Wide-field Fundus Photography Research Articles

The Role of Widefield Optical Coherence Tomography Angiography in Assessing the Severity of Diabetic Retinopathy.

Physicians need an accurate understanding of diabetic retinopathy (DR) severity to optimally manage patients. The aim of this prospective study is to correlate the severity of macular and peripheral retinal vascular abnormalities seen on widefield (WF) optical coherence tomography angiography (OCTA) with DR grading based on WF fundus photography. The study included 150 eyes from 82 patients with treatment-naïve DR. All patients were imaged with WF fundus photography and swept-source WF OCTA. Quantitative and qualitative analyses of the foveal avascular zone (FAZ) size and shape, and measurement of capillary nonperfusion (CNP) areas, were performed from the OCTA images. The mixed-effects model was used to compare the DR grading from WF photography with the vascular changes seen on WF-OCTA, and Bonferroni correction was applied to the gradings. The mean [± standard deviation (SD)] age of patients was 55.5 (± 9.4) years. The WF-OCTA showed that an increasing size of the FAZ (from 0.442 (± 0.059) µm to 0.933 (± 0.086) µm) correlated with increasing severity of the DR (as determined with WF photography). The deep capillary plexus, FAZ size, and CNP areas in eyes with proliferative diabetic retinopathy (PDR) differed from those with mild nonproliferative diabetic retinopathy (NPDR) (p < 0.001). Most eyes with severe nonproliferative DR were found to have CNP in four quadrants [superficial capillary plexus (SCP) 60%, deep capillary plexus (DCP) 50%]. The WF-OCTA detected subtle neovascularization of the disc (NVD) in 7 eyes (10%) and neovascularization elsewhere (NVE) in 13 eyes (18%) that had been diagnosed with only moderate NPDR on WF photography. FAZ and CNP areas as measured by WF-OCTA correlate with DR severity. WF-OCTA can also detect subtle NVE and NVD that cannot be seen with fundus photography.

Head to head comparison of diagnostic performance of three non-mydriatic cameras for diabetic retinopathy screening with artificial intelligence

BackgroundDiabetic Retinopathy (DR) is a leading cause of blindness worldwide, affecting people with diabetes. The timely diagnosis and treatment of DR are essential in preventing vision loss. Non-mydriatic fundus cameras and artificial intelligence (AI) software have been shown to improve DR screening efficiency. However, few studies have compared the diagnostic performance of different non-mydriatic cameras and AI software.MethodsThis clinical study was conducted at the endocrinology clinic of Akdeniz University with 900 volunteer patients that were previously diagnosed with diabetes but not with diabetic retinopathy. Fundus images of each patient were taken using three non-mydriatic fundus cameras and EyeCheckup AI software was used to diagnose more than mild diabetic retinopathy, vision-threatening diabetic retinopathy, and clinically significant diabetic macular oedema using images from all three cameras. Then patients underwent dilation and 4 wide-field fundus photography. Three retina specialists graded the 4 wide-field fundus images according to the diabetic retinopathy treatment preferred practice patterns of the American Academy of Ophthalmology. The study was pre-registered on clinicaltrials.gov with the ClinicalTrials.gov Identifier: NCT04805541.ResultsThe Canon CR2 AF AF camera had a sensitivity and specificity of 95.65% / 95.92% for diagnosing more than mild DR, the Topcon TRC-NW400 had 95.19% / 96.46%, and the Optomed Aurora had 90.48% / 97.21%. For vision threatening diabetic retinopathy, the Canon CR2 AF had a sensitivity and specificity of 96.00% / 96.34%, the Topcon TRC-NW400 had 98.52% / 95.93%, and the Optomed Aurora had 95.12% / 98.82%. For clinically significant diabetic macular oedema, the Canon CR2 AF had a sensitivity and specificity of 95.83% / 96.83%, the Topcon TRC-NW400 had 98.50% / 96.52%, and the Optomed Aurora had 94.93% / 98.95%.ConclusionThe study demonstrates the potential of using non-mydriatic fundus cameras combined with artificial intelligence software in detecting diabetic retinopathy. Several cameras were tested and, notably, each camera exhibited varying but adequate levels of sensitivity and specificity. The Canon CR2 AF emerged with the highest accuracy in identifying both more than mild diabetic retinopathy and vision-threatening cases, while the Topcon TRC-NW400 excelled in detecting clinically significant diabetic macular oedema. The findings from this study emphasize the importance of considering a non mydriatic camera and artificial intelligence software for diabetic retinopathy screening. However, further research is imperative to explore additional factors influencing the efficiency of diabetic retinopathy screening using AI and non mydriatic cameras such as costs involved and effects of screening using and on an ethnically diverse population.

Retinal hemorrhages and damages from tractional forces associated with infantile abusive head trauma evaluated by wide-field fundus photography

We evaluated the distribution and types of retinal hemorrhages (RHs) and other damages in eyes with abusive head trauma (AHT). This retrospective, consecutive case series of AHT and non-AHT conditions involved 54 children with AHT, 43 children with head bruises, and 49 children with blunt eye trauma, each of non-AHT supported by reliable witness accounts. RHs and other damage were evaluated using ophthalmoscopy and wide-field fundus photography. A variety of RH types and other damage were identified in the AHT group but not in the non-AHT group. RHs in AHT extended from the posterior pole to the far periphery in 77% of eyes and on/near the veins in 86% and arteries in 85%, most of which were in the far periphery. Retinoschisis, white-dot lesions, and retinal folds were seen even in the far periphery. RHs on/near the veins and arteries, retinoschisis, and retinal folds suggest a traumatic mechanism of the tractional force of the vitreous that is attached to the entire retinal surface. Identifying the distribution and arterio and venous origins of RHs is a key factor in determining the association with trauma. Thus, wide-field fundus photography is useful to record and evaluate the origin of the RHs and other retinal damage.

Insights into the diagnosis and management of sarcoid uveitis: A review.

Sarcoidosis is a leading cause of non-infectious uveitis that commonly affects middle-aged individuals and has a female preponderance. The disease demonstrates age, sex and ethnic differences in clinical manifestations. A diagnosis of sarcoidosis is made based on a compatible clinical presentation, supporting investigations and histologic evidence of non-caseating granulomas, although biopsy is not always possible. Multimodal imaging with widefield fundus photography, optical coherence tomography and angiography can help in the diagnosis of sarcoid uveitis and in the monitoring of treatment response. Corticosteroid remains the mainstay of treatment; chronic inflammation requires steroid-sparing immunosuppression. Features on multimodal imaging such as vascular leakage may provide prognostic indicators of outcome. Female gender, prolonged and severe uveitis, and posterior involving uveitis are associated with poorer visual outcomes.

Multimodal Imaging Assessment of Typical Sympathetic Ophthalmia: A Case Report

Purpose: Despite the relatively low incidence of sympathetic ophthalmia, prompt diagnosis is essential to prevent visual impairment in both eyes. Here, we present a case of sympathetic ophthalmia, emphasizing distinctive imaging findings.Case summary: A 64-year-old male patient presented with complaints of right ocular pain and visual disturbances occurring 3 weeks after blunt trauma. The patient had a history of ophthalmologic surgeries, including cataract surgery in both eyes and intraocular lens trans-scleral fixation in the right eye. A primary suture was performed in the right eye due to iris prolapse through the scleral incision site and anterior chamber hemorrhage. At 1 week post-surgery, the patient reported diminished visual acuity in the left eye. Further assessment using wide-field fundus photography, optical coherence tomography, and fluorescein angiography revealed multiple focal hyperfluorescence leakage and serous retinal detachment. Consequently, sympathetic ophthalmia was diagnosed. Intravenous administration of high-dose steroids led to significant symptom improvement.Conclusions: We present a patient with a history of multiple ophthalmic surgeries and contralateral eye trauma indicative of sympathetic ophthalmia. Imaging modalities revealed typical findings for this condition. This case report emphasizes the importance of early diagnosis and treatment of sympathetic ophthalmia.

Ophthalmic Artery Occlusion as the Presenting Sign of Acquired Immunodeficiency Syndrome.

Describe a case of severe occlusive vasculitis that led to a diagnosis of acquired immunodeficiency syndrome (AIDS) in a previously healthy middle-aged man. Multimodal imaging including widefield fundus photography, spectral domain optical coherence tomography (OCT), and widefield fluorescein angiography (FA). A healthy 46-year-old man presented with sudden onset vision loss in his left eye with an afferent pupillary defect. His exam revealed signs of retinal vascular disease in both eyes, with an ophthalmic artery occlusion in his affected left eye and a hemiretinal vein occlusion in his asymptomatic right eye. An extensive medical workup was significant for human immunodeficiency virus (HIV) positivity; he was ultimately diagnosed with AIDS and ocular findings were attributed to an associated occlusive vasculitis. He developed anterior segment neovascularization in the left eye for which he received intravitreal bevacizumab and panretinal photocoagulation. He ultimately required cyclophotocoagulation in the left eye for poorly controlled intraocular pressure in the setting of neovascular glaucoma. Although HIV is most classically associated with a retinal microangiopathy, testing should be considered in cases of occlusive retinal vasculitis as it is a rare cause of such findings.

Evaluating spatial dependency of the spectral efficiency in trans-palpebral illumination for widefield fundus photography.

Multi-spectral widefield fundus photography is valuable for the clinical diagnosis and management of ocular conditions that may impact both central and peripheral regions of the retina and choroid. Trans-palpebral illumination has been demonstrated as an alternative to transpupillary illumination for widefield fundus photography without requiring pupil dilation. However, spectral efficiency can be complicated due to the spatial variance of the light property through the palpebra and sclera. This study aims to investigate the effect of light delivery location on spectral efficiency in trans-palpebral illumination. Four narrow-band light sources, covering both visible and near infrared (NIR) wavelengths, were used to evaluate spatial dependency of spectral illumination efficiency. Comparative analysis indicated a significant dependence of visible light efficiency on spatial location, while NIR light efficiency is only slightly affected by the illumination location. This study confirmed the pars plana as the optimal location for delivering visible light to achieve color imaging of the retina. Conversely, spatial location is not critical for NIR light imaging of the choroid.

Clinical features of retinopathy after cardiopulmonary resuscitation

PurposeTo evaluate the clinical patterns of retinopathy in patients who received cardiopulmonary resuscitation (CPR) using wide-field fundus photography and slit-lamp fundus examination.MethodsThe medical records of patients aged ≥ 18 years who survived after receiving CPR and underwent wide-field fundus photography and slit-lamp fundus examination within 3 months were retrospectively analyzed. Fundus findings, including retinal hemorrhage and cotton wool spots, were investigated. The subjects were categorized into the retinopathy and non-retinopathy groups based on the presence of fundus findings. Systemic and CPR-related factors were analyzed to compare the two groups.ResultsTwenty eyes (10 patients) and 28 eyes (14 patients) were included in the retinopathy and non-retinopathy groups, respectively. The retinopathy group had longer CPR time than the non-retinopathy group (15 ± 11 min vs. 6 ± 5 min, p = 0.027). In the retinopathy group, retinal nerve fiber layer hemorrhage was observed in all eyes, and intraretinal hemorrhage was observed in 55% of the eyes. 80% of hemorrhages were located in the peripapillary or posterior pole. There were no interval changes in visual acuity, intraocular pressure, and central retinal thickness for 6 months. The average remission periods of retinal hemorrhage and cotton wool spots were 6.8 ± 2.6 month and 5.6 ± 2.1 months, respectively. No retinopathy progression was observed.ConclusionThe signs of retinopathy, such as retinal hemorrhages and cotton wool spots, which are found after CPR, mainly occur in patients who receive longer time of CPR and improve over time.

Successful Treatment of Von Hippel-Lindau (VHL) Disease-Associated Retinal Capillary Hemangioblastoma (RCH) with Belzutifan in a Pediatric Patient.

The authors describe a case of a Retinal capillary hemangioblastoma (RCH) in a pediatric patient with von Hippel-Lindau (VHL) syndrome that was successfully treated with systemic belzutifan. Case report - The Clinical course was documented with serial fundus exams and multimodal imaging, including Optos wide field fundus photography and optical coherence tomography. A literature review was conducted to look for similar cases and/or discussion. A left RCH was noted on a standard VHL surveillance retinal exam of a then 15-year-old male with VHL syndrome. Over the course of 17 months this RCH was treated with focal laser therapy, photodynamic therapy (PDT), cryotherapy, bevacizumab injection, and endo laser ablation. Complications of these treatments included sub retinal fluid (SRF) and vitreomacular traction (VMT) necessitating laser retinopexy, scleral buckle, and pars plana vitrectomy with membrane stripping. After a 6-month interval from the last local therapy (endo laser treatment), there was minimal regression of the lesion and many concerning features persisted. At 22 months from presentation, the patient started belzutifan 120 mg PO daily with subsequent regression in size and less perfusion to the hemangioblastoma within 4 months. The patient is tolerating the systemic belzutifan with only the expected normocytic anemia and has not required transfusion therapy after 12 months of treatment. VHL disease is a rare and serious condition associated with multiple types of benign and malignant tumors. Belzutifan is tolerated in the adolescent population and can provide a systemic treatment alternative for VHL associated RCH.

Paracentral acute middle maculopathy progressing to central retinal artery occlusion following coronavirus disease vaccination: a multimodal imaging report

Background: Coronavirus disease (COVID-19) vaccines can cause adverse ocular effects, including vascular insults, acute macular neuroretinopathy, paracentral acute middle maculopathy (PAMM), ophthalmic vein thrombosis, Graves’ disease, arteritic anterior ischemic optic neuropathy (A-AION), and nonarteritic AION. Here, we report a case of unilateral PAMM progressing to central retinal artery occlusion (CRAO) after COVID-19 vaccination, identified using multimodal imaging.&#x0D; Case Presentation: A 24-year-old healthy man presented with unilateral progressive blurring of vision in the right eye. He had a recent history of fever without rashes 2 weeks after coronavirus disease vaccination. He was diagnosed with PAMM in the right eye at a local hospital and treated with a tapering dose of oral steroids. At presentation, he showed progressive blurring of vision in the right eye and the best-corrected distance visual acuity (BCDVA) was 20/60. The anterior segment was normal. Fundus examination revealed a pale optic disc with arteriolar attenuation and barrage laser scarring at the inferotemporal periphery. CRAO was diagnosed based on the right eye findings. The patient underwent multimodal imaging, including wide-field fundus photography using Optos® (Optos Carfornia®, Optos Inc., Dunfermline, United Kingdom), multicolor imaging with Spectralis™ (Heidelberg Retinal Angiograph; Heidelberg Engineering, Inc., Dossenheim, Germany), fundus fluorescence angiography (Heidelberg Retinal Angiograph; Heidelberg Engineering, Inc., Dossenheim, Germany), and optical coherence tomography angiography (ANGIOVUE, OPTOVUE, Inc., Fremont, CA, USA) using the split-spectrum amplitude-decorrelation angiography algorithm. The condition progressed from PAMM to CRAO during the oral steroid treatment course. At the 2-month follow-up, the right eye BCDVA had improved to 20/50, with fundus findings remaining the same as at the previous visit.&#x0D; Conclusions: This was the first report of a young patient with PAMM presenting with focal vascular occlusion that evolved to global occlusion in the form of CRAO in the absence of systemic vascular risk factors and with a normal coagulation profile. This case suggests that arterial occlusion may exert a temporary effect secondary to COVISHIELD™ vaccination. Randomized controlled trials and case – control studies on the role of vaccination in precipitating thromboembolic events in healthy individuals would provide insight into the causation.

Portable widefield fundus camera with high dynamic range imaging capability.

Fundus photography is indispensable for the clinical detection and management of eye diseases. Low image contrast and small field of view (FOV) are common limitations of conventional fundus photography, making it difficult to detect subtle abnormalities at the early stages of eye diseases. Further improvements in image contrast and FOV coverage are important for early disease detection and reliable treatment assessment. We report here a portable, wide FOV fundus camera with high dynamic range (HDR) imaging capability. Miniaturized indirect ophthalmoscopy illumination was employed to achieve the portable design for nonmydriatic, widefield fundus photography. Orthogonal polarization control was used to eliminate illumination reflectance artifacts. With independent power controls, three fundus images were sequentially acquired and fused to achieve HDR function for local image contrast enhancement. A 101° eye-angle (67° visual-angle) snapshot FOV was achieved for nonmydriatic fundus photography. The effective FOV was readily expanded up to 190° eye-angle (134° visual-angle) with the aid of a fixation target without the need for pharmacologic pupillary dilation. The effectiveness of HDR imaging was validated with both normal healthy and pathologic eyes, compared to a conventional fundus camera.

Brain fog and vessel clogs

We present a unique case of a 36-year-old female presenting with features suggestive of bilateral combined vascular occlusion, hearing loss, and encephalopathy. Multimodal imaging was done for both eyes fundus evaluation including wide-field color fundus photography, optical coherence tomography, and fundus fluorescein angiography. After extensive ocular and systemic investigations, she was diagnosed to have Susac syndrome (SS). She was referred to a neurologist and otologist for systemic evaluation and underwent laser photocoagulation in both eyes, followed by pars plana vitrectomy in her left eye. Combined bilateral retinal vascular occlusion in association with SS is very rare.

Effective field of view of wide-field fundus photography in the Stanford University Network for Diagnosis of Retinopathy of Prematurity (SUNDROP)

Five-field 130° wide-angle imaging is the standard of care for retinopathy of prematurity (ROP) screening with an ideal hypothetical composite field-of-view (FOV) of 180°. We hypothesized that in many real-world scenarios the effective composite FOV is considerably less than ideal. This observational retrospective study analyzed the effective FOV of fundus photos of patients screened for ROP as part of the Stanford University Network for Diagnosis of Retinopathy of Prematurity (SUNDROP) initiative. Five fundus photos were selected from each eye per image session. Effective FOV was defined as the largest circular area centered on the optic disc that encompassed retina in each of the four cardinal views. Seventy-three subjects were analyzed, 35 without ROP and 34 with ROP. Mean effective FOV was 144.55 ± 6.62° ranging from 130.00 to 153.71°. Effective FOV was not correlated with the presence or absence of ROP, gestational age, birth weight, or postmenstrual age. Mean effective FOV was wider in males compared to females. Standard five-field 130° fundus photos yielded an average effective FOV of 144.54° in the SUNDROP cohort. This implies that an imaging FOV during ROP screening considerably less than the hypothetical ideal of 180° is sufficient for detecting treatment warranted ROP.

Light color efficiency-balanced trans-palpebral illumination for widefield fundus photography of the retina and choroid

A wide-field fundus camera, which can selectively evaluate the retina and choroid, is desirable for better detection and treatment evaluation of eye diseases. Trans-palpebral illumination has been demonstrated for wide-field fundus photography, but its application for true-color retinal imaging is challenging due to the light efficiency delivered through the eyelid and sclera is highly wavelength dependent. This study is to test the feasibility of true-color retinal imaging using efficiency-balanced visible light illumination, and to validate multiple spectral imaging (MSI) of the retina and choroid. 530 nm, 625 nm, 780 nm and 970 nm light emission diodes (LED)s are used to quantitatively evaluate the spectral efficiency of the trans-palpebral illumination. In comparison with 530 nm illumination, the 625 nm, 780 nm and 970 nm light efficiencies are 30.25, 523.05, and 1238.35 times higher. The light efficiency-balanced 530 nm and 625 nm illumination control can be used to produce true-color retinal image with contrast enhancement. The 780 nm light image enhances the visibility of choroidal vasculature, and the 970 nm image is predominated by large veins in the choroid. Without the need of pharmacological pupillary dilation, a 140° eye-angle field of view (FOV) is demonstrated in a snapshot fundus image. In coordination with a fixation target, the FOV can be readily expanded over the equator of the eye to visualize vortex ampullas.

Prophylactic juxtapapillary laser photocoagulation in pediatric morning glory syndrome

To determine the anatomic and visual outcomes of prophylactic juxtapapillary laser photocoagulation treatment alone in the prevention of retinal detachment (RD) in a cohort of pediatric patients diagnosed with morning glory syndrome (MGS). A total of 24 eyes of 22 consecutive patients aged 0-15y diagnosed with MGS treated with prophylactic juxtapapillary laser photocoagulation alone were reviewed. Data including demographics, ocular examination, anatomic and visual outcomes, following treatment and complications were collected. Two patients had bilateral laser treatment and 20 had monocular laser treatment. The age at treatment of 13 (59.1%) patients was less than 12mo. The presenting symptoms included strabismus (6/22, 27.3%), decreased vision (2/22, 9.1%), and routine fundus screening (14/22, 63.6%). Fifteen (68.2%) patients underwent cranial magnetic resonance imaging (MRI) examinations, and 3 of those 15 (20.0%) had abnormal findings in the nervous system. Based on preoperative wide-field fundus photography and B-scan echography, all (100.0%) eyes had no obvious RD. On postoperative 1mo and 6mo and the following follow-ups, the anatomic outcomes of all eyes remained stable. The mean follow-up duration was 27.7±17.5mo. No severe complications were found. Preoperative visual acuity acquired from 2 (9.1%) patients ranged from light perception to 20/200. Postoperative acuity acquired from 11 (50.0%) patients ranged from light perception to 20/125. The preliminary anatomic and visual outcomes of prophylactic juxtapapillary laser treatment alone in pediatric MGS patients are relatively stable in a short-term follow-up. Further long-term clinical observation will be needed to confirm its efficacy and safety.

Wide-field Fundus Imaging and Fluorescein Angiography Findings in Various Pseudoretinoblastoma Conditions.

To report wide-field fundus imaging and fluorescein angiography findings in conditions mimicking retinoblastoma (pseudoretinoblastoma). The clinical and imaging records of 28 patients (36 eyes) imaged with RetCam 3 (Clarity Medical Systems, Inc) wide-field fundus photography and fluorescein angiography who were diagnosed as having various pseudoretinoblastoma disorders between February 2020 and August 2021 were retrospectively evaluated. Most patients were referred with suspicion of retinoblastoma. Other patients had unresolved eye conditions and received initial treatment elsewhere and they were referred later for exclusion of retinoblastoma. On RetCam fundus photography and fluorescein angiography, pseudoretinoblastoma conditions were diagnosed as Coats disease (9 eyes), congenital glaucoma (3 eyes), persistent fetal vasculature (3 eyes), familial exudative vitreoretinopathy (2 eyes), retinopathy of prematurity (2 eyes), myelinated retinal nerve fibers (2 eyes), optic nerve hypoplasia (2 eyes), oculocutaneous albinism (2 eyes), meridional fold (2 eyes), combined hamartoma of retina and retinal pigment epithelium (2 eyes), grouped congenital hypertrophy of the retinal pigment epithelium (1 eye), retinal astrocytic hamartoma (1 eye), morning glory syndrome (1 eye), optic glioma (1 eye), giant choroidal nevus (1 eye), vasculitis (1 eye), and chorioretinitis (1 eye). RetCam imaging and fluorescein angiography demonstrated telangiectasias, microaneurysms, submacular exudation/fibrosis, capillary non-perfusion, and exudation posterior to telangiectasias in Coats disease. Hypofluorescent optic discs, peripheral non-perfusion, and vascular leakage were observed in congenital glaucoma. Large areas of capillary non-perfusion, poor foveal morphology/formation, elongated ciliary processes with hyperfluorescent central core, and hyperfluorescent fibrovascular stalk at the posterior pole were hallmarks of persistent fetal vasculature. RetCam wide-field fundus imaging and fluorescein angiography play an important role in the diagnosis of conditions simulating retinoblastoma. [J Pediatr Ophthalmol Strabismus. 2023;60(1):60-74.].

Retinal Manifestations in Patients with COVID-19: A Prospective Cohort Study

The recent outbreak of the Coronavirus SARS-CoV-2 has been declared a worldwide pandemic. Within various multi-organ involvement, several ocular manifestations have been described, such as conjunctivitis and retinopathy. The prevalence and severity of retinal lesions and their relation to the severity of the systemic disease are unknown. We performed a prospective, observational study on 172 consecutively hospitalized patients with acute confirmed COVID-19 infection. All patients underwent screening widefield fundus photography at the time of hospital admission. Despite no ocular or vision-related symptoms, we found cotton wool spots (CWS) and/or hemorrhages in 19/172 patients (11%). Diabetes history, overweight, and elevated C-reactive protein were more frequently observed among patients with retinal abnormalities, while a history of systemic hypertension was more frequently observed among patients without retinal findings. At a 3-month follow-up visit, CWS had subsided in all patients.

North Carolina Macular Dystrophy: Long-term Follow-up of the Original Family

The phenotype of North Carolina macular dystrophy (NCMD) is highly variable and remains poorly appreciated and understood, often causing misdiagnoses in isolated cases. One of the features of NCMD is the general lack of progression despite its original name, "dominant progressive foveal dystrophy," as reported in 1971 by Lefler etal (W.H.L.). The purpose of this study was to report the long-term follow-up of this condition. Systematic, longitudinal, and detailed documentation along with the imaging of the peripheral retina. We reexamined 27 of the original family members with NCMD in an office setting 30 to 50 years after they were first reported. The evaluation of all the affected subjects included best-corrected visual acuity (BCVA), slit-lamp and dilated-fundus examinations, wide-field fundus and autofluorescent photography, and spectral-domain OCT (SD OCT). Blood was collected for DNA extraction, banking, and sequencing. Best-corrected visual acuity, slit-lamp and dilated-fundus examinations, wide-field fundus and autofluorescent photography, and SD OCT. The 27 subjects examined were a part of the original family with NCMD that was initially reported in 1971. A point mutation (NC_000006.11:g.100040906G>T) (Hg19) in a noncoding region of a deoxyribonuclease I hypersensitivity binding site was found in all the affected subjects. Nine subjects were the affected children of those originally examined 30 to 50 years ago by Kent W. Small (K.W.S.) and W.H.L., and the remaining 17 subjects (34 eyes) had been examined 30 years previously by K.W.S. Of these 17 subjects (34 eyes), 4 of 34 (11%) eyes showed worsening of vision and evidence of fibrosis due to choroidal neovascular membranes (CNVMs). Fourteen of the 27 (51%) patients showed peripheral retinal drusen, which did not seem to correlate with the severity of the macular disease. Most patients with NCMD have stable vision and fundus findings throughout their lives. The ones who experienced BCVA decline did so because of the apparent evidence of CNVMs. Patients with grade 2 NCMD seem to be at an increased risk of further or progressive vision loss due to CNVMs. Intravitreal therapy with vascular endothelial growth factor inhibitors may benefit these patients if they are treated in a timely fashion. Peripheral retina drusen of varying degrees of severity were found in slightly more than half of the affected subjects.

The role of retinal imaging in the management of abusive head trauma cases.

As retinal hemorrhage (RH) is the most frequent and reliable finding of abusive head trauma (AHT), an ophthalmology consultation should be systematically required in suspected cases. Full retinal examination through pharmacologically dilated pupil can detect the type and pattern of RHs, helping to distinguish abusive from non-abusive head trauma. We performed a retrospective analysis of a case series of 6 infants (aged 0.6-10 months) with AHT who were admitted to the Emergency Department of Fondazione Policlinico Universitario A. Gemelli IRCCS in Rome with severe intracranial hemorrhages. Children underwent full multidisciplinary assessment including dilated fundus examination, optical coherence tomography (OCT) and digital wide-field fundus photography (DWFFP - in our case RetCam). In our paper we report the clinical presentation, the ocular findings and outcome at discharge. The mean age at the hospital admission was 6.28 months. In all infants, intracranial hemorrhages were found. Preretinal and intraretinal hemorrhages were detected, collecting good-quality retinal images. Imaging of retinal hemorrhages represents a fundamental moment of AHT diagnosis and documentation. Although RetCam is the gold standard for the acquisition of retinal images in suspected cases, OCT is extremely valuable in forensic evaluation since it can detect even small macular hemorrhages. Therefore, the combination of RetCam and OCT imaging can give relevant hints for the diagnosis of AHT, allowing to evaluate the extent, spread and morphology of RHs.

Developing portable widefield fundus camera for teleophthalmology: Technical challenges and potential solutions.

A portable, low cost, widefield fundus camera is essential for developing affordable teleophthalmology. However, conventional trans-pupillary illumination used in traditional fundus cameras limits the field of view (FOV) in a snapshot image, and frequently requires pharmacologically pupillary dilation for reliable examination of eye conditions. This minireview summarizes recent developments in alternative illumination approaches for widefield fundus photography. Miniaturized indirect illumination has been used to enable compact design for developing low cost, portable, widefield fundus camera. Contact mode trans-pars-planar illumination has been validated for ultra-widefield fundus imaging of infant eyes. Contact-free trans-pars-planar illumination has been explored for widefield imaging of adult eyes. Trans-palpebral illumination has been also demonstrated in a smartphone-based widefield fundus imager to foster affordable teleophthalmology.