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      Laser photocoagulation, intravitreal anti-VEGF, and vitreous surgery for the treatment of juxtapapillary retinal capillary hemangioma

      case-report

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          Abstract

          Juxtapapillary retinal capillary hemangiomas (JRCHs) are benign vascular tumors located on or adjacent to the optic nerve head. A 19-year-old girl presented with epiretinal membrane (ERM) associated with an elevated and round vascular tumoral mass located in the juxtapapillary region of her left eye. She was subsequently diagnosed with isolated JRCH. A combined approach with laser photocoagulation and intravitreal bevacizumab injection was used to facilitate shrinkage of the tumor preoperatively and pars plana vitrectomy was used to remove the tumor and ERM. A small remnant of tumoral mass remained intact and did not show any growth for 7 years.

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          Intravitreal anti-VEGF injection for the treatment of progressive juxtapapillary retinal capillary hemangioma: a case report and mini review of the literature

          We report a case of a patient known to have a von Hippel–Lindau disease with documented progressive juxtapapillary retinal capillary hemangioma (JRCH) with well-preserved visual acuity (VA) and visual field (VF). The patient received a single injection of intravitreal ranibizumab (IVR). Six months after IVR injection, the JRCH showed reduced vascularization, fibrosis, and mild shrinkage, and VA and VF remained unchanged. IVR therapy might therefore be considered as an alternative treatment for progressive JRCH, especially in patients with well-preserved VA and VF.
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            Elevated ocular levels of vascular endothelial growth factor in patients with von Hippel-Lindau disease.

            Von Hippel Lindau disease (VHL) is a rare autosomal dominant inherited disorder characterized by highly vascularized tumors in various organs. The abundant presence of endothelial cells in VHL tumors strongly suggest a role of the VHL tumor suppressor gene in the regulation of angiogenesis. Recently, in vitro studies have shown that the VHL tumor suppressor gene regulates the expression of vascular endothelial growth factor (VEGF). We investigated whether VHL patiens have increased levels of VEGF in their body fluids. The concentration of VEGF was measured in fluid of the anterior chamber of the eye, serum, urine, and fluid from renal cysts of VHL patients and unaffected individuals by ELISA. In addition, levels of basic fibroblast growth factor (bFGF), interleukin-8 (IL-8) and endothelin-1 (ET-1) were measured in urine and serum of VHL patients and control subjects. In 80% of the VHL patients VEGF was detectable in aqueous fluid of the anterior chamber of their eyes. A strong positive correlation (r = 0.90) was found between the age of VHL patients and ocular VEGF concentrations. At comparable age, VEGF levels in ocular fluid of VHL patients were significantly higher (P < 0.001) than in unaffected subjects. No correlation was found between VEGF concentration and the presence of retinal angiomas. A 10 and 16 fold increase of VEGF concentration was seen in fluid from two independent VHL-related cysts as compared with VEGF serum levels of the same patient. The mean concentration of VEGF in serum of VHL patients (n = 15) (319 +/- 84 pg/ml) was higher than in matched controls (238 +/- 68 pg/ml; P = NS). The mean concentration of VEGF in urine of VHL patients (128 +/- 36 pg/ml) was lower than in matched controls (183 +/- 25 pg/ml; P = NS). Concentrations of VEGF did not correlate with the presence of VHL-related tumors. No differences were observed between concentrations of bFGF, IL-8 and ET-1 in serum and urine of VHL patients and matched controls. These findings support a role for the VHL tumor suppressor gene in the in vivo regulation of VEGF.
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              Juxtapapillary Retinal Capillary Hemangioma: New Therapeutic Strategies

              The treatment of juxtapapillary retinal capillary hemangiomas (JRCHs) is still a therapeutic dilemma without established guidelines. Because of the location of these hemangiomas on or adjacent to the optic nerve, treatment is difficult and complex, especially when JRCHs are located in the papillomacular bundle. This manuscript reviews the clinically relevant data on literature regarding the treatment of JRCHs, focusing on novel combined therapies that have shown promising results in these lesions.
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                Author and article information

                Journal
                Indian J Ophthalmol
                Indian J Ophthalmol
                IJO
                Indian Journal of Ophthalmology
                Wolters Kluwer - Medknow (India )
                0301-4738
                1998-3689
                December 2019
                22 November 2019
                : 67
                : 12
                : 2104-2106
                Affiliations
                [1]Department of Ophthalmology, Uludag University School of Medicine, Bursa, Turkey
                [1 ]Department of Ophthalmology, Ozel Jimer Hastanesi, Bursa, Turkey
                Author notes
                Correspondence to: Dr. Gamze Ucan Gunduz, Department of Ophthalmology, Uludag University School of Medicine, Bursa, Turkey. E-mail: gamzeucan@ 123456gmail.com
                Article
                IJO-67-2104
                10.4103/ijo.IJO_1799_18
                6896561
                31755476
                5542b42e-710f-4cd9-a110-0aaa4f8a3751
                Copyright: © 2019 Indian Journal of Ophthalmology

                This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms.

                History
                : 29 October 2018
                : 08 January 2019
                : 18 April 2019
                Categories
                Case Reports

                Ophthalmology & Optometry
                capillary hemangioma,epiretinal membrane,juxtapapillary,optic nerve,retina

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