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Update on Surgery for Epilepsy 356 Arch Dis Child 1999;81:356–359 CURRENT TOPIC Arch Dis Child: first published as 10.1136/adc.81.4.356 on 1 October 1999. Downloaded from Update on surgery for epilepsy J H Cross Surgery for epilepsy is not new, it has been per- area of the brain that is functionally silent formed for over 100 years. However, there has (table 2). The size of the area may be relatively been a reluctance to consider young children small or large, partly dependent on underlying for surgery other than in exceptional circum- pathology and partly the area of brain involved. stances, owing in part to the invasive nature of Complete removal or disconnection of one the presurgical evaluation. Recent advances in cerebral hemisphere (hemispherectomy) may investigative techniques have allowed identifi- be considered where there is a pre-existing cation of candidates early in their natural hemiplegia associated with a structural abnor- history avoiding the long term consequences of mality of the contralateral hemisphere, and sei- chronic epilepsy. Based on a prevalence rate of zures have been proved to arise from that 3–6/1000 and current population figures of hemisphere. With either of these techniques, 60 000 children with epilepsy, about 15 000 presurgical evaluation is aimed at determining are unresponsive to anticonvulsant medication, whether such surgery is likely to cure or and of these as many as 3000 might benefit improve seizures without deterioration in func- from surgery. There are currently six centres in tion. Temporal resection is the most common the UK oVering surgery to children. Of these, operation in paediatric and adult practice, but Great Ormond Street in London operates hemispherectomy comprises up to a quarter of exclusively on children (30 cases/year), al- procedures in paediatric practice, whereas few though for each child who comes to surgery it are performed in adults. Extratemporal resec- is likely four have been evaluated. tion is diYcult in the absence of a known structural lesion, but may be more common The case for early surgery where invasive electroencephalography (EEG) Most adults coming to an epilepsy surgery is available. programme have had a history of seizures since In children there may be some diYculty in childhood,1 the consequences of which are sev- determining when seizures are drug resistant eralfold. Studies of selected groups of children and at what point surgery should be consid- http://adc.bmj.com/ with ongoing seizures suggest that severe ered. In adults, medical intractability may be epilepsy is associated with cognitive decline2 considered as failure to respond to at least two and that early cessation of seizures is associated anticonvulsant drugs over at least two years; with better developmental outcome.3 The psy- these rules may not be appropriate in paediat- chological consequences of recurrent seizures ric practice. In an infant with catastrophic through school and teenage years are also onset epilepsy, seizure frequency may be such apparent, both to the individual and the that a greater number of drugs are tried over a community.4 In addition, recurrent epileptic lesser time. There may be pressure in such on October 1, 2021 by guest. Protected copyright. seizures are not without risk to the individual, cases to suppress seizures as early as possible to both with regard to self injury during a seizure try and reduce the developmental morbidity and the risk of sudden death.5 With advances in Table 1 Types of surgery for epilepsy neurosurgical and neuroanaesthetic tech- niques, morbidity from planned neurosurgical Focal resection—temporal or extratemporal procedures is now low. In addition, postopera- Hemispherectomy Functional procedures tive outcome of seizure control in studies Corpus callosotomy performed to date in children are similar to Multiple subpial transection those in adults.6 There is consequently no jus- tification in conducting a “wait and see” policy Table 2 Criteria for consideration for surgery for epilepsy rather than referral in children with epilepsy, Consultant in particularly in early onset catastrophic epi- Focal resection Paediatric Neurology lepsy, which may have a focal onset. Seizures arise from a single area of the brain that is functionally silent and Honorary Senior Drug resistant Lecturer, Great Candidate selection Hemispherectomy Ormond Street Structural abnormality demonstrable of one cerebral Hospital for Children Procedures on oVer can be divided broadly hemisphere NHS Trust, Institute of into two categories: resective and functional Seizures lateralised to that cerebral hemisphere Child Health, London surgery (table 1). Traditionally, consideration Pre-existing hemiplegia (in the absence of progressive disease) WC1N 3JH, UK is given to focal resection of the seizure focus, J H Cross Drug resistant either temporal or extratemporal. To be Corpus callosotomy considered for such surgery most seizures have Drop attacks, whether atonic, myoclonic or tonic Correspondence to: Specific vocational goals to surgery Dr Cross. to been proved to arise exclusively from one Update on surgery for epilepsy 357 that may occur in association with frequent STRUCTURAL IMAGING seizures in early life, clinical or subclinical. Postoperative follow up studies have shown Arch Dis Child: first published as 10.1136/adc.81.4.356 on 1 October 1999. Downloaded from A further issue may arise in deciding whether more favourable outcome with regard to seizures or seizure syndromes are focal in seizure control where lesions are detected origin and therefore treatable with surgery. within the surgical specimen.11 MRI has Certain syndromes are catastrophic in early enabled the detection of such pathology preop- childhood and the question is often how hard eratively. This has included an increased detec- should we look for a focal or lateralised onset. tion of developmental abnormalities such as Chugani et al highlighted this with the use of dysembryoplastic neuroepithelial tumours and positron emission tomography (PET) in infan- focal cortical dysplasia previously undetected tile spasms, apparently increasing the number on computed tomography, as well as hippo- amenable to surgery7; 30 of 140 of children campal sclerosis, the lesion most commonly presenting with infantile spasms had focal responsible for temporal lobe epilepsy (TLE) lateralised abnormalities. It appears likely how- in adults (fig 1).12 There continues to be ever that lateralisation may be apparent clini- discussion as to the relevance of the hippocam- cally or on EEG or both in patients where sur- pus in childhood epilepsy, and whether hippo- gery is going to be helpful and, in many, such campal sclerosis is an acquired lesion.13 Surgi- abnormalities may be seen on good quality cal series in childhood have indicated a magnetic resonance imaging (MRI).8 This relatively high incidence of tumours and a low does not preclude the fact that all children pre- incidence of hippocampal sclerosis as the senting with infantile spasms should have underlying responsible lesions, but recent data detailed neuroimaging in the form of optimised indicate that this may be equally common in MRI and, where a lateralised structural abnor- children as in adults with TLE, and that it can mality is found, referred for surgical opinion be detected on MRI (as high as 60%).12 14 even if there is initial control of seizures with Visual inspection of the hippocampus and anticonvulsants. This is to allow rapid interven- temporal lobe may also be greatly enhanced, tion should seizures return or developmental and detection of abnormalities increased by progress appear compromised with continuing quantitative and semiquantitative tech- epileptiform activity. Other syndromes that niques.15–17 Further detailed analysis of three require rigorous investigation for a localised dimensional datasets may also provide infor- structural abnormality are those that present mation, particularly in extratemporal epilepsy, with an autistic-type language disorder in not only about localised abnormalities (fig 2) association with epilepsy.9 but also about more widespread developmental Functional procedures involve modification structural abnormalities not initially apparent of brain function rather than tissue removal. from visual inspection.18 This may provide Corpus callosotomy—division (either two some prediction of the likely outcome of thirds or complete) of the corpus callosum—is surgery with regard to seizures.19 Such tech- considered in individuals having frequent niques are generally available only in centres “drop” attacks, whether myoclonic, atonic or specialising in epilepsy and epilepsy surgery, tonic. Another technique recently advocated by and therefore a normal routine MRI in the http://adc.bmj.com/ Morrell et al is multiple subpial transection, presence of clinical or EEG suspicion of focal used in the surgical management of Landau epilepsy does not preclude referral. In children KleVner syndrome (LKS).10 This procedure undergoing evaluation for hemispherectomy, involves transection of transverse fibres leaving MRI also plays a major role, not only in longitudinal fibres intact. For epileptic aphasia (LKS) the technique is performed over Wer- nickes area on what is thought to be the leading side as determined by preoperative investiga- on October 1, 2021 by guest. Protected copyright. tion. The technique may also be considered in individuals where the seizure focus lies within a functionally eloquent area of cortex (such as the motor cortex). Presurgical evaluation The
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