Добавил:
kiopkiopkiop18@yandex.ru t.me/Prokururor I Вовсе не секретарь, но почту проверяю Опубликованный материал нарушает ваши авторские права? Сообщите нам.
Вуз: Предмет: Файл:

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

.pdf
Скачиваний:
0
Добавлен:
30.08.2026
Размер:
57 Мб
Скачать
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