Epilepsy Surgery in Tuberous Sclerosis Complex: Results from the Pediatric Epilepsy Research Consortium Epilepsy Surgery Database
Abstract number :
3.316
Submission category :
9. Surgery / 9B. Pediatrics
Year :
2021
Submission ID :
1826743
Source :
www.aesnet.org
Presentation date :
12/6/2021 12:00:00 PM
Published date :
Nov 22, 2021, 06:56 AM
Authors :
Krista Eschbach, MD - University of Colorado School of Medicine - Children's Hospital Colorado; Samir Karia - Neurology - Norton Children's Hospital; Jeffrey Bolton - Neurology - Boston Children's Hospital; Michael Ciliberto - Pediatrics - University of Iowa Hospitals and Clinics; Jason Coryell - 17Doernbecher Children’s Hospital, Oregon Health and Sciences University; Dewi Depositario-Cabacar - Center for Neuroscience - Children's National Hospital; Satyanarayana Gedela - Pediatrics - Emory College of Medicine, Children's Healthcare of Atlanta; Zachary Grinspan - Weill-Cornell Medicine; Jennifer Koop - Department of Pediatric Neurology - Children’s Hospital of Wisconsin, Medical College of Wisconsin; Ahmad Marashly - Pediatrics - University of Washington, Seattle Children's Hospital; Patricia McGoldrick - Boston Children's Health Physicians; Nancy McNamara - Department of Pediatrics, Division of Pediatric Neurology - Michigan Medicine, University of Michigan; Srishti Nangia - Weill-Cornell Medicine; Adam Ostendorf - Department of Pediatrics, Division of Neurology - Nationwide Children's Hospital; Shilpa Reddy - Neurology - Vanderbilt University Medical Center, Monroe Carell Jr Children’s Hospital; erin Romanowski - Department of Pediatrics, Division of Pediatric Neurology - Michigan Medicine, University of Michigan; Sabrina Shandley - Justin Neuroscience Center - Cook Children’s Medical Center; Rani Singh - Division of Neurology - Atrium Health Levine Children's Hospital; Joseph Sullivan - University of California San Francisco Weill Institute for Neurosciences - Benioff Children’s Hospital; Daniel Shrey - Children's Hospital of Orange County; Priya Tatachar - Pediatrics - Ann and Robert H Lurie Children’s Hospital; Steven Wolf - Boston Children's Health Physicians; Lily Wong-Kisiel - Department of Neurology, Divisions of Child Neurology and Epilepsy - Mayo Clinic College of Medicine; Scott Perry - Justin Neuroscience Center - Cook Children’s Medical Center; Allyson Alexander - Department of Neurosurgery, Division of Pediatric Neurosurgery - University of Colorado Anschutz Medical Campus, Children's Hospital Colorado
Rationale: Tuberous sclerosis complex (TSC) is a leading cause of genetic structural epilepsy in childhood and often results in drug-resistant epilepsy. Center-to-center variations in care are understudied, including pre-surgical evaluation, recommended surgical treatments, the use of palliative resections, and surgical outcomes.
Methods: Patients 0-18 years of age with TSC were included from the Pediatric Epilepsy Research Consortium (PERC) Epilepsy Surgery Database, a prospective cross sectional study enrolling patients undergoing epilepsy surgery evaluation at 20 US pediatric epilepsy centers. Predefined variables collected included demographics, epilepsy characteristics, presurgical treatment, epilepsy evaluation, goal of surgery (palliative vs definitive), and outcome of surgery. Data was obtained from database initiation (1/2018) to present (5/2021).
Results: Of 1037 patients in the database, 36 patients had TSC. The cohort is described in Table 1 and includes 21 males (58%) with a median age of seizure onset at 0.3 years. Patients failed a second anti-seizure medication (ASM) at a median age of 1.5 years (n=28). Referral for phase 1 evaluation occurred at a median age of 4.1 years. Children were on a median of 3 anti-seizure medications at the time of phase 1 evaluation, with daily seizures in 29 (81%). Nine (25%) children had undergone prior epilepsy surgeries including vagus nerve stimulation (VNS), callosotomy, and lesionectomy or more than one of those procedures. Fifteen (42%) children received no additional testing after the Phase 1 monitoring as part of surgical planning. An additional 15 (42%) children received some type of additional presurgical evaluation (PET, MEG, or SPECT) and rarely children received all three modalities of additional testing (Table 1). Epilepsy surgery was offered to 25 (69%) of children (Table 2). Of these, a 2-stage surgery was recommended in 15 (60%) and performed in 13. Sixteen (44%) children underwent epilepsy surgery including lesionectomy or tuberectomy (n=9), lobectomy (n=4), laser ablation (n=2), callosotomy (n=1) and vagus nerve stimulation (n=1). Only 6 (38%) procedures were intended to be definitive and result in sustained seizure freedom. Nine (36%) families either declined surgery or are awaiting surgery at this time. Six children have at least 6 months of follow-up since epilepsy surgery (Table 2), of whom one is seizure free, one has more than 50% seizure reduction, three have less than 50% seizure reduction, and one has undergone an additional surgery.
Conclusions: Children with TSC referred for consideration of epilepsy surgery have a very early onset of drug-resistant epilepsy with a high seizure burden. Epilepsy surgery, which most often consists of resections, is offered to most children who undergo phase 1 evaluation. The extent of additional presurgical testing is variable and surgery for children with TSC is often intended to be palliative. Ongoing follow-up of this cohort will provide insight into the potential benefits of palliative surgery for children with TSC.
Funding: Please list any funding that was received in support of this abstract.: None.
Surgery