OBJECTIVE Despite perioperative risks, epilepsy surgery represents a legitimate curative or palliative treatment approach for children with drug-resistant epilepsy (DRE). Several factors characterizing infants and toddlers with DRE create unique challenges regarding optimal evaluation and management. Epilepsy surgery within children < 3 years of age has received moderate attention in the literature, including mainly case series and retrospective studies. This article presents a systematic literature review and explores multidisciplinary considerations for the preoperative evaluation and surgical management of infants and toddlers with DRE. METHODS The study team conducted a systematic literature review based on Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines, targeting studies that investigated children < 3 years of age undergoing surgical treatment of DRE. Using the PubMed database, investigators selected peer-reviewed articles that reported seizure outcomes with or without developmental outcomes and/or perioperative complications. Studies were eliminated based on the following exclusion criteria: sample size < 5 patients; and inclusion of patients > 3 years of age, when demographic and outcomes data could not be separated from the cohort of patients < 3 years of age. RESULTS The study team identified 20 studies published between January 1990 and May 2017 that satisfied eligibility criteria. All selected studies represented retrospective reviews, observational studies, and uncontrolled case series. The compiled group of studies incorporated 465 patients who underwent resective or disconnective surgery (18 studies, 444 patients) or vagus nerve stimulator insertion (2 studies, 21 patients). Patient age at surgery ranged between 28 days and 36 months, with a mean of 16.8 months (1.4 years). DISCUSSION The study team provided a detailed summary of the literature review, focusing on the etiologies, preoperative evaluation, surgical treatments, seizure and developmental outcomes, and potential for functional recovery of infants and toddlers with DRE. Additionally, the authors discussed special considerations in this vulnerable age group from the perspective of multiple disciplines. CONCLUSIONS While presenting notable challenges, pediatric epilepsy surgery within infants and toddlers (children < 3 years of age) offers significant opportunities for improved seizure frequency, neuro-cognitive development, and quality of life. Successful evaluation and treatment of young children with DRE requires special consideration of multiple aspects related to neurological and physiological immaturity and surgical morbidity.
Keywords: DNET = dysembryoplastic neuroepithelial tumor; DQ = developmental quotient; DRE = drug-resistant epilepsy; ECog = electrocorticography; EEG = electroencephalography; ESM = electrical stimulation mapping; ETLE = extratemporal lobe epilepsy; FCD = focal cortical dysplasia; FDG = fluorodeoxyglucose; ICEEG = intracranial electroencephalography; ICP = intracranial pressure; LTME = long-term monitoring electroencephalography; MCD = malformations of cortical development; MEG = magnetoencephalography; PET = positron emission tomography; PRISMA = Preferred Reporting Items for Systematic Reviews and Meta-Analyses; SEEG = stereotactic electroencephalography; SLA = stereotactic laser ablation; SWS = Sturge-Weber syndrome; TSC = tuberous sclerosis complex; VNS = vagus nerve stimulator; drug-resistant epilepsy; epilepsy surgery; infants; phase I evaluation; phase II monitoring; toddlers.