The relation between angioarchitectural factors of developmental venous anomaly and concomitant sporadic cavernous malformation

Abstract

BackgroundPast studies found that cerebral developmental venous anomaly (DVA) is often concurrent with cavernous malformation (CM). But the reason of the concurrency remains unknown. The purpose of this study was to confirm whether angioarchitectural factors relate to the concurrence and which angioarchitectural factors can induce the concurrency.MethodsDVA cases were selected from the records of the same 3.0 T MR. The DVA cases was divided into two group which are DVA group and DVA concurrent with CM group. 8 angioarchitectural factors of the DVAs were selected and measured. Statistical analysis was performed by the Pearson chi-square statistic,analysis of variance (ANOVA) and multi-factor logistic regression analysis.ResultsFive hundred three DVA lesions were found and 76 CM lesions coexisting with DVA. In the single factor analysis, all the 8 angioarchitectural factors of DVA were related to the concurrency. In the multivariate analysis, 6 angioarchitectural factors. Result of multi-factor logistic regression analysis is Logit(P) = -4.858-0.932(Location) + 1.616(Direction) + 1.757(Torsion) + 0.237(Number) + 2.119(Stenosis rate of medullary vein)-0.015(Angle), goodness of fit is 90.1 %.ConclusionsThe angioarchitectural factors of DVA are related to the concurrency of DVA and CM. 6 angioarchitectural factors may induce the concurrency.

Cerebral cavernous malformations do not fall in the spectrum of PIK3CA-related overgrowth

“…Clinical data obtained from other studies showed that DVAs form dilated, deep medullary veins with caput-medusae formations 90. MRI data obtained from other studies also showed that when DVAs coexist with CCMs, both lesions are connected,88 91 92 reaffirming their close relationship and possible shared signalling pathways. Other studies have reported that when both CCMs and DVAs coexist in the same individual, the haemorrhagic risk increases, 33%–48%,75 93 raising the possibility that CCM signalling cascades merge with PI3K signalling pathways from which DVAs originate.…”

“…However, additional data indicates that the haemodynamic effects of DVAs may be the actual trigger for the initial formation of CCMs 79. Perfusion data generated from a haemodynamic study in DVAs indicated that the restrictive effects on the venous drainage caused by DVAs are sufficient to cause local venous hypertension, leading to CCM formation, or even the propensity of these flanking vessels (or CCM lesions) to bleed, suggesting that initiation of CCMs or occurrence of haemorrhagic CCMs is a consequence of angioarchitectural factors of DVAs 79 88 94 95. These findings raise another possibility that the coexistence of DVAs influence the haemorrhagic occurrence in CCM lesions in which elevated blood pool in the surrounding brain parenchyma as well as increased local venous blood pressure may be key factors for the haemorrhagic events in CCM lesions since CCMs without DVAs tend to bleed at the same frequency.…”

“…It has been long noticed that both CCMs and DVAs coexist in the same individual,83 or that de novo formation of CCMs may occur in the presence of pre-existing DVAs 84. The observed increased systemic or local venous pressure generated by DVAs85–87 may lead to the subsequent formation of CCMs88 with approximately 12% of DVAs having concurrent CCMs 89. Clinical data obtained from other studies showed that DVAs form dilated, deep medullary veins with caput-medusae formations 90.…”

“…Efforts to identify the angioarchitectural factors of DVAs as a trigger for CCM lesion formation in sporadic cases have been made 88. However, additional data indicates that the haemodynamic effects of DVAs may be the actual trigger for the initial formation of CCMs 79.…”

Distant Recurrence of a Cerebral Cavernous Malformation in the Vicinity of a Developmental Venous Anomaly: Case Report of Local Oxy-Inflammatory Events

“…Conversely, mutations in the three known CCM genes were rarely detected in sCCM lesions, which instead resulted associated mainly with somatic mutations in other genes [ 10 , 11 , 12 ], suggesting that fCCM and sCCM lesions may have a distinct genetic origin. Moreover, patients with fCCM often present multiple cerebral cavernomas (up to several hundreds), which are typically not associated with a DVA [ 13 ], while patients with sCCM commonly present a single lesion of various sizes (from mm to several cm) that is frequently associated with a DVA, suggesting the possibility of a different developmental mechanism [ 13 , 14 , 15 , 16 , 17 ].…”