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

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ing vasculature in the clinical expression of a given lesion. In addition, the importance of the parallel maturation of the surrounding brain in children created the concept of optimal therapeutic timing. Finally, the fact that le­sions may not be present at birth certainly further questions the desire to obtain eradication of the AV shunt as the only guarantee of a return to a nor­mal state and protection over time. Such an approach in children is not based on any evidence and on the contrary has often resulted in improper treatment decisions,particularly in young children.The preeminence of the technical challenge to erase any risk and the consequent aggressiveness of the treatment performed has obscured the fact that it might not always be needed immediately and might perhaps be more safely achieved later. Sev­eral years ago,staged treatment procedures entered the interventional are­na. This represented a progression from palliative to secondarily completed surgery and subsequently to planned staged procedures.Little attention was paid to the recovery of the vascular system, and the challenge to achieve to­tal eradication precluded recognition of the reconstructive results obtained. Interventional neuroradiological experience with CAVM management has demonstrated the validity of the following concepts:
There is more than a semantic difference between staged, partial, and
palliative treatment.
The desire to rapidly obtain a complete exclusion of the AVM is often
a compulsory objective, which may indicate a failure of analysis to understand and predict the spontaneous outcome in a given patient.
Incomplete exclusion does not mean the absence of treatment.Postsurgical remnants do not have the same significance as partially
embolized lesions.
5Cerebral Arteriovenous Malformations332
Fig. 5.25E. Legend see p. 331
333Complete Exclusion
Fig. 5.26A–D. A 14-year-old boy presenting with a brutal left hemiplegia associated with IVth nerve palsy and Parinaud syndrome. CT and MRI (A, B) disclosed a right quadrigeminal hematoma. Although the images are suggestive of cavernoma, the ax­ial cut demonstrates a small network on the tegmental portion of the mesencephalon. Selective arteriography demonstrates an arteriovenous malformation (C, D).E–G see p. 334
5Cerebral Arteriovenous Malformations334
Fig. 5.26E–G. (continued) Selective injection into the feeder allowed glue deposition (E). Postembolization follow-up after a few months demonstrates complete stable oc­clusion of this mesencephalic AVM (F, G )
The endovascular approach to CAVMs provides a wider range of op-
tions,both in the degree of completeness and the timing of treatment,
and more so than any other traditional treatment modality.
The complete disappearance of a lesion at imaging follow-up is reliable.To t al stable exclusion of an AVM does not require secondary removal
for preventive purposes. Embolization is a generic name that encompasses many different prac-
tices,objectives,and results.
335Complete Exclusion
Fig. 5.27. A, B A 6-year-old child presenting with an intracerebral hematoma result­ing in an incomplete hemiparesis.B–D Following embolization in one session,the en­tire lesion was excluded.Clinical recovery has started
5.6.2 Partial Treatment
In our practice,the goal of treatment in children has become the preser­vation of normal neurocognitive maturation as well stable protection from acute episodes. In most instances,the morphological goal can be su­perimposed on the clinical goal. In babies, however, as seen in VGAM, these objectives can be separate in time or even contradictory, if the at­tempt to obtain a cure compromises neurological outcome. Staged treat­ment is the planning of steps to reach a more favorable situation and to achieve an exclusion with lower morbidity than if it was accomplished in a single session. Palliative treatment is an incomplete exclusion of a lesion in order to stabilize a critical situation.Partial targeted treatment is an in­complete exclusion motivated by a clinical concern requiring improve­ment and directed toward a specific portion of the lesion when complete exclusion cannot be offered with an acceptable level of risk.It has a clini­cal objective and, in contrast to the palliative objective, its results are morphologically and clinically identifiable and can therefore be evaluat­ed (Fig. 5.28).
A certain group of patients have not yet completed the embolization treatment, making up a new population of patients who did not exist as such in the past.Some are still under treatment (staged),and one may ask why the treatment is not continued more rapidly. The decision to stage a procedure carries some significant advantages, particularly in children, where the length of the procedure is limited by puncture time or fluid volumes. In addition,with the hemodynamic triggers being modified,the correction of some disorders induces a normal, although delayed matu­ration. In this group, the problem is the time between two consecutive sessions. There is no definitive optimal schedule; however, with experi­ence each interventional neuroradiologist knows whether a better techni­cal result can be achieved by waiting for a few weeks or months. This strategy will permit a rearrangement of the remaining anatomy, and some areas of induced angiogenesis may regress, whereas some smaller feeders may enlarge, permitting easier catheterization. This will have to be balanced with the hemodynamic changes induced by embolization and hemodynamic needs, to ensure a proper or preserved maturation process.
There are a number of patients who cannot be cured even with the cur­rent treatment options and capabilities, either because the lesion cannot be reached or because the disease is multifocal. In this subgroup, treat­ment is partial and is repeated in time with long intervals between ses­sions. We do not perform palliative embolization in children with AVM. The aim of partial treatment is to eliminate dangerous portions of the AVM (intranidal aneurysms, false aneurysms seen following a hemor­rhagic episode, and venous ectasias [Figs. 5.24,5.28]) or its effects on the adjacent brain (venous congestion, venous thrombosis) or to reduce seizure activity, progressive deficits, or headaches (Figs. 5.29, 5.30). In some instances,this targeted embolization deals with a dangerous part of the lesion prior to the irradiation of the remainder, to avoid repeated he­morrhage during the 2-year interval and to reduce the size of the target further (Figs. 5.33,5.34).In our experience, partial targeted embolization
5Cerebral Arteriovenous Malformations336
337Partial Treatment
Fig. 5.28. A, B An 8-year-old boy presented with sudden intracerebral hematoma re­sulting in right-sided hemiparesis and hemianopia.C,D The presence of an intranidal aneurysm prompted partial targeted embolization prior to discussing further man­agement.Clinical recovery is incomplete
5Cerebral Arteriovenous Malformations338
Fig. 5.29. A–C A 5-year-old child had had a progressive motor deficit in relation to large multifocal AVMs located in the brain stem since the age of 2 years.Following four distal glue deposits in three sessions (D–F), although the angio­graphic changes were not spectacular, the clinical improvement was dramatic
339Partial Treatment
Fig. 5.30A–E. Legend see p. 340
is an acceptable therapeutic objective only if a complete cure cannot be obtained at a satisfactory level of risk and if the procedure is performed with a permanent agent,such as NBCA.This means that we aim to obtain – by endovascular or other techniques – total exclusion if a good neuro­logical outcome can be guaranteed.The fact that most lesions in patients referred to us are large or multifocal explains the small number of com­plete cures in our series.
5.6.3 Neonates and Infants
Tr eatment objectives in this age group are the same as in VGAM; howev­er,the insult to the cerebral tissue is more rapid,as the venous drainage is usually impaired, and collateral venous circulation is compromised, using as alternative drainage toward subpial veins in most instances. The poor neurological prognosis forces us to try to achieve a signi­ficant reduction of the shunting more rapidly. Early management is, therefore, not so much motivated by the CCF, which is usually mild to moderate, but by the possibility of ensuing irreversible cerebral damage. The patients should be evaluated without angiography in order to assess the degree of interference between the AVM and the brain tis­sue, while rapidly scheduling the first embolization session, almost re­gardless of the existing symptoms. If the CCF is severe, the prognosis is extremely poor, since it combines both systemic and subpial effects (Fig. 5.17).
5Cerebral Arteriovenous Malformations340
Fig. 5.30. A, C Deep-seated AVM located in the head of the caudate nucleus and re­vealed with an intraventricle hemorrhage (A frontal,B early,and C late lateral views). D Distal selective catheterization of the Heubner artery allowed the embolization, thus disconnecting 90% of the lesion (E–G)
Convulsion is a major symptom and should point to the need for rapid treatment, even if the convulsion remains isolated and without perma­nent clinical effect (Tables5.2, 5.3).
In the series of Rodesch et al. (1995a), CAVSs had a poorer prognosis than VGAMs when the onset of symptoms was at neonatal and infant age.
Sixteen neonates or infants were embolized as the primary modality of treatment and four of these eventually died. One died due to multiorgan failure, one following a complementary surgical approach, one from he­morrhage after incomplete treatment (three sessions) for a multifocal perimesencephalic AVM,and one from a posterior fossa hemorrhage de­spite complete occlusion of the AVM.Eight out of 12 had a normal neuro­logical status (scores of 5, 4, or 3), and the remaining four had a score of
2. Eight patients had surgical resection of their CAVS as the first (and only) treatment modality; seven of them were older than 2 years of age at the time of surgery. All patients had their AVM removed and all are still alive; two out of eight have moderate permanent neurological deficits.
5.6.3.1 Hydrodynamic Disorders
Possible hydrodynamic disorders should be managed early through re­duction or occlusion of the AV shunt. Even if pial lesions in infancy pre­sent features that simulate the VGAM pattern, symptoms are significant­ly different, rarely causing hydrodynamic disorders, but frequently focal neurological symptoms and hemorrhage. CAVMs rapidly produce local brain atrophy (focal melting-brain syndrome) (Fig. 5.17). 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.
3415.6.3.1 Hydrodynamic Disorders
Ta ble 5.2. Pial AVMs in children (<16 years): clinical presentation in neonates
Cardial overload 50% Hemorrhage 37.5% Incidental 12%
Ta ble 5.3. Pial AVMs in children (<16 years): clinical presentation in infants
Hemorrhage 30% Macrocrania 26.6% Cardiac overload 23.3% Deficit 6.6% Epilepsy 6.6% Incidental 6.6%