| Objective: To preliminarily explore the relationship between postoperative outcome and related factors in children with drug-refractory West syndrome,and to provide theoretical basis for the selection of surgical indications and timing in children with West syndrome.Methods: The clinical data of 30 children(18 males and 12 females)with West syndrome who underwent surgery in the Epilepsy Center of Shenzhen Children’s Hospital from June 2018 to June 2020 were retrospectively analyzed.Engel grading criteria was used to assess the postoperative outcome,the improvement of postoperative EEG and cognitive function were observed.Univariate analysis and accurate logistic regression analysis were used to find out the relationship between each related factor and postoperative outcome.Results: The age of seizure onset was 4.74 ± 3.24 months,the age at operation was 20.8± 12.67 months,and the disease course from seizure onset to operation was 16.07 ± 11.65 months.Twenty-one children(21/30,70.0%)had multiple seizure patterns,and epileptic spasms mixed with focal seizures were relatively common(11/30,36.7%),all 29 children had daily seizures before surgery,including 20 with>3 daily seizures and 8 with>10 daily seizures.Structural abnormalities were observed on cranial magnetic resonance imaging(MRI)in 25 children,and in 5 children with negative MRI,positron emission tomography magic resonance imaging(PET-MRI)revealed 4 of them suggestive of local metabolic reduction(4/5,80.0%).Of all patients,21(21/30,70.0%)were considered to have congential structural causes,6(6/30,20.0%)had acquired structural causes,and 3(3/30,10.0%)had hereditary causes based on a comprehensive judement of EEG,imaging,and genetic test results.Postoperative pathology showed cortical dysplasia in 17 patients(17/30,56.7%),and others included: glial scar in 7 patients,hemimegalencephaly in1 patient,and ganglioglioma in 1patient.Among all chidren,19(19/30,63.3%)underwent only one operation and 11(11/30,36.7%)had more than two operations,of which 7(7/11,63.6%)had subtotal/total hemispheric dissection.The average postoperative follow-up period was 25 months(15-38 months).There were 22 cases in the significant effect group(Engel grade I + Engel grade II),8 cases in the poor effect group(Engel grade III + Engel grade IV).46.7%(14/30)of the children’s cognitive function and motor function during the follow-up period were improved than before,and 40%(12/30)remained at the preoperative developmental level.The average number of anti-seizure medications(ASMs)used preoperatively in this group of patients was 5.27(2 to 10),and with effective seizure control after surgery,the average number of ASMs decreased to 1.90(0 to 4)at the end of the follow-up period.The correlation analysis between preoperative factors and postoperative efficacy revealed that the course of disease,etiology,positive rate of PET-MRI,operation mode and lesion range were significantly correlated with the postoperative outcome(P < 0.05),while positive rate of PET-MRI and lesion range were independent risk factors affecting postoperative outcomes.Postoperatively,all children had no permanent motor or language function impairment except for hydrocephalus in 2children and hemiplegia in 1 child.Conclusions: Surgery is an effective treament for partially drug-refractory WS.All children with drug-refractory WS should undergo complete preoperative evaluation as early as possible in order to screen for structural causes that can be surgically intervened.The most common cause of drug-refractory WS that can be treated surgically is malformation of cortical development,followed by perinatal brain injury.Hemispheric(Subtotal)disconnection is a better surgical approach for appropriate cases of WS patients.The positive rate of PET-MRI and duration of disease are closely related to the postoperative outcome. |
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