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Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_3644_Библиотеки_им_академика_М_И_Перельмана
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171Dural Sinus Occlusion and Supratentorial Pial Congestion and Reflux
Fig. 3.49A–E. Legend see p. 172
3Vein of Galen Aneurysmal Malformation172
Fig. 3.49. A, B An 8-year-old boy referred to us because of VGAM and significant facial collateral circulation with bilateral proptosis. C, D Angiography demonstrated a
choroidal type of VGAM with bilateral occlusion of the sigmoid sinus.Reflux from the
superior sagittal sinus into the cortical veins joins the cavernous sinus bilaterally and
subsequently the ophthalmic veins. Several transarterial embolization procedures
were performed. A significant decrease in the flow to the malformation led to a dramatic improvement in the facial circulation. e In order to completely occlude the lesion, a transtorcular approach with balloon occlusion of the exit to the vein of Galen
was used. Following successful placement of the balloon, the child had focal
subependymal vein hemorrhage with a thalamocapsular hematoma, leading to rightsided hemiplegia. Progressive improvement occurred with incomplete recovery before discharge. F–I Late follow-up images demonstrated complete occlusion of the lesion.At 19years of age, the child still presents with residual right-sided deficit
173Dural Sinus Occlusion and Supratentorial Pial Congestion and Reflux
Fig. 3.50. A, B Cerebral venous drainage remodeling. Neonate male presented with
mild cardiac insufficiency and secondary macrocrania from a prenatally diagnosed
choroidal VGAM. Embolized at 6 months,he was clinically normal 7 years later.Bilateral cavernous sinus drainage and the phlebitic aspect of the cortical veins despite
complete exclusion of a VGAM was noted. He presented a seizure at the age of 8 years
in relation to a cortical ischemic venous event. Three-dimensional views of pseudophlebitic venous cortical network (C, D)
venous reflux is then demonstrated, and macrocrania may progress to
hydrocephalus and acute focal or diffuse damage may occur (Fig. 3.51):
seizures, deficit, and hemorrhage.
Under these circumstances, emergency endovascular management
should aim to balance the flow of the AV shunt to the capacity of the posterior outlets and functionally split the VGAM drainage from the normal
cerebral one.Unfortunately,this situation is a transient stage,and its progression to completion of the jugular bulb occlusion will occur if treatment is not undertaken immediately. The worsening of macrocrania at
that stage enhances the triggers for further occlusion.The results of such
urgent embolization should be rapidly clinically detectable and recognizable by the progressive disappearance of the facial venous collateral
circulation and the normal neurological status (Fig. 3.48).
The situation is particularly unstable if the occlusion has occurred and
is bilateral, even if it has been present for a few years. The clinical tolerance is dependent on the capacity of the alternative outlets to drain the
VGAM and the brain. These pathways always exist in infancy or childhood; otherwise the condition would be rapidly fatal, similar to neonatal
dural sinus malformations (see Chap. 7, this volume). The risk of hemorrhage and venous infarction is high, as there is significant pial reflux and
the VGAM has become an AVM draining into the pial venous system
(Fig. 3.49). However, if the occlusion has not developed too quickly, the
child may grow without acute hydrovenous failure and with progressive
adaptation of the various collateral pathways.As the venous system compliance is reduced, transient neurological episodes (seizures or deficits)
may express this fragile equilibrium of the venous drainage of the brain
(Fig. 3.52). The neurological prognosis is still excellent if the treatment is
started before the onset of acute symptoms. The hemodynamic goal
might then be complete exclusion of the VGAM from the cerebral circulation.
3Vein of Galen Aneurysmal Malformation174
Fig. 3.51A,B. Ty pical aspect of papilledema on MRI T2 sequence in a case of VGAM
with severe hydrodynamic disorders
175Dural Sinus Occlusion and Supratentorial Pial Congestion and Reflux
Fig. 3.52A–C. Legend see pp. 177
3Vein of Galen Aneurysmal Malformation176
Fig. 3.52D,E. Legend see p.177
177Dural Sinus Occlusion and Supratentorial Pial Congestion and Reflux
Fig. 3.52. A An 8-month-old boy. VGAM was diagnosed due to rapidly progressing
macrocrania.Angiography demonstrated a small choroidal type of lesion with patent
jugular foramen at 9 months. He was embolized 5 months and 1 year later. Although
he showed no delay in neurological aquisition,at the age of 3 years he had several attacks of right-sided hemiplegia lasting for several hours and resolving spontaneously. B, C Although partial stenosis had previously been seen, follow-up angiography
demonstrated bilateral occlusion of jugular bulbs, retrograde opacification of the superior petrosal sinus, and pial reflux in the temporal vein and posterior fossa bilaterally. D, E Emergency embolization was performed, and the malformation was completely occluded.Two years later,the child was clinically normal. Note the spectacular
stagnation of the veins (F) before treatment and their correction on the follow-up angiogram after embolization (G)
3Vein of Galen Aneurysmal Malformation178
Fig. 3.53. A A 10-year-old boy with MRI evidence of long-term effect of bilateral sigmoid sinus thrombosis with congestion of sinuses and intracranial veins. Note the
spectacular bone hypertrophy corresponding to the recruitment of the transcranial
venolymphatic outlets. B, C Note the dural sinus shunt on the torcular and the sigmoid sinus. Following complete embolization of the remaining malformation, the
dural shunts disappeared. D Different child with prenatal diagnosis of VGAM. The
child presented at birth with cardiac overload responding to medical treatment. Instability in the response led to embolization at 22 days of age. Note the spectacular
transdural supply to the lesion
179Dural Sinus Occlusion and Supratentorial Pial Congestion and Reflux
Fig. 3.54. A, B A baby boy diagnosed at the age of 1 year with macrocrania associated
with a small VGAM. Ventricular shunting was performed, and progressive spontaneous thrombosis of the jugular bulbs was demonstrated. Neurological deficits following ventricular shunting were found,as were seizures and mental retardation. The
child was referred to us at the age of 2 years and embolized.A deficit remains and the
child has a score of 1 at age 5.He also shows some psychotic behavior.C Note on MRI
the various transcerebral collateral circulations bridging the cortical system to the
deep venous system. Striate vein collateral circulation calcification of the region is
clearly demonstrated.D see p. 180
Dural AV shunts may develop within the thrombosed portion of the
sinus (Figs. 3.53,3.54). In our series, they caused no specific clinical symptoms.They disappeared following complete obliteration of the VGAM.
The condition of bilateral jugular bulb occlusion and pial venous reflux
may exist for a few years, and some children with undiag-nosed VGAM
may present late with intracerebral hematomas, or subdural or subarachnoid hemorrhagic events.These are rare examples of hemorrhages seen in
children or young adults with true VGAM. It is of interest to note that,after approximately 5 years of age,only subacute or chronic symptoms tend
to occur, as well as sequelae of already preexisting dysfunction. Failure to
thrive, bone hypertrophy, mental retardation, cerebral calcifications, and
some psychiatric syndromes can be noted in older children or young
adults. Once the optimal therapeutic window has been missed, these
symptoms can no longer be reversed, even if the lesion is obliterated.
3.13 Dural Sinus Thrombosis
and Infratentorial Pial Reflux
The infratentorial consequence of the sinus occlusion is tonsillar prolapse
(see Chap. 1, this volume; Fig.3.55). It is secondary to the cerebellar pial
congestion and only appears in its presence.It may disappear with correction of the AV shunt, provided that the prolapse has not existed for a long
time (Fig. 3.56). It does not create any specific symptoms at this age. The
long-term effects of this condition are unknown, since it is likely that most
children that present with this complication of jugular bulb stenosis are in
the worst natural history group; however, a case of a torcular dural sinus
malformation in a child with a tonsillar prolapse revealed the presence of
3Vein of Galen Aneurysmal Malformation180
Fig. 3.54. (continued)
D There is bilateral occlusion
of the sigmoid sinuses
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