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BACKGROUND/OBJECTIVES: To analyze the ophthalmic characteristics of congenital prepapillary vascular loop (PVL) and to propose a new morphologic classification dividing the loops into six types. SUBJECTS/METHODS: Collaborative multinational multicentre retrospective study of PVL cases. RESULTS: There was a total of 49 cases (61 eyes), 37 unilateral (75.5%) and 12 bilateral (24.5%), 32 arterial type (65.3%) and 18 venous type (36.7%) (one patient had either kind in each eye). The mean number of loops per eye was 2.7 (range, 1-7). The loops were asymptomatic in 42 cases (85.7%). Other findings included: the presence of cilioretinal artery (14 cases), retinal vascular tortuosity (26 cases), amaurosis fugax (1 case), branch retinal artery occlusion (1 case) and vitreous haemorrhage (3 cases). Six morphologic loop types could be discerned based on elevation (flat vs. elevated), shape (figure of 8 or corkscrew with hyaline sheath), number (multiple or single), location (central or peripheral), lumen size (arterial vs. arteriolar) and presence of vascular tortuosity or vitreous traction. CONCLUSIONS: PVL are usually asymptomatic and can be divided into six morphologic types with different pathogenesis during early embryogenesis.

Introduction: The prognostic 15-gene expression profile (15-GEP) test for uveal melanoma (UM) predicts metastatic risk based on primary tumor biology. Here we report outcomes from a prospective registry of 15-GEP-tested patients, and a meta-analysis with published cohorts. Objectives: Management and 5-year clinical outcomes following 15-GEP testing were evaluated. Methods: Eighty-nine patients with 15-GEP results were prospectively enrolled at four centers. Physician-recommended management plans were collected, and clinical outcomes tracked every 6 months. Results: Eighty percent of Class 1 (low-risk) patients underwent low-intensity management; all Class 2 (high-risk) patients underwent high-intensity management (p < 0.0001). Median follow-up for event-free patients was 4.9 years. Five Class 1 (10%) and 23 Class 2 (58%) tumors metastasized (p < 0.0001). Five-year Class 1 and 2 metastasis-free survival rates were 90% (81–100%) and 41% (27–62%; p < 0.0001), and melanoma-specific survival rates were 94% (87–100%) and 63% (49–82%; p = 0.0007). Class 2 was the only independent predictor of metastasis and was associated with increased risk for metastasis and mortality by meta-analysis. Conclusions: UM patient management is guided by 15-GEP testing. Class 2 patients were managed more intensely, in accordance with an observed metastatic rate of >50%; Class 1 patients were safely spared intensive surveillance, resulting in appropriate utilization of healthcare resources.

Purpose: We report a case of multiple extramedullary plasmacytoma (EMP) of the choroid and conjunctiva as a sign of multiple myeloma (MM) relapse. Methods: An observational case report with clinicopathologic correlation is presented. Results: A 45-year-old man with a 2.5-year history of MM presented with left eye pain. Examination revealed a conjunctival nodule and choroidal infiltrate in the left eye. Excisional biopsy demonstrated plasmacytoma of the conjunctiva and choroid. Despite radiotherapy and chemotherapy, multiple EMP recurred and the patient died 8 months after initial presentation. Conclusions: Multiple EMP of the choroid and conjunctiva has a poor prognosis and can present as a sign of MM relapse. Our imaging demonstrates the clinical manifestations of this tumor and can aid future diagnoses. Our radiation dose (20 Gy) was inadequate, which is an important reportable factor for future patients.

PURPOSE: Assess systemic vascular endothelial growth factor-A (VEGF) levels after treatment with intravitreous aflibercept, bevacizumab or ranibizumab. DESIGN: Comparative-effectiveness trial with participants randomly assigned to 2-mg aflibercept, 1.25-mg bevacizumab, or 0.3-mg ranibizumab following a retreatment algorithm. PARTICIPANTS: Participants with available plasma samples (N=436) METHODS: Plasma samples were collected before injections at baseline, 4-week, 52-week and 104-week visits. In a pre-planned secondary analysis, systemic free-VEGF levels from an ELISA immunoassay were compared across anti-VEGF agents and correlated with systemic side effects. MAIN OUTCOME MEASURES: Changes in the natural log (ln) of plasma VEGF levels. RESULTS: Baseline free-VEGF levels were similar across all 3 groups. At 4 weeks, mean ln(VEGF) changes were −0.30±0.61, −0.31±0.54, −0.02±0.44 pg/ml for the aflibercept, bevacizumab, and ranibizumab groups, respectively. The adjusted differences between treatment groups (adjusted CI; P-value) were −0.01 (−0.12, +0.10; P=0.89), −0.31 (−0.44, −0.18; P

PURPOSE: For treatment of choroidal melanoma, Palladium-103 ((103)Pd) and Ruthenium-106 ((106)Ru) plaque brachytherapy demonstrates reduced toxicity compared to the historical standard Iodine-125 ((125)I). No report has directly compared clinical outcomes between (103)Pd and (106)Ru, and reasons for selection of one over the other remain purely theoretical. MATERIALS/METHODS: Patients with choroidal melanoma with apical tumor height ≤ 5 mm were included. Patients from Emory University were treated with (103)Pd between 1993 and 2012. Patients from Cleveland Clinic were treated with (106)Ru between 2005 and 2010. Medical records were retrospectively reviewed. We compared post-treatment visual acuity (VA), toxicity, and oncologic outcomes. RESULTS: (103)Pd patients (n = 124) and (106)Ru patients (n = 42) had median follow up of 4.2 and 5.0 years, respectively. Radiation retinopathy-free survival was similar for both radioisotopes but (106)Ru had lower grades of retinopathy (p=0.006). (103)Pd was associated with worse VA preservation (≥20/40) by year 3 (OR 3.8, 95% CI 1.01–14.31, p=0.048). (103)Pd was associated with higher distant metastases-free survival (DMFS) in multivariate analysis (MVA) (HR 0.10, 95% CI 0.02–0.38, p

We report the case of a juvenile patient presenting with periocular eczema, a milky white cataract in the right eye, and iridoschisis. Although cataracts are commonly associated with iridoschisis, only 1 previous case of periocular eczema and iridoschisis has been reported. Our case raises the possibility of a pathophysiologic link between iridoschisis, juvenile cataract formation, and periocular eczema.

Purpose To evaluate outcomes of choroidal melanoma patients treated with 125I or 103Pd plaque brachytherapy. Methods and Materials From 1993 to 2012, our institution treated 160 patients with 103Pd (56.1%) and 125 patients with 125I (43.9%) plaque brachytherapy. Tumor outcomes, visual acuity (VA), and toxicity were compared. Multivariate analyses (MVAs) and propensity score analysis were used to help address differences in baseline characteristics. Results Median followup was longer for 125I patients, 52.7 vs. 43.5 months (p 90% 3-year overall survival and >93% 5-year secondary enucleation-free survival. On MVA, radionuclide was not predictive for tumor outcomes. A higher percentage maintained vision better than 20/40 with 103Pd (63% vs. 35%, p = 0.007) at 3 years. MVA demonstrated 103Pd radionuclide (odds ratio [OR]: 2.12, p = 0.028) and tumor height ≤5 mm (OR: 2.78, p = 0.017) were associated with VA better than 20/40. Propensity score analysis matched 23 125I with 107 103Pd patients. 103Pd continued to predict better VA at 3 years (OR: 8.10, p = 0.014). On MVA for the development of VA worse than 20/200 or degree of vision loss, radionuclide was not significant. Lower rates of radiation retinopathy were seen with 103Pd than 125I (3 years: 47.3% vs. 63.9%, p = 0.016), with radionuclide significant in MVA. Conclusions Both 125I and 103Pd achieve excellent tumor control. An increased probability of long-term VA better than 20/40 and reduced risk of radiation retinopathy is associated with 103Pd.

To describe the development of uveitis and retinal vasculitis in association with pembrolizumab treatment for metastatic uveal melanoma. A case report and a brief review of the literature are presented. Information collected and reported include the patient's clinical course, physical examination findings, fluorescein angiogram images, retinal photographs, and her response to treatment. A 54-year-old woman was diagnosed with a large choroidal malignant melanoma and had the affected eye enucleated. Pathology confirmed a mixed cell choroidal melanoma, and gene expression profiling was Class 2. Seventeen months after enucleation, the patient was diagnosed with metastatic uveal melanoma to the liver. Disease progression was observed during ipilimumab treatment. Pembrolizumab treatment was initiated, and after four infusions, she presented to clinic complaining of floaters and blurred vision. Examination revealed a nongranulomatous panuveitis characterized by perivascular retinal pigment epithelium pigmentary changes, retinal venous sheathing, 1+ anterior chamber and vitreous cellular reaction, 2+ vitreous haze, and optic disk edema. A dexamethasone sustained-release implant was administered and the uveitis regressed. A relapse in symptoms occurred but quickly subsided with a repeat injection. Pembrolizumab may induce a uveitic reaction. There is mounting evidence that patients using prembrolizumab should be educated and monitored for signs of uveitis.

Introduction: Uveal melanoma (UM) is a highly aggressive eye cancer that leads to metastatic death in up to half of patients. UM has a propensity to undergo early micrometastasis prior to treatment of the primary tumor, with later emergence of overt metastatic disease. UM can be stratified into Class 1 (low metastatic risk) and Class 2 (high metastatic risk) using a prospectively validated 12-gene expression profile (GEP) molecular prognostication classifier. The Collaborative Ocular Oncology Group Study 2 (COOG2) is being conducted to prospectively evaluate new prognostic biomarkers in UM, including expression of PRAME, and prognostic driver mutations in BAP1, SF3B1, and EIF1AX. Methods: The COOG2 is a 24 center, prospective study of UM patients undergoing prognostic testing using the CLIA-certified DecisionDx-UM test (Castle Biosciences, Inc) between July 2017 and December 2019. Molecular analysis is performed on residual clinical samples. Data collected include clinical annotations, patient outcomes, GEP, PRAME status, and mutations in BAP1, SF3B1, and EIF1AX. Results: To date, there are 1300 participants enrolled. The baseline characteristics are as follows: 52% male, and 48% female; 87% non-Hispanic, 3% Hispanic, and 10% unknown. GEP classification is 66% class 1 and 34% class 2. PRAME status is 68% PRAME (-) and 32% PRAME (+). Targeted sequencing has been performed on 650 samples and is being analyzed. Conclusions: The COOG2 will establish a new standard of prognostic testing accuracy from a single fine needle biopsy in UM. The resulting integrated prognostic testing platform will be used as a stratification tool for entry into clinical trials of adjuvant therapy in high-risk patients. Citation Format: Christina L. Decatur, Michael A. Durante, Zelia M. Correa, Thomas M. Aaberg, Prithvi Mruthyunjaya, Amy C. Schefler, J. William Harbour. The Collaborative Ocular Oncology Group study number 2: A prospective multi-center evaluation of uveal melanoma prognostic biomarkers [abstract]. In: Proceedings of the Annual Meeting of the American Association for Cancer Research 2020; 2020 Apr 27-28 and Jun 22-24. Philadelphia (PA): AACR; Cancer Res 2020;80(16 Suppl):Abstract nr 5442.

Purpose To describe a new classification of stellate nonhereditary idiopathic foveomacular retinoschisis (SNIFR). Design Retrospective case series and literature review. Participants A total of 17 patients from 5 institutions. Methods Detailed case history, multimodal imaging, and genetic testing were reviewed for patients with macular schisis without a known predisposing condition. Patients with a stellate appearance centered on the fovea with correlating confirmed expansion of the outer plexiform layer (OPL) by optical coherence tomography (OCT) were included. Exclusion criteria included a family history of macular retinoschisis, a known genetic abnormality associated with retinoschisis, myopic traction maculopathy, epiretinal membrane, vitreoretinal traction, optic or scleral pit, or advanced glaucomatous optic nerve changes. Main Outcome Measures Clinical features, anatomic characteristics, and visual acuity. Results A total of 22 eyes from 16 female patients and 1 male patient with foveomacular schisis were reviewed from 5 institutions. Initial visual acuity was ≥20/50 in all eyes (mean, 20/27), but visual acuity in a single eye decreased from 20/20 to 20/200 after the development of subfoveal fluid. The refractive status was myopic in 16 eyes, plano in 3 eyes, and hyperopic in 2 eyes. Three eyes had a preexisting vitreous separation, and 19 eyes had an attached posterior hyaloid. Follow-up ranged from 6 months to >5 years. Conclusions In this largest known series of patients with SNIFR, all patients demonstrated splitting of the OPL in the macula with relatively preserved visual acuity (≥20/40) except in a single patient in whom subretinal fluid developed under the fovea.

To evaluate outcomes of choroidal melanoma patients treated withFrom 1993 to 2012, our institution treated 160 patients withMedian followup was longer forBoth

We report a patient with clinical anophthalmia, partial eyelid fusion and a hypoplastic socket on the right. The left eye has microphthalmia involving the anterior and posterior segments, microcornea, iris coloboma, chorioretinal dysgenesis, macular dysplasia, absence of retinal vessels, and optic nerve aplasia. Systemic abnormalities include microcephaly, bilateral hearing loss, and duodenal atresia. Electrophysiologic testing showed no response from either eye. Cytogenetic testing revealed a de novo interstitial deletion of chromosome 14q22.3q23.1. The literature of similar interstitial deletions and ongoing candidate gene studies are reviewed.

PURPOSE The purpose of this study was to describe an unusual presentation of retinoblastoma in an adult. METHODS Retrospective case report. RESULTS A 48-year-old woman with floaters was found to have an amelanotic intraocular mass with localized vitreous seeds in the superior fundus. Metastatic workup was negative. Cytologic examination of the vitrectomy specimen showed a malignant neoplasm with neuroendocrine differentiation, suspicious for carcinoid tumor. The patient later was found to have a lung lesion presumed to be a carcinoid tumor. She was treated with external beam radiation to the eye, but 1 year later, a new retinal tumor was discovered inferiorly requiring plaque radiotherapy. Soon thereafter, she developed massive intraocular recurrence. The eye was enucleated. Histopathology revealed small round blue cells with round hyperchromatic nuclei. Rosettes were also present within the tumor consistent with retinoblastoma. CONCLUSION Late-onset retinoblastoma can occur in adults, and the diagnosis can be challenging.

Purpose: To report the macular hole closure rate and visual outcomes of patients with residual triamcinolone acetonide in the fovea after macular hole repair. Methods: We reviewed the medical records of consecutive patients who underwent macular hole surgery at our institution between 2005 and 2008. Only patients with visible triamcinolone in the fovea in the first postoperative month were included. Results: Six patients with Stage III or IV macular holes were included. All patients underwent pars plana vitrectomy, internal limiting membrane peeling, and gas tamponade. Triamcinolone acetonide was used to visualize the vitreous in every patient, and diluted indocyanine green was used to stain the internal limiting membrane in five patients. The median preoperative best-corrected visual acuity was 20/200, which improved to a median of 20/40 at the last follow-up. Five patients eventually developed retinal pigment epithelial alterations. Anatomic hole closure was achieved in every patient. The mean follow-up was 23 months (range, 3-36 months). Conclusion: Residual triamcinolone sequestered in the fovea after macular hole surgery did not affect hole closure or prevent improvement in visual acuity. It is unclear whether the retinal pigment epithelial alterations in our patients represent toxicity or are unrelated to the triamcinolone exposure.

Aims To assess the characteristics and outcomes of patients with hydroxychloroquine retinopathy and to review the current screening guidelines. Methods Retrospective chart review of patients diagnosed as having hydroxychloroquine retinopathy at our institution between 2004 and 2008. Results All seven patients were women. While every patient received 400 mg of hydroxychloroquine per day, every patient exceeded the recommended daily dosage allowance (6.5 mg/kg/day). The mean daily dose of hydroxychloroquine was 8.2 mg/kg/day (range: 6.8–13.6 mg/kg/day). The mean duration of usage was 16.3 years (range: 8.5–30 years), and the mean cumulative dose was 2377 g (range: 1241–4380 g). The mean visual acuity at presentation was 20/30, but all patients exhibited significant visual-field defects. Colour vision was impaired in six patients. Four patients exhibited granular hyperpigmentation in the central macula, and three had a bull9s eye appearance. The mean follow-up time was 21 months. The visual outcomes remained stable for every patient except for one patient who experienced an improvement in visual function after drug cessation. Conclusions Hydroxychloroquine retinopathy, although rare, still exists despite current screening guidelines. The authors recommend that physicians dose hydroxychloroquine according to lean body weight and that they use risk stratification to guide their screening regimens.

Thomas M Aaberg Jr,1 RobertW Cook,2 Kristen Oelschlager,2 Derek Maetzold,2 P Kumar Rao,3 John O Mason III41Michigan State University Medical School and Retina Specialists of Michigan, Grand Rapids, MI, 2Castle Biosciences, Friendswood, TX, 3Washington University School of Medicine in St Louis, St Louis, MO, 4Retina Consultants of Alabama and University of Alabama Birmingham, Birmingham, AL, USA Objective: Assess current clinical practices for uveal melanoma (UM) and the impact of molecular prognostic testing on treatment decisions.Design: Cross-sectional survey and sequential medical records review.Participants: Ophthalmologists who treat UM.Methods: (A) Medical records review of all Medicare beneficiaries tested by UM gene expression profile in 2012, conducted under an institutional review board-approved protocol. (B) 109 ophthalmologists specializing in the treatment of UM were invited to participate in 24-question survey in 2012; 72 were invited to participate in a 23-question survey in 2014.Main outcome measures: Responses analyzed by descriptive statistics, frequency analyses (percentages, Tukey, histograms), and Fisher’s exact test. Descriptive presentation of essay answers.Results: The review of Medicare medical records included 191 evaluable patients, 88 (46%) with documented medical treatment actions or institutional policies related to surveillance plans. Of these 88, all gene expression profiling (GEP) Class 1 UM patients were treated with low-intensity surveillance. All GEP Class 2 UM patients were treated with high-intensity surveillance (P

PURPOSE To report a patient with bilateral choroidal metastasis from a malignant paraganglioma. METHODS Clinicopathologic case report and literature review. RESULTS A 68-year-old woman presented with bilateral amelanotic focal choroidal lesions. A thorough systemic work-up for a primary cancer revealed a paraganglioma (extraadrenal pheochromocytoma) and a pheochromocytoma of the left adrenal gland. Fine-needle aspiration biopsy of the choroidal lesion was consistent with metastatic paraganglioma. CONCLUSION Metastatic paraganglioma, although rare, has the ability to metastasize to the choroid.

Purpose To review the clinicopathologic features of eyes enucleated after failed transpupillary thermotherapy. Design Retrospective review. Methods Pathology reports in the L.F. Montgomery Laboratory between 1998 and 2002 were searched for enucleated eyes with choroidal melanoma that had been treated only by transpupillary thermotherapy (TTT) prior to enucleation. The clinical features of the patients, including ultrasonography examination, were reviewed and compared with the pathologic findings. Results Seven eyes from seven patients had been enucleated, representing 8% of eyes treated with TTT at our institute during the period studied. The primary indication for enucleation was tumor growth. The melanomas tended to grow laterally, with minimal if any increase in thickness. Five of the seven eyes histologically demonstrated extrascleral extension, which was detected by ultrasonography prior to enucleation in only one of those eyes. Conclusions Choroidal melanoma may continue to grow along a path of least resistance after TTT. The melanoma may extend laterally in the choroid and through emissary canals. Early extrascleral extension may be difficult to detect by ultrasonography.

OBJECTIVE: To evaluate a method of obtaining conjunctival cells that utilizes aspiration of the conjunctival surface. STUDY DESIGN: Eighteen eyes from 16 patients with epibulbar lesions underwent aspiration of the conjunctival surface using a tuberculin syringe without a needle. The aspirated cells were prepared by the cytocentrifuge or Millipore filter technique. The lesions were subsequently biopsied, and the cytologic and histologic diagnoses were compared. RESULTS: Diagnoses of the conjunctival lesions, in decreasing order of frequency, were conjunctival intraepithelial neoplasia (dysplasia, carcinoma in situ), squamous cell carcinoma, squamous metaplasia, nevus, lymphoma and inflammation. In this feasibility study, the cytologic diagnosis accurately correlated with the histologic diagnosis in most cases. There were two cases in which the cytologic diagnosis led to the correct histologic diagnosis. CONCLUSION: Aspiration cytology of the conjunctival surface is useful in obtaining diagnostic material and may be used as a guide to where a biopsy should be performed. Limitations include inadequate samples and lack of observer experience.

Objective To evaluate the safety and effect on visual acuity of photodynamic therapy with verteporfin (Visudyne, Novartis AG) in patients with subfoveal choroidal neovascularization (CNV) secondary to the ocular histoplasmosis syndrome (OHS). Design Open-label, three-center, noncomparative prospective case series. Participants OHS patients with subfoveal CNV lesions no larger than 5400 μm in greatest linear dimension (GLD) with classic or occult CNV extending under the geometric center of the foveal avascular zone and best-corrected visual acuity letter score of 73 to 34 (approximate Snellen equivalent 20/40–20/200). Methods Twenty-six patients received verteporfin (6 mg/m 2 ) infused IV over 10 minutes. Fifteen minutes after the start of infusion, a laser light at 689 nm delivered 50 J/cm 2 at an intensity of 600 mW/cm 2 over 83 seconds using a spot size with a diameter 1000 μm larger than the GLD of the lesion. At 3-month follow-up examinations, retreatment with the same regimen was applied if angiography showed fluorescein leakage. Safety assessments were also made. Main outcome measures Visual function measurements were the changes from baseline in visual acuity and contrast sensitivity scores and the proportion of patients who, based on best-corrected visual acuity scores, (1) gained 7 or more letters, (2) lost 8 or more letters, and (3) lost 15 or more letters. Results One patient was omitted from the study at the month 3 examination for not meeting the visual acuity eligibility requirements at baseline. By the month 12 examination, but excluding any retreatment at that visit, patients had received an average of 2.9 treatments of a maximum of 4 possible treatments. The month 12 median improvement from baseline in visual acuity of the remaining 25 patients was 7 letters, and median contrast sensitivity improved by 2 letters. Median visual acuity improvement was also 7 letters when three patients, who failed to meet all photographic eligibility requirements at baseline, were excluded. At the month 12 examination, 14 (56%) patients gained 7 or more letters of visual acuity from baseline, whereas 4 (16%) patients lost 8 or more letters, of which 2 (8%) lost 15 or more letters. No serious systemic or ocular adverse events were reported. Conclusions Median visual acuity improved after verteporfin therapy for at least 1 year in a small uncontrolled prospective case series of patients with subfoveal CNV caused by OHS. Verteporfin therapy seemed to be safe and well tolerated in these patients. Two-year data from this study will provide important, additional information on the safety and effect of verteporfin therapy for the treatment of subfoveal CNV secondary to OHS.

Purpose: During the past decade, laser photoablation has supplanted cryotherapy as the standard treatment for threshold retinopathy of prematurity (ROP). We evaluated the progression and the complication rate in a cohort of infants with threshold ROP treated with a confluent technique. Methods: We retrospectively studied 47 patients with ROP (91 eyes), treated with confluent diode laser photoablation. Four main outcomes were evaluated: (1) the rate of progression, (2) the frequency of laser retreatment, (3) postoperative complications, and (4) postoperative refractive error. Results: A mean of 1943 ± 912 laser burns were administered in a confluent pattern to 91 eyes with threshold ROP. Progression to stage 4 or 5 disease occurred in 13 of 29 eyes (44.8%) with zone I and posterior zone II and in 2 of 51 eyes (3.9%) with anterior zone II ROP (P = .01). Eyes with anterior zone II ROP that received more than or equal to 2000 burns progressed more than those that received fewer than 2000 burns. Only 1 eye (1%) needed a supplemental laser treatment. Postoperative complications included corneal edema (2.3%), anterior segment ischemia (2.3%), vitreous hemorrhage (7.9%), posterior synechiae (2.3%), cataract (4.9%), and macular ectopia (12%).The mean spherical equivalent at the last follow-up was −4.52 ± 5.63 D. Conclusions: In our series, infants received more laser burns than infants reported in the literature receiving scatter or near-confluent treatment. While confluent treatment almost eliminated supplemental treatment, it was associated with a similar rate of progression and complications as has been reported with other patterns of laser treatment. (J AAPOS 2002;6:81-5)

PURPOSE: To report the results of macular hole surgery using indocyanine green to improve visualization and facilitate peeling of the internal limiting membrane. METHODS: A retrospective noncomparative review of a consecutive series of 22 patients (22 eyes) who underwent macular hole repair using indocyanine green to facilitate visualization of the internal limiting membrane was performed. One patient was excluded because of a history of a rhegmatogenous retinal detachment. All patients underwent a three-port pars plana vitrectomy with internal limiting membrane peeling. Indocyanine green (0.1% solution) was used to assist in the visualization of the internal limiting membrane. The main outcome measures were postoperative visual acuity, macular hole status, and postoperative retinal pigment epithelial changes. RESULTS: In 21 eyes, the median preoperative best-corrected visual acuity was 20/200 (range, 20/60 to counting fingers at 5 feet). The median postoperative visual acuity was 20/400 (range, 20/60–1/200) with an average follow-up of 13 weeks. The macular hole was closed in 18 eyes (86%) at the most recent follow-up. Ten eyes were found to have atrophic retinal pigment epithelium changes in the area of the previous macular hole. CONCLUSIONS: Indocyanine green assists in visualization of the internal limiting membrane in macular hole surgery. In our series, 10 eyes had unusual atrophic changes in the retinal pigment epithelium at the site of the previous macular hole, or in the area where the indocyanine green solution would have had direct access to the bare retinal pigment epithelium cells. Although the use of indocyanine green improves visualization and assists with peeling of the internal limiting membrane, the safety and potential toxicity of indocyanine green to the retinal pigment epithelium require further investigation.

Keywords: Photomedicine group Reference LPAS-ARTICLE-2001-009 Record created on 2007-07-20, modified on 2016-08-08

Objective To report the authors' clinical experience with submacular surgery for subfoveal membranes in children and to evaluate the histopathologic findings of membranes in children with various etiologies of choroidal neovascularization. Design Retrospective, noncomparative, interventional case series. Participants Twelve eyes of 12 consecutive children with subfoveal choroidal neovascularization treated by vitrectomy and excision of the choroidal neovascular complex. Intervention Vitrectomy, excision of the choroidal neovascular complex, and air-fluid exchange. Main outcome measures Visual acuity and recurrence of choroidal neovascular membrane. Results Preoperative visual acuities ranged from 20/60 to 20/800 (median, 20/300). Postoperative visual acuities ranged from 20/25 to 20/400 (median, 20/80) after an average follow-up of 20 months (range, 7–62 months). Ten of 12 eyes improved from immediate preoperative visual acuity, and four eyes developed recurrence of neovascular membranes over a mean follow-up of 18 months. Histopathologic examination of six excised membranes showed that the most common components of the membranes were retinal pigment epithelium, fibrocytes, vascular endothelium, and collagen. Conclusion Selected eyes of children with subfoveal neovascular membranes and no evidence of membrane regression may benefit from submacular surgery. The histopathologic findings were similar to adult choroidal neovascularization not associated with age-related macular degeneration.

Objective The purpose of the study was to evaluate the incidence of acute-onset (within 6 weeks after surgery) postoperative endophthalmitis and to assess the visual acuity outcomes after treatment over a 10-year period at one institution. Patients and methods This retrospective study reviews all surgical cases performed between January 1, 1984 and December 30, 1994 at the Anne Bates Leach Eye Hospital, Bascom Palmer Eye Institute, University of Miami Medical Center, for the occurrence of nosocomial acute-onset postoperative endophthalmitis. Results The overall 10-year incidence of acute-onset postoperative endophthalmitis after intraocular surgery was 0.093% (54/58, 123). The incidences of culture-proven acute-onset postoperative endophthalmitis by surgical category were as follows: cataract surgery with or without intraocular lens (IOL) (0.082%, 34/41, 654), pars plana vitrectomy (PPV) (0.046%, 3/6557), penetrating keratoplasty (0.178%, 5/2805), secondary IOL placement (0.366%, 5/1367), glaucoma surgeries (0.124%, 4/3233), combined trabeculectomy and cataract surgery (0.114%, 2/1743), and combined penetrating keratoplasty and cataract surgery (0.194%, 1/515). The median visual acuity after endophthalmitis treatment was 20/200. The median visual acuities after endophthal- mitis treatment by procedure were as follows: cataract surgery with or without IOL (20/133), PPV (no light perception), penetrating keratoplasty (2/200), secondary IOL implantation (20/40), glaucoma surgery (20/80), and combined trabeculectomy and cataract surgery with or without IOL (20/150). Conclusions The overall incidence of endophthalmitis after intraocular surgery was 0.093%. The incidence of endophthalmitis was higher after secondary IOL implantation than after cataract extraction ( P = 0.008, Fisher's exact test). After treatment, the visual acuity outcomes were worse in the patients who developed endophthalmitis after PPV than after cataract extraction, glaucoma procedures, or secondary IOL implantation ( P P

To evaluate a tissue sealant (autologous cryoprecipitate activated with bovine thrombin) as an adjuvant in macular hole surgery.Sixty-nine patients with stage 2, 3, or 4 full-thickness macular hole were enrolled consecutively in a prospective pilot study. Anatomic closure of the macular holes with a single operation was the primary outcome. Fifty-eight patients had pre- and postoperative standardized measurements including best refracted visual acuity, reading speed, and contrast sensitivity. Group A patients (45) had primary macular holes; Group B patients (13) had recurrent macular holes or macular holes with "other" retinal pathology. Surgical technique was standardized and membrane dissections were optional.The anatomic closure rate was 80% with a minimum of 6 months follow-up. Mean improvement in visual acuity for Group A (2.9+/-0.4 lines) was significantly better than for Group B (0.8+/-0.5 lines; P = 0.008). Eyes that underwent internal limiting membrane (ILM) dissections had an anatomic closure rate of 96% (23/24), compared with 71% (32/45) in "non-ILM" cases (P = 0.034). Adverse reactions included sterile hypopyon (10%), intraretinal hemorrhage (9%), pigmentary hyperplasia (3%), and retinal detachment (3%).Tissue sealants should be evaluated as an adjuvant in macular hole surgery in a randomized clinical trial. Inflammatory reactions may occur in some patients. Internal limiting membrane dissection may improve anatomic closure rates without adversely affecting the visual acuity.

Purpose To report giant retinal tear and retinal detachment as a complication of attempted removal of intravitreal lens fragments at the time of cataract surgery and to evaluate the anatomic and visual acuity outcomes of pars plana vitrectomy and retinal reattachment surgery. Methods Retrospectively, 10 patients with giant retinal tear, retinal detachment, and intravitreal lens fragments in 10 eyes were reviewed. Results In 10 eyes, retrieval of intravitreal lens fragments using the limbal approach by deep vitrectomy, copious vitreous cavity irrigation, or deep vitreous cavity phacoemulsification had been attempted by the anterior segment surgeon at the time of cataract surgery in each patient. The average interval from cataract surgery to the initial vitreoretinal consultation was 8 days. Each of the 10 eyes had a giant retinal tear involving the inferior retina: in four eyes, of between 90 degrees and 180 degrees; in four, equal to 180 degrees; and in two, of greater than 180 degrees. After pars plana vitrectomy and retinal detachment repair, nine (90%) of 10 eyes had retinal reattachment, but seven patients required more than one operation. One eye had persistent retinal detachment, and one eye had corneal graft failure. Seven (70%) of the 10 eyes had best-corrected final visual acuity of 20/200 or better; three eyes had light-perception, hand-motion, and counting-fingers vision. Conclusions Aggressive attempts at intravitreal lens fragment retrieval from a limbal-based approach should be avoided because these procedures may result in serious retinal complications, such as formation of a giant retinal tear.

Purpose To develop a classification system for mechanical injuries of the eye. Methods The Ocular Trauma Classification Group, a committee of 13 ophthalmologists from seven separate institutions, was organized to discuss the standardization of ocular trauma classification. To develop the classification system, the group reviewed trauma classification systems in ophthalmology and general medicine and, in detail, reports on the characteristics and outcomes of eye trauma, then established a classification system based on standard terminology and features of eye injuries at initial examination that have demonstrated prognostic significance. Results This system classifies both open-globe and closed-globe injuries according to four separate variables: type of injury, based on the mechanism of injury; grade of injury, defined by visual acuity in the injured eye at initial examination; pupil, defined as the presence or absence of a relative afferent pupillary defect in the injured eye; and zone of injury, based on the anteroposterior extent of the injury. This system is designed to be used by ophthalmologists and nonophthalmologists who care for patients or conduct research on ocular injuries. An ocular injury is classified during the initial examination or at the time of the primary surgical intervention and does not require extraordinary testing. Conclusions This classification system will categorize ocular injuries at the time of initial examination. It is designed to promote the use of standard terminology and assessment, with applications to clinical management and research studies regarding eye injuries.

Purpose To determine visual acuity outcome after indocyanine green angiography-guided laser photocoagulation of choroidal neovascularization associated with pigment epithelial detachment in eyes with age-related macular degeneration. Methods We retrospectively reviewed pretreatment and posttreatment visual acuity after laser photocoagulation to well-demarcated hyperfluorescent areas seen with indocyanine green angiography adjacent to or within pigment epithelial detachments in 20 eyes of 20 patients with age-related macular degeneration and suspected choroidal neovascularization. Results Visual acuity before and after laser photocoagulation was followed up for 3 to 24 months (median, 9 months). At 3 months after laser photocoagulation, visual acuity had improved 2 or more Snellen lines in two eyes (10%), worsened by 2 or more lines in 10 (50%), and remained unchanged in eight of 20 (40%). By 6 months after laser photocoagulation, visual acuity had improved by 2 or more lines in two eyes (12%), worsened by 2 or more lines in nine (53%), and remained unchanged in six of 17 (35%). At 9 months after laser photocoagulation, visual acuity had improved by 2 or more lines in one eye (9%), worsened by 2 or more lines in nine (82%), and remained unchanged in one of 11 (9%). Conclusions Indocyanine green angiography-guided laser photocoagulation may temporarily stabilize visual acuity in some eyes with choroidal neovascularization associated with pigment epithelial detachments, but final visual acuity decreases with time.

Purpose: The authors determine if specific features of the clinical presentation of acute postoperative endophthalmitis correlate with the microbiologic culture results. Methods: A total of 420 patients who had clinical evidence of endophthalmitis within 6 weeks after cataract surgery or secondary intraocular lens implantation were evaluated as part of a randomized clinical trial. Results of cultures performed on aqueous and vitreous specimens obtained at presentation were categorized as follows: gram-positive coagulase-negative micrococci, "other" gram-positive, gram-negative, and equivocal/no growth. Results: Eleven features of the initial clinical presentation were associated with significant differences in the microbiologic spectrum ( P Conclusions: The presenting characteristics of acute endophthalmitis after cataract surgery may be helpful in predicting the most likely culture results. Such predictions do not appear sufficiently strong to guide the initial empiric choice of intravitreal antibiotics.

Punctate inner choroidopathyl, multifocal choroiditis2, and multifocal choroiditis associated with progressive subretinal fibrosis3 may all appear quite similar clinically. Patients affected with these conditions, which may represent either variants of single disorder or a spectrum of disease, have a similar profile: they are commonly young myopic females with multiple focal areas of inflammation at the level of the choroid/Bruchs’ membrane/retinal pigment epithelium complex. The aetiology and incidence of these conditions is unknown. Visual outcome in punctate inner choroidopathy is generally good, barring the development of subfoveal choroidal neovascularization (CNV)1.

Background: As part of the design of the Silicone Study, a new classification of proliferative vitreoretinopathy (PVR) was developed that distinguishes the different types of contraction found in PVR. In contrast to the original Retina Society system that emphasized the post-equatorial retinal pathology (posterior PVR), the Silicone Study classification system included the characteristic types of contraction found in both the equatorial region and the pre-equatorial retina and vitreous base (anterior PVR). Methods: The authors contrast (1) preoperative and intraoperative findings and (2) vision and anatomic outcomes in the cohort of anterior PVR eyes with the cohort of posterior-only PVR eyes. For the cohort of eyes randomized to perfluoropropane gas (C 3 F 8 ) or silicone oil, the authors carry out univariate and multivariate analyses to assess the predictive value of baseline and intraoperative parameters on vision and anatomic outcome. Results: Anterior PVR was present in 321 eyes (79%)and was more prevalent in eyes that had undergone an unsuccessful vitrectomy before study entry than in eyes that underwent a primary vitrectomy for PVR (88% versus 73%; P P P = 0.003), (3) have more hypotony (24% versus 11 %; P = 0.03), more edema (8% versus 2%; P = 0.04), more aqueous flare ( P = 0.02), more macular pucker (69% versus 52%; P = 0.005), and more intravitreal contraction (21 % versus 6%; P = 0.002). When compared with eyes that had anterior PVR, eyes with posterior PVR had a better outcome at the 6-month postoperative examination: complete attachment of the retina (76% versus 62%; P = 0.04), visual acuity of 5/200 or better (64% versus 45%; P = 0.006), and normal intraocular pressure (86% versus 71 %; P = 0.04). For eyes with anterior PVR, significant predictors of poor ( 3 F 8 gas as the intraocular tamponade. Conclusion: The Silicone Study classification of anterior PVR permits greater specificity in characterizing PVR and is prognostic of anatomic and vision outcome. Eyes with anterior PVR and clinically significant posterior PVR changes had a better visual prognosis if silicone oil was used. With the current understanding of the pathoanx ~ atomy of anterior PVR and the recent development of new surgical techniques, the incidence of anterior PVR in eyes that previously underwent vitrectomy may decline, and the prognosis in eyes with anterior PVR may improve.

Purpose: To investigate the efficacy of ciliary body endophotocoagulation during pars plana vitrectomy in eyes with medically uncontrolled glaucoma and concomitant vitreoretinal disorders. Methods: The authors compared preoperative and postoperative intraocular pressure (IOP) and visual acuity in 21 eyes of 21 patients undergoing ciliary body endophotocoagulation with pars plana vitrectomy. Results: Preoperative IOPs ranged from 22 to 59 mmHg (median, 46 mmHg). Visual acuity ranged from 20/200 to light perception (median, hand motions). Follow-up ranged from 3 to 18 months (median, 10 months). Postoperative IOPs ranged from 11 to 19 mmHg (median, l 6 mmHg) at 6 weeks, 4 to 20 mmHg (median, l 4 mmHg) at 3 months, 1 to 26 mmHg (median, l 2 mmHg) at 6 months, and 0 to 48 mmHg (median, l2 mmHg) at 12 months. Postoperative visual acuity was statistically improved from preoperative visual acuity by 6 weeks. Conclusion: Ciliary body endophotocoagulation combined with pars plana vitrectomy can effectively treat concomitant glaucoma and vitreoretinal disorders.

Silicone oil has been in use as a vitreoretinal surgical adjunct for more than 30 years. The literature from late 1993 through 1994 is dominated by reports from the Silicone Study, which addresses outcome differences after primary versus repeat vitrectomy, combined retinotomy and retinectomy, and silicone oil removal, as well as the problem of postoperative intraocular pressure abnormalities. Contemporary retrospective series reporting on the use of silicone oil for pediatric, cytomegalovirus retinitis-related, and diabetic retinal detachments are also reviewed.

9575Background: Most uveal melanoma (UM) patients do not present with metastatic disease at diagnosis, yet 50% will develop metastases following primary tumor control. A validated 15-gene expressio...

Angiolymphoid hyperplasia with eosinophilia is a rare benign vascular proliferation with lymphocytic and eosinophilic infiltrate. Angiolymphoid hyperplasia with eosinophilia presents as single or multiple dome-shaped papules or plaques involving the dermis and subcutaneous tissues of the head and neck region. Conjunctival angiolymphoid hyperplasia with eosinophilia is rare, with only two previous case reports.

This study evaluates the prognostic performance of a 15 gene expression profiling (GEP) assay that assigns primary posterior uveal melanomas to prognostic subgroups: class 1 (low metastatic risk) and class 2 (high metastatic risk).Prospective, multicenter study.A total of 459 patients with posterior uveal melanoma were enrolled from 12 independent centers.Tumors were classified by GEP as class 1 or class 2. The first 260 samples were also analyzed for chromosome 3 status using a single nucleotide polymorphism assay. Net reclassification improvement analysis was performed to compare the prognostic accuracy of GEP with the 7th edition clinical Tumor-Node-Metastasis (TNM) classification and chromosome 3 status.Patients were managed for their primary tumor and monitored for metastasis.The GEP assay successfully classified 446 of 459 cases (97.2%). The GEP was class 1 in 276 cases (61.9%) and class 2 in 170 cases (38.1%). Median follow-up was 17.4 months (mean, 18.0 months). Metastasis was detected in 3 class 1 cases (1.1%) and 44 class 2 cases (25.9%) (log-rank test, P10(-14)). Although there was an association between GEP class 2 and monosomy 3 (Fisher exact test, P0.0001), 54 of 260 tumors (20.8%) were discordant for GEP and chromosome 3 status, among which GEP demonstrated superior prognostic accuracy (log-rank test, P = 0.0001). By using multivariate Cox modeling, GEP class had a stronger independent association with metastasis than any other prognostic factor (P0.0001). Chromosome 3 status did not contribute additional prognostic information that was independent of GEP (P = 0.2). At 3 years follow-up, the net reclassification improvement of GEP over TNM classification was 0.43 (P = 0.001) and 0.38 (P = 0.004) over chromosome 3 status.The GEP assay had a high technical success rate and was the most accurate prognostic marker among all of the factors analyzed. The GEP provided a highly significant improvement in prognostic accuracy over clinical TNM classification and chromosome 3 status. Chromosome 3 status did not provide prognostic information that was independent of GEP.

Over the past decade, chemoreduction has become the treatment of choice for the initial management of retinoblastoma1. Yet despite its established use, chemoreduction has not been able to treat diffuse infiltrating retinoblastoma, the least common subtype of retinoblastoma1. Herein we report two cases in which residual intraretinal retinoblastoma exhibiting flat and diffuse infiltration was found on pathological exam after a chemoreduction failure. The chemoreduction reduced some of the tumor and unmasked the intraretinal component.

Purpose: The purpose of this study is to assess the benefit of vitreous surgery in preventing full-thickness macular holes in patients with impending (stage 1) macular holes. Methods: A prospective randomized multicenter clinical trial was conducted on patients with full-thickness macular holes in their first eye (stage 3 or 4) and signs and symptoms of stage 1 macular holes in their fellow eye (study eye). The study eye was randomized to vitreous surgery or observation. Outcome was assessed by standardized measurement of visual acuity, detailed clinical examination, fundus photographs, and fluorescein angiography. Results: A full-thickness macular hole developed in 10 (37%) of 27 patients in the vitrectomy group compared with 14 (40%) of 35 patients randomized to observation ( P = 0.81). This difference of 3% has a 95% confidence interval of (-21%,27%). Conclusion: The study was terminated because of low recruitment. The authors were unable to prove (or disprove) the benefit of vitreous surgery in patients with stage 1 macular holes. The authors can state, however, that should a beneficial effect from vitrectomy exist, it would probably be minimal. Considering the cost and morbidity of vitreous surgery, a conservative approach for stage 1 macular hole might be appropriate.

The ultrastructural features of three surgically excised idiopathic subfoveal neovascular membranes were examined. Ultrastructural features of the cellular components in the membranes included retinal pigment epithelium, endothelial-lined vascular channels, photoreceptor cells, macrophages, myofibroblasts, glial cells, erythrocytes, and ghost erythrocytes. Extracellular constituents included 200-nm to 250− nm collagen fibrils, 100-nm collagen fibrils and fibrin. The findings in this study are consistent with previously reported findings of subfoveal neovascular membranes in age-related macular degeneration and the ocular histoplasmosis syndrome, with the exception of the presence of basal laminar deposit in membranes from age- related macular degeneration.

PURPOSE: To evaluate potential toxic effects of indocyanine green dye on cultured human retinal pigment epithelial cells. METHODS: Controlled laboratory experiment. Cultured human retinal pigment epithelial cells were exposed to balanced saline solution, balanced saline solution with endoillumination, indocyanine green or indocyanine green with endoillumination. Cells were evaluated by light microscopy, electron microscopy, and a mitochondrial dehydrogenase assay. RESULTS: Retinal pigment epithelial cells exposed to indocyanine green showed no histologic or ultrastructural changes. Those exposed to indocyanine green alone or indocyanine green plus light demonstrated a significant decrease in mitochondrial enzyme activity ( P = 0.0002 and 0.005, respectively). CONCLUSION: Brief exposure of cultured human retinal pigment epithelial cells to indocyanine green results in decreased mitochondrial enzyme activity but does not appear to influence cellular morphology or ultrastructure.

The immunohistochemical and histochemical properties of 16 surgically excised subretinal neovascular membranes from 16 patients with age-related macular degeneration were studied. Primary antisera to c-retinaldehyde-binding protein; leukocyte common antigen; factor VIII-related antigen; S-100 protein; glial fibrillary acid protein; muscle-specific actin; neuron-specific enolase; collagen types I, II, III, IV, and V; laminin; and fibronectin were used for immunohistochemical characterization of the membranes. Histochemical staining for lipid and mucopolysaccharide was performed. The results of the staining in conjunction with histologic examination showed the cellular components of the membranes to be composed of retinal pigment epithelium, inflammatory cells, vascular endothelium, glial cells, myofibroblasts, photoreceptor cells, and fibrocytes. The extracellular matrix of the membranes contained collagen types I, III, IV, and V; fibronectin; laminin; mucopolysaccharide; and lipid. These findings are consistent with the concept that subretinal neovascular membranes in age-related macular degeneration are composed of localized intra-Bruch's membrane granulation tissue proliferation associated with diffuse drusen.

Some heredomacular diseases share ophthalmoscopic and morphologic similarities despite being nosologically distinct. In part, this may arise from pathologic reaction patterns such as lipopigment accumulation and drusen deposition which are common to a variety of disorders of the photoreceptor--retinal pigment epithelium--Bruch's membrane complex. Two of these disorders, Stargardt's flavimaculatus and the pattern dystrophies of the retinal pigment epithelium may be especially difficult to differentiate clinically since they are characterised by both a similar ophthalmoscopic appearance and considerable inter and intrafamilial variation in their expression. Stargardt's flavimaculatus may, however, be distinguished from the retinal pigment epithelial pattern dystrophies by certain clinical and morphological features that are outlined below.