Resolution of epiretinal membrane after anti-VEGF and photodynamic therapy of retinal hemangioblastoma.

Purpose: To describe the clinical and multimodal imaging features in a case of resolution of pre-macular fibrosis after photodynamic therapy (PDT) of retinal hemangioblastoma (RH) not related to von-Hippel-Lindau (VHL). Observations: A 25-year-old man presenting with blurred vision and central metamorphopsia in his left eye (LE) due to macular epiretinal membrane secondary to a peripheral RH. The patient had a comprehensive ophthalmic examination, including best corrected visual acuity (BCVA), wide-field fundus photography and autofluorescence (FAF), wide-field optical coherence tomography (OCT) and OCT angiography (OCTA), fluorescein angiography (FA) and indocyanine green angiography (ICGA) and ocular ultrasound. Baseline BCVA was 20/200 in his LE, and it improved to 20/40 nineteen months after anti-VEGF and photodynamic therapy were performed. The treatment led to a progressive shrinkage of the tumor and associated intraretinal exudation, and to a progressive resolution of the epiretinal membrane. Conclusions and importance: Treatments performed on the peripheral retina may result in changes at the level of the vitreo-retinal interface in the macular region. In our patient, after an intravitreal anti-VEGF associated with PDT, a posterior vitreous detachment (PVD) was induced resulting in auto-peeling of the macular epiretinal membrane secondary to a peripheral RH. An accurate assessment of the macular area by OCT is highly recommended in the first days following treatments.

Optical Coherence Tomography in Inflammatory and Neoplastic Lesions Deforming the Choroidal Profile.

The choroid is the main part of the uvea, the vascular layer of the eye that lies between the retina and the sclera. The high vascular component of the choroid makes this structure susceptible to inflammation in multisystemic diseases, as well as the most common site of metastasis in the eye. Therefore, the choroid is involved in many pathological conditions, from uveitis to intraocular tumors. Differentiating between inflammatory and neoplastic lesions deforming the choroidal profile can sometimes be challenging. In addition, scleral disorders can also deform the choroidal profile. Choroidal imaging includes ophthalmic ultrasonography, indocyanine green angiography, and optical coherence tomography (OCT). Recent advances in choroidal imaging techniques, such as enhanced depth imaging optical coherence tomography (EDI-OCT) and swept-source optical coherence tomography (SS-OCT), have facilitated an in-depth analysis of the choroid. The purpose of this review article is to report on and highlight the most common OCT findings to help in the differential diagnosis between inflammatory and neoplastic lesions deforming the choroidal profile.

Choroidal Lymphoma: Diagnostic Value of Combined Indocyanine Green Angiography and Optical Coherence Tomography.

OBJECTIVE: To compare multimodal imaging findings in patients with choroidal lymphoma (CL). METHODS: Multicenter retrospective observational case series. Multimodal imaging features of patients with CL were reviewed with particular attention to the patterns of choroidal infiltration on indocyanine green angiography (ICGA) and optical coherence tomography (OCT). RESULTS: Eighteen eyes of 15 patients were included in this study. Average tumor thickness on ultrasonography was 2.6 mm (range, 1.2-5.7 mm). Choroidal infiltration on ICGA was characterized by multifocal, round areas (300-500 microns diameter) of hypocyanescence in all cases, whereas OCT at the same region disclosed diffuse choroidal infiltration. By OCT, the tumor surface contour was primarily placid (22%), dome-shaped (11%), or undulating (67%). CONCLUSIONS: In this analysis of eyes with CL, ICGA demonstrated multifocal sub-millimeter regions of choroidal hypocyanescence whereas OCT documented diffuse choroidal infiltration. This incongruence could be a distinctive diagnostic feature of choroidal lymphoma, assisting with differentiation from other pathological entities.

Clinical Applications of Optical Coherence Tomography Angiography in Ocular Oncology: Pearls and Pitfalls.

Background: Optical coherence tomography angiography (OCTA) is a valuable imaging tool for the diagnosis of several retinal and choroidal diseases. Its role in ocular oncology is clinically promising but still controversial. In this review, we report the main applications and limits of the use of OCTA for the study of intraocular tumors. Summary: OCTA allows a rapid, safe, low-cost, and high-resolution visualization of the retinal and choroidal vasculature. Attempts have been made to use this technology in ocular oncology to differentiate benign and malignant lesions and to assist physicians in the evaluation and monitoring of post-treatment complications. Main limitations include failure in correct segmentation due to the tumor inner profile or thickness, poor penetration of the laser into the lesion, masking effect from overlying fluid, media opacities and poor fixation. Key Messages: The main applications of OCTA in ocular oncology consist of the documentation of tumor-associated choroidal neovascularizations and the study of vascular changes following tumor treatments. In particular, the diffusion of wide-field protocols makes OCTA suitable for the diagnosis and follow-up of radiation chorio-retinopathy, allowing a detailed visualization of both macular and peripheral ischemic changes. Optimistically, future innovations in OCTA technology may offer new perspectives in the diagnosis and follow-up of intraocular tumors.

DOUBLE FLUENCE PHOTODYNAMIC THERAPY FOR THE TREATMENT OF CIRCUMSCRIBED CHOROIDAL HEMANGIOMA.

PURPOSE: To evaluate the efficacy and safety of double-fluence photodynamic therapy for the treatment of circumscribed choroidal hemangioma. METHODS: Retrospective observational study including patients affected by circumscribed choroidal hemangioma and treated with double-fluence photodynamic therapy. The photodynamic therapy was performed with verteporfin infusion intravenously (dose of 6 mg/m2 body surface area over 10 minutes), followed by the application of two consecutive spots of 50 J/cm2 light at 689 nm for 83 seconds. RESULTS: Twenty-three eyes of 23 patients were included. The mean best-corrected visual acuity increased from 20/45 to 20/28, the mean tumor thickness decreased from 2,758 ± 530 µm to 722 ± 314 µm (P < 0.05), and the mean central retinal thickness decreased from 404 ± 209 µm to 188 ± 56 µm (P < 0.05) in 12 months, respectively. A total reabsorption of macular subretinal fluid, cystoid macular edema, and SRF associated with the tumor was obtained within 6 months in all cases, with persistence of tumor-associated intraretinal fluid up to 12 months only in two patients. No cases of side effects or need for retreatment were reported during the follow-up (average time of 25 months). CONCLUSION: Double-fluence photodynamic therapy is a safe and effective treatment for circumscribed choroidal hemangiomas and should be considered as the first line of treatment for these lesions.

CAVITARY CHOROIDAL NEVUS: A CASE REPORT.

PURPOSE: To describe a case of choroidal nevus with intralesional cavitations detected with enhanced depth imaging optical coherence tomography and to show its changes during an 8-year follow-up. METHODS: A case report. RESULTS: A 55-year-old male patient was found to have a pigmented lesion at the posterior pole of his right eye, just temporal to the fovea. A complete multiimaging examination was obtained and showed a lesion of about 4 mm of diameter with a small amount of subretinal fluid and chronic changes in the retina overlying the lesion. Based on clinical examination, a diagnosis of choroidal nevus was made, but interestingly the enhanced depth imaging optical coherence tomography revealed the presence of intralesional cavitations. Close follow-up with no intervention was planned, and over 8 years of observation, the lesion remained stable in size, with a gradual increase of the central necrosis. CONCLUSION: Benign pigmented choroidal lesions can show internal cavitations likely secondary to internal necrosis. This feature does not represent a sign of malignancy of the lesion. Enhanced depth imaging optical coherence tomography is a feasible imaging tool for the visualization of the internal cavitations and to follow their evolution over time.

EXTENDED FIELD IMAGING OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY FOR THE STUDY OF RETINAL AND CHOROIDAL CHANGES AFTER RADIATION THERAPY FOR CHOROIDAL MELANOMA: Comparison With Wide-Field Angiography.

PURPOSE: Radiation retinopathy is a common side effect of ocular radiotherapy with no long-term effective therapy. Optical coherence tomography angiography (OCTA) and wide-field fluorescein angiography (FA) are widely used for the study of radiation maculopathy and peripheral nonperfusion, respectively. We investigated the role of extended field imaging (EFI-OCTA) for the study of retinal and choroidal alterations after radiotherapy for choroidal melanoma. METHODS: Cross-sectional observational study of 20 eyes of 20 patients diagnosed with radiation retinopathy. All patients underwent a complete imaging evaluation including FA and indocyanine green angiography (ICGA) with 55° and 102° lens (Spectralis Heidelberg Engineering, Heidelberg, Germany). Optical coherence tomography angiography imaging was performed with the Zeiss PlexElite 9000 Swept Source OCTA (Carl Zeiss Meditec, Dublin, CA) using a 12 × 12-mm volume scan pattern centered on the fovea and a +20.00-diopter lens specifically designed to obtain EFI examination. The imaging methods were then compared in terms of visible field of view, extension of nonperfused areas, and vessel density. RESULTS: The mean extension ratio of EFI-OCTA compared to OCTA without EFI, FA/ICGA 55° and FA/ICGA 102° was, respectively, 1.98 ± 0.02, 1.21 ± 0.01 and 0.36 ± 0.003. The mean extension of retinal and choroidal nonperfused areas evaluated by EFI-OCTA (63.03 ± 48.21 and 38.63 ± 30.83 mm2) were significantly higher than with OCTA without EFI (40.40 ± 34.87 and 24.26 ± 21.82 mm2, P < 0.001) but lower than with FA/ICGA 102° (140.7 ± 69.23 and 108.3 ± 69.51 mm2, P < 0.001). No significant differences were found between mean extension of retinal and choroidal ischemic areas measured with EFI-OCTA and FA/ICGA 55° (69.64 ± 51.92 and 47.23 ± 33.59 mm2). The mean vessel density of EFI-OCTA (retina and choroid segmentation) was significantly different compared to OCTA without EFI (P < 0.05). Retinal vessel density was negatively correlated to retinal extension of nonperfused areas (r = -0.5, P = 0.02), and choroidal vessel density was negatively correlated to choroidal nonperfused areas (r = -0.6, P = 0.003) measured with EFI-OCTA. CONCLUSION: In our series, EFI-OCTA captured larger areas than OCTA without EFI and FA/ICGA with 55° lens. EFI-OCTA images showed a good definition of retinal and choroidal vascular changes after radiotherapy, suggesting a possible role of this safe and noninvasive imaging technique in the follow-up of patients with radiation retinopathy.

OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY FINDINGS IN A CASE OF CHOROIDAL NEOVASCULARIZATION SECONDARY TO UNILATERAL RETINAL PIGMENT EPITHELIUM DYSGENESIS TREATED WITH INTRAVITREAL BEVACIZUMAB THERAPY.

PURPOSE: To document by optical coherence tomography angiography the presence of a choroidal neovascularization (CNV) secondary to unilateral retinal pigment epithelium dysgenesis and describe its changes after treatment with intravitreal bevacizumab. METHODS: Case report. RESULTS: A 51-year-old woman referred to our ocular oncology service for a possible choroidal osteoma in her left eye was found to have a unilateral retinal pigment epithelium dysgenesis complicated by CNV. Best-corrected visual acuity at baseline was 20/200. A complete ophthalmological evaluation including color fundus photography, fundus autofluorescence, fluorescein angiography, indocyanine green angiography, optical coherence tomography, and optical coherence tomography angiography was performed. The CNV appeared as a tangled vascular network on optical coherence tomography angiography. After 2 intravitreal injections of bevacizumab, the best-corrected visual acuity improved to 20/50, and optical coherence tomography angiography showed a progressive contraction of the CNV. Unilateral retinal pigment epithelium dysgenesis margins remained stable at consecutive follow-up. CONCLUSION: Optical coherence tomography angiography is a useful imaging technique for the diagnosis and follow-up of CNVs secondary to unilateral retinal pigment epithelium dysgenesis. Anti-vascular endothelial growth factor agents represent an effective therapy for the treatment of CNV in patients with this disease.

OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY FINDINGS IN A CASE OF CONGENITAL RETINAL MACROVESSEL WITH ANOMALOUS RETINAL ANASTOMOSIS ASSOCIATED WITH CONTRALATERAL MYELINATED NERVE FIBERS AND RETINAL VASCULAR ABNORMALITIES.

PURPOSE: To describe a case of congenital retinal macrovessel complicated by cystoid macular edema associated with contralateral myelinated retinal nerve fibers and retinal vascular abnormalities studied with optical coherence tomography angiography (OCTA). METHODS: Case report. RESULTS: A healthy 25-year-old woman with decreased vision in her right eye was found to have a congenital retinal venous macrovessel in the macula associated with cystoid edema. In the contralateral amblyopic eye, the examination revealed a tuft of myelinated retinal nerve fibers along the superotemporal vascular arcade associated with superficial vascular abnormalities. A complete multi-imaging examination was obtained, including fundus color photography, fluorescein angiography, indocyanine green angiography, optical coherence tomography (OCT), and optical coherence tomography angiography. At 1-week follow-up, the optical coherence tomography displayed spontaneous resolution of the edema that remained stable at consecutive 1-month follow-up. CONCLUSION: Congenital retinal macrovessels can be associated with other ocular developmental anomalies. Vascular complications can occur, leading to macular edema and retinal ischemia. Optical coherence tomography angiography can be useful for the diagnosis and follow-up of this condition.

Peripheral exudative haemorrhagic chorioretinopathy: a widefield imaging study.

AIMS: To study widefield imaging features, analyse risk factors for progression and compare treatment modalities of peripheral exudative haemorrhagic chorioretinopathy (PEHCR). METHODS: Clinical and imaging data from each visit were collected, including near-infrared reflectance, fluorescein angiography, indocyanine green angiography, optical coherence tomography using the Spectralis platform (Heidelberg Engineering). Clinical features and treatment performed were recorded. Lesions were classified according to treatments used and according to the prognostic index of macular involvement (MI) and intravitreal bleeding (IVB). RESULTS: 50 eyes of 35 patients were retrospectively enrolled. Using widefield imaging, peripheral subretinal fluid (SRF) was detected in 31 (62%) eyes and peripheral neovascular networks were detected in 42 (84%) eyes and graded as focal, diffuse and subtotal in 22 (44%), 17 (34%) and 11 (22%) eyes, respectively. MI secondary to PEHCR during the follow-up was documented in 17 (34%) eyes, while IVB occurred in 7 (14%) eyes. Both MI and IVB correlated with peripheral SRF and lesion grade. Active treatment included anti-vascular endothelial growth factor (anti-VEGF) injections, photodynamic therapy (PDT) and combined anti-VEGF and PDT for 13 (26%), 1 (2%) and 18 (36%) eyes, respectively. In eyes with risk factors, anti-VEGF and combined treatment inversely correlated with MI incidence. CONCLUSIONS: In our series, peripheral subretinal exudation and the extension of peripheral involvement represented risk factors for MI in eyes with PEHCR. In those high-risk eyes, active treatment is warranted.

OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY FINDINGS IN A CASE OF CHOROIDAL NEOVASCULARIZATION SECONDARY TO TRAUMATIC CHOROIDAL RUPTURE.

BACKGROUND/PURPOSE: To document by optical coherence tomography angiography, the onset of a choroidal neovascularization (CNV) secondary to traumatic choroidal rupture and describe its changes after an intravitreal injection of bevacizumab. METHODS: Case report. RESULTS: A 20-year-old woman presented referring vision loss after a blunt ocular trauma in her left eye. The patient underwent a complete ophthalmic examination. Best-corrected visual acuity was 20/200. Fundus examination, fluorescein angiography, indocyanine green angiography, and optical coherence tomography displayed a choroidal rupture with no evidence of CNV. Optical coherence tomography angiography showed the choroidal rupture as a line of choriocapillaris rarefaction because of the mechanical damage. Six months later, best-corrected visual acuity decreased to 20/300; optical coherence tomography angiography displayed the growth of a CNV, characterized by a tangled vascular network. After one intravitreal injection of bevacizumab, optical coherence tomography angiography documented a contraction of the CNV. CONCLUSION: Optical coherence tomography angiography is a useful imaging technique for the diagnosis and follow-up of patients with choroidal ruptures. Anti-vascular endothelial growth factor agents represent an effective therapy for the treatment of CNVs secondary to this affection.

Diagnosis and Detection.

The aim of the chapter is to provide a practical but exhaustive guide in detecting macular edema and to describe its features according to the retinal condition that causes it. The most useful imaging techniques (biomicroscopy, retinography, optical coherence tomography, and fluorescein/indocyanine-green angiography will be analyzed in order to identify the best diagnostic algorithm in each pathology. There is a table at the end of the chapter which summarizes the important points of the chapter.

Update of intravitreal steroids for the treatment of diabetic macular edema.

Diabetic macular edema is considered the most important factor related to visual impairment in patients with diabetic retinopathy. Together with the use of grid and focal laser photocoagulation, today the intravitreal administration of pharmacotherapies represents the standard of care for the treatment of this complication: anti-vascular endothelium growth factor agents and steroids are the drugs currently used for this aim. Differently from laser therapy, which prevents visual deterioration, the intravitreal approach allows the promotion of visual recovery. However, the intravitreal injections require to be repeated with high frequency, and this carries the risk of drug- and procedure-related adverse effects.

Changes in macular function after ozurdex for retinal vein occlusion.

PURPOSE: To investigate changes in macular function after intravitreal dexamethasone implant (Ozurdex) for macular edema (ME) secondary to retinal vein occlusion (RVO). METHODS: Nineteen treatment-naive patients with RVO-related ME were treated with intravitreal Ozurdex and followed up to 6 months to evaluate functional outcomes, by means of best-corrected visual acuity, microperimetry, and multifocal electroretinography, and their correlations with morphological parameters by enhanced depth imaging optical coherence tomography. RESULTS: Nineteen eyes of 19 patients were included for analysis. At 1 month, mean best-corrected visual acuity, retinal sensitivity, and central macular thickness (CMT) improved from 0.50 ± 0.34 LogMAR, 10.51 ± 4.31 dB, and 762 ± 259 µm (baseline) to 0.38 ± 0.34 LogMAR (p = 0.043), 12.28 ± 5.06 dB (p = 0.025), and 385 ± 191 µm (p = 0.001), respectively. At 3 months, improvement of mean retinal sensitivity and CMT was still significant (11.62 ± 5.05 dB [p = 0.047] and 518 ± 251 µm [p = 0.006]). Multifocal electroretinography measurements also showed (nonsignificant) improvement. No significant changes in choroidal thickness were recorded. Improvements recorded during the first 3 months were no longer significant from month 4. At each time point, we found a negative significant correlation between CMT and retinal sensitivity. Interestingly, 7 eyes did not undergo retreatment of less than 6 months; these eyes showed a significantly better baseline retinal sensitivity than eyes requiring retreatment of less than 6 months (12.27 ± 3.52 dB vs. 9.48 ± 4.53 dB [p = 0.038]). CONCLUSIONS: In eyes with ME secondary to RVO, intravitreal dexamethasone implant provides functional benefits as soon as 1 month after treatment. In most cases, the optimum retreatment interval is less than 6 months from first intravitreal Ozurdex. Microperimetry is a very useful tool to characterize macular function. Baseline macular sensitivity may predict the need for early (<6 months) retreatment.

Central retinal vein occlusion in a young patient following cannabis smoke inhalation.

PURPOSE: To report a case of central retinal vein occlusion (CRVO) following cannabis smoke inhalation in a young patient. METHODS: An otherwise healthy 18-year-old man without risk factors for retinal vein occlusion presented with reduced visual acuity (20/200) secondary to CRVO following cannabis smoke inhalation. Central retinal vein occlusion was diagnosed on the basis of slit-lamp fundus biomicroscopy and fluorescein angiography. RESULTS: Tests for systemic causes were negative. Following one intravitreal dexamethasone implant (Ozurdex) and one ranibizumab injection (Lucentis), functional (20/20 visual acuity) and anatomic improvement was recorded. Fluorescein angiography showed a decrease in the vascular caliber and tortuosity, with no signs of retinal ischemia or edema. CONCLUSIONS: We report CRVO in a young adult following cannabis smoke inhalation. Its occurrence immediately after cannabis smoking further supports a link between the use of cannabis and vascular alterations.