Before the Age of Two Hemispherotomy Allows a Better Neurodevelopmental Outcome Than Posterior Quadrant Disconnection in Large Unilateral Malformations of Cortical Development
Abstract number :
2.439
Submission category :
9. Surgery / 9B. Pediatrics
Year :
2024
Submission ID :
1255
Source :
www.aesnet.org
Presentation date :
12/8/2024 12:00:00 AM
Published date :
Authors :
Rayann Checri, MD – Rothschild Foundation Hospital, Paris, France
Presenting Author: Mathilde Chipaux-Raffo, MD, PhD – Rothschild Foundation Hospital, Paris, France
Suzanne Perguilhem, PhD – Rothschild Foundation Hospital, Paris, France
Sarah D. Rosenberg, MD – Rothschild Foundation Hospital, Paris, France
Marie-Thérèse Dangles, MD – Rothschild Foundation Hospital, Paris, France
Anais Chivet, MD – Rothschild Foundation Hospital, Paris, France
Sarah Ferrand-Sorbets, MD – Rothschild Foundation Hospital, Paris, France
Georg Dorfmüller, MD – Rothschild Foundation Hospital, Paris, France
Christine Bulteau, MD,PhD – Rothschild Foundation Hospital, Paris, France
Emmanuel Raffo, Md, PhD – Rothschild Foundation Hospital
Rationale: Large malformations of cortical development (MCD) represent a frequent etiology of structural Drug Resistant Epilepsy (DRE) in early childhood. They often herald severe motor, cognitive and behavioral impairments. Epilepsy surgery may be performed since the first months of life. Targeting the epileptic zone (EZ) may be uncertain at this age. Our goal is to compare the epileptic and neurodevelopmental outcome of patients that underwent hemispherotomy (HM) vs temporal resection and Posterior Quadrant Disconnection (PQD) before the age of two.
Methods: We retrospectively analyzed the monocentric cohort of our pediatric epilepsy surgery department We considered all toddlers that underwent HM or PQD before the age of 2 for DRE linked to large unilateral MCD between 2008 and 2024
Results: Amongst 14 patient that underwent HM at a median age of 5.9 month (3m to 16m), all of them exhibit hemiparesis, 86% are free of seizure (Engel 1, n=12), 43% show mild to severe cognitive impairment (n=6) and 14% Autistic spectrum disorder (n=2).
Amongst 8 patient that underwent PQD at a median age of 6.2 month (2m to 14m), 50% exhibit hemiparesis (n=4), 25% are free of seizure (Engel 1, n=2), 75% show mild to severe cognitive impairment (n=6), and 63% Autistic spectrum disorder (n=5).
The rate of seizure free patients is statistically different after the two surgical procedures, with an OR of 18 to become Engel 1 (p = 0.007) after HM vs PQD. Cognitive (p=0.13) and social (p=0.019) abilities are significantly better after HM than after PQD. In the PQD subgroup no difference was demonstrate for neurodevelopmental outcome according to the Engel score( cognitive : p=0.42 ; social : p=0.53).
Conclusions: For drug resistant epilepsy linked to large unilateral cortical malformation of cortical development, Posterior Quadrant Disconnection fails to achieve complete seizure freedom and neurodevelopmental benefit in the majority. Hemiparesis is most often due to the underlying pathology. On the other side, our data suggests that a better epileptic and neurodevelopmental outcome is achieved following Hemispherotomy. Therefore for very young patients with large unilateral MCD, Hemispherotomy should not be delayed to benefit from the optimal brain plasticity window.
Funding: none
Surgery