Intraventricular cerebral cavernomas: a series of 12 patients and review of the literature

Abstract

Object: Intraventricular cavernomas (IVCs) occur in only 2-10% of patients with cerebral cavernomas. Reports concerning IVC are scarce and are limited mostly to sporadic case reports. In this paper, the authors present a series of 12 patients with IVCs that were treated at a single neurosurgical department. In addition, the authors reviewed the literature.

Methods: All clinical data were analyzed retrospectively. Follow-up questionnaires were sent to all patients. Outcome was assessed using the Glasgow Outcome Scale. The authors also conducted a PubMed search and found 77 cases of IVC.

Results: The patients' median age was 47 years, and the male/female ratio was 2:1. A cavernoma occurred in the lateral ventricle in 6 patients, in another 5 it was in the fourth ventricle, and 1 had a lesion in the third ventricle. Almost all patients presented with acute headache on admission and in more than half, the symptoms were related to cavernoma bleeding. In total, 8 rebleedings occurred in 5 patients during a median of 0.4 years. Three patients with a cavernoma of the fourth ventricle presented with a cranial nerve deficit. In 8 cases, a cavernoma was surgically treated an average of 1.3 years after the diagnosis. Only 1 patient underwent surgery in the acute phase after a major intraventricular/intracerebral hemorrhage. The median follow-up time was 2 years. No patient was lost to follow-up, and no patient died. In total, on follow-up 9 patients improved and 3 had a persistent neurological deficit, of which 2 existed before surgery.

Conclusions: In the present series, the IVCs had a high tendency for rehemorrhage. Surgery is advocated when hemorrhages are frequent, and the mass effect causes progressive neurological deficits. Microsurgical removal of the IVC is safe, but in the fourth ventricle it can carry increased risk for cranial nerve deficits.