Case Report: Hemispherotomy in the First Days of Life to Treat Drug-Resistant Lesional Epilepsy

Abstract

Background: Neonatal drug-resistant epilepsy is often caused by perinatal epileptogenic insults such as stroke, ischemia, hemorrhage, and/or genetic defects. Rapid seizure control is particularly important for cognitive development. Since early surgical intervention and thus a short duration of epilepsy should lead to an optimal developmental outcome, we present our experience with hemispherotomy in an infant at the corrected age of 1 week. Methods: We report successful hemispherotomy for drug-resistant epilepsy in an infant with hemimegalencephaly at a corrected age of 1 week. Results: The infant was diagnosed with drug-resistant lesional epilepsy due to hemimegalencephaly affecting the left hemisphere. Given congruent electroclinical findings, we performed a left vertical parasagittal transventricular hemispherotomy after critical interdisciplinary discussion. No complications occurred during the surgery. Intraoperatively; 118 ml of red blood cells (30 ml/kg) and 80 ml of plasma were transfused. The patient has been seizure-free since discharge without further neurological deficits. Conclusion: We demonstrate that early epilepsy surgery is a safe procedure in very young infants if performed in a specialized center experienced with age-specific surgical conditions and perioperative management. The specific surgical difficulties should be weighed against the risk of life-long developmental drawbacks of ongoing detrimental epilepsy.

Keywords: EEG; drug-resistant epilepsy; epilepsy; epilepsy surgery; hemispherotomy; infant; pediatrics.

Figure 1

Cranial MRI of the patient. (A) The presurgical axial T2-weighted image shows left hemimegalencephaly with thickened cortex, an irregular gyral pattern and abnormal T2w hypointensity of the white msatter suggestive of accelerated myelination in the entire left hemisphere. Postsurgical coronal (B), axial (C), and sagittal (D) T2-weighted images demonstrate the left sided hemispherotomy.