Hemispherectomy in infants: an institutional experience with 21 patients

Drug-resistant epilepsy (DRE) can arise early in life due to multiple structural abnormalities. In patients with the disorder, seizures can be difficult to control and can significantly impair neurodevelopment. Hemispherectomy is well described as a treatment for refractory hemispheric-onset epilepsy, but its use in infants has been limited due to concerns over surgical risk and physiological tolerance. The aim of this review was to summarize an institutional experience with hemispherectomy in infants to demonstrate the safety and efficacy of the technique.

A retrospective chart review was conducted to identify children younger than 1 year of age who had undergone a modified functional hemispherectomy combining elements of both hemispherotomy and anatomical resection to treat DRE at Seattle Children's Hospital from 2000 to 2024. The primary outcome was favorable seizure outcome, defined as Engel class I-II at the 1-year follow-up. Secondary outcomes included perioperative complications, development of postoperative hydrocephalus, need for long-term nutritional supplementation, and changes in the anti-seizure medication burden.

Twenty-one children who had undergone functional hemispherectomy were identified, revealing a mean age of 6.2 months (range 0.93-12.3 months) at surgery. The most common etiology was hemimegalencephaly (n = 11, 52%). No serious cardiac, pulmonary, or coagulopathic adverse events occurred, although complications included asymptomatic cerebral venous sinus thrombosis (n = 1, 5%), postoperative hydrocephalus (n = 3, 14%), and electrolyte derangements (n = 1, 5%). The mean hospital stay was 12.5 days (range 6-34 days). Hydrocephalus developed > 12 months postoperatively in 2 (67%) of the 3 patients with this complication. The mean estimated blood loss was 345.8 mL (150-700 mL), and all patients received a transfusion during their surgery with a mean transfusion volume of 450.2 mL (60.4 mL/kg). Seizure outcomes were uniformly assessed at the last clinical follow-up, with a mean follow-up duration of 91.9 months (range 6.1-261.6 months). A favorable seizure outcome (Engel class I-II) was attained in 20 patients (95%), with an Engel class I outcome in 19 patients (90%).

An institutional experience demonstrated that functional hemispherectomy is a safe and well-tolerated procedure in infants and offers excellent seizure control outcomes in hemispheric-onset epilepsies. The surgical technique, a focus on minimizing operative blood loss, and multidisciplinary care of these patients are critical elements in ensuring the safety and success of this procedure. Future studies are needed to better characterize long-term functional and neurodevelopmental outcomes in this age group.