Multisystem
& progressive disease with cysts formation
Kidney
enlargement with other organ involvement (liver∼ 80%, pancreas
7-36%, spleen)
Intracranial
aneurysms in ∼6% of pts without family history & ∼20%
with a family history (rupture in 65-75%, usually before age 50)
Cardiac
Valve abnormalities in 25-30%
