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An optic disk melanocytoma is a benign pigmented neoplasm of the optic disk measuring 2.00 mm of diameter in average. Smaller optic disk melanocytomas are rare and have not been studied with multiple imaging studies. A case of a "micro" optic disk melanocytoma measuring 0.71 mm in diameter was studied by multiple imaging modalities, including fundus autofluorescence, ultrasound, visual fields, optical coherence tomography (OCT) and OCT angiography. While clinical appreciation and fundus autofluorescence reveal changes compatible with previously reported cases, OCT, OCT angiography and ultrasound reveal specific changes that could be useful for follow up.

PURPOSE To illustrate the features of unilateral retinal pigment epithelium dysgenesis (URPED) in an African-American male patient. METHODS Case report. RESULTS A 47-year-old asymptomatic African-American man was referred for an atypical subretinal pigmented mass in the left eye. On examination, visual acuity was 20/20 in both eyes. The right eye was unremarkable. The left eye revealed a darkly pigmented grey-black lesion at the level of the RPE with irregular, unraveled fringe-like margins, consistent with URPED. The lesion measured 5 mm in basal dimension and was located 400 μm from the foveola. The dark portion of the lesion was grey-black and demonstrated homogeneous hypoautofluorescence, particularly at the site of grey-white peripheral fringe of fibrous metaplasia. By contrast, there was an additional, subtle lacey arrangement of normal-appearing RPE traversing over the entire lesion demonstrating isoautofluorescence. On fluorescein angiography, the lesion was generally hypofluorescent, particularly in the dark portion of the lesion, but the peripheral fringe of fibrous metaplasia displayed angiographic hyperfluorescent staining, and the subtle lacey normal RPE showed isofluorescence. Optical coherence tomography demonstrated RPE hyperplasia and shallow RPE detachment interspersed with normal-appearing RPE and thinning of outer retina and preservation of the foveola and choroid. CONCLUSION In this case, URPED demonstrated biphasic autofluorescence implying RPE dysfunction in the hypoautofluorescent area and partial RPE function in the lacey isoautofluorescent region.

Purpose: Retinoblastoma is the most frequent intraocular neoplasm of childhood. While survival rates, globe salvage, and disease stage at presentation have significantly improved in the last decades in high-income countries (HICs), retinoblastoma remains a clinical challenge in low and middle-income countries (LMICs). Furthermore, novel therapies such as intraarterial chemotherapy (IAC) are often unavailable, or underused, in middle or low-income countries. Methods: We present a retrospective case series, where we examine the outcomes of 6 eyes in 6 patients who received IAC as primary, or secondary, therapy for retinoblastoma. Results: Four eyes resulted in disease remission, while two ultimately were enucleated due to ophthalmic artery anatomic issues. Nevertheless, all patients remain disease free at last follow-up. Conclusion: Intraarterial chemotherapy is a safe and effective alternative for treating retinoblastoma. Unfortunately, due to disparity in healthcare access between different socioeconomic strata, it is not a common therapy in Mexico. Further work needs to be undertaken, such as increasing awareness of this disease, inter-institutional twinning, and overall improvement in healthcare access.

El retinoblastoma es un tumor que representa aproximadamente el 3% de los cánceres en niños menores de 15 años, siendo el 95% diagnosticado antes 5 años. Por lo general, se limita al ojo, lo que resulta en una tasa de supervivencia que oscila entre 30 - 97%, siendo superior al 97% en los países de ingresos altos (HIC).1,2 El tratamiento del retinoblastoma es complejo y requiere un enfoque multidisciplinario para lograr los objetivos principales de la supervivencia del paciente, globo ocular conservación y optimización de la función visual.

Introduction: To report a case of a Hispanic girl with late-onset Retinoblastoma (Rb) who was misdiagnosed as a pars planitis prior to referral. Nearly 95% of all Rb cases are detected before age 5, and this patient was 8 years-old. Methods: Case report of a late-onset Retinoblastoma with anterior chamber (AC) involvement plus the presence of an Ahmed valve. The patient had a history of a couple of months of topical therapy comprising medication for glaucoma, systemic steroids, and a filtration surgery (Ahmed valve), after that a biopsy was performed prior to referral. Upon arrival at our clinic, we performed an examination under anesthesia (EUA) and a B-scan ultrasound (US). Results: Unilateral Retinoblastoma with an Ahmed valve in an AC filled with Rb seeds was diagnosed with the EUA and US in the left eye. An orbital exenteration with map biopsies of the left orbital cavity was performed with confirmation by histopathology of a poorly differentiated endophytic retinoblastoma with Bruch’s membrane invasion. Follow-up sessions were then arranged as well as subsequent systemic chemotherapy cycles. Conclusion: Given the rare incidence of retinoblastoma in children older than 5 years old, it can be easily mistaken for other differential diagnoses and treated with filtration surgeries that could put the patient’s life at risk. In this report, late-onset Rb diagnosis is highlighted as a differential diagnosis in children and adults with atypical uveitis, which required a multidisciplinary approach.

A 4-year-old girl presented with leukocoria, vision loss, and painless left eye redness. Posterior segment examination revealed a total retinal detachment and abnormal, tortuous retinal vessels in the left eye. No visible mass, vitreous seeding, or hemorrhage were observed. B-mode ultrasound showed a detached retina in a "cinnamon-roll" configuration. Due to poor visual prognosis and high clinical suspicion of malignancy, enucleation was performed. Further histopathological analysis confirmed the diagnosis of diffuse infiltrative retinoblastoma, with an atypical presentation.

Retinoblastoma (Rb) most frequently presents as a unilateral sporadic disease up to 40% of cases, however, arise from a monoallelic germline pathogenic variant. Only 10% of the germline mutations are inherited, and high penetrance is seen in up to 90% of these cases. As an effort to optimize counseling and screening, mutations are classified according to inheritance patterns. However, RB1 spectrum is highly heterogeneous, and information for unaffected carriers remains scarce.The Mexican family of a 5-month-old patient diagnosed with Rb was studied. The family consisted of five individuals (father, mother, and three siblings). Genetic testing using a next-generation sequencing assay targeting RB1 with oligonucleotide baits designed to capture its exons and 20 bases flanking intronic sequences was performed in every family member. Clinical history and a complete ophthalmological examination (best-corrected visual acuity, slit-lamp biomicroscopy, macular optical coherence tomography, fundus autofluorescence, optical coherence tomography angiography, and electrophysiological studies) were performed in members testing positive to RB1 mutation.The father and her five-month-old daughter tested positive for a non-synonymous RB1 mutation c.459del (p.Lys154Serfs*21). The girl presented with bilateral retinoblastoma, successfully treated with cryotherapy and intravenous chemotherapy. The father had no relevant findings on imaging studies or ophthalmologic evaluation.This report describes a rare case of a novel low-penetrance RB1 germline mutation. Long-term follow-up of the father will include periodic evaluation of the eyes and orbits, and surveillance for systemic sarcoma and secondary malignancies. Implications for unaffected individuals need to be further studied.

BACKGROUND AND PURPOSE: Intra-arterial chemotherapy for retinoblastoma has dramatically altered the natural history of the disease. The remarkable outcomes associated with a high safety profile have pushed the envelope to offer treatment for patients weighing ≤10 kg. The purpose was to determine the efficacy and safety of IAC infusions performed in infants weighing ≤10 kg with intraocular retinoblastoma. MATERIALS AND METHODS: A retrospective chart review was performed for patients diagnosed with retinoblastoma and managed with intra-arterial chemotherapy. RESULTS: The total study cohort included 207 retinoblastoma tumors of 207 eyes in 196 consecutive patients who underwent 658 intra-arterial chemotherapy infusions overall. Of these, patient weights were [Formula: see text] 10 kg in 69 (35.2%) and >10 kg in 127 (64.8%) patients. Comparison (≤10 kg versus >10 kg) revealed that the total number of intra-arterial chemotherapy infusions was 222 versus 436. Periprocedural complications were not significantly different (2 [0.9%] versus 2 [0.5%]; P = .49). Cumulative radiation exposure per eye was significantly lower in infants weighing ≤10 kg (5.0 Gym(2) versus 7.7 Gym(2); P = .01). Patients weighing [Formula: see text] 10 kg had a greater frequency of complete tumor regression (82.6% versus 60.9%; P = .02). Mean fluoroscopy time was not significantly different (7.5 versus 7.2; P = .71). There was a significant difference in the frequency of enucleation (16 [21.6%] versus 52 [39.1%]; P = .01). Patients weighing [Formula: see text] 10 kg had greater number of aborted procedures (12 [5.4%] versus 7 [1.6%]; P = .01). On multivariate analysis, weight ≤10 kg was not an independent predictor of complications or procedure failure. CONCLUSIONS: Intra-arterial chemotherapy in patients weighing ≤10 kg is a safe and effective treatment.

Purpose To determine the association between vitreoretinal lymphoma and systemic lymphoma (SL). Methods Single-center retrospective review of medical records. Results Of 95 patients with vitreoretinal lymphoma, 18 (19%) had associated SL (SL group) and 77 (81%) were not associated with SL (no SL group). The most common sites of SL were skin (n = 5), testis (n = 2), liver and breast (n = 2), and others (n = 9). A comparison (SL group vs. [vs.] no SL group) revealed no difference in demographic or ocular findings at initial visit. In the SL group, SL occurred before the onset of ocular symptoms in 14 (78%) patients with mean interval of 86 months (median 61, range 5-286 months) or after ocular symptoms in 4 (22%) patients with mean interval of 19 months (median 12, range 7-44 months). A comparison revealed no difference in overall frequency of pre-existing or eventual central nervous SL (50% vs. 53%, P = 0.99); however, the SL group demonstrated central nervous SL more often after onset of ocular symptoms (78% vs. 17%, P = 0.001). A comparison found no difference in treatment methods, response of vitreoretinal lymphoma to treatment, final visual outcome, or death rate. Conclusion We found 19% of patients with vitreoretinal lymphoma demonstrate related SL, and there was no difference in demographics, clinical features, or response to treatment, compared to those not associated with SL.

Background The introduction of intra-arterial chemotherapy (IAC) for treatment of retinoblastoma considerably changed the paradigm by which this disease is managed, with event-free survival rates being above 70%. Objective To analyze efficacy of IAC treatment using alternative approaches to ophthalmic artery catheterization (OAC), such as external carotid artery approach or balloon-assisted drug delivery. Methods This is a retrospective chart review for subjects receiving IAC for retinoblastoma. The primary approach was OAC. In cases in which selective OAC was not feasible, alternative routes including catheterization of the external carotid artery or use of a balloon-assisted drug infusion were used. Results This study included 197 consecutive patients with 207 retinoblastomas who underwent 658 IAC procedures overall. The mean age at diagnosis was 24 mo, and 54.5% of the study population was male. Success rate with IAC was 97% (639). Alternative approaches to OAC were, in total, 42 cases (6.4%)-external carotid artery catheterization and use of ICA balloon were performed in 22 (3.3%) and 20 (3%) cases, respectively. A mean of 3.1 IAC cycles were performed for each patient. In total, there were 23 technical failures of the primary OAC technique (3.4%). Periprocedural adverse events occurred in 4 procedures (0.6%). Use of an alternative technique for chemotherapy delivery other than selective OAC in at least one IAC cycle was not a predictor of enucleation. Conclusion IAC is a safe and effective treatment option for retinoblastoma. Chemotherapy delivery using alternative techniques is as effective as selective OAC.

PURPOSE The aim of this study was to evaluate retinal microvascular abnormalities following plaque radiotherapy of choroidal melanoma (CM) using wide-field swept-source optical coherence tomography angiography (OCTA). DESIGN Single-centre retrospective review. METHODS Retrospective case series of 105 CM patients treated with I-125 plaque radiotherapy and imaged with wide-field (15 × 9 mm) SS-OCTA from March 2018 to August 2018 at the Ocular Oncology Service, Wills Eye Hospital (Philadelphia, PA). RESULTS At mean follow-up of 49 months (range 4-297) after plaque radiotherapy, there were 52 eyes (50%) with clinically evident radiation retinopathy (CERR) and 53 eyes (50%) without CERR. Comparison (CERR vs controls) revealed foveal avascular zone enlargement (1.7 vs 0.23 mm, P = 0.03) and reduction of capillary vascular density (CVD) in the superficial and deep plexus in the total wide-field (43% vs 47%, P

Purpose The aim of this study was to identify clinical factors predictive of time to central nervous system (CNS) lymphoma or death in patients with vitreoretinal lymphoma (VRL). Design Retrospective cohort study. Methods Patients with VRL (n = 95 patients) from Januray 1, 1984 to July 30, 2018 were identified at a single ocular oncology center and records were retrospectively reviewed. Outcomes included Kaplan-Meier estimated time to CNS lymphoma and death. Results There were 95 patients with VRL diagnosed at mean age 67 years, of which 70 patients had follow-up with the ocular oncology service. Mean time to CNS lymphoma in patients with isolated VRL was 56 months and did not differ by age, sex, bilateral ocular involvement, retinal infiltration, subretinal pigment epithelial (sub-RPE) infiltration, or treatment with prophylactic systemic chemotherapy (P > 0.05). Mean time to death was 66 months and did not differ when comparing those with CNS lymphoma diagnosed before VRL versus after VRL versus no CNS lymphoma at any time (67 vs 60 vs 64 months, P > 0.05). Presence of sub-RPE infiltration was associated with shorter mean time to death (46 vs 76 months, P = 0.04, odds ratio 1.9). Older patient age was associated with increased risk of death (odds ratio 1.0, P = 0.02). The mean time to death did not differ by sex, bilateral ocular involvement, retinal infiltration, timing of CNS or systemic lymphoma, or treatment with prophylactic systemic chemotherapy (P > 0.05). Conclusions Patients with VRL presenting with sub-RPE infiltration could have shorter mean survival time. Further studies are required to confirm these findings and determine whether sub-RPE infiltration is associated with more aggressive CNS lymphoma.

Purpose To determine whether treatment order affects ophthalmic vascular event rates after intra-arterial chemotherapy (IAC) for retinoblastoma. Methods Patients who received IAC as primary or secondary treatment for retinoblastoma from January 2009 to January 2018 were included. All eyes were imaged with fundus photography and fluorescein angiography. Patient characteristics and vascular event rates were compared using t-test and Fisher's exact test. Results There were 196 patients treated with 682 infusions of IAC, divided into primary (no previous therapy, 98 eyes of 98 patients, 328 infusions) and secondary (after other therapy, 105 eyes of 98 patients, 354 infusions) treatment. Overall, ophthalmic vascular events were found after 5% of infusions (17% eyes). A comparison of ophthalmic vascular events (primary vs. secondary IAC), with mean three infusions per eye (median 3, range 1-7), revealed no difference in overall percentage of eyes affected (18% vs. 15%, P = 0.57). Adverse vascular events per eye included retinal vasculature attenuation (1% vs. 0%, P = 0.99), peripheral retinal pruning (1% vs. 0%, P = 0.99), branch retinal artery occlusion (0% vs. 1%, P = 0.99), central retinal artery occlusion (0% vs. 1%, P = 0.99), macular ischemia (0% vs. 2%, P = 0.51), vitreous hemorrhage (2% vs. 3%, P = 0.92), subretinal hemorrhage (1% vs. 0%, P = 0.99), retinal pigment epithelium atrophy (6% vs. 3% P = 0.43), choroidal atrophy (4% vs. 2%, P = 0.92), optic disk pallor (1% vs. 0%, P = 0.99), and ophthalmic artery occlusion (9% vs. 6%, P = 0.35). Conclusion Ophthalmic vascular events after IAC for retinoblastoma affect only 5% of eyes per infusion (17% of treated eyes). Vascular event risk per eye is similar when using IAC as primary or secondary treatment.

Purpose To investigate outcomes in vitreoretinal lymphoma (VRL) presenting with or without sub–retinal pigment epithelial (sub-RPE) infiltration. Design Retrospective, comparative analysis. Subjects Patients with VRL at a single center from January 1, 1984, through July 30, 2018. Methods Record review was conducted for clinical features, treatments, and outcomes of tumor control, visual acuity (VA), and death. Main Outcome Measures Ocular tumor control and VA outcome. Results The study involved 168 eyes of 95 patients with VRL, of which 45 (27%) eyes of 32 patients had sub-RPE infiltration. Comparison (of patients with vs. without sub-RPE infiltration) showed similar presenting features of mean patient age (65 vs. 68 years, P = 0.30), percentage of males (53% vs. 56%, P = 0.83), white race (84% vs. 87%, P = 0.77), bilateral ocular involvement (78% vs. 75%, P = 0.80), and VA of 20/40 or better (40% vs. 50%, P = 0.30), 20/50 to 20/200 (42% vs. 31%, P = 0.20), or worse than 20/200 (18% vs. 19%, P = 0.99). Lymphoma subtype was diffuse large B cell (59% vs. 52%) or unspecified (41% vs. 44%, P = 0.85). Follow-up data were available for 125 eyes of 70 patients. Overall treatment included systemic chemotherapy (53% vs. 64%, P = 0.29), intravitreal chemotherapy (59% vs. 28%, P = 0.005), and external beam radiotherapy (59% vs. 94%, P Conclusion Sub-RPE infiltration in VRL is associated with more persistent or recurrent ocular tumor and poorer VA outcome, but no difference in frequency of central nervous system/systemic lymphoma or death. Further studies are required to determine whether earlier, more aggressive, or prolonged ocular therapy for patients with VRL presenting with sub-RPE infiltration could improve these factors.

Purpose:To analyze risk of nevus transformation into melanoma per millimeter increment.Methods:Retrospective analysis of 3,806 choroidal nevi for transformation into melanoma per incremental millimeter thickness (flat [≤1.0 mm], thin [1.1–2.0 mm], thicker [2.1–3.0 mm], and thickest [>3.0 mm])Results

Purpose:To use multimodal imaging for identification of risk factors for choroidal nevus transformation into melanoma.Methods:Retrospective chart review of 3806 consecutive choroidal nevi with imaging and 2355 choroidal nevi with additional follow up to identify factors predictive of transformation

AimsTo investigate the association of peripheral retinal non-perfusion with retinal haemangioblastoma.MethodsMedical and widefield fluorescein angiography records of patients diagnosed with retinal haemangioblastoma from 1990 to 2018 were reviewed for patient demographics, tumour features, fluorescein angiography features and characteristics of peripheral retinal non-perfusion.ResultsThere were 41 eyes of 40 patients with retinal haemangioblastoma imaged by widefield fluorescein angiography during this time period. Of 41 eyes, 14 (34%) had haemangioblastoma-associated peripheral retinal non-perfusion on fluorescein angiography. A comparison of eyes with versus without non-perfusion revealed younger mean age at presentation (28 vs 43 years old, p=0.05), increased prevalence of von Hippel-Lindau (VHL) disease (62% vs 22%, p=0.01), greater mean largest tumour basal diameter (3.7 vs 2.5 mm, p=0.04), greater tumour distance from optic nerve (8.4 vs 1.9 mm, pConclusionPeripheral retinal non-perfusion can be associated with retinal haemangioblastoma, and could be more common with larger, more peripheral tumours in younger patients with VHL disease. Eyes with haemangioblastoma-associated peripheral non-perfusion could be more likely to develop neovascularisation and lose visual acuity.

AimTo characterise combinations of multimodal imaging risk factors and predictive value for choroidal nevus transformation into melanoma.MethodsThis is a retrospective review of multimodal imaging features for 3806 choroidal nevi from 1 January 2007 through 1 January 2017. Kaplan-Meier estimates and Cox regression analyses were used to calculate 5-year percentages of growth to melanoma and HR.ResultsUsing multimodal imaging, six risk factors predictive of choroidal nevus transformation into melanoma were identified, namely tumour thickness >2 mm, subretinal fluid, symptoms of visual acuity loss to 20/50 or worse, orange pigment, hollow acoustic density and tumour largest basal diameter >5 mm. Kaplan-Meier 5-year estimated tumour growth was found in 1% of nevi with no risk factors, 11% (range 9%–37%) with one factor, 22% (12%–68%) with two factors, 34% (21%–100%) with three factors, 51% (0%–100%) with four factors and 55% (0%–100%) with five factors. HR for growth was 0.1 with no factor, 2.1–7.8 with one factor, 1.8–12.1 with two factors, 4.0–24.4 with three factors, 4.6–170.0 with four factors and 12.0–595.0 with five factors. The highest HR with each combination of two, three, four or five risk factors always included symptoms of visual acuity loss and orange pigment.ConclusionSix risk factors for choroidal nevus transformation into melanoma by multimodal imaging have been identified. Risk for transformation into melanoma is 1% when no factors are present, and approaches 100% with specific combinations of three or more risk factors. Understanding how combinations of factors influence risk of transformation into melanoma can guide counselling and treatment decisions.

Iridociliary ring melanoma is an uncommon type of uveal melanoma. Clinical manifestation varies from asymptomatic cases to masquerade syndromes mimicking refractory glaucoma. Treatment options include radiotherapy and enucleation. Management of metastatic uveal melanoma remains discouraging. Novel therapies using immune checkpoint inhibitors are currently under study. We present a case of a 54-year-old Hispanic woman with progressive vision loss due to metastatic ring melanoma with anterior chamber seeding treated with pembrolizumab.

Retinoblastoma management remains complex, requiring individualized treatment based on International Classification of Retinoblastoma (ICRB) staging, germline mutation status, family psychosocial factors and cultural beliefs, and available institutional resources. For this 2020 retinoblastoma review, PubMed was searched for articles dated as early as 1931, with an emphasis on articles from 1990 to the present day, using keywords of retinoblastoma, chemotherapy, intravenous chemotherapy, chemoreduction, intra-arterial chemotherapy, ophthalmic artery chemosurgery, intravitreal chemotherapy, intracameral chemotherapy, cryotherapy, transpupillary thermotherapy, laser, radiation, external beam radiotherapy, plaque radiotherapy, brachytherapy, and enucleation. We discuss current treatment modalities as used in the year 2020, including intravenous chemotherapy (IVC), intra-arterial chemotherapy (IAC), intravitreal chemotherapy (IvitC), intracameral chemotherapy (IcamC), consolidation therapies (cryotherapy and transpupillary thermotherapy [TTT]), radiation-based therapies (external beam radiotherapy [EBRT] and plaque radiotherapy), and enucleation. Additionally, we present a consensus treatment algorithm based on the agreement of three North American retinoblastoma treatment centers, and encourage further collaboration amongst the world's most expert retinoblastoma treatment centers in order to develop consensus management plans and continue advancement in the identification and treatment of this childhood cancer.

PURPOSE To evaluate spectral domain optical coherence tomography (SD-OCT) features of vitreoretinal lymphoma (VRL). METHODS Review of records and SD-OCT images of vitreoretinal lymphoma evaluated at Ocular Oncology Service, Wills Eye Hospital between July 1, 2000, and April 1, 2019. RESULTS There were 55 eyes of 32 patients included. At presentation, SD-OCT features included vitreous opacities (n = 36, 65%), preretinal deposits (n = 7, 13%), intraretinal deposits (n = 8, 15%), subretinal deposits (n = 20, 36%), retinal pigment epithelium abnormalities (n = 35, 64%), and subretinal pigment epithelium deposits (n = 35, 64%). Of 36 eyes with observed tumor progression, comparison (initial visit vs. time of progression) revealed more intraretinal deposits (17% vs. 50%, P = 0.005) at progression. Of 15 eyes with tumor recurrence, comparison (initial visit vs. time of recurrence) revealed more intraretinal deposits (7% vs. 47%, P = 0.04) at recurrence. At last visit, 39 eyes demonstrated tumor regression. By comparison (initial presentation vs. regression), there were less frequent vitreous opacities (67% vs. 0%, P < 0.001), intraretinal deposits (15% vs. 0%, P = 0.03), subretinal deposits (36% vs. 0%, P < 0.001), and subretinal pigment epithelium deposits (69% vs. 21%, P < 0.001) at regression. CONCLUSION Using SD-OCT in patients with vitreoretinal lymphoma, local tumor regression correlated with a reduction in vitreous opacities, intraretinal deposits, subretinal deposits, and subretinal pigment epithelium deposits. SD-OCT is useful in judging vitreoretinal lymphoma response to therapy.

Early diagnosis and positive outcomes of retinoblastoma in childhood have been positively correlated with the economic wealth of high-income countries (HICs) worldwide. Adequate curability and survival rates, adherence to treatment, presence of poor prognostic initial clinical signs, and metastatic disease at diagnosis appear to have a less favorable picture in low-income countries (LICs). However, this is not always the case. An example is Argentina, where disease-free survival rates of retinoblastoma are notably higher than expected when taking into consideration its economic situation. Unfortunately, as in other Latin American LICs, retinoblastoma outcomes in Mexico are worrisome. Interestingly, the Human Development Index (HDI) in Mexico varies widely between its different geographical regions. While in some states, the HDI resembles those of high-income countries, and in others, the opposite is observed. A unifying picture of Mexico’s developmental status, health resources, indicators, and other factors possibly influencing outcomes in retinoblastoma is currently unavailable. The present review explores the previously mentioned factors in Mexico and compares them to other countries. Additionally, it recommends solutions or enhancements where possible.

Purpose: To report our 5-year experience with intra-arterial chemotherapy (IAC) in the intravitreal chemotherapy (IvitC) era. Methods: Retrospective review of retinoblastoma treated with primary unilateral IAC in the IvitC era (2012–2017). Results: There were 34 eyes treated with IAC alone versus 20 eyes treated with IAC plus IvitC for vitreous seeds. IAC (IAC alone vs. IAC plus IvitC) consisted of melphalan (41 vs. 10%) or melphalan plus topotecan (59 vs. 90%, p = 0.03). IvitC consisted of melphalan (60%) or melphalan plus topotecan (40%). Tumor control and globe salvage were achieved in 100% of group B and C eyes without IvitC. Despite more extensive vitreous seeds in the IvitC group (p < 0.01), comparison of IAC alone versus IAC plus IvitC revealed no difference in tumor control for group D (88 vs. 69%, p = 0.36) or group E (67 vs. 100%, p = 0.25) and no difference in globe salvage for group D (88 vs. 69%, p = 0.36) or group E (58 vs. 57%, p = 0.39). Conclusions: IAC is effective as primary therapy for unilateral group B, C, D, and E retinoblastoma. IvitC is an important adjuvant therapy to achieve comparable globe salvage rates for group D and E eyes with persistent active vitreous seeds.

Fallbericht: Vorgestellt wird der Fall eines 29-jährigen Mannes, der nach Implantation einer Ahmed-Drainage in beiden Augen zur Behandlung eines uveitischen Glaukoms postoperativ eine bilaterale okuläre Dekompressionretinopathie entwickelte. Diskussion: Die okuläre Dekompressionsretinopathie ist eine seltene Komplikation der filtrierenden Glaukomchirurgie; sie verläuft jedoch meist gutartig, die Blutungen bilden sich spontan zurück und es kommt zu einer Erholung der Sehschärfe. Übersetzung aus Case Rep Ophthalmol 2016;7:227-232

Purpose To describe the echographic characteristics of optic disk melanocytoma using a high resolution 10–20 MHz ophthalmic ultrasound. Methods We conducted a 10-year retrospective review finding 9 cases with optic disk melanocytoma. The echographic studies were performed by the same experienced ophthalmologist. The form and density of the tumors were evaluated with B-scan ultrasound. Internal reflectivity and vascularity of the tumors were assessed with a standardized A-scan. Base (vertical and horizontal) and height of the tumor were obtained by using both ultrasounds AB modes. Results The mean age at diagnosis was 43.88 years. There was no evidence of abnormal tumor vascularization in any of the cases. Mean (SD) vertical measurement of the base was 2.53 mm (±1.47). Mean (SD) horizontal measurement of the base was 2.49 mm (±1.03). Mean height (SD) was 1.52 mm (±0.88). Of the assessable cases, 78% had high internal reflectivity, the remaining 22% had medium-high internal reflectivity. All tumors’ internal structure was characteristically homogeneous. Mean follow-up was 33.6 months. Conclusion Melanocytomas are small, benign tumors that are highly assessable by ocular ultrasound when their elevation surpasses 0.5 mm. In our study, the internal reflectivity ranged from high to very high, unlike other malignancies such as choroidal melanoma which tend to present with low internal reflectivity. The avascularity of the tumor is a common finding. Ultrasound is a remarkable tool that helps detect benign characteristics in a pigmented optic disk tumor and helps establish a more reliable diagnosis.

Purpose The aim of this study is to expand the limited knowledge regarding autologous contralateral penetrating keratoplasty. Methods We report the retrospective outcomes of patients who received autokeratoplasty and contralateral opaque corneas in the donor eye at a tertiary care ophthalmology hospital in Mexico City. Results Eleven patients received autokeratoplasty and contralateral opaque corneas in the donor eye at our center from 2010 to 2015. The mean patient age at the time of surgery was 58 years (range, 35-85 yrs), with 4 female and 7 male patients. There were no surgical or immediate postsurgical complications in the autokeratoplasty eye. However, 1 patient had expulsive hemorrhage in the sightless eye. Follow-up duration ranged from 11 to 65 months (mean, 26 mo). During follow-up, 3 of the autokeratoplasty procedures failed because of endothelial attenuation. Identified known risk factors for failure of the eye with visual potential included the presence of an Ahmed glaucoma drainage device in 7/11 patients (63%), history of glaucoma in 8/11 (72%), past heterologous penetrating keratoplasty in 2/11 (18%), Vogt-Koyanagi-Harada syndrome in 1/11 (9%), and 4-quadrant corneal vascularization in 1/11 (9%). Conclusions Autokeratoplasty is a good choice in cases having high risk factors and when fresh corneal tissue is not available. This is the largest study describing outcomes of patients who underwent autokeratoplasty. This technique offers no risk of immune rejection and no need for immunosuppression treatment. This study reports a good prognosis in cases having high risk factors for failure.

Case Report: We report the case of a 29-year-old man who underwent Ahmed valve implantation in both eyes as treatment for uveitic glaucoma, subsequently presenting with bilateral ocular decompression retinopathy in the postoperative period. Discussion: Ocular decompression retinopathy is a rare complication of filtering surgery in patients with glaucoma; however, the course is benign in most cases, with spontaneous resolution of bleedings and improvement of visual acuity.

PURPOSE To evaluate the outcome of photodynamic therapy (PDT) in the management of extrafoveolar choroidal osteoma. METHODS The authors performed a retrospective chart review of all patients with choroidal osteoma that did not involve the foveola and were treated with standard-fluence PDT. RESULTS Nine eyes with extrafoveolar choroidal osteoma were studied. Mean logarithm of the minimum angle of resolution best-corrected visual acuity at initial examination was 0.07 (Snellen ∼20/25). The osteoma was treated with 1 (8/9) or 2 (1/9) PDT sessions using 50 J/cm. After a mean follow-up of 49 months, the treated area of osteoma demonstrated complete (4/9) or partial (5/9) regression, with a mean of 73% regression in the PDT-treated areas. Tumor growth in the region of PDT was noted in 3 cases (3/9) (one tumor toward the foveola and two tumors at the margin away from the foveola), but in no case did the tumor reach the foveola. Therefore, PDT controlled tumor growth in 8 of 9 cases with only 1 of 9 cases showing growth through the PDT scar into foveola. Mean logarithm of the minimum angle of resolution visual acuity at last follow-up was 0.04 (Snellen ∼20/20) (P = 0.59). CONCLUSION Photodynamic therapy is an effective modality for the management of extrafoveolar choroidal osteoma, minimizing tumor growth toward the foveola and preserving visual acuity.

Prostaglandin analogues (PGAs) have become the first-line therapy for patients with glaucoma.1 PGAs promote aqueous humor outflow by the uveoscleral pathway and, to a lesser extent, through the trabecular meshwork and Schlemm’s canal, which are part of the conventional pathway.2 This effect is due to connective tissue remodeling by active metalloproteinases in the ciliary body and trabecular meshwork.3 Recent studies suggest that corneal biomechanical properties may provide important information about glaucoma progression, such as increased deformation of the optic nerve surface.4 Corneal hysteresis (CH) is considered to be a direct measurement of corneal biomechanical properties, with central corneal thickness (CCT) being one of the factors directly related to it.5 Corneal resistance factor (CRF) is also believed to be useful in predicting glaucoma progression and may be correlated with CCT.6,7 Recently Tsikripis et al8 prospectively analyzed the effect of latanoprost and latanoprost/timolol combination on corneal biomechanical properties and CCT and concluded that low CH and a thin cornea may lead to underestimation of intraocular pressure (IOP); they suggested that the Ocular Response Analyzer (ORA; Reichert Ophthalmic Instruments, Depew, NY) be used in combination with Goldmann applanation tonometry (GAT) to obtain more accurate and reliable IOP values. Multiple articles have analyzed the effects on corneal biomechanics of specific PGAs, including latanoprost or bimatoprost.9 Agarwal et al10 retrospectively compared the relationship between CH and the magnitude of IOP reduction with PGA, and they found that baseline CH is independently associated with the magnitude of IOP reduction with PGA therapy. Experimental studies have demonstrated that PGAs alter corneal collagen structure and affect CCT.10 These changes imply that results of GAT could be affected by PGA treatment.11–13 In a study of 136 human eyes, Schlote et al12 found that topical therapy with travoprost was accompanied by a significant reduction of CCT within 1 year of treatment. In a study with F2α prostaglandin, Viestenz et al found that CCT in PGA-treated eyes decreased significantly, suggesting that these changes might be attributed to effects of F2α PGA on the extracellular matrix of corneal stroma via upregulation of matrix metalloproteinases.13 It is not known whether PGAs induce changes in corneal biomechanical structure or, subsequently, affect GAT measures, which assume that there is minimal variation due to the biomechanical properties of the cornea and to CCT. However, in clinical practice, corneal thinning under local effect of PGA treatment could result in underestimation of IOP levels as measured by applanation tonometry.14,15 Bergonzi et al16 used A-scan laser confocal microscopy to analyze 52 eyes that had only been on PGA therapy for 3 years and concluded that PGA therapy increased the keratocyte density in each layer of corneal stroma but more significantly in the anterior stroma than in the midstroma or the posterior stroma. The increased keratocyte density might be the result of diminished extracellular matrix, possibly owing to the activation of matrix metalloproteinases and inhibition of their tissue inhibitors.17 The current interest in improving methods of IOP measurement has led to study of the influence of corneal biomechanical properties on the development of glaucoma. The Ocular Response Analyzer performs measurements of corneal properties such as corneal hysteresis (CH) and corneal resistance factor (CRF) and incorporates both Goldmann-correlated IOP (IOPg) and corneal-corrected IOP (IOPcc), a measurement that is minimally influenced by corneal properties.17 CH is defined as the difference between the air-jet pressure at inward and outward applanation and is considered to be a measure of corneal viscous damping and thus the cornea’s resistance to deformation.18–20 Lower CH values have been linked with progression of visual fields in primary open-angle glaucoma (POAG); lower CH values, with worse visual field damage in patients with asymmetric POAG.11 The purpose of the present study was to determine whether topical application of travoprost 0.004% induces changes in corneal structure and biomechanical properties affecting intraocular pressure measures in rabbits.

Purpose: Intra-arterial chemotherapy (IAC) has become a mainstay in the management of retinoblastoma, especially in advanced or refractory disease. However, IAC is not without complications, and chemotherapy toxic effects can lead to partial or complete choroidal ischemia, often causing vision loss. Methods: This is a case report. Results: A 4-month-old girl with bilateral retinoblastoma was treated with secondary IAC (melphalan 5 mg) for recurrent tumor following intravenous chemotherapy. One month later, complete tumor control was achieved. However, she demonstrated broad choroidal ischemia in the nasal and temporal quadrants but sparing of the watershed zone superior and inferior to the optic disc and in the papillomacular region. Fluorescein angiography revealed poor perfusion of the choriocapillaris with visibility of the large choroidal vessels in the nasal and temporal areas but preserved perfusion of the watershed zone. The watershed zone remained intact on the 10-month follow-up, and the final visual acuity was fix and follow without strabismus. Conclusion: The pathophysiology of choroidal ischemia is not well understood, but the fortuitous watershed zone preservation in this case could represent uneven distribution of the chemotherapeutic drug, resulting in partial chemo-dilution of the medication in the watershed region, which represents the final downstream overlapping choroidal perfusion from both medial and lateral posterior ciliary arteries.