Appearance of pediatric choroidal neovascular membranes on optical coherence tomography angiography

Abstract

Purpose: Compared with fluorescein angiography (FA), the gold standard for diagnosing choroidal neovascularization (CNV) activity, optical coherence tomography angiography (OCTA) is non-invasive without risks associated with fluorescein dye use, and may be especially advantageous in the diagnosis and monitoring of children with CNV.

Methods: Eight eyes from eight patients aged 12 months to 18 years were imaged with the investigational Spectralis OCTA (version 6.9, Heidelberg Engineering, Heidelberg, Germany) and the RTVue XR Avanti (Optovue Inc., Fremont, CA, USA). Two patients were imaged during examination under anesthesia while six patients were imaged in the clinic. Demographic information, ocular characteristics, treatment history, and imaging studies (color photos, fluorescein angiography, OCT) were collected and reviewed.

Results: Three eyes had active CNV while five had quiescent CNV at the time of imaging. CNV was idiopathic or secondary to trauma, retinal vascular dysgenesis versus retinopathy of prematurity, pigmentary retinopathy, Best vitelliform macular dystrophy, panuveitis, morning glory disc anomaly, and optic disc drusen. OCTA of two active CNV demonstrated presence of a main trunk with multiple fine capillaries, vessel loops, and anastomoses. OCTA was repeated after treatment for two CNV and demonstrated a decrease in size with loss of fine capillaries, vessel loops, and anastomoses. For the third active CNV, OCTA verified flow in the CNV complex despite the uncertainty of FA hyperfluorescence in the setting of grossly abnormal retinal vasculature. The five quiescent CNV all lacked fine capillaries, vessel loops, and anastomoses on OCTA.

Conclusion: OCTA demonstrates morphological differences between active and quiescent pediatric CNV.

Keywords: CNV in children; OCTA; Optical coherence tomography angiography; Pediatric choroidal neovascularization.

Fig. 1

Type 1 idiopathic CNV in patient 1. a At presentation, color photo demonstrates a hyperpigmented CNV complex with hemorrhage just inferonasal to the foveal center. b OCT shows the subretinal pigment epithelium CNV complex. The patient was treated with intravitreal anti-VEGF injections. c One year after presentation, the patient had a recurrence of CNV activity. c Early and d late frames of fluorescein angiography illustrate occult leakage of the CNV complex, blocked fluorescence from hemorrhage, and circumferential window defect from retinal pigment epithelium atrophy. A feeder vessel is also visible (red arrows). e The green box and green arrow in the infrared image depict the area captured by the en-face (f) and cross-sectional (g) OCTA, respectively. f En-face OCTA shows a large circular CNV complex with a main trunk (yellow arrow), multiple dense thin capillaries branching from the main trunk in a tree-like manner, and frequent anastomoses in the segmented avascular cube. g Cross-sectional OCTA illustrates flow (arrows) in the CNV complex. h En-face and i cross-sectional OCTA were repeated after anti-VEGF therapy and demonstrate a CNV complex that has decreased in size with loss of thin capillaries and vascular loops

Fig. 2

Type 2 traumatic CNV in patient 2. a Color photo shows a subfoveal CNV complex. b Early and c late frames of fluorescein angiography demonstrate early lacy hyperfluorescence with late leakage typical for classic CNV. A feeding vessel (red arrows) can also be seen. d En-face OCTA demonstrates a CNV complex with a main trunk (yellow arrow) transversing the entire CNV complex with branching capillaries on either side, vessel loops, and anastomoses. e Cross-sectional OCTA shows flow (white arrows) in the CNV. f En-face and g cross-sectional OCTA after two intravitreal bevacizumab injections show a smaller consolidated CNV complex with loss of branching capillaries, vessel loops, and anastomoses

Fig. 3

Type 1 CNV in patient 3 with retinal vascular dysgenesis versus retinopathy of prematurity. At age 2 months, color photos of the a left eye demonstrate vitreous hemorrhage and tractional retinal detachment, and the child underwent vitreoretinal surgery. One month later, she underwent surgery again in the left eye for a recurrent vitreous hemorrhage. At age 12 months, color photo of the b left eye shows attached retina with extensive panretinal photocoagulation endolaser scars. c Early and d late frames of fluorescein angiography in the left eye show two areas of hyperfluorescence in the macula suspicious for leakage (white arrows). e Infrared image with the green box depicting the en-face OCTA shown in f and g and the green arrow depicting the cross-sectional OCTA shown in h. f En-face OCTA of the entire retina shows grossly abnormal vasculature and a possible CNVM complex (red arrow). g Segmentation of the avascular cube was challenging given the abnormal vasculature but shows a possible CNVM complex (red arrow) which is confirmed on h cross-sectional OCTA which shows flow in the pigment epithelial detachment (white arrow)

Fig. 4

Patients 4 to 8. From left to right: fundus photo, late fluorescein angiography, infrared, en-face OCTA, and cross-sectional OCTA. The green box in the infrared image delineates the area captured by the en-face OCTA while the green arrow demonstrates the scan shown by the cross-sectional OCTA. Quiescent CNV complexes in children with pigmentary retinopathy (a), Best vitelliform macular dystrophy (b), panuveitis (c), morning glory disc anomaly (d), and optic nerve drusen (e). The en-face OCTAs show that the CNV complexes in these children lack fine capillaries, vessel loops, and anastomoses while the cross-sectional OCTAs confirmed residual flow in the pre-RPE type 2 (a), sub-RPE type 1 (b, d, e), or mixed with predominantly type 1 component (c) CNV complexes