CHOROIDAL NEOVASCULARIZATION IN TORPEDO MACULOPATHY TREATED BY AFLIBERCEPT: LONG-TERM FOLLOW-UP USING OPTICAL COHERENCE TOMOGRAPHY AND OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY.

PURPOSE: To report a case of torpedo maculopathy (TM) in an elderly patient complicated by choroidal neovascularization (CNV) and that was treated by intravitreal aflibercept injections and monitored during 30 months using optical coherence tomography (OCT) and OCT angiography. METHODS: A 74-year-old woman complained of a metamorphopsia in the left eye. Visual acuity was 20/25 while fundus examination showed a hypopigmented torpedo-shaped chorioretinal lesion in the temporal macula associated with a retinal exudation. RESULTS: Multimodal imaging showed a severe chorioretinal excavation confirming the diagnosis of TM associated with an exudative Type 1 CNV. Intravitreal aflibercept injections allowed an initial good anatomical and functional improvement, but multiple recurrences occurred. At the last visit, 30 months after the start of 14 intravitreal aflibercept injections, a persistent subretinal fluid was present with a decreased visual acuity to 20/32 while the CNV surface grew in OCT angiography. CONCLUSION: This first case of long-term follow-up of CNV secondary to TM showed an anti-VEGF dependency or even a resistance with extension of CNV. Thus, a deep retinochoroidal atrophy could be the late evolution of TM that may result on severe CNV in advanced stages. A regular TM monitoring including OCT and OCT angiography would be necessary to detect any potential CNV for an early and aggressive treatment.

Large-cube 30° × 25° optical coherence tomography in diabetic macular edema.

BACKGROUND: To evaluate the contribution of large-cube 30° × 25° optical coherence tomography (OCT) in the characterization of diabetic macular edema (DME) by assessing its extent and the presence of additional retinal edemas and to evaluate the factors that influenced their occurrence. METHODS: This retrospective study enrolled patients with diabetes who presented with retinal edema detected by horizontal large-cube 30° × 25° (8.7 × 7.3 mm) OCT. Two individualized areas were selected from the thickness map: the area within the 6-mm Early Treatment of Diabetic Retinopathy Study (ETDRS) grid, and that outside the ETDRS grid. Retinal edemas located within the ETDRS grid were designated as "main DME" and those located outside the ETDRS grid were designated as "peripheral retinal edemas." For each area, OCT features were assessed while the extent of the main DME and the presence of peripheral retinal oedema were analysed in the area outside the ETDRS grid. Finally, part of included eyes was followed by the same protocol, of which a part benefited from intravitreal injections. RESULTS: Peripheral events were detected outside the ETDRS area in 279 eyes (74.4%) of the 375 eyes of the 218 patients included in this study: an extension of the main DME outside ETDRS grid in 177 eyes (47.2%) and/or the presence of peripheral retinal edemas in 207 eyes (55.2%). The analysis of associations between main DME and peripheral retinal edemas patterns did not find an association for retinal cyst localization (P = 0.42) while a week association was found fort cyst size (Cramer's V = 0.188, p = 0.028). Nevertheless, a moderate association was found for the presence of microaneurysms (Cramer's V = 0.247, p < 0.001) and strong association for hard exudates (Cramer's V = 0.386, p < 0.001), The binary logistic regression analysis retained the following influencing factors of the occurrence of peripheral events: advanced DR stage (Odds ratio OR = 2.19, p = 0.03), diffuse DME (OR = 7.76, p < 0.001) and its location in outer fields (OR = 7.09, p = 0.006). Likewise, the extension of the main DME outside the ETDRS area in was influenced by the same factors in addition to CMT (OR = 0.98, p = 0.004) while the presence of peripheral retinal edema was influenced by the same factors except the outer location of the Main DME. Finally, from the 94 eyes treated by intravitreal injections, extension of the main DME outside the ETDRS grid was detected in 54 eyes (56.44%) at baseline visit and still remained detectable in 37 eyes (39.36%) after treatment initiation. CONCLUSIONS: Large-cube 30° × 25° OCT allowed for more precise assessment of DME extension and better detection of retinal thickening mainly in the advanced stages of diabetic retinopathy with significant DME whether at the baseline visit or during follow-up. The combination of this protocol with a wider ETDRS grid would enhance DME detection and topography.

Spinal cord infarction associated to retinal vein occlusion in a patient with chronic kidney disease.

A 70-year-old man presented to the emergency department with blood hypotension associated to a sudden paraplegia and thermalgesic analgesia. He had an history of colic and prostatic adenocarcinoma, hypertension and non-dialyzed Chronic Kidney Disease (CKD) related to an idiopathic membranous glomerulonephritis type 1 discovered 9 years ago. Magnetic resonance imaging confirmed a diagnosis of Spinal Cord Infarction (SCI). Few months later, he presented a blurred vision due to central Retinal Vein Occlusion (RVO), which was improved by Anti-VEGF therapy. This is the first reported case of a concomitance of retinal vascular event and SCI highlights the links between the central nervous system and retinal vascularization despite separate involvement of the two events in the arterial and venous systems. Additionally, CKD worsened the risk of cardiovascular incidents by induced oxidative stress, thrombophilia, chronic inflammation, and endothelial dysfunction. SCI occurrence indicates severe vascular dysfunction and elevates the risk of additional vascular disorders.

Features of cotton wool spots in diabetic retinopathy: a spectral-domain optical coherence tomography angiography study.

PURPOSE: To describe the features of cotton wool spots (CWSs) in diabetic retinopathy (DR) by using spectral-domain optical coherence tomography (SD-OCT) and optical coherence tomography angiography (OCT angiography). METHODS: We retrospectively studied patients with DR who presented CWSs and had been imaged by SD-OCT angiography. The retinal layer localisation and dimensions of the CWSs were assessed on SD-OCT, while the decorrelation signal generated by the CWSs and the vascular density (VD) of the superficial capillary plexus (SCP) and deep capillary plexus around the CWSs and in the macula were assessed on OCT angiography. RESULTS: A total of 87 eyes of 57 DR patients were included. All CWSs were located in the retinal nerve fibre layer and exhibited a decorrelation signal on OCT angiography. In 68 eyes (78.1%), hyporeflective cavities appearing as cysts in the CWS were noted, especially when were peripheral and large (P < 0.0001). The thickness averaged 211.8 ± 80.02 mm and was positively influenced by recent diabetes history, glycaemic dysregulation, posterior pole localisation and a high VD of the SCP around the CWS, while the surface area averaged 0.33 ± 0.24 mm2 and was influenced only by a low VD of the SCP around the CWS (P < 0.001). In 72 eyes (82.7%), the non-perfused areas were predominant in the periphery of the CWS. CONCLUSION: The features of CWSs are influenced by systemic factors, such as history and severity of diabetes, in association with local factors, such as localisation and retinal ischaemia. The peripheral localisation of ischaemia confirms the hypothesis that CWSs are sentinel lesions.

Optical Coherence Tomography Angiography of Macula in Chronic Anterior Ischaemic Optic Neuropathy Associated with Giant Cell Arteritis.

A 74-year-old man presented with bilateral optic nerve atrophy related to arteritic anterior ischaemic optic neuropathy (AION) which started 22 years ago. Atrophic papilla was noted in both eyes and optical coherence tomography showed thinning of the retinal nerve fibres layer as well as the macular ganglion cell complex. Optical coherence tomography angiography of the macula found a decreased density of the superficial capillary complex, whereas deep capillary complex was well-preserved. Inner retinal layer atrophy in chronic arteritic AION is followed by a loss of vascularisation in these layers as a consequence of the neurodegeneration.

TREATMENT OF EXUDATIVE CIRCUMSCRIBED CHOROIDAL HEMANGIOMA: Efficacy of Fractionated Proton Therapy (20 Gray Relative Biological Effectiveness in 8 Fractions).

PURPOSE: To evaluate treatment of circumscribed choroidal hemangioma by hyperfractionated proton beam therapy protocol (20 gray relative biological effectiveness in 8 fractions) on tumor control, attachment of retina and visual function. METHODS: Retrospective review of patients treated between January 2010 and April 2015 with at least 6 months of follow-up. RESULTS: Forty-three patients with exudative and symptomatic circumscribed choroidal hemangioma were included. Before treatment, 41 (95%) presented an exudative retinal detachment, median visual acuity was 20/63 and median tumor thickness was 3.3 mm. Mean follow-up was 26 months (7-62). At last follow-up, all patients presented regression of ultrasound tumor thickness and 23/43 (53.5%) a totally flat scar. The mean time to achieve a flat scar was 20 months. Retina was reattached in all patients except one with 9 months of follow-up. Visual acuity was improved or stabilized in 37 patients (86%) and final median visual acuity was 20/25. No patient presented radiation maculopathy or papillopathy. CONCLUSION: Proton beam therapy with a dose of 20 gray relative biological effectiveness delivered in 8 fractions provides excellent anatomical and functional results and are comparable with those obtained with the same dose delivered in 4 fractions. Longer follow-up is required to determine the long-term radiation sequelae.

Retinal cavernous haemangioma treated by proton beam therapy.

As one of intraocular tumours, retinal cavernous haemangioma is a benign vascular lesion that is mostly unilateral. Very few cases about cavernous haemangioma treatment are reported, and there is currently no consensus on the most effective treatment. This clinical case reports on a 40-year-old male, presenting a peripheral retinal cavernous haemangioma, complicated with a repetitive vitreous haemorrhage causing bad vision. Several therapeutic methods were unsuccessfully attempted to stop haemorrhagic recurrences. Thanks to proton beam irradiation, a good collapse of aneurismal dilatations was obtained, with no recurrence of bleeding. This case study confirms that proton beam therapy is a good alternative in treating cavernous haemangiomas, leading to a total tumour regression without complications.

Intravitreal bevacizumab for neovascular glaucoma in uveal melanoma treated by proton beam therapy.

BACKGROUND: To evaluate the efficacy of bevacizumab on reduction of the enucleation rate and control of intraocular pressure (IOP) in neovascular glaucoma (NVG)-complicating proton beam therapy for UM and to identify the determinants of the efficacy of bevacizumab. METHODS: Retrospective comparative study of patients with rubeosis following proton therapy for uveal melanoma. Patients were divided into two groups: a bevacizumab group and a control group which comprised two subgroups: panretinal photocoagulation (PRP)/cryotherapy and observation subgroups. Bevacizumab was administered by three intravitreal injections at 1-month intervals. A second series of injections was administered when necessary. Data concerning IOP and the secondary enucleation rate were collected and compared between the two groups. Univariate and multivariate analyses were performed to determine predictive factors of response to bevacizumab. RESULTS: A total of 169 patients who developed rubeosis following proton therapy between 2006 and 2016 were included: 44 patients in the bevacizumab group and 125 in the control group (38 in the PRP/cryotherapy subgroup and 87 in the observation subgroup). The two groups presented the same baseline characteristics apart from hypertension, retro-equatorial site, and proximity of the optic disk, which were more frequent in the control group, while initial retinal detachment and larger tumor volume were more frequent in the bevacizumab group. After a mean follow-up of 31 months, IOP was less than 21 mmHg in 54.54% of patients after IVB versus 72.7% before treatment (p = 0.06). Statistical analysis did not reveal any statistically significant reduction of the enucleation rate in the bevacizumab group compared to the observational group, whereas the PRP/cryotherapy group showed better eye retention rate (p = 0.15). No enucleation was performed when IOP was < 21 mmHg before IVB. Multivariate analysis identified initial IOP < 21 mmHg and UM situated away from the macula as predictive factors of good response to bevacizumab. CONCLUSION: Despite the improvement of IOP level, intravitreal bevacizumab (IVB) did not reduce the overall enucleation rate in NVG following proton beam therapy. Nevertheless, this treatment was effective in the early phases of NVG or as preventive treatment. PRP remains a valid treatment for NVG.

Detection of an Iris Pigmented Epithelium Cyst in a Child by High Frequency Ultrasonography.

An 18-month-old boy presented with an iris pigmented epithelium cyst. The diagnosis was confirmed using high frequency ultrasonography. The cyst was determined to be in an intermediate location, thus needing no treatment provided that any potential complications were noted. However, continued monitoring is required to detect any changes to adapt management. [J Pediatr Ophthalmol Strabismus. 2017;54:e31-e33.].

Adult ocular medulloepithelioma diagnosed by transscleral fine needle aspiration: A case report.

Ocular medulloepithelioma (ME) is a rare congenital tumor which occurs usually during childhood but is also reported in adults. They have seen an intraocular tumor in an 89 years-old female with a history of small cell lung carcinoma. Transscleral fine needle aspiration was performed. Aspirates were rich and composed of two distinctive populations of cells. The first consisted of epithelioid large cohesive cells with rare rosettes. Nuclei were oval and chromatin was delicate with small nucleoli. The second population consisted of smaller and dispersed cells with regular nuclei and dusty chromatin. Immunohistochemistry performed on paraffin-embedded cell block sections showed that the larger cells and rosettes were cytokeratin AE1/AE3, Synaptophysin, Chromogranin A, CD56, NSE, and EMA positive, whereas the smaller cells were always negative. Interestingly smaller cells expressed only weak nuclear positivity for TTF1, whereas larger cells were TTF1 negative. Melanocytic markers were negative in both populations. Morphological patterns and immunohistochemical staining confirmed ocular ME and allowed to exclude pulmonary metastasis or primary malignant melanoma. The patient was successfully treated by brachytherapy alone and is alive and well 10 months after treatment. Diagn. Cytopathol. 2017;45:561-564. © 2017 Wiley Periodicals, Inc.