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Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_3644_Библиотеки_им_академика_М_И_Перельмана

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271Management
Fig. 4.24A–F. A male neonate presenting in moderate to severe cardiac failure showed on vertebral angiogram on lateral (A) and AP (B) views a high-flow AVF fed by the superior cerebellar artery, which was embolized with glue when the baby was 23 days old. The child became clinically asymptomatic and follow-up vertebral an­giogram in lateral (C) and AP (D) views at 1year and vertebral angiogram in lateral views at 3 years of age (D, E ) demonstrated progressive thrombosis of the AVF. The child was clinically normal at that time and remains so at 2 years follow-up
4Cerebral Arteriovenous Fistulas272
Fig. 4.25A–D. A 20-month-old boy presenting at 3days of age with cardiac failure that was controlled medically with digitalis and diuretics.Ultrasound and MRI of the
left ICA angiogram on lateral view (A) and vertebral angiogram lateral view (B). Em- bolization was started at 3months of age when the child was referred to us,and after the third session the lesion was almost completely excluded from the circulation. Long-term follow-up internal carotid angiogram in lateral view (C) and vertebral an­giogram on lateral view (D) demonstrated permanent occlusion of the AVF
273Management
Fig. 4.26A–D. Neonate presented at birth with acute heart failure. There was a family history of HHT.CT examination (A,B) demonstrated large vascular structure within the sylvian fissure on the right side, which at carotid angiography on AP (C) and lat­eral (D) views proved to be the venous pouch associated with a AVF. Embolization with glue resulted in greatly diminished flow through the lesion,as shown on carotid angiogram in lateral views before (E) and immediately after embolization (F). The child no longer has heart failure,E,F see p. 274
of the AV shunts (Figs. 4.27, 4.28) unless noninvasive imaging already shows evidence of irreversible diffuse brain damage.
Convulsion is a major symptom and should indicate the need for rapid treatment, even if the convulsion remains isolated and without perma­nent effect.
Although pial lesions in infancy present features that simulate the VGAM pattern, symptoms are significantly different, rarely causing hy­drodynamic disorders, but frequently focal neurological symptoms (Fig. 4.29) and hemorrhage. CAVF rapidly produces local brain atrophy (focal melting-brain syndrome) and not diffuse macrocrania with bilat­eral brain damage,in contrast to VGAM.This atrophy represents the sub­acute local effect of abnormal hydrodynamics induced by surrounding pial venous congestion.Dural sinus hyperpressure (Quisling and Mickle 1989; Zerah et al.1992) can induce reversible tonsillar prolapse (Girard et al. 1994), which expresses the posterior fossa hydrovenous disorders (Andeweg 1989). Special attention must be paid to the venous drainage of the brain at each session in order to follow the maturation of the various outlets and their patency.
Therapeutic decision-making is usually complex in children with mul­tiple AV shunts and should be approached on an individual basis (Iizuka et al. 1992;Willinsky et al.1990a,Yoshida et al.2004; Weon et al.2005). The clinical history in combination with the MRI, computed tomography (CT), and angiographic findings, help determine which lesion or lesions cause symptoms and therefore need prompt treatment.
At the neonatal age,the most prominent shunt should be attacked first, as well as the one that gives rise to the most prominent pial congestion, in order to obtain systemic relief and brain drainage improvement,or if the presentation is a hemorrhage,the lesion that produced the bleed must be treated first.
4Cerebral Arteriovenous Fistulas274
Fig. 4.26E,F. Legend see p. 273
275Management
Fig. 4.27A–D. Neonate presented at birth with heart failure (A) and progressive macrocrania. An intracranial bruit was heard at auscultation (B). Tonsillar prolapse was noted on MRI T1 W sagittal view (C). Carotid angiogram on lateral view (D) pri­or to staged embolizations with glue demonstrated a frontal AVF draining into the ectatic sylvian and subtemporal veins (short arrows). E–G see p. 276
4Cerebral Arteriovenous Fistulas276
Fig. 4.27E–G. (continued) At 9years of follow-up, carotid angiogram on lateral (E, F) and AP (G) views demonstrated near complete obliteration of the AVF (long arrow) with incomplete remodeling of the middle cerebral artery trunk (short arrow)
277Management
Fig. 4.28A–D. AP views of vertebral artery (A, B) in a 4-month-old girl demonstrate single-hole AVF. Embolization with pure glue obliterated the AVF, as confirmed by postembolization vertebral angiogram on lateral views (C, D) (same case as Fig.4.1)
4Cerebral Arteriovenous Fistulas278
Fig. 4.29A–D. A 3-year-old girl presented with severe headache and multiple neuro­logical deficits (right IIIrd nerve palsy, left facial weakness, and left motor weakness of the leg). MRI T2 W in sagittal view (A) and MRA (B) showed a large flow void at the interpeduncular cistern level.V-P shunt was performed because of acute ventricular enlargement.Three weeks later vertebral angiography in AP view (C,D) demonstrat­ed an AVF, fed from the right superior cerebellar artery (SCA) and draining into the vein of Galen. E–L see p.279
279Management
Fig. 4.29E–I. (continued) The feeder was thought to be too short to allow for a safe injection of glue without risk of reflux into the parent arterial trunk, and coils were placed in the fistula,reducing the flow by 90%,as shown on se­lective superior cerebellar (E) and postembolization vertebral angiogram (F). Follow-up MRI T1 W sagittal (G), axial (H), and MRA (I) showed com­plete closure of the lesion. Resolution of the neurological deficit occurred over the next few months and complete clinical recovery was noted at 1 year of follow-up
Regarding the management of asymptomatic CAVFs, the therapeutic decision is controversial. it has been suggested that no treatment and simply follow-up is a reasonable option in asymptomatic patients.In con­trast, we recommend the active treatment to close the AVF because of the poorer prognosis of conservatively managed patients.Based on our expe­rience and the high mortality of untreated patients reported in the litera­ture (Nelson et al.1992; five deaths in eight patients who were conserva­tively managed), we highly recommend active intervention.
Tr ea t me n t options to achieve this goal are embolization or open surgery (Nelson et al. 1992; Tada et al. 1986) (Table4.13); there is a very limited role for radiosurgery in older children. Both surgery and em­bolization have proven equally effective in the cure of CAVF.However, we prefer the endovascular approach, since it avoids craniotomy with its concurrent morbidity and reduces the hospitalization time.
In 11% of cases, treatment of the supratentorial CAVFs was performed within the first 30 days of life, 51% within the first 2 years, and the re­mainder between 2 and 15 years of life (Weon et al.2005).This was signif­icantly earlier than the infratentorial CAVFs, which had a median age of
3.5 years (range 5months to 12 years) when first treated.
While open surgery has been performed to obliterate these lesions (Carillo et al. 1984; Barnwell et al.1990;Aoki et al.1991; Nelson et al.1992; Kikuchi et al. 1994; Talamonti 1997), the method of choice over the past two decades has become the endovascular approach. The maturation of this technique in dedicated pediatric neuroendovascular centers has al­lowed for safe and targeted embolization of these CAVFs. Similar to the
4Cerebral Arteriovenous Fistulas280
Ta b l e 4 .13. Selected literature on supratentorial AVFs in children (Weon et al.2005)
Author Age of No. ofAVFs Treatment modalities Clinical Mortality Remarks
reported cases outcome
Carrillo 4 years 1 Surgery Good 0% et al. 1984
Vinuela 12–15 years 2 Combined (1 case), Good 0% et al. 1987 conservative (1 case)
Barnwell 3–7 years 3 Surgery,combined Good 0% 1 case of et al. 1990 HHT1
Aoki 1 year 1 Surgery Good 0% Minimal et al. 1991 leg
weakness
Nelson 1day–-19 years 13 Endovascular (9 cases), 7/11 7.8%
et al. 1992 surgery (1 case), combined complete
(1 case), conservative (2 cases) exclusion
Kikuchi 7 and 16 years 2 Surgery (2 cases) 1 excellent, ? HHT1 et al. 1994 1 unknown
Talamonti 8 months 1 Surgery Normal 0% 1997
We o n £15 years 63 AVFs Endovascular (34 cases), 88.6% 5.6% 11 cases et al. 2005 in 41 endovascular + radiotherapy good of HHT1
patients (1 case) (25%)