Pressure-Lowering Effect of Interventional Fistula Occlusion in Secondary Glaucoma due to Orbital Draining Intracerebral Fistulae. / Drucksenkender Effekt der interventionellen Fistelokklusion bei Sekundärglaukom infolge orbital drainierender intrazerebraler Fisteln.

BACKGROUND: Carotid cavernous sinus fistulas (CSCF) are pathological connections of the internal and/or external carotid artery (and/or its branches) to the cavernous sinus. Ophthalmological symptoms and problems occur particularly when drainage is via the superior ophthalmic vein. MATERIAL AND METHODS: Seven eyes of six patients with a high-grade suspicion of CSCF were included in this retrospective monocentric study. Digital subtraction angiography (DSA) was performed in the included patients, where an interventional fistula closure was performed in the case of CSCF. Four of the six patients received a pre- and post-interventional day-night intraocular pressure profile. Furthermore, medical history, symptoms, visual acuity, slit lamp microscopic findings, and DSA findings were evaluated. RESULTS: The most common symptoms reported by patients were red eyes, diplopia, and exophthalmos. When the intraocular pressure (IOP) was measured, 83.33% of the patients showed increased values. The mean IOP in the day-night intraocular pressure profile in the affected eye before intervention was 23.5 (± 2.7) mmHg compared to 14.1 (± 2.3) mmHg in the healthy eye. A significant difference could thus be demonstrated in side comparison (p = 0.0047). The post-interventional measurement showed a mean IOP of 15.3 (± 1.0) mmHg in the affected eye and thus a significant difference to the pre-interventional measurement in the affected eye (p = 0.0018). Four of the six patients with CSCF were taking antiglaucomatous eye drops before the intervention, and two patients after the intervention. The number of antiglaucoma drugs used could also be reduced. CONCLUSION: Interventional fistula closure is an effective method for treating the secondary increase of IOP in CSCF. Successful closure of the fistula showed a significant reduction in IOP, which was not possible with the sole administration of antiglaucoma drugs. Radius-Maumenee syndrome should be considered as a differential diagnosis.

Investigation of the functional pathogenesis of mild cognitive impairment by localisation-based locus coeruleus resting-state fMRI.

Dementia as one of the most prevalent diseases urges for a better understanding of the central mechanisms responsible for clinical symptoms, and necessitates improvement of actual diagnostic capabilities. The brainstem nucleus locus coeruleus (LC) is a promising target for early diagnosis because of its early structural alterations and its relationship to the functional disturbances in the patients. In this study, we applied our improved method of localisation-based LC resting-state fMRI to investigate the differences in central sensory signal processing when comparing functional connectivity (fc) of a patient group with mild cognitive impairment (MCI, n = 28) and an age-matched healthy control group (n = 29). MCI and control participants could be differentiated in their Mini-Mental-State-Examination (MMSE) scores (p < .001) and LC intensity ratio (p = .010). In the fMRI, LC fc to anterior cingulate cortex (FDR p < .001) and left anterior insula (FDR p = .012) was elevated, and LC fc to right temporoparietal junction (rTPJ, FDR p = .012) and posterior cingulate cortex (PCC, FDR p = .021) was decreased in the patient group. Importantly, LC to rTPJ connectivity was also positively correlated to MMSE scores in MCI patients (p = .017). Furthermore, we found a hyperactivation of the left-insula salience network in the MCI patients. Our results and our proposed disease model shed new light on the functional pathogenesis of MCI by directing to attentional network disturbances, which could aid new therapeutic strategies and provide a marker for diagnosis and prediction of disease progression.

Mutations in PRDM15 Are a Novel Cause of Galloway-Mowat Syndrome.

BACKGROUND: Galloway-Mowat syndrome (GAMOS) is characterized by neurodevelopmental defects and a progressive nephropathy, which typically manifests as steroid-resistant nephrotic syndrome. The prognosis of GAMOS is poor, and the majority of children progress to renal failure. The discovery of monogenic causes of GAMOS has uncovered molecular pathways involved in the pathogenesis of disease. METHODS: Homozygosity mapping, whole-exome sequencing, and linkage analysis were used to identify mutations in four families with a GAMOS-like phenotype, and high-throughput PCR technology was applied to 91 individuals with GAMOS and 816 individuals with isolated nephrotic syndrome. In vitro and in vivo studies determined the functional significance of the mutations identified. RESULTS: Three biallelic variants of the transcriptional regulator PRDM15 were detected in six families with proteinuric kidney disease. Four families with a variant in the protein's zinc-finger (ZNF) domain have additional GAMOS-like features, including brain anomalies, cardiac defects, and skeletal defects. All variants destabilize the PRDM15 protein, and the ZNF variant additionally interferes with transcriptional activation. Morpholino oligonucleotide-mediated knockdown of Prdm15 in Xenopus embryos disrupted pronephric development. Human wild-type PRDM15 RNA rescued the disruption, but the three PRDM15 variants did not. Finally, CRISPR-mediated knockout of PRDM15 in human podocytes led to dysregulation of several renal developmental genes. CONCLUSIONS: Variants in PRDM15 can cause either isolated nephrotic syndrome or a GAMOS-type syndrome on an allelic basis. PRDM15 regulates multiple developmental kidney genes, and is likely to play an essential role in renal development in humans.

Impact of acetylsalicylic acid in patients undergoing cerebral aneurysm surgery - should the neurosurgeon really worry about it?

There has been an increase in the use of acetylsalicylic acid (ASA, Aspirin®) among patients with stroke and heart disease as well as in aging populations as a means of primary prevention. The potentially life-threatening consequences of a postoperative hemorrhagic complication after neurosurgical operative procedures are well known. In the present study, we evaluate the risk of continued ASA use as it relates to postoperative hemorrhage and cardiopulmonary complications in patients undergoing cerebral aneurysm surgery. We retrospectively analyzed 200 consecutive clipping procedures performed between 2008 and 2018. Two different statistical models were applied. The first model consisted of two groups: (1) group with No ASA impact - patients who either did not use ASA at all as well as those who had stopped their use of the ASA medication in time (> = 7 days prior to operation); (2) group with ASA impact - all patients whose ASA use was not stopped in time. The second model consisted of three groups: (1) No ASA use; (2) Stopped ASA use (> = 7 days prior to operation); (3) Continued ASA use (did not stop or did not stop in time, <7 days prior to operation). Data collection included demographic information, surgical parameters, aneurysm characteristics, and all hemorrhagic/thromboembolic complications. A postoperative hemorrhage was defined as relevant if a consecutive operation for hematoma removal was necessary. An ASA effect has been assumed in 32 out of 200 performed operations. A postoperative hemorrhage occurred in one out these 32 patients (3.1%). A postoperative hemorrhage in patients without ASA impact was detected and treated in 5 out of 168 patients (3.0%). The difference was statistically not significant in either model (ASA impact group vs. No ASA impact group: OR = 1.0516 [0.1187; 9.3132], p = 1.000; RR = 1.0015 [0.9360; 1.0716]). Cardiopulmonary complications were significantly more frequent in the group with ASA impact than in the group without ASA impact (p = 0.030). In this study continued ASA use was not associated with an increased risk of a postoperative hemorrhage. However, cardiopulmonary complications were significantly more frequent in the ASA impact group than in the No ASA impact group. Thus, ASA might relatively safely be continued in patients with increased cardiovascular risk and cases of emergency cerebrovascular surgery.

In vivo anatomical mapping of human locus coeruleus functional connectivity at 3 T MRI.

The locus coeruleus (LC) is involved in numerous crucial brain functions and several disorders like depression and Alzheimer's disease. Recently, the LC resting-state functional connectivity (rs-fc) has been investigated in functional MRI by calculating the blood oxygen level-dependent (BOLD) response extracted using Montreal Neurological Institute (MNI) space masks. To corroborate these results, we aimed to investigate the LC rs-fc at native space by improving the identification of the LC location using a neuromelanin sensitive sequence. Twenty-five healthy male participants (mean age 24.8 ± 4.2) were examined in a Siemens MAGNETOM Prisma 3 T MRT applying a neuromelanin sensitive T1TSE sequence and functional MRI. We compared the rs-fc of LC calculated by a MNI-based approach with extraction of the BOLD signal at the exact individual location of the LC after applying CompCor and field map correction. As a measure of advance, a marked increase of regional homogeneity (ReHo) of time series within LC could be achieved with the subject-specific approach. Furthermore, the methods differed in the rs-fc to the right temporoparietal junction, which showed stronger connectivity to the LC in the MNI-based method. Nevertheless, both methods comparably revealed LC rs-fc to multiple brain regions including ACC, bilateral thalamus, and cerebellum. Our results are relevant for further research assessing and interpreting LC function, especially in patient populations examined at 3 T MRI.

Acute symptomatic extracranial internal carotid occlusion - natural course and clinical impact.

Background: To assess the vascular and clinical course of acute symptomatic extracranial internal carotid artery (ICA) occlusion. Patients and methods: Patients with an acute ischemic event in the anterior circulation and corresponding extracranial ICA occlusion at CT angiography and/or color-coded duplex sonography underwent recurrent duplex follow-up for detection of spontaneous recanalization. Stroke recurrence and functional outcome 4.5 months after the ischemic index event assessed by modified Rankin scale served as secondary outcome parameters. Results: 133 patients (91 men, mean age 62.3 years, SD 10.8) demonstrated symptomatic occlusion of the extracranial ICA with open intracranial ICA and open middle cerebral artery and were followed-up for spontaneous recanalization. Twenty-eight recanalized spontaneously, 25 to high-grade focal stenosis within 12 days, revealing an early cumulative recanalization rate of 23 %. Detection of recanalization was independently associated with de novo dual anti-platelet therapy (adjusted odds ratio [OR], 3.24; 95 % confidence interval [CI], 1.34 to 7.80). Ischemic recurrence occurred in 16 patients, of which 10 deemed to be embolic and 5 hemodynamic. Spontaneous ICA recanalization and an exhausted cerebrovascular reserve in the hemisphere distal to the occluded ICA were both independently associated with the occurrence of a recurrent ischemic event at Cox regression. An increasing NIHSS score at admission, a decreasing middle cerebral artery flow velocity and an ischemic recurrence independently predicted poor outcome (modified Rankin scale 3 to 6) in multivariate analysis. Conclusions: Acute symptomatic extracranial ICA occlusion is an unstable condition with frequent spontaneous recanalization to severe stenosis and early embolic stroke recurrence, demanding appropriate prevention especially in those patients with only mild deficit.

Deployment of flow diverter devices: prediction of foreshortening and validation of the simulation in 18 clinical cases.

PURPOSE: Flow diverter (FD) devices show severe shortening during deployment in dependency of the vessel geometry. Valid information regarding the geometry of the targeted vessel is therefore mandatory for correct device selection, and to avoid complications. But the geometry of diseased tortuous intracranial vessels cannot be measured accurately with standard methods. The goal of this study is to prove the accuracy of a novel virtual stenting method in prediction of the behavior of a FD in an individual vessel geometry. METHODS: We applied a virtual stenting method on angiographic 3D imaging data of the specific vasculature of patients, who underwent FD treatment. The planning tool analyzes the local vessel morphology and deploys the FD virtually. We measured in 18 cases the difference between simulated FD length and real FD length after treatment in a landmark-based registration of pre-/post-interventional 3D angiographic datasets. RESULTS: The mean value of length deviation of the virtual FD was 2.2 mm (SD ± 1.9 mm) equaling 9.5% (SD ± 8.2%). Underestimated cases present lower deviations compared with overestimated FDs. Flow diverter cases with a nominal device length of 20 mm had the highest prediction accuracy. CONCLUSION: The results suggest that the virtual stenting method used in this study is capable of predicting FD length with a clinically sufficient accuracy in advance and could therefore be a helpful tool in intervention planning. Imaging data of high quality are mandatory, while processing and manipulation of the FD during the intervention may impact the accuracy.

DOCK6 mutations are responsible for a distinct autosomal-recessive variant of Adams-Oliver syndrome associated with brain and eye anomalies.

Adams-Oliver syndrome (AOS) is characterized by the association of aplasia cutis congenita with terminal transverse limb defects, often accompanied by additional cardiovascular or neurological features. Both autosomal-dominant and autosomal-recessive disease transmission have been observed, with recent gene discoveries indicating extensive genetic heterogeneity. Mutations of the DOCK6 gene were first described in autosomal-recessive cases of AOS and only five DOCK6-related families have been reported to date. Recently, a second type of autosomal-recessive AOS has been attributed to EOGT mutations in three consanguineous families. Here, we describe the identification of 13 DOCK6 mutations, the majority of which are novel, across 10 unrelated individuals from a large cohort comprising 47 sporadic cases and 31 AOS pedigrees suggestive of autosomal-recessive inheritance. DOCK6 mutations were strongly associated with structural brain abnormalities, ocular anomalies, and intellectual disability, thus suggesting that DOCK6-linked disease represents a variant of AOS with a particularly poor prognosis.

DOCK6 Mutations Are Responsible for a Distinct Autosomal-Recessive Variant of Adams-Oliver Syndrome Associated with Brain and Eye Anomalies.

The original article to which this Erratum refers was published in Human Mutation 36(6):593­598(DOI:10.1002/humu22795).The authors realized that a co-author, Nuria C. Bramswig, was left off of the title page of this article at the time of submission. This erratum serves to correct this error by including Dr. Bramswig and Dr. Bramswig's institution in the title page information.The authors regret the error.

[Ischemic stroke--interventional therapy]. / Ischämischer Insult - Interventionelle Therapie.

In addition to the long-established local thrombolysis by means of a catheter, other mechanical procedures for the interventional treatment of acute ischemic stroke have been introduced over the last ten years. These new methods have proved to be effective for the recanalization of occluded vessels supplying the brain and can, in principle, also be employed outside of the therapeutic window valid for intravenous thrombolysis. For just this reason such interventionaltherapies are being increasingly used, also in combination with intravenous thrombolysis. However, higher demands are placed on imaging diagnostics in order to protect the patients from unnecessary risks resulting from these invasive measures. Since this is still a very new therapeutic procedure it has not yet been conclusively demonstrated that the interventional method, in spite of all theoretical advantages in comparison with intravenous thrombolysis, really does lead to a better outcome for the patients. The indication must thus be made on an individual basis at the interdisciplinary level.

3D-visualization of segmented contacts of directional deep brain stimulation electrodes via registration and fusion of CT and FDCT.

OBJECTIVES: 3D-visualization of the segmented contacts of directional deep brain stimulation (DBS) electrodes is desirable since knowledge about the position of every segmented contact could shorten the timespan for electrode programming. CT cannot yield images fitting that purpose whereas highly resolved flat detector computed tomography (FDCT) can accurately image the inner structure of the electrode. This study aims to demonstrate the applicability of image fusion of highly resolved FDCT and CT to produce highly resolved images that preserve anatomical context for subsequent fusion to preoperative MRI for eventually displaying segmented contactswithin anatomical context in future studies. MATERIAL AND METHODS: Retrospectively collected datasets from 15 patients who underwent bilateral directional DBS electrode implantation were used. Subsequently, after image analysis, a semi-automated 3D-registration of CT and highly resolved FDCT followed by image fusion was performed. The registration accuracy was assessed by computing the target registration error. RESULTS: Our work demonstrated the feasibility of highly resolved FDCT to visualize segmented electrode contacts in 3D. Semiautomatic image registration to CT was successfully implemented in all cases. Qualitative evaluation by two experts revealed good alignment regarding intracranial osseous structures. Additionally, the average for the mean of the target registration error over all patients, based on the assessments of two raters, was computed to be 4.16 mm. CONCLUSION: Our work demonstrated the applicability of image fusion of highly resolved FDCT to CT for a potential workflow regarding subsequent fusion to MRI in the future to put the electrodes in an anatomical context.

Correlation of CT-based bone mineralization with drilling-force measurements in anatomical specimens is suitable to investigate planning of trans-pedicular spine interventions.

This interdisciplinary study examined the relationship between bone density and drilling forces required during trans-pedicular access to the vertebra using fresh-frozen thoraco-lumbar vertebrae from two female body donors (A, B). Before and after biomechanical examination, samples underwent high-resolution CT-quantification of total bone density followed by software-based evaluation and processing. CT density measurements (n = 4818) were calculated as gray values (GV), which were highest in T12 for both subjects (GVmaxA = 3483.24, GVmaxB = 3160.33). Trans-pedicular drilling forces F (Newton N) were highest in L3 (FmaxB = 5.67 N) and L4 (FmaxA = 5.65 N). In 12 out of 13 specimens, GVs significantly (p < 0.001) correlated with force measurements. Among these, Spearman correlations r were poor in two lumbar vertebrae, fair in five specimens, and moderately strong in another five specimens, and highest for T11 (rA = 0.721) and L5 (rB = 0.690). Our results indicate that CT-based analysis of vertebral bone density acquired in anatomical specimens is a promising approach to predict the drilling force appearance as surrogate parameter of its biomechanical properties by e.g., linear regression analysis. The study may be of value as basis for biomechanical investigations to improve planning of the optimal trajectory and to define safety margins for drilling forces during robotic-assisted trans-pedicular interventions on the spine in the future.

Diffusion Weighted Imaging in Gliomas: A Histogram-Based Approach for Tumor Characterization.

(1) Background: Astrocytic gliomas present overlapping appearances in conventional MRI. Supplementary techniques are necessary to improve preoperative diagnostics. Quantitative DWI via the computation of apparent diffusion coefficient (ADC) histograms has proven valuable for tumor characterization and prognosis in this regard. Thus, this study aimed to investigate (I) the potential of ADC histogram analysis (HA) for distinguishing low-grade gliomas (LGG) and high-grade gliomas (HGG) and (II) whether those parameters are associated with Ki-67 immunolabelling, the isocitrate-dehydrogenase-1 (IDH1) mutation profile and the methylguanine-DNA-methyl-transferase (MGMT) promoter methylation profile; (2) Methods: The ADC-histograms of 82 gliomas were computed. Statistical analysis was performed to elucidate associations between histogram features and WHO grade, Ki-67 immunolabelling, IDH1 and MGMT profile; (3) Results: Minimum, lower percentiles (10th and 25th), median, modus and entropy of the ADC histogram were significantly lower in HGG. Significant differences between IDH1-mutated and IDH1-wildtype gliomas were revealed for maximum, lower percentiles, modus, standard deviation (SD), entropy and skewness. No differences were found concerning the MGMT status. Significant correlations with Ki-67 immunolabelling were demonstrated for minimum, maximum, lower percentiles, median, modus, SD and skewness; (4) Conclusions: ADC HA facilitates non-invasive prediction of the WHO grade, tumor-proliferation rate and clinically significant mutations in case of astrocytic gliomas.

Radiofrequency lesioning for epileptogenic periventricular nodular heterotopia: a rational approach.

Periventricular nodular heterotopias (PNHs) are frequently associated with pharmacoresistant epilepsy. They are considered part of a dysfunctional network, connected to the overlying cortex. Therefore, removal of the PNHs and additional cortectomy or lobectomy seem to be essential for significant and long-lasting seizure reduction. These procedures, however, can have considerable limitations, especially in patients with functional eloquent cortex adjacent to the PNH. Alternatively, stereotactic neurosurgery can reduce the surgical trauma. Presented is a 56-year-old man who became seizure-free after stereotactically guided radiofrequency lesioning of a solitary PNH.

Impact of stents and flow diverters on hemodynamics in idealized aneurysm models.

Cerebral aneurysms constitute a major medical challenge as treatment options are limited and often associated with high risks. Statistically, up to 3% of patients with a brain aneurysm may suffer from bleeding for each year of life. Eight percent of all strokes are caused by ruptured aneurysms. In order to prevent this rupture, endovascular stenting using so called flow diverters is increasingly being regarded as an alternative to the established coil occlusion method in minimally invasive treatment. Covering the neck of an aneurysm with a flow diverter has the potential to alter the hemodynamics in such a way as to induce thrombosis within the aneurysm sac, stopping its further growth, preventing its rupture and possibly leading to complete resorption. In the present study the influence of different flow diverters is quantified considering idealized patient configurations, with a spherical sidewall aneurysm placed on either a straight or a curved parent vessel. All important hemodynamic parameters (exchange flow rate, velocity, and wall shear stress) are determined in a quantitative and accurate manner using computational fluid dynamics when varying the key geometrical properties of the aneurysm. All simulations are carried out using an incompressible, Newtonian fluid with steady conditions. As a whole, 72 different cases have been considered in this systematic study. In this manner, it becomes possible to compare the efficiency of different stents and flow diverters as a function of wire density and thickness. The results show that the intra-aneurysmal flow velocity, wall shear stress, mean velocity, and vortex topology can be considerably modified thanks to insertion of a suitable implant. Intra-aneurysmal residence time is found to increase rapidly with decreasing stent porosity. Of the three different implants considered in this study, the one with the highest wire density shows the highest increase of intra-aneurysmal residence time for both the straight and the curved parent vessels. The best hemodynamic modifications are always obtained for a small aneurysm diameter.

Endovascular Treatment of Ruptured Middle Cerebral Artery Bifurcation Aneurysms. A Retrospective Observational Study of Short- and Long-Term Follow-Up.

PURPOSE: With the introduction of new devices and the development of approved devices, endovascular techniques are more and more considered as a treatment option for middle cerebral artery aneurysms (MCA). In this study, we present data on ruptured MCA aneurysms that were treated endovascularly in our hospital. MATERIAL AND METHODS: In a retrospective case series of ruptured MCA bifurcation aneurysms, 118 (94%) cases were managed endovascularly between May 2008 and July 2017. Most were managed with coiling (= 62) and the remaining were managed with stent-assisted-coiling (= 35) and endovascular flow disruptor (WEB-device) (= 21). We measured the clinical outcome at the time of discharge and long-term follow-up on the modified Rankin Scale (mRS) and evaluated the rates of periprocedural complications and retreatment. RESULTS: A good clinical outcome (mRS 0-2) at discharge was achieved in 58.5% of these cases. Mortality rate was 19.5%. Nine aneurysms required retreatment. Eighty-three percent demonstrated a good clinical outcome at long-term follow-up (mRS 0-2). In total, 6 (5.1%) procedure-related complications and 10 (8.5%) disease-related complications occurred. No significant difference between reintervention, complications or outcome was found between the employment of different devices (P > 0.05). Endovascular treatment of ruptured MCA aneurysms at our practice showed similar morbidity and mortality to data published about surgical clipping. CONCLUSION: The endovascular device evolution permits a feasible and safe treatment of ruptured MCA bifurcation aneurysms. Endovascular treatment can therefore be considered as an alternative treatment option to microsurgery for this type of aneurysm.

Indirect Flow Diversion for Off-Centered Bifurcation Aneurysms and Distant Small-Vessel Aneurysms, a Retrospective Proof of Concept Study From Five Neurovascular Centers.

Background: Treatment of cerebral aneurysms using hemodynamic implants such as endosaccular flow disruptors and endoluminal flow diverters has gained significant momentum during recent years. The intended target zone of those devices is the immediate interface between aneurysm and parent vessel. The therapeutic success is based on the reduction of aneurysmal perfusion and the subsequent formation of a neointima along the surface of the implant. However, a subset of aneurysms-off-centered bifurcation aneurysms involving the origin of efferent branches and aneurysms arising from peripheral segments of small cerebral vessels-oftentimes cannot be treated via coiling or implanting a hemodynamic implant at the neck level for technical reasons. In those cases, indirect flow diversion-a flow diverter deployed in the main artery proximal to the parent vessel of the aneurysm-can be a viable treatment strategy, but clinical evidence is lacking in this regard. Materials and Methods: Five neurovascular centers contributed to this retrospective analysis of patients who were treated with indirect flow diversion. Clinical data, aneurysm characteristics, anti-platelet medication, and follow-up results, including procedural and post-procedural complications, were recorded. Results: Seventeen patients (mean age: 60.5 years, range: 35-77 years) with 17 target aneurysms (vertebrobasilar: n = 9) were treated with indirect flow diversion. The average distance between the flow-diverting stent and the aneurysm was 1.65 mm (range: 0.4-2.4 mm). In 15/17 patients (88.2%), perfusion of the aneurysm was reduced immediately after implantation. Follow-ups were available for 12 cases. Delayed opacification (OKM A3: 11.8%), reduction in size (OKM B1-3: 29.4%) and occlusion (D1: 47.1%) were observable at the latest investigation. Clinically relevant procedural complications and adverse events in the early phase and in the late subacute phase were not observed in any case. Conclusion: Our preliminary data suggest that indirect flow diversion is a safe, feasible, and effective approach to off-centered bifurcation aneurysms and distant small-vessel aneurysms. However, validation with larger studies, including long-term outcomes and optimized imaging, is warranted.

Resolution of Brainstem Edema after Neurosurgical Occlusion of Dural Arteriovenous Fistulas of the Craniocervical Junction: Report of Three Cases and Review.

OBJECTIVE: Dural arteriovenous fistulas of the craniocervical junction are rare but the most dangerous of spinal fistulas. The concurrent brainstem edema has been attributed to increased pressure within the venous outflow of the brainstem and upper cord, but the differential diagnosis of brainstem edema is demanding. This report presents the details of clinical findings, magnetic resonance imaging (MRI) , surgical techniques, and follow-up of these fistulas. METHODS: Three cases with dural arteriovenous fistulas and edema of the brainstem and upper cord are described plus a review of the literature. Clinical presentation, radiologic findings, and a follow-up of outcomes are presented. RESULTS: All three cases with misleading diagnoses at the time of referral experienced variable degrees of tetraparesis with brainstem edema on MRI. Congested veins on MRI raised the suspicion of a vascular origin of the edema. The diagnosis was established with highly specific angiography with microcatheters identifying a feeder from the ascending pharyngeal artery in two cases and a tentorial feeder from the carotid artery in one case. The operation included temporary clipping of the fistula and neuromonitoring. Follow-up confirmed improvement of clinical signs and resolution of the brainstem edema on MRI. CONCLUSION: Edema of the brainstem and cervical cord may be caused by a dural arteriovenous fistula of the craniocervical junction. Surgical occlusion may be an efficient option for the complete resolution of radiologic and clinical symptoms.

Interactive exploration of a 3D intracranial aneurysm wall model extracted from histologic slices.

PURPOSE: Currently no detailed in vivo imaging of the intracranial vessel wall exists. Ex vivo histologic images can provide information about the intracranial aneurysm (IA) wall composition that is useful for the understanding of IA development and rupture risk. For a 3D analysis, the 2D histologic slices must be incorporated in a 3D model which can be used for a spatial evaluation of the IA's morphology, including analysis of the IA neck. METHODS: In 2D images of histologic slices, different wall layers were manually segmented and a 3D model was generated. The nuclei were automatically detected and classified as round or elongated, and a neural network-based wall type classification was performed. The information was combined in a software prototype visualization providing a unique view of the wall characteristics of an IA and allowing interactive exploration. Furthermore, the heterogeneity (as variance of the wall thickness) of the wall was evaluated. RESULT: A 3D model correctly representing the histologic data was reconstructed. The visualization integrating wall information was perceived as useful by a medical expert. The classification produces a plausible result. CONCLUSION: The usage of histologic images allows to create a 3D model with new information about the aneurysm wall. The model provides information about the wall thickness, its heterogeneity and, when performed on cadaveric samples, includes information about the transition between IA neck and sac.

Stent-assisted coiling of broad-necked intracranial aneurysms with a new braided microstent (Accero): procedural results and long-term follow-up.

Intracranial stents have expanded endovascular therapy options for intracranial aneurysms. The braided Accero stent is available for clinical use since May 2015. To date, no clinical reports on the stent are available. Purpose of this study was the evaluation of the safety and efficacy of the Accero stent in stent-assisted coiling. All patients, in whom implantation of the stent was performed, were included. Primary endpoints were good clinical outcome (mRS ≤ 2) and aneurysm occlusion grades 1 and 2 (Raymond Roy Occlusion Classification). Secondary endpoints were procedural and device-related complications with permanent disability or death, complications in the course, and the recanalization rate. Between September 2015 and August 2018, thirty-four aneurysms were treated with stent-assisted coiling using the Accero. Sixteen aneurysms were untreated, four of these were ruptured. Mild neurological complications occurred in 2/34 (5.9%) treatments. Two stent occlusions occurred during follow-up. No patient had a poor procedure- or device-related outcome. After an average of 15 months of follow-up, 28/30 aneurysms were completely or near-completely occluded. The Accero stent proved to be safe and effective in the treatment of broad-based intracranial aneurysms. The complication rate and the rate of successful aneurysm occlusions are similar to those of other stents.