gms | German Medical Science

Objective: The best strategy to perform follow-up of patients with multiple cerebral cavernous malformations (mCCM) is unclear due to the mainly unknown and 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 2006-2016. Patient data were collected and analyzed retrospectively.

Results: From a total consecutive number of 406 patients with CCM, n = 73 (18.0 %; mean age at first diagnosis 45.2 years (± 2.4); 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). Conservative treatment was suggested in 43 patients over the complete follow-up period. 30 clinically symptomatic patients underwent surgical extirpation of at least one CCM lesion. 43 surgical procedures were performed in total. None of the 4 patients with intractable epilepsy stayed seizure free after surgery. During 500.5 follow-up years in total, routine follow-up MRI in asymptomatic patients lead to an indication for surgery in only 2 occasions and even those 2 were questionable surgical indications.

Conclusion: In patients with mCCM, patient education and imaging studies in the setting of new clinical symptoms may be superior to a rigid imaging follow-up schedule. Intractable epilepsy in mCCM patients should not be considered as a clinical symptom for surgical indication.

Figure 1 [Fig. 1], Figure 2[Fig. 2]