Evolution of an Astrocytic Hamartoma of the Optic Nerve Head in a Patient with Retinitis Pigmentosa – Photographic Documentation over 2 Years of Follow-Up

Aim: To report photographically the evolution of an astrocytic hamartoma of the left optic nerve head over a 2-year follow-up in a patient with retinitis pigmentosa. Methods: A 14-year-old boy was seen in the medical retina clinic with a 3-year history of night blindness. Best corrected visual acuity was 6/18 in both eyes. Colour vision was normal in both eyes and confrontation fields showed peripheral constriction. Fundus examination revealed bone spicule pigmentary changes at the retinal mid periphery typical of retinitis pigmentosa and superficial globules at the margins of both optic nerve heads. Electrodiagnostic tests confirmed moderately severe rod cone dystrophy with macular involvement bilaterally. Results: Two years later, the ocular examination was unchanged except for the appearance of the optic nerve head lesion in the left eye. There was an increase in the size of the lesion which was diagnosed as an astrocytic hamartoma. Further investigations were recommended to exclude neurofibromatosis and tuberous sclerosis. Conclusion: Astrocytic hamartomas of the optic nerve head and optic nerve head drusen have both been described in patients with retinitis pigmentosa. They can be a diagnostic dilemma although drusen are more common (10%). To differentiate these two entities it is very important to document any growth during the follow-up period which is suggestive of astrocytic hamartoma rather than optic disc drusen

Peripapillary intrachoroidal cavitation at the crossroads of peripapillary myopic changes

AIM: To analyze the prevalence of peripapillary intra-choroidal cavitation (PICC) in eyes with gamma peripapillary atrophy (γPPA), in eyes with peripapillary staphyloma (PPS) and in those combining γPPA and PPS and to analyze border tissue discontinuity in PICC. METHODS: This prospective cross-sectional non interventional study included highly myopic eyes. Non-highly myopic eyes were used as control. Radial and linear scans centered on the optic nerve head were performed using spectral-domain optical coherence tomography. Variables were analyzed along the twelve hourly optical coherence tomography sections in both eyes of each subject. RESULTS: A total of 667 eyes of 334 subjects were included: 229 (34.3%) highly myopic eyes and 438 (65.7%) non highly myopic eyes. The mean age of the highly myopic group was 48.99±17.81y. PICC was found in a total of 40 eyes and in 13.2% (29/220) of highly myopic eyes. PICC was found in 10.4% (40/386) of eyes with γPPA, in 20.5% (40/195) of eyes with PPS and in 22.7% (40/176) of those combining γPPA and PPS. All the eyes with PICC showed the co-existence of γPPA and PPS whereas none of the eyes presenting only one of these entities exhibited PICC. A border tissue discontinuity in the γPPA area was found in all eyes with PICC. CONCLUSION: We confirm the presence of a border tissue discontinuity in the γPPA area of all eyes with PICC. These findings suggest the involvement of mechanical factors in the pathogenesis of PICC which may contribute to PICC-related visual field defects

Active Ocular Toxoplasmosis Associated with Simultaneous Multiple Evanescent White Dot Syndrome.

We describe the association between active ocular toxoplasmosis and Multiple Evanescent White Dot Syndrome (MEWDS).info:eu-repo/semantics/publishe

Complete Resolution of a Giant Pigment Epithelial Detachment Secondary to Exudative Age-Related Macular Degeneration after a Single Intravitreal Ranibizumab (Lucentis) Injection: Results Documented by Optical Coherence Tomography

Aim:To describe a patient with a giant pigment epithelial detachment (PED) secondary to exudative age-related macular degeneration (ARMD) successfully treated with a single intravitreal ranibizumab (Lucentis) injection (0.5 mg/0.05 ml).Methods:An 89-year-old woman presented with a six-day history of reduced vision and distortion in the left eye. Best-corrected visual acuity in that eye was 6/15. Fundoscopy revealed a giant PED and exudates temporally to the fovea. Optical coherence tomography showed a PED associated with subretinal and intraretinal fluid. Fluorescein angiography confirmed the diagnosis of an occult choroidal neovascularization. Treatment with intravitreal injections of ranibizumab (Lucentis) was recommended, although the increased risk of retinal pigment epithelium (RPE) rip was mentioned. Results:Four weeks after the first intravitreal Lucentis injection, the visual acuity in the left eye improved to 6/7.5, with a significant improvement of the distortion and a complete anatomical resolution of the PED confirmed by optical coherence tomography. Conclusion:Giant PED secondary to exudative ARMD can be successfully treated with intravitreal ranibizumab, despite the increased risk of RPE rip. To our knowledge, this is the first case presenting with complete resolution of PED after a single ranibizumab injection

Central Serous Chorioretinopathy Associated with Desmopressin Nasal Spray: Causality or Unfortunate Association

Purpose: To describe the possible association between central serous chorioretinopathy (CSCR) and desmopressin use. Methods: The case histories of 2 middle-aged men with CSCR using desmopressin nasal spray were studied. Results: The diagnosis of CSCR was made on the basis of clinical features and ancillary testing (fluorescein angiography and optical coherence tomography). Both patients were using desmopressin nasal spray for polyuria when they developed the first ocular symptoms. Both of them also had an independent risk factor for developing CSCR. Conclusion: We suggest that desmopressin-induced hypercortisolism might implicate the development of CSCR in some patients. A larger study on patients using desmopressin nasal spray would be beneficial to confirm the possible association between this form of therapy and the development of CSCR

Necrotising retinopathy-like lesions as a manifestation of ocular sarcoidosis

Background: A 56-year-old Caucasian man presented with a 2-weeks history of decreased vision in the right eye. Vitritis, papillitis, cystoid macular oedema and inferior diffuse retinal infiltration were noticed. Extensive blood work-up, anterior chamber paracentesis with polymerase chain reaction (PCR) and Goldmann-Witmer coefficient, tuberculin skin test (PPD-test), fluorodeoxyglucose Positron Emission Tomography CT scan (FDG-PET/CT), lymph node biopsy and pars plana vitrectomy were performed. Results: Aqueous and vitreous samples were negative for an infectious and a lymphoproliferative etiology. Enlarged hilar and mediastinal lymph nodes were detected by FDG-PET/CT and subsequently biopsied, allowing to confirm the diagnosis of sarcoidosis. After a few months of oral corticosteroid therapy, the inflammation resolved completely and was replaced by atrophic retinal scars. Conclusion: Necrotising retinopathy-like lesions mimicking an infectious process or a lymphoproliferative disorder can be an atypical manifestation of ocular sarcoidosis.SCOPUS: ar.jinfo:eu-repo/semantics/publishe

Intravitreal methotrexate in vemurafenib-induced uveitis.

Vemurafenib, a BRAF inhibitor, has revolutionized the prognosis of late-stage melanoma patients, rising at the same time concerns about its potential adverse effects. Here is a case of vemurafenib-induced uveitis, peculiar in both its presentation and management.info:eu-repo/semantics/publishe

Effects of SOCS-1 gene transfer in ARPE cells

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Optic neuropathy, renal failure and pulmonary sarcoidosis in a 50-year-old man: where is the link?

Eye disorders are frequently associated with renal diseases, mostly linked to underlying causes such as hypertension, diabetes or autoimmune diseases. Conversely, advanced uraemic states may also lead to progressive vision impairment. The present report concerns a 50-year-old patient who presented with a bilateral, painless, progressive vision loss, a moderate systemic inflammation and chronic renal failure due to hypertension nephrosclerosis. Steroids were given and haemodialysis was initiated, resulting in vision improvement. At 4 months later when the steroids were stopped, the patient developed dyspnoea, cough, fever and fatigue of unclear origin. A lung biopsy showed non-caseating granuloma consistent with pulmonary sarcoidosis. Re-challenge with steroids rapidly improved the respiratory disease. Ophthalmological examinations performed early and later in the course excluded anterior ischaemic optic neuropathy and ocular manifestations of sarcoidosis, leading to a diagnosis of uraemic optic neuropathy. This rare ophthalmological disorder should be promptly recognised since haemodialysis and steroid therapy are highly effective

Analysis of Peripapillary Intrachoroidal Cavitation and Myopic Peripapillary Distortions in Polar Regions by Optical Coherence Tomography

info:eu-repo/semantics/publishe