Indications for surgical evacuation of cerebellar intracerebral hemorrhage: consensus guidelines from the French Society of Neurosurgery (SFNC) and the French Society of Vascular Neurology (SFNV).

BACKGROUND: Cerebellar intracerebral hemorrhage (ICH) is associated with poor functional prognosis and high mortality. Surgical evacuation has been proposed to improve outcome. The purpose of this review was to determine the benefit of surgical evacuation of cerebellar ICH and to establish guidelines for when it should be performed. METHOD: The writing committee comprised 9 members of the SFNV and the SFNC. Recommendations were established based on a literature review using the PICO questions. The American Heart Association (AHA) classification was used to define recommendation level. In case of insufficient evidence, expert opinions were provided. RESULTS: Levels of evidence were low to moderate, precluding definitive recommendations. Based on available data, surgical hematoma evacuation is not recommended to improve functional outcome (Class III; Level B NR). However, based on subgroup analysis, surgical evacuation may be considered in strictly selected patients (Class IIb; Level C-EO): hematoma volume 15-25 cm3, GCS 6-10, and no oral anticoagulation or antiplatelet therapy. Moreover, surgical evacuation is recommended to decrease risk of death (Class IIa; Level B NR) in patients with a hematoma volume >15 cm3 and GCS score <10. CONCLUSION: These guidelines were based on observational studies, limiting the level of evidence. However, except for strictly selected patients, surgical evacuation of cerebellar ICH was not associated with improved functional outcome, limiting indications. Data from RCTs are needed in this field.

Patient Selection in a Pragmatic Study on the Management of Patients with Brain Arteriovenous Malformations.

BACKGROUND: The Treatment of Brain Arteriovenous Malformations Study (TOBAS) is an all-inclusive pragmatic study comprising 2 randomized clinical trials (RCTs). Patients excluded from the RCTs are followed in parallel treatment and observation registries, allowing a comparison between RCT and registry patients. METHODS: The first randomized clinical trial (RCT-1) offers 1:1 randomized allocation of intervention versus conservative management for patients with arteriovenous malformation (AVM). The second randomized clinical trial (RCT-2) allocates 1:1 pre-embolization or no pre-embolization to surgery or radiosurgery patients judged treatable with or without embolization. Characteristics of RCT patients are reported and compared to registry patients. RESULTS: From June 2014 to May 2021, 1010 patients with AVM were recruited; 498 patients were observed and 373 were included in the treatment registries. Randomized allocation in RCT-1 was applied to 139 (26%) of the 512 patients (including 127 of 222 [57%] with unruptured AVMs) considered for curative treatment. RCT-1 AVM patients differed (in rupture status, Spetzler-Martin grade and baseline modified Rankin Score) from those in the observation or treatment registries (P < 0.001). Most patients had small (<3 cm; 71%) low-grade (Spetzler-Martin I-II; 64%) unruptured (91%) AVMs. The allocated management was conservative (n = 71) or curative (n = 68), using surgery (n = 39), embolization (n = 16), or stereotactic radiosurgery (n = 13). Pre-embolization was considered for 179/309 (58%) patients allocated/assigned to surgery or stereotactic radiosurgery; 87/179 (49%) were included in RCT-2. RCT-2 patient AVMs differed in size, eloquence and grade from patients of the pre-embolization registry (P < 0.01). Most had small (<3 cm in 82%) low-grade (83%) AVMs in non-eloquent brain (64%). CONCLUSIONS: Patients included in the RCTs differ significantly from registry patients. Meaningful results can be obtained if multiple centers actively participate in the TOBAS RCTs.

Clinical effectiveness of hypertonic sodium lactate infusion for intraoperative brain relaxation in patients undergoing scheduled craniotomy for supratentorial brain tumor resection: A study protocol of a single center double-blind randomized controlled phase II pilot trial.

INTRODUCTION: Hyperosmolar solutions are prescribed in neurosurgery patients to provide satisfactory intraoperative brain relaxation and to lower cerebral injuries related to surgical retractors. Mannitol is traditionally considered as the first-choice solution for brain relaxation in neurosurgery patients. Hypertonic sodium lactate infusion was reported to provide a higher and longer osmotic effect compared to mannitol in severely brain-injured patients and to prevent impaired cerebral energetics related to brain injuries. To date, the clinical effectiveness of hypertonic sodium lactate infusion has never been studied in neurosurgery patients. The hypothesis of the study is that hyperosmolar sodium lactate infusion may provide satisfactory intraoperative brain relaxation in patients undergoing scheduled craniotomy for supratentorial brain tumor resection. METHODS AND ANALYSIS: We designed a phase II randomized, controlled, double-blind, single-center pilot trial, and aim to include 50 adult patients scheduled for craniotomy for supratentorial brain tumor resection under general anesthesia. Patients will be randomized to receive either mannitol (conventional group) or hypertonic sodium lactate (intervention group) infusion at the time of skin incision. Brain relaxation (primary outcome) will be assessed immediately after opening the dura by the neurosurgeon blinded to the treatment allocated using a validated 4-point scale. The primary outcome is the proportion of satisfactory brain relaxation, defined as brain relaxation score of 3 or 4. ETHICS AND DISSEMINATION: This study was approved by the Ethics Committee (Comité de Protection des Personnes Est III) and authorized by the French Health Authority (Agence Nationale de Sécurité des Médicaments, Saint-Denis, France). The University Hospital of Besancon is the trial sponsor and the holder of all data and publication rights. Results of the study will be submitted for publication in a peer-review international medical journal and for presentation in abstract (oral or poster) in international peer-reviewed congresses. REGISTRATION: The trial is registered with ClinicalTrials.gov (Identifier: NCT04488874, principal investigator: Prof Guillaume Besch, date of registration: July 28, 2020).

The Largest Germline Heterozygous Deletion Encompassing Potocki-Shaffer and WAGR Syndromes Loci to Date: A Case Report.

Potocki-Schaffer syndrome includes multiple exostoses, parietal foramina, and variable developmental delay/intellectual disability. It is associated with a heterozygous deletion of the 11p12p11.2 region. In some cases, the deletion extends to the WAGR locus (11p13p12). We describe here a 9-month-old girl harboring the largest germline heterozygous deletion characterized so far. Oligohydramnios and parietal foramina were noticed during pregnancy. No patient has been diagnosed before with concomitance of these two syndromes during the prenatal period. Cytogenetic diagnosis was anticipated on basis of clinical and radiological signs. Postnatal conventional karyotype confirmed an interstitial 11p deletion: 46,XX,del(11)(p11.2p15.1). Array-comparative genomic hybridization characterized a 29.6 Mb deletion. Our case illustrates the interest of high-resolution genomic approaches to correlate adequately clinical phenotypes with specific genes in suspected contiguous gene deletion syndromes.

Prone Positioning for Severe Acute Respiratory Distress Syndrome in COVID-19 Patients by a Dedicated Team: A Safe and Pragmatic Reallocation of Medical and Surgical Work Force in Response to the Outbreak.

OBJECTIVE: The aim of this study was to define whether rapidly reallocating health care workers not experienced with PP for performing PP in ICU is feasible and safe. SUMMARY BACKGROUND DATA: In the setting of severe acute respiratory distress syndrome (ARDS), the use of prone and supine positioning procedures (PP) has been associated with improved oxygenation resulting in decreased mortality. Nevertheless, applying PP is time consuming for ICU staffs that are at risk of mental of physical exhaustion, especially with the constant surge of admitted COVID-19 patients with severe ARDS. METHODS: This prospective cohort study conducted at a single regional university hospital between March 27 and April 15, 2020. Among 117 patients admitted to ICU, 67 patients (57.3%) presented with proven SARS-CoV-2 infection with severe ARDS requiring PP. After accelerated simulation training, 109 volunteers including surgeons, physicians, nurses and physiotherapists, multiple dedicated teams performed daily multiple PP following a systematic checklist. Patient demographics and PP data were collected. Patient safety and health care workers safety were assessed. RESULTS: Among 117 patients admitted to ICU, 67 patients (57.3%) required PP. Overall, 53 (79%) were male, with a median age of 68.5 years and median body mass index of 29.3 kg/m. A total of 384 PP were performed. Overall, complication occurred in 34 PP (8.8%) and led to PP cancelation in 4 patients (1%). Regarding health care workers safety, four health care workers presented with potential COVID-19 related symptoms and none was positive. CONCLUSIONS: To overcome the surge of critically ill COVID-19 patients, reallocating health care workers to targeted medical tasks beyond their respective expertise such as PP was safe.

A Survey of Microsurgical Technique for Extracranial-to-Intracranial Bypass.

BACKGROUND: Extracranial-to-intracranial bypass surgery is a well-established technique that has been practiced for over 50 years. Since then, numerous technical variants have developed nationally and internationally. OBJECTIVE: Based on a survey, to collect information on cerebrovascular bypass surgeons and their background, surgical volume, and technical steps of extracranial-to-intracranial bypasses with focus on superficial temporal artery to middle cerebral artery (STA-MCA) bypass. METHODS: An electronic survey was distributed among bypass neurosurgeons. Responses were analyzed for national-international variations of STA-MCA bypass surgery techniques. The survey focused on the technical aspects of the surgery itself rather than patient selection or perioperative management. RESULTS: Survey responses were collected from 51 neurosurgeons performing cerebrovascular bypass, from 11 different countries across North America, Europe, and Asia. The largest age block was early-to mid-career (66.7% aged 36-50 years). Most participating surgeons (80.40%) performed less than 20 bypasses annually, whereas a select few surgeons (3) performed more than 50 annually. The most common bypass was STA-M4 MCA bypass with a linear incision (34%) over the parietal branch (44%) and choosing an MCA recipient based on diameter (61.2%). The interrupted anastomosis technique was most common (74%). CONCLUSIONS: The results of this electronic survey will help to identify common patterns in STA-MCA bypass surgery and will serve as a guide to other neurosurgeons to modify and improve their technique. Cerebrovascular bypass is still widely practiced, including by young neurosurgeons, who are actively learning from established masters who share their experience.

Brain Perfusion Before and After Surgical Treatment of Moyamoya Disease.

Moyamoya disease is characterized by bilateral progressive terminal internal carotid arteries steno-occlusion. In this patient, the disease affected middle cerebral arteries and was disclosed after a left frontal cerebral infarction. Brain HMPAO perfusion scintigraphy with acetazolamide challenge, obtained before surgical treatment, demonstrates an extended bilateral frontal hypoperfusion with a vascular steal phenomenon. The same examination obtained after surgical treatment demonstrates a frank improvement in basal brain vascularization, with nearly complete correction of vascular steal phenomenon. HMPAO brain scintigraphy is a very useful tool in the assessment of moyamoya disease severity before surgery and for the evaluation of treatment efficiency.

A Giant Tumefactive Virchow-Robin Space: A Rare Cause of a Homonymous Quadrantanopia.

A 53-year-old woman was found to have a left inferior homonymous quadrantanopia. Brain MRI disclosed a giant Virchow-Robin space compressing the right optic tract. After fenestration of this cystic lesion, most of the visual field loss resolved. Giant Virchow-Robin spaces may cause homonymous field defects which, with appropriate management, may improve.

Research Progresses in Understanding the Pathophysiology of Moyamoya Disease.

BACKGROUND: The pathogenesis of moyamoya disease (MMD) is still unknown. The detection of inflammatory molecules such as cytokines, chemokines and growth factors in MMD patients' biological fluids supports the hypothesis that an abnormal angiogenesis is implicated in MMD pathogenesis. However, it is unclear whether these anomalies are the consequences of the disease or rather causal factors as well as these mechanisms remain insufficient to explain the pathophysiology of MMD. The presence of a family history in about 9-15% of Asian patients, the highly variable incidence rate between different ethnic and sex groups and the age of onset support the role of genetic factors in MMD pathogenesis. However, although some genetic loci have been associated with MMD, few of them have been replicated in independent series. Recently, RNF213 gene was shown to be strongly associated with MMD occurrence with a founder effect in East Asian patients. However, the mechanisms leading from RNF213 mutations to MMD clinical features are still unknown. SUMMARY: The research on pathogenic mechanism of MMD is in its infancy. MMD is probably a complex and heterogeneous disorder, including different phenotypes and genotypes, in which more than a single factor is implicated. KEY MESSAGE: Since the diagnosis of MMD is rapidly increasing worldwide, the development of more efficient stratifying risk systems, including both clinical but also biological drivers became imperative to improve our ability of predict prognosis and to develop mechanism-tailored interventions.

Radiological pitfalls of a large intracranial dissecting aneurysm.

We report the case of a large dissecting aneurysm of the anterior cerebral artery revealed by cerebral infarction in 38-year-old man. The volume and aspect of the aneurysm initially led us to the diagnosis of saccular aneurysm. Given the complete thrombosis, the risk of bleeding was low and antithrombotic therapy was started. Surgery could be discussed later. However radiological monitoring by MRI (magnetic resonance imaging) showed a rapid decrease in volume of the aneurysm. The final angiography found an aspect of stenosis followed by a little arterial dilatation. The diagnosis of dissecting anterior cerebral aneurysm was a posteriori established.

Preoperative simulation for the planning of microsurgical clipping of intracranial aneurysms.

INTRODUCTION: The safety and success of intracranial aneurysm (IA) surgery could be improved through the dedicated application of simulation covering the procedure from the 3-dimensional (3D) description of the surgical scene to the visual representation of the clip application. We aimed in this study to validate the technical feasibility and clinical relevance of such a protocol. METHODS: All patients preoperatively underwent 3D magnetic resonance imaging and 3D computed tomography angiography to build 3D reconstructions of the brain, cerebral arteries, and surrounding cranial bone. These 3D models were segmented and merged using Osirix, a DICOM image processing application. This provided the surgical scene that was subsequently imported into Blender, a modeling platform for 3D animation. Digitized clips and appliers could then be manipulated in the virtual operative environment, allowing the visual simulation of clipping. This simulation protocol was assessed in a series of 10 IAs by 2 neurosurgeons. RESULTS: The protocol was feasible in all patients. The visual similarity between the surgical scene and the operative view was excellent in 100% of the cases, and the identification of the vascular structures was accurate in 90% of the cases. The neurosurgeons found the simulation helpful for planning the surgical approach (ie, the bone flap, cisternal opening, and arterial tree exposure) in 100% of the cases. The correct number of final clip(s) needed was predicted from the simulation in 90% of the cases. The preoperatively expected characteristics of the optimal clip(s) (ie, their number, shape, size, and orientation) were validated during surgery in 80% of the cases. CONCLUSIONS: This study confirmed that visual simulation of IA clipping based on the processing of high-resolution 3D imaging can be effective. This is a new and important step toward the development of a more sophisticated integrated simulation platform dedicated to cerebrovascular surgery.

Clinical and imaging follow-up after surgical or endovascular treatment in patients with unruptured carotid-ophthalmic aneurysm.

BACKGROUND: Carotido-ophthalmic aneurysms are complex and their treatment is challenging. Few data are available on patient follow-up after endovascular or surgical treatment. OBJECTIVE: To evaluate outcome of patients with unruptured carotido-ophthalmic aneurysm after endovascular or surgical treatment. MATERIALS AND METHODS: This series included 52 consecutive patients in a single center treated for an unruptured carotido-ophthalmic aneurysm at Lille University Hospital between 2000 and 2011. Visual disturbances were present in 5 patients. Treatment option (endovascular or microsurgical) was decided for each patient in a multidisciplinary meeting. We recorded age and the American Society of Anesthesiology score (ASA) before treatment and the modified Rankin Scale score (mRS) at 3 months after treatment. All patients had conventional angiography performed before and immediately after treatment. Long-term imaging follow-up was performed at 3 years after treatment. RESULTS: Treatment was endovascular in 29 patients and microsurgical in 23. The mean follow-up was 4.6 years. Conventional angiograms showed multiple intracranial aneurysms in 26 patients. Age, pre-therapeutic ASA score and mRS score at 3 months after treatment showed no significant difference between microsurgery and endovascular treatment. Imaging follow-up showed aneurysm recurrence after endovascular treatment in 6 patients including 3 with major recurrence that required further treatment by microsurgery. In these 3 major recurrences, the initial conventional angiography demonstrated the origin of the ophthalmic artery at the neck or from the aneurysmal sac in 3 cases. After microsurgery, conventional angiography showed a remnant neck in 2 patients including 1 treated by further endovascular procedure. CONCLUSION: Endovascular treatment remains the first therapeutic option when the ophthamic artery originates at a distance from the neck, but microsurgery should be considered for large aneurysms with optic nerve compression, or when the ophthalmic artery arises from the neck of the aneurysm.

Decompressive surgery for malignant middle cerebral artery infarcts: the results of randomized trials can be reproduced in daily practice.

BACKGROUND: In clinical randomized controlled trials (RCTs), decompressive surgery (DS) for malignant middle cerebral artery (MMCA) infarcts leads to a 50% absolute risk reduction in mortality, and improves the 1-year functional outcome. The reproducibility of these results in routine practice has never been evaluated. The purpose of this study was to test the hypothesis that the results of DS for MMCA in practice are similar to those observed in the surgical group of RCTs. METHODS: We prospectively included the first 31 patients who underwent DS for MMCA. They were screened based on similar criteria as in the meta-analysis. The primary outcome was a modified Rankin Scale (mRS) score of ≤4, and secondary outcomes were mRS of ≤3 and death at 1 year. RESULTS: Thirty-one patients underwent DS for MMCA. The 1-year mRS was ≤4 in 22 patients (71.0%) and ≤3 in 16 (51.6%). Seven patients died (22.6%). CONCLUSION: This observational study showed that DS for MMCA in a center without previous experience provides similar results as those obtained in the surgical arm of RCTs.

Relevance of expandable titanium cage for the treatment of cervical spondylotic myelopathy.

BACKGROUND: In patients with cervical spondylotic myelopathy, ventral disease and loss of physiological cervical lordosis are indications for anterior approach. As bone graft and titanium cage present many drawbacks, expandable titanium cage has been recently introduced for this indication. The authors present the clinical and radiological outcomes in patients undergoing the placement of an expandable cage in the treatment of spondylotic myelopathy with straight or kyphotic cervical spine alignment. METHODS: This was a retrospective review of prospectively collected data. A total of 26 patients underwent cervical corpectomy and reconstruction using an expandable titanium cage and anterior plate between 2005 and 2008. Pain and functional disability were measured using VAS and mJOA preoperatively and at 3 months, 6 months, 1 year and 2 years. Kyphosis was measured using lateral radiographs at the same points of follow-up. Fusion was evaluated on flexion-extension radiographs at 2 years. RESULTS: The mean VAS improved from 4.2 to 1.7 and the mean mJOA increased from 12.85 to 16.04 at 2 years postoperatively (p < 0.05). The mean kyphosis angle decreased from 17° to 2° at the last follow-up (p < 0.05). The fusion rate was 100% at 2 years. Three complications were reported including a transient dysphagia, an epidural hematoma and an early hardware migration. CONCLUSION: Expandable titanium cage is an effective device, which achieves good clinical and radiological outcomes at a minimum 2-year follow-up.

Surgery for unruptured intracranial aneurysms in the ISAT and ISUIA era.

BACKGROUND: The ISAT and ISUIA studies, along with the improvement of endovascular treatment (EVT) have strongly influenced the management of intracranial aneurysms (IAs). We present our experience in the microsurgical treatment of unruptured IAs (UIAs) in this context. METHODS: We retrospectively reviewed a consecutive series of non-giant UIAs selected for surgery during a five-year period. Patients and aneurysms characteristics, surgical results and outcome assessed by the Glascow Outcome Scale (GOS) at three month follow-up were studied. RESULTS: Eighty-five patients underwent 93 surgical procedures to obliterate 113 UIAs. Those were incidental in 89% of the cases and mainly located on the middle cerebral artery (65%). Patients were assigned to surgery according to their medical history (young, previous subarachnoid haemorrhage), aneurysm characteristics (wide neck, branch at the neck, "small" size, associated "surgical" aneurysm) or failure of EVT (5%). Operatively, 48% of UIAs had thin wall or blebs and 71% were occluded with one titanium clip. Thrombectomy or temporary clipping were necessary in 4% and 11% of the cases, three aneurysms peroperatively ruptured, four were deemed unclippable, three paraclinoid UIAs had an intracavernous residue and 16% were wrapped because of a small neck remnant (class 2). The mortality rate was 0% and 4% of the patients experienced a definitive major neurological deterioration. Final GOS was unchanged in 96% of the patients. CONCLUSIONS: Despite reduction in operative cases and in appropriately selected patients ineligible to EVT, microsurgical clipping of non-giant anterior circulation UIAs can still achieve good outcome with very low mortality and neurological morbidity.

Management of delayed oesophagus perforation and osteomyelitis after cervical spine surgery: review of the literature.

Oesophagus perforation is a possible complication of anterior cervical spine surgery, which generally occurs during the first postoperative days. Delayed perforation is very rare and its management remains controversial. We report the successful management of a delayed oesophagus perforation associated with an osteomyelitis.

Challenges in the management of ruptured and unruptured brainstem arteriovenous malformations: outcome after conservative, single-modality, or multimodality treatments.

BACKGROUND: Brainstem arteriovenous malformations are challenging lesions, and benefits of treatment are uncertain. OBJECTIVE: To study the clinical course of Brainstem arteriovenous malformations and the influence of treatments on outcome. METHODS: We reviewed a prospective series of 31 brainstem arteriovenous malformations. Demographic, morphological, and clinical characteristics were recorded. Factors determining initial and final outcomes (modified Rankin Scale), results of treatments (cure rates, complications), and disease course were analyzed. RESULTS: Brainstem arteriovenous malformations were symptomatic and bled in 93% and 61% of cases, respectively. Examination was abnormal and initial modified Rankin Scale score was < 3 in 71% and 86% of patients, respectively. The average follow-up time was 6.2 years, and 26% of patients rebled (5.9 %/y). Treatment modalities included conservative, radiosurgical, endovascular, surgical, and multimodality treatment in 13%, 58%, 35%, 16%, and 26% of cases, respectively. The obliteration rate was 60% overall and 39% after radiosurgery, 40% after embolization, and 75% after microsurgery, with respective complication-free cure rates of 71%, 50%, and 0%. Overall procedural mortality and morbidity were 2.3% and 18.6%, respectively. Final modified Rankin Scale score was < 3 in 77% of cases. Neurological deterioration (35%) was related to treatment complications in 74% of cases with a negative impact of surgery (P = .04), palliative embolization (odds ratio = 16), and multimodality treatments (odds ratio = 24). Radiosurgery was inversely associated with worsening (odds ratio = 0.06). CONCLUSION: Brainstem arteriovenous malformations require individualized treatment decisions. Single-modality treatments with a reasonable chance of complete cure and low complication rate (such as radiosurgery) should be favored.

Retrosigmoid approach for meningiomas of the cerebellopontine angle: results of surgery and place of additional treatments.

BACKGROUND: Meningiomas of the cerebellopontine angle (CPA), although homogenous in terms of location, present different surgical challenges depending on their site of dural origin and extension. Complete tumor resection sometimes leads to high morbidity. The objective of this work is to evaluate the results of surgery and the place of additional treatments. METHODS: In a series of 115 patients with CPA meningiomas, we retrospectively studied 69 patients operated on between 1994 and 2007 using a retrosigmoid approach. Clinical presentation, operative findings and functional outcome were reviewed for a mean follow-up time of 36 months. RESULTS: Usual presentation was hearing loss associated with gait disturbance (50%) and vertigo (35%). Preoperative cochlear evaluation was abnormal in 40% of the cases. Most tumors (90%) originated on the posterior face of the petrous part of the temporal bone. In one-third of the cases, the meningioma had invaded the internal acoustic meatus. Total or subtotal tumor removal was achieved in 91% of patients without perioperative mortality. Fourteen patients received additional treatment with radiotherapy or radiosurgery for a small residual tumor, often deliberately left in place to reduce operative morbidity. At long-term follow-up, facial nerve function was preserved in 91% of the cases. Hearing worsened in 17% of patients. The tumor recurred in only one case. CONCLUSION: The retrosigmoid approach is a safe surgical procedure. The modern management of CPA meningiomas should achieve as complete a resection as possible within reasonable limits, considering that a small residual tumor can be controlled fairly easily with radiosurgery for a relatively long time.

Subacute involvement of the medulla oblongata and occipital neuralgia revealing an intracranial dural arteriovenous fistula of the craniocervical junction.

A 58-year-old woman with cervicomedullary dural arteriovenous fistula (AVF) presenting with myelopathy, ipsilateral occipital neuralgia, and signs of involvement of the brainstem is reported and the previously published cases have been reviewed. The dural AVF was successfully treated surgically after an attempt of embolization.

Ruptured cerebral oncotic aneurysm from choriocarcinoma: report of two cases and review of the literature.

The study design involved case reports and review of the literature. Oncotic aneurysm from choriocarcinoma is an extremely rare event that should be known by all neurosurgeons and suspected in women of childbearing age. The purpose of this article is to report the authors' experience and to provide insight on clinical presentation and radiological signs to aware the reader to this entity and then to prevent misdiagnosis. The authors report two cases of ruptured oncotic aneurysm treated at their institution in 2010. A review of the literature was performed to discuss the pathogenesis and the role of the neurosurgeon. Chemotherapy is the treatment of choice making surgery necessary only for patients with large intracerebral haematoma that represents an immediate threat.