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Purpose: To describe a new retinal phenotype characterized by bilateral, multifocal, subretinal vitelliform lesions along the vascular arcades that we refer to as multifocal vitelliform paravascular retinopathy (MVPR)., Design: Observational case series., Methods: Multimodal retinal imaging including color fundus photography, fundus autofluorescence and cross sectional and en-face optical coherence tomography was performed to evaluate and characterize the lesions of MVPR., Results: Thirteen asymptomatic patients aged 10 to 78 [mean 49 ± 24, 49% under 50] were evaluated for bilateral retinal lesions. Initial visual acuity was 20/30 or better in 22 (85%) eyes. Of the 20 eyes with follow-up, 14 (70%) exhibited visual acuity 20/30 or better at final follow-up. Multifocal small round yellow lesions with distinct borders were identified along the vascular arcades in all patients. The vitelliform lesions were brightly hyperautofluorescent and consisted of focal areas of subretinal hyperreflective material on optical coherence tomography (OCT) that in some cases evolved to hyporeflective spaces (or retinal pigment epithelium atrophy) with associated hypoautofluorescence. When performed, electroretinography (ERG) and electrooculography (EOG) testing were normal and genetic testing was negative for variants in BEST1 and other genes associated with vitelliform retinopathies., Conclusions: MVPR may represent a novel entity of vitelliform disorders with a distinct clinical presentation and phenotype and generally favorable prognosis., (Published by Elsevier Inc.)

Purpose: To survey the impact of directional reflectivity on structures within optical coherence tomography images in retinal pathology., Methods: Sets of commercial optical coherence tomography images taken from multiple pupil positions were analyzed. These directional optical coherence tomography sets revealed directionally reflective structures within the retina. After ensuring sufficient image quality, resulting hybrid and composite images were characterized by assessing the Henle fiber layer, outer nuclear layer, ellipsoid zone, and interdigitation zone. Additionally, hybrid images were reviewed for novel directionally reflective pathological features., Results: Cross-sectional directional optical coherence tomography image sets were obtained in 75 eyes of 58 patients having a broad range of retinal pathologies. All cases showed improved visualization of the outer nuclear layer/Henle fiber layer interface, and outer nuclear layer thinning was, therefore, more apparent in several cases. The ellipsoid zone and interdigitation zone also demonstrated attenuation where a geometric impact of underlying pathology affected their orientation. Misdirected photoreceptors were also noted as a consistent direction-dependent change in ellipsoid zone reflectivity between regions of normal and absent ellipsoid zone., Conclusion: Directional optical coherence tomography enhances the understanding of retinal anatomy and pathology. This optical contrast yields more accurate identification of retinal structures and possible imaging biomarkers for photoreceptor-related pathology.

Purpose: To report a case of concurrent melanoma metastasis to the orbit and MEK inhibitor-associated retinopathy., Methods: Case report of a 66-year-old female patient undergoing mutation targeted MEK-inhibitor treatment for metastatic melanoma with retinal manifestations., Results: After initiating MEK-inhibitor therapy our patient presented with bilateral blurred vision. Clinical examination and OCT imaging revealed multifocal bilateral serous retinal detachments consistent with MEKAR. Extrafoveal subretinal fluid foci resolved after discontinuation of MEK-inhibitor therapy, but her foveal lesions persisted for over 18 months. Years after her initial evaluation for MEKAR, our patient developed metamorphopsia in the left eye resulting from chorioretinal folds secondary to intraconal orbital metastasis. MRI revealed additional intracerebral and lung metastases, and the patient expired in 2 months., Conclusion: We report a case of prolonged MEKAR with concomitant orbital metastasis from melanoma. Multimodal imaging in conjunction with history and clinical features allows for disease identification and monitoring.

Background/purpose: The purpose of this report was to describe the use of multimodal imaging to establish the diagnosis of Bartonella henselae -associated optic neuropathy in a patient who presented with a central scotoma without overt evidence of optic nerve involvement., Methods: This was a case report. Main outcome measures included clinical, optical coherence tomography, and fluorescein angiography findings., Observations: A 72-year-old woman presented with a 3-day history of central scotoma in the left eye. Her examination was remarkable for faint exudation in the nasal macula of the left eye but was otherwise normal for her age. Spectral domain optical coherence tomography of the macula revealed mild thickening of the papillomacular bundle with scattered small cystoid spaces and several intraretinal exudates, none of which were visible clinically. Fluorescein angiography revealed localized leakage of the inferotemporal optic disc. When prompted, the patient recalled being scratched multiple times by her two pet kittens. Serial testing showed rising anti- B. henselae ( B. henselae ) immunoglobulin G antibody titers to 1:1,280, confirming the suspected diagnosis of B. henselae -associated optic neuropathy., Conclusion: Bartonella -associated optic nerve involvement can occur without overt evidence of optic disc swelling. Multimodal imaging can be used to suggest the diagnosis and support appropriate serologic testing., (Copyright © 2022 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the Opthalmic Communications Society, Inc.)

Purpose: To describe a case of nonsyndromic retinitis pigmentosa caused by presumed compound heterozygous A615T and T522M mutations in HGSNAT, characterized by bilateral cystoid macular edema and retinal neovascularization., Methods: Case report. The patient underwent clinical evaluation, multimodal imaging, and next-generation panel sequencing. In silico analysis was performed with PolyPhen-2, SIFT, and MutationTaster. Segregation analysis was not available., Results: A 35-year-old hypertensive man presented with nyctalopia, photopsia, and difficulty reading for six months. He had no family history of visual deficits. The best-corrected visual acuity was 20/25 in the right eye and 20/20 in the left eye. Examination revealed midperipheral bone spicules and macular neovascularization in both eyes. Multimodal imaging demonstrated cystoid macular edema, ellipsoid band loss outside the central macula, and leakage from the neovascularization in both eyes. Sequencing detected four mutations in three genes, including two heterozygous mutations in HGSNAT (c.1843G>A, p.A615T and c.1565C>T, p.T522M). A615T is a pathogenic, hypomorphic mutation. T522M has not been previously phenotypically described. It is predicted damaging by in silico analysis and occurs at a conserved position near the eighth transmembrane domain, adjacent to residues in which missense mutations result in protein misfolding., Conclusion: This is, to the best of our knowledge, the first reported case of retinal neovascularization in a case of nonsyndromic retinitis pigmentosa due to HGSNAT mutation. The T522M variant likely functions as a severe mutation alongside the hypomorphic A615T mutation. These findings expand the genotypic and phenotypic spectrum of nonsyndromic retinitis pigmentosa.

Purpose: To report two patients who developed full-thickness macular holes following panretinal photocoagulation for proliferative diabetic retinopathy., Methods: Case series., Results: Case 1 was a 58-year-old woman with Type 1 diabetes mellitus and proliferative diabetic retinopathy and Case 2, a 72-year-old man with Type 2 diabetes mellitus and proliferative diabetic retinopathy. Before panretinal photocoagulation, each patient had an epiretinal membrane and an irregular foveal contour noted on optical coherence tomography. At 2 months follow-up, each patient showed foveal floor thinning and Case 2 also had a lamellar macular hole. At 6 months follow-up, each patient had a full-thickness macular hole. Each were successfully repaired with vitrectomy, membrane peeling, and gas tamponade., Conclusion: The relationship between hole formation and previous panretinal photocoagulation in the patients reported here is unclear and possibly coincidental.

Purpose: To report an increasingly prevalent fundoscopic manifestation of syphilitic uveitis., Methods: Case report of a patient with acute retinal necrosis secondary to syphilis., Results: A 48-year-old man presented with decreased vision, anterior segment inflammation, and a wedge-shaped retinal lesion in the superior periphery, with a diaphanous leading edge extending down toward the superior arcade. The patient was HIV+ and poorly compliant with therapy. The top three differential diagnoses were herpetic necrotizing retinitis, syphilis, and lymphoma. An extensive lab workup was positive for syphilis. The patient was treated with IV penicillin G and demonstrated improvement in visual acuity and examination., Conclusion: There have been an increasing number of reports of syphilis patients, especially in the population of men who have sex with men, who present with fundus findings similar to acute retinal necrosis. These findings include a characteristic ground glass, translucent appearance of unifocal or multifocal lesions, primarily affecting the inner retina and sometimes associated with co-localizing occlusive vasculitis. Treatment with IV penicillin G is warranted and has demonstrated good visual recovery.

Purpose: To describe a patient with metastatic clear cell renal cell carcinoma in remission on maintenance nivolumab therapy who developed late-onset ocular toxicity manifesting as creamy chorioretinal lesions with exudative retinal detachment concerning for choroidal metastasis., Method: Case report. Main outcome measures include ophthalmoscopic examination, fundus photography, fundus autofluorescence, fluorescein angiography, indocyanine green angiography, spectral domain optical coherence tomography, and B-scan ultrasonography., Results: A 49-year-old woman with a medical history of metastatic clear cell renal cell carcinoma in remission for two years after immunotherapy with four cycles of ipilimumab and nivolumab followed by maintenance nivolumab infusions developed lesions concerning for choroidal metastases in her right eye. Optical coherence tomography of the lesions revealed a bacillary layer detachment containing possible fibrinous exudate organized into layers and underlying choroidal thickening with chorioretinal folds. Later, choroidal thickening and chorioretinal folds also occurred in the left eye. Given that pan imaging detected no metastasis and the posterior segment abnormalities resolved after cessation of nivolumab and treatment with systemic corticosteroids, the patient was diagnosed with nivolumab-induced Vogt-Koyanagi-Harada-like uveitis., Conclusion: This case expands on the clinical spectrum of nivolumab-induced Vogt-Koyanagi-Harada-like uveitis, a condition that can also present with bacillary layer detachment mimicking an early choroidal metastasis, manifest asymmetrically in each eye, and develop after long-standing treatment.

Purpose: To report a case of Purtscher-like retinopathy in a patient with milk-alkali syndrome and pancreatitis., Methods: Case report and review of the literature., Results: A 46-year-old woman presented with decreased vision following discharge from the intensive care unit, where she had been admitted for milk-alkali syndrome secondary to long-term calcium supplementation and over-the-counter antacid use, and pancreatitis. Dilated examination showed ischemic retinal whitening and retinal hemorrhages in the posterior pole bilaterally consistent with Purtscher-like retinopathy. Over three months, the retinopathy resolved and her vision improved., Conclusion: Milk-alkali syndrome is the clinical triad of hypercalcemia, renal failure, and metabolic alkalosis and occurs secondary to the consumption of large amounts of calcium and alkali. The hypercalcemia associated with milk-alkali syndrome may cause pancreatitis, which can lead to Purtscher-like retinopathy.

Purpose: To describe two cases of catastrophic, bilateral retinal vascular occlusion after intravitreal (IVT) bevacizumab injection., Methods: Case series. Main outcome measures included clinical and fluorescein angiography findings., Results: Case 1-A 65-year-old woman with calcinosis, Raynaud phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasis syndrome developed acute, severe, bilateral visual loss 2 weeks after bilateral IVT bevacizumab injection for proliferative diabetic retinopathy. Examination and fluorescein angiography revealed moderate anterior chamber inflammation, bilateral perivascular retinal hemorrhages, and near total retinal vascular occlusion. Extensive testing revealed moderately elevated anti-B2 glycoprotein (antiphospholipid) antibodies. Case 2-An 85-year-old man with polymyalgia rheumatica and left eye exudative age-related macular degeneration experienced severe, bilateral, sequential visual loss in the left eye and then right eye approximately 3 weeks after IVT bevacizumab left eye injection. Examination revealed bilateral panuveitis, diffuse perivascular exudates, and intraretinal hemorrhages. Fluorescein angiography showed diffuse venous leakage. Extensive testing revealed an elevated antinuclear antibody and mildly elevated anticardiolipin antibody., Conclusion: Patients with underlying retinal vascular vulnerabilities may be at increased risk of catastrophic, bilateral retinal vascular occlusion after treatment with IVT bevacizumab. The moderate-to-severe intraocular inflammation in both cases and the contralateral involvement after unilateral IVT injection in Case 2 suggest a possible delayed immune-mediated mechanism.

Purpose: To assess the impact of submacular fluid (SMF) after pneumatic retinopexy for primary rhegmatogenous retinal detachment repair., Methods: Retrospective review of consecutive patients treated with pneumatic retinopexy for primary rhegmatogenous retinal detachment repair. 387 eyes (374 patients) were included, of which 166 underwent optical coherence tomography imaging after successful pneumatic retinopexy. Foveal-centered optical coherence tomography scans were reviewed., Results: SMF occurred in 59 eyes (35.5%) and was associated with macular detachment ( P ≤ 0.001) and phakic lens status ( P = 0.007). Submacular fluid resolved over an average of 9.39 months and was associated with worse preprocedure best-corrected visual acuity and delayed visual recovery. The mean final best-corrected visual acuity was 0.277 logarithm of the minimum angle of resolution (20/40) in eyes with SMF and 0.162 logarithm of the minimum angle of resolution (20/30) in those without SMF ( P < 0.001). Submacular fluid was associated with discontinuity of the interdigitation zone ( P = 0.003), ellipsoid zone ( P = 0.005), and external limiting membrane ( P ≤ 0.001) after SMF resolution. Ellipsoid zone discontinuity was associated with worse visual prognosis ( P = 0.009)., Conclusion: Trace SMF detected by optical coherence tomography is common after successful pneumatic retinopexy and resulted in delayed visual recovery and increased rates of outer retinal discontinuity after SMF resorption, although the final difference in best-corrected visual acuity in those with and without SMF was minimal.

Purpose: To report a sporadic case of a familial adenomatous polyposis (FAP) discovered in a patient with bilateral retinal pigment epithelial lesions., Methods: Case report., Results: A 30-year-old Asian woman presented for evaluation of bilateral pigmented lesions at the level of the retinal pigment epithelium. She had no personal or family history of colonic polyps or colon cancer. Colonoscopy revealed innumerable adenomatous polyps and genetic testing revealed a mutation in the adenomatous polyposis coli gene consistent with FAP. She subsequently underwent prophylactic total colectomy., Conclusion: The pigmented retinal pigment epithelium lesions of FAP have a characteristic appearance and it is vital for the retinal specialist to be familiar with them. De novo mutations in the APC gene are responsible for 20% to 30% of FAP cases. In the presence of the characteristic retinal pigment epithelium lesions, it is important to send the patient for work up of FAP even in the absence of family history of FAP.

Purpose: To investigate the clinical and anatomic characteristics of soccer ball-induced posterior segment injuries in the era of modern multi-modal imaging., Methods: Retrospective case series of patients with soccer ball injury and diagnostic imaging from 2007 to 2020 at a single vitreoretinal practice., Results: Eight patients met inclusion criteria. Fundus photographs (FP) and optical coherence tomography (OCT) were obtained in eight patients, fundus autofluorescence (FAF) in five patients, fluorescein angiography (FA) in three patients, and en-face OCT and OCT-Angiography (OCT-A) were obtained in two patients each. FA and FAF identified traumatic pigment epitheliopathy secondary to commotio. Increased hypo-autofluorescence was associated with shallow, peripheral retinal detachment on FAF. OCT of the macula displayed outer retinal changes associated with commotio, and offered insight into the acute and subacute changes of traumatic macular hole formation. A patient displayed foveal hyper-reflectivity in the shape of an hourglass with retinal cyst at the level of the external limiting membrane (ELM) as seen on OCT and En-face OCT. A patient with commotio involving the macula lacked microvascular changes on OCT-A., Conclusion: OCT, FA, and FAF imaging may aid in the work-up and management of the soccer ball-related posterior segment injuries.

Purpose: To discuss the mechanism of injury and characterize the clinical features of ocular trauma associated with elastic cord exercise equipment band injuries in a consecutive series of patients seen at a single vitreoretinal surgery practice., Methods: We performed a retrospective review of all patients who were treated for blunt trauma from 2013 to 2020 at a single vitreoretinal practice., Results: Thirteen eyes from 11 patients met the inclusion criteria of possessing ocular trauma secondary to recoil from exercise bands. Presenting visual acuity ranged from 20/16 to HM (median: 20/32). The most frequently observed anterior segment pathologies were traumatic iritis (54%) and angle recession (31%). The most common posterior segment findings were vitreous hemorrhage (54%) and peripheral commotio retinae (54%). Three eyes (23%) required surgical intervention. Follow-up intervals ranged from 0 to 10 months (median: 1.75 months). Visual acuity at last examination ranged from 20/13 to 20/400 (median: 20/40)., Conclusion: A wide spectrum of serious ocular injuries requiring medical and surgical intervention can result from this form of blunt ocular trauma. The frequency of this event would be decreased by the use of sports goggles and careful inspection of equipment for wear and over use., (Copyright © 2020 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the Opthalmic Communications Society, Inc.)

Purpose: To present select cases of paracentral acute middle maculopathy (PAMM) demonstrating progressive vascular occlusion and the ischemic cascade and illustrate scenarios in which therapeutic intervention may be considered., Design: Perspective., Methods: Review and interpretation of selected literature, with perspective on the evaluation and management of patients with PAMM lesions. Multimodal imaging of 3 illustrative cases of PAMM are presented, including cross-sectional and en face optical coherence tomography (OCT), with progressive vascular occlusion and evidence of the ischemic cascade noted in 2 of the cases., Results: All 3 cases showed evidence of PAMM at baseline, including perivenular PAMM in 2 cases. Progression from a mild central retinal vein occlusion (CRVO) to a more severe CRVO was noted in the first case, while the second case progressed from an incomplete to complete central retinal artery occlusion (CRAO). In the third case, there was resolution of PAMM lesions associated with partial CRAO after the patient began taking aspirin., Conclusions: Perivenular PAMM can be the only presenting sign of retinal vascular occlusion. In such cases, it is important to differentiate between partial CRVO and incomplete CRAO as the underlying cause. We provide guidance on making this important distinction, which plays a critical role in directing the nature and urgency of the systemic work-up. In patients with PAMM caused by partial retinal arterial occlusion, a benign empirical approach, such as low-dose aspirin (81 mg), may be reasonable to reduce the likelihood of subsequent vision loss from progression to complete arterial occlusion. A clinical trial, however, is necessary to support such a strategy., (Copyright © 2021 Elsevier Inc. All rights reserved.)

A 78-year-old woman underwent 25-gauge pars plana vitrectomy and scleral fixation for a dislocated intraocular lens in the left eye under general anesthesia. She developed massive hemorrhagic choroidal detachment 1 day later. A giant retinal tear detachment and proliferative vitreoretinopathy was noted at postoperative Week 7 and repaired with choroidal drainage, 240° peripheral retinectomy, perfluorocarbon liquid, endolaser, and silicone oil. Hypotony associated with the 25-gauge sclerotomies used for suture fixation was the suspected etiology for this complication. Use of 27-gauge instrumentation or application of fibrin glue to superior sclerotomies may help reduce the incidence of postoperative hypotony. [ Ophthalmic Surg Lasers Imaging Retina. 2021;52:288-292.] .

Background and Objective: Ellipsoid zone (EZ) reflectivity on optical coherence tomography (OCT) is affected by the orientation of the scanning beam. The authors sought to determine how directional reflectivity changes in dry age-related macular degeneration (AMD)., Patients and Methods: Retrospective image analysis included 17 control and 20 dry AMD subjects. Directional OCT (D-OCT) was performed using multiple displaced pupil entrance positions. EZ pixel values and apparent incidence angles were measured., Results: EZ reflectivity decreased in off-axis scans in controls ( P < .001), AMD areas between drusen ( P < .001), and AMD areas overlying drusen ( P < .001). The magnitude of decrement in EZ reflectivity was significantly higher when incidence angles exceeded 10° in controls than in AMD areas between drusen ( P = .024)., Conclusion: EZ reflectivity in dry AMD may vary by incident angle of light less than in controls, possibly indicating alteration of photoreceptor orientation or integrity. [ Ophthalmic Surg Lasers Imaging Retina. 2021;52:145-152.] .

Purpose: To describe the appearance of concentric, fingerprint-like waves within the Henle fiber layer (HFL) using en face optical coherence tomography in patients with tractional pathologies of the retina., Methods: Retrospective analysis of six eyes of six patients imaged by optical coherence tomography with volumetric slabs positioned at the level of the HFL., Results: Optical coherence tomography data from six patients with tractional vitreoretinal pathology were reviewed. Concentric, fingerprint-like microwaves were visualized through en face optical coherence tomography in all six study eyes at the level of the HFL. This finding resembled the finding of HFL waves previously noted histopathologically from force exerted on this layer., Conclusion: In retinal pathologies in which specific physical forces act on the retina, volumetric optical coherence tomography may permit visualization of en face concentric, fingerprint-like hyperreflective rings within the HFL. This "fingerprint sign" may represent a biomechanical consequence of traction on the retina and allow clinical decision making based on improved recognition of the existence of such traction.

Background Historically, CRX mutations have been associated with cone-rod dystrophy, cone dystrophy, Leber's congenital amaurosis, and retinitis pigmentosa. There is recent emerging evidence of an adult-onset macular dystrophy phenotype. We review the published literature and discuss the first case of heterozygous CRX R90 W macular dystrophy. Materials and Methods The patient received serial ophthalmic examination and imaging. Genetic testing was performed by MyRetinaTracker with the use of a retinal dystrophy panel. Results A 55-year-old Caucasian male patient without a prior medical history presented for evaluation of decreased vision in the right eye. Visual acuity was 20/32 both eyes, and his fundus examination was notable for an incomplete ring-shaped macular atrophy with foveolar sparing in both eyes. Fundus autofluorescence was notable for hypo-autofluorescence of the ring and fluorescein angiography for transmission hyperfluorescence. Full-field ERG and EOG were normal, while mfERG showed central depression. His lesion was clinically diagnosed as benign concentric annular macular dystrophy, but genetic testing revealed a heterozygous mutation in CRX (c.268 C > T, p.R90 W). A three-generation family tree did not reveal other members with known macular dystrophy. Given the lack of documentable autosomal dominant inheritance and the presence of a CRX mutation, the patient's diagnosis was revised to adult-onset macular dystrophy. Conclusions We believe this to be the first case of a dult onset macular dystrophy associated with heterozygous CRX R90 W mutation.

Background: Autosomal recessive bestrophinopathy is part of the diverse spectrum of retinal diseases caused by mutations in the BEST1 gene., Methods: A case report., Results: We present a case that highlights the classic retinal findings of autosomal recessive bestrophinopathy with an emphasis on modern multimodal imaging., Conclusion: We describe modern multimodal imaging in an individual with a BEST1 gene mutation and clinical findings consistent with an autosomal recessive bestrophinopathy.

There is an increased incidence of heart failure in individuals with diabetes mellitus (DM). The coexistence of kidney disease in DM exacerbates the cardiovascular prognosis. Researchers have attempted to combine the critical features of heart failure, using transverse aortic constriction, with DM in mice, but variable findings have been reported. Furthermore, kidney outcomes have not been assessed in this setting; thus its utility as a model of heart failure in DM and kidney disease is unknown. We generated a mouse model of obesity, hyperglycemia, and mild kidney pathology by feeding male C57BL/6J mice a high-fat diet (HFD). Cardiac pressure overload was surgically induced using transverse aortic constriction (TAC). Normal diet (ND) and sham controls were included. Heart failure risk factors were evident at 8-wk post-TAC, including increased left ventricular mass (+49% in ND and +35% in HFD), cardiomyocyte hypertrophy (+40% in ND and +28% in HFD), and interstitial and perivascular fibrosis (Masson's trichrome and picrosirius red positivity). High-fat feeding did not exacerbate the TAC-induced cardiac outcomes. At 11 wk post-TAC in a separate mouse cohort, echocardiography revealed reduced left ventricular size and increased left ventricular wall thickness, the latter being evident in ND mice only. Systolic function was preserved in the TAC mice and was similar between ND and HFD. Thus combined high-fat feeding and TAC in mice did not model the increased incidence of heart failure in DM patients. This model, however, may mimic the better cardiovascular prognosis seen in overweight and obese heart failure patients.

Purpose: To assess the spectrum of perivenular ischemia in eyes with retinal vascular obstruction (typically central or hemicentral retinal vein obstruction) using en face optical coherence tomography (OCT)., Design: Retrospective observational case series., Methods: Eyes with recent retinal vascular occlusion illustrating paracentral acute middle maculopathy (PAMM) in a perivenular fern-like pattern with en face OCT were evaluated in this study. Multimodal retinal imaging including en face OCT segmentation of the inner nuclear layer was performed in all patients. Color fundus photography and fluorescein angiography (FA) images were used to create a vascular overlay of the retinal veins vs the retinal arteries to map the distribution of PAMM with en face OCT analysis., Results: Multimodal retinal imaging was performed in 11 eyes with acute retinal vascular obstruction. While 7 eyes demonstrated obvious findings of retinal vein obstruction (5 with central and 2 with hemicentral retinal vein occlusion), 4 eyes were unremarkable at presentation. En face OCT analysis demonstrated a spectrum of perivenular PAMM illustrating a fern-like pattern with sparing of the periarteriolar area in all cases., Conclusion: En face OCT may illustrate a remarkable perivenular pattern of PAMM in eyes with retinal vascular obstruction even in the absence of significant funduscopic findings. Perivenular PAMM with en face OCT demonstrates a wide spectrum of variation with narrow fern-like perivenular lesions at the mildest end and more diffuse lesions with only periarterial sparing at the most severe end of the spectrum. Arterial hypoperfusion secondary to outflow obstruction from a central retinal vein obstruction appears to be the most common cause of this presentation, although primary arterial hypoperfusion may also be an etiology., (Published by Elsevier Inc.)

Purpose: To describe a case of an isolated Group 3 retinal arteriovenous malformation (AVM)., Methods: Observational case report., Results: A 15-year-old girl with no significant medical history presented with no light perception vision in her right eye and gradually decreasing vision in her left eye over several years. She was found to have a large AVM in the posterior pole of the right eye with associated perivascular sheathing in the periphery. Her left eye seemed totally unremarkable. Neuroimaging revealed no intracranial or intraorbital abnormalities. A diagnosis of isolated Group 3 retinal AVM was made., Conclusion: Retinal AVMs are congenital, nonhereditary, and sporadic vascular anomalies that develop in the posterior pole. A number of patients with this finding can have coexisting AVMs in the brain, mandible, skin, or orbit, which comprises the Wyburn-Mason syndrome. Because of these systemic associations, neuroimaging should always be considered when a retinal AVM is seen. Severe vision loss can occur from these AVMs secondary to a number of ocular complications, including central retinal vein occlusions.