on behalf of the EPISTOP consortium †Objective To correlate fetal brain magnetic resonance imaging (MRI) findings with epilepsy characteristics and neurodevelopment at 2 years of age in children with tuberous sclerosis complex (TSC) to improve prenatal counseling.Study design This retrospective cohort study was performed in a collaboration between centers of the EPISTOP consortium. We included children with definite TSC, fetal MRIs, and available follow-up data at 2 years of age. A pediatric neuroradiologist masked to the patient's clinical characteristics evaluated all fetal MRIs. MRIs were categorized for each of the 10 brain lobes as score 0: no (sub)cortical lesions or doubt; score 1: a single small lesion; score 2: more than one small lesion or at least one large lesion (>5 mm). Neurologic manifestations were correlated to lesion sum scores.Results Forty-one children were included. Median gestational age at MRI was 33.3 weeks; (sub)cortical lesions were detected in 97.6%. Mean lesion sum score was 4.5. At 2 years, 58.5% of patients had epilepsy and 22% had drug-resistant epilepsy. Cognitive, language, and motor development were delayed in 38%, 81%, and 50% of patients, respectively. Autism spectrum disorder (ASD) was diagnosed in 20.5%. Fetal MRI lesion sum scores were significantly associated with cognitive and motor development, and with ASD diagnosis, but not with epilepsy characteristics.Conclusions Fetal cerebral lesion scores correlate with neurodevelopment and ASD at 2 years in children with TSC.

Background Cerebrospinal fluid (CSF) leak is widely recognized as a challenging and commonly occurring postoperative complication of transsphenoidal surgery (TSS).The primary objective of this study is to benchmark the current prevalence of CSF leak after TSS in the adult population. Methods The authors followed the PRISMA guidelines. The PubMed, Embase, and Cochrane Library databases were searched for articles reporting CSF leak after TSS in the adult population. Meta-analysis was performed using the Untransformed Proportion metric in OpenMetaAnalyst. For two between-group comparisons a generalized linear mixed model was applied. Results We identified 2,408 articles through the database search, of which 70, published since 2015, were included in this systematic review. These studies yielded 24,979 patients who underwent a total of 25,034 transsphenoidal surgeries. The overall prevalence of postoperative CSF leak was 3.4% (95% confidence interval or CI 2.8–4.0%). The prevalence of CSF leak found in patients undergoing pituitary adenoma resection was 3.2% (95% CI 2.5–4.2%), whereas patients who underwent TSS for another indication had a CSF leak prevalence rate of 7.1% (95% CI 3.0–15.7%) (odds ratio [OR] 2.3, 95% CI 0.9–5.7). Patients with cavernous sinus invasion (OR 3.0, 95% CI 1.1–8.7) and intraoperative CSF leak (OR 5.9, 95% CI 3.8–9.0) have increased risk of postoperative CSF leak. Previous TSS and microscopic surgery are not significantly associated with postoperative CSF leak. Conclusion The overall recent prevalence of CSF leak after TSS in adults is 3.4%. Intraoperative CSF leak and cavernous sinus invasion appear to be significant risk factors for postoperative CSF leak.

Background and purpose Patient and aneurysm characteristics have been reported to differ between patients with familial and non-familial intracranial aneurysms (IAs), although results are inconsistent. We systematically reviewed and meta-analyzed the literature to identify and quantify patient- and aneurysm characteristics associated with familial IAs. Methods We searched PubMed and EMBASE for case-control and cohort studies comparing patient- and aneurysm characteristics between familial and non-familial IAs. Two observers independently assessed study eligibility and appraised quality with the Newcastle Ottawa Scale. With univariable weighted linear regression analysis we calculated β-coefficients with corresponding 95% confidence intervals (CIs) for ruptured and unruptured IAs combined and for ruptured IAs only. Heterogeneity was assessed with Higgins I 2 . Results A total of 15 articles were included in the meta-analysis in which 16,346 patients were analyzed with a total of 14,225 IAs. For ruptured and unruptured IAs combined, multiple IAs were more prevalent in familial (28.5%) than in non-familial IAs (20.4%; β = 0.10, 95% CI, 0.04 to 0.16; I 2 0%). For ruptured IAs only, in familial patients IAs were more prevalent on the middle cerebral artery (41.1% versus 29.5%; β = 0.12, 95% CI, 0.01 to 0.24; I 2 12%) and ruptured at a younger age (46.5 years versus 50.8 years; β = -5.00, 95% CI, -9.31 to -0.69; I 2 98%) than in non-familial patients. No significant differences were found for the proportion of women, size of the aneurysm at time of rupture, smoking or hypertension. Conclusion These results suggest that characteristics of familial and non-familial IAs show considerable overlap, yet differ on specific aspects. However, results for age at rupture showed considerable heterogeneity. These findings should be taken into consideration for future etiological research into IAs.