constriction of the visual field sparing central vision was found. Fluorescein angiography revealed severe peripheral retinal vasoocclusion, panretinal degeneration, and cystoid macular edema. Optical coherence tomography demonstrated intraretinal fluid. Electroretinography showed decreased amplitude of a- and b-waves in both scotopic and photopic tests. The implicit time was generally within normal limits. The amplitude of flicker response was also diminished. Multifocal electroretinography showed declined signal all over the macular region. Systemic workup including whole body positron emission tomography/computed tomography for cancer screening showed negative results. Only antirecoverin antibody was positive. Under the impression of nonparaneoplastic autoimmune retinopathy, systemic and local immunosuppressive therapy was given and showed improvement. CONCLUSION: Nonparaneoplastic autoimmune retinopathies are uncommon retinal degenerations with visual loss associated with unique clinical symptoms and findings and with serum antiretinal autoantibodies. Severe peripheral retinal vasoocclusion was not reported. Early detection and timely treatment with immunosuppression could result in clinical improvement.

Choroidal infarction after photodynamic therapy combined with bevacizumab and triamcinolone reversed by tissue plasminogen activator: case report.

PURPOSE: To present a case of choroidal infarction after combined therapy of photodynamic therapy, intravitreal bevacizumab, and sub-Tenon triamcinolone injection salvaged by tissue plasminogen activator administration. METHODS: Interventional case report. RESULTS: Combined therapy of full-fluence photodynamic therapy, intravitreal bevacizumab, and sub-Tenon triamcinolone injection was given on the same day to treat neovascular age-related macular degeneration in an 82-year-old patient. Subsequent acute visual loss and marked macular edema as a result of choroidal infarction were noted. Intravitreal and sub-Tenon administration of tissue plasminogen activator were performed 9 days later to salvage the choroidal infarction, and visual improvement was noted. Fundoscopy and optical coherent tomography showed almost complete resolution of macular edema. Indocyanine green angiography revealed reperfusion of the choroidal vessels. CONCLUSION: Choroidal infarction may happen after combined therapy. Tissue plasminogen activator administration may reverse the infarction and help visual recovery.

Combination treatment of pediatric coats' disease: a bicenter study in Taiwan.

PURPOSE: To present the clinical outcome of different combination treatment modalities in pediatric Coats' disease in two Taiwan medical centers. METHODS: A retrospective review of clinical records was done of pediatric patients with Coats' disease treated at National Taiwan University Hospital and Changhua Christian Hospital. Data regarding the age at the time of diagnosis, initial presentation, methods of treatment, visual and anatomic results, and complications were recorded. Changes in vision and retinal status with the different methods of treatment were specifically evaluated. RESULTS: From 2005 through 2011, 10 eyes of 9 patients were treated under the diagnosis of Coats' disease. The clinical manifestations varied from localized vascular abnormalities with subretinal fluid and hard exudates to extensive detachment with massive exudates and retinal hemorrhage. The main treatment modalities include argon laser photocoagulation, micropulse laser, and cryotherapy. The adjunctive therapies included intravitreal triamcinolone, bevacizumab, and ranibizumab. The mean follow-up was 40.50 ± 20.52 months (range: 14 to 72 months). Best corrected visual acuity at last follow-up was light perception to 1.0 (20/20 Snellen). Anatomic improvement was achieved in 9 eyes (90%). Visual improvement was noted in 7 eyes (70%), visual stabilization in 2 eyes (20%), and visual deterioration in 1 eye (10%). Vitreous fibrosis evolving into tractional retinal detachment occurred in 1 patient receiving cryotherapy combined with intravitreal bevacizumab injections. No enucleation was ultimately necessary. CONCLUSIONS: Pediatric Coats' disease varies greatly in severity. Carefully selected treatment modalities can improve most eyes with different conditions. Intravitreal anti-vascular endothelial growth factor agents may act as useful adjuncts to improve anatomic and functional outcome. Cryotherapy combined with the intravitreal bevacizumab injection in severe cases of exudative retinal detachment may carry the risk of vitreoretinal traction and tractional retinal detachment.

Unusual optical coherence tomography and fundus autofluorescence findings of eclipse retinopathy.

A 63-year-old female patient complained of dimness in the central field of vision in the left eye after viewing an annular partial eclipse without adequate eye protection on 22 July 2009. Fundoscopy showed a wrinkled macular surface. Fundus autofluorescence study revealed well-demarcated hyperautofluorescence at the fovea. Optical coherence tomography demonstrated tiny intraretinal cysts. Fluorescein angiography and indocyanine green angiography were unremarkable. Epimacular membrane developed in the following month with deteriorated vision. Vitrectomy, epiretinal membrane and internal limiting membrane peeling were performed. Vision was restored to 20/20 after the operation. Direct sun-gazing may damage the retinal structures resulting in macular inflammation and increased focal metabolism, which explains the hyperautofluorescence. It may also induce epimacular membrane. Fundus autofluorescence might represent a useful technique to detect subtle solar-induced injuries of the retina. The visual prognosis is favorable but prevention remains the mainstay of treatment. Public health education is mandatory in reducing visual morbidity.

Different refractive errors in triplets with retinopathy of prematurity treated with bevacizumab.

The authors report refractive errors possibly resulting from intravitreal bevacizumab injection. Triplet A presented with stage 3 retinopathy of prematurity, was treated with intravitreal bevacizumab, and high refractive errors were noted. Triplet B presented with stage 2 retinopathy of prematurity in the right eye and stage 3 retinopathy of prematurity in the left eye, which regressed spontaneously. Triplet C presented with stage 2 retinopathy of prematurity in the right eye and stage 3 retinopathy of prematurity in the left eye, which were treated with intravitreal bevacizumab.

Clinical characteristics and surgical management of familial exudative vitreoretinopathy-associated rhegmatogenous retinal detachment.

PURPOSE: The purpose of this study was to report the clinical characteristics and surgical outcome of familial exudative vitreoretinopathy-associated rhegmatogenous retinal detachment. METHODS: Retrospective interventional case series of patients with familial exudative vitreoretinopathy-associated rhegmatogenous retinal detachment. All cases had preoperative and postoperative clinical evaluations. Eyes were divided into three groups: no, moderate, and severe foveal dragging according to the status of fovea. Scleral buckling procedures or vitrectomy was performed to attach the retina. RESULTS: Twenty-four eyes in 22 patients were included in this study. The male to female ratio was 18:4. The average age was 16.42 ± 5.48 years. There were 14, 5, and 5 eyes in the no, moderate, and severe foveal dragging groups, respectively. Proliferative vitreoretinopathy was present in only one eye in the severe foveal dragging group. In the subgroup of eyes without foveal dragging, neither proliferative vitreoretinopathy, posterior located breaks, nor giant tears were noted. All eyes needed only scleral buckle to reattach the retina. In the subgroup with moderate foveal dragging, posterior located break was noted in one eye, which was the only eye in this group that needed vitrectomy besides scleral buckle to attach the retina. In eyes with severe foveal dragging, Stage D proliferative vitreoretinopathy was noted in one eye and posterior located break in one eye. Encircling buckle and vitrectomy were necessary in all eyes. The average number of operation is 1.1, 1.2, and 2.4 for each of the three groups, respectively. Final visual acuity improved in 23 of 24 eyes. Final retinal attachment was obtained in 95.8% of eyes (23 of 24). CONCLUSION: Male predominance and juvenile onset are the main characteristics in familial exudative vitreoretinopathy-associated rhegmatogenous retinal detachment. Surgical success rates are comparable to rhegmatogenous retinal detachment unassociated with familial exudative vitreoretinopathy in cases without severe foveal dragging and are worse in the group with severe foveal dragging.

Unusual maculopathy secondary to hypergammaglobulinemia in a patient with systemic lupus erythematosus.

PURPOSE: To report the unusual findings of fluorescein angiography and optical coherence tomography in a case of systemic lupus erythematosus with hypergammaglobulinemia. DESIGN: Interventional case report. RESULTS: A 59-year-old woman had systemic lupus erythematosus but had stopped taking medication for 6 months. Blurred vision was noted, and the fundoscopic findings revealed serous macular detachment. Results of optical coherence tomography demonstrated intraretinal and subretinal fluid, but fluorescein angiography showed no leakage. Her best-corrected visual acuity improved after posterior sub-Tenon triamcinolone injection. CONCLUSION: Patients with atypical macular detachment may be afflicted with a serum immunogammopathy such as multiple myeloma, Waldenström's macroglobulinemia, and benign polyclonal gammopathy. Systemic lupus erythematosus should also be ruled out.

Intravitreal ranibizumab as salvage therapy in an extremely low-birth-weight infant with rush type retinopathy of prematurity.

We report the effects of intravitreal ranibizumab as salvage therapy in an extremely low-birth-weight (ELBW) infant with rush type retinopathy of prematurity (ROP). This case was a girl of 23 weeks gestational age weighing 480 g at birth. At a postconceptual age of 33 weeks, she presented with zone 1, stage 3 ROP with plus disease. Despite intravitreal bevazucimab and laser photocoagulation, extraretinal fibrovascular proliferation persisted. Intravitreal 0.25 mg (0.025 ml) ranibizumab was injected OU. After treatment, extraretinal fibrovascular proliferation disappeared. Fundus examination showed flat retinas and normal vasculature in both eyes. She has been followed up for 2 years. Intravitreal ranibizumab injection seems effective and well tolerated as salvage therapy in an ELBW infant with rush type ROP. No short-term ocular or systemic side effects were identified. More cases and longer follow-up are mandatory.

Reactive macular edema and acute visual loss after photodynamic therapy on the same day of fluorescein angiography.

Reactive macular edema can occur following photodynamic therapy (PDT) in patients who have undergone fluorescein angiography (FA) on the same day. It might be better not to perform PDT on the same day of FA to avoid side effects. The safety interval between FA and PDT should be at least 24 hours, considering fluorescein retention in the body.

Macular thickness measurements in central retinal artery occlusion by optical coherence tomography.

PURPOSE: To evaluate the structural features of central retinal artery occlusion by means of optical coherence tomography. METHODS: Eleven consecutive patients with acute central retinal artery occlusion and at least 3-month follow-up were enrolled prospectively in this study. The main outcome measures included best-corrected visual acuity, foveal thickness (FT), and total macular volume by optical coherence tomography. RESULTS: Five male and six female patients with central retinal artery occlusion were examined. The mean age was 71.2 ± 8.7 years (range, 59-83 years). Mean FT and total macular volume were 195 ± 26 µm and 6.41 ± 0.33 mm, respectively, in normal fellow eyes, increased to 299 ± 76 µm and 7.90 ± 0.93 mm, respectively, at initial examination and declined to 167 ± 30 µm and 4.85 ± 0.68 mm, respectively, at 3-month follow-up. Mean reduction in FT was 181 ± 61 µm (range, 120-270 µm) in eyes with poor visual outcome and 43 ± 25 µm (range, 15-74 µm) in eyes with fair visual outcome. Statistical analysis revealed that the relationships among visual prognosis and initial FT and initial total macular volume were not significant. CONCLUSION: Optical coherence tomography provides special information about central retinal artery occlusion. There were marked and variable changes in FT and total macular volume. Limited reduction in macular thickness might indicate a fair visual outcome.

Influence of systemic steroids on subretinal fluid after scleral buckle surgery for macula-off retinal detachment.

PURPOSE: To investigate the effect of postoperative systemic steroid therapy on the incidence and duration of the subretinal fluid (SRF) and its correlation with the visual outcome after scleral buckle surgery for macula-off rhegmatogenous retinal detachment. METHODS: Prospective interventional study of sixty patients with macula-off rhegmatogenous retinal detachment who undertook scleral buckle surgery. Those patients who had recurrent retinal detachment, proliferative preretinal membrane, or primary surgical failure were excluded. Patients were assigned to Group 1 (with prescription of oral prednisolone for 3 days postoperatively) and Group 2 (with no such prescription).Furthermore, patients in Group 1 were subdivided into Group 1A (0.5 mg prednisolone/kg)and Group 1B (1 mg prednisolone/kg) according to the dosage of prednisolone. Patients were followed-up initially 1 week and 2 weeks after surgery and then on a monthly basis for at least 1 year. After the operation, all patients had corrected visual acuity and also received examinations of indirect ophthalmoscopy and optical coherence tomography. We compared the incidence, duration of SRF, and the correlation with the visual outcome between the groups. RESULTS: Lower incidence of SRF was noted in Group 1 than in Group 2 (56.7% vs. 80%;P = 0.052), and the duration of SRF was significantly shorter in Group 1 than in Group 2 (218 days vs. 286 days; P = 0.039). There was no significant difference in the final BCVA between the two groups. However, patients in Group 1 had greater improvements of BCVA 1 year after operation (13 lines vs. 7 lines in Groups 1 and 2, respectively; P = 0.026). Statistically,there were no difference between Groups 1A and 1B, in terms of the incidence of SRF,duration of SRF, final BCVA, and improvement of BCVA. CONCLUSION: Postoperative systemic steroid treatment may decrease the incidence of SRF and facilitate the absorption of SRF. Patients treated with steroids had greater improvement of BCVA.

Treatment of inferior rhegmatogenous retinal detachment by pneumatic retinopexy technique.

PURPOSE: The purpose of this study was to evaluate the effectiveness of pneumatic retinopexy as an alternative technique for repairing inferior rhegmatogenous retinal detachments. DESIGN: Retrospective, noncomparative, interventional case series. METHODS: A review on 13 patients (13 eyes) who had undergone pneumatic retinopexy as the initial procedure for primary retinal detachments with causative break(s) in the inferior one third of retina. After gas injection, all patients were instructed to maintain a lateral recumbent posture with head tilting 10 cm to 30 cm downward. RESULTS: Nine male and 4 female patients (mean age 28.1 years, ranging from 14 to 57) were included in this study. Eleven eyes (84.6%) had myopia of -3 diopters or higher. Macular detachment was found in eight eyes. Pneumatic retinopexy alone resulted in reattachment in 10 eyes (76.9%). Three other eyes needed additional scleral buckling. Final retinal reattachment was achieved in all 13 subjects. CONCLUSION: Inferior rhegmatogenous retinal detachment can be treated by pneumatic retinopexy with proper head position. More attention to the postoperative stage are required to ensure surgical success.