Intrazerebrale Blutung aus einer DVA mit arteriovenösem Shunt: Transitionelle arteriovenöse Malformation

Zusammenfassung

Hintergrund:

Neben den klassischen Formen der zerebralen Gefäßmalformationen—venöses Angiom oder auch DVA (developmental venous anomaly), kavernöses Angiom, kapilläre Teleangiektasie und arteriovenöse Malformation (AVM)—existieren Übergangsformen, die morphologische Kriterien von mehreren Unterformen aufweisen und sich bei existierendem AV-Shunt durch ein erhöhtes Blutungsrisiko auszeichnen.

Fallbeschreibung:

Vorgestellt wird die Kasuistik einer 73-jährigen Patientin, bei der es zu einer intrazerebralen Blutung (ICB) aus einer transitionellen venösen Anomalie gekommen ist. Angiographisch ließen sich Charakteristika einer AVM in Form eines AV-Shunts mit früher Kontrastierung der Zentralvene der DVA, jedoch kein AVM-typischer Nidus nachweisen.

Schlussfolgerung:

Der vorgestellte Fall unterstützt die These anderer Autoren, von denen eine gemeinsame Pathogenese und ein fließender morphologischer Übergang zwischen DVA und AVM postuliert wird, der in den zitierten Fallserien und Einzelfallbeschreibungen—neben histologischen Merkmalen—wie auch in unserem Fall im häufigen Nachweis von ICBs aus transitionellen AVMs oder DVA-assoziierten AVMs zum Ausdruck kommt.

Abstract

Background:

In addition to the four classic types of cerebral vascular malformations introduced by McCormick—arteriovenous malformation (AVM), cavernous malformation, capillary teleangiectases and venous angioma (or developmental venous anomaly, DVA)—a number of transitions or mixed patterns have been recognized by different authors. Apart from the more common coexistence of venous angioma and cavernous malformation, there have been descriptions of combined AVM and venous angioma or venous angioma that exhibit arteriovenous shunting angiographically. These are usually referred to as mixed angiomas, arterialized venous angioma, hybrid angioma, transitional AVM or as venous angioma with pericapillary AVM.

Case Report:

We report a case of a 73-year-old woman who presented to our hospital after a right-sided cerebellar hemorrhage that clinically resulted in hemiataxia, dysphasia and instability of gait with a tendency to fall to the right side. Thorough neurological workup also revealed a hemianopia to the left that had previously not been noticed by the patient or her relatives but had been present for an uncertain period prior to the recent event. Imaging studies, CT and MRI, revealed a subacute hemorrhage in the right cerebellar hemisphere and a posthemorrhagic defect in the right parietooccipital region (Figure 1). Adjacent to this posthemorrhagic defect, contrast enhanced studies showed the presence of a large draining vein, suggesting a venous angioma. This was proven by angiography (Figure 2) which not only demonstrated the venous angioma itself but also showed evidence of arteriovenous shunting with opacification of the draining central vein in the arterial angiographic phase (Figure 2 [1a/b, 2a/b]). This finding led to the assumption that the previous parietooccipital hemorrhage was due to AV-shunting through the transitional AVM that could be demonstrated angiographically.

Conclusion:

In our opinion, the present case demonstrates a morphologic transition between two of the classic cerebral vascular malformations and thereby supports the hypothesis that venous angiomas (or DVA) and AVMs, despite their morphological differences, may have a similar pathogenesis. DVAs with an arteriovenous shunt carry the risk of an intracerebral hemorrhage.