Deep venous communication in vein of Galen malformations: incidence, Imaging, and Implications for treatment.

BACKGROUND: Failure to appreciate deep venous drainage pathways is a major cause of severe complications in the endovascular treatment of vein of Galen aneurysmal malformations (VOGMs). OBJECTIVE: To report deep venous drainage patterns in patients with VOGM, emphasizing the internal cerebral veins, and to describe the challenges in evaluating these. METHODS: Patients with VOGM presenting to our institute between 2000 and 2018 were retrospectively analyzed. Patients with complete and good quality imaging datasets were included in the study. Three neuroradiologists with expertise in the subject independently analyzed the deep venous drainage patterns on multi-sequence MRI and digital subtraction angiography. Follow-up imaging studies were analyzed for alterations in deep venous drainage patterns that occurred following endovascular treatment. Descriptive statistics were used to report findings. RESULTS: Twenty-three patients had optimal quality MRI imaging and 25 had optimal quality DSA imaging available. In 14/23 (61%) patients, internal cerebral vein (ICV) communication could be reliably identified on MRI and in 8/25 (32%) patients on DSA. Deep venous communication with the VOGM was demonstrated in 8/26 (30.8%) patients. One (3.8%) patient demonstrated ICV communication with the VOGM only on postoperative imaging, while in 2 (8%) patients the ICV drainage route changed from VOGM to alternative pathways after the procedure. Other variant pathways included lateral mesencephalic vein, superior or inferior sagittal sinus, anterior mesencephalic vein, tentorial sinus, deep Sylvian vein, and superior vermian vein. CONCLUSION: ICV communication with the VOGM is not uncommon and requires dedicated preprocedural imaging to identify it. However, there are significant challenges in assessing this communication in the presence of high-flow fistulae, vessel tortuosity and size, and contrast limitations in this population.

Congenital spine and spinal cord malformations--self-assessment module.

The educational objectives of this self-assessment module are for the participants to exercise, self-assess, and improve their understanding of the most important features of congenital spine and spinal cord malformations.

Predicting factors for the follow-up outcome and management decisions in vein of Galen aneurysmal malformations.

PURPOSE: Vein of Galen aneurysmal malformations (VGAMs) are choroidal arteriovenous malformations that develop during an early embryonic stage. Although recent reports have shown improved outcome for these patients, the overall outcome still is poor. In this study, we evaluated the clinical, imaging, and angiographic features that may predict the outcome in VGAM patients. METHODS: Twenty-five patients diagnosed with VGAM were reviewed for clinical symptoms, including neonatal scoring systems, imaging findings, angioarchitecture, treatment decision, initial treatment age, follow-up timing, and follow-up outcome. RESULTS: Factors that were significantly associated with a poor outcome (p < 0.05) included neurological symptoms at presentation, a medium-to-low overall neonatal score (<12/21), a very poor score (<2/5) in one (or more) categories, focal parenchymal changes, calcifications, tonsillar herniation, arterial steal, or more than two groups of multiple arterial feeders. The venous drainage pattern and treatment age were not significantly associated with the overall outcome. CONCLUSIONS: The presence of multiple factors that are related with poor outcome may warrant withholding aggressive treatment, while a small subgroup of carefully selected patients without any of these factors who are clinically asymptomatic may have a good outcome even with conservative management and close follow-up. For all other patients in which treatment is considered, the optimal treatment time is at 4-5 months of age; however, urgent treatment, regardless of age, should be indicated in those that do not have permanent brain damage on imaging with deteriorating congestive heart failure, evidence of arterial steal, or progressive occlusion of the venous outflow.

Malformations of cortical development.

Malformations of cortical development are an important cause of developmental delay and epilepsy. Proper identification of these malformations can greatly help in accurately counseling affected families and, in some cases, in the treatment of the epilepsy. Modem neuroimaging is an important tool in the diagnosis of these malformations.

Neuroimaging in disorders of cortical development.

Malformations of cortical development are important causes of developmental delay and epilepsy. They are classified by the presumed stage during which normal development is interrupted: neuronal proliferation and differentiation, neuronal migration, and late migration/cortical organization. This article discusses the important malformations in each of these groups, how and why the malformations develop, and their imaging findings. A better understanding of these disorders helps in genetic counseling of the parents and may help in the treatment of associated epilepsy.

Metabolic and electrophysiological alterations in subtypes of temporal lobe epilepsy: a combined proton magnetic resonance spectroscopic imaging and depth electrodes study.

PURPOSE: This study compared the metabolic regional alterations, characterized by proton magnetic spectroscopic imaging ((1)H-MRSI), with electrophysiological abnormalities recorded by using depth electrodes and with structural lesions, in patients with several subtypes of temporal lobe epilepsy (TLE). METHODS: Twenty-five subjects were investigated, including 15 controls and 10 patients with drug-resistant unilateral TLE, nine of whom had structural abnormalities identified by MRI. All patients underwent noninvasive presurgical evaluation and then stereoelectroencephalography (SEEG). We performed an original metabolic exploration combining two (1)H-MRS imaging acquisitions associated with two single-voxel acquisitions (temporal poles) to map the most informative regions of interest (ROIs) including mesial and neocortical localizations. The N-acetyl aspartate/(choline+creatine) ratio was chosen as a metabolic index. SEEG analysis allowed the classification of each ROI as electrically normal or abnormal (i.e., involved in ictal and/or interictal discharges). Groups were compared by using a nonparametric Mann-Whitney U test. RESULTS: N-Acetyl aspartate/(choline+creatine) was significantly lower in all regions involved in SEEG electrophysiological epileptic abnormalities than in controls (p < 0.05). In contrast, the regions without any electrophysiological abnormalities were not metabolically different from those in controls (p > 0.05) except in one ROI. No differences between the metabolic profiles of epileptogenic and irritative zones were found. The metabolic alterations included, but also extended beyond, the lesions. The presence of metabolic abnormalities in mesial structures was not specific for the mesial subtype and generally extended outside the mesial structures. CONCLUSIONS: These results indicate that metabolic abnormalities are linked to ictal and interictal epileptiform activities rather than to structural alterations in TLE.