------. 2 o A girl born at 30 weeks and 5 days5 and weeks 30 at born girl A linear structures that may representmay that structures linear hyperreflective and clefts, cholesterol bemay that fluid subretinal in dots seenthose to similar macrophages chorioretinopathy.serous central in studiesdemonstrate these Together, evalua the in OCT using of benefit the disease.Coats with patients of tion CASE N g1,560 weighing and age gestational anes under examination underwent reti for screening after (EUA) thesia Retinal(ROP). prematurity of nopathy membranesrevealed examination vascular and disc optic the overlying indetachments retinal causing arcades, vascuretinal anomalous and eye, each eye. each in disease plus without lature neo and avascularity diffuse showed FA 2).(Figure eye each in vascularization they may represent lipids, inflammatorylipids, represent may they alsostudy This cells. blood red or cells, histopath with findings OCT correlated hyperreflectiveincluding analysis, ologic C

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- Gupta et al found al et Gupta 1,2 Ong et al also documented the ben the documented also al et Ong Recent studies have demonstratedhave studies Recent B OCT. These dots are of unclear etiology;unclear of are dotsThese OCT. thatspeculated authorsthe however, OCT correlated with visual acuity, visualacuity, visual with correlated OCT staging. disease clinical and prognosis, funduswith along OCT using of efits disease. Coats in FA and photography formcan exudates that found They particularlyretina, the of layers all in nuclearouter the of half upper the in pat star macular a with eyes in layer describedalso They exudation. of tern aboveatrophy retinal outer extensive ofpresence the and nodules fibrotic ondots hyperreflective preretinal small the ellipsoid zone and the externalthe and zone ellipsoid the membrane.limiting managementthe in OCT of utility the disease. Coats of abnormali retinal microstructural that intraretinalas such disease, Coats in ties bemay fluid, subretinal and edema OCTwith frequently more identified Theseexamination. clinical with than onseen abnormalities microstructural - - - - Case studies illustrate the utility of new imaging technologies in children. new imaging utility of studies illustrate the Case MSC, FACS MD; AND R.V. PAUL CHAN, MD, BY MICHAEL J. HEIFERMAN,

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A dus examination (B), and FA (C) helped clinicians (A), fundus examination (B), and FA (C) helped clinicians Figure 1. A 3-year-old patient presented to the clinic with no light perception in his right eye. External examination identify a . A 3-year-old boy was referred forreferred was boy 3-year-old A The cases we describe in this articlethis in describe we cases The ation of pediatric patients. Givenpatients. retina pediatric of ation pediatrics,in challenges unique the aug in helpful particularly is imaging clinicaland histories patient menting magingevalu the of part integral an is Examination revealed an exudativean revealed Examination fluoresceinand detachment, retinal multipleshowed (FA) angiography demon OCT aneurysms. light-bulb fluid subretinal and intraretinal strated ofdisruption as well as exudates, and of retinal imaging in pediatric patients.pediatric in imaging retinal of CASE N percep light no with 1).(Figure eye right his in vision tion on clinical examination and to monitorto and examination clinical on forguidance provide activity, disease toola as serve and decisions, treatment families.their and patients educating for usespractical the of some demonstrate examinations in this patient population.patient this in examinations helpcan patients pediatric in Imaging seennot pathology detect to clinicians RETINAL IMAGING RETINAL UPDATES IN PEDIATRIC PEDIATRIC IN UPDATES I RETINA TODAY

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in the right eye. The patient was born A B full-term with normal birth weight and had no history of ocular trauma, sur- gery, systemic illness, or similar family history. Examination revealed a large peripapillary subretinal lesion with asso- ciated retinal edema (Figure 3). OCT confirmed subretinal hyperreflective material with associated intraretinal and subretinal fluid. FA demonstrated vascularization of the lesion with leak- C D age. OCT angiography (OCTA) was performed to show flow within the sub- retinal hyperreflective material. A diag- nosis of choroidal neovascularization (CNV) was made, and serial anti-VEGF intravitreal injections were performed, with subsequent improvement in the intraretinal and subretinal fluid. Ong et al reported eight patients with pediatric CNV evaluated with OCTA.7 Figure 2. During examination for ROP, a patient was found to have retinal detachment in her right (A) and left eyes These authors compared active and (B). Diffuse avascularity and neovascularization were noted in the patient’s right (C) and left eyes (D). quiescent CNV and demonstrated the presence of fine capillaries, anastomoses, Gupta et al reported a series of three pediatric patients. They also speculated and vessel loops in active cases. These moderately premature neonates with that subclinical retinal vasculitis may findings may be helpful in monitoring for aggressive posterior vitreoretinopathy contribute to the worse prognosis in CNV recurrence and may indicate when (APVR).3 This entity, more consistent pediatric posterior-involving . repeat FA and treatment is appropriate. with familial exudative vitreoretinopathy Chee et al reported on the safety of Another potential role for OCTA is (FEVR) than with ROP, is characterized FA specifically in the pediatric popula- in screening for retinal pathology. Alam by the presence of severe retinal pathol- tion.6 These authors found no significant et al used machine learning–based ogy despite relatively older gestational adverse events during 214 FAs and no artificial intelligence to differentiate age at birth and larger birth weight. change in 5-minute preinjection and and stage OCT angiograms of patients APVR is characterized by an absence postinjection physiologic parameters in with nonproliferative diabetic retinopa- of prominent plus disease and is more the 27 FAs that were conducted during thy and sickle cell .8 Using aggressive than the entity known as EUA. The most common inpatient diag- their screening tool, they were able to ROPER (also called ROP vs FEVR).4 EUA nosis was ROP followed by Coats dis- quantify blood vessel tortuosity, caliber, with FA should be considered early ease, FEVR, and nonaccidental trauma. density, and branch-point geometry, as and can help distinguish this entity The most common outpatient diagnosis well as the foveal avascular zone con- from ROP. was Coats disease, followed by FEVR and tour irregularity and area. Using these The role of FA in pediatric retina sickle cell retinopathy. They found that OCTA parameters, their screening tool patients has been an area of recent inves- FAs were done as inpatient procedures achieved 94.84% sensitivity to identify tigation in other disorders. Abraham et more often for younger patients, given retinopathy from the general pool of al evaluated pediatric patients with pos- patient cooperation issues and the controls. These findings support the terior-involving uveitis on immunosup- need to coordinate with intervention use of artificial intelligence in screen- pressive therapy deemed quiescent on under anesthesia. ing vulnerable groups without access clinical examination.5 They performed These studies, taken together, demon- to retinal specialists, including the FA on these patients and found that strate the safety and importance of FA in pediatric population. 11 of 14 (79%) had persistent subclini- managing pediatric retina patients. The role of OCTA in the manage- cal inflammation requiring a change in ment of pediatric retinal patients their immunosuppressive therapy. These CASE No. 3 remains to be fully explored, but the authors emphasized the importance A 9-year-old girl with an studies noted here contribute to the of FA for monitoring disease activity in was found to have counting fingers VA limited volume of knowledge to date.

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. Graefes Arch J Clin Med Ophthalmol Retina Retina Today . 2020;258:89-98. . 2020;4(6):595-601. [email protected] Financial disclosure: Consultant/advisor (Alcon, Fellow, University of Illinois College of Medicine [email protected] Financial disclosure: None The John H. Panton, MD, Professor of of The John H. Panton, MD, Professor Editorial Advisory Board Member, Novartis), Scientific advisory board (Phoenix Novartis), Scientific advisory board (Phoenix Technology), Grant support (Novartis, Alcon [XOVA], National Institutes of Health, National Science Foundation, Research to Prevent Blindness, iNsight Foundation, Blind Children’s Center, United States Agency for International Development) ; Vice Chair, Global Ophthalmology; Global Ophthalmology; Ophthalmology; Vice Chair, ROP Service; and Director, Pediatric Retina and Co-Director, Vitreoretinal Fellowship, all at Illinois Eye and Ear Infirmary, Department of Ophthalmology and Visual Sciences, University of Illinois at Chicago      n n MICHAEL J. HEIFERMAN, MD n n n 5. Abraham A, Saboo US, Ducca BL, et al. The detection of occult retinal US, Ducca BL, et al. The detection of occult 5. Abraham A, Saboo angiography in pediatric uveitis. vasculitis on fluorescein 2020;4:198-203. systemic Valikodath NG, et al. Evaluation of potential 6. Chee RI, Gupta MP, patients. to in pediatric adverse events related Ophthalmol Retina of pediatric choroidal neovas 7. Ong SS, Hsu ST, Grewal D, et al. Appearance angiography. cular membranes on optical coherence tomography Clin Exp Ophthalmol machine learning based 8. Alam M, Le D, Lim JI, et al. Supervised of . multi-task artificial intelligence classification 2019;8(6):pii:E872. FACS R.V. PAUL CHAN, MD, MSC, n n - - . JAMA Ophthalmol . 2019;50:201-207. . 2019;39:1177-1185. . 2016;47:14-19. Retina n Ophthalmic Surg Lasers Imaging Retina B 1. Gupta MP, Dow E, Jeng-Miller KW, et al. Spectral domain optical coherence tomography findings in Coats disease. 2. Ong SS, Cummings TJ, Vajzovic L, et al. Comparison of optical coherence tomography with fundus photographs, fluorescein angiography, and histopathologic analysis in assessing Coats disease. 2019;137:176-183. 3. Gupta MP, Yonekawa Y, Campbell JP, et al. Early diagnosis and manage ment of aggressive posterior vitreoretinopathy presenting in premature neonates. 4. John VJ, McClintic JI, Hess DJ, et al. Retinopathy of prematurity versus famil ial exudative vitreoretinopathy: report on clinical and angiographic findings. Ophthalmic Surg Lasers Imaging Retina treatment algorithms and to improveto and algorithms treatment care. patient - - C A Figure 3. A large peripapillary subretinal lesion was detected in a 9-year-old patient with counting fingers vision in a 9-year-old patient with counting fingers vision Figure 3. A large peripapillary subretinal lesion was detected subretinal hyperreflective material was observed on (A). Vascularization of the lesion was noted on FA (B), and OCTA (C). Pediatric retinal imaging has becomehas imaging retinal Pediatric color fundus photography, FA, OCT,FA, photography, fundus color includingtechniques imaging new and modalitiesimaging novel These OCTA. pedi redefine to potential the have andclassifications disease retinal atric CONCLUSION CONCLUSION develop the with research of focus a andmodalities imaging new of ment pediatricMany applications. clinical toaccess have now specialists retina

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