Ultra-widefield Fundus Imaging Research Articles

Nystagmus associated with macular dysplasia

ABSTRACT Purpose To describe Optical Coherence Tomography (OCT) findings of the macula in patients with nystagmus, mainly the relationship between spectral-domain OCT (SD-OCT) images and nystagmus in macular dysplasia. Methods In this study, 17 cases (29 eyes) with congenital macular abnormalities in patients with albinism, macular heterotopias, congenital aniridia, foveal hypoplasia, congenital macular coloboma, and congenital retinoschisis were retrospectively analyzed. Patients underwent multimodal retinal imaging examinations including ultra-widefield fundus imaging, SD-OCT, autofluorescence, and visual field. When the pit was not clearly presented, SD-OCT imaging was centered at the expected foveal center. Results In cases of oculocutaneous albinism SD-OCT showed the absence of the foveal pit and increased foveal thickness, with nystagmus. Their fundus revealed a lack of pigment in retinal pigment epithelium with visible large choroidal vessels. SD-OCT in congenital aniridia showed a planar fovea in the macula with the lack of a foveal pit and nystagmus. SD-OCT showed the absence of a foveal pit in foveal hypoplasia with nystagmus. In cases of monocular macular heterotopia, no nystagmus was found; the fellow eye had good vision and the macular morphology was usually normal. Nystagmus was not found in patients with congenital macular coloboma and congenital retinoschisis in this study. Conclusion SD-OCT plays an important role in the diagnosis and prognosis of macular dysplasia in patients with nystagmus. Absence of a normal foveal pit is an OCT-imaging characteristic of macular dysplasia associated with nystagmus.

Degenerative Peripheral Retinoschisis: Observations From Ultra-Widefield Fundus Imaging.

To describe the ultra-widefield (UWF) imaging characteristics of patients with degenerative peripheral retinoschisis (DPR) using Optomap technology. In this multicenter, retrospective, noncomparative, consecutive case series, eligible patients underwent detailed retinal examination including indirect ophthalmoscopy. UWF fundus imaging, including color fundus photography, autofluorescence, and angiography, was performed using standardized protocols and findings were recorded and reviewed and analyzed. A total of 35 patients (58 eyes) with DPR were identified who underwent 55 sessions of UWF imaging. Mean age was 65 years, and the inferotemporal quadrant was most commonly affected (74% of eyes). Of these patients, 31 underwent fluorescein angiography and 90% of these studies illustrated abnormalities in the area affected by the schisis. The most common finding was retinal vascular leakage originating from the deep capillary plexus observed in 29 eyes (93.5%). UWF imaging enables a more detailed identification of the clinical features associated with DPR and provides simple, practical, and noninvasive tools to monitor progression of disease. The breadth of retinal vascular complications identified with fluorescein angiography may suggest an important vascular component associated with the pathogenesis of this entity. [Ophthalmic Surg Lasers Imaging Retina. 2019;50:557-564.].

Retinal Vascular Abnormalities in Phakomatosis Pigmentovascularis

To describe the spectrum of retinal vascular abnormalities in patients with phakomatosis pigmentovascularis (PPV). Multicenter, retrospective, noncomparative, consecutive case series. Eligible patients underwent detailed retinal examination including indirect ophthalmoscopy. Ultra-widefield fundus imaging, including color fundus photography and angiography, was performed using standardized protocols, and findings were recorded and reviewed and analyzed. Three patients with a clinical diagnosis of PPV are presented. Evaluation of all patients (n = 6 eyes of 3 patients) with widefield fluorescein angiography showed several retinal vascular abnormalities, including peripheral retinal nonperfusion (n = 3 eyes), peripheral vascular leakage (n = 3 eyes), aberrant retinal vessels (n = 1 eyes), vascular tortuosity (n = 1 eyes), and disruption of the foveal avascular zone including fovea plana (n = 3 eyes). In addition, 2 eyes demonstrated peripheral retinal vascular straightening and leakage similar to the features of familial exudative vitreoretinopathy. One of the patients was a carrier of a somatic GNA11 R183C pathogenic variant that has been associated with PPV. Fluorescein angiography, especially with widefield capability, reveals numerous retinal vascular abnormalities in patients with PPV. Considering the association of GNA11 pathogenic variants with PPV and allied disorders, these observations may suggest a role of guanine-binding proteins (G-proteins) in retinal vascular development. Supplemental material available at www.ophthalmologyretina.org.

Panretinal Photocoagulation along the Long Ciliary Nerve in Diabetic Retinopathy

Abstract Patients with a history of pan retinal photocoagulation for proliferative diabetic retinopathy underwent ultra-widefield imaging with identification of the long ciliary nerve. This study found high prevalence of photocoagulation along the nerve despite current guidance. Proliferative diabetic retinopathy is a prevalent disease associated with significant morbidity, reduced quality of life, high costs, and significant visual loss. 1 Panretinal photocoagulation (PRP) is a widely-used technique to decrease neovascularization and bleeding and reduce severe vision loss in proliferative diabetic retinopathy (PDR). 2 , 3 Despite its uses, multiple cases have linked peripheral laser photocoagulation near the long ciliary nerve (LCN) to corneal ulceration, transient accommodative paresis and pupillary disturbances 4 , 5 , 6 . Current guidelines from the Basic and Clinical Science Course recommend that “the long ciliary nerves in the 3 o’clock and 9 o’clock meridians should be avoided” during PRP. 7 The development of ultra-widefield (UWF) fundus imaging offers a unique opportunity to visualize the long ciliary nerve. The aim of this study is to establish the frequency of inadvertent panretinal photocoagulation along the long ciliary nerve through confirmation of laser marks on widefield fundus imaging.

Phenotypic Features of Oguchi Disease and Retinitis Pigmentosa in Patients with S-Antigen Mutations: A Long-Term Follow-up Study

To present phenotypic features of 22 patients with S-antigen (SAG) mutations. Retrospective cohort study. Twenty-one Japanese patients from 16 families with a homozygous c.924delA mutation and 1 patient with a homozygous c.636delT mutation in the SAG gene. Clinical records on symptoms; best-corrected visual acuity; and Goldmann perimetry, fundus photography, fundus autofluorescence (FAF), OCT, and electroretinography results were reviewed. Best-corrected visual acuity, Goldmann perimetry results, imaging findings, and electroretinography results. Ten patients had Oguchi disease and 12 had retinitis pigmentosa (RP) with mean follow-up periods of 13.8 and 10.2 years, respectively. Retinitis pigmentosa patients were older (mean age, 56.0 years) than those with Oguchi disease (mean age, 22.1 years; P < 0.001) at the initial visit. Night blindness noted in childhood was the most common initial symptom for both Oguchi disease (80.0%) and RP (91.7%) patients. Best-corrected visual acuity in the logarithm of the minimum angle of resolution (logMAR) was well preserved in Oguchi disease patients (mean, 0.02 logMAR in both eyes) but reduced in most RP patients (mean, 1.32 logMAR [right eye] and 1.35 logMAR [left eye]). Similarly, the visual field in the retinal area was preserved in Oguchi disease patients (mean, 677 mm2 right eye and 667 mm2 left eye) and reduced in RP patients (mean, 369 mm2 right eye and 294 mm2 left eye). Fundus images revealed a characteristic golden sheen with no retinal degeneration in Oguchi disease patients, excluding 2 with macular degeneration detected by FAF, OCT, or both and 1 with mild retinal degeneration confirmed by OCT and fluorescein angiography. Pigmentary retinal degeneration most evident posteriorly was observed in RP patients, accompanied by a characteristic golden sheen in 12 of 14 patients undergoing ultra-widefield fundus imaging. OCT showed disrupted macular structure, and FAF revealed variable hypofluorescence. Electroretinography identified absent rod responses in both diseases, along with relative preservation of cone responses in Oguchi disease patients. Three patients showed progressive loss of the golden sheen based on fundus images, including 1 who demonstrated RP 26 years after the initial diagnosis of Oguchi disease. Retinitis pigmentosa with SAG mutations often shows a characteristic golden sheen surrounding posterior pigmentary retinal degeneration. Oguchi disease can show progressive degeneration in adulthood, rarely resulting in RP.

Abstract P400: Screening for Hypertension Using Retinal Vascular Calibre in Ultra-Widefield Fundus Imaging

Fundus images from the left eyes of 440 subjects aged 50-59 years enrolled in the Northern Ireland Cohort of Longitudinal Ageing were analyzed. Subjects were categorized as normotensive or hypertensive, according to thresholds on systolic/diastolic blood pressure measurement (140/90 mm Hg) averaged over two sitting measurements in a clinical setting. A fully automatic system to analyze each image used conventional and deep neural network machine learning techniques to locate retinal landmarks and detect, classify and measure retinal vessels. From this data, a measure of the arteriolar-venular ratio (AVR) in the peripheral retina was calculated. Semi-automatic analysis was also performed. Results are presented in Table 1. Subjects had mean age of 54.6 ± 2.9 years; 56.1% (247 of 440) females, with 34.3% (151 of 440) subjects categorized as hypertensive. Narrower arterioles and smaller AVR were observed in subjects with hypertension. This was also observed in fully-automated analysis, however 4% (17 of 440) subjects failed to be processed by the system. In fully-automated analysis the area under a receiver operator characteristic curve of AVR for hypertensive status was 0.69 (95% CI, 0.63 to 0.74). Table 1 - Results for semi-automated and automated analysis of retinal vessel parameters. *p&lt;0.005 Automated measurement of AVR in ultra-widefield fundus imaging was associated with hypertension. With further development, such as evaluation against diagnosis of hypertension obtained from ambulatory blood pressure monitoring clinics, this system could become a test for undiagnosed hypertension in people attending routine eye health check-ups.

Mechanistic Evaluation of Panretinal Photocoagulation Versus Aflibercept in Proliferative Diabetic Retinopathy: CLARITY Substudy.

PurposeThe purpose of this study was to study the effects of panretinal photocoagulation (PRP) and intravitreal aflibercept on retinal vessel oxygen saturations, area of retinal nonperfusion, and area of neovascularization in proliferative diabetic retinopathy.MethodsThis is a prospective randomized single center study. Forty patients with proliferative diabetic retinopathy were randomized to PRP or intravitreal aflibercept treatment for 52 weeks. Retinal oximetry and ultra-widefield angiography were performed at baseline and week 52. Ultra-widefield color fundus imaging was performed at baseline, week 12, and week 52. The outcomes were retinal arterio-venous oximetry differences (AVD), area of retinal nonperfusion, and area of neovascularization in disc areas (DA).ResultsThe AVD in the PRP group increased from 36.7% at baseline to 39.7%, whereas it decreased from 33.4% to 32.5% in the aflibercept group. The difference in AVD between groups at week 52 was 4.0% (95% confidence interval, −0.08, 8.8; P = 0.10). The baseline mean area of retinal nonperfusion of 125.1 DA and 131.2 DA in the PRP and aflibercept groups increased to 156.1 DA and 158.4 DA, respectively, at week 52 (P = 0.46). The median baseline area of neovascularization decreased from 0.98 DA to 0.68 DA in the PRP group and from 0.70 DA to 0 DA in the aflibercept group at week 12 (P = 0.019). At week 52, this measured 0.24 DA in the PRP group and 0 DA in the aflibercept group (P = 0.45).ConclusionsIntravitreal aflibercept achieved an earlier and complete regression of neovascularization in proliferative diabetic retinopathy compared with PRP. There were no significant differences in global change in intravascular oxygen saturation or areas of retinal nonperfusion between the two groups by 52 weeks.

Air tamponade in retinal detachment surgery followed by ultra-widefield fundus imaging system

To report the surgical result of pars plana vitrectomy (PPV) with air tamponade for rhegmatogenous retinal detachment (RRD) by ultra-widefield fundus imaging system. Of 25 consecutive patients (25 eyes) with fresh primary RRD and causative retinal break and vitreous traction were presented. All the patients underwent PPV with air tamponade. Visual acuity (VA) was examined postoperatively and images were captured by ultra-widefield scanning laser ophthalmoscope system (Optos). Initial reattachment was achieved in 25 cases (100%). The air volume was >60% on the postoperative day (POD) 1. The ultra-widefield images showed that the retina was reattached in all air-filled eyes postoperatively. The retinal break and laser burns in the superior were detected in 22 of 25 eyes (88%). A missed retinal hole was found under intravitreal air bubble in 1 case (4%). The air volume was range from 40% to 60% on POD 3. A double-layered image was seen in 25 of 25 eyes with intravitreal gas. Retinal breaks and laser burns around were seen in the intravitreal air. On POD 7, small bubble without effect was seen in 6 cases (24%) and bubble was completely disappeared in 4 cases (16%). Small oval bubble in the superior area was observed in 15 cases (60%). There were no missed and new retinal breaks and no retinal detachment in all cases on the POD 14 and 1mo and last follow-up. Air disappeared completely on a mean of 9.84d postoperatively. The mean final postoperative best-corrected visual acuity (BCVA) was 0.35 logMAR. Mean final postoperative BCVA improved significantly relative to mean preoperative (P<0.05). Final VA of 0.3 logMAR or better was seen in 13 eyes. PPV with air tamponade is an effective management for fresh RRD with superior retinal breaks. The ultra-widefield fundus imaging can detect postoperative retinal breaks in air-filled eyes. It would be a useful facility for follow-up after PPV with air tamponade. Facedown position and acquired visual rehabilitation may be shorten.

Cytomegalovirus retinitis in the post-cART era.

To review the epidemiology, diagnosis, and management of cytomegalovirus retinitis (CMVR) in the post-combined antiretroviral era (cART) era. Although cART has dramatically reduced CMVR incidence and morbidity in the HIV population, CMVR continues to cause significant vision loss in both HIV and non-HIV patients, especially amongst patients without immune reconstitution. Advances in imaging including ultra-widefield fundus and autofluorescence imaging, optical coherence tomography, and adaptive optics may reflect CMVR activity; however, the diagnosis remains a clinical one. There have been minimal advances in therapy, with several agents no longer available due to market concerns. Despite reduced incidence and morbidity in the post-cART HIV population, CMVR continues to cause vision loss amongst HIV and non-HIV patients. Diagnosis remains primarily clinical, and therapy centers upon immune reconstitution along with systemic and/or intravitreal antivirals. Further studies are necessary to determine whether advanced imaging can influence management, and whether novel antiviral agents or adoptive immune transfer have a role in treatment of drug-resistance CMVR.

Pathologic myopia

Pathologic myopia is the major cause of the loss of the best-corrected visual acuity (BCVA) worldwide, especially in East Asian countries. The loss of BCVA is caused by the development of myopic macula patchy, myopic traction macula patchy, and myopic optic neuropathy (or glaucoma). The development of such vision-threatening complications is caused by eye deformity, characterized by a formation of posterior staphyloma. The recent advance in ocular imaging has greatly facilitated the clarification of pathologies and pathogenesis of pathological myopia and myopia-related complications. These technologies include ultra-wide field fundus imaging, swept-source optical coherence tomography, and 3D MRI. In addition, the new treatments such as anti-VEGF therapies for myopic choroid all neovascularization have improved the outcome of the patients. Swept-source OCT showed that some of the lesions of myopic maculopathy were not simply chorioretinal atrophy but were Bruch’s membrane holes. Features of myopic traction maculopathy have been analyzed extensively by using OCT. The understanding the pathophysiology of complications of pathologic myopia is considered useful for better management of this blinding eye disease.

Assessment of vitreous haze using ultra-wide field retinal imaging.

BackgroundConventional fundus imaging has been used to assess vitreous haze (VH) in patients with uveitis. Ultra-wide field (UWF) retinal imaging that uses scanning laser technology has not been evaluated for the detection of VH. This pilot study evaluates the ability of UWF imaging in detecting VH.Patients with intermediate, posterior, or panuveitis were examined to assess the level of VH using slit-lamp biomicroscopy. Colored fundus images were acquired using a Carl Zeiss FF450 camera. The same photographer obtained fundus images of the same eyes during the same visit by Optos UWF P200Tx retinal camera. Two graders independently analyzed UWF fundus images for presence or absence of VH, without quantifying the degree of VH using any scale. The images were analyzed using the composite red plus red-free wavelengths utilized by the Optos UWF camera and by using each wavelength exclusively. These findings were compared to clinical detection of VH and detection of VH using conventional fundus photography.ResultsNinety-two eyes were included in the study. For composite UWF images, sensitivity was 0.27, specificity was 0.88, PPV was 0.31, NPV was 0.86, positive LR was 2.25, and negative LR was 0.83. For the conventional Zeiss images, sensitivity was 0.5, specificity was 0.84, PPV was 0.33, NPV was 0.91, positive LR was 3.13, and negative LR was 0.6.Agreement between the composite UWF and Zeiss techniques was substantial with k = 0.64. Inter-observer agreement for composite UWF images was also substantial with k = 0.65. Inter-observer agreement for Zeiss images was moderate with k = 0.471. Intra-observer agreement for both imaging modalities was substantial with a composite UWF k = 0.76 and Zeiss k = 0.7.ConclusionsUWF fundus imaging using scanning laser technique may be used to assess VH and employed in the management of intermediate, posterior, and panuveitis.

ULTRA-WIDEFIELD FUNDUS IMAGING: A Review of Clinical Applications and Future Trends.

To review the basic principles of ultra-widefield fundus imaging and discuss its clinical utility for a variety of retinal and choroidal disorders. A systematic review of the PubMed database was performed using the search terms Optos, optomap, panoramic, ultra-widefield, wide-angle, and ellipsoid mirror. This yielded 158 publications of which 128 were selected based on content and relevance. A total of 128 articles pertaining to ultra-widefield imaging were cited in this review. Optos ultra-widefield imaging has become an essential tool for the identification of peripheral retinal and vascular pathology. The high resolution and multimodal capabilities of this device are also providing new insights into a variety of disorders, even those that primarily involve the posterior pole. Although the presence of artifact and the need for clinical validation are significant hurdles to more widespread use, ultra-widefield is evolving to become the standard-of-care imaging modality for many diseases and is finding new clinical and research applications such as for screening and telemedicine.

Ultra widefield fundus imaging for diabetic retinopathy.

For decades, the standard method for screening and grading severity of diabetic retinal disease has relied upon a montage of photographs using normal angle fundus cameras. With the development of ultrawide field (UWF) fundus imaging, more of the retina can be imaged with fewer pictures, less dependence on photographer skill, and, often, greater ease on the patient. Recent studies have shown comparability between traditional and UWF imaging for standard grading of diabetic retinopathy. Moreover, UWF images can detect peripheral pathology not typically seen in standard photographs, which may enlighten our understanding of disease severity and suggest new indications for treatment.

PERIPHERAL VASCULAR ABNORMALITIES IN β-THALASSEMIA MAJOR DETECTED BY ULTRA WIDE-FIELD FUNDUS IMAGING.

To describe peripheral retinal vascular abnormalities in two patients with β-thalassemia major. Visual acuity testing, slit-lamp biomicroscopy, dilated fundus examination, ultra wide-field fundus photography, and fluorescein angiography were performed in two consecutive patients with β-thalassemia major. Both patients had excellent visual acuity (20/20 and 20/25). Anterior segment examination was unremarkable. There were no lenticular changes noted in either patient. Dilated fundus examination was only remarkable for peripheral neovascular changes with preretinal hemorrhage in one patient and white without pressure in the other patient. Ultra wide-field fluorescein angiography revealed peripheral ischemia, vascular remodeling, active neovascularization, and arteriovenous anastomosis at the junction of perfused and nonperfused peripheral retina. One patient had sea fan neovascularization with active vitreous hemorrhage requiring peripheral laser photocoagulation. As with patients with sickle cell disorder, patients with β-thalassemia major may require surveillance for peripheral vascular abnormalities, with treatment of these abnormalities before they result in permanent visual loss.

Ultra Wide-Field Imaging of Choroidal Metastasis Secondary to Primary Breast Cancer

Purpose: To demonstrate the ability of ultra wide-field fundus imaging to document the response to treatment of a choroidal metastatic lesion from primary breast carcinoma. Design: Observational case report. Methods: A 45 year old female with a history of metastatic breast cancer and progressive vision loss in the right eye was noted to have an elevated, minimally pigmented choroidal lesion in the superior fundus periphery associated with a serous retinal detachment involving the macula. Ultra wide-field imaging allowed for comparison of the lesion size and secondary serous retinal detachment in response to systemic chemotherapy. Results: Over a 3 month follow up period of observation while the patient received systemic chemotherapy the lesion demonstrated increased growth and increased subretinal fluid. Conclusion: Ultra wide-field fundus photography allowed for accurate documentation of growth of a peripheral choroidal metastatic lesion and associated serous retinal detachment.