von-Hippel Disease

Benign retinal or optic disc capillary hemangioma that may present as unilateral or bilateral and single or multiple tumors.
When multiple, bilateral retinal tumors associated with central nervous system and other organ involvement are found, the condition is referred to as von Hippel-Lindau disease.

Clinical Features

Ophthalmoscopic appearance: grayish or orange-reddish peripheral retinal or optic disc capillary tumors of various sizes.
Early finding may be appreciable as slightly dilated retinal arteriole and venule, which show fine tortuosity feeding the tumor.
As the tumor progresses, large, dilated, tortuous feeding and draining vessels may extend all the way to the disc and may be easily recognizable as the clue to peripheral retinal vascular tumors.
Larger tumors commonly associated with intraretinal or subretinal exudation, which may be adjacent to the tumor margin or may be remote from the tumor (stellate exudation of the macula).
Fluorescein angiography: the tumor is fluorescent within seconds. It remains fluorescent and leaks dye into the vitreous in the late phase.
Differential diagnoses include: Coat's disease, sickle-cell retinopathy, racemose retinopathy, retinoblastoma, and malignant melanoma.

A case of von-Hippel disease (capillary hemangioma) involving the optic disc:

Usually the afferent and efferent vessels are not apparent
Can be complicated with:
- Lipid exudation or hard exudates in the macular area
- Exudative retinal detachment
- Vitreous hemorrhage
- The overlying vitreous changes with fibrovascular tissue formation can be a source of traction and result in tractional or rhegmatogenous retinal detachment