gms | German Medical Science

Objective: The role of associated venous malformations (VM), respective adjacent venous angioarchitecture, in the pathomechanism and treatment of cerebral cavernous malformations (CCM) is still discussed controversial. We analyzed high-resolution SWI at 7T in a consecutive series of sporadic CCM in previous work [1] and found different repetitive patterns of associated VM. In this study we analyzed the venous angioarchitecture of multiple CCMs for the first time in high anatomical detail.

Method: 13 patients with symptomatic or asymptomatic multiple (> 3) CCM diagnosed using 1.5 T MRI were additionally scanned using a 7T MRI scanner (Magnetom 7T, Siemens Healthcare, Erlangen, Germany) equipped with a gradient coil (125 cm length) capable of 45 mT/m maximum amplitude and a slew rate of 220 mT/m/ms. Sequence parameters for SWI were the following: TE: 15 sec, TR: 27 sec, in plane resolution 250 µm, slice thickness 1 mm, flip angle 14°. Image data were analysed regarding presence and formation of the CCM associated venous vessel structures. Genetic analysis of underlying germline mutation in all patients is still ongoing. Clinical patient data was assessed according to the reporting standards of CCM.

Results: In the 13 patients (9 women, 4 men, 23–72y, mean age: 49 y) overall 771 CCM lesion were analyzed. In 7 patient generalized CCM lesions (mean n=101), equally distributed within the brain, were found. In these patients no associated VM was depicted. In 2 patients uni- or bilateral generalized venous dysplasia with associated clusters of CCM (mean n=7) were found. In 4 patients local VM with associated clusters of CCM (n=5) were displayed.

Conclusions: In this consecutive group of patients high-resolution SWI revealed three different patterns of multiple CCM with or without associated VM. Image data suggests two different manifestations of multiple CCMs. A generalized form affecting the whole brain with numerous lesions (Fig.1A) and a cluster-type form with fewer CCMs associated with generalized (Fig.1B) or local (Fig. 1C) VM. Genetic analysis is still ongoing.