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. 2016 Aug 30;5(1):1434.
doi: 10.1186/s40064-016-3096-2. eCollection 2016.

Vertical parasagittal hemispherotomy for Sturge-Weber syndrome in early infancy: case report and literature review

Xiangyu Liu  1 , Taisuke Otsuki  2 , Akio Takahashi  2 , Takanobu Kaido  3
Affiliations

Vertical parasagittal hemispherotomy for Sturge-Weber syndrome in early infancy: case report and literature review

Xiangyu Liu et al. Springerplus. .

Abstract

Introduction: 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). 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.

Discussion: It 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.

Conclusion: 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.

Keywords: Epilepsy surgery; Seizure; Sturge–Weber syndrome; Vertical parasagittal hemispherotomy.

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Figures

Fig. 1
Fig. 1
a Facial port-wine stain affecting the facial skin; b CT scan revealing cortical calcification in the cerebral hemisphere; c MR imaging demonstrating leptomeningeal venous angioma and enlargement of the choroid plexus; d An immediate postictal FDG-PET scan showing significant intense hypermetabolism in the left frontal lobe
Fig. 2
Fig. 2
a Interictal scalp EEG showing markedly asymmetry with spikes observed on F3&F7; b Ictal scalp EEG revealing changing of the background activities and bilateral rhythmic slow waves with no localized value
Fig. 3
Fig. 3
a Intraoperative image demonstrating leptomeningeal angioma; b Postoperative CT scan showing brain edema; c, d, e Postoperative MR images (axial, sagittal and cornonal view) confirming complete disconnection
See this image and copyright information in PMC

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