CLINICAL ARTICLE J Neurosurg Pediatr 26:157–164, 2020 S eizure disorders in childhood can either follow a be- nign and relatively inconsequential course or have signifcant deleterious effects on neurocognitive de- velopment. These latter epilepsies are so-called catastroph- ic. 1–4 Despite maximal medical management, a signifcant number of infants (roughly 25%) continue to progress with seizures. 5–8 The considerable adverse effects of unrelenting seizure activity on the developing brain result in cognitive, psychological, and functional impairment and have been associated with up to a 20% risk of mortality in some se- ries. 7,9 Epilepsy surgery has played an important role in the treatment of patients affected by medically intractable sei- zures, resulting in excellent seizure control and improve- ment in quality in life in a greater number of patients with hemispheric epileptogenic conditions. 7,10–15 In cases of catastrophic epilepsy, it has been suggested that neurosur- gical treatment can alter the natural history of the disease ABBREVIATIONS DQ = developmental quotient. SUBMITTED September 14, 2019. ACCEPTED January 28, 2020. INCLUDE WHEN CITING Published online April 24, 2020; DOI: 10.3171/2020.1.PEDS19537. Long-term outcomes after surgery for catastrophic epilepsy in infants: institutional experience and review of the literature Vincent C. Ye, MD, 1 Ashish H. Shah, MD, 2 Samir Sur, MD, 2 Justin K. Achua, MS, 2 Shelly Wang, MD, MPH, 3 George M. Ibrahim, MD, PhD, 1 Sanjiv Bhatia, MD, 3 and John Ragheb, MD 2,3 1 Division of Neurosurgery, Department of Surgery, Hospital for Sick Children, University of Toronto, Ontario, Canada; 2 Department of Neurosurgery, University of Miami; and 3 Division of Neurosurgery, Nicklaus Children’s Hospital, Miami, Florida OBJECTIVE Uncontrolled epilepsy is associated with serious deleterious effects on the neurological development of infants and has been described as “catastrophic epilepsy.” Recently, there has been increased emphasis on early surgi- cal interventions to preserve or rescue neurodevelopmental outcomes in infants with early intractable epilepsy. The en- thusiasm for early treatments is often tempered by concerns regarding the morbidity of neurosurgical procedures in very young patients. Here, the authors report outcomes following the surgical management of infants (younger than 1 year). METHODS The authors performed a retrospective review of patients younger than 1 year of age who underwent sur- gery for epilepsy at Miami (Nicklaus) Children’s Hospital and Jackson Memorial Hospital between 1994 and 2018. Pa- tient demographics, including the type of interventions, were recorded. Seizure outcomes (at last follow-up and at 1 year postoperatively) as well as complications are reported. RESULTS Thirty-eight infants (median age 5.9 months) underwent a spectrum of surgical interventions, including hemispherectomy (n = 17), focal resection (n = 13), and multilobe resections (n = 8), with a mean follow-up duration of 9.1 years. Hemimegalencephaly and cortical dysplasia were the most commonly encountered pathologies. Surgery for catastrophic epilepsy resulted in complete resolution of seizures in 68% (n = 26) of patients, and 76% (n = 29) had a greater than 90% reduction in seizure frequency. Overall mortality and morbidity were 0% and 10%, respectively. The latter included infections (n = 2), infarct (n = 1), and immediate reoperation for seizures (n = 1). CONCLUSIONS Surgical intervention for catastrophic epilepsy in infants remains safe, ef fcacious, and durable. The authors’ work provides the longest follow-up of such a series on infants to date and compares favorably with previously published series. https://thejns.org/doi/abs/10.3171/2020.1.PEDS19537 KEYWORDS infant; epilepsy; surgery; outcomes; hemispherectomy J Neurosurg Pediatr Volume 26 • August 2020 157 ©AANS 2020, except where prohibited by US copyright law Unauthenticated | Downloaded 10/14/23 01:28 PM UTC