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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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181Dural Sinus Thrombosis and Infratentorial Pial Reflux
Fig. 3.55A–D. A 2-year-old boy presented with a VGAM with macrocrania.The child
was referred to us at the age of 7 years. He already had severe mental retardation.
A Initial angiography demonstrated a choroidal type of VGAM. B Consecutive an-
giography (during embolization sessions) demonstrated the progressive occlusion of
the jugular bulbs with the reflux into the cortical veins and progressive cerebral congestion. The child was embolized twice with significant improvement in clinical
status.At 10years of age, he still had a score of 1. C,D Note the tonsillar prolapse and
the perimedullary veins on angiography
3Vein of Galen Aneurysmal Malformation182
Fig. 3.56A–D. Legend see p.183
183Dural Sinus Thrombosis and Infratentorial Pial Reflux
Fig. 3.56A–G. Neonatal diagnosis of VGAM. A male neonate weighting 3,310 g presented with cardiac failure responding to medical treatment.He was referred to us at
3months.A–D He had a mural type of VGAM. Some jugular stenosis can already be
seen. D, E He developed progressive occlusion of the remainder of the jugular foramen as well as asymptomatic tonsillar prolapse.F, G Embolization led to complete oc-
clusion of the malformation.At that time, some degree of reopening of one sigmoid
sinus was noted. Six years later, MRI showed complete occlusion of the lesion. There
is partial shrinkage of the mass and resolution of the tonsillar prolapse
syringomyelia on 10-year follow-up (Apsimon 1993; see Fig. 2.28). It is
most important to understand that the prolapse is not related to global intracranial hypertension,even if the mechanism that creates it is also able to
produce supratentorial hydrodynamic failure (Fig. 3.57). Therefore, evidencing a prolapse does not indicate emergency ventricular shunting, but
rather embolization to diminish the relative importance of the VGAM
drainage in the overall venous pathways.In fact,progressive ventricular enlargement is usually not associated with tonsillar prolapse.In addition,this
anomaly does not occur at the neonatal age (see Chap. 2, this volume); it is
not related to the volume of the VGAM ectasia (Fig. 3.58; see also Fig. 3.59),
but to the venous changes that have occurred over time and to their effect
on water dynamics. It also does not represent an associated Chiari-1 malformation.On the contrary,some secondary or associated Chiari-1 malformations might very well have the same physiopathological explanation as
some skull base craniostenoses. The significance of tonsillar prolapse in
VGAM is the same as in CAVMs or dural AV shunts (see Chaps. 4, 5, 7, this
volume) and develop in the same age group.Jugular bulb occlusion occurring after the age of 5 years would not have the same hydrovenous consequences as those described above. Yet congestion of posterior fossa veins
will lead to brain dysfunction and/or hemorrhages supporting an active
endovascular approach (Fig. 3.60) (Scheme 3.1F).
3.13.1 Spontaneous Thrombosis
Spontaneous thrombosis of the VGAM is rare (Fig. 3.61). In our experience, 2.5% of patients showed spontaneous thrombosis, but only half of
them are neurologically normal, which is less than what proper treat-
3Vein of Galen Aneurysmal Malformation184
Fig. 3.57A,B. Unusual severe supratentorial melting-brain syndrome predominantly
affecting the occipital lobes and tonsillar prolapse. Both express the hydrovenous
constraints exerted and their morphological consequences (A, B)
ment can now accomplish.In addition, this thrombosis is mostly unpredictable, although the tentorial edge compression of the arterial feeders
together with the secondary intraluminal thrombosis in the stenosed
draining veins might be an indication of such a development. In any
event, this thrombosis tends to occur late, when cerebral damage may
already be irreversible.It is possible to make a retrospective diagnosis of
a completely excluded VGAM by recognition of the persistent embryonic arrangement of the deep cerebral veins. Spontaneous thrombosis
should not be considered as a favorable outcome, and expecting it to occur is not a therapeutic strategy and now constitutes an unacceptable
choice.
185Spontaneous Thrombosis
Fig. 3.58A–C. A 6-month-old boy with VGAM diagnosed because of macrocrania.
A He was shunted at the age of 8 months, with a significant increase in the vein of
Galen size. The patient was referred to us at the age of 10 months. BThe malformation
was excluded in one session. One year later, a follow-up angiogram confirmed complete occlusion of the lesion and MRI demonstrated shrinkage of the mass. C Note the
persisting subependymal atrophy and the absence of tonsillar prolapse despite the
size of the supratentorial ectasia prior to its embolization
3Vein of Galen Aneurysmal Malformation186
Fig. 3.59A–C. Legend see p. 187
187Spontaneous Thrombosis
Fig. 3.59. A Male infant with
a VGAM presented with significant macrocrania.Ventricular
shunting was performed shortly
after diagnosis. Facial collateral
circulation and moderate mental retardation were observed.
The child was referred to us at
the age of 2 years.Embolization
of the malformation was performed in one session. B, C Immediately after embolization,
a significant reduction in the
flow was noted,with whirling
phenomena inside the pouch
and complete stagnation.
D–F Following embolization,
he was kept asleep for 1day.
It took him 5 days to wake up.
Note the fluid level within the
pouch.F Complete shrinkage
of the mass was observed at
1-year follow-up.At the age of
13, the child had a score of 5
3Vein of Galen Aneurysmal Malformation188
Fig. 3.60A–D. Legend see p.189
189Spontaneous Thrombosis
Fig. 3.60. A, B A child who was a few months old with a well-tolerated VGAM was referred to radiation therapy and lost for follow-up. He was referred to us at the age of
10 years with rapidly progressive brain stem symptoms related to venous congestion
secondary to outlet restrictions (C, D).Angiographic aspects (E, F)
Scheme 3.1F. Infratentorial disorders
Neonate Infant Child <5 Years Child >5 Years
Congestive
cardiac
failure
Multiorgan
failure
Encephalomalacia
Decrease in head
circumference
Infratentorial pial
reflux and congestion
Optimal therapeutic window
F
Macrocrania
hydrocephalus
Hydrodynamic
Dural venous thrombosis
disorders
(sigmoid s., jugular bulb
To ns i llar prolapse
Hydromyelia or
syringomyelia
Neurocognitive
delay
Subependymal atrophy
(pseudo ventriculomegaly)
calcifications (chronic venous
ischaemia)
Convulsions
Neurological deficits
Cerebro-meningeal
haemorrhages
Epilepsy
Neurological
deficits
3Vein of Galen Aneurysmal Malformation190
Fig. 3.61. A–B „Spontaneous“ occlusion ofVGAM revealed by macrocrania with ventriculomegaly shunted at 7months of age. C, D Follow-up angiography performed at
1year demonstrates complete occlusion of the VGAM with calcification of the wall of
the pouch. The deep venous drainage pattern of the brain confirms the diagnosis.
Arrows,deep venous drainage,and arrowhead,torcular stump,in D
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