Late meningitis and a nonabsorbable stent in recurrent Rathke's cleft cyst: illustrative case.

BACKGROUND: The management of recurrent Rathke's cleft cysts (RCCs) remains challenging. The off-label application of steroid-eluting bioabsorbable or nonabsorbable intracystic stents has been recently described. Early outcomes and complications of this treatment have been described as well, but long-term data are lacking. OBSERVATIONS: A 31-year-old woman who had undergone 3 prior transsphenoidal surgeries for recurrent RCC at other institutions was admitted for methicillin-susceptible Staphylococcus aureus meningitis. Five years earlier, a nonabsorbable stent had been placed in the cyst using a microsurgical transsphenoidal approach. RCC recurrence had been radiologically evident after 2 years but was clinically asymptomatic. Acute cerebrospinal fluid leakage and other causes of meningitis were ruled out; the stent was occluded. After antibiotic therapy and resolution of the meningitis, the patient underwent endoscopic removal of the catheter, excision of the cyst, and reconstruction with a nasoseptal flap. At the 18-month follow-up, she remained neurologically intact, under replacement therapy, and with no signs of recurrence. LESSONS: The authors report the first case of late meningitis due to a nonabsorbable stent positioned for the treatment of a recurrent RCC. Currently, data on the long-term outcomes, failure rates, and complications of stents for RCC are limited. https://thejns.org/doi/10.3171/CASE2477.

The oculomotor cistern and pituitary adenomas: anatomical and clinical study.

OBJECTIVE: The oculomotor cistern (OMC) is a meningeal cuff filled with CSF that contains the oculomotor nerve (cranial nerve [CN] III) at the level of the lateral wall of the cavernous sinus. Only a few studies have investigated the involvement of the OMC by pituitary adenomas (pituitary neuroendocrine tumors [PitNETs]), mainly with relatively small case series. The aim of this study was to perform a histomorphological description of the OMC and systematically analyze its involvement by PitNETs from radiological, clinical, and surgical perspectives. METHODS: Ten hemisellae from formalin-fixed specimens were studied with 3-µm sections. Digital image analysis software was used for morphological and quantitative assessments. Clinical, radiological, surgical, and histological data of patients undergoing endoscopic transsphenoidal surgery for PitNETs at the University of Brescia, Italy, between 2014 and 2021 were recorded. OMC involvement was graded as not compressed, compressed, and invaded. The same surgical team operated on all patients. RESULTS: The OMC had an elliptical shape with an average area of 3.1 mm2 and a length of 5.5 mm. No cisternal points of weakness were recognized in the histomorphological study. Of 315 patients, 246 had complete data: apoplexy and CN III palsy were documented in 6.9% and 8.5%, respectively. OMC compression and invasion were recorded in 106 (43.1%) and 23 (9.3%) patients. Significant associations between OMC involvement and PitNET dimensions (p < 0.001), Knosp grade (p < 0.001), preoperative oculomotor palsy (p < 0.001), Ki-67 percentage (p = 0.009), and recurrence/progression of residual tumor (p = 0.008) were found. A new postoperative CN III palsy was evident in 2%: transient in 4 cases, and persistent in 1 patient treated for a recurrent PitNET who experienced a local infection complication. Preoperative CN III palsy improved in 10 cases. CONCLUSIONS: Significant OMC involvement by PitNETs might be underrecognized, but it can be treated using the endoscopic transsphenoidal approach, and it affects patient outcomes.

Neuroanatomical photogrammetric models using smartphones: a comparison of apps.

OBJECTIVES: A deep knowledge of the surgical anatomy of the target area is mandatory for a successful operative procedure. For this purpose, over the years, many teaching and learning methods have been described, from the most ancient cadaveric dissection to the most recent virtual reality, each with their respective pros and cons. Photogrammetry, an emergent technique, allows for the creation of three-dimensional (3D) models and reconstructions. Thanks to the spreading of photogrammetry nowadays it is possible to generate these models using professional software or even smartphone apps. This study aims to compare the neuroanatomical photogrammetric models generated by the two most utilized smartphone applications in this domain, Metascan and 3D-Scanner, through quantitative analysis. METHODS: Two human head specimens (four sides) were examined. Anatomical dissection was segmented into five stages to systematically expose well-defined structures. After each stage, a photogrammetric model was generated using two prominent smartphone applications. These models were then subjected to both quantitative and qualitative analysis, with a specific focus on comparing the mesh density as a measure of model resolution and accuracy. Appropriate consent was obtained for the publication of the cadaver's image. RESULTS: The quantitative analysis revealed that the models generated by Metascan app consistently demonstrated superior mesh density compared to those from 3D-Scanner, indicating a higher level of detail and potential for precise anatomical representation. CONCLUSION: Enabling depth perception, capturing high-quality images, offering flexibility in viewpoints: photogrammetry provides researchers with unprecedented opportunities to explore and understand the intricate and magnificent structure of the brain. However, it is of paramount importance to develop and apply rigorous quality control systems to ensure data integrity and reliability of findings in neurological research. This study has demonstrated the superiority of Metascan in processing photogrammetric models for neuroanatomical studies.

Dissecting and fusiform aneurysms of the superior cerebellar artery: anatomy, clinical presentation, and treatment outcomes.

The management of superior cerebellar aneurysms is still controversial. Although several techniques are available, the deep localization of the lesion and the limited number of cases increase the complexity of decision-making for optimal treatment. Only a few cases of dissecting and fusiform aneurysms of the superior cerebellar artery (SCA) are described in the literature, many of which are without long follow-ups. The study aims to offer an exhaustive retrospective analysis of patients affected by SCA fusiform and dissecting aneurysms treated at our institution from 2008 to 2023, highlighting outcomes and complications. Moreover, a comprehensive narrative review was performed. A total of seven patients were treated at our institution. After a title and abstract screen, fifty-five papers met the criteria for inclusion in the review. In our case series, conservative treatment was proposed as the first therapeutic option in four cases (57.1%), while endovascular treatment (EVT) was in three cases (42.9%). A good recovery was observed in 66% of patients presenting with subarachnoid hemorrhage (SAH), while every patient without SAH achieved a good clinical outcome. A poor outcome was observed only in one patient with a dissecting aneurysm causing SAH, who also suffered a pontine infarction. In the literature review, conservative treatment was proposed as a first therapeutic option in eleven cases (16.6%), open microsurgical techniques in 19 patients (28.8%), and EVT in 31 patients (46.9%). Fatal outcome was documented in five patients (7.5%), all characterized by the rupture of the vascular lesion, while 6.1% of cases had non-fatal poor outcomes.

Endoscopic Extended Transsphenoidal Surgery for Transbasal Tuberculum Sellae Meningioma: 2-Dimensional Operative Video.

Transbasal meningiomas1 are a rare group of tumors that grow intracranially, invade the skull base, and propagate extracranially; they are usually located at the level of the olfactory cribra and sphenoid wing.1-4 Transbasal tuberculum sellae meningiomas are a subset of these tumors, which extend into the suprasellar and sphenoid regions. The traditional transcranial approach for some transbasal meningiomas has recently evolved toward the transnasal endoscopic approach, which allows the removal of both the intranasal and intracranial components, reducing the manipulation of the optic nerves and neurovascular structures. Nevertheless, specific anatomical criteria must be present; the knowledge of specific anatomy5,6 and the surgeon's experience are fundamental for reaching the most favorable outcome.7-11 In this video, we present the case of a 50-year-old woman with a history of progressive visual impairment and scotoma in the left inferotemporal quadrant. Radiological examinations suggested a transbasal tuberculum meningioma that extended into the suprasellar region, sphenoid sinus, and optic canals. An endoscopic transtuberculum approach with intraoperative visual-evoked potentials12 was performed, allowing an early devascularization with the preservation of the superior hypophyseal arteries and the removal of the component extending in the medial portion of both optic canals. The postoperative MRI documented a gross total resection without complications. The patient reported an immediate improvement of the visual symptoms. Histopathology documented a meningioma (CNS World Health Organization grade 1) with bone invasion. The patient consented to the procedure and to the publication of her image; Institutional Review Board approval was not required for this single case report.

3-Dimensional Printed Model of the Temporal Bone for Neurosurgical Training.

BACKGROUND AND OBJECTIVES: The development of neurosurgical skills stands out as a paramount objective for neurosurgery residents during their formative years. Mastery of intricate and complex procedures is a time-intensive process marked by a gradually ascending learning curve. Consequently, the study and simulation on surgical models assume significant importance. One of the most intricate neuroanatomical regions includes the petrous and mastoid portions of the temporal bone. These regions host critical, highly functional, and vital neurovascular structures, including the facial nerve, cochlea, semicircular canals, internal carotid artery, and middle ear. This fully open-source 3-dimensional (3D) model of the temporal bone, created for educational purposes, should be easily and economically reproducible using a 3D printer, offering all residents the opportunity to understand the spatial location, three-dimensional anatomical structures, and fundamental intricacies of mastoidectomy. METHODS: A 3D model of the temporal bone was fabricated using a computed tomography (CT) scan derived from an actual human body. The CT scan of the model was meticulously juxtaposed with the reference sample CT scan. Neurosurgical residents were recruited as participants for this study. Each participant was tasked with executing a mastoidectomy on 2 separate occasions, with a 2-week interval between attempts. Throughout these sessions, various parameters, including the time taken for task completion, the volume of bone removal, and any potential complications, were systematically registered. RESULTS: The mean volume of bone removed increased by 34.5%, and the mean task time and the mean number of complications decreased by 10.3% and 25%, respectively, during the training. CONCLUSION: Engaging in training with cost-effective anatomical models constitutes a valuable tool for refining technical skills during residency. We posit that this type of model training should be incorporated as part of the trainee's curriculum during the residency program because of the myriad advantages evidenced by the findings of this study.

The Coexistence of Carotico-Clinoid Foramen and Interclinoidal Osseous Bridge: An Anatomo-Radiological Study With Surgical Implications.

BACKGROUND AND OBJECTIVES: The coexistence of complete carotico-clinoid bridge (CCB), an ossification between the anterior (ACP) and the middle clinoid (MCP), and an interclinoidal osseous bridge (ICB), between the ACP and the posterior clinoid (PCP), represents an uncommonly reported anatomic variant. If not adequately recognized, osseous bridges may complicate open or endoscopic surgery, along with the pneumatization of the ACP, especially when performing anterior or middle clinoidectomies. METHODS: According to Preferred Reporting Items for Systematic Reviews and Meta-Analyses for Scoping Reviews guidelines, a systematic scoping review was conducted up to June 5, 2023. PubMed, Scopus, Web of Science databases, and additional citations were searched. Two hundred high-resolution noncontrast computed tomography (CT) scans (400 sides) and 41 dry skulls (82 sides) were analyzed to identify the different morphology of sellar bridges, focusing on the coexistence of complete CCF and ICB. Two embalmed latex-injected heads with coexisting CCF and ICB were dissected step-by-step to show the anatomic relationship with the surrounding structures from an endoscopic and microscopic perspective. RESULTS: A total of 19 articles were included. The review identified a complete CCF and ICB rate ranging from 4.92% to 6.3%. The analysis of 200 CT scans revealed a rate of coexistence in 4% of the cases, all encountered in White women. Two different types of interclinoid bridges were identified based on the degree of bone mineralization. Both endoscopic and macroscopic step-by-step dissections highlighted variability in morphology and consistency of the sellar bridges and the close relationship with the cavernous sinus neurovascular structures. CONCLUSION: The coexistence of CCF and ICB is an anatomic variation found in 4% of cases. Preoperative knowledge of the degree of mineralization and its relationship with surrounding structures is essential to performing safe surgery and minimizing cranial nerve and vascular injuries. Preoperative high-resolution CT scans can adequately identify these anatomic variations.

SupraPetrous InfraTemporal Approach: A Supplemental Approach to Supracerebellar Infratentorial for Inferior Amygdala and Hippocampal Head Access-A Cadaveric Study With Case Illustrations.

BACKGROUND AND OBJECTIVES: Access to the amygdala and hippocampus (A/H) is complex. To address the limitations and invasiveness of traditional approaches, including the Transsylvian, Subtemporal, and Supracerebellar infratentorial approaches, we developed the suprapetrous infratemporal (SPIT) approach. This study describes the nuances of this approach in both cadaveric studies and clinical cases. METHODS: Three unilateral exposures were performed using microscopic and endoscopic methodologies in the SPIT approach. After cadaveric investigation, this approach was successfully implemented in representative clinical cases. RESULTS: The SPIT approach enabled direct access to the inferior A/H, circumventing the requirement for temporal lobe retraction and detachment of the temporal lobe from the dura through a subtemporal route by drilling the upper part of the mastoid, consequently mitigating tension on the vein of Labbé. This enabled a bottom-up view because one would gain with a zygomatic osteotomy and forward projection like a mini-posterior petrosal view by using a transmastoid view, without cutting down the zygomatic arch and opening the dura subtemporally, limiting patient pain and preventing case comorbidity. The SPIT approach was performed in 2 cases of mesial temporal cavernoma presenting with seizures. The lesion was visualized intraoperatively and was successfully removed in these cases. The postoperative course was excellent with no complications, and gross total resection was radiographically confirmed with Engel Class 1a seizure freedom. CONCLUSION: The SPIT approach is a complementary approach for inferior A/H disease, combining the combined middle fossa approach modified for intradural pathology. Limited drilling of the upper aspect of the mastoid with a medial dural opening at the level of the arcuate eminence provides a direct trajectory with minimal brain retraction. Additional research encompassing a larger patient cohort and extended follow-up periods is required to substantiate the advantages of SPIT in the management of inferior A/H lesions.

Topography and Radiological Variables as Ancillary Parameters for Evaluating Tissue Adherence, Hypothalamic-Pituitary Dysfunction, and Recurrence in Craniopharyngioma: An Integrated Multidisciplinary Overview.

Craniopharyngiomas continue to present a challenge in clinical practice due to their heterogeneity and unpredictable adherence to vital neurovascular structures, particularly the hypothalamus. This results in different degrees of hypothalamus-pituitary axis dysfunction and a lack of uniform consensus and treatment guidelines regarding optimal management. MRI and CT are complementary techniques in the preoperative diagnostic phase, enabling the precise definition of craniopharyngioma size, shape, and consistency, as well as guiding classification into histopathological subtypes and topographical categories. Meanwhile, MRI plays a crucial role in the immediate postoperative period and follow-up stages by identifying treatment-related changes and residual tumors. This pictorial essay aims to provide an overview of the role of imaging in identifying variables indicative of the adherence degree to the hypothalamus, hypothalamic-pituitary dysfunction, the extent of surgical excision, and prognosis. For a more comprehensive assessment, we choose to distinguish the following two scenarios: (1) the initial diagnosis phase, where we primarily discuss the role of radiological variables predictive of adhesions to the surrounding neurovascular structures and axis dysfunction which may influence the choice of surgical resection; (2) the early post-treatment follow-up phase, where we discuss the interpretation of treatment-related changes that impact outcomes.

3D Printing for Customized Bone Reconstruction in Spheno-Orbital Meningiomas: A Systematic Literature Review and Institutional Experience.

Background: The treatment of spheno-orbital meningiomas (SOMs) requires extensive bone resections, creating significant defects in a complex geometrical space. Bone reconstruction represents a fundamental step that optimizes long-term aesthetic and functional outcomes. In recent years, 3D printing technology has also been exploited for complex skull base reconstructions, but reports remain scarce. Methods: We retrospectively analyzed four consecutive patients who underwent SOM resection and one-step 3D PEEK customized reconstruction from 2019 to 2023. A systematic review of 3D printing customized implants for SOM was then performed. Results: All patients underwent a frontotemporal craniotomy, removal of SOM, and reconstruction of the superolateral orbital wall and pterional region. The aesthetic outcome was extremely satisfactory in all cases. No orbital implant malposition or infectious complications were documented. Eleven papers were included in the literature review, describing 27 patients. Most (23) patients underwent a single-stage reconstruction; in three cases, the implant was positioned to correct postoperative delayed enophthalmos. Porous titanium was the most used material (16 patients), while PEEK was used in three cases. Prosthesis malposition was described in two (7.4%) patients. Conclusions: Single-step reconstruction with a personalized 3D PEEK prosthesis represents a valid reconstruction technique for the treatment of SOMs with good aesthetic outcomes.

A Systematic Review of the Metabolism of High-Grade Gliomas: Current Targeted Therapies and Future Perspectives.

High-grade glial tumors (HGGs) exhibit aggressive growth patterns and high recurrence rates. The prevailing treatment approach comprises radiation therapy (RT), chemotherapy (CMT), and surgical resection. Despite the progress made in traditional treatments, the outlook for patients with HGGs remains bleak. Tumor metabolism is emerging as a potential target for glioma therapies, a promising approach that harnesses the metabolism to target tumor cells. However, the efficacy of therapies targeting the metabolism of HGGs remains unclear, compelling a comprehensive review. This study aimed to assess the outcome of present trials on HGG therapies targeting metabolism. A comprehensive search of PubMed, Ovid MEDLINE, and Ovid EMBASE was conducted until November 2023. The search method used pertinent Medical Subject Heading (MeSH) terminologies and keywords referring to "high-grade gliomas", "metabolism", "target therapies", "monoclonal antibodies", "overall survival", and "progression-free survival". The review analyzed studies that focused on therapies targeting the metabolism of HGGs in human subjects. These studies included both randomized controlled trials (RCTs) and non-randomized controlled trials (NRCTs). Out of 284 articles identified, 23 trials met the inclusion criteria and were thoroughly analyzed. Phase II trials were the most numerous (62%). Targeted metabolic therapies were predominantly used for recurrent HGGs (67%). The most common targeted pathways were the vascular endothelial growth factor (VEGF, 43%), the human epidermal growth factor receptor (HER, 22%), the platelet-derived growth factor (PDGF, 17%), and the mammalian target of rapamycin (mTOR, 17%). In 39% of studies, the subject treatment was combined with CMT (22%), RT (4%), or both (13%). The median OS widely ranged from 4 to 26.3 months, while the median PFS ranged from 1.5 to 13 months. This systematic literature review offers a thorough exploration of the present state of metabolic therapies for HGGs. The multitude of targeted pathways underscores the intricate nature of addressing the metabolic aspects of these tumors. Despite existing challenges, these findings provide valuable insights, guiding future research endeavors. The results serve as a foundation for refining treatment strategies and enhancing patient outcomes within the complex landscape of HGGs.

Full-Extension Eyebrow Approach with Supraorbital Nerve Preservation for Frontal Sinus Tumors.

Frontal sinus surgery still represents a challenge due to its complex and highly variable anatomy. In this manuscript, we present a detailed anatomical description of an eyebrow approach that allows full exposure of the frontal sinus with a large osteoplastic bone flap and preservation of the supraorbital nerve. Laryngoscope, 134:1633-1637, 2024.

Aggressive PitNETs and Potential Target Therapies: A Systematic Review of Molecular and Genetic Pathways.

Recently, advances in molecular biology and bioinformatics have allowed a more thorough understanding of tumorigenesis in aggressive PitNETs (pituitary neuroendocrine tumors) through the identification of specific essential genes, crucial molecular pathways, regulators, and effects of the tumoral microenvironment. Target therapies have been developed to cure oncology patients refractory to traditional treatments, introducing the concept of precision medicine. Preliminary data on PitNETs are derived from preclinical studies conducted on cell cultures, animal models, and a few case reports or small case series. This study comprehensively reviews the principal pathways involved in aggressive PitNETs, describing the potential target therapies. A search was conducted on Pubmed, Scopus, and Web of Science for English papers published between 1 January 2004, and 15 June 2023. 254 were selected, and the topics related to aggressive PitNETs were recorded and discussed in detail: epigenetic aspects, membrane proteins and receptors, metalloprotease, molecular pathways, PPRK, and the immune microenvironment. A comprehensive comprehension of the molecular mechanisms linked to PitNETs' aggressiveness and invasiveness is crucial. Despite promising preliminary findings, additional research and clinical trials are necessary to confirm the indications and effectiveness of target therapies for PitNETs.

Surgical Anatomy of the Microscopic and Endoscopic Transorbital Approach to the Middle Fossa and Cavernous Sinus: Anatomo-Radiological Study with Clinical Applications.

BACKGROUND: The transorbital approaches (TOAs) have acquired growing notoriety, thanks to their ability to offer alternative corridors to the skull base. However, the limited access and the unfamiliarity with this surgical perspective make recognition of key landmarks difficult, especially for less experienced surgeons. The study wants to offer a detailed description of the anatomy to comprehend the potential and limitations of TOAs. METHODS: Measurements of the orbit region and the surrounding areas were performed on two hundred high-resolution CT scans and thirty-nine dry skulls. Five specimens were dissected to illustrate the TOA, and one was used to perform the extradural clinoidectomy. Three clinical cases highlighted the surgical applications. RESULTS: A step-by-step description of the key steps of the TOA was proposed and a comparison with the transcranial anterior clinoidectomy was discussed. The mean work distance was 6.1 ± 0.4 cm, and the lateral working angle increased 20 ± 5.4° after removing the lateral orbital rim. CONCLUSIONS: TOAs are indicated in selected cases when tumor involves the lateral portion of the cavernous sinus or the middle skull base, obtaining a direct decompression of the optic nerve and avoiding excessive manipulation of the neurovascular structures. Comprehension of surgical anatomy of the orbit and its surrounding structures is essential to safely perform these approaches.

The "candy wrapper" of the pituitary gland: a road map to the parasellar ligaments and the medial wall of the cavernous sinus.

PURPOSE: The anatomy of the medial wall of the cavernous sinus (MWCS) and parasellar ligaments (PLs) has acquired increasing importance in endoscopic endonasal (EE) surgery of the cavernous sinus (CS), including resection of the MWCS in functioning pituitary adenomas (FPAs). Although anatomical studies have been published, it represents a debated topic due to their complex morphology. The aim is to offer a description of the PLs that originate from the MWCS and reach the lateral wall of the cavernous sinus (LWCS), proposing the "candy wrapper" model. The relationships between the neurovascular structures and histomorphological aspects were investigated. METHODS: Forty-two CSs from twenty-one human heads were studied. Eleven specimens were used for EE dissection; five underwent a microscopic dissection. Five specimens were used for histomorphological analysis. RESULTS: Two groups of PLs with a fan-shaped appearance were encountered. The anterior group included the periosteal ligament (55% sides) and the carotico-clinoid complex (100% sides), formed by the anterior horizontal and the carotico-clinoid ligaments. The posterior group was formed by the posterior horizontal (78% sides), and the inferior hypophyseal ligament (34% sides). The periosteal ligament originated inferiorly from the MWCS, reaching the periosteal dura. The anterior horizontal ligament was divided in a superior and inferior branch. The superior one continued as the carotid-oculomotor membrane, and the inferior branch reached the CN VI. The carotico-clinoid ligament between the middle and anterior clinoid was ossified in 3 sides. The posterior horizontal ligament was related to the posterior genu and ended at the LWCS. The inferior hypophyseal ligament followed the homonym artery. The ligaments related to the ICA form part of the adventitia. CONCLUSION: The "candy wrapper" model adds further details to the previous descriptions of the PLs. Understanding this complex anatomy is essential for safe CS surgery, including MWCS resection for FPAs.

3-Dimensionally Printed Affordable Nose Model: A Reliable Start in Endoscopic Training for Young Neurosurgeons.

BACKGROUND: Training neurosurgical skills is one of the most important tasks of a residency program. Techniques' complexity and pathology rarity define a long learning curve for mastering different surgical skills for which simulation on anatomic samples is extremely important. For this purpose, cadaver laboratory training is the most reliable tool. However, since access to cadaveric specimens is limited, due to costs and availability, surgical skills could be developed using inanimate models. This work aimed to develop a printable 3-dimensional model of the nasal cavity and sellar floor using an open-source downloadable file, to give residents the opportunity to improve their endoscopic surgical skills in a low-risk atmosphere with little cost. METHODS: The 3D model was realized taking as a sample a real-case CT scan imaging from which the sellar floor was removed. A quail egg was placed underneath the printed model covering the sellar floor opening. Under endoscopic visualization, the "sellar floor" was drilled by each participant with the goal of sparing the egg's inner membrane. Once the task was achieved, surgeons were asked to participate in a satisfaction survey. RESULTS: The total cost for printing was 6.31€ (6,72$). A satisfaction survey showed technical improvement (90%), increased confidence (80%), and bringing learned skills into the operating room (70%), leading to a 100% agreement in introducing this project into residency programs. CONCLUSIONS: Training on affordable anatomic models represents a useful tool in technical skills improvement. We believe this model could help residents bring their technical capabilities to more sophisticated levels.

Brain Arteriovenous Malformations and Pregnancy: A Systematic Review of the Literature.

BACKGROUND: The bleeding risk and outcome of pregnant women harboring intracranial arteriovenous malformations are still unclear, and no consensus has been achieved on management timing and strategy. METHODS: We searched PubMed, MEDLINE, and EMBASE from 1990 to 2022 for studies evaluating the bleeding risk and the outcome of women with intracranial arteriovenous malformations. Our primary end point was the hemorrhage rate. The secondary end points were pregnancy outcome and treatment safety for the mother and the fetus. RESULTS: Nine studies reporting on 2426 women were included. The overall hemorrhage rate in untreated women was 2.6%. The rate of first bleeding during pregnancy and postpartum was greater than the respective fertile period in unpregnant women (11% vs. 6.7%). The risk of first bleeding was greater in the II and III trimesters (4.5% and 2.9%), while was lower during delivery and puerperium (0.1% and 0.2%). The majority of the women did not report any complications after pregnancy and early postpartum death occurred in 4.1% of cases. The overall miscarriage rate was 12.4%. CONCLUSIONS: Women harboring intracranial arteriovenous malformations appear to have a greater risk of hemorrhage during pregnancy. There is an increased bleeding risk in the later stages of gestation, whereas delivery and puerperium are less risky phases. Outcomes are relatively good for the mother, with low rates of mortality and unfavorable sequelae, but there is a risk of miscarriage for the fetus. Intervention should be undertaken prophylactically before pregnancy or during early gestation if possible. For pregnant women who deferred treatment, multidisciplinary management is advised.