Frontal Disconnection in a Case of Mild Malformation of Cortical Development with Oligodendroglial Hyperplasia and
Alessandro De Benedictis1, Davide Ferlito2, Matteo Barba1
1Neurosurgery Unit, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.
Insights
Frontal disconnection surgery successfully treated drug-resistant epilepsy in a child with MOGHE. The patient achieved seizure freedom and neurocognitive improvement post-procedure.
Area of Science:
- Neurosurgery
- Epileptology
- Neuropathology
Background:
- Drug-resistant epilepsy associated with lobar encephalopathies poses significant treatment challenges.
- Frontal disconnection is a surgical technique targeting white matter connectivity for epilepsy treatment.
Purpose of the Study:
- To describe the surgical technique and outcomes of frontal disconnection in a pediatric case.
- To highlight the histopathological finding of MOGHE in drug-resistant epilepsy.
Main Methods:
- Case report of a 7-year-old child undergoing right prefrontal disconnection and anterior insular resection.
- Detailed description of surgical steps to interrupt anterior white matter connectivity.
- Histopathological analysis and neuroimaging review.
Main Results:
- The patient remained seizure-free at 11-month follow-up.
- No residual motor deficits were observed.
- Neurocognitive improvement was noted post-surgery.
- Histopathology revealed MOGHE, a rare epilepsy-associated entity.
Conclusions:
- Frontal disconnection is an effective surgical option for specific drug-resistant epilepsies.
- Early identification and surgical intervention can lead to favorable outcomes.
- MOGHE is an important histopathological consideration in refractory epilepsy cases.
Abstract:
Frontal disconnection is an effective technique for the treatment of different forms of drug-resistant epilepsy associated with lobar encephalopathies. We describe the case of a 7-year-old child who underwent right prefrontal disconnection and anterior insular resection. The procedure involves sequential steps aiming to interrupt both interhemispheric and intrahemispheric anterior white matter connectivity. Histopathologic analysis revealed a MOGHE (mild malformation of cortical development with oligodendroglial hyperplasia and epilepsy), a recently recognized histopathologic entity associated with multiple types of drug-resistant epilepsy.1-3 At the early 11-month follow-up, the patient remained seizure free without residual motor deficit and showed neurocognitive improvement. The video illustrates the main surgical steps, incorporating intraoperative footage and advanced 3-dimensional neuroimaging modeling. The patient's parents consented to the procedure. The participants and any identifiable individuals consented to publication of his/her image. Considering the retrospective nature of the analysis, the current study did not require the approval of the local ethics committee according to current legislation, but a notification was sent.
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