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Presumed retinal pericapillary astrocytic hamartoma: multimodal imaging findings of a novel hamartomatous lesion

Ledesma-Gil, Gerardo; Essilfie, Juliet; Onishi, Alex; Wald, Kenneth J; Fisher, Yale L; Fawzi, Amani A; Shields, Carol L; Freund, K Bailey; Chhablani, Jay
PURPOSE/OBJECTIVE:To describe the multimodal imaging findings of retinal lesions that clinically resemble retinal astrocytic hamartomas (RAHs), but also have unique characteristics that we believe represent a novel variant. METHODS:Observational study. Five eyes in five patients with solitary retinal lesion evaluated at the retina division of three institutions. We describe the multimodal imaging findings including fundus photography, fundus autofluorescence, fluorescein angiography, spectral-domain optical coherence tomography (OCT), swept-source OCT, swept-source OCT angiography and ultrasonography. RESULTS:The retinal lesions described shared similar appearance to RAHs but demonstrated unique features such as glistening granular appearance on fundus photographs with perivascular hyperreflectivity with OCT and OCT angiography. CONCLUSION/CONCLUSIONS:The lesions described herein appear to have unique characteristics that warrant a designation as a novel RAH variant. The name presumed retinal pericapillary astrocytic hamartoma is suggested.
PMID: 32980819
ISSN: 1468-2079
CID: 4616332

Detection of Occult Arteriovenous Malformation With Annular Array Ultrasonography

Ledesma-Gil, Gerardo; Essilfie, Juliet; Freund, K Bailey; Yannuzzi, Lawrence A; Fisher, Yale L
Retinal vascular tortuosity may occur in a wide range of ocular disorders. When retinal vascular tortuosity involves both arteries and veins, and presents unilaterally and without hemorrhage, a diagnosis of Wyburn Mason syndrome (WMS) should be considered due to the potential morbidity and mortality associated with cerebral involvement. Magnetic resonance imaging (MRI) and MRI angiography (MRA) are important tools for identifying cerebral arteriovenous malformations (AVMs), but these imaging modalities have limited spatial resolution to detect very small vascular lesions. Annular array contact ocular ultrasound is a new imaging modality capable of detecting small intraorbital AVMs. [Ophthalmic Surg Lasers Imaging Retina. 2020;51:239-243.].
PMID: 32348541
ISSN: 2325-8179
CID: 4412422

Multimodal Imaging of Punctate Outer Retinal Toxoplasmosis

Yannuzzi, Nicolas A; Gal-Or, Orly; Motulsky, Elie; Swaminathan, Swarup S; Cunningham, Emmett T; Davis, Janet L; Fisher, Yale; Gregori, Giovanni; Rosenfeld, Philip J; Freund, K Bailey
BACKGROUND AND OBJECTIVE/OBJECTIVE:To describe the multimodal imaging characteristics associated with punctate outer retinal toxoplasmosis (PORT). PATIENTS AND METHODS/METHODS:Multicenter, retrospective, observational case series of three patients who presented with PORT. Multimodal imaging was reviewed including optical coherence tomography (OCT), fundus autofluorescence, optical coherence tomography angiography, and conventional dye-based angiography. RESULTS:Patient ages ranged from 13 years to 55 years. Each patient had multiple white, punctate outer retinal lesions in the affected eye at initial diagnosis. OCT showed both inner and outer retinal changes, including disruption of the ellipsoid and interdigitation zones and retinal pigment epithelium/Bruch's membrane complex, as well as punctate, preretinal, hyperreflective lesions at the vitreoretinal interface, which regressed with treatment. CONCLUSION/CONCLUSIONS:Multimodal imaging is useful in diagnosing and monitoring treatment response in PORT, an uncommon presentation of ocular toxoplasmosis that must be differentiated from white dot syndromes or other causes of unilateral retinitis. [Ophthalmic Surg Lasers Imaging Retina. 2019;50:281-287.].
PMID: 31100158
ISSN: 2325-8179
CID: 3920072

PRESUMED RETINAL PIGMENT EPITHELIUM TUMOR ORIGINATING FROM UNILATERAL RETINAL PIGMENT EPITHELIUM DYSGENESIS

Gal-Or, Orly; Finger, Paul T; Fisher, Yale L; Yannuzzi, Lawrence A; Freund, K Bailey
PURPOSE: To describe a patient with a presumed retinal pigment epithelium (RPE) tumor originating from unilateral RPE dysgenesis. METHODS: Case report. RESULTS: A 30-year-old woman with an unremarkable medical and ocular history was referred for an evaluation of progressive central metamorphopsia in her left eye. Visual acuity was 20/20 in her right eye and 20/25 in her left eye. Funduscopic examination of the left eye revealed an elevated mass within an area of unilateral RPE dysgenesis showing hyperpigmentation and hypopigmentation with scalloped margins. Fundus autofluorescence of the lesion showed a marginal pattern of hyperautofluorescence and hypoautofluorescence that was the inverse of the fluorescein angiography pattern. A well-circumscribed subretinal mass appeared to originate from the unilateral RPE dysgenesis lesion with surrounding subretinal fluid extending beneath the fovea. Ultrasonography showed medium-to-high reflectivity of the mass with no evidence of choroidal involvement. Optical coherence tomography showed a subretinal hyporeflective mass consistent with a tumor of RPE origin. The tumor appeared to invade the overlying retina where fluorescein angiography showed hyperfluorescent leakage and OCT angiography showed retinal vascular deformation. Indocyanine green angiography showed no evidence of choroidal neovascularization. Findings in the right fundus were normal. Over a 5-month follow-up, intravitreal anti-vascular endothelial growth factor therapy induced a resolution of subretinal exudation and modest reduction in tumor thickness. CONCLUSION: To the authors' knowledge, this is the first report of a presumed RPE tumor described as originating from unilateral RPE dysgenesis. Multimodal imaging was crucial for establishing the diagnosis and showing that the patient's visual symptoms were a product of the exudation produced by the tumor's invasion of the retina.
PMID: 28333854
ISSN: 1937-1578
CID: 2499572

Diagnostic and Therapeutic Challenges

Ahmad, Meleha; Dolz-Marco, Rosa; Fisher, Yale L; Yannuzzi, Lawrence A; Zhang, Wenlan; Materin, Miguel; Jaffe, Glenn J
PMID: 28991861
ISSN: 1539-2864
CID: 3067652

40TH MEETING OF THE ATLANTIC COAST RETINA CLUB MACULA 2016 [Meeting Abstract]

Fisher, Yale L; Yannuzzi, Lawrence A
PMID: 28005717
ISSN: 1937-1578
CID: 3095912

Real-Time In-Vivo Correlation of Retained Vitreoretinal Adjuvants Poly(Dimethyl siloxane) and Perflouro-n-octane by Handheld High-Resolution B-Scan Ultrasound Biomicroscopy [Meeting Abstract]

McCann, Jesse T; Bala, Chandra; Fisher, Yale
ISI:000394174004085
ISSN: 0146-0404
CID: 2507092

Panuveitis With Exudative Retinal Detachments After Vaccination Against Human Papilloma Virus

Dansingani, Kunal K; Suzuki, Mihoko; Naysan, Jonathan; Samson, C Michael; Spaide, Richard F; Fisher, Yale L
A 20-year-old white woman presented with bilateral acute visual loss (visual acuity: 20/60), panuveitis, and exudative retinal detachments 3 weeks after a second dose of quadrivalent human papillomavirus (HPV4) vaccine. She was treated with oral prednisolone for 6 weeks and responded rapidly. By week 4, vision had normalized and clinical signs resolved. Uveitis after HPV4 vaccination has been reported in two cases. Although the differential diagnosis includes Harada disease, temporal correlation with HPV4 and definitive response to a short course of treatment implicate the vaccine in this case. Vaccine-induced uveitis is rare and difficult to distinguish from coincidental autoimmune disease. [Ophthalmic Surg Lasers Imaging Retina. 2015;46:967-970.].
PMID: 26469238
ISSN: 2325-8179
CID: 1839682

Dissecting the Intravitreal Injection: The Role of Silicones [Meeting Abstract]

McCann, Jesse T; Fisher, Yale; Kiss, Szilard; Nemiroff, Samuel
ISI:000362891102375
ISSN: 0146-0404
CID: 2274992

Direct visualization of silicone oil removed from vitrectomized eyes with transmission electron microscopy reveals microemulsions and nanoemulsions [Meeting Abstract]

McCann, J T; Fisher, Y
Purpose Poly(dimethylsiloxane) polymers (commonly known as silicone oil) have been frequently used in retinal surgery as a tamponade. Emulsification of these oils has been a difficult and persistent problem. Persistence of the oil following vitrectomy with fluid exchange has been confirmed by contact B-scan ultrasonography. In addition, infiltration of the silicone oil into the retina and optic nerve has been demonstrated by SD-OCT and adaptive optics imaging. This study intends to directly visualize the emulsions formed by silicone oil used in retinal surgery by multimodal imaging. Methods Silicone oil that was removed from eyes by fluid exchange and vitrectomy having been used as a surgical tamponade was subjected to chemical and electron micrographic analysis. 0.1 mL of silicone oil was washed with 2 mL of sterile irrigating solution (BSS, Alcon, TX). The mixture was then vortexed for 60 s and allowed to sit at 25 degreeC for 24 hours. The supernatant was decanted and placed on carbon-coated copper grids for transmission electron microscopy (TEM) and cryo-TEM analysis. Results BSS was suficient to emulsify the removed silicone oil. Transmission electron microscopy revealed nano-size (<100 nm) and micron-sized (<1 mum) oil-in-water emulsions. These emulsions are several orders of magnitude smaller than previously reported. These droplet sizes are similar to the size of silicone oil droplets that have been found in the tissues of the retina and optic nerve using optical coherence tomography and adaptive optics imaging. Conclusions This demonstrates that silicone-soluble components of the eye partition into the silicone oil after its instillation as a surgical tamponade. These silicone-soluble components are alone suficient to cause emulsification. The equilibration of the silicone oil with lipid-soluble components in the eye is consistent with phase-equilibrium behavior. Further analysis of the silicone oil to determine the identity of the emulsifying agents is ongoing
EMBASE:616118484
ISSN: 0146-0404
CID: 2565502