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Catastrophic Epilepsy and Hemispherotomy

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Textbook of Pediatric Neurosurgery

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Abstract

The catastrophic epilepsies encompass a number of severe epilepsies that are intractable, result in intellectual and behavioral disability, and have the highest incidence of sudden unexpected death in epilepsy (SUDEP). For the catastrophic hemispheric epilepsies such as Sturge-Weber syndrome, hemimegalencephaly, Rasmussen’s encephalitis, extensive unilateral cortical dysplasia, perinatal hemispheric infarct, and other unilateral refractory epilepsy, hemispheric disconnection has long been used to treat these types of epilepsies. Krynauw reported on the first major series of anatomical hemispherectomy (AH) for intractable seizures in 1950. Complications encouraged modifications to the original technique. Rasmussen introduced the functional hemispherectomy (FH) in 1974, which allowed much less brain excision and introduced the concept of hemispheric disconnection. Further modifications continued, all based on the concept of maximal disconnection and minimal excision. Therefore, future modifications became known as variants of the “hemispherotomy.” In 1995, Villemure introduced the peri-insular hemispherotomy (PIH). Here, we will discuss the catastrophic hemispheric epilepsies and surgical indications, preoperative planning, operative steps, and postoperative management of patients undergoing PIH – our modification of the approach described by Villemure and Mascott and Shimizu and Maehara.

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Authors and Affiliations

  1. Pappajohn Biomedical Institute, University of Iowa Carver College of Medicine, Iowa City, IA, USA

    Brian J. Dlouhy

  2. Department of Neurosurgery, University of Iowa Hospitals and Clinics, Iowa City, IA, USA

    Brian J. Dlouhy

  3. Washington University, St. Louis, MO, USA

    Matthew D. Smyth

Authors
  1. Brian J. Dlouhy
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  2. Matthew D. Smyth
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Corresponding author

Correspondence to Matthew D. Smyth .

Editor information

Editors and Affiliations

  1. International Neuroscience Institute, Hannover, Germany

    Concezio Di Rocco

  2. Oakland Medical Center, Oakland, CA, USA

    Dachling Pang

  3. University of Toronto Hospital for Sick Children, Toronto, ON, Canada

    James T. Rutka

Section Editor information

  1. Division of Neurosurgery, The Arthur and Sonia Labatt Brain Tumour Research Centre, The Hospital for Sick Children, University of Toronto, Toronto, ON, Canada

    James T. Rutka

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Dlouhy, B.J., Smyth, M.D. (2020). Catastrophic Epilepsy and Hemispherotomy. In: Di Rocco, C., Pang, D., Rutka, J. (eds) Textbook of Pediatric Neurosurgery. Springer, Cham. https://doi.org/10.1007/978-3-319-31512-6_98-1

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  • DOI: https://doi.org/10.1007/978-3-319-31512-6_98-1

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-31512-6

  • Online ISBN: 978-3-319-31512-6

  • eBook Packages: Springer Reference Biomedicine and Life SciencesReference Module Biomedical and Life Sciences

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