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.

Epilepsy surgery in early infancy: A retrospective, multicenter study

“…The youngest patient was operated at a corrected age of 1 week. 10 Cranial MRIs revealed malformation of cortical development as the most common cause (n = 14).…”

“…Median seizure onset was 13 days (IQR: 56, range 1‐128), and first surgery was performed at an age of 134 days (IQR: 58, range 61‐186), (Figure 1A). The youngest patient was operated at a corrected age of 1 week 10 . Cranial MRIs revealed malformation of cortical development as the most common cause (n = 14).…”

Epilepsy surgery in the first six months of life: A systematic review and meta-analysis

“…Additionally, the type of lesion found may be associated with often-necessary larger surgical interventions [16,17]. Only few studies, which are mostly case series, report on patients who undergo epilepsy surgery in the first months of life [16,[18][19][20]. Because of this, there is a lack of data on epilepsy surgery safety, outcome, and functional development postoperatively.…”

Family Burden and Epilepsy Surgery in Children with Drug-Resistant Epilepsy

“…Besides high risks of cognitive and psychosocial impairments in children with DRE with longer epilepsy duration, these results underline the necessity of epilepsy surgery evaluation as soon as possible. 2,[12][13][14] Presurgical seizure frequency did not significantly influence the postsurgical decrease of FB. However, with a seizure freedom rate of 87.1%, we only have a few patients with continuous seizure activity after surgery.…”