Surgery is considered for children with medically intractable epilepsy declining neurological function, or syndromes for which medical treatment is known to be ineffective. These determinations require accurate classification of the epilepsy syndrome, knowledge of the natural history, response to anti-epileptic drug trials, and serial assessments of development. Many types of infantile and early childhood epilepsies are hard to classify and of uncertain prognosis.

Although evidence shows that children (even under age 3) who undergo epilepsy surgery soon after epilepsy has started have better outcomes, surgery is not offered as commonly as it should be. [Pindrik: 2018] [Kadish: 2018] [Englot: 2012] Also, there can be significant delays before referral; often, up to 60 months and a process where 6 anti-epileptic medications have been tried before referral to an epilepsy surgery team. [Prideaux: 2018] Drug-Resistant Epilepsy (ILAE) defines drug resistance as failing to respond to 2 well-selected anti-epileptic drugs used in isolation or any combination. Also see [Kwan: 2010].

Because the frequency of seizures in children is often high, the determination of drug resistance need not take years and, often, can be established within a year of epilepsy onset.

Vagal nerve stimulation (VNS) can be used in generalized or focal epilepsy. [Welch: 2018] A VNS reduces seizure frequency by sending regular, low-amplitude pulses of electricity to the brain via the vagus nerve. A stimulator device is implanted under the skin in the chest and is about the same size as a cardiac pacemaker. A wire from the device is wound around the vagus nerve in the neck. It is a palliative, not curative, procedure that has been performed in adults and in some children with intractable complex partial seizures or generalized tonic seizures who were not candidates for definitive surgical cure. Why this works is not well understood.

Although the VNS has been placed in children younger than 3 years old [Zamponi: 2008], it is usually placed in older children. The VNS is generally well-tolerated and safe for children and adults. The median reduction of seizure frequency is about a third at 1 year and 40% by 18 months. Several months may go by before there is any change, followed by a slow but steady improvement in seizure frequency. In 1 study, 60-90% of children showed a reduction in seizure frequency. [Zamponi: 2008] [Fan: 2018] Complications that occur with VNS use include failure of the device to work (3%), infections (3%), and stimulus-associated hoarseness and swallowing problems. [Smyth: 2003] Sleep-related breathing problems have also been described. [Hsieh: 2008] Evaluation for and treatment with VNS for children is performed in epilepsy clinics. The VNS is reserved for children with intractable seizures. Frequent follow-up with pediatric neurology will be necessary after insertion of the VNS.

Deep brain stimulation (DBS) involves implanting a device to stimulate the anterior nucleus of the thalamus involved in the spread of an initially localized seizure. [Yan: 2018]

Responsive neuro-stimulation (RNS) is a relatively new therapy. A device is placed in the skull that is connected to electrodes on top of the brain or inside the brain with the placement tailored to the individual. It monitors brain waves, and if seizure activity is detected, it stimulates the brain to try to counter the seizure activity.

Lobectomy/lesionectomy (most frequently involving the temporal lobe) is a procedure in which a specific lobe (or lesion within a lobe) of the brain is removed. This is successful in many cases, especially when a lesion is identified on MRI. [Stevelink: 2018] The percentage of cure or significant seizure reduction varies with different criteria/methods.

Corpus callosotomy is a palliative surgery in which the main connection between the right and left halves of the brain is cut. It is used most often to reduce injuries related to abrupt drop seizures, but seizure freedom should not be expected.

Hemispherectomy/hemispherotomy is a procedure in which half of the brain is removed or disconnected from the surrounding brain. This is a palliative surgery that may result in a more significant functional loss (peripheral vision loss, weakness) on the opposite side of the body, but it also has remarkable success in stopping seizures (60-80% depending on circumstances). This has shown to be particularly effective when a prior stroke has caused epilepsy or in epilepsy caused by hemimegalencephaly or Rasmussen’s encephalitis.

If surgery is being considered for a child with seizures, the families can have a reasonable expectation of seizure elimination or substantially fewer disabling seizures, which should improve the quality of life and perhaps help development. There should be minimal risk of significant loss of neurologic function. Children with complete resections of focal structural lesions identified by MRI fare best and have seizure-free rates as high as 80%. [Mehvari: 2019]

Many of these lesions are congenital, slow-growing tumors or cerebral dysgenesis. Mesial temporal sclerosis (a common indication in adult epilepsy surgery) is less frequent in children and adolescents, but it has a very favorable surgical outcome. Without a clear lesion on MRI, complete long-term seizure freedom following surgery is less likely (at times only 30-50%); however, it still has a higher likelihood of seizure freedom when compared to continued medication trials (estimated at less than 10%).