Long-term outcomes after surgery for catastrophic epilepsy in infants: institutional experience and review of the literature

Abstract

<jats:sec><jats:title>OBJECTIVE</jats:title><jats:p>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 surgical interventions to preserve or rescue neurodevelopmental outcomes in infants with early intractable epilepsy. The enthusiasm 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).</jats:p></jats:sec><jats:sec><jats:title>METHODS</jats:title><jats:p>The authors performed a retrospective review of patients younger than 1 year of age who underwent surgery for epilepsy at Miami (Nicklaus) Children’s Hospital and Jackson Memorial Hospital between 1994 and 2018. Patient 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.</jats:p></jats:sec><jats:sec><jats:title>RESULTS</jats:title><jats:p>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).</jats:p></jats:sec><jats:sec><jats:title>CONCLUSIONS</jats:title><jats:p>Surgical intervention for catastrophic epilepsy in infants remains safe, efficacious, 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.</jats:p></jats:sec>

Epilepsy surgery in infants up to 3 months of age: Safety, feasibility, and outcomes: A multicenter, multinational study

“…Similar outcomes were reported for temporal lobe surgery in infants (up to 4.5 years of age) 31 . Ye et al reported a 68% Engel I among 38 infants who underwent surgery before 1 year of age 49 . Honda et al reported 66% seizure‐free among 12 infants up to 9 months old undergoing hemispherotomy 34 .…”

“…In a survey by Steinbock et al, 56% of infants (1–35 months old) received intra‐ or postoperative blood products 25 . Ye et al reported no need for intraoperative blood product transfusion and no hydrocephalus following 38 surgeries in infants younger than 1 year of age 49 . Gröppel et al compared the safety of epilepsy surgery before 1 year of age (3–9 months) to elder infants (16–32 months) and reported no complications in either age groups 28 .…”

Prenatal diagnosis and delivery of megalencephaly–capillary malformation syndrome

“…The most common clinical findings of affected infants are intractable epilepsy, psychomotor sequelae and contralateral motor deficit 3. Most symptomatic cases are managed via surgical hemispherectomy9 or temporising endovascular embolic hemispherectomy 10. Rarely, some patients may be stable on antiepileptic therapy11 and, more recently, mTOR inhibitors such as rapamycin 12.…”

Functional hemispheric disconnection procedures for chronic epilepsy: history, indications, techniques, complications and current practice in Europe. A consensus statement on behalf of the EANS functional neurosurgery section

“…Schramm et al reported a mortality rate of 1.0 % in a large retrospective pediatric series while in the adult series of the same center the reported mortality rate was 0 % ( Schramm et al, 2012 ; Althausen et al, 2013 ). Likewise, several recent pediatric series have reported 0 % mortality ( Dorfer et al, 2013b ; Moosa et al, 2013 ; Ye et al, 2020 ). The transformation of the procedure from an extensive resection to a minimal disconnection, the avoidance of cortical vessel coagulation of the pathological hemisphere, and the advances in neuroanesthesia during the last two decades may well explain the minimization of the associated mortality.…”