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Ocular melanocytoma in children. Analysis of 25 cases
Volume 21 Number 4 / August 2017 034 Ocular melanocytoma in children. Analysis of 25 cases. Jerry A. Shields, Carol L. Shields Introduction: Ocular melanocytoma is a type of magnocellular nevus that can closely resemble melanoma. It can occur in the uvea or optic disc as a circumscribed pigmented lesion, typically in adults. In this report, we describe the clinical variations and outcomes of this rare tumor in children. Methods: Retrospective chart review of children (\21 years) with ocular melanocytoma. Results: The melanocytoma mainly involved iris (n 5 12) or optic disc (n 5 13). Of the 12 children with iris melanocytoma, mean age at diagnosis was 12 years, race was Caucasian (n 5 11), and features included symptoms of spot on iris (n 5 6), blurred vision (n 5 1), photophobia (n 5 1), and findings of a black iris mass with granular surface (n 5 12) of mean 4 mm diameter ciliary body extension (n 5 2), oculo(dermal) melanocytosis (n 5 2), and stromal (n 5 8) and angle (n 5 7) seeding. There was no case of secondary glaucoma. Outcomes included tumor enlargement (n 5 5) by mean 1 mm, vision loss (n 5 2), and development of melanoma (n 5 2) at mean 16 months follow-up. Of those 13 with optic disk melanocytoma, mean age was 14 years, race was Caucasian (n 5 8), and features included symptoms of chronic headache (n 5 2), blurred vision (n 5 2), photopsia (n 5 1), and findings of oculo(dermal) melanocytosis (n 5 5), pigmented optic disk tumor (n 5 13) of median diameter of 2 mm, and disc edema/pallor (n 5 5). Outcomes included tumor enlargement (n 5 2) by mean 0.5 mm, vision loss (n 5 2) and no case of retinal vascular obstruction or development of melanoma. There was 1 case of iris/optic disc melanocytoma with massive choroidal melanocytoma in a child with ocular melanocytosis. Discussion: Ocular melanocytoma can be associated with congenital ocular melanocytosis and both can predispose to melanoma even at a young age. Conclusions: Ocular melanocytoma can occur in the iris, ciliary body, choroid, and optic disk and can lead to vision loss with rare transformation into melanoma. 035 Evaluation of a telemedicine program for pediatric diabetic retinopathy. Sasha Strul, Sean Donahue, Karishma Datye, William Russell Introduction: Annual screening for pediatric diabetic retinopathy (DR) is recommended by the ADA for type 1 diabetes starting at age 10, once duration of diabetes is over 3-5 years, and starting at diagnosis of type 2. We instituted a telemedicine program for pediatric DR and analyzed epidemiologic and disease factors of screened children. Methods: Retrospective chart review. Results: Over 1 year, 494 childreen meeting ADA criteria for ophthalmic evaluation had non-mydriatic fundus screening in the diabetes clinic in an ongoing program. Prevalence of diabetic retinopathy was 8.3%. The first twenty-nine patients with abnormal results were compared to 35 randomly selected normals. Duration of diabetes (median of 12 and 7 years in the abnormal and normal groups respectively, P 5 0.003) and age (median of 17 and 16 respectively, P 5 0.04) were different in univariate analysis (Wilcoxon test). Average A1c, duration of disease, age and sex showed some importance as predictors of an abnormal screening, but none reached statistical significance in a penalized logistic regression model, likely due to limited power.
Journal of AAPOS
e13 Discussion: The prevalence of diabetic retinopathy in children screened is substantially higher than that previously reported from retrospective reviews of eye clinic records, likely due to poor compliance of most-at-risk children with formal eye examination. This program has decreased the number of children needing eye examinations by 90% compared to ADA recommendations. Conclusions: Telemedicine is an attractive and cost-effective means of evaluating children at risk for diabetic retinopathy. 036 Retinopathy of prematurity twin concordance in the G-ROP Study. Lauren A. Tomlinson, Gui-shuang Ying, Gil Binenbaum, on behalf of the G-ROP Study group Introduction: We sought to determine concordance of retinopathy of prematurity (ROP) between premature twins and identify risk factors for inter-sibling discordance. Methods: Retrospective cohort study of twin pairs receiving ROP examinations at 30 North-American hospitals between 2006 and 2012 (G-ROP Study). Higher-order multiples (triplets,etc.) were excluded. Outcomes were proportions of twin pairs who were concordant for any ROP and for severe ROP (ETROP type 1 or 2). Birth weight (BW) and comorbidities of prematurity were evaluated as risk factors for discordance. Results: Of 7,483 infants, 663 twin pairs (1,326 infants) were studied; median GA 28 wks(range, 23-35), mean BW 1172 g (SD, 349 g), mean BW-difference between twins 179 g (SD, 180 g; range, 0-1140 g). 110 (8.3%) infants developed severe ROP. Overall twin-pair concordance was 80.3% (kappa 5 0.59) for ROP and 92.5% (kappa 5 0.50) for severe ROP. Severe-ROP-concordance was similar regardless of percentage difference in BW (90%-95%, kappas 5 0.47-0.58). In 17/21 twin pairs discordant for both sepsis/NEC and severe ROP, the infant with sepsis/NEC had severe ROP (10 larger-twin, 7 smaller-twin). Nine of these 21 pairs had high discordance (one twin had severe ROP, the other twin had no ROP); in 8/9 of twin pairs with high discordance, the twin with severe ROP had sepsis and/or surgical NEC but the sibling did not. Discussion: Twin siblings develop severe ROP with high concordance, generally irrespective of BW difference. Discordance is associated with systemic infection in the twin with severe ROP. Conclusions: Ophthalmologists examining twins can expect similar ROP diagnoses but should suspect discordance when one infant develops sepsis or NEC. 037 Visual improvement in amblyopic eye following radiationinduced vision loss in dominant eye with uveal melanoma. Aldo Vagge, Bruce M. Schnall, Renelle P. Lim, Jerry A. Shields, Carol L. Shields Introduction: Visual improvement in amblyopic eyes of adults can occur following visual loss in the dominant eye. The purpose of this study is to determine the frequency and amount of this improvement. Methods: Retrospective review of adult patients with visual loss following radiotherapy for uveal melanoma with a history of amblyopia in the fellow eye. Best corrected visual acuity (BCVA) of treated and amblyopic eye were examined over time. BCVA improvement was defined as $ 2 logMAR lines. The patients were divided into 2 groups dependent on the severity of visual loss in the treated melanoma eye (BCVA melanoma eye . or # amblyopic eye).
Journal of AAPOS
e13 Discussion: The prevalence of diabetic retinopathy in children screened is substantially higher than that previously reported from retrospective reviews of eye clinic records, likely due to poor compliance of most-at-risk children with formal eye examination. This program has decreased the number of children needing eye examinations by 90% compared to ADA recommendations. Conclusions: Telemedicine is an attractive and cost-effective means of evaluating children at risk for diabetic retinopathy. 036 Retinopathy of prematurity twin concordance in the G-ROP Study. Lauren A. Tomlinson, Gui-shuang Ying, Gil Binenbaum, on behalf of the G-ROP Study group Introduction: We sought to determine concordance of retinopathy of prematurity (ROP) between premature twins and identify risk factors for inter-sibling discordance. Methods: Retrospective cohort study of twin pairs receiving ROP examinations at 30 North-American hospitals between 2006 and 2012 (G-ROP Study). Higher-order multiples (triplets,etc.) were excluded. Outcomes were proportions of twin pairs who were concordant for any ROP and for severe ROP (ETROP type 1 or 2). Birth weight (BW) and comorbidities of prematurity were evaluated as risk factors for discordance. Results: Of 7,483 infants, 663 twin pairs (1,326 infants) were studied; median GA 28 wks(range, 23-35), mean BW 1172 g (SD, 349 g), mean BW-difference between twins 179 g (SD, 180 g; range, 0-1140 g). 110 (8.3%) infants developed severe ROP. Overall twin-pair concordance was 80.3% (kappa 5 0.59) for ROP and 92.5% (kappa 5 0.50) for severe ROP. Severe-ROP-concordance was similar regardless of percentage difference in BW (90%-95%, kappas 5 0.47-0.58). In 17/21 twin pairs discordant for both sepsis/NEC and severe ROP, the infant with sepsis/NEC had severe ROP (10 larger-twin, 7 smaller-twin). Nine of these 21 pairs had high discordance (one twin had severe ROP, the other twin had no ROP); in 8/9 of twin pairs with high discordance, the twin with severe ROP had sepsis and/or surgical NEC but the sibling did not. Discussion: Twin siblings develop severe ROP with high concordance, generally irrespective of BW difference. Discordance is associated with systemic infection in the twin with severe ROP. Conclusions: Ophthalmologists examining twins can expect similar ROP diagnoses but should suspect discordance when one infant develops sepsis or NEC. 037 Visual improvement in amblyopic eye following radiationinduced vision loss in dominant eye with uveal melanoma. Aldo Vagge, Bruce M. Schnall, Renelle P. Lim, Jerry A. Shields, Carol L. Shields Introduction: Visual improvement in amblyopic eyes of adults can occur following visual loss in the dominant eye. The purpose of this study is to determine the frequency and amount of this improvement. Methods: Retrospective review of adult patients with visual loss following radiotherapy for uveal melanoma with a history of amblyopia in the fellow eye. Best corrected visual acuity (BCVA) of treated and amblyopic eye were examined over time. BCVA improvement was defined as $ 2 logMAR lines. The patients were divided into 2 groups dependent on the severity of visual loss in the treated melanoma eye (BCVA melanoma eye . or # amblyopic eye).