When to Consider Surgery
Because infantile spasms can be catastrophic to development, surgery should be considered as soon as possible for children with focal, including unilateral or unihemispheric, brain malformations. If your child has failed vigabatrin (Sabril) and adrenocorticotropphic hormone (ACTH) or prednisolone, and the seizures are coming from a zone of cortical malformation on one side of the brain, or a specific location of the brain – do not delay! It is time for a surgical evaluation.
Referral to a surgeon for a surgical evaluation should occur when:
Your child has failed first-line treatments
No longer considered a last resort, epilepsy surgery is now a viable treatment option to stop seizures when the child’s infantile spasms are considered drug-resistant (also known as intractable or refractory). The most recent practice guidelines from the American Academy of Neurology and the Child Neurology Society for the medical treatment of infantile spasms state that adrenocorticotropic hormone (ACTH, sold as Acthar gel) and Vigabatrin (also sold as Sabril) are the first line drugs for infantile spasms. There is also some evidence that high-dose oral prednisolone can be an effective treatment as well.
One third of children presenting with infantile spasms before 36 months of age will be drug-resistant – and the largest group are children with infantile spasms. Children with focal or hemispheric lesions or malformations, such as hemimegalencephaly and cortical dysplasia, (the children most often considered for surgery) also carry a high risk of medical intractability.
Your child has a focal cortical abnormality
The ILAE recommends a surgical evaluation for that infants with drug resistant epilepsy who have focal-onset seizures – particularly those with a unilateral structural brain abnormality such as hemimegalencephaly, or focal cortical abnormalities such as the cortical dysplasias, tuberous sclerosis, and porencephaly. A presurgical evaluation will attempt to map the cortical abnormality so that the surgeon knows what part of the brain to remove.
Your child does not have a metabolic or degenerative brain condition
Children with degenerative brain conditions or metabolic disorders which cause seizures typically are not candidates for epilepsy surgery.
Your child exhibits delay or developmental arrest
In order to be a candidate for surgery, your child must be progressing very slowly or development has “frozen in time”. Most children with drug-resistant infantile spasms exhibit significant developmental delay or development arrest.
There is no unacceptable loss of function if surgery is selected
Very difficult decisions must be made – and fast – for parents considering brain surgery to stop their child’s infantile spasms. The most sobering decision to make is how much function are you willing to lose in exchange for stopping the infantile spams? Hemispherectomy, for example, results in (among other things) partial blindness and partial paralysis; however, this may be acceptable if the infantile spasms have caused or will cause serious developmental regression or arrest and, in many cases, death.
The sooner the better
Research shows that surgery should be considered as soon as possible rather than waiting months or years. This is because seizures, especially in infancy, can be catastrophic – this means that they are strongly associated with intellectual disability (formerly known as mental retardation) and poor developmental outcome.
If your child has uncontrolled infantile spasms, you are already aware that your child will probably always face physical, developmental, and/or behavioral challenges regardless of the treatments that you choose. Moderate or severe learning difficulties may be present in 70–90% of children with infantile spasms. Autism is present in up to 30% of children with epilepsy, especially in children who had seizures in the first two years of life.
It is unlikely that your child will “grow out” of infantile spasms
You may be wondering if you should wait and see if your child’s infantile spasms go away on their own. It is true that the spasm seizure type stops by three years of age in approximately one-half of children with infantile spasms and rarely continues after age five; however, 50 to 90 percent of patients with infantile spasms will go on to develop other seizure types. In general, patients with infantile spasms caused by stroke, brain malformations, or other known cause more likely to develop other seizure types than those with cryptogenic infantile spasms (57.5 versus 35.3). Approximately 27 to 50 percent of patients with infantile spasms develop a severe form of epilepsy known as Lennox-Gastaut syndrome.
Reading about the long-term consequences of infantile spasms is disheartening for any parent. However, patients with infantile spasms who are also epilepsy surgery candidates are offered a unique opportunity to permanently stop or reduce spasms and future seizure types and lower the number of anti-epileptic medications that they need to take. Seizure and medication reduction can lead to improved development and behavior that can change your child’s and your family’s quality of life for the better.
Infantile spasms and hemispherectomy surgery
W. Donald Shields, M.D., ret’d, Emeritus Professor of Neurology and Pediatrics, David Geffen School of Medicine at the University of California at Los Angeles, discusses infantile spasms, hemispherectomy, as well as the underlying etiologies that often require removing or disconnecting half the brain to stop catastrophic epilepsy. This segment provides an excellent introduction to infantile spasms, their catastrophic consequences if unabated, and hemispherectomy surgery to stop them.
For more information
Infantilespasmsproject.org: The Infantile Spasms Project is a program of the Division of Pediatric Neurology and Department of Pediatrics at the David Geffen School of Medicine and Mattel Children’s Hospital at the University of California, Los Angeles (UCLA). The information presented is intended as a straightforward and organized guide to parents and caregivers of children with infantile spasms, as well as health care professionals.
PubMed: PubMed is a service of the US National Library of Medicine® that: Provides free access to MEDLINE®, the NLM® database of indexed citations and abstracts to medical journal articles.
UpToDate: UpToDate® is an evidence-based, physician-authored resource for physicians and patients. Some content is subscription-based while others are available for a fee.
The ILAE was founded in 1909 and has more than 100 chapters worldwide. It is an association of physicians and other health professionals working toward a world where no person’s life is limited by epilepsy. ILAE’s mission is to ensure that health professionals, patients, and their care providers, governments, and the public world-wide have the educational and research resources that are essential in understanding, diagnosing and treating persons with epilepsy.
Connect with other parents
There are several websites and social media groups created by parents, for parents, that focus on infantile spasms and brain surgery, including:
- The Brain Recovery Project
- Web site: http://www.brainrecoveryproject.org/
- Facebook: https://www.facebook.com/BrainRecoveryProject/
- Twitter: @BrainRecoveryP
- Epilepsy Surgery Facebook support groups:
- Pediatric epilepsy surgery support: https://www.facebook.com/groups/254742961378861/
- Small steps – seize the day: https://www.facebook.com/groups/smallstepsseizetheday
- Groupe fermé Hémispherotomie France: https://www.facebook.com/groups/1448325795196382/
- Fundacion de Hemispherectomia en Espanol: https://www.facebook.com/groups/570764702973077/https://www.facebook.com/groups/570764702973077/
- Piece of Mind: Seizures, epilepsy, and brain surgery: https://www.facebook.com/Piece-Of-Mind-SeizuresEpilepsy-and-Brain-Surgery-127094857354002/
- Hemimegalencephaly Family Support Network
- Infantile Spasms Community
- Web site: http://www.iscommunity.org/
- Facebook: https://www.facebook.com/groups/infantilespasmscommunity/
- The Lennox-Gastaut Syndrome Foundation
- Tuberous Sclerosis Alliance Web site: http://www.brainrecoveryproject.org/
- Hemispherectomy Foundation
- Web site: http://hemifoundation.homestead.com/
- Facebook: https://www.facebook.com/groups/29122689930/
Pellock JM, Hrachovy R, Shinnar S. Infantile spasms: a U.S. consensus report. Epilepsia. 2010 Oct;51(10):2175-89.