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The two surgical options used most commonly for patients with LGS are corpus callosotomy or vagus nerve stimulation (VNS) therapy. Rarely, patients with LGS may be a candidate for focal removal of the brain tissue initiating the seizures.
Corpus callosotomy has been used for several decades as a beneficial procedure to help reduce the drop attacks and the convulsive seizures in children with LGS. For the child with LGS, a complete corpus callosotomy is performed. The child will still have an ongoing need for medical therapy after the surgical procedure, but many children have dramatic improvement in their drop attacks and the frequency or strength of their convulsive seizures. Before surgery these children are typically evaluated in an Epilepsy Monitoring Unit to document all their seizure types and to clarify which seizure types are expected to respond to corpus callosotomy.
The child is typically admitted to surgery the day of the corpus callosotomy. The procedure normally takes about 4-5 hours to perform. The recovery in the hospital after surgery can vary from 5 days up to one week. Some children may have a temporary need for increased rehabilitation services (speech, physical and occupational therapies) after this procedure. However, essentially, all children should return to their level of functioning prior to surgery.
Vagus nerve stimulation (VNS) therapy has also been shown to be effective for multiple seizure types associated with LGS. Again, the family should be given information on this procedure and be directed to the website (www.vnstherapy.com).
Vagus nerve stimulation seems to be most effective as a treatment for drop attacks but can also be effective for the partial seizures and the generalized seizures. Some children may require both corpus callosotomy and vagus nerve stimulation, performed typically at separate times, as they could have a beneficial response to one and less dramatic response to the other, and unfortunately, this cannot be predicted in advance. Additionally, some children show improvement in seizure control after each procedure. The response to corpus callosotomy is noted within days after the surgery and the ongoing control of seizures is usually evident over the first 3-6 months after surgery.
With vagus nerve stimulation, the family again has indication of the extent of improvement of seizure control over the first six months but there may be continued improvement over the next twelve months. As such, the family and their pediatric neurologist can evaluate the response that has occurred the first six months after either procedure, and make decisions if they need to proceed to the other procedure at that time, or other therapies.
Once a decision has been made to pursue vagus nerve stimulation, the child is typically admitted to day surgery for a short hospital stay. The procedure itself typically takes 40 minutes to one hour to complete. The child may be observed overnight at the hospital or discharged home from day surgery. The child is then seen for follow up by the neurologist who makes adjustments to the vagus nerve stimulator, with increases in the dose of the device as tolerated by the child, similar to what is done with medications. The current battery life for the vagus nerve stimulator is 4-8 years, depending on the device settings. In children who respond to this treatment, a generator replacement is required before the battery runs out. This is a brief surgical procedure taking approximately 10-15 minutes to perform. Vagus nerve stimulation tends to be well tolerated. Some children may have a change in their voice or have a tickling sensation with coughing during stimulation, but adjustments to the device’s parameters can minimize or prevent these side effects. Some families note improvement in alertness, and behavior, in addition to seizure control with vagus nerve stimulation.
Author: James Wheless, M.D.
Topic Editor: Robert Fisher, M.D., Ph.D.
Last Reviewed: 8/7/09
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