Australasian Journal of Neuroscience Volume 30 ● Number 2 ● Oct 2020

Epilepsy Surgery: A Paediatric Perspective

Lauren Bollard 1, Emily Moore 1, Rebecca Paff 1

1Clinical Nurse Educator, Commercial Travellers Ward, The Children's Hospital at Westmead.

Abstract

250,000 people in Australia live with and more than 40% of those are children. Medication is a first line, effective treatment. However, not all patients have the desired outcome of re- duction or cessation. In fact, 1 in 3 do not gain full seizure control with medication alone.

Epilepsy surgery, while not a new concept of treatment for seizure management, has gained signifi- cant traction in the past decade and has become a particular focus of the Neurology Department at The Children’s Hospital, Westmead. This is evident by the expansion of the Neuroscience ward for the precise purpose of surgical intervention for the treatment of Epilepsy and other seizure condi- tions.

The paediatric patient journey to surgery is a complex and intricate one. It involves a collaborative approach of the multidisciplinary teams from diagnosis and beyond, whilst maintaining a high stand- ard of holistic, family centred care. This paper aims to discuss this journey and the impact it has on the patients, families and the nurses involved.

Key Words

Epilepsy, , surgery, Stereotactic Electroencephlagram (EEG)

Introduction: 20 years of age. (Epilepsy Action Australia, 2019; Sheng, et al. 2017; Kwan et al., 2010). Seizures are the most common neurological The consequences of uncontrolled seizures condition worldwide and the most common in a child can differ greatly due to the group of neurological emergency in children. While symptoms that vary in frequency and intensi- these medical emergencies are stressful, ty. The most obvious is recurrent seizure ac- they are not all the same and can be divided tivity and the disruption this has to everyday into two categories based on chronicity life, cognitive impairment, poor school attend- (Stafstrom & Carmant, 2015; Chiou & Hsieh, ance and subsequent educational challenges. 2008). A “seizure” is a transient, paroxysmal Even children with normal intelligence levels alteration of neurologic function which may be have a higher risk of learning difficulties. Ad- provoked by a non-epileptic or reversible in- ditionally, uncontrolled seizures can lead to sult such as trauma, hypoglycemia, or more emotional and behavioural disturbances such commonly in children, febrile illness as increased risk of anxiety, depression, irri- (Stafstrom & Carmant, 2015). Whereas, tability, hyperactivity, aggression and atten- “epilepsy” (or epilepsy syndrome) is an en- tion disorders. These challenges combined during repetitive predisposition to unprovoked with social stigma, disability and death further seizures due to complex genetic or structural impact on the overall quality of a child’s life causes, or damage to the delicate tissues (Epilepsy Action Australia, 2019; Sheng, et al. and structures of the brain (Stafstrom & Car- 2017; Kwan et al., 2010). mant, 2015). Seizures have a lifetime inci- dence of almost 9% and epilepsy has a life- Questions or comments about this article should be time incidence of 3% worldwide (Epilepsy directed to Vicki Evans Action Australia, 2019). Email address: [email protected]

Epilepsy effects250 thousand people in Aus- DOI: 10.21307/ajon-2020-007 tralia alone and half of these present before Copyright © 2020ANNA

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70% of children presenting to an emergency An Overview of Stereoelectroencephalog- department in NSW with a non- raphy (SEEG) Monitoring: event will respond to a single medication. The other 30% require poly-pharmaceutical SEEG has been used in Europe since the management to control seizure activity. 1960’s (Batchelder, 2017). The Cleveland (Epilepsy Action Australia, 2019; Sheng, et Clinic was the first centre to use this proce- al. 2017; Kwan et al., 2010). Of this 30%, half dure in North America in 2009, and The Chil- will have refractory or intractable Epilepsy; dren’s Hospital Westmead was the first to where seizure control is not achieved despite perform a paediatric case in Australia in 2011 multiple interventions including trial of two or (Alomar, Mullin, Smithason & Gonzalez- more anti-epileptic medications and dietary Martinez, 2018; Sala-Pedro et al., 2019). changes such as Ketogenic and Modified SEEG is an invasive surgical procedure used Atkins Diet (Epilepsy Action Australia, 2019; to localize the patient’s epileptogenic zone Sheng, et al. 2017; Kwan et al., 2010). It is (the area where a seizure starts), determine this cohort of paediatric patients who may its relationship with the eloquent cortex, and benefit from surgical treatment, as their determine whether the candidate is suitable brains are capable of reorganizing their neu- for a tailored surgical resection (Minotti, Mon- rologic function post-operatively (Sheng et al, tavont, Scholly, Tyvaert & Taussig, 2018). It 2017; Cross et al., 2006). Many types of epi- involves using three-dimensional imaging to lepsy surgery exist including temporal resec- place a stereotactic frame around the skull to tions, excision of seizure provoking lesions or plan intracerebral trajectories of depth elec- developmental malformations, sectioning of trodes (Batchelder, 2017; Ho et al., 2018). the corpus callosum and hemispherectomies These electrodes have a precision of 3mm of (Dwivedi, etal. 2017). Without such interven- the epileptogenic zone (Mullin et al., 2016). tion, uncontrolled seizures may create signifi- Consequently, if the area of cerebral dysfunc- cant disruptions to cognition, achievement of tion can be identified, the potential risks of developmental milestones, and mental surgery-related deficits are deemed to be health; and impair the individual’s quality of acceptable, and it is safe to remove the asso- life particularly when the seizures are pro- ciated brain tissues, then surgery will be un- longed, frequent and associated with Status dertaken with a view to eliminating the area Epilepticus (Sugano & Arai, 2015; Harden, of misfiring of neurons which in turn prevents Black & Chin, 2016). the undesired electrical activity from com- mencing and spreading, and therefore elimi- Epilepsy surgery has been shown to be a nates seizure activity altogether (Ho et al., highly effective method of achieving seizure 2018; Ritaccio, Brunner & Schalk, 2018). freedom in children with focal, drug re- sistance epilepsy (Jayakar, et al. 2014). Un- Advantages of SEEG: fortunately, not all children with refractory epilepsy are candidates for surgical interven- The advantages of SEEG include reduced tion due to the multi structure involvement. morbidity, lower discomfort, better tolerability Hence, a detailed surgical work-up which of electrodes, the capacity for deeper map- involves a multitude of non-invasive tests ping of brain structures and accuracy while such as Video Electroencephalogram moni- completing a surgical evaluation for epilepsy toring (VEEG), magnetic resonance imaging (Minotti, et al. 2018). Patients can be cared (MRI) scans, functional MRI, Positron Emis- for in the ward and have a shorter length of sion Tomography (PET) scan, Single-Photon stay (Iida & Otsubo, 2017). Emission Computerized Tomography Another advantage of SEEG monitoring is (SPECT) scan, a neuropsychology assess- the capacity to perform cortical stimulation. ment, and a baseline physiotherapy assess- The purpose of cortical stimulation (also ment, is necessary to determine whether the known as electrical stimulation mapping), is child would benefit from epilepsy surgery to guide neurosurgical resective strategies by (Chassoux, Navarro, Catenoix, Valton & identifying areas of cortical dysfunction and Vignal, 2017). Should these non-invasive mapping the electrophysiological pathways assessments fail to correctly localize the epi- associated with the eloquent functions of the leptogenic zone, or if further information is individual’s brain so that post-operative sen- required to clarify and support the data col- sorimotor and linguistic deficits are minimized lected through previous testing, invasive Ste- or eliminated (Ritaccio, Brunner & Schalk, reoelectroencephalography (SEEG) monitor- 2018). This is achieved via application of ing may be required (Alomar, Mullin, electrical stimuli via the depth electrodes in- Smithason & Gonzalez-Martinez, 2018). hibit or excite brain functions, usually while the patient is doing a language or behavioral test and observing the EEG and patient re-

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sponse (So & Alwaki, 2018; Ritaccio, Brunner Neurophysiologists to pinpoint the epilepto- & Schalk, 2018). Hence, if a child is not ac- genic zone of the brain responsible for sei- tively having seizures, targeted parts of the zure events (The Children’s Hospital at West- brain can be stimulated to generate a seizure mead, 2012). Between 2011 and October response (Ritaccio, Brunner & Schalk, 2018; 2019, the team at The Children’s Hospital at So & Alwaki, 2018). This helps to identify the Westmead performed 116 epilepsy surgery focal point if the child is not having spontane- operations and completed 23 SEEG evalua- ous seizure activity. The procedure is carried tions. A multitude of resources are required out with the Neurology consultant and a team to ensure that the best service possible is of doctors and Neurophysiologists at the bed- provided and expedite surgery. side. In 2011, the largest room on the Neurosci- Disadvantages of SEEG: ence unit was converted into an Epilepsy Monitoring Unit with the capacity for Video Due to its invasive nature, SEEG Monitoring Electroencephalogram (VEEG) monitoring for carries inherent risks including dislodgement up to four concurrent patients. Stafstrom, & of leads, electrode failure, intracranial hemor- Carmant (2015) explain VEEG is a video re- rhage and infection (Kamitaki, Billakota, cording of the brain’s electrical activity. It can Bateman & Pack, 2018; Mullin et al, 2016). In detect abnormal electrical activity, such as the paediatric population, the child’s compli- focal spikes or waves (consistent with focal ance is also a significant consideration for epilepsy), or diffuse bilateral spike waves SEEG suitability as their movements are re- (consistent with ). Straf- stricted to the bed or the immediate sur- strom & Carmant further state that VEEG rounding areas for a minimum of seven days allows recording of longer periods of time due to the electrodes, and they need to be including wakefulness, drowsiness, and sleep tethered to the monitoring unit so that data because the prevalence of epileptiform ab- can be collected (Batchelder, 2017). A bed- normalities varies in these different states of side sitter is therefore required at all times to consciousnesst includes infrared cameras for ensure that the patient’s intracranial elec- better viewing of nocturnal events, continu- trodes are not displaced or dislodged as a ous back to base cardiac, respiratory and result of normal childhood behaviours, or saturation monitoring and remote access confusion as a part of the post-ictal period monitoring so the scientific officers and doc- (Kamitaki, et al., 2018). The procedure is tors on call can view events when not on hos- contraindicated in patients with a skull thick- pital grounds. The establishment of the EMU ness of less than 2mm (usually children un- also required the implantation of an after- der three years of age), as their fragile crani- hours troubleshooting service so valuable um can fracture and prevent the intracranial recording time is not lost should a disruption electrode from being adequately secured occur out of hours. The neuroscience depart- (Minotti et al., 2018, Ho et al., 2018). ment also offers a portable VEEG monitoring outreach service, which enables patients in Development of a Specialized Paediatric other areas of the hospital such as the emer- Epilepsy Monitoring Unit at The Children’s gency department or Paediatric Intensive Hospital at Westmead (CHW) : Care Unit to be monitored. (SCHN, 2019). In addition to the four-bed EMU, a single moni- The International League Against Epilepsy toring room was upgraded to accommodate (ILAE) Paediatric Epilepsy Surgery Recom- VEEG monitoring and newly available Stere- mendations, determined that dedicated pae- otactic Electroencephalogram (SEEG). diatric epilepsy surgery centers were neces- sary as the neurobiological aspects of child- Furthermore, the proximity and layout of hood epilepsy are unique to children and thus Westmead Hospital and The Children’s Hos- require specialized care (Cross et al., 2006). pital Westmead creates a unique position of The Children’s Hospital at Westmead (CHW), having a paediatric and adult epilepsy unit in located in New South , Australia, con- the one campus. This allows for a network of tains one such example of this; a 22-bed spe- experts to meet regularly and discuss cases. cialized paediatric Neuroscience unit that Families are often comforted by this treats and cares for patients between the ag- knowledge that their child is getting excellent es of 0-17 with a range of Neurological and care in a state-of-the-art location with multiple Neurosurgical concerns (SCHN, 2019). With- experts covering the full patient journey. The in the ward sits a four-bed Epilepsy Monitor- complexity of presenting cases is equal to ing unit and laboratory that was upgraded in that of leading surgical centres world-wide 2011 to include innovative technologies that and the surgical outcomes are on par with enable the Neurologists, Neurosurgeons and those centres at around 70% to 80% for spe-

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cific types of Epilepsy such as Temporal cus on the surgical rationale and to manage Lobe Epilepsy (Lee & Lee, 2013). patient and family expectations. Patient and family-centred care is an approach to health The CHW Stereotactic Electroen- care that empowers patients and their fami- cephlagram (SEEG_ Protocol): lies and fosters independence. It supports family care-giving and decision-making. The adoption of SEEG has required wide- (Harden, Black, & Chin, 2016), It respects spread changes in the nursing care of pa- patient and families’ choices, their values, tients on the ward including the development beliefs and cultural backgrounds. It builds on of new policies and procedures to ensure that individual and family strengths and involves best practice is maintained. Post operatively, patients and their families in the planning, the patient is admitted to the EMU from re- delivery and evaluation of health care ser- covery into the specialised single room and vices. (Harden, Black, & Chin, 2016), nursed at a 1:1 ratio for the first 48 hours. After this time, the patient is reassessed to Case Study: determine if the 1:1 ratio is still required. If it is deemed safe without a nurse in the room, Patient X is a 9-year-old girl who has had then a sitter must always be present, and this epilepsy since the age of 5. She has had no is usually a parent or guardian. Kamitaki, prior , febrile event and no Billakota, Bateman, L., Pack, (2018) explain family history of seizures. She had failed four the increase in safety for the patient with medication trials and in March 2012 had epi- these measures in place. The patient is lepsy surgery for resection of right temporal placed on a strict regime of neurological ob- cortical dysplasia. She remained seizure free servations including continuous cardio respir- for 6 months on Carbamazepine. atory and oxygen saturation monitoring. They also receive prophylactic antibiotics and In September 2012 she began to have recur- aperients and are on bed rest with toilet privi- rent weekly events which were difficult to leges. The patient remains on their regular characterise. She had changes to her vision anti-epileptic medications at the discretion of where “things became very blurry and ap- the and diet as tolerated. If peared close up”. She sometimes saw medication withdrawal is deemed necessary “rainbow colours” at the start of a seizure and the patient has intravenous cannula for im- felt “like clouds were all around her”. She al- mediate access should an adverse event oc- so had non-sustained head turning to the cur. Furthermore, nurses carry out hourly right. These ongoing events required further documentation of functioning equipment, investigating. maintain a seizure safe environment and re- ceive widespread education on VEEG moni- After significant testing in 2013 it was hypoth- toring and how to safeguard equipment and esised that there was an area of dysplasia proper functioning. (SCHN, 2019) around the previous surgical site but that The Neurosurgical Registrar and Epilepsy more extensive exploration of other areas Fellow are on call and must be notified of any would need to be considered. It was then seizure activity. These practices have been decided that she would proceed to Stereotac- developed in consultation with the entire tic EEG monitoring in 2014. Patient X had Neuroscience team and are regularly re- SEEG implantation in April 2014 and had viewed to ensure they are the most appropri- seven days of monitoring. This included stim- ate and safest course of action. This is ulation to elicit seizure response and to ce- demonstrated during monthly Neurosurgical ment all previous findings of a residual epi- safety meetings. (SCHN, 2019). lepogenic area. The electrodes were re- moved at the conclusion of monitoring and Benefits of the Family Centred Care Phi- she was discharged home the following day. losophy of CHW: Her sutures were removed by her GP and she continued on Carbamazepine. Five The goal of all patient interventions is to im- weeks later she underwent a craniotomy and prove overall quality of life and lessen the resection of epileptogenic right posterior tem- seizure burden. Comprehensive patient care poral lobe. She had an uneventful recovery involves developing a relationship with both and was discharged home 5 days later on the child and family, understanding the child Carbamazepine. To date the patient remains and the dynamics of their particular family, seizure free. working within these challenges and main- taining clear communication. This builds trust Patient X’s parents conveyed that decision and a mutually respective relationship and making was the hardest part. It was “a gut- enhances the outcomes for the child. Keep- wrenching decision” but expressed a sense ing lines of communication open helps to fo- of relief once the decision was made. They

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stated that they “placed enormous faith in Tripathi, M. (2017). Surgery for drug-resistant both the neurologist and neurosurgeon”. . New Journal of They stated feeling safe in the familiarity of Medicine, 377 (17). 1639-1647. doi:10.1056/ the hospital environment they had become nejoa161335 used to and the clinicians that they already knew. They felt comforted by the reassur- Epilepsy Action Australia, (2019, Sept). Facts ance and support they were given throughout and statistics. Retrieved from https:// the process. www.epilepsy.org.au/about-epilepsy/facts- and-statistics/ Conclusion: Epilepsy Action UK, (March, 2019) Education Due to the extensive influence of refractory and epilepsy. Retrieved from https:// Epilepsy on the life and future of a child, after www.epilepsy.org.uk/info/education/learning- other treatment options have been exhaust- and-behaviour. ed, surgical intervention is considered. The diagnosis, dynamic management plan and Harden, J., Black, R., & Chin, R., (2016), possibility of surgery can be a daunting and Families’ experiences of living with paediatric complex construct for any one with epilepsy. epilepsy: a qualitative systematic review. Epi- The disease pathway of a child can be even lepsy & Behaviour, 60. pp. 225-237. doi: more complex and has its unique challenges. http://dx.doi.org/10.1016/j.yebeh.2016.04.034 With the assistance of advanced imaging techniques, a multidisciplinary as well as a Ho, A., Feng, A., Kim, L., Pendharkar, A., family centred approach, clinicians can offer Sussman, E., Halpern, C., & Grant, G. progressive and radically life changing op- (2018). Stereoelectroencephalography in tions that shift this disease from a purely children; a review. Neurosurgical Focus, 45 management perspective to a disease with a (3). doi: http://thejns.org/doi/ possible cure in the form of surgical interven- abs/10.3171/2018.6.FOCUS18226 tion. Iida, K & Otsubo, H. (2017) Stereoenceph- References: alography: indication and efficacy. Neurologi- ca medico-chirurgica. 57 (8), 375-385. Alomar, S., Mullin, J., Smithason, S., & Gon- Jayakar, P., Gaillard, W. D., Tripathi, M., zalez-Martinez, J. (2018). Indications, tech- Libenson, M. H., Mathern, G. W., & Cross, J. nique, and safety profile of insular stereoe- H. (2014). Diagnostic test utilization in evalu- lectroencephalography electrode implanta- ation for resective epilepsy surgery in chil- tion in medically intractable epilepsy. Journal dren. Epilepsia, 55(4), 507–518. doi:10.1111/ of Neurosurgery, 182. pp. 1147-1157. epi.12544 doi:10.3171/2017.1.JNS161070 Kamitaki, B., Billakota, S., Bateman, L., Pack, Batchelder, P. (2017). Surgical Management A., (2018). Addition of a hospital bedside sit- of Epilepsy. In C. Cartwright, & D. Wallace ter during intracranial monitoring improves (eds), Nursing care of the Pediatric Neuro- safety and seizure responses in an adult epi- surgical Patient, pp. 457-492, Springer Inter- lepsy monitoring unit. Epilepsy & Behaviour, national Publishing, AG. 86. 15-18. doi: https://10.1016/ Chassoux, F., Navarro, V., Catenoix, H., Val- j.beh.2018.07.002 ton, L, Vignal, J. (2018). Planning and man- Kwan, P., Arzimanoglou, A., Berg, A.T., Bro- agement of SEEG. Neurophysiologie die, M.J., Allen H.W., Mathern, G.,…,French, Clinique/Clinical Neurophysiology, 48. 25-37. J. (2010). Definition of drug resistant epilep- doi: https://doi.org/10.1016/ sy: consensus proposal by the ad hoc task j.neucli.2017.11.007 force of the ILAE commission on therapeutic Cross, J.H., Jayakar, P., Nordli, D., strategies. Epilepsia, 51(6), 1069-1077. doi: Delalande, O., Duchowny, M., Weisre, H.,…, http://dx.doi.org/10.1111/ j.1528- Mathern, G., (2006). Proposed criteria for 1167.2009.02397.x referral and evaluation of children for epilep- Lee, Y.J., Lee J.S. (2013) Temporal lobe sy surgery: recommendations from the sub- epilepsy surgery in children versus adults: commission for pediatric epilepsy surgery. from etiologies to outcomes. The Korean Epilepsia, 47 (60). doi:https:// Paediatric Society, 2013;56(7):275-281 doi.org/10.1111/j.1528-1167.2006.00569.x Minotti, L., Montavont, A., Scholly, J., Dwivedi, R., Ramanujam, B., Chandra, P., Tyvaert, L., & Taussig, D. (2018), Indications Sapra, S., Gulati, S., Kalaivani, M., …

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and limits of stereoelectroencephalography So, E., & Alwaki, A. (2018). A guide for corti- (SEEG). Neurophysiologie Clinique/Clinical cal electrical stimulation mapping. Journal of Neurophysiology, 48. pp 15-24. doi: https:// Clinical Neurophysiology, 35 (2). pp 98-105. doi.org/10.1016/j.neucli.2017.11.006 doi:10.1097/wnp.0000000000000435

Mullin, J.P., Shriver, M., Alomar, S., Najm, I., Stafstrom, C., & Carmant, L. (2015). Sei- Bullicio, J., Chauvel, P. & Gonzalez- zures and epilepsy: an overview for neurosci- Martinez,J. (2016) Is SEEG safe? A system- entists. Cold Spring Harbor Perspectives in atic review and meta-analysis if stereo- Medicine, 5 (6). doi:10.1101/ -related com- cshperspect.a022426 plications. Epilepsia, 57 (3): 386-401. Sugano, H., &Arai, H. (2015). Epilepsy sur- Ritaccio, A, Brunner, P., & Schalk, G., gery for pediatric epilepsy: optimal timing of (2018). Electrical stimulation mapping of the surgical intervention. Neurologia Medico- brain: basic principles and emerging alterna- chirurgica, 55(5). 399-406.doi: 10.2176/ tives. Journal of Clinical Neurophysiology, 35 nmc.ra.2014-0369 (2). doi:10.1097/WNP.0000000000000440 The Children’s Hospital at Westmead (2012). Sala-Padro, J., Fong, M., Rahman, Z., Bart- The Children’s Hospital at Westmead 2012- ley, M., Gill, D., Dexter, M.,…, Wong, C. Fundraising Annual Review. Retrieved from (2019). A study of perfusion changes with https://d2qicjz8gxf4ne.cloudfront.net/ insula epilepsy using SPECT. Seizure: Euro- eeb81662db562f6dc1446b27e8a1fd23.pdf pean Journal of Epilepsy, 69, pp.44-50. doi: https://doi.org/10.1016/j.seizure.2019.03.021 The Sydney Children’s Hospital Network (accessed Oct 2019). Patient and family cen- Sheng, J., Liu, S., Qin, H., Li, B., & Zhang, X. tred care fact sheet. Retrieved from https:// (2018). Drug-resistant epilepsy and surgery. www.schn.health.nsw.gov.au/files/ Current Neuropharmacology, 16. 17-28. doi: attachments/ 10.2174/1570159X15666170504123316 patient_and_family_centred_care.pdf

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