Abstract
Background: The best strategy to perform follow-up of patients with multiple cerebral cavernous malformations (mCCM) is unclear due to the unpredictable clinical course. Still, serial radiological follow-up is often performed. The objective of this work was to critically question whether active follow-up by serial imaging is justified and has an impact on clinical decision making.Methods: We included all consecutive patients with mCCM treated and followed at our Department between 2006 and 2016. Patient data were collected and analyzed retrospectively.Results: From a total number of 406 patients with CCM, n = 73 [18.0%; mean age at first diagnosis 45.2 years (±2.4 SE); n = 42 male (57.5 %)] were found to harbor multiple lesions (≤5 CCM in 58.9%; 6–25 in 21.9%; ≥ 25 in 19.2%). All of them were followed for a mean of 6.8 years (±0.85 SE). Conservative treatment was suggested in 43 patients over the complete follow-up period. Thirty patients underwent surgical extirpation of at least one CCM lesion. Forty-three surgical procedures were performed in total. During 500.5 follow-up years in total, routinely performed follow-up MRI in asymptomatic patients lead to an indication for surgery in only two occasions and even those two were questionable surgical indications.Conclusion: Routinely performed follow-up MRI in asymptomatic patients with mCCM is highly questionable as there is no evidence for therapeutic relevance.
Highlights
cerebral cavernous malformations (CCM) are the most common vascular malformations in the brain with an incidence of ∼0.4–0.6% [1, 2]
Routinely performed follow-up MRI in asymptomatic patients with multiple cerebral cavernous malformations (mCCM) is highly questionable as there is no evidence for therapeutic relevance
Since no data exists in the literature on followup strategies of patient with mCCM, the objective of this study was to critically question whether active follow-up by serial imaging has an impact on clinical decision making in patients with mCCM and can prevent CCM hemorrhage in the further course
Summary
CCM are the most common vascular malformations in the brain with an incidence of ∼0.4–0.6% [1, 2]. The principle danger inherent to CCM is the hemorrhage risk that has been estimated around 0.6–2% per lesion-year or 4.3–13% per patient-year in prospective studies with FCCM [3,4,5]. Whereas in other vascular malformations (i.e., aneurysms, arteriovenous malformations) grading systems have been established to estimate the risk of intracerebral hemorrhage (ICH), so far no grading system exists in the literature to serve as a valuable aid in the clinical decision as to whether perform conservative or surgical treatment in patients with CCM [8,9,10]. The best strategy to perform follow-up of patients with multiple cerebral cavernous malformations (mCCM) is unclear due to the unpredictable clinical course. The objective of this work was to critically question whether active follow-up by serial imaging is justified and has an impact on clinical decision making
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