Impact of Vaccination Status on Outcome of Patients With COVID-19 and Acute Ischemic Stroke Undergoing Mechanical Thrombectomy.

BACKGROUND: Data on impact of COVID-19 vaccination and outcomes of patients with COVID-19 and acute ischemic stroke undergoing mechanical thrombectomy are scarce. Addressing this subject, we report our multicenter experience. METHODS AND RESULTS: This was a retrospective analysis of patients with COVID-19 and known vaccination status treated with mechanical thrombectomy for acute ischemic stroke at 20 tertiary care centers between January 2020 and January 2023. Baseline demographics, angiographic outcome, and clinical outcome evaluated by the modified Rankin Scale score at discharge were noted. A multivariate analysis was conducted to test whether these variables were associated with an unfavorable outcome, defined as modified Rankin Scale score >3. A total of 137 patients with acute ischemic stroke (48 vaccinated and 89 unvaccinated) with acute or subsided COVID-19 infection who underwent mechanical thrombectomy attributable to vessel occlusion were included in the study. Angiographic outcomes between vaccinated and unvaccinated patients were similar (modified Thrombolysis in Cerebral Infarction ≥2b: 85.4% in vaccinated patients versus 86.5% in unvaccinated patients; P=0.859). The rate of functional independence (modified Rankin Scale score, ≤2) was 23.3% in the vaccinated group and 20.9% in the unvaccinated group (P=0.763). The mortality rate was 30% in both groups. In the multivariable analysis, vaccination status was not a significant predictor for an unfavorable outcome (P=0.957). However, acute COVID-19 infection remained significant (odds ratio, 1.197 [95% CI, 1.007-1.417]; P=0.041). CONCLUSIONS: Our study demonstrated no impact of COVID-19 vaccination on angiographic or clinical outcome of COVID-19-positive patients with acute ischemic stroke undergoing mechanical thrombectomy, whereas worsening attributable to COVID-19 was confirmed.

Initial Experience with a New Self-Expanding Open-Cell Stent System with Antithrombotic Hydrophilic Polymer Coating (pEGASUS Stent) in the Treatment of Wide-Necked Intracranial Aneurysms.

PURPOSE: We report our initial experience with endovascular embolization of intracranial aneurysms using this new self-expanding open-cell stent system (pEGASUS stent system) with the antithrombogenic hydrophilic polymer coating. MATERIALS AND METHODS: We retrospectively reviewed all patients treated with stent-assisted coiling or the Woven EndoBridge device using the pEGASUS stent system between September 2022 and June 2023. Demographic, clinical, and angiographic data were analyzed as well as short-term follow-up, including procedural complication rates and aneurysmal occlusion rates using the Raymond-Roy occlusion classification (RROC). RESULTS: Twelve patients with 12 wide-necked intracranial aneurysms were treated with the pEGASUS stent system, including 2 acutely ruptured aneurysms embolized in an emergency setting. The treated aneurysms were located at the anterior communicating artery (25.0%), the basilar artery (50.0%), the middle cerebral artery (16.7%), and the internal carotid artery (8.3%). All stents were deployed successfully. Immediate complete aneurysmal occlusion (RROC class I) was achieved in 83.3% (10/12) and near-complete occlusion (RROC II) in 16.7% (2/12). No periprocedural complications occurred in patients treated in the elective setting. A single case of intraoperative in-stent thrombus formation occurred during the treatment of an acutely ruptured basilar aneurysm and was resolved with intravenous Tirofiban. No other periprocedural complications occurred. Eleven out of 12 patients were available for follow up (mean 7.4 months). Complete aneurysmal occlusion without in-stent stenosis (ISS) was seen in 10 patients (90.9%). One patient (9.1%) showed aneurysmal reperfusion (RROC IIIb) with asymptomatic moderate ISS. CONCLUSION: Our initial results demonstrate that the pEGASUS stent system appears to be a safe and effective device for stent assisted embolization of wide-necked intracranial aneurysms. More data is necessary to evaluate long-term follow-up.

Embolization of an Intracranial Vertebral Artery Aneurysm via the Deep Cervical Artery.

Treatment of vertebral artery aneurysms can be challenging due to the unusual vascular anatomy or unfeasibility of traditional endovascular techniques. We describe a novel approach for endovascular treatment of a ruptured intracranial vertebral artery aneurysm with bilateral vertebral artery occlusions and hypoplasia of the posterior communicating arteries. Successful coil embolization was performed using a collateral pathway for microcatheterization via anastomosis between the deep cervical artery and the vertebral artery. This case report highlights a novel alternative endovascular treatment approach for vertebrobasilar aneurysms in case of a poor vascular status with occlusion or lack of traditional endovascular access routes.

Early clinical experience with the new generation Pipeline Vantage flow diverter in the treatment of unruptured saccular aneurysms using short-term dual antiplatelet therapy.

PURPOSE: The Pipeline Vantage flow diverter with Shield technology (PV) used in this study is a 4th-generation flow diverter (FD) designed to reduce thrombogenicity, promote endothelialization of the implant and increase efficiency in achieving aneurysm closure. In this study, we report the aneurysm occlusion rate, complication rate and clinical outcome with short-term dual antiplatelet therapy (DAPT) in the treatment of unruptured intracranial saccular aneurysms using the PV. METHODS: We retrospectively identified patients treated between September 2021 and January 2023 with the PV and subsequently underwent short-term DAPT for 3 months. Patient and aneurysm characteristics, peri- and post-procedural complications, clinical outcomes and the grade of aneurysm occlusion were documented. RESULTS: Thirty patients with 32 aneurysms were treated. Successful FD implantation was achieved in all cases (100%). No periprocedural complications were documented. The overall symptomatic complication rate was 10% and the neurologic, treatment-related symptomatic complication rate was 6.6%. Only one symptomatic complication (3.3%) was device-related. Permanent clinical deterioration occurred in 2/30 patients (6.6%), leading to deterioration of the mRS within the first 3 months after treatment. No mortality was documented. The rate of complete aneurysm occlusion after 3 months and after a mean imaging follow-up of 9.9 months was 65.6% and 75%, respectively. CONCLUSION: Implantation of the PV for the treatment of saccular intracranial aneurysms achieves a good aneurysm occlusion rate with a low rate of complications. In addition, the use of short-term DAPT after PV implantation appears to be safe.

Feasibility and safety of ADAPT in acute distal posterior cerebral artery occlusions.

PURPOSE: The direct aspiration first pass technique (ADAPT) is an effective and safe endovascular treatment for distal medium vessel occlusions (DMVO) of the anterior circulation. Clinical experience with ADAPT in the distal posterior circulation, however, is still limited and published data is scarce. In this original work, feasibility, safety and efficacy of ADAPT with distal access catheters (DAC) for treatment of acute distal posterior cerebral artery occlusions (DPCAOs) is evaluated. METHOD: All acute ischemic stroke patients between 2017 and 2022 with primary or secondary DPCAOs in the P2 or P3 segment, that underwent thrombectomy of the DPACO using ADAPT with DACs as frontline therapy, were identified. Demographic data, recanalization rates, procedural safety, and clinical outcome were assessed. RESULTS: Twenty-four patients with primary (n = 6) or secondary (n = 18) DPCAOs (P2: 21/24; P3: 3/24) were included. Median NIHSS score at admission was 14.5 (IQR 9.5). In all cases, the DPCAO could be reached with the DAC. Successful revascularization (DMVO-p-TICI ≥ 2b) with ADAPT was achieved in 79.2% (19/24), including a first pass effect of 62.5% (15/24), leading to complete recanalization (DMVO-p-TICI 3). Median number of passes was 1 (range 1-2). No complications related to distal PCA aspiration thrombectomy occurred. Median NIHSS and mRS scores at discharge were 4 (IQR 8) and 3 (IQR 2), respectively. CONCLUSIONS: ADAPT appears to be feasible, safe and effective for the treatment of acute DPCAOs in the setting of different occlusion patterns. High revascularization rates without procedural complications can be achieved. Further studies are needed to consolidate these results.

Long-term results of wide-necked intracranial bifurcation aneurysms treated with stent-assisted coiling using low-profile acandis acclino stents.

PURPOSE: Little data exists on endovascular treatment of complex intracranial aneurysms with the Acandis Acclino low-profile self-expanding closed-cell stent systems and is mainly limited to short- or midterm results. We report our long-term, single-centre experience with three generations of Acclino stents treating complex intracranial aneurysms. METHODS: 62 wide-necked intracranial aneurysms were treated electively using 88 Acclino stent systems. Single stent-assisted coiling was the preferred treatment in 38 cases and the kissing-Y stenting technique in 24 cases. We analysed demographic data and long-term follow-up results. RESULTS: All stents were successfully deployed with immediate complete (Raymond Roy occlusion classification, RROC I) or near-complete occlusion (RROC II) achieved in 93,5%. Follow-up was available in 55 cases with a mean follow-up of 36 months (range 9-80 months). Long-term RROC I or II was achieved in 49 cases (89,1%). Three cases of stable residual aneurysmal filling were observed (5,5%). Seven aneurysms (12,7%) demonstrated a worsening on follow-up leading either to a neck remnant (4 cases, 7,3%) or to an aneurysm recurrence (3 cases, 5,5%). One recurrent aneurysm was retreated with coilembolization (1,8%). The directly procedural-related complication rate was 4,8%. Seven cases of clinically silent in-stent stenosis (12,7%; morbidity n = 0) were detected on long-term follow-up, six of them using the kissing-Y stenting technique. CONCLUSION: Endovascular treatment of various intracranial aneurysms using the Acandis Acclino stent systems is safe and efficient with high aneurysm occlusion rates combined with low complication rates on long-term follow-up. Overall, rates of in-stent stenosis are low but may depend on the treatment technique (single stent-assisted coiling versus kissing-Y stenting with coiling).

Endovascular treatment of distal medium vessel occlusions using microcatheter aspiration thrombectomy.

BACKGROUND AND PURPOSE: Recent studies suggest that endovascular treatment (EVT) in distal medium vessel occlusion (DMVO) stroke is beneficial even beyond middle cerebral artery (MCA) - M2 segment. However, data about aspiration thrombectomy of DMVOs is scarce since common state-of-the-art aspiration catheters are usually too large for small distal intracranial arteries. We report our initial experiences using the microcatheter aspiration thrombectomy (MAT) technique as frontline therapy for acute DMVOs in the MCA territory. METHODS: We retrospectively analyzed all acute ischemic stroke (AIS) patients that underwent MAT of a primary or secondary DMVO in the M3 or M4 segment between January 2019 and October 2021. Recanalization rates, procedural safety and outcome data were recorded. RESULTS: MAT of acute M3 and M4 occlusions was performed in 19 patients with AIS. Six had isolated DMVO strokes, 13 had secondary DMVOs during EVT of a proximal large vessel occlusion. Successful revascularization to DMVO TICI ≥ 2b was achieved in 58% (11/19) with a single pass in all of them. The median National Institutes of Health Strokes Scale (NIHSS) score at admission and discharge was 12 and 3, respectively. 68% (13/19) of the patients had a good clinical outcome at discharge (modified Rankin Scale 0-2). No symptomatic complications related to MAT occurred. CONCLUSIONS: MAT of DMVOs in the MCA territory is technically feasible and effective. Compared to stent retriever-based thrombectomy in DMVOs the hemorrhagic complication rate appears notably lower. Further studies are needed to validate the benefit of mechanical thrombectomy in the distal intracranial vasculature.

Mechanical thrombectomy for acute ischemic stroke in COVID-19 patients: multicenter experience in 111 cases.

BACKGROUND: Data on the frequency and outcome of mechanical thrombectomy (MT) for large vessel occlusion (LVO) in patients with COVID-19 is limited. Addressing this subject, we report our multicenter experience. METHODS: A retrospective cohort study was performed of consecutive acute stroke patients with COVID-19 infection treated with MT at 26 tertiary care centers between January 2020 and November 2021. Baseline demographics, angiographic outcome and clinical outcome evaluated by the modified Rankin Scale (mRS) at discharge and 90 days were noted. RESULTS: We identified 111 out of 11 365 (1%) patients with acute or subsided COVID-19 infection who underwent MT due to LVO. Cardioembolic events were the most common etiology for LVO (38.7%). Median baseline National Institutes of Health Stroke Scale score and Alberta Stroke Program Early CT Score were 16 (IQR 11.5-20) and 9 (IQR 7-10), respectively. Successful reperfusion (mTICI ≥2b) was achieved in 97/111 (87.4%) patients and 46/111 (41.4%) patients were reperfused completely. The procedure-related complication rate was 12.6% (14/111). Functional independence was achieved in 20/108 (18.5%) patients at discharge and 14/66 (21.2%) at 90 days follow-up. The in-hospital mortality rate was 30.6% (33/108). In the subgroup analysis, patients with severe acute COVID-19 infection requiring intubation had a mortality rate twice as high as patients with mild or moderate acute COVID-19 infection. Acute respiratory failure requiring ventilation and time interval from symptom onset to groin puncture were independent predictors for an unfavorable outcome in a logistic regression analysis. CONCLUSION: Our study showed a poor clinical outcome and high mortality, especially in patients with severe acute COVID-19 infection undergoing MT due to LVO.

Mechanical thrombectomy of M2 occlusions with distal access catheters using ADAPT.

BACKGROUND AND PURPOSE: The direct aspiration first pass technique (ADAPT) using distal access catheters (DAC) has proven to be an effective and safe endovascular treatment strategy of acute ischemic stroke with large vessel occlusions (LVO). However, data about direct aspiration using DAC in M2 segment occlusions is limited. We assess the safety and efficacy of DACs in acute M2 occlusions using ADAPT with large bore (5 French /6 French) aspiration catheters as the primary method for endovascular recanalization. MATERIALS AND METHODS: From January 2017 to July 2018, 52 patients with an acute ischemic stroke due to M2 occlusions underwent mechanical thrombectomy using ADAPT with DACs (SOFIA 5 French/Catalyst 6) as frontline therapy. Patient demographics, technical parameters and outcome data were recorded. RESULTS: Median National Institutes of Health Strokes Scale (NIHSS) Score was 12 at admission. Successful revascularization to mTICI 2b-3 with ADAPT alone was achieved in 45 of 52 patients (86.5%) with mTICI 3 achieved in 32 patients (61.5%). Additional stent retrievers were used in 6 patients and led to an overall successful revascularisation of 92.3% (48/52). Median NIHSS at discharge was 4. 29 of 52 (55.8%) patients had a modified Rankin Scale (mRS) Score 0-2 at three months. Symptomatic intracranial hemorrhage did not occur. CONCLUSION: DACs can safely be used for mechanical thrombectomy of acute M2 occlusions by the ADAPT approach. Their use alone can be a high efficacious treatment of distal intracranial thromboembolic occlusions.

Craniofacial venous malformations treated by percutaneous sclerotherapy using polidocanol: a single-center experience.

BACKGROUND: Percutaneous therapy with various sclerosants is an established treatment of venous malformations in general. We investigated the safety and effectiveness of polidocanol in the craniofacial region. PURPOSE: To present and evaluate our subjective and objective mid- and long-term results of patients with craniofacial venous malformations (CFVM) after percutaneous sclerotherapy using polidocanol. MATERIAL AND METHODS: Twenty patients with CFVM treated by percutaneous sclerotherapy were followed up and asked to fill in a questionnaire comparing levels of the following CFVM-related symptoms before and after treatment: pain; functional impairment; cosmetic deformities; and impairment in daily life. Additionally, both size reduction as well as procedural-related complication rates were analyzed. RESULTS: Evaluation of the questionnaire revealed an improvement or complete relief of CFVM-related symptoms with significant reduced impairment in daily life after percutaneous sclerotherapy. Eighteen (90%) patients noticed a post-sclerotherapy improvement of at least one of their corresponding symptoms and expressed satisfaction with regard to their treatment. For 13 patients, a > 50% size reduction of the CFVM could be observed, while seven exhibited a < 50% size reduction. One minor complication was encountered in 56 treatment sessions (1.8%). CONCLUSION: Percutaneous sclerotherapy using polidocanol is a well-tolerated treatment for CFVM with a low complication rate. Size reduction and positive results with improvement of different clinical symptoms can be achieved.

Neuroimaging of Mitochondrial Cytopathies.

Mitochondrial diseases are a complex and heterogeneous group of genetic disorders that occur as a result of either nuclear DNA or mitochondrial DNA pathogenic variants, leading to a decrease in oxidative phosphorylation and cellular energy (ATP) production. Increasing knowledge about molecular, biochemical, and genetic abnormalities related to mitochondrial dysfunction has expanded the neuroimaging phenotypes of mitochondrial disorders. As a consequence of this growing field, the imaging recognition patterns of mitochondrial cytopathies are continually evolving. In this review, we describe the main neuroimaging characteristics of pediatric mitochondrial diseases, ranging from classical to more recent and challenging features. Due to the increased knowledge about the imaging findings of mitochondrial cytopathies, the pediatric neuroradiologist plays a crucial role in the diagnosis and evaluation of these patients.

Delayed and incomplete treatment may result in dural fistula development in children with Vein of Galen malformation.

The association of dural arteriovenous fistulas (DAVF) in children with Vein of Galen malformation (VGM) has recently been reported for the first time. In a larger series of cases treated with transarterial NBCA embolization, 30% had DAVF. We wanted to analyze the development of DAVF in our cohort of children with VGM and to evaluate whether their occurrence depends on different treatment timing and embolic materials. We analyzed 43 VGM cases treated with a combined transarterial and transvenous approach between 2003 and 2016. In our early series until 2011, we used coils solely in 21 children. Since 2012, 22 children were treated with the combination of coils and Onyx. In the early series treated with coils solely, no case presented initially with or developed DAVF over time on follow-up angiograms. In our recent series we found four cases (9%) with DAVF. In two patients (5%), DAVF were found on the initial angiogram. Both patients presented at our department at age >2 years and were not treated elsewhere before. One patient (2%) presented at our department with too proximal occlusion of arterial feeders performed at another institution before. Only one patient (2%) developed DAVF in our department after the transarterial use of Onyx. Interestingly, this child did not develop DAVF as long as we used coils solely and his DAVF was localized exactly where an Onyx cast was identified. In conclusion, delayed and incomplete treatment may have a considerable impact on the occurrence of DAVF in VGM.

Vascular angular remodeling by kissing-Y stenting in wide necked intracranial bifurcation aneurysms.

INTRODUCTION: Single stent deployment leads to a change in vascular geometry in wide necked bifurcation aneurysms. In some complex cases, the use of the single stent technique might not be sufficient or may not be feasible. The kissing-Y stenting technique appears to be an alternative endovascular treatment option. The aim of this study was to evaluate the effects of the kissing-Y stenting technique on vascular angular remodeling. METHODS: 21 patients with wide necked intracranial bifurcation aneurysms at different sites (10 anterior communicating artery, 6 middle cerebral artery, 3 basilar artery, 1 vertebral artery/posterior inferior cerebellar artery, 1 internal carotid artery/posterior communicating artery) were treated with 44 closed cell stents (follow-up 2012-2016) using the kissing-Y stenting technique. We analyzed vascular angle geometry between the mother and both affected daughter vessels by digital subtraction angiography, before and after stent deployment, using standard working projections. RESULTS: Endovascular treatment of wide necked intracranial aneurysms using the kissing-Y stenting technique significantly decreased the angle between the bifurcation branches from 130.4±9.5° to 91.5±9.1° (p<0.0001). CONCLUSIONS: Kissing-Y stenting in wide necked bifurcation aneurysms leads to vascular angular remodeling of both affected branches. The resulting straightening of the bifurcation angle may prevent aneurysmal recurrence.

Endovascular treatment of head and neck arteriovenous malformations: long-term angiographic and quality of life results.

AIM: To present the long-term angiographic and subjective results of patients with head and neck arteriovenous malformations (HNAVMs) after endovascular treatment. METHODS: We retrospectively analyzed the medical files of 14 patients with HNAVM who were treated between 2000 and 2014. The treatment of choice was a transarterial superselective microcatheter-based approach followed by embolization using liquid embolic agents. The patients were asked to answer a quality of life questionnaire about the following symptoms before and after treatment: pain, functional impairment, cosmetic deformity, impairment in daily life, and bleeding. RESULTS: Complete or >90% closure of the AVM was achieved in 6 of 14 patients (43%). >50% shunt reduction was achieved in 10 patients (71%). Three complications were encountered in a total of 86 interventional procedures. Six patients presented with bleeding which was cured in all cases (100%). Four of the 14 patients (29%) specified pain which was resolved in two of them. Another six patients (43%) presented with functional impairment; four were cured and two noted an improvement. All 14 patients presented with cosmetic concerns; four were cured and eight experienced a clearly visible improvement. Nine of 13 patients (69%) presented with impairment in daily life which was resolved in five patients and four reported an improvement. CONCLUSIONS: Endovascular embolization is a well-tolerated therapy for HNAVM with a low complication rate. Good angiographic results, positive subjective results, and improvement in different aspects of quality of life can be achieved.

Endovascular treatment of complex intracranial aneurysms using Acandis Acclino stents.

OBJECTIVE: To determine the safety and effectiveness of a new low-profile, laser-cut, closed-cell stent system in the treatment of complex intracranial aneurysms. METHODS: A total number of 43 patients with complex intracranial aneurysms were treated using 60 Acandis Acclino stent systems (follow-up 2012-2016; mean 11 months). 36 patients presented with wide-necked intracranial aneurysms, dissecting aneurysms were seen in 7 patients. 39 patients received stent-assisted coiling. We analyzed demographic data and follow-up results. RESULTS: Sixty stents were successfully deployed. In one paraophthalmic internal carotid artery aneurysm the stent could not be placed. Thirty-three wide necked aneurysms were treated by single or multiple stent-assisted coiling. Complete occlusion was achieved in 31 of those cases (94% Raymond-Roy occlusion classification, RROC 1). Two patients showed stable residual aneurysmal filling (RROC 3). In three wide-necked aneurysms, sole stenting was the preferred treatment. For dual stent-assisted procedures the kissing-Y stenting technique was successfully performed in 11 aneurysms. In all dissecting aneurysms constructive therapy with stenting and preservation of the affected parent artery was achieved. Additional subsequent coil embolization was intentionally planned and successfully performed in 6 of the 7 dissecting aneurysms. The overall directly procedure-related complication rate was 7%, including one death. CONCLUSIONS: Endovascular treatment of complex intracranial aneurysms using Acclino stents is a feasible and safe procedure with low complication rates. Even severe cases can be treated among others using the kissing-Y stenting technique, with good mid-term results.

Fistulous-type vein of Galen malformation phantom model for endovascular training and research.

INTRODUCTION: Vein of Galen malformation (VGM), a high-flow intracranial arteriovenous shunt, is among the most severe neurovascular diseases in childhood. In many cases untreated children die or survive only severely disabled. Endovascular embolization is the preferred treatment. OBJECTIVE: To develop a simple fistulous-type VGM phantom model for teaching and training of different endovascular treatment methods and to investigate new treatment options and devices. METHODS: An experimental in vitro pulsatile phantom model was developed imitating a high-flow fistulous-type VGM, which is typical, especially in the neonatal phase. Pressure measurements at different arterial sites were performed before and after closure of the VGM. Closure of the VGM was achieved by coiling using a combined microcatheter-based transvenous and transarterial approach called 'kissing microcatheter technique'. RESULTS: The behaviour of the phantom model in vitro under fluoroscopy and under angiographic runs was extremely similar to that in in vivo conditions in children. The results showed that intra-arterial pressures changed and increased statistically significantly at all measurement sites after embolization, as in human arteriovenous malformation. We also demonstrated different and complementary visualizations of hemodynamics and angioarchitecture by antegrade and retrograde microcatheter injections. CONCLUSIONS: Our phantom model behaves like a typical fistulous-type VGM and can be used in vitro for teaching and training and for further research. It offers a new and better understanding of hemodynamics and angioarchitecture in the endovascular management of VGM.

Hydrocephalus in vein of Galen malformation: etiologies and therapeutic management implications.

BACKGROUND: Up to now, only little is known about hydrocephalus (HC) in vein of Galen malformation (VGM). We want to present the different etiologies and our long-term experience (1992-2015) in the management of HC. METHODS: Out of 44 treated children with VGM, we retrospectively reviewed all cases with HC. We analyzed the etiologies, our treatment results and complications. RESULTS: Twenty-one children (48 %) presented either with HC or developed it over time. In 21 % of those cases, high venous pressure was presumably the sole cause. Until 2009, seven of them received ventriculoperitoneal (VP) shunting; six of those resulted in severe postoperative complications. The remaining children have been treated successfully by endovascular embolization. Five out of the 44 children (11 %) developed HC after intraventricular hemorrhage. In four cases, those children were treated with positive results by using transient external ventricular drainages. In one case a VP shunt with highest valve pressure was inserted. Another four children (9 %) presented with aqueductal stenosis-related HC caused by either dilated venous outflow or space-occupying coil masses after embolization. The latter case was successfully treated by ventriculocisternostomy, whereas endovascular treatment decreased the venous outflow in size and thus resolved the HC in the other cases. In the remaining cases (7 %), atrophy due to melting brain syndrome led to HC ex vacuo. CONCLUSIONS: HC in VGM is a common phenomenon with several etiologies requiring different treatments. In most cases, embolization of the VGM as sole treatment is completely sufficient in order to decrease high venous pressure. However, certain other causes of HC should be treated in an interdisciplinary setting by specialized neurosurgeons.

Caroli disease associated with vein of Galen malformation in a male child.

We report the first case of a male child with both Caroli disease and vein of Galen malformation. The neonate presented to our department with congestive heart failure as a result of the intracranial arteriovenous high-flow shunt. Over time, several endovascular embolizations led to a complete angiographic occlusion of the shunt. Additionally, the diagnosis of Caroli disease was made at the age of 2 months. He developed choledocholithiasis necessitating endoscopic sphincterotomy and stone extraction. As a prolonged medical treatment he received ursodeoxycholic acid and antibiotics. A coincidence of Caroli disease and vein of Galen malformation has not yet been described. Both diseases are very rare, leading to the question of whether there is a link in the pathogenesis. Based on the few previously described underlying mechanisms, we develop hypotheses about the relationship between both rare diseases. We consider overexpression of vascular endothelial growth factor and its receptors as a possible common molecular mechanism in their pathogenesis. We also highlight the critical role of increased expression of the Notch ligand Jagged 1 both in the development of cerebral arteriovenous malformations in general and in the formation of dilated intrahepatic bile ducts (eg, in Caroli disease).