von Hippel-Lindau disease is a member of the group of phakomatoses. It is characterized by formation of angiomas or hemangioblastomas in the retina, cerebellum, and occasionally in other tissues. Pheochromocytoma and renal cell carcinoma may also occur. Polycythemia can be seen, usually with cerebellar hemangioblastomas, renal cell carcinomas, or pheochromocytomas.1
The retinal tumors usually enlarge, and exudation from these lesions may lead to retinal detachment. Less commonly, peripheral retinal breaks may occur, leading to rhegmatogenous detachment.2 This may occur after treatment of an angioma.
I have observed a case with several unusual features. Preretinal membranes were present in both eyes, with spontaneous peeling in one eye and peeling after treatment of a peripheral angioma in the other eye. In both eyes, macular breaks resulted. In the treated eye, rhegmatogenous detachment occurred as a result of the macular break, along with traction originating from the treated lesion. The patient
Loewenstein JI. Bilateral Macular Holes in von Hippel-Lindau Disease. Arch Ophthalmol. 1995;113(2):143-144. doi:10.1001/archopht.1995.01100020021016