Peripapillary Neovascular Membrane in a Young Pregnant Woman and Prompt Response to Ranibizumab Injections following Uneventful Delivery

Purpose: Occurrence of choroidal neovascularization (CNV) during pregnancy has been reported as a complication of presumed ocular histoplasmosis syndrome or punctuate inner chorioretinopathy. To our knowledge, idiopathic CNV (ICNV) during pregnancy has only been reported once in the relevant literature. Bevacizumab has been used for the treatment of ICNV in small case series. However, there is limited experience regarding the use of ranibizumab for the management of ICNV. Case Report: A 31-year-old woman in the eighth month of her second pregnancy was diagnosed with mild macular and papillary edema. She was followed up using biomicroscopy, fluorescein angiography (FA), and optical coherence tomography (OCT). After 3 months, visual acuity further deteriorated and funduscopy, FA and OCT findings revealed a juxtapapillary choroidal neovascular membrane (CNVM). After two ranibizumab injections, best-corrected visual acuity increased significantly, physiological macular anatomy was restored and no subretinal fluid was observed. Discussion: In this case report, we present a young pregnant patient with peripapillary ICNV and neurosensory detachment involving the macula, and treatment of the eye with intravitreal ranibizumab following uneventful delivery. Increased angiogenic factor levels associated with pregnancy may contribute to the onset of CNV although this relationship has to be investigated experimentally. The rapid response to ranibizumab suggests that this anti-VEGF agent may be an alternative treatment option in the management of peripapillary ICNV

Retinal Dystrophy in Long-Chain 3-hydroxyacyl-CoA Dehydrogenase Deficiency

[No abstract available

Myopexie retro-equatoriale, après transposition selon Hummelsheim, dans le traitement de la paralysie du VIème nerf crânien [Retro-equatorial myopexy following Hummelsheim transposition in treatment of 6th cranial nerve paralysis]

INTRODUCTION: Vertical muscle transpositions are considered as the classic procedures for total VIth nerve palsy, whereas weakening of the controlateral medial rectus muscle requires a residual function of the paretic muscle. The aim of this study is to investigate the cumulative effects of these two different surgical approaches applied to the same patients. PATIENTS AND METHODS: 2 patients (M 48 years and F 55 years) with posttraumatic total VIth nerve paralysis were operated in two steps more than 1 year after trauma. The first procedure consisted of a Hummelsheim transposition of the vertical recti and was followed by a posterior fixation of the controlateral medial rectus. RESULTS: The vertical transposition resulted in the improved position of the paretic eye and in the disappearance of diplopia in primary position. The posterior fixation considerably improved the motility in abduction of the paretic eye and consequently provided the patients with widened binocular field of fusion. CONCLUSION: Posterior fixation of the controlateral medial rectus efficiently complements the vertical transposition in cases of total VIth nerve palsy. This combined effect remained stable during a 2 years follow-up in our patients

Description d'un melanome de l'uvee dans le cadre d'une neurofibromatose de type 2. [Choroidal melanoma in neurofibromatosis type 2: description of a case]

To the best of our knowledge this is the first description of a choroidal melanoma with documented growth in neurofibromatosis type 2 (NF2). A 20-year-old patient with NF2 presenting deafness due to bilateral acoustic neurinomas and unilateral amaurosis due to a meningioma of the optic nerve developed a pigmented parapapillary choroidal tumor. Despite signs indicating the diagnosis of a melanoma, periodic observation was chosen in order to postpone functional amputation following optic nerve irradiation. The tumor growth was slow during the 5 years that followed, and once progression became rapid, the tumor was treated by accelerated proton beam radiotherapy. One year later, visual acuity diminished due to actinic optic neuropathy and was stabilized at 0.3 for the 2 following years. The tumor presented objective signs of regression, and no sign of metastatic disease was observed. The therapeutic approach in this case provided local control of the tumor while preserving useful visual function

Surgical approach in a case of unilateral retinal pigment epithelium dysgenesis and literature review

Purpose: To present a case of unilateral retinal pigment epithelium dysgenesis (URPED) complicated with tractional retinal detachment and macular hole formation, and highlight the successful anatomical and functional restoration following surgical repair. To conduct an updated review of the literature. Observations: A 16-year-old asymptomatic female presented with a unilateral atypical peripapillary lesion of the retinal pigment epithelium (RPE) in the left eye. At baseline, best corrected visual acuity (BCVA) was 20/20 and anterior segment examination was unremarkable. Fundus examination revealed an irregularly shaped atrophy of the RPE adjacent to the optic disc with scalloped border of RPE hyperplasia and a fibroglial proliferation in the overlying retina. Optical coherence tomography demonstrated mild changes of the RPE and the outer retina layers. Three years after initial diagnosis, the patient was referred to our clinic due to blurry vision. Complete ophthalmological evaluation revealed tractional retinal detachment with full thickness macular hole formation. Pars plana vitrectomy with epiretinal membrane removal and internal limiting membrane peeling led to anatomical recovery of the macular area with BCVA of 20/32 at four-months postoperatively. Conclusions and importance: This is the first report of tractional retinal detachment and macular hole as rare complications of URPED. Systematic follow-up examinations seem to be essential for the prevention of permanent visual loss, whereas prompt surgical intervention can contribute to visual acuity restoration in complicated cases. © 2021 The Author

Ranibizumab in the management of advanced Coats disease Stages 3B and 4: long-term outcomes.

BACKGROUND: Laser photocoagulation and cryotherapy to completely destroy telangiectatic vessels and ischemic retina in Coats disease is barely applicable in advanced cases with total retinal detachment, and globe survival is notoriously poor in Stages 3B and 4. Anti-vascular endothelial growth factor intravitreal injections may offer new prospects for these patients. METHODS: This study is a retrospective review of all consecutive patients with Coats disease treated with neoadjuvant or adjuvant intravitreal ranibizumab plus conventional and amblyopia treatment as appropriate. RESULTS: Nine patients (median age, 13 months) presenting Coats Stages 3B and 4 (5 and 4 eyes, respectively) were included. Iris neovascularization resolved within 2 weeks and retinal reapplication within 4 months in all patients. At last follow-up, globe survival was 100% with anatomical success in 8 of the 9 eyes. With a median follow-up of 50 months, fibrotic vitreoretinopathy was developed in 5 of the 9 cases, one leading to tractional retinal detachment and ultimately phthisis bulbi. The remaining 4 of the 9 eyes achieved some vision (range, 0.02-0.063). CONCLUSION: To the best of the authors' knowledge, this is the largest reported series of late-stage Coats undergoing anti-vascular endothelial growth factor therapy, a homogenous cohort of patients treated with a single agent and with the longest follow-up. This study supports the role of ranibizumab in advanced disease by transient restoration of the hemato-retinal barrier and suppression of neovascularization to facilitate classic treatment. At the last follow-up, the authors report unprecedented anatomical success and functional outcome

Choroidal thickness and ocular growth in childhood

The involvement of the choroid in ocular growth regulation has been postulated in studies showing that refractive errors correlate with alterations in choroidal thickness (ChT). The advent of optical coherence tomography imaging has enabled qualitative and quantitative assessment of the choroid. In children, ChT changes correlate with a number of ocular pathologies, including myopia, retinopathy of prematurity, and amblyopia. We synthesize mechanisms and evidence regarding choroidal thickness variation during childhood. Subfoveal ChT is influenced by a number of factors including age, ethnicity, gender, axial length, and intraocular pressure. Myopic eyes have thinner choroids compared to emmetropic and hyperopic eyes. ChT may in fact serve as a marker of myopic progression, as ChT thinning occurs early during myopic development, but this association has not been established quantitatively. In addition, subfoveal ChT appears thicker in amblyopic eyes, while prematurity and retinopathy of prematurity may be associated with thinner ChT. Overall, both animal models and clinical research indicate that ChT induces or reflects physiological changes in the eye pertaining to ocular growth or maturation. © 2020 Elsevier Inc

Phenotypic variability of retinocytomas: preregression and postregression growth patterns.

AIM: To describe the incidence of retinocytomas, their variability at presentation and their growth patterns both before and after regression.¦METHODS: Medical notes of the 525 patients of the Jules-Gonin Eye Hospital Retinoblastoma Clinic between 1964 and 2008 were reviewed and the charts of 36 patients with retinocytomas and/or phthisis bulbi were selected.¦RESULTS: The proportion of patients with retinocytomas and/or phthisis bulbi was 3.2%. The mean age at diagnosis was 28.7±17 years. Five tumours presented a cystic pattern (5.8%). Evidence of aggressive exophytic disease prior to spontaneous regression was documented in two eyes, and of invasive endophytic disease (regressed vitreous seeding or internal limiting membrane disruption) in three eyes. Twenty patients were followed with a mean follow-up of 44±60 months. Tumour growth was observed in 16% cases, benign cystic enlargement in 4% and malignant transformation in 12%.¦CONCLUSION: This large study of retinocytomas substantially expands the published features of retinocytoma by describing the cystic nature of some retinocytomas as well as clinical characteristics of the endophytic and exophytic preregression growth patterns. The authors report two different patterns of reactivation: benign cystic enlargement and malignant transformation with or without cystic growth. Higher than previously reported frequency of growth and possible life-threatening complications impose close lifetime follow-up of retinocytoma patients