Comparative studies of the diagnosis and treatment of cerebral cavernous malformations in adults: systematic review

Abstract

Background: Cerebral cavernous malformation (CCM) management decisions are usually made after CCM diagnosis is suspected or definitively diagnosed on axial imaging by indirectly comparing a surgeon's estimate of operative morbidity and mortality against published estimates of CCM untreated clinical course.

Methods: We used comprehensive electronic strategies to search OVID Medline and EMBASE for original studies published before 2011 of ≥20 adults with CCM that (a) evaluated diagnostic test accuracy, or (b) compared treatment with microsurgery or stereotactic radiosurgery against conservative management in a concurrent or historical control group and reported clinical outcome(s). We used the Grading of Recommendations Assessment, Development and Evaluation (GRADE) Working Group's approach to identify level 1 or level 2 studies according to the Oxford Centre for Evidence-Based Medicine's 2011 criteria.

Results: We found one eligible diagnostic test accuracy study of 72 patients with brain masses accompanied by vasogenic edema and substantial amounts of blood, which found that hyperintense perilesional signal on T1-weighted magnetic resonance imaging could differentiate CCM from other causes with excellent specificity (98 %) and reasonable sensitivity (62 %). We found five potentially eligible observational studies of adults with a CCM that had already bled, but none met level 2 criteria for a "dramatic" effect (the conventionally calculated probability of the two groups of observations coming from the same population should be less than 0.01 and a rate ratio greater than 10). We found 11 potentially eligible observational studies of adults with CCM and epilepsy, but nine studies did not demonstrate dramatic effects and the remaining two studies showed dramatic effects, but they were at high risk of bias.

Conclusions: To address the absence of level 1 or 2 evidence to support CCM treatment decisions, there is a need for large studies of CCM treatment with a concurrent control group, ideally with randomized treatment allocation.