Retina Imaging Conference Denis Jusufbegovic, M.D. University of Louisville Department of Ophthalmology and Visual Sciences 2/11/16

Case Presentation CC: “ Left eye turning in x few weeks” HPI: 5 yo boy was brought to clinic by parents who noticed that his left eye was turning in for few weeks. No recent illness or trauma. Child had no complaints. After evaluation by a pediatric ophthalmologist, patient was referred to retina clinic.

Case Presentation PMH: Negative, term-baby, vaccination up to date : None POH: None FH: Non-contributory MEDS MEDS: None ALL: NKDA ROS: Negative

Case Presentation BCVA: 20/20 20/400 BCVA: 20/20 20/400 Pupils: 4 No APD 4 Pupils: 4 No APD 4 IOP: 12 mmHg 12 mmHg IOP: 12 mmHg 12 mmHg Anterior segment: normal Anterior segment: normal ODOS

Fundus Photos Right eye is normal. Color fundus photograph of the left eye demonstrates subfoveal hemorrhage..

Fluorescein Angiography FA of the right eye is normal

Fluorescein Angiography FA demonstrates subfoveal choroidal neovascularization in the left ye

OCT

Assessment 5 year-old boy with subfoveal choroidal neovascularization OS. ? Etiology DDXTraumaInfectionInflammationIdiopathic

Management Lab work: RPR negative, Toxocara IgG and IgM negative, Toxoplasma IgG and IgM negative Intravitreal Bevacizumab injection OS Patching (2hrs/day) OD for deprivational amblyopia

Follow up Initial visit 1 mo s/p Avastin 3 mo s/p Avastin #2

3 mo follow up BCVA improved to 20/100 OS

Pediatric CNV Development of CNV in young children is rare Development of CNV in young children is rare CNV lesions and their etiologies significantly differ between children and adults CNV lesions and their etiologies significantly differ between children and adults Most common causes of CNV in adults are AMD and high myopia, which are generally not seen in children Most common causes of CNV in adults are AMD and high myopia, which are generally not seen in children CNV lesions in young children are focal and mostly located in subretinal space (type 2 or classic membranes) CNV lesions in young children are focal and mostly located in subretinal space (type 2 or classic membranes)

Pediatric CNV- Etiology Inflammatory/infectious - most common cause of CNV in children (unclassified focal choroiditis, POHS, toxoplasmosis, toxocara) Inflammatory/infectious - most common cause of CNV in children (unclassified focal choroiditis, POHS, toxoplasmosis, toxocara) Retinal dystrophies - the second common cause identified in several case series was Best’s disease Other dystrophies such as Stargardt disease, choroideremia, North Carolina macular dystrophy may cause CNV Retinal dystrophies - the second common cause identified in several case series was Best’s disease Other dystrophies such as Stargardt disease, choroideremia, North Carolina macular dystrophy may cause CNV Idiopathic - large percentage of CNV lesions in children have no identifiable cause Idiopathic - large percentage of CNV lesions in children have no identifiable cause Traumatic – choroidal rupture Traumatic – choroidal rupture Others – ON drusen, angioid streaks, choroidal osteoma, combined hamartoma of the retina and RPE Others – ON drusen, angioid streaks, choroidal osteoma, combined hamartoma of the retina and RPE

Management Anti-VEGF is the mainstay therapy for CNV Anti-VEGF is the mainstay therapy for CNV Kozak I et al. Outcomes of treatment of pediatric choroidal neovascularization with intravitreal antiangiogenic agents: the results of the KKESH International Collaborative Retina Study Group. Retina Oct;34(10): Retrospective review of 39 cases (45 eyes) Majority treated with Avastin, few with Lucentis Mean and median number of injections was 2.2 and 1 22 eyes (48%) gained more than 3 lines 27 eyes (60%) had final visual acuity 20/50 or better (20%) did not improve and had severe vision loss (20/200 or worse) No systemic side-effects reported

Thank you

References 1. Rishi P, Gupta A, Rishi E, Shah BJ. Choroidal neovascularization in 36 eyes of children and adolescents. Eye (Lond) Oct;27(10): Kozak I et al. Outcomes of treatment of pediatric choroidal neovascularization with intravitreal antiangiogenic agents: the results of the KKESH International Collaborative Retina Study Group. Retina Oct;34(10): Kohly RP et al. Management of pediatric choroidal neovascular membranes with intravitreal anti-VEGF agents: a retrospective consecutive case series. Can J Ophthalmol Feb;46(1): Wilson ME, Mazur DO. Choroidal neovascularization in children: report of five cases and literature review. J Pediatr Ophthalmol Strabismus Jan-Feb;25(1):23-9. Review.