Long-term developmental outcome after early hemispherotomy for hemimegalencephaly in infants with epileptic
Ryoko Honda1, Takanobu Kaido, Kenji Sugai
1Department of Child Neurology, National Center of Neurology and Psychiatry (NCNP), Tokyo, Japan.
Insights
Early hemispherotomy surgery in infants with hemimegalencephaly (HME) and epileptic encephalopathy can lead to seizure freedom and improved developmental outcomes. Shorter seizure duration before surgery is linked to better postoperative developmental quotients.
Area of Science:
- Pediatric Neurology
- Neurosurgery
- Developmental Neuroscience
Background:
- Hemimegalencephaly (HME) is a rare congenital brain malformation.
- Epileptic encephalopathy in infants with HME presents significant developmental challenges.
- Early surgical intervention is being explored for severe pediatric neurological conditions.
Purpose of the Study:
- To evaluate the impact of early hemispherotomy on neurodevelopment in infants with HME and epileptic encephalopathy.
- To determine the correlation between seizure control and developmental outcomes post-surgery.
- To identify factors influencing postoperative development in this patient cohort.
Main Methods:
- A consecutive series of 12 infants diagnosed with HME and epileptic encephalopathy underwent vertical parasagittal hemispherotomy.
- Surgical outcomes, including seizure status and developmental quotient (DQ), were assessed postoperatively.
- Developmental quotients were compared between seizure-free and persistent seizure groups, and correlated with preoperative seizure duration.
Main Results:
- Hemispherotomy was performed without mortality or severe morbidity.
- Eight out of 12 patients (66.7%) achieved seizure freedom.
- Seizure-free patients demonstrated significantly higher postoperative developmental quotients (DQ) compared to those with ongoing seizures (p=0.02).
- In the seizure-free group, a shorter preoperative seizure duration correlated with a better postoperative DQ (r=-0.811, p=0.01).
Conclusions:
- Early hemispherotomy is a viable surgical option for infants with HME and epileptic encephalopathy, offering seizure control and improved neurodevelopmental trajectories.
- Minimizing seizure duration in early infancy is crucial for optimizing postoperative developmental outcomes in these patients.
- This study highlights the potential benefits of timely surgical intervention in improving long-term development for infants with complex brain malformations and intractable epilepsy.
Abstract:
This study aimed to identify the effect of early hemispherotomy on development in a consecutive series of 12 infants with hemimegalencephaly (HME) demonstrating epileptic encephalopathy. Mean age at onset was 20.4 days (range, 1-140), mean age at surgery was 4.3 months (range, 2-9), and mean follow-up time was 78.8 months (range, 36-121). Eleven patients had a history of early infantile epileptic encephalopathy. Vertical parasagittal hemispherotomy was performed without mortality or severe morbidities. At follow-up, seizure freedom was obtained in 8 patients (66.7%), who showed significantly higher postoperative developmental quotient (DQ) (mean, 31.3; range, 7-61) than those with seizures (mean, 5.5; range, 3-8) (p=0.02). Within the seizure-free group, postoperative DQ correlated with preoperative seizure duration (r=-0.811, p=0.01). Our results showed that shorter seizure duration during early infancy could provide better postoperative DQ in infants with HME and epileptic encephalopathy.
