Melanocytic Nevus of the Superior Conjunctival Fornix: A Case Report.

Introduction: Conjunctival nevi are benign tumors that are commonly located at the nasal or temporal limbus and rarely in the fornix or tarsus. We report a case of a patient presenting with a solitary compound cystic nevus of the conjunctival fornix in the background of bilateral complexion-associated melanosis. Case Presentation: A 71-year-old African-American female was referred for evaluation of an incidentally noted melanocytic lesion of the right conjunctival fornix. The patient underwent an excisional biopsy, revealing histological features consistent with a compound cystic nevus. Conclusion: This finding is noteworthy due to the rarity of conjunctival nevi originating in the fornix. The case underscores the importance of excisional biopsy in evaluating conjunctival forniceal melanocytic lesions to exclude malignant melanoma, a critical consideration for prognosis.

Transitioning from a COMS-based plaque brachytherapy program to using eye physics plaques and plaque simulator treatment planning system: A single institutional experience.

The aim of this work is to describe the implementation and commissioning of a plaque brachytherapy program using Eye Physics eye plaques and Plaque Simulator treatment planning system based on the experience of one institution with an established COMS-based plaque program. Although commissioning recommendations are available in official task groups publications such as TG-129 and TG-221, we found that there was a lack of published experiences with the specific details of such a transition and the practical application of the commissioning guidelines. The specific issues addressed in this paper include discussing the lack of FDA approval of the Eye Physics plaques and Plaque Simulator treatment planning system, the commissioning of the plaques and treatment planning system including considerations of the heterogeneity corrected calculations, and the implementation of a second check using an FDA-approved treatment planning system. We have also discussed the use of rental plaques, the analysis of plans using dose histograms, and the development of a quality management program. By sharing our experiences with the commissioning of this program this document will assist other institutions with the same task and act as a supplement to the recommendations in the recently published TG-221.

A CASE OF HIGH-RISK CILIARY BODY MELANOMA ARISING IN AN EYE AFTER RESECTION OF AN IRIDOCILIARY MELANOCYTOMA.

PURPOSE: To report a case of a benign iridociliary melanocytoma recurring as malignant melanoma after excision. METHODS: Observational case report with clinical data, slit-lamp examination findings, ultrasound biomicroscopy results, and histopathological and genetic analyses. RESULTS: A 40-year-old African American woman initially presented with a superonasal iridociliary mass with a maximal thickness of 2.5 mm. Visual acuity of the involved eye was 20/25, intraocular pressure was 52 mmHg on maximal pressure-lowering medications, and Humphrey visual field testing revealed an inferior altitudinal defect. Fine-needle aspiration biopsy and incisional biopsy followed by tumor excision confirmed a benign melanocytoma. After 5 years of stability, possible recurrence was detected on ultrasound biomicroscopy as an increase in ciliary body thickness. The new lesion grew to a thickness of 5.1 mm over the next 18 months of observation. Fine-needle aspiration biopsy and gene expression profile of the recurrent lesion diagnosed a malignant melanoma with high metastatic potential (Class 2). The patient underwent plaque brachytherapy and has ongoing regression of the tumor. CONCLUSION: Transformation of benign iridociliary melanocytoma to melanoma is rare. To the best of the authors' knowledge, this is the first documented case of a melanoma arising in an eye after initial excision of a melanocytoma. Close monitoring of these patients is warranted even years after the initial excision.

Preliminary Results of Uveal Melanoma Treated With Iodine-125 Plaques: Analysis of Disease Control and Visual Outcomes With 63 Gy to the Target Volume.

Purpose: Our purpose was to review the preliminary outcomes of patients with uveal melanoma treated with iodine-125 plaques using a novel treatment planning approach. Methods and Materials: This was a single institution, retrospective review of patients treated with iodine-125 brachytherapy for uveal melanoma from November 2016 to February 2019. We used 3-dimensional treatment planning with the Eye Physics Plaque Simulator to ensure that a minimum of 63 Gy covered a 2-mm circumferential tumor margin and the apex height of the tumor over 94 hours. Primary endpoints were local failure, systemic metastasis, final visual acuity (VA), and radiation retinopathy. Associations between primary endpoints and tumor characteristics/radiation dose were performed using univariate analysis. Results: Sixty-nine patients were included in the analysis. Mean largest basal diameter was 11.67 mm (range, 6-18; median, 12), and the average tumor thickness to the inner sclera was 3.18 mm (range, 0.5-9.3; median, 2.8). Molecular testing that was successfully performed in 59 patients revealed that 27% (16 of 59) had class 2 gene expression profile designation. Average follow-up posttreatment was 28.3 months (range, 4-46; median, 29), with 6% (4 of 69) developing local failure and 6% (4 of 69) developing metastasis over this duration. Average final VA (0.57 logMAR [Snellen 20/74]; range, 0-2.9; median, 0.3) was decreased from baseline (0.34 logMAR [Snellen 20/44]; range, 0-2.3; median, 0.1), and 48% (33 of 69) developed radiation retinopathy. Fifty percent of patients had a final VA 20/40 or better and 22% had a final VA 20/200 or worse. Conclusions: In patients with uveal melanoma, preliminary results with brachytherapy using Eye Physics plaques with a treatment plan that delivers 63 Gy to a 2-mm circumferential tumor margin and the tumor apex suggest effective disease control and favorable VA outcomes.

Improved Prognostic Precision in Uveal Melanoma through a Combined Score of Clinical Stage and Molecular Prognostication.

Introduction: Prognosis of uveal melanoma (UM) is assessed using clinical staging or molecular testing. Two modalities often used for prognostication are the American Joint Committee on Cancer (AJCC) staging and a tumor gene expression profile (GEP), the outcomes of which are often discordant. This article discusses a total risk score created to combine the discordant information from both sources. Methods: A retrospective case series was conducted of all patients presenting with UM over 6 years to 2 referral centers. Each tumor was classified using the AJCC and the GEP. A total risk score was calculated for each patient using results from both AJCC and GEP. Kaplan-Meier analysis of metastasis-free survival was used to compare groups. Results: A total of 294 patients were included in the study. Kaplan-Meier estimates showed significant curve separation between individual AJCC and GEP risk groups. The combined total risk score provided an accurate estimate of prognosis that incorporated results from both AJCC and GEP. Conclusions: Clinical staging and molecular prognostication of UM can be discordant. There is important information provided by each system that is not provided by the other. The total risk score provides a simple method to combine information from both AJCC stage and the GEP class in order to provide patients and care teams with a more complete understanding of metastatic risk.

Dual-source strength seed loading for eye plaque brachytherapy using eye physics eye plaques: A feasibility study.

Purpose: This study quantifies the dosimetric impact of incorporating two iodine-125 (125I) seed source strengths in Eye Physics eye plaques for treatment of uveal melanoma. Material and methods: Plaque Simulator was used to retrospectively plan 15 clinical cases of three types: (1) Shallow tumors (< 5.5 mm) with large base dimensions (range, 16-19 mm); (2) Tumors near the optic nerve planned with notched plaques; and (3) Very shallow (< 3.0 mm) tumors with moderate base dimensions (range, 13.5-15.5 mm) planned with larger plaques than requested by the ocular oncologist. Circular plaques were planned with outer ring sources twice the source strength of inner sources, and notched plaques with the six seeds closest to the notch at twice the source strength. Results: In cases of type (1), the dual-source strength plan decreased prescription depth, and doses to critical structures were lower: inner sclera -25% ±2%, optic disc -7% ±3%, and fovea -6% ±3%. In four out of five cases of type (2), the dual-source strength plan decreased prescription depth, and dose to inner sclera was lower (-22% ±5%), while dose to optic disc (17% ±7%) and fovea (20% ±12%) increased. In cases of type (3), a smaller dual-source strength plaque was used, and scleral dose was lower (-45% ±3%), whereas dose to optic disc (1% ±14%) and fovea (5% ±5%) increased. Conclusions: Dual-source strength loading as described in this study can be used to cover tumor margins and decrease dose to sclera, and therefore the adjacent retina, but can either decrease or increase radiation dose to optic disc and fovea depending on location and size of the tumor. This technique may allow the use of a smaller plaque, if requested by the ocular oncologist. Clinical determination to use this technique should be performed on an individual basis, and additional QA steps are required. Integrating the use of volumetric imaging may be warranted.

Recurrence of Primary Breast Lymphoma Presenting as Bilateral Vitreoretinal Lymphoma.

PURPOSE: This report describes a case of relapsed primary breast lymphoma (PBL) presenting as vitreoretinal lymphoma (VRL). METHODS: We describe the clinical and hematopathologic findings in a patient with relapsed PBL involving the vitreous of both eyes. RESULTS: A 59-year-old woman was treated for PBL with systemic and intrathecal chemotherapy 5 years prior to presentation. Three years later, she presented to an outside clinic with blurred vision in both eyes and bilateral vitritis. She was referred to our clinic with concern for ocular lymphoma. On presentation, the patient's best-corrected visual acuity was 20/40 in the right eye and 20/25 in the left eye with 3+ vitreous cells in the right eye and 2+ vitreous cells in the left eye. Vitreous biopsy of the right eye revealed CD5-negative/CD10-negative B-cell lymphoma cells on flow cytometry. She had no evidence of disease on brain MRI, lumbar puncture, bone marrow biopsy, or full-body CT scans. She was treated with a regimen of rituximab, methotrexate, procarbazine, and vincristine for central nervous system penetration as well as multiple intraocular injections of methotrexate and rituximab with improvement in vision and ocular inflammation bilaterally. CONCLUSION: Relapsed PBL can present as bilateral VRL.

Optical Coherence Tomography Angiography Microvascular Variations in Pre- and Posttreatment of Retinoblastoma Tumors.

INTRODUCTION: The purpose of this study is to describe variations in microvasculature before and after treatment of treatment-naive lesions and during consolidation therapy of retinoblastoma lesions using an investigational portable optical coherence tomography angiography (OCTA) system. METHODS: This study is a single-center, prospective, observational case series. Recruited subjects were either undergoing surveillance for retinoblastoma or had newly detected retinoblastoma. Nine tumors from 7 eyes in 6 patients were included. During exams under anesthesia, the tumors were imaged with an investigational portable OCTA system. OCTA images were analyzed to assess vascular changes before and after treatment. RESULTS: In all 6 presented cases, OCTA imaging revealed distinctive vascular patterns, such as dilated feeder arteries and draining veins, disorganized and complex branching patterns, irregular vessel calibers, and dilation and tortuosity of vessels. After treatment, OCTA imaging revealed decreased intrinsic tumor vascularity and reduced dilation of draining and feeder vessels. Tumor relapse demonstrated prominent vascularity (n = 1) that resolved on repeat OCTA after transpupillary thermotherapy treatment. Type 2 (n = 1), 3 (n = 6), and 4 (n = 1) tumor regression patterns were seen in our patients after treatment, and OCTA findings were consistent with a previously published report. Interestingly, in one of the presented cases, OCTA demonstrated clear feeder, draining, and intrinsic tumor vessels that were not as evident on fluorescein angiography. CONCLUSIONS: OCTA may offer a noninvasive and sensitive technique to evaluate the vasculature of both the tumor and the surrounding retina in retinoblastoma. With additional research and development into its use in patients with retinoblastoma, OCTA may one day be useful in assessing treatment response and residual tumor activity.

Choroidal nevus with retinal invasion, clinical and imaging features.

PURPOSE: To report a clinically challenging case of a choroidal nevus with retinal invasion with accompanying ancillary testing. OBSERVATIONS: A 60-year-old Caucasian female was referred for a suspicious melanocytic choroidal lesion in her left eye. Ophthalmoscopic examination revealed a melanocytic choroidal lesion, measuring 10 mm × 10 mm in basal diameter. The lesion had a clinically evident area of retinal invasion seen as a protruding choroidal mass at its center, darker in appearance compared to the rest of the lesion, obscuring retinal vessels. The choroidal nevus had associated chronic retinal changes and the absence of overlying orange pigment or subretinal fluid. On fundus autofluorescence, there was a hypoautofluorescent area showing the site of retinal invasion. Fluorescein angiography at the lesion site exhibited central blocked perfusion corresponding to the area of retinal invasion. Ultrasonography showed a dome-shaped choroidal lesion that was optically dense with a medium-high internal reflectivity measuring 3.3mm in thickness. The optical coherence tomography showed a choroidal mass extruding through a break in Bruch's membrane with inner retinal invasion. A watchful waiting strategy was adopted, and at 28 months follow-up, the choroidal lesion did not show growth or presence of new suspicious features of malignant transformation. CONCLUSION AND IMPORTANCE: This case highlights the importance of recognizing the key features of choroidal nevi with retinal invasion, which can prevent the treatment of a benign condition and assist in the arrival of a correct diagnosis. These lesions should be monitored for long-term.

CHOROIDAL MELANOMA DURING PREGNANCY: A CASE REPORT AND REVIEW OF THE LITERATURE.

PURPOSE: To report a case of a woman diagnosed with choroidal melanoma during pregnancy, as well as review similar cases published in the literature. The prevalence, risk factors, treatment, prognosis, and outcomes of choroidal melanoma in pregnancy are addressed. METHODS: An extensive search of the literature was conducted by accessing PubMed, MEDLINE, and Scopus databases in January 2018 to identify cases of choroidal or uveal melanoma diagnosed during pregnancy. PATIENT: A 37-year-old white woman pregnant with twins and diagnosed with choroidal melanoma. RESULTS: Choroidal melanoma in pregnancy has a prevalence ranging from 4.3% to 7.5% among women of childbearing age with the disease. Pregnancy has been linked to increased incidence of choroidal melanoma, but female hormones have not been shown to play a role in the pathogenesis of the disease. Other factors such as melanocyte-stimulation hormone and fetal-induced angiogenesis may be implicated. Choroidal melanoma in pregnancy has not resulted in a worse maternal or fetal outcome. CONCLUSION: Although rare, choroidal melanoma in pregnancy is a clinical challenge. Management of these patients should be tailored to maternal and fetal needs.

Uveal Melanoma Metastatic to the Cavernous Sinus: A Case Report.

A woman in her early 50s previously treated 7 years prior with iodine-125 plaque brachytherapy without a biopsy for gene expression profiling for uveal melanoma in the left eye presented with a 3-week history of intermittent diplopia and headache. Ophthalmic examination was significant for limitation in left eye upward gaze; otherwise, examination revealed a stable, regressed tumor in the left eye, and normal vision, pressure, and pupils in both eyes. Neuroimaging showed a left cavernous sinus lesion, suggestive of a meningioma. Excisional biopsy revealed metastatic melanoma. The patient was treated with radiotherapy, and her diplopia resolved. Slight enlargement of the lesion was noted on neuroimaging 20 months later, and was treated with stereotactic radiosurgery. Serial neuroimaging in the following 6 months did not reveal any recurrences or new metastases. This case demonstrates the importance of investigating persistent diplopia in a patient with a history of uveal melanoma, and the possibility of metastases occurring in organs besides the liver or lung.

Multiple Uveal Melanoma.

INTRODUCTION: Multiple uveal melanoma is a rare occurrence and includes bilateral melanoma, unilateral multiple/multifocal melanoma, or melanoma with metastasis to the ipsilateral or contralateral eye. METHODS: A chart review of patients diagnosed with uveal melanoma between January 2013 and January 2019 at the Duke University Eye Center Ophthalmic Oncology Service was performed. RESULTS: Three patients with multiple, sequential melanoma were identified; patient 1 had bilateral choroidal melanoma and patients 2 and 3 had 2 choroidal melanomas occurring in the same eye. In all 3 patients, both the first and sequential choroidal melanomas were treated with I-125 radioactive plaque brachytherapy (PBT). Two patients were found to have developed secondary metastatic uveal melanoma as a presenting sign of systemic metastases. Patient 4, initially treated with PBT, was diagnosed with ipsilateral metastatic choroidal melanoma, also treated with PBT. Patient 5 had right eye enucleation for choroidal melanoma and developed vision-threatening metastasis in the left eye, which was treated with PBRT. None of the patients had history of cancer prior to their first diagnosis. Patients 1 and 5 were tested with a systemic melanoma panel; both were negative for BAP1, but patient 1 had a variant of unknown significance in BRCA2. Patient 3 had oculodermal melanocytosis, an established risk factor of uveal melanoma. CONCLUSION: Although rare, the possibility of multiple uveal melanoma does exist. Examination of the treated and contralateral eye on a regular basis is crucial, not only to identify local failure but also new metastases from the primary tumor and additional primary tumors.

Unique Geospatial Accumulations of Uveal Melanoma.

PURPOSE: The main purpose of this paper was to describe the unique accumulation of cases of uveal melanoma (UM). All patients were white and did not have known occupational risk factors. From the authors' standpoint, there were no lifestyle factors in common in the reported cases. Results of more extensive analyses, including geospatial analysis, are currently being conducted and will be presented in a separate paper. DESIGN: Observational case series. METHODS: Descriptive data from medical records, patient interviews, and questionnaires were obtained from 5 patients from North Carolina, 6 patients from Alabama, and 14 patients from New York. Standard incidence ratio (SIR) calculations were provided by the respective states' cancer registries. UM is the most common primary malignant eye tumor in adults, although it is rare, with 2,500 cases diagnosed annually in the United States. Despite a growing understanding of the molecular characteristics, there remains uncertainty regarding epidemiologic trends and environmental risk factors. This study identified 3 geographic accumulations of UM: 1) Huntersville, NC; 2) Auburn, AL; and 3) Broome and Tioga Counties, New York. Investigation of these groups will guide ongoing efforts to discover potential risk factor and assist with future treatment and prevention. RESULTS: In North Carolina, 5 females who were identified as living in Huntersville, NC, were diagnosed with UM at ages 20, 22, 24, 30, and 31. The SIR calculations considering the observed and expected incidence ratios was 0.7 (95% confidence interval [CI], 0.5-0.9) in Mecklenburg County. In Alabama, 6 individuals who were identified as either attending Auburn University or employed there from 1989 to 1993 had diagnoses of UM. Initial SIR calculations for white females of all ages was 1.15 (95% CI, 0.989-1.328). In New York, SIR for Broome and Tioga counties were 0.93 and not significant. However, in Tioga county, for males and females and females alone, SIRs were 2.00 (P = .04) and 3.33 (P = .006). CONCLUSIONS: Although most of the conclusions that the SIR does not meet statistical criteria that defines these accumulations as true "cancer clusters," considering the incidence and demographics of UM, these accumulations of cases is unexpected and worth additional exploration. Further investigation into these cases with additional geospatial analyses and blood and tumor testing is ongoing. Information learned from the study of these unique populations may inform a better understanding of the pathogenesis of UM.

NCCN Guidelines Insights: Uveal Melanoma, Version 1.2019.

The NCCN Guidelines for Uveal Melanoma include recommendations for staging, treatment, and follow-up of patients diagnosed with uveal melanoma of the choroid or ciliary body. In addition, because distinguishing between uveal melanoma and benign uveal nevi is in some cases difficult, these guidelines also contain recommendations for workup of patients with suspicious pigmented uveal lesions, to clarify the tests needed to distinguish between those who should have further workup and treatment for uveal melanoma versus those with uncertain diagnosis and low risk who should to be followed and later reevaluated. These NCCN Guidelines Insights describe recommendations for treatment of newly diagnosed nonmetastatic uveal melanoma in patients who have already undergone a complete workup.

CORRELATION OF GENE EXPRESSION PROFILE STATUS AND AMERICAN JOINT COMMISSION ON CANCER STAGE IN UVEAL MELANOMA.

PURPOSE: To study the relationship between gene expression profile (GEP) subclass and American Joint Committee on Cancer (AJCC) stage in patients with uveal melanoma (UM). METHODS: A retrospective, multicenter study was undertaken with patients entered from nine major ocular oncology centers from across the United States. Three hundred sixty eligible patients had UM and underwent I-125 plaque brachytherapy with concurrent tumor biopsy with GEP testing between January 1, 2010, and October 28, 2014. Patient demographics and UM features were analyzed by both GEP and AJCC status. RESULTS: Gene expression profile class divided the cohort into three groups: Class 1a (n = 186), Class 1b (n = 77), and Class 2 (n = 113). When classified using AJCC staging criteria, we found the following: Stage I in 91 cases (25.3%), Stage IIA in 143 cases (39.7%), Stage IIB in 89 cases (24.7%), Stage IIIA in 36 cases (10%), and Stage IIIB in 1 case (0.3%). There were no Stage IV cases, as lymph node and metastatic data were not collected as a part of this study. Among Stage I tumors, both high tumor height and high largest basal diameter were associated with a higher frequency of Class 2 status (P < 0.05). As UMs progress to a larger AJCC tumor group (T1-T4), the odds ratio of having a worse prognosis based on GEP class was 1.75 (95% CI, 1.36-2.25; P < 0.001). Similarly, as UMs progress to a higher AJCC stage, the odds ratio of having a worse prognosis based on GEP class was 1.69 (95% CI, 1.36-2.10; P < 0.001). CONCLUSION: This report details the differences in clinical features between GEP subclasses and how they are distributed among the AJCC stages. When the tumors were grouped by AJCC staging criteria, both larger AJCC tumor (T) group and worsening AJCC stage were associated with worsening predicted prognosis, based on GEP subclass.

Multi-center analysis of intraocular biopsy technique and outcomes for uveal melanoma: Ocular Oncology Study Consortium report 4.

PURPOSE: To investigate the relationship between surgical approach for intraocular tumor biopsy of uveal melanoma and tumor morphologic features such as size and intraocular location and the effect of these variables on diagnostic yield and biopsy outcome. METHODS: Consecutive patients from nine Ocular Oncology centers with uveal melanoma (UM) undergoing tumor biopsy immediately preceding I125 plaque brachytherapy with tissue sent for gene expression profiling (GEP) testing were reviewed retrospectively. RESULTS: Three hundred sixty patients were included (50% men, mean age 60.2 years). Overall biopsy yield was 99% and 83% for GEP and cytopathology, respectively. Surgeon choice of biopsy approach (trans-vitreal vs. trans-scleral) was found to associate with both tumor location and tumor thickness. A trans-scleral rather than trans-vitreal approach was used more commonly for anteriorly located tumors (92% vs. 38% of posterior tumors, p < 0.001) and thicker tumors (86% vs. 55% of thin tumors, p < 0.001). When performing trans-vitreal biopsies, ocular oncologists with previous vitreoretinal surgery fellowship training were more likely to use wide-field surgical viewing systems, compared with indirect ophthalmoscopy (82.6% vs. 20.6%, p < 0.001). Surgical complications were rare and occurred more frequently with trans-vitreal biopsies (3.6% vs. 0.46%, p = 0.046). CONCLUSIONS: In this multi-center analysis of UM tumor biopsy, surgical yield was high for obtaining tumor tissue for GEP and cytopathology analysis with both trans-scleral and trans-vitreal techniques. Fellowship-trained ocular oncologists' preferred intraocular biopsy techniques associated strongly with tumor location, tumor thickness, and fellowship training of the surgeon. Short-term complication rates were low.

Assessment of Macular Microvasculature in Healthy Eyes of Infants and Children Using OCT Angiography.

PURPOSE: To assess macular vasculature in healthy infants and children using OCT angiography (OCTA). DESIGN: Prospective cross-sectional study. PARTICIPANTS: One hundred thirty-five normal maculae of 89 healthy infants and children (mean age, 8.5±5.3 years; range, 9 weeks-17 years) treated at the Duke University Eye Center. METHODS: We imaged 135 maculae of 89 pediatric patients using the standard Spectralis tabletop and investigational Spectralis with Flex module devices, both equipped with investigational OCTA software (Heidelberg Engineering, Heidelberg, Germany). OCT angiography images of the superficial vascular complex (SVC) and deep vascular complex (DVC) were analyzed for foveal avascular zone (FAZ) area and superficial and deep vessel density. We assessed effects of age, gender, race, axial length (AL), and central subfield thickness on FAZ and vessel density. Patients with both eyes imaged were assessed for agreement between the FAZ and vessel densities of the left and right eyes. MAIN OUTCOME MEASURES: The FAZ area, as well as vessel area density (VAD) and vessel length density (VLD) in the SVC and DVC. RESULTS: The FAZ varied significantly with race; white patients showed a significantly smaller FAZ than black patients (mean difference, 0.11 mm2; P = 0.004). The FAZ did not vary with age, gender, or AL (P > 0.05). In the SVC, VAD and VLD varied significantly with age (P < 0.001) and AL (R2 = 0.46; P < 0.001) but not gender (P > 0.05). The SVC VLD was significantly different between races and ethnicities (P = 0.037), but VAD was not (P < 0.05). In the DVC, VAD and VLD also varied significantly with age (P < 0.001) and AL (R2 = 0.46; P < 0.001) but not gender or race (P > 0.05). There was excellent agreement between the right and left eyes for FAZ (intraclass correlation [ICC], 0.97), SVC VLD (ICC, 1.00), and DVC VLD (ICC, 1.00). CONCLUSIONS: Quantitative studies of pediatric perifoveal vasculature should consider age, race, and AL. In eyes with unilateral disease, the perifoveal vasculature in the unaffected eye may be used as a control comparison because there is excellent agreement between eyes.