J Clin Neurol.  2014 Apr;10(2):101-107. 10.3988/jcn.2014.10.2.101.

Long-Term Outcomes of Hemispheric Disconnection in Pediatric Patients with Intractable Epilepsy

• Lee YJ
• Kim EH
• Yum MS
• Lee JK
• Hong S
• Ko TS

• KMID: 2287527
• DOI: http://doi.org/10.3988/jcn.2014.10.2.101

Abstract

BACKGROUND AND PURPOSE
Hemispherectomy reportedly produces remarkable results in terms of seizure outcome and quality of life for medically intractable hemispheric epilepsy in children. We reviewed the neuroradiologic findings, pathologic findings, epilepsy characteristics, and clinical long-term outcomes in pediatric patients following a hemispheric disconnection.
METHODS
We retrospectively studied 12 children (8 males) who underwent a hemispherectomy at Asan Medical Center between 1997 and 2005. Clinical, EEG, neuroradiological, and surgical data were collected. Long-term outcomes for seizure, motor functions, and cognitive functions were evaluated at a mean follow-up of 12.7 years (range, 7.6-16.2 years) after surgery.
RESULTS
The mean age at epilepsy onset was 3.0 years (range, 0-7.6 years). The following epilepsy syndromes were identified in our cohort: focal symptomatic epilepsy (n=8), West syndrome (n=3), and Rasmussen's syndrome (n=1). Postoperative histopathology of our study patients revealed malformation of cortical development (n=7), encephalomalacia as a sequela of infarction or trauma (n=3), Sturge-Weber syndrome (n=1), and Rasmussen's encephalitis (n=1). The mean age at surgery was 6.5 years (range, 0.8-12.3 years). Anatomical or functional hemispherectomy was performed in 8 patients, and hemispherotomy was performed in 4 patients. Eight of our 12 children (66.7%) were seizure-free, but 3 patients with perioperative complications showed persistent seizure. Although all patients had preoperative hemiparesis and developmental delay, none had additional motor or cognitive deficits after surgery, and most achieved independent walking and improvement in daily activities.
CONCLUSIONS
The long-term clinical outcomes of hemispherectomy in children with intractable hemispheric epilepsy are good when careful patient selection and skilled surgical approaches are applied.

Keyword

seizure; hemispherectomy; hemispherotomy; psychomotor outcomes

MeSH Terms

Child
Chungcheongnam-do
Cohort Studies
Electroencephalography
Encephalitis
Encephalomalacia
Epilepsy*
Follow-Up Studies
Hemispherectomy
Humans
Infant
Infant, Newborn
Infarction
Paresis
Patient Selection
Quality of Life
Retrospective Studies
Seizures
Spasms, Infantile
Sturge-Weber Syndrome
Walking

Figure

• 

  Fig. 1 Presurgical MRI scans of patients with different etiologies (A-D). Left encephalomalacia (patient 3) (A), right frontotemporal cortical dysplasia (patient 4) (B), Sturge-Weber syndrome involving the right hemisphere (patient 6) (C), and left hemimegalencephaly (patient 11) (D). Axial (upper) and coronal (lower) MRI scans demonstrating the main surgical procedure for hemispheric disconnection (E-H). Anatomical hemispherectomy produced by removal of the entire hemisphere including most of the deep structures in patient 3 (E). Functional hemispherectomy based on a combination of partial anatomic excision and disconnection of the remaining lobes in patient 4 (F). Modified peri-insular hemispherotomy and removal of the frontotemporoparietal operculum and underlying deep structures in patient 6 (G). Vertical parasagittal hemispherotomy performed in patient 11 (H).

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