Surgical outcomes of drug-refractory infantile epileptic spasms syndrome and related prognostic factors: a retrospective study

Abstract

Background: In this study, we aimed to assess the efficacy of surgical treatment in children with drug-refractory infantile epileptic spasms syndrome (IESS) and examine the factors influencing the post-surgical outcomes.

Methods: The clinical data of 30 children (18 males and 12 females) with epileptic spasms (ES) who underwent surgery at the Epilepsy Center of Shenzhen Children's Hospital between June 2018 and June 2020 were retrospectively analyzed. Post-surgical outcomes were evaluated using the Engel Epilepsy Surgery Outcome Scale. Scalp electroencephalography and developmental quotient were assessed preoperatively and postoperatively. Univariate analysis and exact logistic regression analyses were used to identify the factors affecting the postoperative efficacy.

Results: Of the 30 patients who underwent surgical resection, 22 (73.3%) achieved Engel's class I-II outcomes. Additionally, motor and cognitive functions improved in 14 patients (46.7%). The development of 12 (40%) patients remained at the preoperative development level. The median number of antiseizure medications taken preoperatively was 5.27 (range 2-10), which decreased to 1.90 (range 0-4) at the last follow-up. Seizure duration, etiology, positive positron emission tomography-magnetic resonance imaging (PET-MRI), surgery type, and lesion location were significantly correlated with the postoperative efficacy (P < 0.05). Positive PET/MRI findings and lesion location predicted independently the postoperative outcomes. Permanent impairments of motor or language function were rare, with only two cases reporting hydrocephalus and one reporting hemiplegia.

Conclusions: Surgery is an effective treatment option for children with IESS. Early referral and comprehensive preoperative evaluation are essential for identification of surgically treatable structural lesions. The primary surgically treatable cause is cortical malformation, followed by perinatal brain injury. Hemispheric disconnection is a preferred surgical approach. Positive PET/MRI findings and lesion location predicted the postoperative outcomes.

Keywords: Drug-refractory epilepsy; Hemispherotomy; Infantile epileptic spasms syndrome; Post-surgical outcome; Structural lesions; Surgical treatment.