| 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
- 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
- Differential diagnoses include: Coat's disease, sickle-cell retinopathy,
racemose retinopathy, retinoblastoma, and malignant melanoma.
- Laser photocoagulation therapy.
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