Primary intraventricular hemorrhage (IVH), bleeding in the ventricular system without a discernable parenchymal component, is a rare neurological disorder. To better define the features of primary IVH and the yield of diagnostic angiography in this condition, we retrospectively analyzed all cases of primary IVH evaluated at a tertiary referral hospital over a 6-year period and performed a systematic review of the literature.Methods
For the retrospective case series, all patients with primary IVH admitted to the neurovascular service at a single tertiary referral center over a 6-year period were identified by screening a departmental database. For the systematic review of the literature, all case series of patients with primary IVH diagnosed by computed tomography were identified in the Medline database.Results
From the systematic review, the majority of patients with spontaneous primary IVH presented with headache (69%), nausea/vomiting (53%), and altered mental status (66%). Most primary IVH patients had associated hydrocephalus (62%), and about one-third required ventricular drainage (34%). Diagnostic cerebral angiography was positive for a bleeding source in 56%. The two most common causes identified by angiography were arteriovenous malformations (58% of positive angiograms) and aneurysms (36% of positive angiograms). Approximately one-third of patients with primary IVH do not survive hospital discharge (39%). Patient age and amount of IVH independently predicted in-hospital mortality.Conclusions
Primary IVH is a rare form of intracerebral hemorrhage, with varying short-term outcomes that depend on patient age and the extent of intraventricular hemorrhage. The yield of diagnostic cerebral angiography in the setting of primary IVH is very high. The two most common causes of primary IVH identified on angiography are arteriovenous malformations and aneurysms. Routine catheter angiography in the setting of primary IVH is warranted.