Von Hippel-Lindau Disease and the Eye
Retinal hemangioblastoma (also referred to as retinal capillary hemangioma) is a benign lesion originating from the endothelial and glial components of the neurosensory retina and optic nerve head. Historically known as a manifestation of the von Hippel-Lindau (VHL) disease, it can be seen as an iso...
| Main Authors: | , , , |
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| Format: | Article |
| Language: | English |
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Knowledge E
2020-02-01
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| Series: | Journal of Ophthalmic & Vision Research |
| Subjects: | |
| Online Access: | https://doi.org/10.18502/jovr.v15i1.5950 |
| _version_ | 1811248885997240320 |
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| author | Saeed Karimi Amir Arabi Toktam Shahraki Sare Safi |
| author_facet | Saeed Karimi Amir Arabi Toktam Shahraki Sare Safi |
| author_sort | Saeed Karimi |
| collection | DOAJ |
| description | Retinal hemangioblastoma (also referred to as retinal capillary hemangioma) is a benign lesion originating from the endothelial and glial components of the neurosensory retina and optic nerve head. Historically known as a manifestation of the von Hippel-Lindau (VHL) disease, it can be seen as an isolated finding or in association with some rare ocular conditions. In addition to characteristic ophthalmoscopic features, results of numerous ancillary tests including angiography, ultrasound, optical coherence tomography, and genetic tests may support the diagnosis and differentiate it from similar conditions. Because of serious life-threatening complications of VHL disease, every ocular approach to retinal hemangioblastomas should be in relationship with additional multidisciplinary diagnostic and therapeutic efforts. In addition, any patient with actual or probable diagnosis of VHL disease should be screened for ocular involvement. Unfavorable visual loss can occur early, and ocular complications of VHL range from exudative retinopathy to tractional retinal detachment, neovascular glaucoma, and phthisis bulbi. Accordingly, various treatment methods have been tested with overall acceptable responses, including photocoagulation, cryotherapy, photodynamic therapy, plaque radiotherapy, vitrectomy, and more novel intravitreal injections of anti-vascular endothelial growth factors and propranolol. |
| first_indexed | 2024-04-12T15:35:37Z |
| format | Article |
| id | doaj.art-77ded26e588a41ed924177b92fed2682 |
| institution | Directory Open Access Journal |
| issn | 2008-322X |
| language | English |
| last_indexed | 2024-04-12T15:35:37Z |
| publishDate | 2020-02-01 |
| publisher | Knowledge E |
| record_format | Article |
| series | Journal of Ophthalmic & Vision Research |
| spelling | doaj.art-77ded26e588a41ed924177b92fed26822022-12-22T03:26:57ZengKnowledge EJournal of Ophthalmic & Vision Research2008-322X2020-02-0115789410.18502/jovr.v15i1.5950Von Hippel-Lindau Disease and the EyeSaeed Karimi0Amir Arabi1Toktam Shahraki2Sare Safi3Ophthalmic Epidemiology Research Center, Shahid Beheshti University of Medical Sciences, Tehran, IranOphthalmic Epidemiology Research Center, Shahid Beheshti University of Medical Sciences, Tehran, IranOphthalmic Epidemiology Research Center, Shahid Beheshti University of Medical Sciences, Tehran, IranOphthalmic Epidemiology Research Center, Shahid Beheshti University of Medical Sciences, Tehran, IranRetinal hemangioblastoma (also referred to as retinal capillary hemangioma) is a benign lesion originating from the endothelial and glial components of the neurosensory retina and optic nerve head. Historically known as a manifestation of the von Hippel-Lindau (VHL) disease, it can be seen as an isolated finding or in association with some rare ocular conditions. In addition to characteristic ophthalmoscopic features, results of numerous ancillary tests including angiography, ultrasound, optical coherence tomography, and genetic tests may support the diagnosis and differentiate it from similar conditions. Because of serious life-threatening complications of VHL disease, every ocular approach to retinal hemangioblastomas should be in relationship with additional multidisciplinary diagnostic and therapeutic efforts. In addition, any patient with actual or probable diagnosis of VHL disease should be screened for ocular involvement. Unfavorable visual loss can occur early, and ocular complications of VHL range from exudative retinopathy to tractional retinal detachment, neovascular glaucoma, and phthisis bulbi. Accordingly, various treatment methods have been tested with overall acceptable responses, including photocoagulation, cryotherapy, photodynamic therapy, plaque radiotherapy, vitrectomy, and more novel intravitreal injections of anti-vascular endothelial growth factors and propranolol.https://doi.org/10.18502/jovr.v15i1.5950diagnosisretinal capillary hemangiomatreatmentvon hippel-lindau |
| spellingShingle | Saeed Karimi Amir Arabi Toktam Shahraki Sare Safi Von Hippel-Lindau Disease and the Eye Journal of Ophthalmic & Vision Research diagnosis retinal capillary hemangioma treatment von hippel-lindau |
| title | Von Hippel-Lindau Disease and the Eye |
| title_full | Von Hippel-Lindau Disease and the Eye |
| title_fullStr | Von Hippel-Lindau Disease and the Eye |
| title_full_unstemmed | Von Hippel-Lindau Disease and the Eye |
| title_short | Von Hippel-Lindau Disease and the Eye |
| title_sort | von hippel lindau disease and the eye |
| topic | diagnosis retinal capillary hemangioma treatment von hippel-lindau |
| url | https://doi.org/10.18502/jovr.v15i1.5950 |
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