S28 Jornal de
Pediatria - Vol. 78, Supl.1, 2002
0021-7557/02/78-Supl.1/S28
Jornal de Pediatria
Copyright © 2002 by Sociedade Brasileira de Pediatria
REVIEW ARTICLE
Surgical treatment of epilepsies in children
Jaderson Costa da Costa*
Abstract
Objective: to review the literature on surgical treatment of epilepsy in children.
Sources: this review article is based on critical analysis of literature concerning epilepsy surgery for
children.
Summary of the findings: in children and adolescents, developmental abnormalities and low-grade
tumors predominate as causes and types of epilepsies. Extratemporal resections and hemispherectomies are
common in pediatric series, and hippocampal sclerosis is rare. Seizure-free outcome is significantly less
frequent after extratemporal or multilobar resection than after temporal resection in children than in adults,
but the results are gratifying in both groups. Also, a global outcome, including parental satisfaction,
developmental and social outcome, as well as activities of daily living (ADL), schooling, and behavioral
changes should be considered. Pediatric epilepsy surgical series show that 60-100% of the patients have
a good seizure outcome. Roughly 30 to 40% of patients improve some aspects of their behavior such as
attention, aggressiveness, and hyperactivity and 16% of those start to attend school after surgery. Parents
noted improvement of their social life in about 2/3 of children.
Conclusions: surgery for epilepsy has now become a realistic therapeutic option for selected children
and the field is likely to increase in the near future. Surgical therapy should not be considered unless there
is a reasonably good chance of improving the patient’s quality of life.
J Pediatr (Rio J) 2002; 78 (Supl.1): S28-S39: refractory epilepsy, epilepsy surgery, global outcome.
Introduction
Despite the fact that over the last ten years approximately
10 new antiepileptic drugs (AED) have been introduced in
the national and/or international market, 20% to 30% of the
pediatric patients still present epilepsies refractory to clinical
treatment. The present article will focus on such patients.
The development of the surgical treatment of epilepsy
necessarily involves the technological advances seen over
the last two decades, and more remarkably, over the last
years. Consequently, we have observed a growing number
of medical centers dedicated to the surgical treatment of
epilepsy in children.1-10 This ongoing interest is due to at
least two factors: the positive results seen in most patients,
and the introduction of new investigation techniques,
especially neuroimaging.11 Epilepsy surgery in children is
particularly more complex than in adults, since: 1) surgery
is performed in a developing human being and, therefore,
with constant changes in neurobiological characteristics; 2)
as seizures and intervention occur early on in life, there is a
higher potential of repercussion on the child’s development;
and 3) surgery occurs in a moment of great plasticity and,
therefore, of greater post-surgical reorganization/adaptation.
* Professor of Neurology, PUCRS School of Medicine.
S28
Surgical treatment of epilepsies in children - da Costa JC
At least three conditions must be fulfilled in order to
consider a surgical treatment for epilepsies: the presence of
epileptic seizures refractory to drug treatment; the actual
probability of a satisfactory result, both in relation to the
control of seizures and in relation to the quality of life; and
the actual possibility of performing the surgical treatment
without adding a significant functional deficit.10
Epilepsies refractory to clinical treatment
Epilepsy refractory to clinical treatment or
pharmacoresistant epilepsy is defined as the inadequate
control of seizures despite proper drug therapy with AED,
or the adequate control of epileptic seizures, but with
unacceptable side effects.12-14 The recognition of what an
inadequate control is and which side effects are unacceptable
is somehow subjective. Therefore, there is no precise number
of epileptic seizures for a patient to be considered eligible
for surgery. Some patients can bear extremely well a certain
number of seizures in a year, especially pre-school children,
while this same number can be completely debilitating to
another group of patients, compromising their social or
school life, or determining a greater risk of injuries, or even
a more serious life threat.12 With regard to unacceptable
side effects, we understand them as the tolerability to
AEDs, which can definitely limit the patient’s activities,
and even impose a life threat. At no moment should this
criterion mean convenience of treatment. As a general rule,
a patient is considered to be refractory to drug treatment and
eligible for epilepsy surgery when, despite being properly
treated for a period of two to three years with the several
therapeutic schemes available, still presents disabling
epileptic seizures.13,15-18 A two to three years’ follow-up
may be completely inadequate in catastrophic epilepsies of
childhood, with a progressive feature, such as the case of
patients with chronic Rasmussen encephalitis, or in the case
of patients who present a progressive injury process (e.g.,
brain tumor).
It must be clear that, because of the risks involved in the
surgical treatment of epilepsy, drug treatment must be the
first therapeutic option for the complete control of epileptic
seizures. Therefore, a sufficient amount of time must be
spent with conventional therapy before surgery is considered.
Clinical therapeutics must be as intense as possible,
performed over the shortest possible period of time.10,19
Refractivity
Prior to considering a patient as refractory to drug
treatment, we must judiciously10,12,19,20:
1. review the diagnosis of epilepsy. Sometimes syncopes
or non-epileptic events are treated as epilepsies (de
Paola & Gates, 1998);
2. to eliminate any possible mistakes in the classification
of seizures, since this can lead to inadequate treatment
or to unsuccessful therapy;
Jornal de Pediatria - Vol. 78, Supl.1 , 2002 S29
3. identify the presence of triggering factors such as, for
instance, video game/intermittent light stimulus (strobe
effect) and sleep deprivation, which can incite the
occurrence of seizures and determine the refractivity to
drug treatment;
4. reevaluate the patient in order to eliminate the presence
of a progressive neurological disorder associated with
convulsive seizures, such as, for instance, brain tumors,
degenerative disorders of the central nervous system
and metabolic disorders;
5. reevaluate the drug treatment. Some of the reasons for a
failure in the response to AEDs include poor adherence
to treatment, use of inadequate AEDs, use of AED at an
improper rate or in insufficient dosages, or, occasionally,
with inadequate associations. This is one of the few
situations in which the serum concentration of the AED
is indicated in order to verify adherence to treatment.21
The flow chart in Figure 1 suggests an approach to
patients with epilepsies refractory to treatment with
AEDs.
Refractivity index and indicators12,19,20
A population study carried out in Finland, with a 30year follow-up, examined children with ages equal to or less
than 15, who presented recurrent epileptic seizures. Authors
verified that 76% of the children did not present any
seizures for over three years. This rate was slightly reduced
(74%) when a five-year period was analyzed. It is important
to highlight that 23% of the surviving patients presented
refractory epilepsy.22 In this study, the occurrence of status
epilepticus, the high frequency of epileptic seizures at
initial stages, and the epileptic seizures associated with
static encephalopathies (non-progressive) due to insult
during the perinatal period, were the indicative signs of
refractory epileptic seizures. Also, the frequency of epileptic
seizures, especially cluster seizures,23 an early onset,24
neural migration disorders,25-27 and other injuries such as
tumors and vascular malformations determine, in general,
the presence of refractory seizures. These patients are
probable candidates for an early evaluation and surgical
indication (Table 1).19,20
Chance of remission
One of the concerns with pediatric patients presenting
epilepsy is their chance of remission with or without drug
treatment. In fact, if the rate of remission is demonstrably
high, there is no reason to indicate surgery, or such indication
will probably occur at later stages during life. On the other
hand, epilepsies with a low rate of remission may occasionally
suggest an earlier surgical approach. In the next paragraphs,
we shall consider some examples of epilepsies susceptible
to surgical treatment, and compare them with non-surgical
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Surgical treatment of epilepsies in children - da Costa JC
Figure 1 - Suggested approach to patients with epilepsy refractory to treatment with AED
situations. Benign idiopathic partial epilepsies have a high
remission rate. In the most common of these conditions,
benign childhood epilepsy with centrotemporal paroxysms,
practically all patients will present a spontaneous remission
prior to the ages of 13 to 16.28-31 Therefore, in these cases,
surgery is absolutely contraindicated.
Remission rate for symptomatic or cryptogenic partial
epilepsies varies from 10% to 62%.5,32-36 In the specific
group of lobo-temporal epilepsies, we find low rates of
remission.5,36
Important aspects for surgical decision-making
Wishfulness and collaboration
It is fundamental that parents are aware of the risk and
benefits associated with epilepsy surgery, and the need of
reinterventions to complement the treatment. If possible,
the child should not participate in this decision. The patient’s
cooperation throughout the process of investigation and in
Table 1 -
Refractivity indicators
– Occurrence of status epilepticus
– Partial epilepsy with frequent and/or clustered seizures
– Early onset (below 2 years)
– Associated structural injury (non-progressive perinatal injuries,
neural migration disorders, tumors and vascular malformation)
the postoperative period is essential for therapeutic success.
Such collaboration is determinant in those procedures in
which an active involvement of the patient is necessary,
such as in cases of resection in regions close to the eloquent
area of the brain, in which it is necessary to locate the
epileptic focus, and to map the motor, sensitive, sensorial
and language functions by means of extra or intraoperative
electrical stimulation.12
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Surgical treatment of epilepsies in children - da Costa JC
Psychomotor development
For newborns and children in general, the progressive
pattern of epilepsy can represent an even greater threat than
it represents for adults, since it can affect the normal
development of the nervous system. Therefore, a surgical
treatment of refractory infantile spasms associated with
focal abnormality can determine not only the control of
seizures, but also a reversion in the regressive status of the
child’s psychomotor development, which suggests the
important role of seizures in this neurological condition.7,37
In addition, the great potential of the still immature brain
tissue to compensate for the functions of the areas resected
during the procedure, known as neural plasticity, notably
reduces the chances of permanent neurological deficits.19,20
Nature of the lesion
After identifying a specific lesion, it is necessary to
confirm whether there is an actual relation between the
lesion and the ictus. In addition, despite the tendency
towards early surgery, it is necessary to be sure that the
seizures will not present a spontaneous remission, and that
the position where the epileptic discharges occur will not
change over time. Moreover, it is necessary to demonstrate
clearly whether the area where the resection will be
performed presents some eloquent function, since its removal
may lead to even more serious damage to the patient. In
practice, basically two functions are considered to be
untouchable: the first is language, and the second, memory.
The localization of these areas must be the main objective
of the pre-surgical assessment.19,20,38-40
Objectives of the epilepsy surgery
Significant differences can be observed in relation to the
objectives and expectations of an epilepsy surgery, when
we compare pediatric and adult patients (Table 2). Thus, in
children, our goals are: 1) to control epileptic seizures with
minimal or no functional repercussion (neurological
sequelae); 2) to interrupt the catastrophic course of some
epilepsies; 3) to resume or to preserve the psychomotor
development; 4) to improve behavior; and 5) to promote a
cognitive improvement associated with an adequate school
performance. In short, we do not seek only to control
seizures, but also to obtain a psychosocial integration of
patients, promoting a visible repercussion on their quality
of life.10,41,43
Contraindications
A clinical status of chronic psychosis is considered a
contraindication for the surgical treatment of epilepsy. On
the other hand, a post-ictal psychotic state should not be
considered as a criterion for excluding this procedure, since
this phenomenon generally disappears after surgery. Mental
retardation is no longer a contraindication; we should keep
Table 2 -
Objectives and expectations regarding the surgical
treatment of epilepsies in children and adults43
Children
Adults and Adolescents
Seizure control
Seizure control
Interruption of the catastrophic
evolution of some epilepsies
License to drive motor
vehicles
Psychomotor development
Job
Behavioral improvement
Independence
Cognitive/educational progress
in mind that refractivity of seizures is an additional problem
in this condition, and that, sometimes, it is considered by the
family as the most severe problem in a patient with mental
retardation. The imminence of a permanent neurological
deficit due to a resection of an eloquent area has been
generally considered as a contraindication for surgical
treatment. However, when such condition already exists,
this criterion has no validity. Moreover, children, especially
under the age of seven, are able to recover an initial deficit
with time. The surgical treatment of epilepsy is
contraindicated in patients presenting a progressive disorder
of the central nervous system (for example, metabolic
encephalopathies or a demyelinating disorder, etc.), as long
as they can not be potentially controlled by surgery, such as
in the case of brain tumors. Other progressive pathologies,
such as Rasmussen syndrome, or potentially progressive,
such as Sturge-Weber syndrome, can also be surgically
treated.
Surgical timing
In epilepsy surgery, as well as in several other procedures,
the outcome can be considerably influenced by the surgical
timing. Four aspects must be considered in order to establish
an adequate surgical timing: the potential harmful effect of
the epileptic seizures on the human brain; the plasticity of
the immature nervous system; the effect of the epileptic
discharges on the developing human brain; and the potentially
harmful effect of AEDs on the child’s neurological
development.
The influence of repetitive epileptic seizures, frequent
or not, clustered or not, separated or not from other factors,
such as underlying lesion, drugs, nutritional conditions,
etc., is very difficult to assess. Experimental models are
being used to evaluate the susceptibility to seizures and
their repercussion on the immature brain.44 Data obtained
so far do not allow us to state that brief seizures or status
epilepticus cause a structural lesion on the brain of a young
animal;45-47 however, there is some evidence suggesting
that recurrent generalized seizures during the early stages of
development can produce a transient reduction in brain
growth.48
S32 Jornal de Pediatria - Vol. 78, Supl.1, 2002
The immature brain presents a greater plasticity and a
greater capacity to reorganize itself.49 Thus, if the language
area suffers a destructive lesion prior to the age of five its
functions can be relocated to the opposite hemisphere.50
Therefore, children who present eventual neurological
deficits, caused by the underlying pathology and/or the
surgical resection, have greater chance of recovery.
AEDs can eventually compromise the patient’s quality
of life due to their side effects.51 Children with behavioral
problems and children with mental retardation seem to be
more susceptible to side effects.52 This effect is greater in
polytherapy than it is in monotherapy. The earlier the
surgery takes place, the fewer are the risks associated with
AEDs.
After delaying surgical indication for the pediatric group
for many years, the centers for the surgical treatment of
epilepsy are seeking to anticipate the procedure, avoiding
more harmful psychosocial sequelae.53
Presurgical investigation
The objective of a presurgical investigation is the
topographic diagnosis, the conditio sine qua non for a
surgical candidate. This diagnosis relies on the clinical,
electrographic, neuropsychological, and neuroimaging
(structural and functional) diagnosis. 10,19,20,54 The
prognosis for surgical outcome will depend on the degree of
convergence of these several topographic diagnoses:
1) Clinical topographic diagnosis: the anamnesis will
help with the establishment of etiological factors, such as
the history of obstetric trauma, anoxic-ischemic injury,
extended febrile seizure (complicated), etc. Other clinical
details must be obtained, such as familial history and
psychomotor development.55 The characterization of the
aura (simple partial seizure) helps with the establishment of
the initial symptomatogenic area. Also, the convulsive
pattern will allow for an outline of the progression of the
epileptic discharges. Reflex asymmetry and muscle power
suggest the possibility of a focal pathology; the presence of
a cutaneous manifestation, such as incontinentia pigmenti,
angiomatosis in the distribution area of the trigeminal
nerve, and cafe-au-lait spots or positive ultraviolet
hypochromic spots should arise the suspicion of a neuronal
migration disorder (hemimegalencephaly), Sturge-Weber
syndrome, and tuberous sclerosis, respectively.56
2) Neuropsychological topographic diagnosis: allows
for the definition of cortical dysfunction areas; the
determination of the language-dominant hemisphere, using
the sodium amytal test (Wada test); and the evaluation of
visual and verbal memory, as well as the memory store
related to the hippocampus contralateral to the side to be
operated.38-40,57
3) Neurophysiologic topographic diagnosis: both
interictal and ictal electroencephalograms are used to locate
Tratamento cirúrgico das epilepsias na criança - da Costa JC
the epileptogenic zone, the latter being used during the
epileptic seizure, and the former one out of the epileptic
seizure. The video-electroencephalogram (video-EEG),
which allows for the simultaneous documentation of the
electroencephalographic report and the patient’s image, is
the main exam used to determine the origin of epileptic
seizures.
On the other hand, when seizures can not be located
using surface electrodes, or when the single-photon emission
computed tomography (SPECT), the nuclear magnetic
resonance (NMR) or the neuropsychological data are not in
accordance with video-EEG findings, intracranial electrodes
are used, such as depth electrodes, subdural or epidural
grids and strips, and epidural pegs.25,28
Depth electrodes are used when one wants to register the
electrographic activity of distant structures in the cerebral
cortex, especially in the amygdala and the anterior and
posterior hippocampus. These electrodes are constituted by
a polyurethane rod, with multiple contact points that allow
for the registration and stimulation of the targets in which
they are implanted. They can also be used in extratemporal
epilepsies in order to evaluate the frontal lobe, the orbitofrontal and medial area (cingulum).3 There are few published
studies about the use of these electrodes in children, although
it is clear that they are increasingly being used in
neuropediatric centers for epilepsy surgery.59 The grids or
strips of subdural electrodes consist of electrodes embedded
in a thin layer of inert or transparent plastic (teflon or
silastic). This layer is moldable and can be easily inserted
into the subdural space. These electrodes are particularly
useful for planning a safe surgical approach, especially in
cases in which the epileptogenic area is near a sensorialmotor area or a language area, since, through these electrodes,
it is possible to register the electrical activity and to
electrically stimulate the cerebral cortex for brain mapping.60
Epidural pegs are useful for the evaluation of two or more
epileptogenic areas in distant neocortical zones. Electrodes
are placed through a small trephine hole, resting the electrode
disc over the dura mater surface. Comparatively to the
electrodes grids and strips, epidural pegs can be placed
more diffusely and in more distant areas; however, they can
only be used in studies of the cerebral convexity.61 Willie
et al. did not find significant differences in the use of
subdural electrodes in children, when compared to the
results obtained with adults.50
4) Topographic diagnosis through neuroimaging:
through exams that allow for an anatomic/functional
diagnosis, such as encephalic computed tomography (CT),
NMR, SPECT, and Positron Emission Tomography (PET),
we seek to establish the areas of structural and/or functional
abnormality. Considering the cost-benefit ratio, NMR offers
advantages over CT in the investigation of epilepsies.62
The improvement of techniques related to magnetic
resonance, notably the volumetric studies of limbic structures
and spectroscopy, have decisively contributed not only to
the diagnosis of structural/functional abnormalities, but
Surgical treatment of epilepsies in children - da Costa JC
also to a better understanding of the physiopathology of
some forms of epilepsy associated with structural
abnormalities, which are sometimes subtle.63,64 Ictal
SPECT, which is more sensitive than the interictal in
locating the zone where seizures begin, is easy to obtain,
due to the low stability of the HMPAO in vitro. However,
the use of ethyl cysteinate dimer has allowed easily obtaining
ictal SPECTs, constituting a more important instrument in
the establishment of the epileptogenic zone.65
“Catastrophic” epileptic syndromes of childhood
Some epileptic syndromes can evolve catastrophically,
not only because of the refractivity of seizures, but also
because of their relevant impact on cognitive and neuromotor
development of patients. Infantile spasms, Sturge-Weber
syndrome, and the Rasmussen syndrome, initially described
as a chronic encephalitis, are some of the most important
catastrophic epilepsies of childhood.
Infantile spasms: we are evidently not referring to
cases of infantile spasms with a positive response to ACTH/
corticoids, pyridoxine and/or antiepileptic drugs. We are
making reference only to infantile spasms that are usually
resistant to habitual therapeutics, and that are associated
with an injury process, such as choroid plexus papilloma,
temporal astrocytoma and the porencephalic cysts, which
can benefit from surgical treatment.61 More recently,
Chugani et al. have identified, with PET, a focal area of
cortical dysplasia in cases of cryptogenic infantile spasms.37
These patients presented a unilateral area of hypometabolism
involving the parieto-occipito-temporal region, and
benefited from the resection of this area, without new
seizures.
Sturge-Weber Syndrome (SWS): SWS is classified as
a neurocutaneous syndrome, characterized by facial capillary
hemangioma, distributed in one or more divisions of the
trigeminal nerve, and ipsilateral leptomeningeal
angiomatosis. These patients develop epilepsy, cerebral
calcification and contralateral hemiparesis. Forty percent
of these patients are believed to be candidates for epilepsy
surgery.66 PET evidence of deep depression with the use of
glucose in the cerebral hemisphere ipsilateral to the facial
angioma, often extending over the affected area - as
determined by MNR or CT - associated with the clinical
observation of a progressive injury,67 has been causing a
debate about early surgical treatment in these cases.
Rasmussen syndrome (RS): RS is a progressive
neurological disease, characterized by partial seizures with
or without secondary generalization, and hemiparesis. In
the past, RS was supposed to have a viral etiology and, more
recently, antiglutamate antibodies (AntiGluR3) were
implicated in the etiology of this syndrome, suggesting an
autoimmune process.68 Drug treatment is rarely sufficient,
and RS is often associated with a clinical status of epilepsia
partialis continua.69 In these cases, a lower-cost resection
Jornal de Pediatria - Vol. 78, Supl.1 , 2002 S33
can be experimented, when the exact area where seizures
begin can be observed.70 However, in patients with more
diffuse lesions, a hemispherectomy must be considered.71
Tuberous sclerosis: tuberous sclerosis complex is a
cellular differentiation, proliferation and migration disorder
that can be acquired hereditarily, as a dominant autosomal
inheritance, or as the result of a mutation. Despite the fact
that lesions are usually multiple, it is possible, sometimes,
to determine which of them is epileptogenic. In these cases,
the resection of the hamartoma or tuber is indicated.
Occasionally, after resection of the main epileptogenic
lesion, other tuber may manifest its epileptogenicity,
demanding a new surgical intervention.19,21
Surgical approach
Several surgical approaches are available for the
treatment of epilepsies. We can classify them into two
groups: resective surgeries, in which the goal is to remove
the area responsible for initiating epileptic seizures and,
thus, obtain the complete control of seizures; and palliative
or functional surgeries, in which the objective is to interrupt
or limit the dissemination of epileptic discharges, minimizing
the clinical manifestations and its consequences. In these
surgeries, the epileptogenic area is preserved and, therefore,
the main objective is not the complete control of seizures,
although some patients can achieve such goal. Callostomies,
sections of the corpus callosum and the multiple subpial
transections (Morell’s technique) are some of these palliative
surgeries that seek to block the propagation of seizures.72,73
Results
Evaluation of surgical outcome
In addition to controlling epileptic seizures, changes in
the neuropsychological parameters usually assessed in the
preoperative period, such as intelligence, language and
memory, must also be considered. Neurological sequelae,
such as paresis and changes in the visual field, must be
evaluated, as well as the impact in the patient’s psychosocial
profile.
With regard to seizures, the most simple form of assessing
these data consists in distributing patients into three
categories: without seizures, improved, or unaltered.
Nevertheless, since the first Palm Desert conference, in
February 1986, most centers have preferred to use a
classification that includes four classes. Thus, class I requires
that patients be free from seizures for two years or since the
time of surgery, although it allows residual auras (simple
partial seizures) and seizures during washout. There is a
semantic difficulty to differentiate patients who are free
from seizures from those who still present auras, which are
simple partial seizures. Class II includes patients who rarely
present disabling seizures; class III includes patients with a
significant improvement; and class IV indicates patients
S34 Jornal de Pediatria - Vol. 78, Supl.1, 2002
without a significant improvement (Table 3). Non-resective
surgeries, considered palliative or functional,29 especially
disconnection surgeries, notably callostomy, must be
evaluated in different terms, since often only a partial
control is obtained and, therefore, there is a need for a
judicious evaluation of the frequency of seizures and the
quality of life in the pre- and postoperative periods. A scale
that can contribute to the evaluation of the number of
seizures is one that is concerned only with the frequency of
seizures75 (Table 4). The objective of this scale is to
standardize the description of the frequency of epileptic
seizures among several authors, in order to evaluate and
compare the results of the surgical treatment in terms of how
many levels, up or down, the patient has moved after
treatment.
There are not many specific and validated instruments
to evaluate the psychosocial aspects related to the quality of
life in epileptic patients. The first and most widely known
is the Washington Psychosocial Seizure Inventory
(WPSI).41,42 This questionnaire was developed in order to
evaluate the psychosocial profile of epileptic patients in
general, and not specifically of patients operated on due to
refractory epilepsy. Nevertheless, in February 1992, the
second Palm Desert conference, in California, 66% of the
Table 3 - Classification of results75
Class I - Free from harmful seizures*
A. Completely free from seizures after surgery
B. Only harmless simple partial seizures after surgery
C. Some harmful seizures after surgery, however free from
harmless seizures in the last 2 years
D. Generalized convulsive seizures only with the discontinuation
of antiepileptic drug treatment
Class II - Rare harmful seizures (almost free from seizures)
A. Free from harmful seizures at the beginning, but now with
rare seizures
B. Rare harmful seizures since surgery
C. Very rare harmful seizures after surgery, but rare seizures in
the last 2 years
D. Only nighttime seizures
Class III - Significant improvement**
A. Significant reduction in the number of seizures
B. Long period free from seizures, longer than 50% of the
follow-up period, but shorter than 2 years
Class IV - No significant improvement
A. With reduction in the number of seizures
B. Without observed improvement regarding the preoperative
period
C. Worsening of seizures after surgery
* Seizures in the immediate postoperative period (first weeks) are not
included.
** The definition of significant improvement requires quantitative analysis of
additional data, with percentage of seizure reduction, cognitive function
and quality of life.
Surgical treatment of epilepsies in children - da Costa JC
Table 4 - Seizure frequency scale75
0.
1.
2.
3.
4.
5.
6.
7.
8.
9.
10.
11.
12.
No seizures, no antiepileptic drugs
No seizures, use of antiepileptic drugs
No seizures, need of antiepileptic drugs
Simple partial seizures (harmless)
Only nighttime seizures
1 to 3 seizures per year
4 to 11 seizures per year
1 to 3 seizures per month
1 to 6 seizures per week
1 to 3 seizures per day
4 to 10 seizures per day
> 10 seizures per day, but no status epilepticus
status epilepticus, if not maintained in drug-induced coma
centers for the surgical treatment of epilepsy that performed
a psychosocial evaluation of their patients had used the
WPSI. 76 More recently, another questionnaire was
developed at UCLA for the evaluation of epileptic patients
treated with surgery, the ESI-55.77 Today a series of scales
are available to be used according to the age group, approach
and strategies for data collection.78
In the published pediatric series, the rate of positive
results, considering temporal, temporal or extratemporal,
and temporal or hemispheric resections, has varied from
50% to 100%. In Table 5, we compare our results with the
results seen in the available literature, and Table 6 and 7
present the results according to surgical approach and
etiology, respectively.
The temporal and extratemporal designations do not
take into consideration some of the epileptic subsyndromes
that can be related to a better or worse surgical prognosis.
Also the option for the surgical approach respects the
definition of the subsyndromes, and this has also been the
object of the latest publications in the area.97
It is a well-known fact that children with refractory
epilepsy tend to present an inferior psychosocial performance
when compared with children in the same age group,
including behavioral and cognition problems, as well as
other limitations to their social relations. These patients
also present a greater incidence of psychiatric disorders,
notably aggressiveness and hyperactivity. Table 8 lists the
main behavioral, cognitive and social aspects that comprise
what we call “non-convulsive aspects”, which should also
be considered for this group of patients, as part of the global
follow-up.43 In our group of patients, the postoperative
interview showed that 35% of children presented an
improvement in their attention deficit; 29% presented a
more stable behavior; and 37% stopped being hyperactive.
We could say that approximately 30% to 40% of patients
presented an improvement in behavioral aspects, including
attention, aggressiveness, and hyperactivity.
Jornal de Pediatria - Vol. 78, Supl.1 , 2002 S35
Surgical treatment of epilepsies in children - da Costa JC
Table 5 - Surgical results in pediatric studies
Authors
Patients
(n)
Age at
surgery
Resection
% of
good results
Davidson & Falconer, 197579
40
2-34
Temporal
60
Rasmussen, 1977
77
6-15
Temporal
73
12
3-15
Temporal
83
32
2-18
Temporal
93
Jensen & Vaernet,
Green,
197780
197781
198082
40
<15
Temporal
57
Whittle, 198183
8
5-18
Temporal
62
Green & Pootrakul, 198284
32
<18
Temporal
81
35
<21
Temporal / Extratemporal
78
Polkey,
Blume et al.,
198285
Goldring & Gregorie,
198486
29
0.5-14
Temporal / Extratemporal
62
Lindsay et al., 198487
13
7-36
Temporal / Hemispheric
100
Meyer et al., 198688
50
7-18
Temporal
78
198789
40
<15
Temporal / Extratemporal
65
Drake et al.,
198790
48
6-16
Temporal
100
Wyllie et al.,
19885
Goldring,
23
3-18
Temporal / Extratemporal
70
Hopkins & Klug, 199191
11
1-9
Temporal
90
Ribaric et al., 199192
34
2-15
Temporal / Extratemporal
85
16
<12
Temporal
88
Duchowny et al.,
Adelson et al.,
19921
199293
33
<18
Temporal / Extratemporal
87
Wyllie et al., 19936
18
<12
Temporal
83
Wyllie et al., 199694
12
<2.5
Temporal / Extratemporal / Hemispheric
75
31
<3
Temporal / Extratemporal / Hemispheric
76
62
74
0.3-12
13-20
Temporal
Extratemporal / Hemispheric
79
89
73
0.4-16
Temporal / Extratemp/multilobar
Hemispheric
78
Duchowny et al.,
Wyllie et al.,
199895
199896
Da Costa et al., 200143
Table 6 -
Surgical results in pediatric patients. Program of
Epilepsy Surgery of Porto Alegre, Hospital São
Lucas of PUCRS. Follow-up from 2 to 8 years
Procedure
% of good results
Temporal resection
92
Extratemporal/multilobar resection
40
All procedures
78
In our series, 67% of the children presented an
improvement in their social performance; 24% remained at
the same level of performance; and 9% got worse (Table 9).
Those patients who got worse were the same who did not
present a good control of their seizures after surgery.
Parents of children over the age of three answered a
quite simple questionnaire in order to evaluate everyday
activities (EDA), mostly related with self-care. Those
patients with a more independent behavior were considered
as A-B classes; those with a low performance in EDAs
(Table 10) were considered as classes C and D (Table 10).
Table 7 - The most frequent etiology in pediatric studies43
Cortical development disorder
31.5
Low malignity/fetal brain tumors
20.5
Hyppocampal sclerosis
8
S36 Jornal de Pediatria - Vol. 78, Supl.1, 2002
Surgical treatment of epilepsies in children - da Costa JC
Table 8 - Behavioral changes due to surgical treatment: interview with parents
Behavior
Patients with
preoperative
behavioral problems
Persistent
behavioral
problems
Attention deficit
26 (36%)
17
9 (35%)
Emotional instability/ lack of control
17 (23%)
12
5 (29%)
Hyperactivity
19 (26%)
12
7 (41%)
Ten percent of our patients moved from classes C/D to
classes A/B (Table 11). Although this is a low percentage,
it is important to indicate that 1/3 of our patients presented
a significant delay in their psychomotor development, and/
or were mentally retarded. Occasionally, a child with dozens
of seizures with falls per day or week, totally unable to
perform the simplest EDA, begins to take care of himself or
herself, a fact that generates an important impact on the
family.
In one year of follow-up, 16% of the children oldern
than seven who did not go to school had begun their school
activities, even though most of them required a specialized
pedagogical support. Patients with severe mental retardation
did not acquire the minimal requirements for beginning
their pedagogical activity.
Table 9 - Sociability
Improved
28 (67%)
Same
10 (24%)
Deteriorated
Table 10 - Daily activities (DA)
1.
2.
3.
4.
5.
6.
To take a shower without help
To get dressed
To eat
To brush one’s teeth
To help with housework
Initiative to perform activities
Class A:
Class B:
Class C:
Class D:
6 items
4 to 5 items
2 to 3 items
1 item
4 (9%)
Improvement
Table 11 - Daily activities
Class
Presurgical
Postsurgical
A+B
68%
78%
C+D
32%
22%
All these aspects are important in the follow-up of
children with refractory epilepsy who are submitted to
surgical treatment. Regardless of the control of seizures, the
other aspects involved in the establishment of good quality
of life must be evaluated and valued. Remembering Wilder
Penfield, the pioneer of the epilepsy surgery: “It is not
enough to know whether a radical surgical procedure has
stopped attacks or not. We must know its effect upon the
patient’s.... behavior and on the happiness of the patient and
friends. When all the features of his life are considered, it
still remains for the physician to ask the final question: in the
opinion of the patient and those who love him, was the
operation a success or failure?”
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Corresponding author:
Dr. Jaderson Costa da Costa
Av. Ipiranga, 6690 - Centro Clínico PUCRS - sala 202
CEP 90610-000 – Porto Alegre, RS
E-mail: [email protected]