Optical Coherence Tomography Angiography Features of Iris Racemose Hemangioma in 4 Cases.

Importance: Optical coherence tomography angiography (OCTA) allows visualization of iris racemose hemangioma course and its relation to the normal iris microvasculature. Objective: To describe OCTA features of iris racemose hemangioma. Design, Setting, and Participants: Descriptive, noncomparative case series at a tertiary referral center (Ocular Oncology Service of Wills Eye Hospital). Patients diagnosed with unilateral iris racemose hemangioma were included in the study. Main Outcomes and Measures: Features of iris racemose hemangioma on OCTA. Results: Four eyes of 4 patients with unilateral iris racemose hemangioma were included in the study. Mean patient age was 50 years, all patients were white, and Snellen visual acuity was 20/20 in each case. All eyes had sectoral iris racemose hemangioma without associated iris or ciliary body solid tumor on clinical examination and ultrasound biomicroscopy. By anterior segment OCT, the racemose hemangioma was partially visualized in all cases. By OCTA, the hemangioma was clearly visualized as a uniform large-caliber vascular tortuous loop with intense flow characteristics superimposed over small-caliber radial iris vessels against a background of low-signal iris stroma. The vascular course on OCTA resembled a light bulb filament (filament sign), arising from the peripheral iris (base of light bulb) and forming a tortuous loop on reaching its peak (midfilament) near the pupil (n = 3) or midzonal iris (n = 1), before returning to the peripheral iris (base of light bulb). Intravenous fluorescein angiography performed in 1 eye depicted the iris hemangioma; however, small-caliber radial iris vessels were more distinct on OCTA than intravenous fluorescein angiography. Conclusions and Relevance: Optical coherence tomography angiography is a noninvasive vascular imaging modality that clearly depicts the looping course of iris racemose hemangioma. Optical coherence tomography angiography depicted fine details of radial iris vessels, not distinct on intravenous fluorescein angiography

Optical Coherence Tomography of Retinal and Choroidal Tumors

Optical coherence tomography (OCT) has revolutionized the field of ophthalmology since its introduction 20 years ago. Originally intended primarily for retina specialists to image the macula, it has found its role in other subspecialties that include glaucoma, cornea, and ocular oncology. In ocular oncology, OCT provides axial resolution to approximately 7 microns with cross-sectional images of the retina, delivering valuable information on the effects of intraocular tumors on the retinal architecture. Some effects include retinal edema, subretinal fluid, retinal atrophy, photoreceptor loss, outer retinal thinning, and retinal pigment epithelial detachment. With more advanced technology, OCT now provides imaging deeper into the choroid using a technique called enhanced depth imaging. This allows characterization of the thickness and reflective quality of small (<3 mm thick) choroidal lesions including choroidal nevus and melanoma. Future improvements in image resolution and depth will allow better understanding of the mechanisms of visual loss, tumor growth, and tumor management

Comparative Analysis of Circulating Endothelial Progenitor Cells in Age-Related Macular Degeneration Patients Using Automated Rare Cell Analysis (ARCA) and Fluorescence Activated Cell Sorting (FACS)

BackgroundPatients with age-related macular degeneration (ARMD) begin with non-neovascular (NNV) phenotypes usually associated with good vision. Approximately 20% of NNV-ARMD patients will convert to vision debilitating neovascular (NV) ARMD, but precise timing of this event is unknown. Developing a clinical test predicting impending conversion to NV-ARMD is necessary to prevent vision loss. Endothelial progenitor cells (EPCs), defined as CD34+VEGR2+ using traditional fluorescence activated cell sorting (FACS), are rare cell populations known to be elevated in patients with NV-ARMD compared to NNV-ARMD. FACS has high inter-observer variability and subjectivity when measuring rare cell populations precluding development into a diagnostic test. We hypothesized that automated rare cell analysis (ARCA), a validated and FDA-approved technology for reproducible rare cell identification, can enumerate EPCs in ARMD patients more reliably. This pilot study serves as the first step in developing methods for reproducibly predicting ARMD phenotype conversion.MethodsWe obtained peripheral venous blood samples in 23 subjects with NNV-ARMD or treatment naïve NV-ARMD. Strict criteria were used to exclude subjects with known angiogenic diseases to minimize confounding results. Blood samples were analyzed in masked fashion in two separate laboratories. EPCs were independently enumerated using ARCA and FACS within 24 hours of blood sample collection, and p<0.2 was considered indicative of a trend for this proof of concept study, while statistical significance was established at 0.05.ResultsWe measured levels of CD34+VEGFR2+ EPCs suggestive of a trend with higher values in patients with NV compared to NNV-ARMD (p = 0.17) using ARCA. Interestingly, CD34+VEGR2+ EPC analysis using FACS did not produce similar results (p = 0.94).ConclusionsCD34+VEGR2+ may have predictive value for EPC enumeration in future ARCA studies. EPC measurements in a small sample size were suggestive of a trend in ARMD using ARCA but not FACS. ARCA could be a helpful tool for developing a predictive test for ARMD phenotype conversion

Dome-shaped macula simulating choroidal hemangioma in a myopic patient

To describe a case of dome-shaped macula simulating a choroidal hemangioma and discuss multimodal imaging features to distinguish between these disorders. A 52-year-old myopic male with a refraction of –8.00 D in both eyes developed blurred vision in the left eye OS) over 4 years. Fundus examination of the right eye (OD) was unremarkable. In OS, there was a subtle orange-colored mass in the macular region with subretinal fluid, suggestive of choroidal hemangioma. Upon referral, our examination disclosed an echodense mass on ultrasonography of OS, measuring 1.0 mm thickness, and additional staphyloma was noted. Enhanced depth imaging optical coherence tomography (EDI-OCT) confirmed subfoveal fluid over a dome-shaped mass that originated in the sclera and not the choroid, consistent with the dome-shaped macula. The OD showed similar features, but to a lesser degree. Both eyes demonstrated choroidal thinning on EDI-OCT, related to high myopia. Dome-shaped macula can masquerade as choroidal hemangioma, especially when associated with subretinal fluid. In these cases, EDI-OCT can document "tumor" origin from within the sclera and not the choroid

Stones, bones, groans, thrones, and psychiatric overtones: Systemic associations of sclerochoroidal calcification.

Sclerochoroidal calcification (SCC) is a frequent masquerader of choroidal melanoma with important systemic associations such as hyperparathyroidism and parathyroid adenoma. Herein, we describe a case of a 67-year-old male who presented with an amelanotic choroidal lesion in the right eye (OD) and a history of kidney stones. Ultrasonography showed the lesion to be flat and calcified OD. Incidentally, a subclinical calcified plaque was also found in the fellow eye. Optical coherence tomography showed an elevated suprachoroidal mass in a table mountain configuration OD and flat configuration left eye, consistent with type 4 and type 1 SCC. The patient was referred for metabolic testing to rule out the underlying electrolyte imbalance and was found to be normal

doi:10.1155/2011/385058 Review Article Optical Coherence Tomography of Retinal and Choroidal Tumors

License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Optical coherence tomography (OCT) has revolutionized the field of ophthalmology since its introduction 20 years ago. Originally intended primarily for retina specialists to image the macula, it has found its role in other subspecialties that include glaucoma, cornea, and ocular oncology. In ocular oncology, OCT provides axial resolution to approximately 7 microns with cross-sectional images of the retina, delivering valuable information on the effects of intraocular tumors on the retinal architecture. Some effects include retinal edema, subretinal fluid, retinal atrophy, photoreceptor loss, outer retinal thinning, and retinal pigment epithelial detachment. With more advanced technology, OCT now provides imaging deeper into the choroid using a technique called enhanced depth imaging. This allows characterization of the thickness and reflective quality of small (&lt;3 mm thick) choroidal lesions including choroidal nevus and melanoma. Future improvements in image resolution and depth will allow better understanding of the mechanisms of visual loss, tumor growth, and tumor management. 1

Role of pars plana vitrectomy and membrane peel in vitreomacular traction associated with retinal vasoproliferative tumors.

To report a case of retinal vasoproliferative tumor (VPT) with secondary epiretinal membrane (ERM) formation and vitreo-macular traction managed by pars plana vitrectomy (PPV) and membrane peel. A 29-year-old male was referred for management of decreased vision in the right eye (OD) for 1 week. Presenting visual acuity was 20/50 Snellen feet (ft) OD, and fundus examination showed an ERM associated with a reddish-yellow mass in the inferotemporal quadrant with overlying exudation, hemorrhage, and subretinal fluid consistent with VPT, and cryotherapy was recommended. Two months later, there was complete tumor regression, but there was decreased vision from progressive vitreomacular traction to 20/400 ft. PPV with combined ERM and internal limiting membrane (ILM) peel were performed with resolution of vitreomacular traction and improvement of visual acuity to 20/50 ft at 6 months. PPV with combined ERM and ILM peel is effective for vision loss secondary to ERM and vitreomacular traction associated with retinal VPT

Progressive Growth of Bilateral Choroidal Osteomas in a Child

A 7-year-old girl was referred for retinal dystrophy. On examination, visual acuity was 20/20 in the right eye and 20/30 in the left eye. Fundus examination revealed bilateral orange-colored subfoveal choroidal tumors. Ultrasonography showed calcification, confirming the diagnosis of choroidal osteoma. Because vision was preserved without subretinal fluid or choroidal neovascularization, observation with calcium supplementation was recommended. Three years later, there was minimal tumor growth and visual acuity was maintained. Choroidal osteomas are rare, benign tumors that may decrease vision through decalcification, particularly when subfoveal. In these cases, cautious observation and possibly calcium supplementation may help protect vision

Comparative analysis of circulating endothelial progenitor cells in age-related macular degeneration patients using automated rare cell analysis (ARCA) and fluorescence activated cell sorting (FACS).

BACKGROUND: Patients with age-related macular degeneration (ARMD) begin with non-neovascular (NNV) phenotypes usually associated with good vision. Approximately 20% of NNV-ARMD patients will convert to vision debilitating neovascular (NV) ARMD, but precise timing of this event is unknown. Developing a clinical test predicting impending conversion to NV-ARMD is necessary to prevent vision loss. Endothelial progenitor cells (EPCs), defined as CD34(+)VEGR2(+) using traditional fluorescence activated cell sorting (FACS), are rare cell populations known to be elevated in patients with NV-ARMD compared to NNV-ARMD. FACS has high inter-observer variability and subjectivity when measuring rare cell populations precluding development into a diagnostic test. We hypothesized that automated rare cell analysis (ARCA), a validated and FDA-approved technology for reproducible rare cell identification, can enumerate EPCs in ARMD patients more reliably. This pilot study serves as the first step in developing methods for reproducibly predicting ARMD phenotype conversion. METHODS: We obtained peripheral venous blood samples in 23 subjects with NNV-ARMD or treatment naïve NV-ARMD. Strict criteria were used to exclude subjects with known angiogenic diseases to minimize confounding results. Blood samples were analyzed in masked fashion in two separate laboratories. EPCs were independently enumerated using ARCA and FACS within 24 hours of blood sample collection, and p<0.2 was considered indicative of a trend for this proof of concept study, while statistical significance was established at 0.05. RESULTS: We measured levels of CD34(+)VEGFR2(+) EPCs suggestive of a trend with higher values in patients with NV compared to NNV-ARMD (p = 0.17) using ARCA. Interestingly, CD34(+)VEGR2(+) EPC analysis using FACS did not produce similar results (p = 0.94). CONCLUSIONS: CD34(+)VEGR2(+) may have predictive value for EPC enumeration in future ARCA studies. EPC measurements in a small sample size were suggestive of a trend in ARMD using ARCA but not FACS. ARCA could be a helpful tool for developing a predictive test for ARMD phenotype conversion

Management of retinal vasoproliferative tumor associated with ROP by plaque brachytherapy.

The authors report a secondary exudative vasoproliferative tumor of the retina associated with retinopathy of prematurity, managed by plaque radiotherapy. A 15-year-old girl with a history of mild, stable retinopathy of prematurity in both eyes noted progressively reduced visual acuity to hand motions in the left eye. Examination disclosed a vasoproliferative tumor of 10-mm base and 3.5-mm thickness with near total (macula-off) exudative retinal detachment. She was treated with iodine-125 plaque radiotherapy, giving apex dose of 40 Gy over 4 days. The tumor regressed to 1.9 mm and retinal detachment resolved 8 months following therapy. Epiretinal membrane with significant macular pucker and edema later developed and was managed with pars plana vitrectomy and membrane peeling. Visual acuity was counting fingers due to photoreceptor loss. Retinopathy of prematurity is a rare cause of vasoproliferative tumor. Iodine-125 plaque radiotherapy can lead to tumor regression and reattachment of the retina in such cases