The neurovascular triad: mixed cavernous, capillary, and venous malformations of the brainstem

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Object

The four types of cerebrovascular malformations may sometimes be combined and more often occur in pairs; triads are exceptional. The authors present six patients with the clinicoradiographic profile of mixed vascular malformations of the brainstem, including cavernous malformation (CM), capillary telangiectasia, and developmental venous anomaly (DVA).

Methods

Five patients (one of whom was a child) suffered from hemorrhage, suggesting that this complex association has a high bleeding potential. Progressive growth, rebleeding, and de novo occurrence of the associated CM were documented in three cases. Magnetic resonance imaging of the brain was obtained in all patients by using one or more of the following modalities: T1-weighted sequences before and after gadolinium administration; T2-weighted sequences; T2-weighted fluid attenuated inversion recovery; T1-weighted fast spin echo; and diffusion weighted, diffusion tensor, and perfusion imaging in three cases.

Results

Three patients were surgically treated with the intention of excising the hemorrhagic lesion, but only two patients had their malformations successfully removed. In the third case, diffuse pontine telangiectasia precluded the safe excision of the CM. Histological examination demonstrated a blended pathological milieu characterized by coalescent telangiectasia and venules associated with loculated endothelial chambers resembling an immature or de novo CM. Three patients were treated conservatively; recurrent minor hemorrhage occurred in one case. The authors found these malformations to be arranged in two basic relationships: CM inside the telangiectasia and CM in the radicles of the DVA. Stenosis of the main venous collector and dilation of the medullary veins were important findings.

Conclusions

The pathogenesis of this malformation may be referred to a developmental deviance of the brainstem capillary–venous network associated with transitional vessels and loculated endothelial vascular spaces related to genetic and acquired origins, probably in a restrictive venous outflow milieu.

Abbreviations used in this paper:CM = cavernous malformation; CT = computed tomography; DVA = developmental venous anomaly; FSE = fast spin echo; MR = magnetic resonance.

Article Information

Address correspondence to: Eugenio Pozzati, M.D., Department of Neurosurgery, Bellaria Hospital, Bologna, Italy 40139. email: eugenio.pozzati@ausl.bo.it.

© AANS, except where prohibited by US copyright law.

Headings

Figures

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    Case 1. Axial FSE T1-weighted MR images obtained without (a) and with (b) contrast medium. The hemorrhagic lesion in the right middle cerebellar peduncle is not enhanced by gadolinium, whereas the capillary telangiectasia and the DVA were highly enhanced, without deformation of the pons profile. Axial FSE (c) and fluid attenuated inversion recovery (d) T2-weighted MR images demonstrating a round hemorrhagic lesion in the subacute phase, a faint venous drainage anomaly, and, only on T2-weighted images, a hypointense pontine lesion (e) consistent with a telangiectasia. Postoperative T1-weighted MR image (f) with addition of contrast medium demonstrating total removal of the hemorrhagic lesion with preservation of the DVA and of the telangiectasia.

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    Case 1. Photomicrographs using endothelial cell marker CD 31. A: Loculated assemblage of irregular vascular spaces (arrows) lined by endothelial cells, with budding of new sinusoids resembling a cavernomatous structure. B: Telangiectasia characterized by capillary vessels interspersed in the brain parenchyma. C: Aggregates of endothelial channels and venous vessels with incomplete elastic wall (arrow). Van Gieson staining for elastic fibers, original magnification × 50.

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    Case 4. Axial CT scan (a) demonstrating a calcification in the pons. Axial fluid attenuated inversion recovery T1-weighted (b) and FSE T2-weighted (c) MR imaging studies demonstrating gradations of hypointensity due to hemosiderin deposition consistent with a CM around the calcification. Axial (d) and coronal (f) FSE T1-weighted MR images obtained after addition of gadolinium showing the CM is surrounded by telangiectasia. In this case the telangiectasia is slightly visible also in an FSE T2-weighted sequence obtained without contrast injection. The effect of the hemosiderin is increased on the coronal T2-weighted sequence (e). The telangiectasia is evident on T1-weighted sequences after gadolinium administration. Venous caput medusae arises from the DVA and drains into the lateral recess (g).

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    Case 4. Left: Follow-up MR image obtained at 12 months after initial presentation confirming the typical radiographic findings of the three vascular malformations. Right: Same examination, lower section, demonstrating enlarged venous radicles in the fourth ventricle.

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    Case 5. Sagittal (left) and coronal (right) FSE T1-weighted MR images obtained after gadolinium administration revealing a CM surrounded by a capillary telangiectasia and DVA, with multiple drainages through the pons.

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    Case 6. Postoperative sagittal T1-weighted MR image obtained after contrast administration revealing focal stenosis of the main venous collector (arrow) draining into the superior petrosal sinus through the cerebellopontine cistern.

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    Case 6. Left: Postoperative sagittal MR image obtained after contrast administration demonstrating the faint peripheral radicles of the DVA. Right: After 1 year, progressive dilation of the terminal radicles of the DVA and a hypointense lesion (arrow) consistent with a de novo CM are evident.

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