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Updates in Pediatric Retinal Imaging s IMAGING UPDATES IN PEDIATRIC RETINAL IMAGING Case studies illustrate the utility of new imaging technologies in children. BY MICHAEL J. HEIFERMAN, MD; AND R.V. PAUL CHAN, MD, MSC, FACS maging is an integral part of the evalu- the ellipsoid zone and the external they may represent lipids, inflammatory ation of pediatric retina patients. Given limiting membrane. cells, or red blood cells. This study also the unique challenges in pediatrics, Recent studies have demonstrated correlated OCT findings with histopath- imaging is particularly helpful in aug- the utility of OCT in the management ologic analysis, including hyperreflective menting patient histories and clinical of Coats disease.1,2 Gupta et al found linear structures that may represent Iexaminations in this patient population. that microstructural retinal abnormali- cholesterol clefts, and hyperreflective Imaging in pediatric patients can help ties in Coats disease, such as intraretinal dots in subretinal fluid that may be clinicians to detect pathology not seen edema and subretinal fluid, may be macrophages similar to those seen on clinical examination and to monitor identified more frequently with OCT in central serous chorioretinopathy. disease activity, provide guidance for than with clinical examination. These Together, these studies demonstrate treatment decisions, and serve as a tool microstructural abnormalities seen on the benefit of using OCT in the evalua- for educating patients and their families. OCT correlated with visual acuity, visual tion of patients with Coats disease. The cases we describe in this article prognosis, and clinical disease staging.1 demonstrate some of the practical uses Ong et al also documented the ben- CASE NO. 2 of retinal imaging in pediatric patients. efits of using OCT along with fundus A girl born at 30 weeks and 5 days photography and FA in Coats disease.2 gestational age and weighing 1,560 g CASE NO. 1 They found that exudates can form underwent examination under anes- A 3-year-old boy was referred for in all layers of the retina, particularly thesia (EUA) after screening for reti- leukocoria with no light percep- in the upper half of the outer nuclear nopathy of prematurity (ROP). Retinal tion vision in his right eye (Figure 1). layer in eyes with a macular star pat- examination revealed membranes Examination revealed an exudative tern of exudation. They also described overlying the optic disc and vascular retinal detachment, and fluorescein extensive outer retinal atrophy above arcades, causing retinal detachments in angiography (FA) showed multiple fibrotic nodules and the presence of each eye, and anomalous retinal vascu- light-bulb aneurysms. OCT demon- small preretinal hyperreflective dots on lature without plus disease in each eye. strated intraretinal and subretinal fluid OCT. These dots are of unclear etiology; FA showed diffuse avascularity and neo- and exudates, as well as disruption of however, the authors speculated that vascularization in each eye (Figure 2). A B C Figure 1. A 3-year-old patient presented to the clinic with no light perception in his right eye. External examination (A), fundus examination (B), and FA (C) helped clinicians identify a retinal detachment. 38 RETINA TODAY | JULY/AUGUST 2020 IMAGING s 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 uveitis. 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 retinopathy.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 exophoria 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. JULY/AUGUST 2020 | RETINA TODAY 39 s IMAGING 5. Abraham A, Saboo US, Ducca BL, et al. The detection of occult retinal A B vasculitis on fluorescein angiography in pediatric uveitis. Ophthalmol Retina. 2020;4:198-203. 6. Chee RI, Gupta MP, Valikodath NG, et al. Evaluation of potential systemic adverse events related to fluorescein angiography in pediatric patients. Ophthalmol Retina. 2020;4(6):595-601. 7. Ong SS, Hsu ST, Grewal D, et al. Appearance of pediatric choroidal neovas- cular membranes on optical coherence tomography angiography. Graefes Arch Clin Exp Ophthalmol. 2020;258:89-98. 8. Alam M, Le D, Lim JI, et al. Supervised machine learning based multi-task artificial intelligence classification of retinopathies. J Clin Med. 2019;8(6):pii:E872. R.V. PAUL CHAN, MD, MSC, FACS C n The John H. Panton, MD, Professor of Ophthalmology; Vice Chair, Global Ophthalmology; Director, Pediatric Retina and ROP Service; and Co-Director, Vitreoretinal Fellowship, all at Illinois Eye and Ear Infirmary, Department of Ophthalmology and Visual Sciences, University of Illinois at Chicago n Editorial Advisory Board Member, Retina Today Figure 3. A large peripapillary subretinal lesion was detected in a 9-year-old patient with counting fingers vision n (A). Vascularization of the lesion was noted on FA (B), and subretinal hyperreflective material was observed on [email protected] OCTA (C). n Financial disclosure: Consultant/advisor (Alcon, Novartis), Scientific advisory board (Phoenix Technology), Grant support (Novartis, Alcon CONCLUSION treatment algorithms and to improve [XOVA], National Institutes of Health, National Pediatric retinal imaging has become patient care. n Science Foundation, Research to Prevent a focus of research with the develop- Blindness, iNsight Foundation, Blind Children’s ment of new imaging modalities and 1. Gupta MP, Dow E, Jeng-Miller KW, et al. Spectral domain optical coherence tomography findings in Coats disease.
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