Cavernous malformations associated with dural arteriovenous shunts in the central nervous system

Abstract

Introduction: Some cerebral cavernous malformations (CCMs) may be associated with vascular malformations that occur on the capillary-venous side of the cerebral circulation. The association between CCMs and dural arteriovenous shunts (DAVSs) is not well known. The purpose of this study is to investigate the relationship between CCMs and DAVSs.

Methods: We retrospectively analyzed 179 patients diagnosed as intracranial DAVSs and performed with digital subtraction angiography (DSA). The DSA images were investigated for the location, cortical venous reflux (CVR), multiplicity, and treatment method/result of DAVS. MR images were investigated for the location, number, and size of CCMs and correlated with the DSA images.

Results: Six of the 179 patients with DAVSs presented with coexistent CCMs (3.4 %; M/F = 3:3; mean age, 38 ± 22 years). Five of the six DAVS patients with CCMs were associated with CVR (83 %). The total number of CCMs associated with DAVS was 20. Multiple CCMs (range, 2-7) were seen in four (66 %) of six patients. Eighteen (90 %) of the 20 CCMs were located on the ipsilateral hemisphere of the CVR (n = 10) or adjacent to the deep venous reflux (n = 8). Five de novo CCMs were detected in two patients. All de novo CCMs were located on the ipsilateral hemisphere of the DAVS with CVR or juxtapositioned to abnormally dilated deep vein.

Conclusion: CCMs may develop in association to DAVSs. The relationship between CCMs and DAVSs suggests the venous pathogenic origin of CCMs and the role of venous hypertension in the de novo development of CCMs.