Focal lesionectomy as surgical treatment of epilepsy in patients with Sturge-Weber syndrome: a case-based systematic review and meta-analysis

Abstract

OBJECTIVE Sturge-Weber syndrome (SWS) is a rare neurocutaneous disorder presenting mostly with a facial port-wine stain and leptomeningeal angiomatosis. More than 85% of the patients are affected by epilepsy by the age of 2 years. Seizure and symptom control is the focus of SWS treatment, since no causal therapy exists yet. For pharmacologically intractable epilepsy, surgery is a treatment option. The aim of this systematic review and meta-analysis was to provide an overview of the literature regarding lesionectomy in SWS with a focus on seizure outcome, complications, and motor and cognitive development. METHODS The PubMed and Embase databases were searched using a systematic search strategy to identify studies on SWS from their inception until 2021. Two independent researchers assessed the studies for inclusion and quality. Outcome measures were seizure outcome, postoperative complications, and motor and cognitive development. Thereafter, a systematic review was conducted, and a meta-analysis was performed for all included cohort studies. Risk of bias was assessed using the Newcastle-Ottawa Scale. Forest plots have been generated for all outcomes; risk ratio was used for pooled outcomes. A p value < 0.05 was considered as statistically significant. RESULTS After removal of duplicates, the authors screened 439 articles, of which 9 articles with 150 patients were included. Our case and 5 case reports and 4 retrospective cohort studies were included for systematic review. The latter 4 studies qualified for the meta-analysis. In these 4 articles, 144 patients received surgical treatment: 81 (56%) underwent focal lesionectomy and 63 (44%) hemispherectomy. Pooled outcome analysis for postoperative favorable seizure outcome showed a nonsignificant difference between lesionectomy and hemispherectomy (69.2% vs 87.3%; RR 0.73, 95% CI 0.50–1.08; t = −2.56, p = 0.08). Lesionectomy showed a significantly lower rate for developmental delay and postoperative hemiparesis in comparison with hemispherectomy (29.8% vs 76.3%; RR 0.41, 95% CI 0.28–0.59; z = −4.77, p < 0.0001 and 18.1% vs 100%; RR 0.11, 95% CI 0.06–0.21; z = −6.58, p < 0.0001, respectively). CONCLUSIONS Based on the limited literature available, lesionectomy leads to a nonsignificant lower seizure control rate, while postoperative developmental or motor deficits are significantly lower compared with hemispherectomy. Therefore, focal lesionectomy remains a valid alternative to hemispherectomy in SWS with a clearly localized epileptogenic area; however, individual case-based decisions in a specialized multidisciplinary team are of paramount importance.

Seizure, Motor, and Cognitive Outcomes After Epilepsy Surgery for Patients With Sturge-Weber Syndrome

“…6,10,11 A previous meta-analysis reported a pooled seizure-free rate of 69.2%. 15 This study revealed similar seizure-free probabilities to our previous single-center experience (79.5% for 2 years of follow-up), 14 but the rates observed were slightly greater than those of earlier studies, 6,10,11 potentially due to the increased proportions of full resection in this study. 11,23 Some studies have suggested that FR leads to worse seizure outcomes than HS and results in only limited seizure-free periods, 9,26 while other studies and current findings have indicated that FR could also result in similar long-term seizure control as HS.…”

Sturge Weber Syndrome Roach Type I: A case report

“…Convulsions manifesting in early childhood, are the commonest neurological feature of SWS, which are usually focal complex or secondary generalized partial in type. 14 The index case presented with convulsions from one year of age with new onset of left hemiparesis in adulthood. However, his mental scores were normal, and had no developmental delay or headache.…”

“…Endocrine dysfunctions in the form of growth hormone insufficiency and secondary hypothyroidism have been reported. 13,14 Medical treatment of seizures has widely been adopted as a means of seizure control, but some patients continue to have intractable convulsions in spite of optimum treatment. In them, surgery is a presumed alternate.…”

“…However, a case-based systematic review and meta-analysis failed to demonstrate any significant improvement in seizure control after lesionectomy in SWS patients; but postoperative cases showed improvement in hemiparesis. 15 The presence of brain calcification provides important evidence of leptomeningeal angiomas, and tissue diagnosis is usually not warranted.…”

Sturge–Weber syndrome: Updates in pathogenesis, diagnosis, and treatment

“…In a study of 10 patients with SWS who underwent functional hemispherectomy, 80% of children became seizure‐free, and those who did not achieve seizure freedom had incomplete resection of epileptic regions 121,127 . Functional lesional surgeries risk less postoperative developmental and motor deficits than hemispheric surgeries 128 but are less likely to result in full seizure control, especially when ictal regions are not completely removed 129,130 . Vagus nerve stimulation may also aid seizure control in drug‐resistant epilepsy, but only anecdotal reports are available for SWS 131 .…”