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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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201Infants and Children
Fig. 3.63A–F. Prenatal diagnosis of VGAM at 34 weeks of gestation. The baby was
born by caesarian section weighing 3,300 g and was macrocephalic with a head circumference of 36 cm (more than 2 SD). Cardiac failure responded to medication,with
a neonatal score at 16. A Because of the instability of the response to medical treat-
ment and the severity of the macrocrania, embolization was performed at 3 months
and 4 months.B Complete occlusion of the malformation was achieved.C–F One year
later, complete occlusion was confirmed,but the child had severe mental retardation,
indicating underestimated consequences of the early hydrovenous disorders despite
the absence of true hydrocephalus.Note the pseudo-phlebitic appearance of the cortical cerebral veins bilaterally. D–E see p. 202
3Vein of Galen Aneurysmal Malformation202
Fig. 3.63D–F. Legend see p. 201
3.15 Technical Management
3.15.1 General Remarks
We favor the transarterial femoral approach to deliver glue in situ as the
first treatment modality in every case. In some rare cases, we have had to
perform the femoral puncture with the help of Doppler ultrasound. The
smallest baby that was embolized weighed 2.0 kg.No cut-down has been
necessary in our experience. We try to obtain complete exclusion in the
lowest number of sessions, but this desire is primarily guided by the
clinical stability observed in the infant.On one occasion,we failed to embolize a young infant as we did not achieve a safe catheter a safe position
and he died shortly thereafter.
An average of 2.4 sessions per child is needed to obtain the expected
therapeutic goal. The venous route was used in 5% of cases when it became impossible to achieve effective embolization via the arterial route
or to specifically disconnect a sinus reflux in order to protect the brain.In
each case, the child was in a clinical condition that required immediate
treatment; no deliberate attempt was made to completely exclude the
VGAM drainage considering the hemorrhagic risk related to the sudden
congestion of nonvisualized subependymal anastomoses.
The largest arterial contributors to the lesion should be embolized
first.The microcatheters to be used are a combination of flow-guided and
over-the-wire devices, their size depends upon the flow of the feeders to
be excluded and the agent selected. In high-flow lesions, one of us (PL)
uses the Baltacci P1,8 (Balt Extrusion, Montmorency, France) specially
designed for high-flow lesions in babies. This microcatheter is used directly through the 4-F sheath, without a guiding catheter; therefore, no
co-axial system is used in 90% of neonatal or infant cases.Catheterization
is usually rapid,since these devices can straighten the vertebral and internal carotid cervical skull base kinks. Microcatheters that are too soft are
often unstable during fast injection of pure glue, while the over-the-wire
microcatheters tend to be stiff or kink in these loopy vessels. The occlusion of the VGAM can often be obtained in one or two sessions; however,
the purpose is not the fastest time to obtain 100% exclusion of the lesion
but rather the safest reduction that guarantees normal cerebral maturation and neurocognitive development (Table 3.5).
Whenever the occlusion of the malformation of the Galen vein is complete or almost complete, we recommend keeping neonates and infants
under general anesthesia for the next 24 h in the ICU. This protocol has
been used since the beginning of our experience in order to avoid the unnecessary agitation of a baby awakening.Babies are woken up the following day. There are no specific postoperative measures taken in the management of most of these patients, except for in the situation mentioned
above; in particular, no heparin or steroids are utilized; blood pressure is
kept at a normal level while the child is asleep in the ICU.
Endovascular treatment sessions are arranged every 3–6 months
depending on the clinical status and response to the embolization.
Femoral punctures should be avoided in the ICU as should scalp or
jugular venous lines.
203General Remarks
The technical aspects of VGAM embolization at Bicêtre Hospital are summarized below:
General anesthesia
Femoral puncture 20-gauge Teflon needle. In tiny patients, arterial
access may be a significant obstacle,which may require catheterization
of the umbilical artery shortly after birth (Berenstein et al. 1997) or the
assistance of Doppler ultrasound to localize and cannulate the femoral
artery
A 4-F sheath (Terumo),6 ml contrast/kg pure contrast for runs,diluted
at 50% for fluoroscopic control)
A4-F thin wall guiding catheter,if necessary
Pure NBCA + tantalum powder + Lipiodol (for slow-flow shunts)
Intraoperative blood pressure 70 mm systolic or lower if possible at the
time of high-flow fistula embolization
One to three angiographic runs in neonates 3 ml/s, for a total of 6 ml
(vertebral Towne’s projection or biplane when possible, followed by
lateral view of both internal carotid arteries, ICA). If one posterior
cerebral artery is not seen on the first run, choose the corresponding
ICA side on the second run,and the opposite one for expected cerebral
venous information
No additional arterial line
No heparinis used
Alternate femoral puncture side at each session
Usual length of procedure is 45 min, maximum length of procedure,
2h
Recovery room (a few hours)
Pediatric ICU (24 h GA) if the occlusion incomplete or almost com-
plete (secondary thrombosis expected)
No induced low blood pressure, but controlled blood pressure
(nicardipine if high blood pressure)
Pediatric neurology ward
3Vein of Galen Aneurysmal Malformation204
Ta ble 3.5. Bicêtre Admission and Outcome Score
a
Score Condition
5Normal (N)
4Minimal non-neurological symptoms (MS),not treated and/or
asymptomatic enlargement of the cardiac silhouette
3Transient neurological symptoms (TNS),not treated and/or asymptomatic
cardiac overload with treatment
2Permanent minor neurological symptoms (MNS),mental retardation of up
to 20%; nonpermanent neurological symptoms with treatment; normal
school with support and/or cardiac failure stabilized with treatment
1Severe neurological symptoms,mental retardation of more than 20%
(SNS); specialized school and/ or cardiac failure unstable despite treatment
0 Death (D)
a
Does not apply to neonates.
3.15.2 Follow-Up
From our experience with the use of bucrylate in CAVM patients, we
consider results to be stable at 6 months to 1 year following the final embolization. We have not observed revascularization at later follow-up when
angiographic evaluation at 6months to 1 year was completely normal.
When slight hyperemia is demonstrated at 6months, even without
evidence of AV shunting, another follow-up angiogram is taken 1 and
2years later.Presently,we do not rely on the immediate postembolization
angiographic appearance. Magnetic resonance angiography (MRA) is
likely to satisfactorily document the stability of a complete occlusion.
During follow-up, all children are clinically evaluated by the referring
physicians or the pediatric neurologists. Clinical assessment is based on
neurocognitive examination. After treatment is completed, children are
followed up with a clinical examination every year and MRI every 2 years
(Fig. 3.64). This management has created a population of children that
did not exist 20 years ago. This ongoing clinical follow-up is therefore
mandatory; in the pediatric population, therapeutic success can only be
truly evaluated when brain maturation is complete and functionally evaluated over time.
Our series of 317 VGAM patients (Table 3.6) include the cases referred
and those for whom embolization was not indicated to overcome the
clinical situation or predictable outcome (Tables 3.7, 3.8)
To t al exclusion of a VGAM is a clear and simple observation,as demonstrated by a repeated negative angiogram (Fig. 3.64). Total or nearly total
obliteration of the lesion is already obtained in 55% of the children who
were embolized and alive (Tables 3.9, 3.10),which means that a significant
number of children do not have an eradicated VGAM. In 97% of the patients in whom the treatment of the lesion was considered complete,total
exclusion of the shunt was obtained and confirmed. In only 3% was the exclusion incomplete, but the persisting remaining shunt is less of a risk in
comparison to the technical difficulty in completely obliterating it.
In many instances, complete disappearance is not achieved at the end
of embolization,and some slow flow inside the lesion can still be demonstrated (Fig. 3.65). We have never seen any rupture of the VGAM under
these circumstances.
Among the children partially treated, with the exception of the complications described below, none developed any permanent neurological
symptoms that they did not have before, e.g., seizure, deficit, and hemorrhage. As will be seen with CAVFs and CAVms, staging and progressive
exclusions of the active portions of the lesion make it possible to follow
over time incompletely embolized lesions until complete exclusion is
deemed feasible and necessary; the presence of subependymal supply is
not a contraindication to embolization as soon as they have shown no
regression following the highest flow shunts (Figs. 3.66,3.67).
Among the goals to reach is the presence of a faint subependymal reflux
in an incompletely excluded lesion.Spontaneous thrombosis of the pouch
could lead to hemorrhagic risk in the thalamic region (similar to what is
observed following transvenous approach and packing of the venous ectasia distal to these anastomoses) (Fig. 3.68). Partial targeted sessions or
deposition should be directed to the control of this reflux (Fig. 3.69).
205Follow-Up
3Vein of Galen Aneurysmal Malformation206
Fig. 3.64A–C. Legend see p. 207
207Follow-Up
Fig. 3.64A–F. Male neonate presenting with cardiac failure and rapidly progressing
macrocrania initially related to a small VGAM.A Following ventricular shunting, the
size of the lesion dramatically increased. The child came to us at 10 months of age and
mild mental retardation was noted. Tonsillar prolapse was also noted.B–D In two ses-
sions of embolization, 1 week apart, complete occlusion of the shunt was achieved.
One year later, complete occlusion was confirmed. E The supply from the basilar tip
branches and, in particular,the choroidal and subependymal arteries regressed spontaneously.F At 8 years of follow-up, the child had a score of 5
Ta ble 3.6. VGAM:patients (1981–2002)
Age at diagnosis Age at first consultation
Fetus 93 (29.3%) 18 (5.7%)
Neonates (<1 month) 119 (37.5%) 122 (38.5%)
Infants (>1 month <2 years) 82 (25.9%) 125 (39.4%)
Children (2–16 years) 23 (7.3%) 52 (16.4%)
To tal 317 317
Ta ble 3.7. VGAM therapeutic decision and proposed treatment
Embolization Abstention Lost to follow-up Total
Neonates 88 (5)
a
45 7 140
Infants 103 (8) 16 6 125
Children 42 (4) 6 4 52
To tal 233 (17) 67 17 317
To tal (%) 73.5% 21.1% 5.4%
a
Numbers in parentheses denote embolization done elsewhere.
3Vein of Galen Aneurysmal Malformation208
Ta ble 3.8. Reasons for therapeutic abstention
Neonates Infants Children
Therapeutic abstention 45 Therapeutic abstention 16 Therapeutic abstention 6
Encephalomalacia 25 (56%) Encephalomalacia 9 (56%) Bicêtre Admission Score 1 3 (50%)
NN score <8
a
17 (38%) Technical failure 1 (6%) Surgery 1 (17%)
Therapeutic interruption 3 (6%) Spontaneous occlusion 6 (38%) Spontaneous occlusion 2 (33%)
of pregnancy
a
Score 8: four patients; score 7:six patients; score 6: three patients; score 5:four patients.
Ta ble 3.9. VGAM therapeutic results 1981–2002: patients referred for management
To tal Death Alive
Neonates 23 12 11
Infants 153 11 142
Children 40 – 40
To tal 216 23 193
a
a
Seven patients with angiography without embolization and five patients with
angiography and spontaneous occlusion.
Ta b l e 3.10. VGAM therapeutic results 1981–2002: morphological results (surviving
children)
a
100% 82
95% 8
90% 16
±50% 75
<50% 12
To tal 193
a
55% of patients have a 90%–100% occlusion; 38.5% of patients have a 50%–90%
occlusion;6.2% of patients have a <50% occlusion.
209Follow-Up
Fig. 3.65A–E. A Male infant presented with macrocrania at the end of neonatal age.
He was referred for consultation at 10months. He had a score of 4 at that time. B, C
The lesion was embolized in two sessions.An immediate response of the head circumference was observed; however, a minimal shunt was still seen on the wall of the
VGAM.C Note the presence of nonocclusive glue inside the sigmoid sinus.D, E Finally, 2years after the last embolization, the shunt finally disappeared, both sigmoid sinuses opened in patent jugular bulbs. E see p. 210
3.15.3 Complications: Morbidity
There are non-neurological complications related to the embolization
procedure and the technical difficulty of injecting pure NBCA glue in
6.7% of cases. On three occasions,a drop of glue caused an asymptomatic
partial occlusion of the internal iliac artery during removal of the
catheter. In all patients, follow-up showed progressive reconstitution of
the vessel lumen; in one additional patient, the catheter became glued in
place, resulting in a homonymous hemianopsia from which he recovered
at 3 years of follow-up. In this situation, the catheter should be cut as
short as possible at the femoral entrance and be pushed forward to float
in the aorta. The catheter rapidly became extraluminal and incorporated
in the vessel wall (Fig. 3.70). It has not led to any thrombotic or embolic
manifestations in our experience, nor has it required preventive anticoagulation treatment. In an additional patient, a drop of glue remained at
the tip of the catheter and could not be passed through the sheath
(Fig. 3.71). Despite the small size of the drop, it occluded the distal aorta
of a 4.6-kg baby girl. The microcatheter tip and its attached glue were
pushed into a standby position in the suprarenal aorta. Immediate
surgery by transperitoneal approach to the aortic bifurcation was performed. After gentle pulling on the catheter to bring the tip and the glue
droplet under direct visual control, a small aortotomy was done; the
microcatheter was cut and its tip removed directly (Fig. 3.72).After completion of the direct surgery,the sheath was removed and the postoperative course was uneventful. The aorta and the femoral artery demonstrated no detectable anomaly at angiographic follow-up performed during a
3Vein of Galen Aneurysmal Malformation210
Fig. 3.65E. Legend see p. 209
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