Predictors of Progression in a Series of 81 Adult Patients Surgically Managed for an Intracranial Hemangioblastoma: Implications for the Postoperative Follow-Up.

The aim was to identify predictors of progression in a series of patients managed for an intracranial hemangioblastoma, in order to guide the postoperative follow-up modalities. The characteristics of 81 patients managed for an intracranial hemangioblastoma between January 2000 and October 2022 were retrospectively analyzed. The mean age at diagnosis was of 48 ± 16 years. Eleven (14%) patients had von Hippel-Lindau disease. The most frequent tumor location was the cerebellar hemispheres (n = 51, 65%) and 11 (14%) patients had multicentric hemangioblastomas. A gross total resection was achieved in 75 (93%) patients. Eighteen (22%) patients had a local progression, with a median progression-free survival of 56 months 95% CI [1;240]. Eleven (14%) patients had a distant progression (new hemangioblastoma and/or growth of an already known hemangioblastoma). Local progression was more frequent in younger patients (39 ± 14 years vs. 51 ± 16 years; p = 0.005), and those with von Hippel-Lindau disease (n = 8, 44% vs. n = 3, 5%, p < 0.0001), multiple cerebral locations (n = 3, 17% vs. n = 2, 3%, p = 0.02), and partial tumoral resection (n = 4, 18% vs. n = 1, 2%, p = 0.0006). Therefore, it is advisable to propose a postoperative follow-up for at least 10 years, and longer if at least one predictor of progression is present.

Endovascular treatment of brain arteriovenous malformations: clinical outcomes of patients included in the registry of a pragmatic randomized trial.

OBJECTIVE: The role of endovascular treatment in the management of patients with brain arteriovenous malformations (AVMs) remains uncertain. AVM embolization can be offered as stand-alone curative therapy or prior to surgery or stereotactic radiosurgery (SRS) (pre-embolization). The Treatment of Brain AVMs Study (TOBAS) is an all-inclusive pragmatic study that comprises two randomized trials and multiple registries. METHODS: Results from the TOBAS curative and pre-embolization registries are reported. The primary outcome for this report is death or dependency (modified Rankin Scale [mRS] score > 2) at last follow-up. Secondary outcomes include angiographic results, perioperative serious adverse events (SAEs), and permanent treatment-related complications leading to an mRS score > 2. RESULTS: From June 2014 to May 2021, 1010 patients were recruited in TOBAS. Embolization was chosen as the primary curative treatment for 116 patients and pre-embolization prior to surgery or SRS for 92 patients. Clinical and angiographic outcomes were available in 106 (91%) of 116 and 77 (84%) of 92 patients, respectively. In the curative embolization registry, 70% of AVMs were ruptured, and 62% were low-grade AVMs (Spetzler-Martin grade I or II), while the pre-embolization registry had 70% ruptured AVMs and 58% low-grade AVMs. The primary outcome of death or disability (mRS score > 2) occurred in 15 (14%, 95% CI 8%-22%) of the 106 patients in the curative embolization registry (4 [12%, 95% CI 5%-28%] of 32 unruptured AVMs and 11 [15%, 95% CI 8%-25%] of 74 ruptured AVMs) and 9 (12%, 95% CI 6%-21%) of the 77 patients in the pre-embolization registry (4 [17%, 95% CI 7%-37%] of 23 unruptured AVMs and 5 [9%, 95% CI 4%-20%] of 54 ruptured AVMs) at 2 years. Embolization alone was confirmed to occlude the AVM in 32 (30%, 95% CI 21%-40%) of the 106 curative attempts and in 9 (12%, 95% CI 6%-21%) of 77 patients in the pre-embolization registry. SAEs occurred in 28 of the 106 attempted curative patients (26%, 95% CI 18%-35%, including 21 new symptomatic hemorrhages [20%, 95% CI 13%-29%]). Five of the new hemorrhages were in previously unruptured AVMs (n = 32; 16%, 95% CI 5%-33%). Of the 77 pre-embolization patients, 18 had SAEs (23%, 95% CI 15%-34%), including 12 new symptomatic hemorrhages [16%, 95% CI 9%-26%]). Three of the hemorrhages were in previously unruptured AVMs (3/23; 13%, 95% CI 3%-34%). CONCLUSIONS: Embolization as a curative treatment for brain AVMs was often incomplete. Hemorrhagic complications were frequent, even when the specified intent was pre-embolization before surgery or SRS. Because the role of endovascular treatment remains uncertain, it should preferably, when possible, be offered in the context of a randomized trial.

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.

Surgical treatment of brain arteriovenous malformations: clinical outcomes of patients included in the registry of a pragmatic randomized trial.

OBJECTIVE: The Treatment of Brain Arteriovenous Malformations Study (TOBAS) is a pragmatic study that includes 2 randomized trials and registries of treated or conservatively managed patients. The authors report the results of the surgical registry. METHODS: TOBAS patients are managed according to an algorithm that combines clinical judgment and randomized allocation. For patients considered for curative treatment, clinicians selected from surgery, endovascular therapy, or radiation therapy as the primary curative method, and whether observation was a reasonable alternative. When surgery was selected and observation was deemed unreasonable, the patient was not included in the randomized controlled trial but placed in the surgical registry. The primary outcome of the trial was mRS score > 2 at 10 years (at last follow-up for the current report). Secondary outcomes include angiographic results, perioperative serious adverse events, and permanent treatment-related complications leading to mRS score > 2. RESULTS: From June 2014 to May 2021, 1010 patients were recruited at 30 TOBAS centers. Surgery was selected for 229/512 patients (44%) considered for curative treatment; 77 (34%) were included in the surgery versus observation randomized trial and 152 (66%) were placed in the surgical registry. Surgical registry patients had 124/152 (82%) ruptured and 28/152 (18%) unruptured arteriovenous malformations (AVMs), with the majority categorized as low-grade Spetzler-Martin grade I-II AVM (118/152 [78%]). Thirteen patients were excluded, leaving 139 patients for analysis. Embolization was performed prior to surgery in 78/139 (56%) patients. Surgical angiographic cure was obtained in 123/139 all-grade (89%, 95% CI 82%-93%) and 105/110 low-grade (95%, 95% CI 90%-98%) AVM patients. At the mean follow-up of 18.1 months, 16 patients (12%, 95% CI 7%-18%) had reached the primary safety outcome of mRS score > 2, including 11/16 who had a baseline mRS score ≥ 3 due to previous AVM rupture. Serious adverse events occurred in 29 patients (21%, 95% CI 15%-28%). Permanent treatment-related complications leading to mRS score > 2 occurred in 6/139 patients (4%, 95% CI 2%-9%), 5 (83%) of whom had complications due to preoperative embolization. CONCLUSIONS: The surgical treatment of brain AVMs in the TOBAS registry was curative in 88% of patients. The participation of more patients, surgeons, and centers in randomized trials is needed to definitively establish the role of surgery in the treatment of unruptured brain AVMs. Clinical trial registration no.: NCT02098252 (ClinicalTrials.gov).

Purely cystic meningioma: Case report and systematic review of the literature.

Meningiomas are the most common benign intracranial tumors. They are generally asymptomatic, and discovered incidentally during cerebral imaging. The vast majority of meningiomas are solid, highly cellular and well-vascularized neoplasms. However, in several cases, they can be partially or, even rarely, almost completely cystic making their differential diagnosis and management challenging. In this paper, we present the rare case of a 59-year-old female patient, presenting with persistent headaches, who was diagnosed with a left parieto-occipital purely cystic lesion. The patient underwent a complete resection of this cystic lesion because of increasing headaches and volumetric progression. Interestingly, the histological assessment confirmed a cystic WHO grade I meningioma. The evolution was favorable and there was no recurrence after 3 years of follow-up. We also perform a systematic review of the literature concerning purely cystic meningiomas and we discuss the particular histological features of cystic meningiomas as well as the possible pathogenesis. This challenging clinical entity can easily be misdiagnosed as hemangioblastoma or glial/metastatic tumor with cystic component.

Full cervical cord tractography: A new method for clinical use.

Despite recent improvements in diffusion-weighted imaging, spinal cord tractography is not used in routine clinical practice because of difficulties in reconstructing tractograms, with a pertinent tri-dimensional-rendering, in a long post-processing time. We propose a new full tractography approach to the cervical spinal cord without extensive manual filtering or multiple regions of interest seeding that could help neurosurgeons manage various spinal cord disorders. Four healthy volunteers and two patients with either cervical intramedullary tumors or spinal cord injuries were included. Diffusion-weighted images of the cervical spinal cord were acquired using a Philips 3 Tesla machine, 32 diffusion directions, 1,000 s/mm2 b-value, 2 × 2 × 2 mm voxel size, reduced field-of-view (ZOOM), with two opposing phase-encoding directions. Distortion corrections were then achieved using the FSL software package, and tracking of the full cervical spinal cord was performed using the DSI Studio software (quantitative anisotropy-based deterministic algorithm). A unique region of avoidance was used to exclude everything that is not of the nervous system. Fiber tracking parameters used adaptative fractional anisotropy from 0.015 to 0.045, fiber length from 10 to 1,000 mm, and angular threshold of 90°. In all participants, a full cervical cord tractography was performed from the medulla to the C7 spine level. On a ventral view, the junction between the medulla and spinal cord was identified with its pyramidal bulging, and by an invagination corresponding to the median ventral sulcus. On a dorsal view, the fourth ventricle-superior, middle, and inferior cerebellar peduncles-was seen, as well as its floor and the obex; and gracile and cuneate tracts were recognized on each side of the dorsal median sulcus. In the case of the intramedullary tumor or spinal cord injury, the spinal tracts were seen to be displaced, and this helped to adjust the neurosurgical strategy. This new full tractography approach simplifies the tractography pipeline and provides a reliable 3D-rendering of the spinal cord that could help to adjust the neurosurgical strategy.

Outcomes of Unruptured Low-Grade Brain Arteriovenous Malformations Using TOBAS (Treatment of Brain Arteriovenous Malformations Study) Criteria.

BACKGROUND: Treatment of an unruptured brain arteriovenous malformation (bAVM) is a matter of debate, especially for low-grade bAVM (Spetzler-Martin grade I and II). The aim is to compare the outcomes of patients with low-grade unruptured bAVM after interventional or medical management in a pragmatic manner. METHODS: Adults with unruptured low-grade bAVM diagnosed between 2006 and 2016 were included. The primary end points were death from all causes and disabling stroke that resulted in a modified Rankin Scale (mRS) score >2 at last follow-up. RESULTS: Eighty-four patients presented with an unruptured Spetzler-Martin low-grade bAVM. Among these patients, 55 (65.5%) were treated and 29 (34.5%) were untreated, with no differences regarding clinical and radiologic characteristics. The modality of treatment was embolization in 25.5%, radiosurgery (alone, 30.9%; with embolization, 18.2%), and surgery (alone, 5.5%; with embolization, 20%). The rupture rate was 6.7% person-year in the untreated group; 12.7% (n = 7) of treated and 16.7% (n = 5) of untreated patients achieved the primary evaluation criteria (P = 0.744). Using a Kaplan-Meier curve, the probability of reaching this criterion at 5 years was not different between groups (P = 0.07). Complications resulting in an mRS score >2 at last follow-up occurred in 9.1%, in 80% of cases after embolization. CONCLUSIONS: This study shows no differences between treated and untreated low-grade bAVM. Embolization seems to carry a high risk of complication and should be used with caution. The small number of cases must encourage cautious interpretations especially because of the spontaneous high-rupture rate. One major interest is to investigate center habits in pathology when treatment standards are limited.

Characteristics and management of hydrocephalus in adult patients with cerebellar glioblastoma: lessons from a French nationwide series of 118 cases.

The characteristics of hydrocephalus associated with cerebellar glioblastoma (cGB) remain poorly known. The objectives were to describe the occurence of hydrocephalus in a French nationwide series of adult patients with cGB, to identify the characteristics associated with hydrocephalus and to analyze the outcomes associated with the different surgical strategies, in order to propose practical guidelines. Consecutive cases of adult cGB patients prospectively recorded into the French Brain Tumor Database between 2003 and 2017 were screened. Diagnosis was confirmed by a centralized neuropathological review. Among 118 patients with cGB (mean age 55.9 years), 49 patients (41.5%) presented with pre-operative hydrocephalus. Thirteen patients (11.0%) developed acute (n=7) or delayed (n=6) hydrocephalus postoperatively. Compared to patients without hydrocephalus at admission, patients with hydrocephalus were younger (52.0 years vs 58.6 years, p=0.03) and underwent more frequently tumor resection (93.9% vs 73.9%, p=0.006). A total of 40 cerebrospinal-fluid diversion procedures were performed, including 18 endoscopic third ventriculostomies, 12 ventriculoperitoneal shunts and 10 external ventricular drains. The different cerebrospinal-fluid diversion options had comparable functional results and complication rates. Among the 89 patients surgically managed for cGB without prior cerebrospinal-fluid diversion, 7 (7.9%) were long-term shunt-dependant. Hydrocephalus is frequent in patients with cGB and has to be carefully managed in order not to interfere with adjuvant oncological treatments. In case of symptomatic hydrocephalus, a cerebrospinal-fluid diversion is mandatory, especially if surgical resection is not feasible. In case of asymptomatic hydrocephalus, a cerebrospinal-fluid diversion has to be discussed only if surgical resection is not feasible.

Alterations of cerebral microcirculation in peritumoral edema: feasibility of in vivo sidestream dark-field imaging in intracranial meningiomas.

BACKGROUND: Intracranial meningiomas display a variable amount of peritumoral brain edema (PTBE), which can significantly impact perioperative morbidity. The role of microcirculatory disturbances in the pathogenesis of PTBE is still debated. The aim of this study was to microscopically demonstrate and intraoperatively quantify, for the first time, the alterations to microcirculation in PTBE using sidestream dark-field (SDF) imaging. METHODS: Adult patients with WHO grade I meningiomas were recruited over a 9-month period and divided into 2 groups depending on the absence (NE group) or the presence (E group) of PTBE. In vivo intraoperative microcirculation imaging was performed in the peritumoral area before and after microsurgical resection. RESULTS: Six patients were included in the NE group and 6 in the E group. At the baseline in the NE group, there was a minor decrease in microcirculatory parameters compared to normal reference values, which was probably due to the mass effect. In contrast, microcirculatory parameters in the E group were significantly altered, affecting both vessel density and blood flow values, with a drop of approximately 50% of normal values. Surgical resection resulted in a quasi-normalization of microcirculation parameters in the NE group, whereas in the E group, even if all parameters statistically significantly improved, post-resection values remained considerably inferior to those of the normal reference pattern. CONCLUSION: Our study confirmed significant alterations of microcirculatory parameters in PTBE in meningiomas. Further in vivo SDF imaging studies may explore the possible correlation between the severity of these microcirculatory alterations and the postoperative neurological outcome.

Arteriovenous malformation drained into a developmental venous anomaly: A case report and up-dated literature review.

INTRODUCTION: Although the association of developmental venous anomalies (DVAs) with cavernous malformations is well documented, the association with arteriovenous malformations (AVM) is unusual. The aim is herein to report an additional case and to review the concepts associated to these mixed malformations in order to guide patient management. METHODS: A case of AVM associated with a DVA was identified and a literature review was performed according to PRISMA guidelines. CASE REPORT: In an 18-year-old man presenting with sub-acute headache but with a normal neurological examination, the MRI-scan showed a right occipital DVA associated with hemosiderin spots evocative of earlier asymptomatic bleedings. The Digital Subtraction Angiography revealed a right parieto-occipital Spetzler-Martin Grade III AVM, fed by branches from the right middle and posterior cerebral arteries, with a superficial drainage flowing into a DVA that then joined the superior sagittal sinus. Multistep embolization was performed, leading to a partial reduction of the nidus, but preserving the DVA permeability. After a six-year follow-up. bleeding did not recur and the MRI aspect of the malformation was perfectly stable. CONCLUSION: The co-occurrence of a DVA and an AVM is rare but has a higher bleeding risk than AVM alone (69% vs 38%) and must consequently be suspected when a DVA is revealed by a haemorrhage, in the absence of associated cavernoma. These mixed malformations represent a therapeutic challenge which has to be tailored to the venous anatomy and to the malformation Spetzler-Martin grade. DVA permeability should be preserved to avoid deleterious venous infarction.

How I do it: dorsolateral approach for ventrolateral intramedullary cavernoma.

BACKGROUND: For small and lateral lesions, in order to avoid postoperative sequelae related to dorso-median myelotomy, we propose to describe the use of a ventrolateral approach for intramedullary lesions. METHOD: Performing this approach entails that the denticulate ligament is cut from its dural attachment and retracted. Rotation of the spinal cord must be achieved with great caution and under electrophysiological monitoring. After pia mater incision, hydrodissection is useful to gently dissect the cavernoma and promote a cleavage plane. CONCLUSION: In the case of lateral intramedullary lesions, using this approach maximized the absence of postoperative deficit.

Could propranolol be beneficial in adult cerebral cavernous malformations?

Surgery is the only therapeutic option for cerebral cavernous malformations (CCM) and is proposed, whenever possible, after haemorrhagic events, neurological symptoms, or epilepsy, radiosurgery being a controversial alternative in some cases. However, there is no treatment for non-accessible lesions, such as brainstem CCM, multiple CCM, or those located in functional areas. Propranolol, a non-selective beta-blocker used as first-line treatment for infantile haemangiomas, has proved spectacularly effective in a few cases of adult patients with CCM. We herein review the histological, in vitro data and clinical findings that support the idea of propranolol as a potential treatment for CCM. Since one retrospective study has not been conclusive, we support the idea that prospective trials are necessary.

Insular gliomas: a surgical reappraisal based on a systematic review of the literature.

INTRODUCTION: Insular gliomas are heterogeneous lesions whose management presents multiple challenges for their tendency to affect young patients in good neurological and cognitive conditions, their deep anatomic location and proximity with critical functional and vascular structures. The appropriate management of insular gliomas requires a multidisciplinary evidence-centred teamwork grounded on the best anatomic, neurophysiological and oncological knowledge. The present study provides a reappraisal of the management of insular gliomas based on a systematic review of the literature with the aim of guiding clinicians in the management of such tumors. EVIDENCE ACQUISITION: A systematic review of the literature from the Medline, Embase and Cochrane Central databases was performed. From 2006 to 2016, all articles meeting specific inclusion criteria were included. EVIDENCE SYNTHESIS: The present work summarizes the most relevant evidence about insular gliomas management. The anatomy and physiology of the insula, the new WHO 2016 classification and clinico-radiological presentation of insular gliomas are reviewed. Surgical pearls of insular gliomas resection as well as oncologic and functional outcomes after insular gliomas treatment are discussed. CONCLUSIONS: Management of insular gliomas remains challenging despite improvement in surgical and oncological techniques. However, the literature review supports a growing evidence that recent developments in the multidisciplinary care account for constant improvements of survival and quality of life.

Biomechanical Characterization of Intracranial Aneurysm Wall: A Multiscale Study.

OBJECTIVE: Aneurysm wall biomechanics are not yet an integral part of aneurysm rupture risk evaluation. We aimed to develop a new technique describing the biomechanical properties of aneurysm wall and correlating them to rupture status. METHODS: Aneurysm wall samples collected during surgery were submitted before and after freezing to tensile tests or as fresh samples to indentation tests. The lateral stiffness or the Young's modulus of the different samples was determined as a function of the mechanical test used. The impact of freezing on biomechanical properties was evaluated. The correlation of clinical and radiologic data with the biomechanical profile of the aneurysm samples was investigated. Two-photon microscopy was used to study collagen fiber organization. RESULTS: Sixteen aneurysm samples (11 unruptured and 5 ruptured) were included. Freezing decreased tissue stiffness. No significant difference was found between ruptured and unruptured aneurysm wall samples regarding demographic characteristics, ethnicity, smoking status, arterial hypertension, site, size and shape of the aneurysm, PHASES score, mechanical profile, or overall Young's modulus. Indentation tests found that the rupture occurred in a restricted area of increased elastic capacity and unruptured areas had increased stiffness. Two-photon microscopy found disruption of the collagen fiber network in rupture zones. CONCLUSIONS: The indentation test of fresh aneurysm wall samples described the heterogeneity of biomechanical properties of the tissue and found increased elastic capacity in the rupture zone and increased stiffness in the remainder of the aneurysm. This study could be a basis for further research aimed at building a biomechanical-based model of aneurysm rupture risk.

Intracranial dural arteriovenous fistulas: a review of their current management based on emerging knowledge.

Intracranial dural arteriovenous fistulas are a rarely diagnosed type of vascular malformations, yet they are clinically relevant in a subspecialized neurovascular setting because a misdiagnosis may lead to permanent morbidity and mortality. Modern brain imaging techniques such as flat panel detector computed tomographic angiography and magnetic resonance imaging angiography have a significant role in the preoperative work-up, still digital subtraction angiography remains the neuroimaging mainstay. The most important factor guiding their management is the presence of cortical venous reflux, significantly associated with aggressive symptoms due to cerebral or spinal cord venous congestion and hemorrhage. Cutting-edge developments in endovascular and neurosurgical treatment of these vascular malformations, which should be undertaken in specialized referral centers, have substantially improved their prognosis. While transarterial or transvenous endovascular techniques represent frequently the therapy of choice, surgery remains a very valuable option both as a first line treatment and after partial embolization has been carried out. A significant neurosurgical advance is the introduction of indocyanine green video angiography, which allows precise identification of the arterialized draining vein of the dural fistula to be disconnected and confirms interruption of the arteriovenous shunt. Stereotactic radiosurgery may be considered in case of intracranial dural arteriovenous fistulas without cortical venous drainage for which surgical and endovascular options have been consumed or for palliation.

Pleomorphic pineocytoma associated with normal pineal parenchyma: report of a case in a 70-year-old man.

Pineocytomas (PCs) most frequently occur in adults, but only three cases have been reported in women older than 70 years. In PCs, cytologic pleomorphism, accompanied by ganglion cells intensely expressing neuronal markers, has been described and the presence of pleomorphic cells may lead to an erroneous upgrading of the tumor. We report an unusual case of pleomorphic pineocytoma in an older patient who presented with a slowly growing tumor adjacent to residual pineal gland. The immunohistological markers of the tumoral tissue and the remnant normal pineal tissue were evaluated and compared. In the neoplasm, the large number of cells labeled for neuronal markers, including many pleomorphic cells, confirmed previous findings that a neuronal immunophenotype is common in PC. Reactivity for synaptophysin was stronger in the tumor than the pineal gland, whereas neurofilament protein reactivity was stronger in the pineal gland than the tumor. The neoplastic cells, but not the pineal gland, were reactive for chromogranin A. This dense core vesicle-associated protein immunolabeling is an interesting diagnostic marker for PCs, which makes it possible to distinguish normal pineal parenchyma with low or negative expression from tumoral tissue. This case illustrates that, even though PCs are low-grade tumors, they can increase in size and surgery appears a valuable option.

Symptomatic Moyamoya disease: clinical features and outcome after indirect bypass surgery in four French adults.

BACKGROUND: There are a few reports of moyamoya disease (MMD) in the European Caucasian adult population. We present the clinical manifestations, the neuroradiological aspects, the treatment, and the outcome after surgical revascularization of four French patients with MMD. PATIENTS AND METHODS: We identified four adults (age >18 years; three women and one man) with MMD who underwent digital subtraction catheter angiography at our institution from 1997 through 2006. The median age at symptom onset was 35 years (range, 22 to 41 years). The initial clinical presentation was intracerebral hemorrhage in three patients and ischemic stroke in one patient. RESULTS: Three patients underwent bilateral surgical revascularization and one patient underwent unilateral surgical revascularization. All patients underwent the same surgical revascularization procedure (encephalo-duro-arterio-myo-synangiosis). The mean (+/-SD) period of follow-up after diagnosis of MMD was 6 years and 9 months (+/-3 years and 5 months). No patient experienced any recurrent hemorrhagic or ischemic stroke. No perioperative stroke occurred. No patient was severely disabled or unable to walk. Three patients out of four were employed. CONCLUSION: Our data suggest safety and a potential benefit of surgical revascularization (indirect bypass surgery) in European adult patients with symptomatic MMD. Further long-term prospective multicenter studies are needed. The establishment of a registry would be useful in order to accumulate data in large numbers of European patients with this uncommon disease.

[Comparative study for ruptured aneurysms treated by surgery and endovascular method]. / Estudo comparativo entre aneurismas rotos tratados por cirurgia e por via endovascular.

The management of intracranial aneurysms has truly evolved after the introduction of the endovascular treatment. In this paper we compare patients that were operated or embolized for intracranial aneurysms. Between 1995 and 1999, 78 grade I to III ruptured aneurysms were treated in our service: 52 patients were operated, 21 were embolized and 5 were submitted to combinated endovascular and surgical treatment. In the surgical group, clinical outcome was very good in 80.8% of cases with 5% of mortality with 96.2% of total exclusion of the aneurysm. In the endovascular group, 95% of cases the clinical outcome was very good with only 42.8% of total exclusion of the aneurysm. By the endovascular method for treatment of aneurysms, we can obtain a good clinical outcome but a poor radiological outcome and sometimes need a complementary surgical procedure to treat residual aneurysm.

Estudo comparativo entre aneurismas rotos tratados por cirurgia e por via endovascular / Comparative study for ruptured aneurysms treated by surgery and endovascular method

Realizamos análise comparativa dos resultados clínicos e radiológicos após o tratamento de aneurisma roto, localizado no setor anterior, por cirurgia ou por via endovascular. Entre 1995 e 1999, 78 pacientes foram tratados em nosso serviço por apresentarem ruptura de aneurisma intracraniano com grau clínico (Hunt & Hess) variando entre I e III. Dentre estes pacientes, 52 foram operados, 21 embolizados e 5 fizeram tratamento combinado. Dos casos cirúrgicos, o resultado clínico foi considerado ótimo em 80,8 por cento com mortalidade de 5 por cento e oclusäo completa do aneurisma em 96,2 por cento. Dos pacientes embolizados, 95 por cento dos casos o resultado foi considerado ótimo com oclusäo completa do aneurisma em 42,8 por cento. O tratamento endovascular de aneurismas intracranianos apresenta uma vantagem no resultado clínico em relaçäo aos aneurismas operados. Porém, apresentam taxa de exclusäo completa baixa e podem necessitar de tratamento complementar