Surgical procedures for refractory epilepsy

Children are a high-risk group for epilepsy, and once 2 or more regular antiepileptic drug regimens fail to effectively control seizures, there is less than a 5% chance that another trial of other drugs will terminate the seizures in the long term. Thus, approximately 30% of patients will not be able to effectively control their seizures even with regular medication and will eventually develop drug-refractory epilepsy. Cohort studies have found that 8-10% of children with newly diagnosed epilepsy develop drug-refractory epilepsy after 2 years. Furthermore, further follow-up reveals that a proportion of children who previously responded well to medication will again develop drug-refractory epilepsy. If imaging reveals a structural lesion in the child, the chances that the drug will control the epilepsy are even lower. Neurodevelopmental impairment in children with epilepsy is another problem in Xuanwu Hospital of Capital Medical University. Epilepsy control early in brain development is critical for intellectual development, and in infants and children, poorly controlled epilepsy can lead to significant neurodevelopmental impairments, including cognitive, behavioral, psychosocial, and psychiatric deficits. Studies have shown an 83% prevalence of mental retardation in children with epilepsy occurring within 1 year of age; refractory epilepsy within 2 years of age, especially when seizures occur daily, is a risk factor for severe mental retardation. Because epilepsy can induce mental retardation, some researchers now view epilepsy as a progressive “brain disease”. Adolescent children with intractable epilepsy may have significant psychosocial impairment. In addition, we need to consider seizure-related disability and mortality. The mortality rate for poorly controlled childhood epilepsy is 0.5% per year, and causes include sudden epileptic death, aspiration pneumonia, trauma, and persistent status epilepticus, among others. In recent years, as epilepsy surgery continues to advance, controlling seizures in children through surgical means has become increasingly important to physicians and parents of patients. Approximately 50% of pediatric patients with drug-refractory epilepsy can have their seizures completely eradicated or effectively controlled by surgical treatment. Considering the effects of epilepsy on neurological function and the toxic effects of high doses of antiepileptic drugs, it is then an unwise choice to delay surgery by trying too many various antiepileptic drugs. Surgery: The main surgical procedures in epilepsy surgery include focal focal resection, cerebral hemispherectomy, palliative surgery, and neuromodulation surgery. Focal focal resection: This is the most commonly used and most effective surgical procedure for focal epilepsy, and the goal is to completely remove the epileptogenic focus. It can be anatomically divided into temporal lobe resection and extratemporal lobe resection. Overall, about 65% to 70% of patients can achieve good results after focal resection. Hemispherectomy: This is a special case procedure for patients with very extensive lesions involving the entire cerebral hemisphere that cannot be focally resected, such as Rasmussens encephalitis, Struge-Weber syndrome, hemispheric giant gyrus, hemispheric hemorrhage and ischemic sequelae. Hemispherectomy is one of the most characteristic surgical approaches in pediatric epilepsy surgery. It is important to note that the function of the contralateral and affected hemispheres must be evaluated preoperatively and is a good indication for surgery in patients with epilepsy who have very poor function on the affected side and whose corresponding function has been partially or mostly transferred to the contralateral hemisphere. The plasticity of the infant and child brain is more conducive to the recovery and reorganization of neurological function after surgery, providing favorable conditions for this procedure. The main surgical approaches include anatomic hemispherectomy, functional hemispherectomy with partial preservation of the lobes, and hemispheric fiber dissection with preservation of most of the lobes, which is sequentially less invasive. However, in general, these procedures are more invasive and risky than focal resections and require adequate perioperative preparation, especially in younger children. However, the efficacy of this type of surgery is positive, with a seizure disappearance rate of more than 80%. Palliative surgery: Some patients who cannot be treated with the two radical surgical procedures mentioned above may be considered for palliative surgery, which aims to reduce the number of seizures or to reduce their severity. The main application is corpus callosotomy, which is mainly used for fall attacks caused by tonic or atonic seizures. About 80% of children with fall attacks can benefit from corpus callosotomy. In patients with Landau-Kleffner syndrome or other epileptogenic foci located in important functional areas that cannot be resected, subcallosal transverse fiber dissection can be used. The most common application of neuromodulation surgery in children is vagus nerve electrical stimulation, which is a product of contemporary technological development and opens up a new idea for the future of epilepsy treatment. The vagus nerve stimulator is placed under the skin on the left side of the chest, and electrodes are implanted in the left vagus nerve, and different stimulation parameters are used for intermittent stimulation to achieve seizure control. Surgical efficacy: The effectiveness of surgical treatment of childhood epilepsy has been reported differently, which is related to the different composition of cases enrolled in each epilepsy center. In 2012, Xuanwu Hospital reported in the European Journal of Epilepsy a group of 222 children with epilepsy treated by surgical removal of the epileptogenic focus. The proportion of seizure disappearance after surgery in temporal lobe epilepsy was 77.8%, which was better than that in extratemporal lobe epilepsy. However, the proportion of children with extratemporal lobe epilepsy was higher than that of temporal lobe epilepsy. In patients with extratemporal lobe epilepsy, accurate localization of the epileptic focus and adequate resection of the epileptic focus without damaging the functional areas are sometimes difficult, which affects the surgical efficacy. On the other hand, although children with hemispheric injury are very resistant to the drug, seizures disappear in up to 89.5% of cases after undergoing hemispherectomy. The efficacy of vagus nerve stimulation in children with epilepsy, although reported inconsistently, is roughly comparable to that of adults, with typically 50% of patients having more than 50% reduction in seizure frequency and seizure severity and improvement in some types of generalized seizures. Vagus nerve electrical stimulation is generally considered more appropriate for children with catastrophic epilepsy who cannot be treated by focal resection surgery. Of course, seizure control is only one aspect of surgical efficacy. Children with epilepsy often also have learning disabilities, developmental delays, psychobehavioral abnormalities, and psychosocial problems. These problems are more common in children with early onset epilepsy and frequent seizures. Surgical procedures may not only stop seizures, but may also improve co-morbidities. Vagus nerve electrical stimulation may likewise improve the child’s social interaction and behavior, and even aggression, even if the seizures are not significantly controlled. A shift in treatment thinking: The current guideline for the treatment of refractory epilepsy in children is changing, with a rapid increase in children receiving early surgical treatment. This is based on several advances: 1. Early diagnosis of drug-refractory epilepsy. Early detection of “medication-refractory” features is important in the early treatment of childhood epilepsy. In order to make a surgical decision within a reasonable time frame, the epileptologist must have a clear understanding of the natural course of the childhood epilepsy syndrome and must distinguish between self-limiting symptoms, where remission is possible, and refractory cases, where remission is unlikely. The presence of a clear lesion is often indicative of a poor outcome. In addition, young age of onset, intellectual disability, neurological abnormalities, persistent status epilepticus, multiple seizure types, frequent seizures and significant focal EEG abnormalities at the onset of epilepsy are also risk factors for progression to drug-refractory epilepsy in children.2 Advances in epileptic focus localization techniques continue. The EEG in children is more complex and ambiguous than in adults, and advances in neuroimaging are more important for localizing epileptic foci in children. High-resolution MRI can identify more gangliogliomas, embryonic dysplastic neuroepitheliomas, focal cortical dysplasia, and other neuro-migratory disorders. The increasing familiarity of pediatric epileptologists with such lesions has also greatly improved their diagnostic rates. advances in functional imaging such as PET-CT examinations and interictal and seizure SPECT examinations have also led to increasing localization of epileptic foci in children. 3. experience and confidence in surgical treatment of infants and children is gradually accumulating. Decades of research and experience have confirmed that the efficacy of surgery for childhood epilepsy is at least comparable to that of adults. And there are specific epilepsy procedures that should be used almost exclusively in pediatric patients, such as cerebral hemispherectomy. Current data show that overall the risk of epilepsy surgery is not significantly higher in children and infants than in adult patients. However, special attention needs to be paid to the fact that infants with poor physical development have a higher perioperative mortality rate than adult patients.4. Expanded indications for surgery in children. In some children and infants, epilepsy may be due not only to severe congenital anomalies, but also to conditions that cause progressive neurological dysfunction, such as tuberous sclerosis, Sturge-Weber syndrome, Rasmussen’s encephalitis, and others. Some children may also have so-called “catastrophic epilepsy” or epileptic encephalopathy, such as West syndrome and Lennox-Gastaut syndrome. In these syndromes, in addition to refractory epilepsy, the child also shows a generalized hypofunction of the higher cortex. Therefore, in these children, early surgical treatment is associated with salvage of developmental and cognitive function in addition to seizure control.5 There is evidence that early surgical intervention improves the long-term prognosis. Clearly, if children achieve satisfactory long-term outcomes with early surgical intervention, not only can the incidence of epilepsy-related events and the adverse effects of long-term antiepileptic medication be reduced, but psychosocial aspects can also be significantly improved. It has been reported that infants and children with “catastrophic epilepsy” up to 3 years of age can experience “compensatory” rapid development after successful surgical treatment. Children with shorter histories are more likely to show postoperative increases in verbal and operational IQs. Conversely, patients approaching adulthood do not show IQ improvement despite a high rate of seizure disappearance after surgery. Issues to note: There are certainly some arguments against early surgery, such as the possibility of spontaneous remission in children with epilepsy, the possibility of future pharmacological control, the possibility of genetic metabolic disorders, the possibility of serious complications from surgery, and concerns about higher surgical disability/mortality in infants. This is a reminder that early surgical treatment of childhood epilepsy is a very complex and rigorous issue that requires not only advanced technical equipment but also extensive knowledge and experience.