Optical coherence tomography angiography features of choroidal neovascularization secondary to angioid streaks.

PURPOSE: To characterize the features of choroidal neovascularization (CNV) secondary to angioid streaks (AS) with optical coherence tomography angiography (OCT-A) and to assess its sensitivity in CNV detection in this particular context. METHODS: Consecutive patients, both with treatment-naïve and recurrent CNV associated with angioid streaks were prospectively analyzed. All patients underwent macular imaging by fluorescein angiography (FA), indocyanine green angiography (ICGA), spectral-domain (SD)-OCT, and OCT-A (AngioVue, Optovue, Optovue Inc., Freemont, CA, USA). OCT-A detection rate of CNV associated to AS was evaluated by two independent observers. We studied the association between OCT-A feature and either exudative or active status using Fisher exact test. RESULTS: A total of 32 eyes of 18 consecutive patients were included in the analysis. OCT-A was able to detect CNV associated with angioid streaks in 87.5 % (28/32) eyes. OCT-A phenotypes of CNV were classified into interlacing pattern in 9 eyes, pruned vascular tree pattern in 7 eyes, and combined pattern in 12 eyes (Interuser agreement: 0.871 ± 0.071). CNV were not detectable in 4/32 eyes. There was a statistically significant association between the presence in OCT-A of densely ramified networks with both previous treatment status in the last 6 months (p < 0.001) and with exudative signs on SD-OCT (p = 0.014). CONCLUSION: OCT-A appears as a sensitive tool for detection of CNV secondary to AS. The interlacing pattern was significantly associated with active and exudative features.

[Treatment with ranibizumab for choroidal neovascularization secondary to a pseudoxanthoma elasticum: Results of the French observational study PiXEL]. / Traitement par ranibizumab des néovascularisations choroïdiennes secondaires à un pseudoxanthome élastique : résultats de l'étude observationnelle française PiXEL.

INTRODUCTION: Pseudoxanthoma elasticum (PXE), a rare hereditary connective tissue disorder, may be complicated by angioid streaks (AS) and choroidal neovascularization (CNV), which may lead to irreversible loss of visual acuity (VA). Here we describe the safety and efficacy of ranibizumab in patients with CNV secondary to PXE. METHODS: A multicenter (n=23), observational study of a retrospective/prospective cohort, performed under real world conditions in France in all patients with CNV secondary to PXE who received at least one ranibizumab injection as of October 2011. The study objectives were to describe the mean annual number and reason for ranibizumab injections since initiation, evolution of best-corrected visual acuity (BCVA by Early Treatment Diabetic Retinopathy Study [ETDRS] letters), and safety. RESULTS: Patients (n=72; 98 eyes) had a mean age of 59.6±8.3years and consisted of 54.2% men. The criterion for retreatment was based mainly on loss of VA, progression of CNV and angiographic leakage. CNV was primarily subfoveal or juxtafoveal (73.4%), and the initial mean VA was 64.6 ETDRS letters. On average, visual acuity of all eyes analyzed was relatively stable during the 2-year follow-up (62.3 letters vs 64.6 letters at the first injection), and 88.6% of eyes maintained VA between -15 and +15 letters or gained over 15 letters. No deaths or new intolerances were described. CONCLUSIONS: These results showed that ranibizumab was able to maintain stable VA in clinical practice for at least 2years in patients with CNV secondary to PXE, and to significantly reduce the frequency of neovascularization relapses, with a limited number of injections. The treatment was well tolerated by the patients.

High-resolution spectral domain optical coherence tomography features in adult onset foveomacular vitelliform dystrophy.

PURPOSE: To describe the different morphological features in adult onset foveomacular vittelliform dystrophy (AOFVD) using high-resolution spectral domain optical coherence tomography (OCT). DESIGN: Prospective observational case series. METHODS: Complete ophthalmologic examination, including spectral domain OCT, was performed in 49 consecutive AOFVD patients (60 eyes). RESULTS: In 28/60 eyes, spectral domain OCT showed hyper-reflective clumps within the outer plexiform and outer nuclear layers. In 9/60 eyes, the photoreceptor inner segment/outer segment (IS/OS) interface appeared highly reflective like a shell all around the vitelliform material, and appeared irregular and discontinued in 27/60 eyes. The Verhoeff membrane was clearly visible at the border of the lesion, disappeared over the vitelliform lesion in 20/60 eyes, became thickened and less defined on the outer aspect of the lesion in 11/60 eyes, appeared without noticeable alterations in 10/60 eyes and not well defined in 19/60 eyes. The vitelliform material appeared as a highly reflective dome-shaped lesion (homogeneous in 14/60 eyes and heterogeneous in 36/60 eyes) located between the photoreceptor layer and the retinal pigment epithelium (RPE). In 10/60 eyes, the macular lesion appeared as hypo/a-reflective. The RPE appeared irregular in 14/60 eyes, with hyper-reflective mottling on its inner aspect. We observed discrete RPE detachments in 29/60 eyes. CONCLUSIONS: We hypothesise that early changes involve the layer between RPE and the IS/OS interface, first with vitelliform material accumulation beneath the sensory retina, and then with IS/OS alterations, pigments migration towards inner layers and fluid accumulation. These changes come with RPE alterations such as hypertrophy or sub-RPE deposits.

Cost-effectiveness sequential modeling of ranibizumab versus usual care in age-related macular degeneration.

AIMS: To assess effectiveness, cost, and cost-effectiveness of ranibizumab versus the current medical practices of treating age-related macular degeneration in France. METHODS: A simulation decision framework over 1 year compared ranibizumab versus the usual care using two effectiveness criteria: the "visual acuity improvement rate" (greater than 15 letters on the ETDRS scale) and the "rate of legal blindness avoided". Two decision trees included various sequences of current treatments, with or without ranibizumab. RESULTS: Ranibizumab appeared significantly more effective than the usual care (p < 0.001), providing greater treatment success rate of visual acuity improvement (48.8% versus 33.9%). The cost of the ranibizumab strategy was higher (9,123 euros over 1 year for ranibizumab versus 7,604 euros for the usual care) but the average cost-effectiveness was lower--18,721 euros/success for ranibizumab versus 22,543 euros/success for usual care (p < 0.001). Considering the "legal blindness avoided" success criterion, the ranibizumab strategy appeared significantly more effective (p < 0.001), providing greater treatment success rate for of legal blindness avoided than usual care (99.7% versus 93.1%) although it was more expensive (9,196 euros over 1 year for ranibizumab versus 5,713 euros for the usual care). CONCLUSION: Ranibizumab significantly improved the rate of visual acuity improvement and reduced the rate of legal blindness. Ranibizumab appeared significantly more cost-effective than the usual treatments in terms of visual acuity improvement.

[Surgical treatment of persistent macular edema in retinal vein occlusion]. / Traitement chirurgical de l'oedème maculaire persistant des occlusions veineuses rétiniennes.

INTRODUCTION: Persistent macular edema (ME) is the main cause of poor visual outcome in nonischemic retinal vein occlusion (RVO). The aim of this study was to evaluate the results of surgery in this disorder. PATIENTS AND METHODS: Retrospective nonrandomized comparative pilot study. Visual acuity measurement, fluorescein angiography, and optical coherence tomography were performed before and after treatment, at 3 months, 6 months, and 1 year. RESULTS: The study included 42 eyes: 15 eyes underwent vitrectomy with peeling of the posterior hyaloid and/or the internal limiting membrane, 19 received intravitreous injection of triamcinolone (IVT), and four had a combination of both treatments. In the vitrectomy group, 63% of the eyes showed a progressive decrease of ME throughout the year of follow-up (p<0.05 at 1 month and 1 year) and an increase in visual acuity (p<0.05 at 3 and 6 months); no complication occurred. In the IVT group, the eyes showed a rapid improvement in vision and in ME at 1 month (p=0.007 and p=0.001, respectively) but with a frequent ME recurrence at 4 months; however, 31% of eyes showed durable improvement. Complications after IVT were pseudo-endophthalmitis (one eye), transient hypertony (53%), and a full-thickness macular hole (one eye). In the group treated with vitrectomy combined with IVT, ME decreased rapidly and durably, but vision did not improve in this small subgroup of long-lasting RVO with prior pigment epithelial changes. CONCLUSION: In some eyes, vitrectomy seems to have a more durable effect than IVT alone. Further randomized and controlled studies are needed to confirm these results and to compare them to the natural course of the disease.

[Fundus flavimaculatus and choroidal neovascularization]. / Fundus flavimaculatus et néovascularisation choroïdienne.

OBJECTIVE: Fundus flavimaculatus is an aspect of Stargardt disease, which is characterized by juvenile onset, rapidly progressive deterioration, and poor visual outcome due to atrophic areas or flecks. Recently, a late-onset form has been described, on the borderline with AMD. However, choroidal neovascular complications are not frequent and rarely described in Stargardt's disease. CASE REPORT: Late discovery of FFM in a patient over 50 years old. During her follow-up, she presented a decrease in visual acuity due to juxtafoveal choroidal neovascularization. In spite of laser treatment, a retrofoveal recurrence appeared, which was treated with perifoveal laser. DISCUSSION: Confusion between AMD and a late-onset Stargardt may occur because of their many clinical similarities. The population and the fundus results can be similar to AMD and an examiner may be confused. In the literature, choroidal neovascular complications appear in patients over 50 years of age who may suffer from AMD. In fundus flavimaculatus, pigmentary epithelium alterations are due to progressive lipoproteic scrap storage that predisposes to Bruch's membrane rupture and choroidal neovascularization. This physiopathology resembles the that of AMD. CONCLUSION: Choroidal neovascular complications are possible in late-onset Stargardt disease. Their appearance together may be greater than previously believed.

Surgical removal of subfoveal choroidal neovascularization: visual outcome and prognostic value of fluorescein angiography and optical coherence tomography.

PURPOSE: To study the functional results of macular surgery and determine pre-operative features associated with better final visual outcome. METHODS: Forty-two consecutive patients underwent surgical removal of subfoveal choroidal neovascularization (CNV), related to age-related macular degeneration (AMD) in 8 eyes, degenerative myopia in 14 eyes, multifocal choroiditis (MFC) in 10 eyes, idiopathic CNV in 6 eyes and other etiologies in 4 eyes. Mean age was 49 years. Pre-operative visual acuity (VA) was 20/200 or less in 30 eyes (71.4%) and never better than 20/40. Fluorescein angiography was analyzed before and after surgery. Pre-operative optical coherence tomographs (OCT) were studied in a masked fashion. Mean follow-up was 12 months (range 4-48 months). RESULTS: Final VA was 20/200 or less in 25 eyes (60%). According to the CNV etiology, the percentage were 87.5%, 80%, 57.1% and 20% respectively in eyes with AMD, MFC, high myopia, and idiopathic or other diseases. Post-operative VA improved in 21 eyes (50%) but subsequently declined in 7% by the final examination. Patients younger than 50 years had better functional results (p=0.006). Lack of retinal pigment epithelium (RPE) changes on pre-operative angiography was correlated with good visual outcome (p<0.001). The OCT study confirmed some features already described and showed some different CNVpatterns: above and usually separated from the RPE, below and not separated from the RPE, and ungradable. Eyes with the first OCT pattern had the best visual outcome. Main complications included 4 (10%) retinal detachments and 9 (21%) recurrences. OCT was also useful to confirm CNV recurrences post-operatively. CONCLUSIONS: CNV surgical excision results vary depending on the underlying disease, the RPE and choriocapillaris function, and the features observed on pre-operative OCT images.

A new approach of geodesic reconstruction for drusen segmentation in eye fundus images.

Segmentation of bright blobs in an image is an important problem in computer vision and particularly in biomedical imaging. In retinal angiography, segmentation of drusen, a yellowish deposit located on the retina, is a serious challenge in proper diagnosis and prevention of further complications. Drusen extraction using classic segmentation methods does not lead to good results. We present a new segmentation method based on new transformations we introduced in mathematical morphology. It is based on the search for a new class of regional maxima components of the image. These maxima correspond to the regions inside the drusen. We present experimental results for drusen extraction using images containing examples having different types and shapes of drusen. We also apply our segmentation technique to two important cases of dynamic sequences of drusen images. The first case is for tracking the average gray level of a particular drusen in a sequence of angiographic images during a fluorescein exam. The second case is for registration and matching of two angiographic images from widely spaced exams in order to characterize the evolution of drusen.

[Vitrectomy-laser-gas for treating optic disk pits complicated by serous macular detachment]. / Vitrectomie-laser-gaz pour le traitement des fossettes colobomateuses de la papille compliquées de décollement séreux rétinien maculaire.

PURPOSE: Optic nerve pit is a rare congenital anomaly. In two third of the patients, the severity of the disease is increased by the apparition of a serous macular detachment, which may compromise the visual prognosis. The aim of this study is to propose a therapy appropriate to such complication. METHODS: A prospective study was performed including 10 patients with a serous macular detachment caused by optic nerve pit. All patients underwent intraocular surgery including vitrectomy, peripapillary photocoagulation and intravitreal injection of gas. The mean postoperative follow-up period was 9.5 months. RESULTS: Therapy success was based on anatomical and functional results. Serous macular detachment reattached in 7 patients out of 10 and a recurrence was observed in one case. Visual acuity improved from 0 to 18 lines of the EDTRS chart (mean increase: 6.7 lines). CONCLUSION: These results confirm that intraocular surgery including vitrectomy-photocoagulation-gas injection is a valuable treatment for serous macular detachment associated with optic nerve pit.

[Collar-button choroidal melanoma. Anatomo-radiologic correlations]. / Mélanome choroïdien en bouton de col. Confrontations anatomo-radiologiques.

Malignant melanomas of the choroid are the most frequent symptomatic eye tumours in adults. They often have a pathognomonic appearance, being collar-button or mushroom shaped due to rupture of Bruch's membrane by the tumoral mass. The ultrasonographic image of collar-button melanoma is well known: the head of the tumour is hyperechogenic and its base hypoechogenic. According to some authors, this is caused by difference in blood supply between the two parts. At MRI strongly pigmented melanomas emit a high-intensity signal on T1-weighted sequences and a low-intensity signal on T2-weighted sequences, but these characteristic features are inconstant. We present a case of collar-button melanoma explored by ultrasonography, colour Doppler Flow Imaging (CDFI) ultrasound and MRI, then enucleated. Flows and signals were different in front of, or behind the rupture of Bruch's membrane: ultrasounds showed a hyperechogenic image at the head and a hypoechogenic image at the base; on T2-weighted MRI sections intensity was greater in the head than in the base (head: 69 ms, base 180 ms) on CDFI, no flow was detectable in the head and very high flows were seen in the base of the tumour. Comparisons of these images with pathological findings, where there was no difference between head and base in melanin concentration and in cellular type (mixed or mainly epithelioid), led us to believe that the differences observed in images were essentially due to differences in blood supply between the two parts of the tumour constricted by the sides of the ruptured Bruch's membrane.

Clinical risk factors for proliferative vitreoretinopathy after retinal detachment surgery.

PURPOSE: To identify the clinical risk factors for the development of severe proliferative vitreoretinopathy (PVR) after retinal detachment surgery. METHODS: A retrospective study of 1020 patients with either no PVR or with PVR of grade C1 or less at initial examination was conducted. After surgery, severe PVR was defined as grade C2 or worse. The data relating to 94 variables were evaluated by univariate analysis and stepwise logistic regression. RESULTS: Severe PVR developed after surgery in 107 patients (10.5%). Ten significant predictive variables were identified: minor intra- or postoperative hemorrhage, grade A preoperative PVR, preoperative choroidal detachment, giant tears, air tamponade, detachment involving more than 2 quadrants, cumulative break area larger than 3 optic disks, postoperative choroidal detachment, signs of uveitis at initial examination, and grade B preoperative PVR. CONCLUSION: The results indicate that in addition to the size of breaks, extent of detachment, and presence of preoperative inflammation or low-grade PVR, iatrogenic problems also are important factors in the pathogenesis of severe PVR after surgery for retinal detachment.

Clinical experience with digitized fluorescein angiography.

Directly acquired images of fluorescein angiography may be helpful in the treatment of retinal diseases. We report our experience with a commercially available image processing system. The major advantage of this system is that the pictures are instantly displayed on a television monitor allowing immediate treatment of the patient. However, further development of complementary software procedures is necessary to obtain an instrument capable of performing useful image-analysis of fundus lesions.

[Significance of the digital image in the diagnosis and classification of macular drusen]. / Intérêt de l'image numérisée dans le diagnostic et la classification des drusen maculaires.

Macular drusen, hard or soft, have been often observed at the early stages of age-related macular degeneration. Clinically, it has been shown that soft drusen and macular subretinal new vessels are associated in a statistically significative manner. The question is to recognize and to determine drusen at greatest risk for developing choroidal neovascularization? To answer to this question, a method of digital images analysis of fluorescein angiography had been perfected. About ten eyes, preliminary results show, an objective and quantitative difference of the evolution of the grey level during the same angiogram between the different retinal structures. This method would bring a prognostic feature for the long term follow-up of patients with drusen.

[Surgical treatment of the Marcus-Gunn syndrome. Indications and results. Apropos of 15 cases]. / Traitement chirurgical du syndrome de Marcus-Gunn. Indications et résultats. A propos de 15 cas.

Fifteen cases of jaw-winking blepharoptosis treated, are reported, to suggest the management of the surgical methods. The degree of the ptosis, the eyelid retraction, the surgical procedures are reviewed. Patients with a wild retraction required a levator muscle resection. Patients with a moderate or severe retraction required an unilateral levator denervation with frontalis suspension. In this case, the most satisfactory surgical results were obtained with unilateral levator denervation on the affected side compared with a bilateral frontalis suspension with fresh autogenous fascia. Then, the surgical procedure seems to depend especially on the eyelid retraction.