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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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In the Bicêtre series, the symptoms tended to progress fairly quickly,
requiring investigation and hospitalization. The most frequent clinical
symptoms noted at admission were cardiac insufficiency (46.3%), neurological deficit (34.1%), macrocrania (31.7%), and epilepsy (29.3%)
(Tables 4.8, 4.12).Among the 52 pediatric patients with CAVFs, there were
14 with posterior fossa AVFs (Figs. 4.18, 4.19).In this series,the diagnosis
was not made at prenatal or neonatal age. Eight cases (8/14, 57%) were
diagnosed in infancy and six out of 14 (43%) in children under 12 years
of age. The mean age at diagnosis was 3.5 years. The male:female ratio
was 9:5 (Table 4.10). Revealing symptoms were macrocrania (28%),
hemorrhage (22%), headache (22%), nonhemorrhagic neurological
deficit (14%), and cardiac overload (14%) (Table 4.12).
261Presentation
Fig. 4.17A–F. A 3-month-old baby presented with two convulsions and a mild postictal right-sided deficit. T1 W MRI in sagittal views (A, B) demonstrated multiple flow
voids along the medial aspect left frontal-parietal cortex (arrow, A) and evidence of
hemorrhagic infarction deep within the left frontal lobe (arrow, B).A LIC angiogram
in lateral view (C) showed two distinct high-flow shunting zones (arrowheads, aster-
isks) as well as a third slow-flow shunt (arrow). Delayed venous opacification of the
left frontal lobe is noted on the lateral view LIC (D). Embolization was done a few days
later and completed in three further sessions. Two and a half years later,the lesion is
almost completely occluded, as shown on the LIC angiogram in lateral views (E, F).
The neurocognitive evaluation was normal at that time and the right-sided deficit had
almost disappeared
▲

4Cerebral Arteriovenous Fistulas262
Fig. 4.18A–D. A 6-year-old boy presented with grade II subarachnoid hemorrhage
without focal neurological deficit. The T1 W MRI axial view (A) revealed a large flow
void along the lateral aspect of the right cerebellar hemisphere.Vertebral angiogram
in lateral view demonstrated an AVF from the right PICA, which was embolized with
glue in one session with excellent outcome, as shown on follow-up vertebral angiogram in lateral views (C, D)

263Presentation
Fig. 4.19A–E. Legend see p. 264

4Cerebral Arteriovenous Fistulas264
Fig. 4.19A–G. An 11-year-old girl with a family history of HHT suffered from occasional epistaxis and migraines. Neurological examination revealed moderate left
spasticity and nystagmus. Cranial auscultation revealed a systolic bruit that was not
perceived by the patient.T1 W MRI in sagittal view (A) showed a large flow void along
the posterior aspect of the left cerebellum and a small rounded flow void (arrows)
near the vein of Galen region.Vertebral angiogram, lateral views (B–D),demonstrate
a large AVF arising from the posterior inferior cerebellar artery (arrow,B).Embolization with glue resulted in complete occlusion of the lesion,as shown on the follow-up
MRI T1 W sagittal view (E) performed several weeks later, demonstrating increased
signal within the previous venous pouch (asterisk,E).Postembolization right (F) and
left vertebral (G) angiograms in AP views confirmed closure of the AVF.The child has
become neurologically normal on follow-up

4.4.1 Natural History
The natural history of CAVF after presentation remains mostly speculative, as in our experience we have rarely found such a lesion in a neonate
or infant– incidentally or with minor symptoms– that we were able to
follow and that continued to be well tolerated without treatment. On the
other hand, in particular with posterior fossa locations and depending on
the age at diagnosis, their tolerance in young children can be surprising,
when one considers their size and flow.
As was mentioned in Chap. 2 of this volume,this would suggest a spectrum of natural history rather then a single one. In other words, the natural history after presentation is likely determined by the individual host
response to the presence of the AVF, systemically (heart, liver, kidney,
etc.), regionally (brain, dural sinus, CSF, etc.), and the decompensation
that may occur at the level of the AVF itself (hemorrhage, etc.). The in
utero demonstration of CAVF is rare and by itself does not have any clinical implications. Noninvasive imaging by ultrasound or MRI may, however, demonstrate evidence in utero or at neonatal age of regional,hemispheric, or diffuse cerebral malacia. Such findings in our experience are
not reversible,despite curative treatment,and in fact are a contraindication to active treatment when extensive encephalomalacia is present, as
the natural history under those circumstances is extremely poor
(Figs. 4.20–4.23).
The reason for the comparatively low frequency of CAVFs in adults
would suggest either their early expression,their spontaneous (asymptomatic) thrombosis, the early progression toward symptomatology, or
even incompatibility with survival.From our combined experience,there
is little evidence to suggest the occurrence of spontaneous asymptomatic
thrombosis and we therefore favor the postulate that CAVFs have a natural history that is highly age- and host-specific and unlikely to be tolerated for a long time.
265Natural History
Fig. 4.20A,B. T2 W MRI in
coronal (A) and sagittal (B)
views in a 2-week-old girl
demonstrates right-sided
cingular gyrus AVF draining
into the deep venous system
and associated with hemispheric atrophy

4Cerebral Arteriovenous Fistulas266
Fig. 4.21A–C. A baby girl presenting as a neonate with acute
heart failure related to a CAVF.
Digitalis and diuretic treatment
was given and improved the
clinical situation. Ultrasound,
computed tomography,and angiography (not shown) led to
diagnosis of a sylvian AVF,and
embolization was attempted
three times at another institution.Because of these technical
difficulties, the child was transferred at 1 year of age to our
institution. Heart failure was
under control but developmental delay of about 3 months had
occurred as well as a right
hemiparesis. MRI was performed (A) and showed
hemispheric atrophy. LICA
angiogram in lateral views
(B, C) demonstrated high-flow
AVF from left MCA with venous
congestion affecting the left
hemisphere.An endovascular
procedure was performed with
difficulty and partially occluded
the shunt.The remaining lesion
was subsequently surgically
resected but the child died
shortly afterward

267Natural History
Fig. 4.22A–C. Prenatal diagnosis. Neonate presented in
acute heart failure,which was
difficult to manage,as well as
macrocrania and an intracranial bruit. There was systemic
manifestation of hepatic and
renal failure.CT revealed large,
intracerebral venous pouches
associated with ventricular
dilatation and subcortical
leukoencephalomalacia.
The child was in severe neurological distress. The true nature
(pial or dural) of the lesion
could not be assessed.
The baby died rapidly

4Cerebral Arteriovenous Fistulas268
Fig. 4.23A–F. Legend see p. 269

269Natural History
Fig. 4.23A–K. Color flow Doppler examination (A) demonstrated evidence of large
AV shunt at posterior fossa level at 36 weeks gestation in utero. MRI examination at
5days of age showed, on sagittal (B) and axial views (C, D), evidence of a very large
flow void at lower aspect posterior fossa as well as second lesion along right sylvian
region. Cerebral atrophy was already present at that time, while the child clinically
was in moderate to severe heart failure. Vertebral angiogram demonstrated in AP
views (E,F) evidence of large AVF fed by right PICA. Selective PICA angiogram in AP
(G) and lateral (H) views confirmed AVF prior to obliteration with coils (long arrow),
as shown on postembolization vertebral angiograms in AP (I) and lateral (J) views.
Additionally, a small AVF was shown fed by the right MCA (small arrow). Follow-up
MRI 1 week later in axial view (K) demonstrates closure of the AVFs as well as progressive cerebral atrophy.The baby progressively clinically worsened and died 1week
later
▲

4.5 Management
The indications, the time,and the method of treatment chosen for management of CAVFs depend on a variety of factors such as the age at presentation,presenting symptoms,the status of the brain, and the status of
the other organs. Treatment may be contraindicated because of already
existing severe brain damage: melting brain syndrome (cortical or
subependymal) and/or diffuse parenchymal calcifications (Fig. 4.23).
These can be noted either at the time of diagnosis or can develop rapidly
between the initial consultation and the actual admission of the child for
treatment.This was the case in 7% of patients with supratentorial CAVFs
(Weon et al. 2005).
Tr eatment objectives in neonates and infants are the same as in VGAM;
however,the insult to the cerebral tissue is more rapid,as the drainage occurs to subpial veins in most instances. Because of the poor neurological
prognosis, one should attempt to achieve total occlusion or a significant
reduction of the shunting through the AVF (more than 75%) more rapidly (Figs. 4.24–4.26). Early management is dictated by the danger of brain
impairment, and less so by the congestive heart failure, which is usually
mild. Patients should be evaluated clinically, by noninvasive imaging, in
order to assess the degree of interference between the AVF and the brain
tissue. Angiography is done with intent to treat, planning the first
embolization session,almost regardless of the existing symptoms.
If congestive heart failure is severe, the prognosis is extremely poor,
since it combines both systemic and subpial effects. Possible hydrodynamic disorders should be managed early through reduction or occlusion
4Cerebral Arteriovenous Fistulas270
Fig. 4.24A,B. Legend see p. 271
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