Postoperative development of children after hemispherotomy

doi:10.1016/S0387-7604(02)00010-4

Brain and Development

Volume 24, Issue 3, April 2002, Pages 155-160

https://doi.org/10.1016/S0387-7604(02)00010-4 Get rights and content

Abstract

We studied the postoperative development of 14 children with cortical dysgenesis who underwent modified functional hemispherectomy (hemispherotomy) at the age of 6 years or younger. At follow-up of 24–72 months (median of 47 months), six (43%) were seizure-free, six achieved>90% reduction, one achieved 50–90% reduction, and one achieved 0–50% reduction in seizure frequency. At the preoperative and final postoperative examinations, mean scores of developmental quotient (DQ) were as follows: 25.5 and 31.9 in total DQ, 26.0 and 33.7 in intellectual DQ, and 27.4 and 22.9 in motor DQ. Children scoring >50 points in preoperative intellectual DQ score obtained near-normal intellectual DQ postoperatively, while, those scoring <10 DQ preoperatively remained at a low developmental level. Among children with DQ scores in the range from 10 to 50, two children who obtained seizure-free outcome and were operated upon in the first 3 years of life achieved marked developmental progress. The present study indicated that high preoperative intellectual DQ and cessation of seizures seem to be associated with better postoperative intellectual development. However, long-term observation of postoperative development and an accumulation of more cases will be needed before we can reach a firm conclusion.

Introduction

Cortical dysgenesis has recently been recognized as an important pathology of children with medically refractory epilepsy [1]. As their epilepsy is often catastrophic due to the frequency and intractability of the seizures, hemispherectomy is applied to selected children, most often to infants with hemimegalencephaly (HME) [2], [3], [4]. However, in assessing benefits for the developmental process in these children, seizure control alone is not the best measure of the effectiveness of epilepsy surgery [5], [6] since the children inevitably have a combination of mental retardation and motor dysfunction. For adequate measurement of the success of hemispherectomy, postoperative assessments of intellectual development and motor function are mandatory [4], [7].

In this study we examined the postoperative development of hemispherectomized children with cortical dysgenesis, focusing on factors associated with better intellectual development.

Section snippets

Patients

Between October 1994 and March 1998, we performed a modified functional hemispherectomy (hemispherotomy) on 15 children aged 6 years or less who had intractable epilepsy caused by cortical dysgenesis. We employed a modified version [8] of the peri-insular hemispherotomy reported by Villemure [9]. One child became severely disabled due to acute brain swelling and was excluded from the study, so, our final series consisted of 14 children (six left and eight right sides). All of the children were

General information

Table 1 shows characteristics of 14 children who underwent hemispherectomy. MRI demonstrated eight holohemispheric HMEs, five focal cortical dysplasias (FCDs), and one polymicrogyria (PMG), all of which were subsequently confirmed by pathological examination. In one child with FCD, dysplastic areas were observed throughout an entire hemisphere. In one child with PMG and two children with FCD, the frontal, temporal, and parietal lobes were affected. In the remaining two children with FCD,

Discussion

In the present series of hemispherotomies, we examined total, intellectual, and motor development after surgery. Our results indicated that worthwhile seizure reduction following hemispherotomy effectively facilitates the steady development of children with cortical dysgenesis, especially the development of intellectual function.

Numerous recent reports have confirmed that functional hemispherectomy can assure steady postoperative development in children by reducing their seizures [2], [6], [12]

Acknowledgements

This work was supported by the Japan Epilepsy Research Foundation (1996).

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Challenges of Epilepsy Surgery
2020, World Neurosurgery
Though frequently effective in the management of medically refractory seizures, epilepsy surgery presents numerous challenges. Selection of the appropriate candidate patients who are likely to benefit from surgery is critical to achieving seizure freedom and avoiding neurocognitive morbidity. Identifying the seizure focus and mapping epileptogenic networks involves an interdisciplinary team dedicated to formulating a safe and effective surgical plan. Various strategies can be employed either to eliminate the epileptic focus or to modulate network activity, including resection of the focus with open surgery or laser interstitial thermal therapy; modulation of epileptogenic firing patterns with responsive neurostimulation, deep brain stimulation, or vagus nerve stimulation; or non-invasive disconnection of epileptic circuits with focused ultrasound, which is also discussed in greater detail in the subsequent chapter in our series. We review several challenges of epilepsy surgery that must be thoughtfully addressed in order to ensure its success.
Physical Growth of the Contralateral Cerebrum is Preserved After Hemispherotomy in Childhood
2019, Pediatric Neurology
Hemispherotomy can be an effective treatment for refractory childhood epilepsy. However, the extent of postoperative brain development after hemispherotomy remains incompletely understood. This study aims to provide an anatomic foundation in assessing development of the contralateral hemisphere, by measuring volumetric growth after hemispherotomy.
Eleven patients with hemimegalencephaly, Rasmussen's encephalitis, and cerebral infarction who underwent hemispherotomy before age 12 years, an immediate preoperative magnetic resonance imaging, and at least three years of follow-up magnetic resonance imagings were retrospectively analyzed. The volume of the contralateral hemisphere was measured before and after surgery. Growth curves were compared with those of healthy individuals from an open database. The growth rate relative to the healthy individuals (“catch-up rate”) was calculated.
A positive volumetric growth of the contralateral hemisphere was observed across all pathologies. The hemimegalencephaly subgroup underwent hemispherotomy at the earliest time and had the largest postoperative growth rate, which exceeded that of healthy individuals. The Rasmussen subgroup underwent surgery at the second earliest time and had an intermediate growth rate, which was similar to that of healthy individuals. The infarction subgroup underwent surgery at the latest time and had the slowest growth rate, which was less than that of healthy individuals.
The contralateral hemisphere continues to increase in volume after hemispherotomy in childhood. Further studies with a larger sample size and correlation with cognitive outcomes may aid in characterizing the prognosis after hemispherotomy.
Epilepsy surgery for children with severe developmental delay: An ethical double jeopardy
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Functional hemispherectomy is safe and effective in adult patients with epilepsy
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Citation Excerpt :
This is especially true in patients with acquired or progressive lesions without the typical hemispastic syndrome with preserved hand function, whereas in patients with inborn or in early childhood acquired anomalies showing the typical hemispastic syndrome with noticeably limited hand function, hemiparesis will not exacerbate postoperatively. However, recovery of postoperative initially aggravated motor impairment has been reported in children as they are able to compensate potentially new deficits, most probably due to brain plasticity [21–24]. Less is known concerning functional reorganization of motor cortex and language in the adult nervous system [1,5,6,25].
Functional hemispherectomy (FH) is a well-established therapeutic option for children with epilepsy with parenchymal damage confined to one hemisphere, yet its application in adults remains rare. The intention of our study was to investigate postoperative clinical and epileptological outcome in adults who received FH for intractable epilepsy.
We retrospectively analyzed 12 adult patients (18–56 years) with intractable epilepsy due to unihemispheric pathology. All patients underwent FH. Postoperative neurological and cognitive outcome as well as seizure status were evaluated with a mean follow-up period of 4.9 years.
Ten patients (83%) were seizure-free (Engel I), and two (17%) had recurrent seizures at last follow-up. Apart from one patient requiring operative revision for bone flap infection, no perioperative morbidity or mortality occurred. Postoperative functional assessment revealed deterioration of motor function in 7 patients, whereas 5 remained unchanged. Language was unchanged in 8 patients. The absence of background slowing in preoperative electroencephalogram (EEG) as well as ictal and interictal EEG patterns located ipsilateral to the side of surgery was associated with favorable seizure outcome.
Favorable seizure control and acceptable functional outcome can be achieved by FH in adults with intractable epilepsy. The risk of postoperative deficits is moderate and even older patients are able to manage postoperative motor impairment. Therefore, FH should be considered in case of unihemispheric lesions also in adults.
An observational study on outcome of hemispherotomy in children with refractory epilepsy
2016, International Journal of Surgery
Citation Excerpt :
When compared for the seizure outcome between the groups, while few studies have reported non-significant difference between functional hemispherectomy and hemispherotomy [15], multi center trials with large series have reported significantly more number of patients undergoing hemispherotomy as seizure-free as the functional hemispherectomy groups. Encouragingly, an increasing number of studies show that hemispherotomy is associated with improvements in post-operative development, quality of life (QOL) and adaptive behaviors in children by reducing the seizure frequency [16–18]. Disparities in aetiology have been reported in developing countries and even within a given developing country [19].
The current study aimed to evaluate the clinical characteristics and outcome of hemispherotomy in children with refractory hemispherical epilepsy.
Retrospective analysis of data in twenty one children aged ≤12 years who underwent hemispherotomy and had at least two years post surgery follow-up was performed. Sixteen children underwent Delalande's vertical para-sagittal hemispherotomy (VPH), while lateral peri-insular functional hemispherotomy was performed in the rest.
The average age of onset for epilepsy in the study population was 2.9 ± 2.4 years; the average duration of epilepsy was 4.0 ± 2.9 years. The mean age at surgery of the study population was 6.8 ± 2.8 years. Six (28.5%) children were girls. Gliosis due to presumed childhood infarct was most common etiology, observed in 13 (62.0%) of the children, followed by Rasmussen's encephalitis in six (28.5%). There was no significant difference between the surgery groups for the reported acute post operative seizures (APOS) (20.0% vs. 25.0%; p = 1.000). At last follow up 90.5% patients were seizure free; there was no difference between the groups for seizure freedom (60.0% vs. 87.5%; p = 0.228). When analyzed for outcome between the etiologies, seizure freedom was similar for gliosis due to infarct (76.9%), Rassmussens encephalitis (83.3%) and malformations of cortical development (MCD) (100.0%). Moreover, improved quality of life in epilepsy (QOLIE) scores was observed in 80.0% of the lateral peri-insular functional hemispherotomy group and 87.5% children in VPH group at the last follow-up.
Gliosis due to presumed childhood infarct was the leading cause of medically refractory epilepsy caused by hemispheric lesions in the current study. Encouragingly, hemispherotomy offers seizure freedom (in 90.5% patients) and improvement in QOLIE scores at two years follow up.
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To evaluate whether aggressive nutrition can improve long-term neurodevelopmental outcomes and growth in extremely low birth weight (ELBW) infants born appropriate for gestational age (AGA).
This single-center cohort study included 137 ELBW AGA infants born in two epochs. The first group received standard nutrition (SN; n = 79) consisting of amino acids started at 0.5 g/kg/day on Day 4 of life and increased to 1.0 g/kg/day. The second aggressive nutrition (AN) group received amino acids started at 1.5–2.0 g/kg/day within 24 h of life and increased to 3.5 g/kg/day. Parenteral and enteral feedings were combined in both groups. Neurodevelopmental outcomes by the Kyoto Scale of Psychological Development and growth were followed up to 18 months of corrected age or 3 years of age and compared by univariate and multivariate analyses.
Baseline characteristics were similar between the two groups. At 3 years of age, AN children had a significantly greater mean value of head circumference, but not length or weight, than SN children (49.1 vs 48.0 cm, p = 0.014). The cognitive-adaptive (C-A) score in the AN group was also significantly higher than that in the SN group (98.3 vs 91.9 at 18 months, p = 0.039 and 89.5 vs 83.1 at 3 years, p = 0.047). AN infants born ≥ 26 weeks of gestation were less likely to develop borderline disability in C-A, language-social and overall developmental scores compared to gestational age-matched SN infants.
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Original articlePostoperative development of children after hemispherotomy

Abstract

Introduction

Section snippets

Patients

General information

Discussion

Acknowledgements

Pediatr Neurol

Pediatr Neurol

Focal neuronal migration disorders and intractable partial epilepsy: a study of 30 patients

Ann Neurol

The role of hemispherectomy in the treatment of holohemispheric hemimegalencephaly

J Neurosurg

Hemimegalencephaly and intractable epilepsy: benefits of hemispherectomy

Epilepsia

Why would you remove half a brain? The outcome of 58 children after hemispherectomy – The Johns Hopkins Experience: 1968 to 1996

Pediatrics

Surgery for catastrophic localization-related epilepsy in infants

Epilepsia

Epilepsy surgery in the first three years of life

Epilepsia

Functional hemispherectomy for children aged 2 years or less for the treatment of intractable epilepsy caused by cortical dysgenesis (in Japanese)

No To Hattatsu (Tokyo)

Modification of peri-insular hemispherotomy and its surgical results

Neurosurgery

Peri-insular hemispherotomy: surgical principles and anatomy

Neurosurgery

Original article
Postoperative development of children after hemispherotomy