The pathogenesis of optic nerve drusen. A hypothesis.

Myriads of tiny perivascular drusen were found at histologic examination of the eyes of a 19-year-old patient who had pseudopapilledema and who died of a ruptured intracranial aneurysm. The possibility that they arose from constituents leaking from abnormal blood vessels prompted a review of 53 fluorescein retinal angiograms of patients with optic nerve drusen. As compared to a control group, many of these angiograms revealed the following abnormalities: (1) an abnormal branching pattern on the disc; (2) the presence of relatively large blood vessels connecting the superficial and deep disc circulations; and (3) increased disc capillarity. We believe that the tendency to develop optic nerve drusen results, at least in part, from a congenitally abnormal disc vasculature that allows transudation of plasma proteins that in turn serve as a nidus for the deposition of extracellular materials.