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Neuropediatrics 2006; 37(1): 26-31
DOI: 10.1055/s-2006-923945
Original Article

Georg Thieme Verlag KG Stuttgart · New York

Early Periinsular Hemispherotomy in Children with Sturge-Weber Syndrome and Intractable Epilepsy - Outcome in Eight Patients

C. Schropp1 , N. Sörensen2 , J. Krauß2
  • 1University Children's Hospital Würzburg, Würzburg, Germany
  • 2Department of Pediatric Neurosurgery, University of Würzburg, Würzburg, Germany
Further Information

Publication History

Received: August 3, 2005

Accepted after Revision: January 25, 2006

Publication Date:
15 March 2006 (online)

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Abstract

Background: Periinsular hemispherotomy is used to treat catastrophic epilepsy in hemispheric lesions. It avoids complications of tissue resection performed in other hemispherectomy procedures. We report on the effectiveness, complications, and neurological sequelae in patients with Sturge-Weber syndrome (SWS). Methods: Eight patients (mean age at operation 15 months, mean surveillance time 7.1 years, 5 girls) were operated between 1994 and 2000, one with additional subpial resections of the insular cortex. Preoperatively hemiparesis was evident in all patients, mental retardation in six. Charts were reviewed for pre- and perioperative data. Outcome data were collected by structured telephone interview and questionnaires. Results: No severe complications occurred. Five patients with unilateral angiomatosis were seizure-free without medication, rare non-disabling seizures occurred in one. Two patients with bilateral angiomatosis had seizure reduction of > 90 %. Hemiparesis deteriorated in all patients with a marked deficit of hand function and less severe deficit in the lower extremity and trunk. Seven became ambulatory and achieved community language. Mental development improved with seizure control. Six patients have learning disability, two are mentally handicapped. Conclusion: Periinsular hemispherotomy is a valuable treatment modality in this patient group. The sequelae of hemiparesis, hemianopsia, and developmental delay must be seen in relation to the natural course in severe SWS.

Key words

Sturge-Weber syndrome - childhood epilepsy - hemispherectomy - hemispherotomy - epilepsy surgery - bilateral angiomatosis

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Christian Schropp

University Children's Hospital Würzburg

Josef-Schneider-Str. 2

97080 Würzburg

Germany

Email: schropp_c@klinik.uni-wuerzburg.de

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