Von hippel-lindau syndrome (retinocerebral angiomatosis; angiomatosis retinae; cerebelloretinal hemangioblastomatosis; lindau syndrome; retinal capillary hamartoma) 1

General: Dominant inheritance; angiomata in the cerebellum and the walls of the fourth ventricle; young adults.

Ocular: Secondary glaucoma; angiomatosis of the iris; vitreous hemorrhages; tortuosity of dilated retinal artery and vein (feeder vessels); retinal exudates and hemorrhages; retinitis proliferans; angiomata of optic nerve and retina; papilledema; retinal detachment; lipid accumulation in macula; keratoconus; bilateral macular holes; choroid plexus papilloma; bilateral optic nerve hemangioblastomas.

Clinical: Cerebellar angiomatosis; epilepsy; psychic disturbances to dementia.

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