Clinical outcomes of posterior fossa arteriovenous malformations: a single center experience.

BACKGROUND: Posterior fossa arterio-venous malformations (pfAVMs) are challenging lesions due to the anatomical particularities of the posterior fossa, and the high incidence of hemorrhagic presentation. The two most important goals when treating AVMs are preserving neurological function and preventing rupture, or a second hemorrhage. The aim of this study was to analyze the clinical and imaging features of pfAVMs to identify the factors that influence the prognosis of these patients. METHODS: We conducted a single-center retrospective observational study that included patients treated at our institution with pfAVMs between January 1997 and December 2021. RESULTS: A total of 48 patients were included. A good modified Rankin score (mRS) was observed in 33 cases (69%) at presentation. Thirty-four patients (71%) presented with a ruptured AVM. Out of these, 19 patients (40%) had intraventricular hemorrhage. Microsurgical resection was performed in 33 cases (69%), while in the other cases, the patients opted for conservative management (7 cases, 15%), stereotactic radiosurgery (SRS) (6 cases, 12%), or endovascular treatment (2 cases, 4%). Patients ≤ 30 years old were more prone to hemorrhagic presentation (OR: 5.23; 95% CI: 1.42-17.19; p = 0.024) and this remained an independent risk factor for rupture after multivariate analysis as well (OR: 4.81; 95% CI: 1.07-21.53; p = 0.040). Following multivariate analysis, the only factor independently associated with poor prognosis in the surgically treated subgroup was a poor clinical status (mRS 3-5) at admission (OR: 96.14; 95% CI: 5.15-1793.9; p = 0.002). CONCLUSIONS: Management of posterior fossa AVMs is challenging, and patients who present with ruptured AVMs often have a poor clinical status at admission leading to a poor prognosis. Therefore, proper and timely management of these patients is essential.

A comprehensive analysis of patients with cerebral arteriovenous malformation with headache: assessment of risk factors and treatment effectiveness.

BACKGROUND: Due to the high mortality and disability rate of intracranial hemorrhage, headache is not the main focus of research on cerebral arteriovenous malformation (AVM), so research on headaches in AVM is still scarce, and the clinical understanding is shallow. This study aims to delineate the risk factors associated with headaches in AVM and to compare the effectiveness of various intervention treatments versus conservative treatment in alleviating headache symptoms. METHODS: This study conducted a retrospective analysis of AVMs who were treated in our institution from August 2011 to December 2021. Multivariable logistic regression analysis was employed to assess the risk factors for headaches in AVMs with unruptured, non-epileptic. Additionally, the effectiveness of different intervention treatments compared to conservative management in alleviating headaches was evaluated through propensity score matching (PSM). RESULTS: A total of 946 patients were included in the analysis of risk factors for headaches. Multivariate logistic regression analysis identified that female (OR 1.532, 95% CI 1.173-2.001, p = 0.002), supply artery dilatation (OR 1.423, 95% CI 1.082-1.872, p = 0.012), and occipital lobe (OR 1.785, 95% CI 1.307-2.439, p < 0.001) as independent risk factors for the occurrence of headaches. There were 443 AVMs with headache symptoms. After propensity score matching, the microsurgery group (OR 7.27, 95% CI 2.82-18.7 p < 0.001), stereotactic radiosurgery group(OR 9.46, 95% CI 2.26-39.6, p = 0.002), and multimodality treatment group (OR 8.34 95% CI 2.87-24.3, p < 0.001) demonstrate significant headache relief compared to the conservative group. However, there was no significant difference between the embolization group (OR 2.24 95% CI 0.88-5.69, p = 0.091) and the conservative group. CONCLUSIONS: This study identified potential risk factors for headaches in AVMs and found that microsurgery, stereotactic radiosurgery, and multimodal therapy had significant benefits in headache relief compared to conservative treatment. These findings provide important guidance for clinicians when developing treatment options that can help improve overall treatment outcomes and quality of life for patients.

Repeat single-session stereotactic radiosurgery for arteriovenous malformation: a systematic review and meta-analysis.

BACKGROUND: Stereotactic radiosurgery is the preferred option for treating brain arteriovenous malformation (AVM) when the risks associated with surgery outweigh the potential benefits. However, some patients require repeat radiosurgery due to residual AVM after the first procedure. This systematic review and meta-analysis aimed to investigate the safety and efficacy of repeated procedure of radiosurgery for AVM. METHOD: A systematic review was conducted according to the PRISMA guideline. The search was conducted on PubMed, Scopus, Embase, and Web of Science, using a pre-designed search string. Studies investigating the efficacy of repeat radiosurgery for residual AVM following initial single session radiosurgery were included. The risk of bias was assessed using the JBI tool. Meta-analysis and met-regression were performed to pool and inspect data. RESULTS: Our meta-analysis, with a mean follow-up of 45.57 months, reveals repeat radiosurgery as a viable option for arteriovenous malformations (AVMs), achieving a 60.82% obliteration rate with a mean time to obliteration of 33.18 months. Meta-regression identifies AVM volume and Spetzler-Martin (SM) grade as factors influencing obliteration, with smaller volume and lower SM grades associated with higher rates. Complications include 10.33% radiation-induced changes, 5.26% post-radiosurgery hemorrhage, 2.56% neurologic deficits, and 0.67% cyst formation. Heterogeneity in complications is primarily attributed to male proportion and SM grade, while factors influencing post-radiosurgery hemorrhage remain unclear. The type of radiosurgery, whether Gamma Knife Radiosurgery (GKRS) or LINAC, does not significantly impact outcomes. CONCLUSION: Repeat radiosurgery is a feasible, effective, and safe treatment for AVMs following failure of initial radiosurgery. When utilized in appropriate patient subgroups, it provides an acceptable risk-to-benefit profile. Feature studies are required to clarify its clear indications.

Treatment of Brain Arteriovenous Malformations.

Brain arteriovenous malformations (AVMs) are a rare entity of vascular anomalies, characteristic of anatomical shunting where arterial blood directly flows into the venous circulation. The main aim of the active treatment policy of brain AVMs is the prevention of haemorrhage. There are well-established treatment strategies that continually improve in their safety and efficacy, primarily due to the advances in imaging modalities, targeted and novel techniques, the development of alternative treatment approaches, and even better experience with the disease itself. There are interesting imaging novelties that may be prospectively applicable in the decision-making and planning of the most effective treatment approach for individual patients with intracranial AVM. Surgery is often considered the first-line treatment; however, each patient should be evaluated individually, and the risks of the active treatment policy should not overcome the benefits of the spontaneous natural history of the disease. All treatment modalities, i.e., surgery, radiosurgery, endovascular embolization, and observation, are justified but need to be meticulously selected for each individual patient in order to deliver the best treatment outcome. This chapter deals with historical and currently applied dogmas, followed by introductions of advances in each available treatment modality of AVM management.

Location-based clinical and angiographic profile of brain arteriovenous malformations - a single-center observational study.

BACKGROUND: The location of brain arteriovenous malformations (bAVM) is one of the most relevant prognostic factors included in surgical, endovascular and radiosurgical scores. However, their characteristics according to location are seldom described. The goal of this study was to describe the clinical and angiographic characteristics of bAVM classified according to their location. METHODS: This retrospective observational study included patients diagnosed with bAVM and attending a national referral hospital during the period 2010-2020. Data regarding clinical and angiographic variables were extracted, including characteristics on nidus, arterial afferents, venous drainage and associated aneurysms. BAVM were classified in 8 groups according to their location: frontal, temporal, parieto-occipital, periventricular, deep, cerebellar, brainstem and mixed. Data distribution for each group was determined and between-group differences were assessed. RESULTS: A total of 269 bAVM (in 258 patients) were included. The most frequent location was parieto-occipital; and the least frequent, brainstem. Statistically significant differences were observed between groups for most studied variables, including: clinical presentation, functional status at admission; nidus size and density, classification according to the Spetzler-Martin, Buffalo and modified Pollock-Flickinger scales; number, diameter, origin and type of afferents; number, diameter, type and direction of venous drainage, retrograde venous flow; and presence and size of flow-related aneurysms. CONCLUSION: The clinical and angiographic differences observed between brain AVM groups allow the formulation of profiles according to their location.

The role of surgical disconnection for posterior fossa pial arteriovenous fistulas and dural fistulas with pial supply: an illustrative case series.

BACKGROUND: Pial arteriovenous fistulas (pAVFs) are rare vascular malformations characterized by high-flow arteriovenous shunting involving a cortical arterial supply directly connecting to venous drainage without an intermediate nidus. Dural arteriovenous fistulas (dAVFs) can infrequently involve additional pial feeders which can introduce higher flow shunting and increase the associated treatment risk. In the posterior fossa, arteriovenous fistula (AVF) angioarchitecture tends to be particularly complex, involving either multiple arterial feeders-sometimes from both dural and pial origins-or small caliber vessels that are difficult to catheterize and tend to be intimately involved with functionally critical brainstem or upper cervical cord structures. Given their rarity, published experience on microsurgical or endovascular treatment strategies for posterior fossa pAVFs and dAVFs with pial supply remains limited. METHODS: Retrospective chart review from 2019-2023 at a high-volume center identified six adult patients with posterior fossa pAVFs that were unable to be fully treated endovascularly and required microsurgical disconnection. These cases are individually presented with a technical emphasis and supported by comprehensive angiographic and intraoperative images. RESULTS: One vermian (Case 1), three cerebellopontine angle (Cases 2-4) and two craniovertebral junction (Cases 5-6) posterior fossa pAVFs or dAVFs with pial supply are presented. Three cases involved mixed dural and pial arterial supply (Cases 1, 4, and 6), and one case involved a concomitant microAVM (Case 2). Endovascular embolization was attempted in four cases (Cases 1-4): The small caliber and tortuosity of the main arterial feeder prevented catheterization in two cases (Cases 1 and 3). Partial embolization was achieved in Cases 2 and 4. In Cases 5 and 6, involvement of the lateral spinal artery or anterior spinal artery created a prohibitive risk for endovascular embolization, and surgical clip ligation was pursued as primary management. In all cases, microsurgical disconnection resulted in complete fistula obliteration without evidence of recurrence on follow-up imaging (mean follow-up 27.1 months). Two patients experienced persistent post-treatment sensory deficits without significant functional limitation. CONCLUSIONS: This illustrative case series highlights the technical difficulties and anatomical limitations of endovascular management for posterior fossa pAVFs and dAVFs with pial supply and emphasizes the relative safety and utility of microsurgical disconnection in this context. A combined approach involving partial preoperative embolization-when the angioarchitecture is permissive-can potentially decrease surgical morbidity. Larger studies are warranted to better define the role for multimodal intervention and to assess associated long-term AVF obliteration rates in the setting of pial arterial involvement.

Most Promising Approaches to Improve Brain AVM Management: ARISE I Consensus Recommendations.

Brain arteriovenous malformations (bAVMs) are complex, and rare arteriovenous shunts that present with a wide range of signs and symptoms, with intracerebral hemorrhage being the most severe. Despite prior societal position statements, there is no consensus on the management of these lesions. ARISE (Aneurysm/bAVM/cSDH Roundtable Discussion With Industry and Stroke Experts) was convened to discuss evidence-based approaches and enhance our understanding of these complex lesions. ARISE identified the need to develop scales to predict the risk of rupture of bAVMs, and the use of common data elements to perform prospective registries and clinical studies. Additionally, the group underscored the need for comprehensive patient management with specialized centers with expertise in cranial and spinal microsurgery, neurological endovascular surgery, and stereotactic radiosurgery. The collection of prospective multicenter data and gross specimens was deemed essential for improving bAVM characterization, genetic evaluation, and phenotyping. Finally, bAVMs should be managed within a multidisciplinary framework, with clinical studies and research conducted collaboratively across multiple centers, harnessing the collective expertise and centralization of resources.

Enhancing the quality of evidence, comparability, and reproducibility in brain arteriovenous malformations treated with open surgery research: a systematic review and proposal of a reporting guideline for surgical and clinical outcomes.

Brain Arteriovenous Malformations (bAVMs) are rare but high-risk developmental anomalies of the vascular system. Microsurgery through craniotomy is believed to be the mainstay standard treatment for many grades of bAVMs. However, a significant challenge emerges in the existing body of clinical studies on open surgery for bAVMs: the lack of reproducibility and comparability. This study aims to assess the quality of studies reporting clinical and surgical outcomes for bAVMs treated by open surgery and develop a reporting guideline checklist focusing on essential elements to ensure comparability and reproducibility. This is a systematic literature review that followed the PRISMA guidelines with the search in Medline, Embase, and Web of Science databases, for studies published between January 1, 2018, and December 1, 2023. Included studies were scrutinized focusing on seven domains: (1) Assessment of How Studies Reported on the Baseline Characteristics of the Patient Sample; (2) Assessment and reporting on bAVMs grading, anatomical characteristics, and radiological aspects; (3) Angioarchitecture Assessment and Reporting; (4) Reporting on Pivotal Concepts Definitions; (5) Reporting on Neurosurgeon(s) and Staff Characteristics; (6) Reporting on Surgical Details; (7) Assessing and Reporting Clinical and Surgical Outcomes and AEs. A total of 47 studies comprising 5,884 patients were included. The scrutiny of the studies identified that the current literature in bAVM open surgery is deficient in many aspects, ranging from fundamental pieces of information of methodology to baseline characteristics of included patients and data reporting. Included studies demonstrated a lack of reproducibility that hinders building cumulative evidence. A bAVM Open Surgery Reporting Guideline with 65 items distributed across eight domains was developed and is proposed in this study aiming to address these shortcomings. This systematic review identified that the available literature regarding microsurgery for bAVM treatment, particularly in studies reporting clinical and surgical outcomes, lacks rigorous scientific methodology and quality in reporting. The proposed bAVM Open Surgery Reporting Guideline covers all essential aspects and is a potential solution to address these shortcomings and increase transparency, comparability, and reproducibility in this scenario. This proposal aims to advance the level of evidence and enhance knowledge regarding the Open Surgery treatment for bAVMs.

Brain Arteriovenous Malformation In Vitro Model for Transvenous Embolization Using 3D Printing and Real Patient Data.

BACKGROUND AND PURPOSE: Transvenous embolization has emerged as a novel technique for treating selected brain AVMs with high reported occlusion rates. However, it requires anatomic and technical skills to be successful and to ensure patient safety. Therefore, training and testing are essential for preparing clinicians to perform these procedures. Our aim was to develop and test a novel, patient-specific brain AVM in vitro model for transvenous embolization by using 3D printing technology. MATERIALS AND METHODS: We developed a brain AVM in vitro model based on real patient data by using stereolithography resin 3D printing. We created a closed pulsed circuit with flow passing from the arterial side to the venous side, and we tested the effect of mean arterial pressure on retrograde nidal filling with contrast injections. Transvenous embolization simulations were conducted for each of the 12 identical models divided into 2 groups (2×6). This involved the use of an ethylene-vinyl alcohol liquid embolic agent injected through microcatheters either without or with a coil in the vein (groups 1 and 2, respectively). RESULTS: Retrograde contrast advance to nidus was directly related to lower mean arterial pressure. Transvenous embolization tests with a liquid embolic agent adequately reproduced the usual embolization plug and push technique. We found no differences between the 2 group conditions, and additional venous coil neither increased nidus penetration nor reduced injection time in the model (57.6 versus 61.2% nidus occlusion rate, respectively). CONCLUSIONS: We were able to develop and test a functional in vitro brain AVM model for transvenous embolization by using 3D printing to emulate its conditions and characteristics. Better contrast penetration was achieved with less mean arterial pressure, and no embolization advantage was found by adding coil to the vein in this model.

Management of sporadic intracranial vascular malformations in pregnancy: a retrospective analysis.

BACKGROUND: Sporadic intracranial vascular malformations can pose significant risk to parturients, and additional reports of management may inform patient care. Here we describe the peripartum management of parturients with intracranial vascular malformations. METHODS: After Institutional Review Board approval, we performed a retrospective analysis of parturients with a known sporadic intracranial vascular malformation including cavernous malformation, developmental venous anomaly, or arteriovenous malformation who delivered at our institution between 2007 and 2020. RESULTS: We identified 10 parturients (five cavernous malformations, three developmental venous anomalies, and two arteriovenous malformations) with 16 deliveries. Among all deliveries, 13 (81.3%) were cesarean deliveries without trial of labor; 11 of these (84.6%) received a single-shot spinal and two (15.4%) received an epidural for surgical anesthesia. Two deliveries (12.5%) began with attempted trial of labor but ultimately required cesarean delivery for failure to progress; one of these cases received epidural anesthesia and the other received combined spinal-epidural anesthesia. One delivery was via spontaneous vaginal delivery with epidural analgesia. Overall, our study's cesarean delivery rate was 93.8% and spontaneous vaginal delivery rate was 6.2%. Three of 16 pregnancies were complicated by seizure, obstructive hydrocephalus, or intracranial hemorrhage. There were no intensive care unit admissions or maternal deaths. CONCLUSIONS: In our case series of 16 deliveries, there were no complications directly resulting from neuraxial procedures. It remains unclear whether intracranial developmental venous anomalies or unruptured arteriovenous malformations impart increased risk during pregnancy. Antepartum planning with a multidisciplinary team approach enables risk stratification and optimal management.

[Expert consensus on multidisciplinary diagnosis and treatment of brain arteriovenous malformation].

Brain arteriovenous malformation (BAVM) is a cerebral vasculature disorder caused by gene mutation. Current available treatment measures include surgical resection, interventional embolization and stereotactic radiosurgery. The three therapeutic methods have their own advantages for different vascular structures.However, due to the complex vascular architecture of the lesion and its close anatomical relationship with brain tissue, any single treatment can not safely and effectively treat all BAVM cases. Therefore, in order to better regulate and guide the clinical diagnosis and treatment of BAVM patients in China, the National Medical School for Neurological Diseases, the Professional Committee of Neurointervention of the Chinese Medical Doctor Association and the radio-neurosurgery Expert Committee of the World Chinese Neurosurgeons Association jointly discussed and formulated this expert consensus. After in-depth analysis of the evidence of evidence-based medicine at home and abroad, the expert group combined with the specific situation of China, and gave 33 recommendations on specific clinical diagnosis and treatment issues such as predictive factors of cerebral arteriovenous malformation hemorrhage, clinical risks during pregnancy, imaging diagnosis measures, and clinical treatment strategies, in order to provide guidance for the diagnosis and treatment of BAVM nationwide.

Stereotactic radiosurgery with versus without embolization for intracranial dural arteriovenous fistulas: a systematic review and meta-analysis.

OBJECTIVE: Stereotactic radiosurgery (SRS) has been established as a safe and alternative treatment for dural arteriovenous fistulas (dAVFs). While embolization alone is the most commonly used modality for the treatment of dAVFs, the adjunctive use of embolization with SRS, with the growing use of SRS, has gained increasing interest in the past few years. However, the relative efficacy and safety of SRS combined with embolization versus SRS alone for dAVFs remains uncertain. Hence, this systematic review aimed to evaluate the efficacy of SRS with adjunctive embolization for intracranial dAVFs. METHODS: A systematic review and meta-analysis was conducted by searching electronic databases, including PubMed, Embase, and the Cochrane Library, up to August 2023. All studies evaluating the utilization of adjunctive embolization and SRS for dAVFs were included. Risk of bias was assessed using the Newcastle-Ottawa Scale. A meta-analysis was conducted on the suitable outcomes. RESULTS: Eighteen studies involving 715 patients were included. The mean age of the participants in the study was 64.30 years in the adjunctive embolization group and 60.51 years in the SRS-alone group. In the adjunctive embolization group 41.3% of patients were female, compared with 47.1% in the SRS-only group. The dAVF obliteration rates were 64.7% and 65.7% in the adjunctive embolization and SRS-alone groups, respectively. These obliteration rates were comparable between the two groups (p = 0.96), as were the symptom improvement rates (p = 0.35). Adverse events were rare, and were more commonly associated with the adjunctive embolization procedure, although further causal analysis was not possible. CONCLUSIONS: This study provides evidence that adjunctive embolization plus SRS provides similar obliteration and symptom improvement rates compared with SRS alone, with both having very limited SRS-related adverse events. Considering the added burden and adverse events of additional endovascular treatment, the authors recommend embolization be reserved for more complex dAVFs or when embolization can potentially be curative alone or provide more rapid symptomatic relief or protection during the radiosurgical latency period.

[Presurgical Simulation for Brain Arteriovenous Malformation].

Surgical extirpation of brain arteriovenous malformations(AVMs)requires precise pre-surgical simulation. Utilizing image software, widely used with picture archiving and communication systems(PACS), surgeons can generate simulation images that precisely illustrate the proper feeders, passing arteries, and drainers. The crucial steps for creating informative simulation images include: (1)the free rotation of reconstructed 3D digital subtraction angiography(DSA)images; (2)removal of irrelevant arteries(the most important procedure); and(3)construction of stereo imagery of the "core images." This article presents a detailed description of these procedures.

Fractal-Based Analysis of Arteriovenous Malformations (AVMs).

Arteriovenous malformations (AVMs) are cerebrovascular lesions consisting of a pathologic tangle of the vessels characterized by a core termed the nidus, which is the "nest" where the fistulous connections occur. AVMs can cause headache, stroke, and/or seizures. Their treatment can be challenging requiring surgery, endovascular embolization, and/or radiosurgery as well. AVMs' morphology varies greatly among patients, and there is still a lack of standardization of angioarchitectural parameters, which can be used as morphometric parameters as well as potential clinical biomarkers (e.g., related to prognosis).In search of new diagnostic and prognostic neuroimaging biomarkers of AVMs, computational fractal-based models have been proposed for describing and quantifying the angioarchitecture of the nidus. In fact, the fractal dimension (FD) can be used to quantify AVMs' branching pattern. Higher FD values are related to AVMs characterized by an increased number and tortuosity of the intranidal vessels or to an increasing angioarchitectural complexity as a whole. Moreover, FD has been investigated in relation to the outcome after Gamma Knife radiosurgery, and an inverse relationship between FD and AVM obliteration was found.Taken altogether, FD is able to quantify in a single and objective value what neuroradiologists describe in qualitative and/or semiquantitative way, thus confirming FD as a reliable morphometric neuroimaging biomarker of AVMs and as a potential surrogate imaging biomarker. Moreover, computational fractal-based techniques are under investigation for the automatic segmentation and extraction of the edges of the nidus in neuroimaging, which can be relevant for surgery and/or radiosurgery planning.

Virtual Planning and Augmented Reality-Guided Microsurgical Resection of a Frontal Arteriovenous Malformation.

Arteriovenous malformations (AVMs) are complex vascular lesions that can pose significant risk for spontaneous hemorrhage, seizures, and symptoms related to ischemia and venous hypertension.1 Microsurgical management of AVMs requires a deep understanding of the surrounding anatomy and precise identification of the lesion characteristics. We demonstrate the use of augmented reality in the localization of arterial feeders and draining veins in relation to bordering normal structures (Video 1). A 66-year-old man presented with several episodes of severe right frontal headaches. Magnetic resonance imaging revealed an AVM along the right frontal pole. Subsequent computed tomography angiography demonstrated arterial supply from the right anterior cerebral artery with venous drainage to the superior sagittal sinus. Due to the size, noneloquent location, and superficial pattern of venous drainage, the patient elected to proceed with microsurgery. A virtual planning platform was used in preparation for surgery. Augmented reality integrated with neuronavigation was used during microsurgical resection. Postoperative angiography showed complete resection of the AVM. The patient was discharged home on postoperative day 3 with no complications. He remains neurologically well at 4 months of follow-up.

Risk of intracranial hemorrhage in brain arteriovenous malformations: a systematic review and meta-analysis.

BACKGROUND AND OBJECTIVE: Brain arteriovenous malformations (bAVMs) carry a risk of hemorrhage. We aim to identify factors associated with subsequent hemorrhages. METHODS: Systematic searches were conducted across the ScienceDirect, Medline, and Cochrane databases. Assessed risk factors included bAVM size, bAVM volume, hemorrhage and seizure presentations, presence of deep venous drainage, deep-seated bAVMs, associated aneurysms, and Spetzler-Martin grade. Subgroup analyses were conducted on prior treatments, hemorrhage presentation, AVM size, and type of management. RESULTS: The meta-analysis included 8 cohort studies and 2 trials, with 4,240 participants. Initial hemorrhage presentation (HR 2.41; 95% CI 1.94-2.98; p < 0.001), any deep venous drainage (HR 1.52; 95% CI 1.09-2.13; p = 0.01), and associated aneurysms (HR 1.78; 95% CI 1.41-2.23; p < 0.001) increased secondary hemorrhage risk. Conversely, higher Spetzler-Martin grades (HR 0.77; 95% CI 0.68-0.87; p < 0.001) and larger malformation volumes (HR 0.87; 95% CI 0.76-0.99; p = 0.04) reduced risk. Subgroups showed any deep venous drainage in patients without prior treatment (HR 1.64; 95% CI 1.25-2.15; p < 0.001), bAVM > 3 cm (HR 1.79; 95% CI 1.15-2.78; p = 0.01), and multimodal interventions (HR 1.69; 95% CI 1.12-2.53; p = 0.01) increased risk. The reverse effect was found for patients initially presented without hemorrhage (HR 0.79; 95% CI 0.67-0.93; p = 0.01). Deep bAVM was a risk factor in > 3 cm cases (HR 2.72; 95% CI 1.61-4.59; p < 0.001) and multimodal management (HR 2.77; 95% CI 1.66-4.56; p < 0.001). Kaplan-Meier analysis revealed increased hemorrhage risk for initial hemorrhage presentation, while cumulative survival was higher in intervened patients over 72 months. CONCLUSION: Significant risk factors for bAVMs hemorrhage include initial hemorrhage, any deep venous drainage, and associated aneurysms. Deep venous drainage involvement is a risk factor in cases without prior treatment, those with bAVM > 3 cm, and cases managed with multimodal interventions. Deep bAVM involvement also emerges as a risk factor in cases > 3 cm and those managed with multimodal approaches.

Hemangioblastoma and arteriovenous malformation in the same patient: a not random association or two isolated entities? Systematic review starting from a unique case.

BACKGROUND: The association between intracranial hemangioblastomas and arteriovenous malformations has been documented in very few cases in literature since 1965 and might present in three modalities: "intermixed, adjacent and separated (spatially and temporally)". Often, the pattern of presentation is "intermixed". According to our systematic review, we propose an adjustment of the previous classification, specifically for these entities. We describe the first case of a truly "spatially separated" association between these two lesions. METHODS: Our study encompassed all adult patients diagnosed with both intracranial hemangioblastoma and AVM who were evaluated in the last 20-year period, from 2003 to 2023 at Helsinki University Hospital. Cases of this coexistence were retrospectively identified and collected from clinical records. For the systematic review, studies reporting the coexistence of hemangioblastoma and AVM in adult patients (>18 years old) were selected. Given the rarity of this pattern, case reports were also included. RESULTS: The combined analysis of our systematic review and institutional retrospective study revealed a total of only seven identified cases. We applied the classification of neoplasms and AVM by Yano, modifying and adapting it into our screened patient series. We systematically reclassified "adjacent" and genuinely "spatially separated" patterns based on the vascular axis supplying both lesions. CONCLUSIONS: Hemangioblastomas and AVMs rarely coexist in the same patient. Our study reports the first instance of a truly "spatially separated" sporadic association between these vascular lesions. The rarity of such coexistence underscores the need for a nuanced and systematic classification to guide the management of these infrequent cases.

Coexistence of moyamoya syndrome with arteriovenous malformation. Systematic review and illustrative case report.

The coexistence of Moyamoya Syndrome with Arteriovenous Malformation is exceedingly rare. Here, we present the case of a 37-year-old female patient diagnosed with AVM in the right parietal lobe, accompanied by severe stenosis of the right middle cerebral artery and right anterior cerebral artery, along with moyamoya collateral induction. Our objective was to investigate the frequency and mutual influence of these conditions, and to determine a preferable treatment strategy by conducting a comprehensive review of previous case reports. We conducted a thorough search of PubMed, Scopus, and Web of Science databases, adhering to Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Our review encompassed 36 publications, reporting a total of 64 cases of AVM coexisting with Moyamoya Syndrome. Notably, bilateral stenosis was observed in half of the cases. Among patients with unilateral stenosis, AVM was localized in the opposite hemisphere only in 2 cases. Treatment approaches varied, with 18 cases undergoing active treatment for both stenosis and AVM, 11 cases treating stenosis prior to AVM, 5 cases addressing AVM first, and 3 cases treating both AVM and stenosis simultaneously. Embolization, either standalone or supplemented by stereotactic radiosurgery, was employed in six cases. Stereotactic radiosurgery alone was utilized in 12 cases, while 15 patients underwent surgical removal of the AVM. Our findings provide valuable insights for neurosurgeons managing patients with concurrent AVM and Moyamoya Syndrome. The variety of treatment approaches observed in the literature underscores the complexity of these cases, emphasizing the need for individualized strategies. This information may guide future systematic reviews and meta-analyses, contributing to a better understanding of the optimal management of these rare coexisting vascular pathologies.