Abstract
Retinal capillary hemangiomas are benign vascular tumors of the retina. They can be unifocal, multifocal, unilateral or bilateral, sporadic or inherited. It is heritable as autosomal dominant inheritance when associated with von Hippel-Lindau (VHL) disease. Treatment of retinal capillary hemangioma can be observation, laser photocoagulation, cryotherapy and/or plaque radiotherapy. VHL disease can be associated with central nervous system hemangioma, renal cell carcinoma and visceral tumors. VHL gene is present on chromosome 3p25-26 and suspected individuals can therefore undergo genetic testing and focused systemic surveillance. This chapter describes the ophthalmic and systemic manifestations associated with retinal capillary hemangioma.
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References
Maher ER, Iselius L, Yates JR, et al. Von Hippel-Lindau disease: a genetic study. J Med Genet. 1991;28(7):443–7.
Singh AD, Shields CL, Shields JA. von Hippel-Lindau disease. Surv Ophthalmol. 2001;46(2):117–42.
Singh AD, Rundle PA, Rennie I. Retinal vascular tumors. Ophthalmol Clin N Am. 2005;18(1):167–76. x
Melmon KL, Rosen SW. Lindau’s disease. Review of the literature and study of a large kindred. Am J Med. 1964;36:595–617.
Magee MA, Kroll AJ, Lou PL, Ryan EA. Retinal capillary hemangiomas and von Hippel-Lindau disease. Semin Ophthalmol. 2006;21(3):143–50.
Lonser RR, Glenn GM, Walther M, et al. von Hippel-Lindau disease. The Lancet. 2003;361(9374):2059–67.
Lamiell JM, Salazar FG, Hsia YE. von Hippel-Lindau disease affecting 43 members of a single kindred. Medicine (Baltimore). 1989;68(1):1–29.
Maher ER, Yates JR, Harries R, et al. Clinical features and natural history of von Hippel-Lindau disease. Q J Med. 1990;77(283):1151–63.
Neumann HP, Wiestler OD. Clustering of features of von Hippel-Lindau syndrome: evidence for a complex genetic locus. The Lancet. 1991;337:1052.
Salazar FG, Lamiell JM. Early identification of retinal angiomas in a large kindred von Hippel-Lindau disease. Am J Ophthalmol. 1980;89(4):540–5.
Carr RE, Noble KG. Retinal angiomatosis. Ophthalmology. 1980;87(9):956–9.. 961
Augsburger JJ, Shields JA, Goldberg RE. Classification and management of hereditary retinal angiomas. Int Ophthalmol. 1981;4(1–2):93–106.
Gass JD, Braunstein R. Sessile and exophytic capillary angiomas of the juxtapapillary retina and optic nerve head. Arch Ophthalmol. 1980;98(10):1790–7.
Schmidt D, Natt E, Neumann HP. Long-term results of laser treatment for retinal angiomatosis in von Hippel-Lindau disease. Eur J Med Res. 2000;5(2):47–58.
Webster AR, Maher ER, Moore AT. Clinical characteristics of ocular angiomatosis in von Hippel-Lindau disease and correlation with germline mutation. Arch Ophthalmol. 1999;117(3):371–8.
Vail D. Angiomatosis retinae, eleven years after diathermy coagulation. Am J Ophthalmol. 1958;46(4):525–34.
Haining WM, Zweifach PH. Fluorescein angiography in von Hippel-Lindau disease. Arch Ophthalmol. 1967;78(4):475–9.
Shields CL, Shields JA, De Potter P. Patterns of indocyanine green videoangiography of choroidal tumours. Br J Ophthalmol. 1995;79(3):237–45.
Goes F, Benozzi J. Ultrasonography of haemangioma of the optic disc. Bull Soc Belge Ophtalmol. 1980;190:87–97.
Lieb WE, Shields JA, Cohen SM, et al. Color Doppler imaging in the management of intraocular tumors. Ophthalmology. 1990;97(12):1660–4.
Kremer I, Gilad E, Ben-Sira I. Juxtapapillary exophytic retinal capillary hemangioma treated by yellow krypton (568 nm) laser photocoagulation. Ophthalmic Surg. 1988;19(10):743–7.
Lane CM, Turner G, Gregor ZJ, Bird AC. Laser treatment of retinal angiomatosis. Eye. 1989;3:33–8.
Bonnet M, Garmier G. Treatment of retinal capillary angiomas of von Hippel’s disease. J Fr Ophtalmol. 1984;7(8–9):545–55.
Finger PT, Chin KJ, Yu G-P, Palladium-103 for Choroidal Melanoma Study Group. Risk factors for radiation maculopathy after ophthalmic plaque radiation for choroidal melanoma. Am J Ophthalmol. 2010;149(4):608–15.
Kreusel KM, Bornfeld N, Lommatzsch A, Wessing A, Foerster MH. Ruthenium-106 brachytherapy for peripheral retinal capillary hemangioma. Ophthalmology. 1998;105(8):1386–92.
von Buelow M, Pape S, Hoerauf H. Systemic bevacizumab treatment of a juxtapapillary retinal haemangioma. Acta Ophthalmol Scand. 2007;85(1):114–6.
Slim E, Antoun J, Kourie HR, Schakkal A, Cherfan G. Intravitreal bevacizumab for retinal capillary hemangioblastoma: a case series and literature review. Can J Ophthalmol. 2014;49(5):450–7.
Albiñana V, Escribano RMJ, Soler I, et al. Repurposing propranolol as a drug for the treatment of retinal haemangioblastomas in von Hippel-Lindau disease. Orphanet J Rare Dis. 2017;12(1):122.
Dahr SS, Cusick M, Rodriguez-Coleman H, et al. Intravitreal anti-vascular endothelial growth factor therapy with pegaptanib for advanced von Hippel-Lindau disease of the retina. Retina. 2007;27(2):150–8.
Choyke PL, Glenn GM, Walther MM, Patronas NJ, Linehan WM, Zbar B. von Hippel-Lindau disease: genetic, clinical, and imaging features. Radiology. 1995;194(3):629–42.
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Maheshwari, A., Newman, H., Finger, P.T. (2020). Ocular Von Hippel-Lindau Disease. In: Khetan, V. (eds) Intraocular Tumors. Springer, Singapore. https://doi.org/10.1007/978-981-15-0395-5_10
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