Control #: 1261 Poster#: EP - 40 Altered Hemodynamic of Developmental Venous Anomalies with Unusual Appearance Angel Mironov MD, PhD Alegent Creighton University Medical Center Omaha, Nebraska

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Background While the developmental venous anomalies (DVA) are widely considered congenital segmental variations in the venous drainage pathway, there is unclear and controversial emphasis regarding the often association with cavernous malformations, the underlying venous hypertension as causative factor in associated hemorrhage & ischemia, as well the imaging parenchymal abnormalities and clinical characteristic

Purpose The pathophysiologic patterns attributed to DVA have been reflected in recently reports discussing perfusion abnormalities like as increased or decreased mean transit time (MTT), cerebral blood fluid (CBF) and cerebral blood volume (CBV) The purpose is to present three cases with peculiar patterns and completely divergent hemodynamic performance like representing three major groups of DVA with distinct patterns selected from a large data base, aiming to address natural history & possible pathogeneses of DVA especially with associated cavernous malformation (CM)

Material and Methods We analyzed the drainage patterns of 205 patients with DVA (62.4% association with CM) identified by retrospective & prospective studies and confirmed by MR & CT & catheter brain angiography Especially the venous patterns & angiographic circulation time of catheter angiography were addressed. The exclusion criterion was an association with other vascular brain lesions

Case 1 45 year-old female Presented with intermittent headaches for last 3 years MRI T2 shows DVA left frontal with probably centrifugal distribution

A B C D

E F G H

Sequential catheter angiography Case 1 Sequential catheter angiography A – D: already early arterial phases show parenchymal opacity due to increased number of enlarged medullary veins converging to large centripetal directed vein merging the anterior part of superior sagittal sinus E – F: late venous phases show timely drainage of the medullary veins in the area of DVA compare to the normal parietal & occipital brain G – H: oblique views show abnormal collecting vein with patent perfusion without visible stenotic deformities

Case 2 42 year-old male Presented with stroke-like sudden ataxia & altered mental status T2 T2 diffusion MRI shows segmental restricted diffusion in the distribution of the right superior cortical area

A B C D

Sequential catheter angiography A – D: Case 2 Sequential catheter angiography A – D: Already early arterial phases show parenchymal opacity due to increased number of early opacification of enlarged medullary veins draining to dysplastic collecting vein into a short tentorial venous sinus. The angiographic circulation time appears to be normal The patterns are consistent with DVM of right cerebellum with centrifugal distribution

E F G H

Selective angiography of the right superior cerebellar artery E – F: Case 2 Selective angiography of the right superior cerebellar artery E – F: Arterial and early venous AP views show intense early arterial opacity of medullary veins and dysplastic veins converging into dysplastic collecting vein to a short tentorial sinus G – H: Lateral views of early and late venous phase show dysplastic appearance of the medullary veins with timely drainage without signs of circulation delay

I J K L

5 months follow up I – K: Sequential catheter angiography Case 2 5 months follow up I – K: Sequential catheter angiography There is completely normal presentation of the angiomorphology with normal circulation time. The DVA does not appear more. There is no visualization of abnormal medullary veins and no abnormal collecting vein. The short tentorial sinus in the area shows the same shape L: MRI There is chronic evolution of primary lesion with vasogenic edema showing now segmental cortical atrophy

Case 3 58 year-old female Presented with slowly progressive trigeminal neuralgia and progressive ataxia for many years MRI shows extreme shrinking of the right cerebellar hemisphere with extensive uptake of hemosiderin and inhomogeneous performance of the central areas The findings are consistent with a giant cavernous malformation associated with marked right cerebellar atrophy

A B C D

Sequential catheter angiography – venous phase A – B: Case 3 Sequential catheter angiography – venous phase A – B: Sequential early venous phases show progressive increasing parenchymal opacity due to delay of venous drainage in the right cerebellar hemisphere. A large collecting vein shows a late and distinct delayed opacity compare to the other venous circulation C – D (oblique): Sequential late venous phases show a further increasing opacity of the collecting vein. There is definitely evidence of impressive venous stasis of dysplastic “medusa” & enlarged collecting vein due to focal stenosis close to the short tentorial sinus The patterns are consistent with DVA of right cerebellum with centrifugal distribution & distinct focal stenosis of collecting vein at the level of merging into a short tentorial sinus

Results The blood flow patterns in sequential catheter angiographies show distinct values of relative hemodynamic characteristics : Case 1: increased relative CBV increased relative CBF normal relative MTT Case 2: increased relative CBV normal relative MTT & drainage obstruction Case #: increased relative CBV increased relative MTT & high grade drainage stenosis

Discussion The hemodynamic analysis of sequential catheter angiographies of 205 patients with DVA show three major groups of relative hemodynamic patterns with many transitional appearances, but relatively constant characteristic: Group 1: There is early opacity of the medullary vein and resp. collecting vein due to increased relatively CBF and CBV, but normal circulation time of the venous phase without delay of the venous circulation (often mistaken as AV shunts of mixed lesions). This DVAs do not harbor stenosis of the collecting veins and do not show compromise of venous drainage in the area (case 1)

Discussion continued Group 2: There is early opacity of dysplastic medullary veins related to increased relative CBV and CBF; there are also different grades of stenotic deformities of the drainage, but normal circulation time. The clinical evolution may lied to different scenarios of vascular events – hemorrhage, infarction, or even some partial occlusion with partial or definite self improvement (case 2) Group 3: There is well definite stenosis of the collecting vein with delay of the venous drainage and consequent delayed circulation through the DVA or increased relative MTT. This type of DVA may be associated with CM like as causative consequence (case 3)

Conclusion The patterns of perfusion abnormalities determined in sequential brain angiography may suggest a complex DVA pathogenesis evolving primary as well the venous angioarchitecture as inducing a secondary developmental disorder of arterial morphology While an in uterus thrombosis of the developing venous system may constitute the distinct DVA (as considered), there is a second simultaneous or late consequent pathogenetic circle which may reflect an induced dilatation of arterioles, attributed causally to a respond to the primary venous restriction

In non complicated DVA there is normal mean transit time (case 1) Conclusion continued While the mechanism of a consequent arterial dilatation remains unclear, this effect may explain the inherently associated increasing blood pool consistent with increased angiographic circulation which corresponds to the increased CBV and CBF, but preserved normal MTT In non complicated DVA there is normal mean transit time (case 1) The development of stenosis along the peculiar drainage will harbor an increasing resistance with delay of the MTT Depending on morphologic venous capacity the induced venous hypertension may be compensated (asymptomatic DVA, case 1) or compromised (propensity toward infarction, bleeding or creation of CM, case 2, 3)

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