| Identifier |
vascular_malformations_eyes_brain_lee |
| Title |
Vascular Malformations Involving the Eyes and Brain |
| Creator |
Andrew G. Lee, MD; Jae Eun Lee |
| Affiliation |
(AGL) Chairman, Department of Ophthalmology, The Methodist Hospital, Houston, Texas; Professor of Ophthalmology, Weill Cornell Medicine, New York City, New York; (JEL) Class of 2023, Baylor College of Medicine, Houston, Texas |
| Subject |
Hemorrhage; Venous Malformations; Wyburn-Mason Syndrome; Cavernous Malformation; Capillary Malformation |
| Description |
Summary: • Vascular malformations with both intraocular and intracranial presentations o Can include phakomatoses. o Most worrisome and the highest risk for hemorrhage and vascular malformation is the arterial venous malformation o AVM in the eye called the Wyburn-Mason Syndrome. > May look like a spaghetti like tangle of vessels • Cavernous Malformation o Series of endothelial live channels o Less likely to bleed due to lack of arterial feeder o May be in the orbit > Appear as an intraconal mass with variable enhancement • Risks of bleeding stratified o Highest risk: arteriovenous malformation > Due to it being high flow o Cavernous malformation > Low flow o Venous malformation > Very low flow o Capillary malformation > Extremely low flow |
| Transcript |
Today we're just talking about the vascular malformations and the ones that have both intraocular and intracranial presentations including phakomatoses. So of course the most worrisome and the highest risk for hemorrhage and vascular malformation that we see is the arterial venous malformation- the AVM. So the AVM has a feeding artery and then a nidus and then one or more draining veins. So it's an arteriovenous malformations with a nidus rather than a capillary bed. This is a high flow malformation which means it's a very high risk of bleeding, and so if we see that in the brain, we definitely want to look in their eye, and if they have an AVM in their brain, we want to be looking for AVM in the eye, and that disorder is called the Wyburn-Mason Syndrome. In the eye it looks like a spaghetti like a tangle of vessels so it's like the artery and the vein are both dilated and there's no intervening capillary bed. So it looks similar to what you would think AVM looks like in the brain- a tangle of blood vessels artery to vein direct connection. The second malformation that we see in the brain and in the eyes is the cavernous malformation. The cavernous malformation is a series of endothelial live channels, so it's like a single endothelium, and inside is the hemorrhage. And so this is a low flow malformation- it can still bleed, but because it doesn't have the arterial feeder, it's less likely to bleed, and even it does bleed, it's not likely to produce a catastrophic intraparenchymal or intraventricular hemorrhage like an AVM hemorrhage will be localized here. And in fact when you scan most cavernous malformations you can see that there's been bleeding and there's the hemorrhages of different ages-old, new, and intermediate. And so just like with the AVM, which could be in the brain, in the eye, or in fact AVM can occur in the orbit, the cavernous confirmation can occur in your brain and in your eye, and in the eye it looks like clusters of grapes, and the same thing with the brain cavernous malformation different ages of hemorrhage and sclerosis and FA may show the classic cluster of grapes appearance. And the cavernous malformation can be in your orbit. In the orbit it usually looks like an intraconal mass with variable enhancement that can be delayed because it takes time for the contrast material to get inside those cavernous spaces. And this has no name, so as opposed to that eponyms that are used for the other conditions this has no name. And then we've got the venous malformation-the venous malformation in the head is called the developmental venous anomaly or DVA. So it's basically a dilated vein- it's the big vein. It is a low risk but not a no risk for hemorrhage, so AVM highest risk cavernous malformation next and then low risk for the venous malformation. We can see this in the eye too, but it's not a phakomatoses and it doesn't have a name. And then we have the capillary malformation. The capillary telangiectasia. And that can be in your brain as well, and it looks like little tiny spider, because it's the capillary that's dilated telangiectasia. And that same thing can be seen in your eye and that doesn't have a name, and the same thing can be seen in your conjunctiva or your mucosal surfaces. That telangiectasia does have a name- that is Osler Weber Rendu, which is also called hemorrhagic hereditary telangiectasia. So we see that in the conjunctiva and sometimes in the retina and mucosal surfaces. The other telangiectasia condition in the conjunctiva is the ataxia telangiectasia, sometimes called Lewis Barr. So this Lewis Barris the ataxia telangiectasia. And so when you see a vascular malformation in the brain, you should be looking for the same vascular malformation in the eye and in the orbit, and if you have it in your brain and your eye or your orbit, it's much more likely that you should be thinking about a phakomatoses, some of which have names, like Wyburn-Mason, Osler Weber Rendu, and Lewis Barr, and some have no names like the cavernous malformation. And the highest risk for bleeding is the arteriovenous malformation because it's high flow, cavernous malformation low flow, venous malformation very low flow, and capillary malformation extremely low flow. So in order, those are the risks for the hemorrhage with the highest being AVM. |
| Date |
2021-04 |
| Language |
eng |
| Format |
video/mp4 |
| Type |
Image/MovingImage |
| Collection |
Neuro-Ophthalmology Virtual Education Library: Andrew G. Lee Collection: https://novel.utah.edu/Lee/ |
| Publisher |
North American Neuro-Ophthalmology Society |
| Holding Institution |
Spencer S. Eccles Health Sciences Library, University of Utah, 10 N 1900 E SLC, UT 84112-5890 |
| Rights Management |
Copyright 2019. For further information regarding the rights to this collection, please visit: https://NOVEL.utah.edu/about/copyright |
| ARK |
ark:/87278/s6vb45p8 |
| Setname |
ehsl_novel_lee |
| ID |
1680636 |
| Reference URL |
https://collections.lib.utah.edu/ark:/87278/s6vb45p8 |
