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The presence of retinal capillary hemangioblastoma and cerebellar hemangioblastoma in the context of Von Hippel-Lindau syndrome (VHL) is not characteristically associated with other ophthalmologic conditions. Here, we report the case of a 22-yearold female with a history of bilateral primary congenital glaucoma who presented with a right juxtapapillary retinal capillary hemangioblastoma and an old hemiretinal vein occlusion in which the retinal capillary hemangioblastoma was likely the contributing factor. Her systemic work up was positive for VHL syndrome and revealed the presence of a fatal large brainstem hemangioblastoma. To our knowledge, the association of VHL and congenital glaucoma and/or retinal venous occlusion has not been reported. [ABSTRACT FROM AUTHOR]

Abstract: An 18year-old male with no antecedent of trauma, systemic syndrome or myopia was referred for surgical treatment of a full thickness macular hole in the left eye. A more careful inspection revealed discrete foveal cystic changes in the fellow eye and subtle peripheral depigmented retinal pigment epithelial changes in both eyes. A spectral-domain optical coherence tomography (SD-OCT) scan confirmed, in addition to the full thickness macular hole in the left eye, microcystic spaces in the nuclear layers of both retinae. The diagnosis of X-linked retinoschisis was confirmed with a full field electroretinogram displaying the typical negative ERG. Macular holes are uncommon in the young and those complicating X-linked retinoschisis are rare. This report highlights the importance of investigating the presence of a macular hole in a young patient and illustrates the clinical and SD-OCT clues beyond the foveal center which led to the correct diagnosis of X-linked juvenile retinoschisis. [Copyright &y& Elsevier]

Idiopathic juxtafoveolar retinal telangiectasis (IJFT), also known as parafoveal telangiectasis or idiopathic macular telangiectasia, refers to a heterogeneous group of well-recognized clinical entities characterized by telangiectatic alterations of the juxtafoveolar capillary network of one or both eyes, but which differ in appearance, presumed pathogenesis, and management strategies. Classically, three groups of IJFT are identified. Group I is unilateral easily visible telangiectasis occurring predominantly in males, and causing visual loss as a result of macular edema. Group II, the most common, is bilateral occurring in both middle-aged men and women, and presenting with telangiectasis that is more difficult to detect on biomicroscopy, but with characteristic and diagnostic angiographic and optical coherence tomography features. Vision loss is due to retinal atrophy, not exudation, and subretinal neovascularization is common. Group III is very rare characterized predominantly by progressive obliteration of the perifoveal capillary network, occurring usually in association with a medical or neurologic disease. This paper presents a current review of juxtafoveolar retinal telangiectasis, reviewing the classification of these entities and focusing primarily on the two most common types encountered in clinical practice, i.e., groups I and II, describing their clinical features, histopathology, natural history, complications, latest results from imaging modalities and functional studies, differential diagnosis, and treatment modalities. [ABSTRACT FROM AUTHOR]

Objective and Design: A retrospective review study was designed to describe five cases of optic disc melanocytomas with tumor-related visual impairment. Participants: Five patients with optic disc melanocytoma presented with visual complaints to a tertiary eye hospital in Saudi Arabia. Materials and Methods: Demographic and clinical data were analyzed, including the results of ocular examination, lesion laterality, best-corrected Snellen visual acuity, pupillary reflex, visual field testing, color fundus photography, fundus fluorescein angiography, and ophthalmic ultrasound. Results: Visual dysfunction secondary to optic disc melanocytoma was identified. Case 1 had macular star edema with mild tumor enlargement, Case 2 had optic atrophy, Case 3 had juxtapapillary choroidal neovascular membrane with macular involvement, Case 4 had optic disc swelling with an enlarged blind spot, and Case 5 had a large altitudinal visual field defect. Conclusion: Although melanocytomas of the optic disc tend to have a benign behavior with slow evolution and stable vision, they may adversely affect visual function through a variety of mechanisms. [ABSTRACT FROM AUTHOR]

Background: Neovascular glaucoma (NVG) is a severe form of secondary glaucoma caused by the growth of newvessels over the trabecular meshwork. The principal causes are associated with retinal ischemia. Ablative treatmentof the retina can prevent, halt, and even reverse the growth of new vessels on the iris and angle. It is an essential partof the management in most cases. Aims: To determine the causes of NVG among Saudi patients, presented at the King Khaled Eye Specialist Hospital. Methods: A retrospective review of 337 Saudi patients with NVG was obtained. All cases were reviewed for theevidence and causes of the disease, and their basic demographic information. A subset of 100 diabetic patients withPDR was further studied in greater detail for clinical findings and treatment history. Results: The most common primary etiologic associations for NVG included diabetic retinopathy (DR) (56.06%), retinalvenous obstruction (26.40%), and chronic retinal detachment (03.56%). A history of diabetes mellitus was reported in65.04%, systemic arterial hypertension was noted in 61.00%, and evidence of renal impairment was documented in22.00%. Vision was markedly reduced in most eyes with NVG (median: hand motion). The median best visual acuity inthe fellow eye was 20/160. Among the 100 cases, with DR as a cause of NVG, 43 patients had bilateral neovascularizationof the iris (NVI) and 72 had bilateral PDR. Sixty-one patients had no previous laser treatment before the diagnosis of NVG. Among these, who received treatment, the median number of total laser spots was 1,003. Conclusions: Diabetes is a major cause of NVG presented to this tertiary eye care center in the Kingdom of Saudi Arabia followed by retinal venous obstruction. Close monitoring and full pan-retinal photocoagulation (PRP) were absent in most of the diabetic cases. It is important to recognize that the "unaffected" fellow eye, particularly in diabetic patients, may require fairly urgent treatment as well. [ABSTRACT FROM AUTHOR]

Many risk factors have been linked to retinal vein occlusions (RVOs) whether central or branch retinal vein occlusion. Ocular risk factors include glaucoma and hypermetropia. Controversy exists to whether short axial length is a risk factor for retinal vein occlusions. We report an extreme case that supports the latter hypothesis. A 33-year-old male presented with decreased visual acuity in the left eye. He turned out to have nanophthalmos with hemiretinal vein occlusion and macular edema with unremarkable systemic work up for retinal vein occlusion except for a glycated hemoglobin (HbA1c) level of 7%. To our knowledge this is the first case report of hemiretinal vein occlusion in the setting of nanophthalmos and suggests that short axial length may be a risk factor for retinal vein occlusion. [ABSTRACT FROM AUTHOR]