| von-Hippel Disease |
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- 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.
- Management:
- Laser photocoagulation therapy.
- Cryotherapy.
- Diathermy.
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