Vein of Galen aneurysmal malformations: critical analysis of the literature with proposal of a new classification system

A review

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Vein of Galen aneurysmal malformations are a rare and diverse group of entities with a complex anatomy, pathophysiology, and serious clinical sequelae. Due to their complexity, there is no uniform treatment paradigm. Furthermore, treatment itself entails the risk of serious complication. Offering the best treatment option is dependent on an understanding of the aberrant anatomy and pathophysiology of these entities, and tailored therapy is recommended. Herein, the authors review the current concepts related to vein of Galen aneurysmal malformations and suggest a new classification system excluding mesodiencephalic plexiform intrinsic arteriovenous malformations from this group of malformations.

Abbreviations used in this paper:AV = arteriovenous; AVF = AV fistula; AVM = AV malformation; VGAM = vein of Galen aneurysmal malformation.

Article Information

Address correspondence to: Martin M. Mortazavi, M.D., Division of Neurosurgery, University of Alabama at Birmingham, 1530 3rd Ave. S., Birmingham, AL 35209. email: m_mortazavi@hotmail.com.

Please include this information when citing this paper: published online July 26, 2013; DOI: 10.3171/2013.5.PEDS12587.

© AANS, except where prohibited by US copyright law.

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Figures

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    Illustration of a VGAM. Note pericallosal feeding vessel overlying the corpus callosum and entering the posterior aspect of the malformation. Also, note the thalamus in the anterior aspect of the malformation. The P1 segment with its feeders is seen superior to the superior cerebellar arteries. The P1 feeders, along with the P2 and P3 feeders, enter the malformation. This illustrates a Type I or choroidal type according to the Lasjaunias classification because the feeders enter the malformation at its anteroposterior aspect. According to Yaşargil's classification it is a Type III malformation because there is a mixture of feeders from both pericallosal/P3 and P1–2 segments. Note the enlarged straight sinus and torcula posterior to the malformation. (Illustration from Microneurosurgery, Volume IIIB by M. G. Yaşargil, published by Thieme Verlag in 1988. Reprinted with permission.)

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    Schematic illustration of the development of a VGAM and its tributaries. A: Note the normal anastomosis between the posterior branches of the pericallosal arteries and distal branches of the posterior cerebral arteries (PCA) during fetal life. B: In VGAMs, those anastomoses degenerate and end up in the enlarged and malformed median prosencephalic vein of Markowski (MPV), traditionally called a VGAM. Associated malformations are stenosis, fenestration, duplication, or absence of straight sinus (SS), and decrease or absence of torcula (T). Sometimes there is an aberrant presence of falcine sinus (FS) and an accessory torcula (AT).

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    Angiograms. A: Note the stenotic straight sinus (arrow) with poststenotic dilation of the torcula (T). The transverse sinus (TS) is visible. The arrowheads show P1 feeders. B: Duplicated straight sinus. C: Absence of straight sinus, with enlarged parietooccipital veins acting as an accessory straight sinus. Accessory torcula seen. Note the curved arrow showing the reflux anteriorly toward the internal cerebral vein. D: Stenotic left transverse sinus (arrow). (Figures from Microneurosurgery, Volume IIIB by M. G. Yaşargil, published by Thieme Verlag in 1988. Reprinted with permission.)

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    Lasjaunias types of malformation. A: Type I. Choroidal type with the AVF at the anterior aspect of the malformation. The arrow indicates the distal pericallosal feeder to the dilated vein of Galen (VG). The straight sinus is also seen. B: Type II. Mural type with the AVF appearing in the inferolateral aspect of the malformation. The arterial feeders are from the P3 segments (arrows). (Figures from Microneurosurgery, Volume IIIB by M. G. Yaşargil, published by Thieme Verlag in 1988. Reprinted with permission.)

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    Yaşargil Type I malformation. A: Note the arterial feeders from the pericallosal and P3 segment to the engorged vein of Galen. The thalamoperforating vessels have no fistulous connection to the malformation. The question mark illustrates possible anterior drainage through the internal cerebral and atrial veins. B: Lateral carotid angiogram showing a vein of Galen aneurysmal malformation (VG) with pericallosal feeder (arrow). The straight sinus is visible. Shading designates the corpus callosum (also in Figs. 610). (Illustration and figure from Microneurosurgery, Volume IIIB by M. G. Yaşargil, published by Thieme Verlag in 1988. Reprinted with permission.)

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    Yaşargil Type II malformation. A: Note arterial feeders only from the P1 segment along with the thalamoperforators. Drainage occurs both through the aneurysmally enlarged vein of Galen posteriorly and through the internal cerebral and atrial veins anteriorly. B: Vertebral angiogram showing P1 feeders (arrowheads). Also note stenotic straight sinus (arrow). (Illustration and figure from Microneurosurgery, Volume IIIB by M. G. Yaşargil, published by Thieme Verlag in 1988. Reprinted with permission.)

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    Yaşargil Type III malformation. A: Note feeders, both from the pericallosal and P3 segments, and from the P1 segment and thalamoperforators. Drainage is through the dilated vein of Galen as well as anteriorly through internal cerebral and atrial veins, which remain unvisualized. B: Note P3 feeders (single arrows) and P1 feeders (double arrows). Note the enlarged straight sinus; the torcula and transverse sinus are also seen. (Illustration and figure from Microneurosurgery, Volume IIIB by M. G. Yaşargil, published by Thieme Verlag in 1988. Reprinted with permission.)

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    Yaşargil Type IVA malformation—thalamic AVM. A: Note P1 feeders to the thalamic AVM. Note the venous drainage through the dilated vein of Galen. B: Lateral vertebral angiogram showing a thalamic AVM with venous drainage through the dilated vein of Galen. Note the duplicated straight sinus. (Illustration and figure from Microneurosurgery, Volume IIIB by M. G. Yaşargil, published by Thieme Verlag in 1988. Reprinted with permission.)

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    Yaşargil Type IVB malformation—mesencephalic AVM. A: Note the mesencephalic AVM with venous drainage through the vein of Galen. B: Lateral angiogram showing a mesencephalic AVM with venous drainage through the vein of Galen. (Illustration and figure from Microneurosurgery, Volume IIIB by M. G. Yaşargil, published by Thieme Verlag in 1988. Reprinted with permission.)

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    Yaşargil Type IVC malformation—mesodiencephalic AVM. A: Note the feeders from posterior communicating artery and P1 segments, and drainage to the dilated vein of Galen. There is also a separate fistulous connection from the pericallosal and P3 segments to the dilated vein of Galen. B: Note thalamomesencephalic AVM as well as a separate fistulous connection between the distal pericallosal artery and the vein of Galen. The internal cerebral vein is usually dilated. (Figure and original illustration by P. Roth in Microneurosurgery, Volume IIIB by M. G. Yaşargil, published by Thieme Verlag in 1988. Illustration modified by C. J. Griessenauer and M. M. Mortazavi. Reprinted with permission.)

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    Angiogram showing dilated septal and internal cerebral veins and vein of Galen secondary to an adjacent cingulocallosal AVM. Note the difference in the shape of the mildly dilated vein of Galen because that vessel itself is not involved, in contrast to a Yaşargil Type IV malformation. (Courtesy of Dr. Laughlin Dawes, Prince of Wales Hospital, Randwick, Australia.)

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    Diagram showing the treatment paradigm. Transarterial embolization has emerged as the most widely used treatment modality.

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    Illustrations of surgical technique and pre- and postoperative angiograms obtained in a case of successful surgery of a Yaşargil Type I malformation. A: Lateral view of the relationship between the malformation and its arterial tributaries, the falx, and the tentorium. The black line indicates the surgical window. The dotted line indicates the location for the falcine incision superior to the straight sinus to gain access to the contralateral side. B: Craniotomy showing the malformation. Note the falcine incision enabling access to the contralateral side. C: Preoperative angiogram demonstrating a Yaşargil Type I malformation with its P3 feeders (arrows). D: Postoperative vertebral angiogram showing resolution of the malformation. Note that the P3 perforators are intact and only their fistulous connections with the malformation are occluded. The patient was neurologically intact and stable on follow-up. Surgery was performed by M. G. Yaşargil. (Illustrations and figures from Microneurosurgery, Volume IIIB by M. G. Yaşargil, published by Thieme Verlag in 1988. Reprinted with permission.)

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