Abstract
IntroductionThe authors here present a rare case of a 3-month-old infant with unilateral Sturge-Weber syndrome (SWS) who had excellent seizure control and no aggravation of previous existed neurological deficits after vertical parasagittal hemispherotomy (VPH). To our knowledge, this patient with SWS was the youngest one who received VPH.Case description The use of VPH results in a successful treatment of intractable epilepsy in a patient with seizure onset in early infancy. At follow-up, the patient’s neurodevelopmental status has been improved since the surgery.DiscussionIt is generally accepted that early-onset seizures in children with SWS are associated with worse neurological and developmental outcomes. However, when surgical treatment should be considered and how it should be performed remain a longstanding controversy. We promote early surgery in children with SWS and early-onset epilepsy.ConclusionWe suggest that VPH may be a useful adjuvant in the management of SWS with refractory epilepsy in early infancy and this procedure carries low neurological risk.
Highlights
DiscussionIt is generally accepted that early-onset seizures in children with Sturge-Weber syndrome (SWS) are associated with worse neurological and developmental outcomes
The authors here present a rare case of a 3-month-old infant with unilateral Sturge-Weber syndrome (SWS) who had excellent seizure control and no aggravation of previous existed neurological deficits after vertical parasagittal hemispherotomy (VPH)
We suggest that VPH may be a useful adjuvant in the management of SWS with refractory epilepsy in early infancy and this procedure carries low neurological risk
Summary
This case demonstrates the utility of VPH as an effective surgical treatment for SWS patients with medically refractory epilepsy. Delalande (2007) reported the first series of 83 patients who underwent VPH for refractory epilepsy with shorter operative times and fewer early postoperative complications (Delalande et al 2007) His surgical technique can achieve complete disconnection of the affected hemisphere and preserve an intact vessel supply Previous authors reported that early-onset seizures occurring in patients younger than 1 year of age may be more difficult to control and are associated with worse neurological and developmental outcomes (Alkonyi et al 2011; Jagtap et al 2013; Thomas et al 2012). The surgical techniques of VPH provide smaller skin incision and bone flap, which reduces blood loss and avoids the exposure of large venous sinuses It allows complete disconnection of the hemisphere through a cortical window with good results in terms of seizure outcome and a relatively low complication rate
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