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

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  • 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
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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 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).

METHODS

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.

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, 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.

ABBREVIATIONS DQ = developmental quotient.

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Contributor Notes

Correspondence John Ragheb: University of Miami, FL. jragheb@med.miami.edu.

INCLUDE WHEN CITING Published online April 24, 2020; DOI: 10.3171/2020.1.PEDS19537.

Disclosures The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.

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