WHY WOULD YOU REMOVE HALF A BRAIN - THE OUTCOME OF 58 CHILDREN AFTER HEMISPHERECTOMY - THE JOHNS-HOPKINS EXPERIENCE - 1968 TO 1996

Purpose. To report the outcomes of the 58 hemispherectomies performed at Johns Hopkins between 1968 and January 1996. Methods. Charts were r eviewed of the 58 hemispherectomies performed at Johns Hopkins Medical Institutions by the Pediatric Epilepsy Group during-the years 1968 to 1996. Twenty-seven operations were done for Rasmussen's syndrome, 24 operations for cortical dysplasias/hemimegalencephalies, and 7 for Stu rge-Weber syndrome or other congenital vascular problems. Seizure cont rol alone did not seem to adequately describe the outcomes of the proc edure. Therefore, a score was constructed that included seizure freque ncy, motor disability, and intellectual handicap. This burden of illne ss score better described the child's handicap before and after surger y. Results. Perioperative death occurred in 4 out of 58 children. Of t he 54 surviving children, 54% (29/54) are seizure-free, 24% (13/54) ha ve nonhandicapping seizures, and 23% (12/54) have residual seizures th at interfere to some extent with function. Reduction in seizures was r elated to the etiology of: the unilateral epilepsy. Eighty-nine percen t of children with Rasmussen's, 67% of those with dysplasias, and 67% of the vascular group are seizure-free, or have occasional, nonhandica pping seizures. All operations were considered by the parents and the physicians to have been successful in decreasing the burden of illness . In 44 the procedure was very successful, in 7 it was moderately succ essful, and in 3 it was minimally successful. Success was related to t he etiology, and early surgery was preferable. Conclusion. Hemispherec tomy can be a valuable procedure for relieving the burden of seizures, the burden of medication, and the general dysfunction in children wit h severe or progressive unilateral cortical disease. Early hemispherec tomy, although increasing the hemiparesis in children with Rasmussen's syndrome, relieves the burden of constant seizures and allows the chi ld to return to a more normal life. In children with dysplasias, early surgery can allow the resumption of more normal development.