Relationship of A1 segment hypoplasia to anterior communicating artery aneurysm morphology and risk factors for aneurysm formation

J Neurosurg 127:89–95, 2017

Hypoplasia of the A1 segment of the anterior cerebral artery is frequently observed in patients with anterior communicating artery (ACoA) aneurysms. The effect of this anatomical variant on ACoA aneurysm morphology is not well understood.

METHODS Digital subtraction angiography images were reviewed for 204 patients presenting to the authors’ institution with either a ruptured or an unruptured ACoA aneurysm. The ratio of the width of the larger A1 segment to the smaller A1 segment was calculated. Patients with an A1 ratio greater than 2 were categorized as having A1 segment hypoplasia. The relationship of A1 segment hypoplasia to both patient and aneurysm characteristics was then assessed.

RESULTS Of 204 patients that presented with an ACoA aneurysm, 34 (16.7%) were found to have a hypoplastic A1. Patients with A1 segment hypoplasia were less likely to have a history of smoking (44.1% vs 62.9%, p = 0.0410). ACoA aneurysms occurring in the setting of a hypoplastic A1 were also found to have a larger maximum diameter (mean 7.7 vs 6.0 mm, p = 0.0084). When considered as a continuous variable, increasing A1 ratio was associated with decreasing aneurysm dome-to-neck ratio (p = 0.0289). There was no significant difference in the prevalence of A1 segment hypoplasia between ruptured and unruptured aneurysms (18.9% vs 10.7%; p = 0.1605).

CONCLUSIONS Our results suggest that a hypoplastic A1 may affect the morphology of ACoA aneurysms. In addition, the relative lack of traditional risk factors for aneurysm formation in patients with A1 segment hypoplasia argues for the importance of hemodynamic factors in the formation of ACoA aneurysms in this anatomical setting.

 

Results of the ANSWER Trial Using the PulseRider for the Treatment of Broad-Necked, Bifurcation Aneurysms

Neurosurgery 81:56–65, 2017

The safety and probable benefit of the PulseRider (Pulsar Vascular, Los Gatos, California) for the treatment of broad-necked, bifurcation aneurysms was studied in the context of the prospective, nonrandomized, single arm clinical trial— the Adjunctive Neurovascular Support of Wide-neck aneurysm Embolization and Reconstruction (ANSWER) Trial.

OBJECTIVE: To present the results of the United States cases employing the PulseRider device as part of the ANSWER clinical trial.

METHODS: Aneurysms treated with the PulseRider device among sites enrolling in the ANSWER trial were prospectively studied and the results are summarized. Aneurysms arising at either the carotid terminus or basilar apex thatwere relatively broad neckedwere considered candidates for inclusion into the ANSWER study.

RESULTS: Thirty-four patients were enrolled (29 female and 5 male) with a mean age of 60.9 years (27 basilar apex and 7 carotid terminus).Mean aneurysm height ranged from 2.4 to 15.9 mm with a mean neck size of 5.2 mm (range 2.3-11.6 mm). In all patients, the device was delivered and deployed. Immediate Raymond I or II occlusion was achieved in 82.4% and progressed to 87.9% at 6-month follow-up. A modified Rankin Score of 2 or less was seen in 94% of patients at 6 months.

CONCLUSION: The results from the ANSWER trial demonstrate that the PulseRider device is safe and offers probable benefit as for the treatment of bifurcation aneurysms arising at the basilar apex or carotid terminus. As such, it represents a useful addition to the armamentarium of the neuroendovascular specialist.

Clinical Experience and Results of Microsurgical Resection of Arteriovenous Malformation in the Presence of Space-Occupying Intracerebral Hematoma

Neurosurgery 81:75–86, 2017

Management of ruptured arteriovenous malformations (AVMs) with a mass-producing intracerebral hematoma (ICH) represents a surgical dilemma.

OBJECTIVE: To evaluate the clinical outcome and obliteration rates of microsurgical resection of AVM when performed concomitantly with evacuation of an associated spaceoccupying ICH.

METHODS: Data of patients with AVMwere collected prospectively. Cases were identified in which an AVM was resected and an associated space-occupying ICH was evacuated at the same time, and divided into “group 1,” in which the surgery was performed acutely within 48 h of presentation (secondary to elevated intracranial pressure); and “group 2,” in which selected patients were operated upon in the presence of a liquefying ICH in the “subacute”stage. Clinical outcomes were assessed using themodified Rankin Scale, with a score of 0 to 2 considered a good outcome. Obliteration rateswere assessed using postoperative angiography.

RESULTS: From 2001 to 2015, 131 patients underwent microsurgical resection of an AVM, of which 65 cases were included. In “group 1” (n = 21; Spetzler-Ponce class A = 13, class B = 5, and class C = 3), 11 of 21 (52%) had a good outcome and in 18 of 19 (95%) of those who had a postoperative angiogramthe AVMswere completely obliterated. In “group 2”(n=44; Spetzler-Ponce class A=33, class B=9, and class C=2), 31 of 44 (93%) had a good outcome and 42 of 44 (95%) were obliterated with a single procedure. For supratentorial AVMs, the ICH cavity was utilized to provide an operative trajectory to a deep AVM in 11 cases, and in 26 cases the ICH cavity was deep to the AVM and hence facilitated the deep dissection of the nidus.

CONCLUSION: In selected patients the presence of a liquefying ICH cavity may facilitate the resection of AVMs when performed in the subacute stage resulting in a good neurological outcome and high obliteration rate.

Preoperative planning of hemangioblastoma using 3D imaging

 

J Neurosurg 127:139–147, 2017

Successful resection of hemangioblastoma depends on preoperative assessment of the precise locations of feeding arteries and draining veins. Simultaneous 3D visualization of feeding arteries, draining veins, and surrounding structures is needed.

The present study evaluated the usefulness of high-resolution 3D multifusion medical imaging (hr-3DMMI) for preoperative planning of hemangioblastoma. The hr-3DMMI combined MRI, MR angiography, thin-slice CT, and 3D rotated angiography. Surface rendering was mainly used for the creation of hr-3DMMI using multiple thresholds to create 3D models, and processing took approximately 3–5 hours. This hr-3DMMI technique was used in 5 patients for preoperative planning and the imaging findings were compared with the operative findings. Hr-3DMMI could simulate the whole 3D tumor as a unique sphere and show the precise penetration points of both feeding arteries and draining veins with the same spatial relationships as the original tumor.

All feeding arteries and draining veins were found intraoperatively at the same position as estimated preoperatively, and were occluded as planned preoperatively. This hr-3DMMI technique could demonstrate the precise locations of feeding arteries and draining veins preoperatively and estimate the appropriate route for resection of the tumor. Hr-3DMMI is expected to be a very useful support tool for surgery of hemangioblastoma.

Impact of Weekend Presentation on Short-Term Outcomes and Choice of Clipping vs Coiling in Subarachnoid Hemorrhage

Neurosurgery 81:87–91, 2017

Presentation on a weekend is commonly associated with higher mortality and a decreased likelihood of receiving invasive procedures.

OBJECTIVE: To determine whether weekend presentation influences mortality, discharge destination, or type of treatment received (clip vs coil) in subarachnoid hemorrhage (SAH).

METHODS: We performed a serial cross-sectional retrospective study using the Nationwide Inpatient Sample. All adult discharges with a primary diagnosis of SAH (ICD-9-CM 435) from 2005 to 2010 were included, and records with trauma or arteriovenous malformation were excluded. Unadjusted and adjusted associations between weekend presentation and 3 outcomes (in-hospital mortality, discharge destination, and treatment with clip vs coil) were estimated using chi-square tests and multilevel logistic regression.

RESULTS: A total of 46 093 admissions for nontraumatic SAH were included in the sample; 24.6% presented on a weekend, 68.9% on a weekday, and 6.5% had unknown day of presentation. Weekend admission was not a significant predictor of inpatient mortality (25.4% weekend vs 24.9% weekday; P = .44), or a combined poor outcome measure of mortality or discharge to long-term acute care or hospice (30.3% weekend vs 29.4% weekday; P = .23). Among those treated for aneurysm obliteration, the proportion of clipped vs coiled did not change with weekend vs weekday presentation (21.5% clipped with weekend presentation vs 21.6% weekday, P = .95; 21.5% coiled with weekend presentation vs 22.4% weekday, P = .19).

CONCLUSION: Presentation with nontraumatic SAH on a weekend did not influence mortality, discharge destination, or type of treatment received (clip vs coil) compared with weekday presentation.

 

Transdural arterial recruitment to brain arteriovenous malformation

J Neurosurg 127:51–58, 2017

The occurrence of transdural arterial recruitment (TDAR) in association with brain arteriovenous malformation (bAVM) is uncommon, and the reason for TDAR is not understood. The aim of this cohort study was to examine patient and bAVM characteristics associated with TDAR and the implications of TDAR on management.

METHODS A prospective surgical database of bAVMs was examined. Cases previously treated elsewhere or incompletely examined by digital subtraction angiography (DSA) assessment were excluded. Three studies of this cohort were performed, as follows: characteristics associated with TDAR, the relationship between TDAR and neurological deficits unassociated with hemorrhage (NDUH), and the impact of TDAR on outcome from surgery. Regression models were performed.

RESULTS Of 769 patients with complete DSA who had no previous treatment, 51 (6.6%) were found to have TDAR. The presence of TDAR was associated with increasing age (p < 0.01; OR 1.05; 95% CI 1.02–1.07); presentation with NDUH (p < 0.01; OR 2.71; 95% CI 1.29–5.71); increasing size of the bAVM (p < 0.01; OR 1.57; 95% CI 1.29–1.91); and combined supply from both anterior and posterior circulations (p = 0.02; OR 2.37; 95% CI 1.17–4.78). Further analysis of TDAR cases comparing those with and without NDUH found an association of larger size (6.6 cm [2.9 SD] compared with 4.7 cm [1.8 SD]; p < 0.01) and combined supply from both anterior and posterior circulations (relative risk 2.5; 95% CI 1.0–6.2; p = 0.04) to be associated with an NDUH presentation. For the 632 patients undergoing surgery there was an increased risk of complications (where this produced a new permanent neurological deficit at 12 months represented by a modified Rankin Scale score of > 1) with the following variables: size; location in eloquent brain; deep venous drainage; increasing age; and no presentation with hemorrhage. The presence of TDAR was not associated with an increased risk of complications from surgery.

CONCLUSIONS The authors found that TDAR occurs in older patients with larger bAVMs, and that TDAR is also more likely to be associated with bAVMs presenting with NDUH. The likely explanation for the presence of TDAR is a secondary recruitment arising as a consequence of shear stress, rather than a primary vascular supply present from the earliest development of the bAVM.

Bilateral chronic subdural hematoma: unilateral or bilateral drainage?

J Neurosurg 126:1905–1911, 2017

Bilateral chronic subdural hematoma (bCSDH) is a common neurosurgical condition frequently associated with the need for retreatment. The reason for the high rate of retreatment has not been thoroughly investigated. Thus, the authors focused on determining which independent predictors are associated with the retreatment of bCSDH with a focus on surgical laterality.

METHODS In a national database of CSDHs (Danish Chronic Subdural Hematoma Study) the authors retrospectively identified all bCSDHs treated in the 4 Danish neurosurgical departments over the 3-year period from 2010 to 2012. Univariate and multivariate analyses were performed to determine the relationship between retreatment of bCSDH and clinical, radiological, and surgical variables.

RESULTS Two hundred ninety-one patients with bCSDH were identified, and 264 of them underwent unilateral (136 patients) or bilateral (128 patients) surgery. The overall retreatment rate was 21.6% (57 of 264 patients). Cases treated with unilateral surgery had twice the risk of retreatment compared with cases undergoing bilateral surgery (28.7% vs 14.1%, respectively, p = 0.002). In accordance with previous studies, the data also showed that a separated hematoma density and the absence of postoperative drainage were independent predictors of retreatment.

CONCLUSIONS In bCSDHs bilateral surgical intervention significantly lowers the risk of retreatment compared with unilateral intervention and should be considered when choosing a surgical procedure.

 

Radiosurgery for Unruptured Brain Arteriovenous Malformations: An International Multicenter Retrospective Cohort Study

Neurosurgery 80:888–898, 2017

The role of intervention in the management of unruptured brain arteriovenous malformations (AVM) is controversial.

OBJECTIVE: To analyze in a multicenter, retrospective cohort study, the outcomes following radiosurgery for unruptured AVMs and determine predictive factors.

METHODS: We evaluated and pooled AVM radiosurgery data from 8 institutions participating in the International Gamma Knife Research Foundation. Patients with unruptured AVMs and ≥12 mo of follow-up were included in the study cohort. Favorable outcome was defined as AVM obliteration, no postradiosurgical hemorrhage, and no permanently symptomatic radiation-induced changes.

RESULTS: The unruptured AVM cohort comprised 938 patients with a median age of 35 yr. The median nidus volume was 2.4 cm3, 71% of AVMs were located in eloquent brain areas, and the Spetzler-Martin grade was III or higher in 57%. The median radiosurgical margin dose was 21 Gy and follow-up was 71 mo. AVM obliteration was achieved in 65%. The annual postradiosurgery hemorrhage rate was 1.4%. Symptomatic and permanent radiation-induced changes occurred in 9% and 3%, respectively. Favorable outcome was achieved in 61%. In the multivariate logistic regression analysis, smaller AVM maximum diameter (P = .001), the absence of AVM-associated arterial aneurysms (P = .001), and higher margin dose (P = .002) were found to be independent predictors of a favorable outcome. A margin dose ≥ 20 Gy yielded a significantly higher rate of favorable outcome (70% vs 36%; P < .001)

CONCLUSION: Radiosurgery affords an acceptable risk to benefit profile for patients harboring unruptured AVMs. These findings justify further prospective studies comparing radiosurgical intervention to conservative management for unruptured AVMs.

 

The Superior Cerebellar Artery Aneurysm: A Posterior Circulation Aneurysm with Favorable Microsurgical Outcomes

Neurosurgery 80:908–916, 2017

Superior cerebellar artery (SCA) aneurysms are usually grouped with aneurysms that arise from the upper basilar artery or more broadly, the posterior circulation. However, the SCA aneurysm has distinctive anatomy that facilitates safe surgical management, notably few associated perforating arteries, and excellent exposure in the carotid-oculomotor triangle.

OBJECTIVE: To demonstrate the outcomes of patients treated with microsurgery in a continuous surgical series.

METHODS: Sixty-two patients harboring 63 SCA aneurysmswere retrospectively reviewed from a prospectively maintained database, focusing on clinical characteristics, surgical techniques, and clinical outcomes.

RESULTS: Of 31 patients (49%) presenting with subarachnoid hemorrhage, the SCA aneurysm was the source in 16 (25%). Thirty-three aneurysms were complex (52%) and 43 patients (59%) had multiple aneurysms. Fifty-seven SCA aneurysms (90.5%) were clipped and 5 were bypassed/trapped or wrapped. Complete angiographic occlusion was achieved in 91.7%. Permanent neurological morbidity occurred in 3 patients and 3 patients that presented in coma after subarachnoid hemorrhage died. All patients with “simple” aneurysms and without subarachnoid hemorrhage had improved or unchangedmodified Rankin scale scores. Overall, outcomes were stable or improved in 82.5% of patients.

CONCLUSION: SCA aneurysms are favorable for microsurgical clipping with low rates of permanent morbidity and mortality. Microsurgery should be considered alongside endovascular techniques as a treatment option in many patients.

Comparison Between CTA and Digital Subtraction Angiography in the Diagnosis of Ruptured Aneurysms

Computerized tomography angiography (CTA) is commonly used to diagnose ruptured cerebral aneurysms with sensitivities reported as high as 97% to 100%. Studies validating CTA accuracy in the setting of subarachnoid hemorrhage (SAH) are scarce and limited by small sample sizes.

OBJECTIVE: To evaluate the diagnostic accuracy of CTA in detecting intracranial aneurysms in the setting of SAH.

METHODS: A single-center, retrospective cohort of 643 patients was reviewed. A total of 401 patients were identified whose diagnostic workup included both CTA and confirmatory digital subtraction angiography (DSA). Aneurysms missed by CTA but diagnosed by DSA were further stratified by size and location.

RESULTS: Three hundred and thirty aneurysms were detected by CTA while DSA detected a total of 431 aneurysms. False positive CTA results were seen for 24 aneurysms. DSA identified 125 aneurysms that were missed by CTA and 83.2% of those were <5 mm in diameter. The sensitivity of CTA was 57.6% for aneurysms smaller than 5 mm in size, and 45% for aneurysms originating from the internal carotid artery. The overall sensitivity of CTA in the setting of SAH was 70.7%.

CONCLUSION: The accuracy of CTA in the diagnosis of ruptured intracranial aneurysm may be lower than previously reported. CTA has a low sensitivity for aneurysms less than 5 mm in size, in locations adjacent to bony structures, and for those arising from small caliber parent vessels. It is our recommendation that CTA should be used with caution when used alone in the diagnosis of ruptured intracranial aneurysms.

Cerebral Revascularization for Aneurysms in the Flow-Diverter Era

Neurosurgery 80:759–768, 2017

Cerebral bypass has been an important tool in the treatment of complex intracranial aneurysms. The recent advent of flow-diverting stents (FDS) has expanded the capacity for endovascular arterial reconstruction.

OBJECTIVE: We investigated how the advent of FDS has impacted the application and outcomes of cerebral bypass in the treatment of intracranial aneurysms.

METHODS: We reviewed a consecutive series of cerebral bypasses during aneurysm surgery over the course of 10 years. FDS were in active use during the last 5 years of this series. We compared the clinical characteristics, surgical technique, and outcomes of patients who required cerebral bypass for aneurysm treatment during the preflow diversion era (PreFD) with those of the postflow diversion era (PostFD).

RESULTS: We treated 1061 aneurysms in the PreFD era (from July 2005 through June 2010) and 1348 in the PostFD era (from July 2010 through June 2015). Eighty-five PreFD patients (8%) and 45 PostFD patients (3%) were treated with cerebral bypass. PreFD patients had better baseline functional status compared to PostFD patients with average preoperative modified Rankin Scale score of 0.55 in PreFDand 1.18 in PostFD.

CONCLUSION: After the introduction of FDS, cerebral bypass was performed in a lower proportion of patients with aneurysms. Patients selected for bypass in the flow-diverter era had worse preoperative modified Rankin Scale scores indicating a greater complexity of the patients. Cerebral bypass in well-selected patients and revascularization remains an important technique in vascular neurosurgery. It is also useful as a rescue technique after failed FDS treatment of aneurysms.

Is there an inherited anatomical conformation favoring aneurysmal formation of the anterior communicating artery?

J Neurosurg 126:1598–1605, 2017

The pathophysiological mechanisms responsible for the formation of intracranial aneurysms (IAs) remain only partially elucidated. However, current evidence suggests a genetic component. The purpose of this study was to investigate the specific anatomical variations in the arterial complex that are associated with the presence of anterior communicating artery (ACoA) aneurysms in the familial forms of IAs.

METHODS This multicenter study investigated bifurcation IAs in patients who had a sporadic ACoA IA without a family history of IA (SACAA group), in patients who had an ACoA IA with a family history of IA (FACAA group), and in their healthy first-degree relatives (HFDRs). Through the use of MR angiography (MRA) reconstructions, the symmetry of the A1 segments and the angle between the A1 and A2 segments were analyzed on 3D models for each group. These measurements were then compared among the 3 groups.

RESULTS Twenty-four patients with SACAA, 24 patients with FACAA, and 20 HFDRs were included in the study. Asymmetrical configuration of the A1 segments was more frequent in the FACAA group than in the HFDR group (p = 0.002). The aneurysm-side A1-A2 angle was lower in the FACAA group (p = 0.003) and SACAA group (p = 0.007) than in the HFDR group. On the contralateral side, there was no difference in A1-A2 angles between groups.

CONCLUSIONS The anatomical shape of the ACoA complex seems to be similarly associated with the presence of ACoA IAs in both the FACAA and SACAA groups. This highlights the role played by hemodynamic constraints in aneurysm formation and questions the hypothesis of the hereditary character of these anatomical shapes.

Surgical treatment of anterior cranial fossa dural arterio-venous fistulas (DAVFs)

Acta Neurochir (2017) 159:823–830

Anterior cranial fossa dural arterio-venous fistulas (DAVFs) represent 6% of all intracranial DAVFs; characteristically they show an aggressive behaviour with high risk of intracranial haemorrhage. Peculiar anatomical features, such as feeding by the ethmoidal arteries and the pattern of venous drainage (frequently with varices that mimic aneurysmal dilatation), can be evaluated in detail only by digital subtraction angiography (DSA), which represents the Bgold standard^ in the diagnosis of such cranial fistulas. Recent technological developments in endovascular management of this type of DAVF have partially reduced the morbidity risk related to this modality of treatment. Our purpose is to present our experience in the surgical management of 14 patients with anterior cranial fossa DAVFs, with attention paid to the possible role of preoperative embolisation in these cases and to the surgical technique.

Method Between 1999 and 2015, 14 patients with anterior cranial fossa DAVFs were submitted to surgery in two neurosurgical departments; the mean age was 63 years old; nine DAVFs caused intracranial haemorrhage (subarachnoid haemorrhage in three cases, intracerebral haemorrhage in six cases). Pre-operative embolisation was attempted in an early case and was successfully done in one recent case. In all patients, the surgical approach chosen was a pterional craniotomy with a lowmargin on the frontal bone in order to gain the exposure of the anterior cranial fossa and especially of the olphactory groove region; the resection of the falx at its insertion on the crista galli was needed in five cases in order to get access to the contralateral afferent vessels. Cauterisation of all the dural feeders on and around the lamina cribrosa was needed in all cases; venous dilatations were evident in eight patients (in seven out of nine patients with ruptured DAVF and in one out of five patients with unrupturedDAVF) andwere removed in all cases. One patient harboured an ophthalmic artery aneurysm, which was excluded by clipping.

Results One patient died 5 days after surgery due to the severity of the pre-operative haemorrhage. Postoperative DSA showed the disappearance of the DAVF and of the venous pseudo-aneurysms in all cases. Clinical outcome was favourable (without neurological deficits) in 11 patients; three patients presented an unfavourable clinical outcome, due to the severity of the initial haemorrhage.

Conclusions Surgical exclusion of the anterior cranial fossa DAVFs still represents the gold standard for such lesions, due to low post-operative morbidity and to complete protection against future rebleedings; endovascular techniques may help the surgeon in complex cases.

Guidelines for the Clinical Management of Cerebral Cavernous Malformations

Neurosurgery 80:665–680, 2017

Despite many publications about cerebral cavernous malformations (CCMs), controversy remains regarding diagnostic and management strategies.

OBJECTIVE: To develop guidelines for CCM management.

METHODS: The Angioma Alliance (www.angioma.org), the patient support group in the United States advocating on behalf of patients and research in CCM, convened a multidisciplinary writing group comprising expert CCM clinicians to help summarize the existing literature related to the clinical care of CCM, focusing on 5 topics: (1) epidemiology and natural history, (2) genetic testing and counseling, (3) diagnostic criteria and radiology standards, (4) neurosurgical considerations, and (5) neurological considerations. The group reviewed literature, rated evidence, developed recommendations, and established consensus, controversies, and knowledge gaps according to a prespecified protocol.

RESULTS: Of 1270 publications published between January 1, 1983 and September 31, 2014, we selected 98 based on methodological criteria, and identified 38 additional recent or relevant publications. Topic authors used these publications to summarize current knowledge and arrive at 23 consensus management recommendations, which we rated by class (size of effect) and level (estimate of certainty) according to the American Heart Association/American Stroke Association criteria. No recommendation was level A (because of the absence of randomized controlled trials), 11 (48%)were level B, and 12 (52%) were level C. Recommendations were class I in 8 (35%), class II in 10 (43%), and class III in 5 (22%).

CONCLUSION: Current evidence supports recommendations for the management of CCM, but their generally low levels and classes mandate further research to better inform clinical practice and update these recommendations. The complete recommendations document, including the criteria for selecting reference citations, a more detailed justification of the respective recommendations, and a summary of controversies and knowledge gaps, was similarly peer reviewed and is available on line www.angioma.org/CCMGuidelines.

Reappearance of arteriovenous malformations after complete resection of ruptured arteriovenous malformations

J Neurosurg 126:1088–1093, 2017

Ruptured arteriovenous malformations (AVMs) are often obliterated after emergency microsurgical treatment. However, some studies have reported AVM recurrence after the obliteration of ruptured AVMs. The authors report their experience with AVM recurrence after successful microsurgical treatment of ruptured AVMs.

METHODS The authors reviewed the medical data of 139 consecutive patients who underwent microsurgery at the authors’ institution for ruptured AVM between 2002 and 2012. Each patient underwent a conventional cerebral angiography examination immediately after the surgery. Subsequent follow-ups were performed with MR angiography after 6 months, and, if there was no indication of AVM recurrence, patients were followed up with conventional cerebral angiography between 1 and 2 years after the treatment; pediatric patients were followed up until age 18 years. Recurrence was defined as new radiological evidence of an AVM at the site of a ruptured AVM or a new hemorrhage in patients with angiographically documented AVM obliteration on postoperative angiograms.

RESULTS The mean age of the patients at the time of ruptured AVM diagnosis was 30.8 years (SD ± 5, range 4–69 years), and 44 of the patients were younger than 18 years (the mean age at diagnosis in this pediatric subgroup was 11.4 years [range 4–17.9 years]). Complete AVM obliteration after the initial microsurgery was observed in 123 patients (89.5%). Reappearance of an AVM was noted in 7 patients between 12 and 42 months after the treatment, and all of these patients were younger than 18 years. The recurrent AVM was located in an eloquent zone in 4 patients, and deep venous drainage was noted in 3 patients. Radiosurgery was performed in 6 of these patients, and 1 patient underwent another microsurgical procedure. The authors noted only one rebleeding due to an AVM recurrence during the latency period after radiosurgery.

CONCLUSIONS The recurrence of an AVM is fairly rare and affects mostly pediatric patients. Therefore, especially in children, long-term angiographic follow-up is required to detect AVM recurrence or an AVM remnant. The authors stress the need for discussion involving a multidisciplinary neurosurgical team to decide on treatment in cases of any AVM recurrence or remnant.

Early diffusion-weighted MRI lesions after treatment of unruptured intracranial aneurysms:

J Neurosurg 126:1070–1078, 2017

Diffusion-weighted MRI was used to assess periprocedural lesion load after repair of unruptured intracranial aneurysms (UIA) by microsurgical clipping (MC) and endovascular coiling (EC).

METHODS Patients with UIA were assigned to undergo MC or EC according to interdisciplinary consensus and underwent diffusion-weighted imaging (DWI) 1 day before and 1 day after aneurysm treatment. Newly detected lesions by DWI after treatment were the primary end point of this prospective study. Lesions detected by DWI were categorized as follows: A) 1–3 DWI spots < 10 mm, B) > 3 DWI spots < 10 mm, C) single DWI lesion > 10 mm, or D) DWI lesion related to surgical access.

RESULTS Between 2010 and 2014, 99 cases were included. Sixty-two UIA were treated by MC and 37 by EC. There were no significant differences between groups in age, sex, aneurysm size, occurrence of multiple aneurysms in 1 patient, or presence of lesions detected by DWI before treatment. Aneurysms treated by EC were significantly more often located in the posterior circulation (p < 0.001). Diffusion-weighted MRI detected new lesions in 27 (43.5%) and 20 (54.1%) patients after MC and EC, respectively (not significant). The pattern of lesions detected by DWI varied significantly between groups (p < 0.001). Microembolic lesions (A and B) found on DWI were detected more frequently after EC (A, 14 cases; B, 5 cases) than after MC (A, 5 cases), whereas C and D were rare after EC (C, 1 case) and occurred more often after MC (C, 12 cases and D, 10 cases). No procedure-related unfavorable outcomes were detected.

CONCLUSIONS According to the specific techniques, lesion patterns differ between MC and EC, whereas the frequency of new lesions found on DWI is similar after occlusion of UIA. In general, the lesion load was low in both groups, and lesions were clinically silent. Clinical trial registration no.: NCT01490463 (clinicaltrials.gov)

Pathophysiology of Intracranial Aneurysm: The ICAN Project

Neurosurgery 80:621–626, 2017

Understanding the pathophysiologic mechanism of intracranial aneurysm
(IA) formation is a prerequisite to assess the potential risk of rupture. Nowadays, there are neither reliable biomarkers nor diagnostic tools to predict the formation or the evolution of IA. Increasing evidence suggests a genetic component of IA but genetics studies have failed to identify genetic variation causally related to IA.

OBJECTIVE: To develop diagnostic and predictive tools for the risk of IA formation and rupture.

METHODS: The French ICAN project is a noninterventional nationwide and multicentric research program. Each typical IA of bifurcation will be included. For familial forms, further IA screening will be applied among first-degree relatives. By accurate phenotype description with high-throughput genetic screening, we aim to identify new genes involved in IA. These potential genetic markers will be tested in large groups of patients. Any relevant pathway identified will be further explored in a large cohort of sporadic carriers of IA, which will be well documented with clinical, biological, and imaging data.

EXPECTED OUTCOMES: Discovering genetic risk factors, better understanding the pathophysiology, and identifying molecular mechanisms responsible for IA formation will be essential bases for the development of biomarkers and identification of therapeutic targets.

DISCUSSION: Our protocol has many assets. A nationwide recruitment allows for the inclusion of large pedigrees with familial forms of IA. It will combine accurate phenotyping and comprehensive imaging with high-throughput genetic screening. Last, it will enable exploiting metadata to explore new pathophysiological pathways of interest by crossing clinical, genetic, biological, and imaging information.

Hemorrhage from cerebral cavernous malformations

J Neurosurg 126:1079–1087, 2017

The aim of this paper is to define an overall cavernous malformation (CM) hemorrhage rate and risk factors for hemorrhage.

METHODS The authors performed a systematic, pooled analysis via the PubMed database through October 2015 using the terms “cavernoma,” “cavernous malformation,” “natural history,” “bleeding,” and “hemorrhage.” English-language studies providing annual rates and/or risk factors for CM hemorrhage were included. Data extraction, performed independently by the authors, included demographic data, hemorrhage rates, and hemorrhage risk factors.

RESULTS Across 12 natural history studies with 1610 patients, the mean age at presentation was 42.7 years old and 52% of patients (95% CI 49%–55%) were female. Presentation modality was seizure in 30% (95% CI 25%–35%), hemorrhage in 26% (95% CI 17%–37%), incidental in 17% (95% CI 9%–31%), and focal deficits only in 16% of cases (95% CI 11%–23%). CM location was lobar in 66% (95% CI 61%–70%), brainstem in 18% (95% CI 13%–24%), deep supratentorial in 8% (95% CI 6%–10%), and cerebellar in 8% (95% CI 5%–11%). Pooling 7 studies that did not assume CM presence since birth, the annual hemorrhage rate was 2.5% per patient-year over 5081.2 patient-years of follow-up (95% CI 1.3%–5.1%). Pooling hazard ratios across 5 studies that evaluated hemorrhage risk factors, prior CM hemorrhage was a significant risk factor for hemorrhage (HR 3.73, 95% CI 1.26–11.1; p = 0.02) while younger age, female sex, deep location, size, multiplicity, and associated developmental venous anomalies (DVAs) were not.

CONCLUSIONS Although limited by the heterogeneity of incorporated reports and selection bias, this study found prior hemorrhage to be a significant risk factor for CM bleeding, while age, sex, CM location, size, multiplicity, and associated DVAs were not. Future natural history studies should compound annual hemorrhage rate with prospective seizure and nonhemorrhagic neurological deficit rates.

Volume-Staged Stereotactic Radiosurgery for Intracranial Arteriovenous Malformations

Neurosurgery 80:543–550, 2017

Radiation-based treatment options of large intracranial arteriovenous malformations (AVM) must balance the likelihood of obliteration with the risk of adverse radiation effects (ARE).
OBJECTIVE: To analyze the efficacy and risks of volume-staged stereotactic radiosurgery (VS-SRS) for AVM.

METHODS: Retrospective study of 34 AVM patients having VS-SRS between 1997 and 2012. A median of 2 stages (range, 2-4) was used to treat a median AVM volume of 22.2 cm3 (range, 7.4-56.7). The median AVM margin dose was 16 Gy (range, 14-18); the median radiosurgery- based AVM score was 2.81 (range, 1.54-6.45). The median follow-up after VS-SRS was 8.2 years (range, 3-13.3).

RESULTS: Nidus obliteration was noted in 18 patients (53%) after VS-SRS. The rate of obliteration was 14% at 3 years, 54% at 5 years, and 75% at 7 years. Six patients (18%) had 11 bleeds after VS-SRS. Two patients (6%) remained neurologically stable, 2 (6%) patients had significant deficits, and 2 patients (6%) died. The actuarial risk of a first bleed after VS-SRS was 6% at 1 year, 12% at 3 years, and 19% at 7 years. Six patients (18%) underwent repeat SRS; all achieved nidus obliteration for an overall cure rate of 71%. Two patients (6%) had a permanent ARE after VS-SRS or repeat SRS.

CONCLUSION: VS-SRS permitted large volume intracranial AVM to be treated with a low rate of ARE. Further study is needed on dose escalation and decreasing the treatment volume per stage to determine if this will increase the rate of obliteration with this technique.

Microsurgery for cerebral arteriovenous malformations: subgroup outcomes in a consecutive series of 288 cases

J Neurosurg 126:1056–1063, 2017

The objective of this study was to review the outcomes after microsurgical resection of cerebral arteriovenous malformations (AVMs) from a consecutive single-surgeon series. Clinical and imaging data were analyzed to address the following questions concerning AVM treatment in the post-ARUBA (A Randomized Trial of Unruptured Brain Arteriovenous Malformations) era. 1) Are the patients who present with unruptured or ruptured AVMs doing better at long-term follow-up? 2) Is the differentiation between Ponce Class A (Spetzler-Martin Grade I and II) patients versus Ponce Class B and C patients (Spetzler-Martin Grade III and IV) meaningful and applicable to surgical practice? 3) How did the ARUBA-eligible patients of this surgical series compare with the results reported in ARUBA?

METHODS Two hundred eighty-eight patients with cerebral AVMs underwent microsurgical resection between 1983 and 2012 performed by the same surgeon (J.S.). This is a prospective case collection study that represents a consecutive series. The results are based on prospectively collected, early-outcome data that were supplemented by retrospectively collected, follow-up data for 94% of those cases. The analyzed data included the initial presentation, Spetzler-Martin grade, obliteration rates, surgical and neurological complications, and frequency of pretreatment with embolization or radiosurgery. The total cohort was compared using “small-AVM,” Spetzler-Martin Grade I and II, and ARUBA-eligible AVM subgroups.

RESULTS The initial presentation was hemorrhage in 50.0% and seizures in 43.1% of patients. The series included 53 Spetzler-Martin Grade I (18.4%), 114 Spetzler-Martin Grade II (39.6%), 90 Spetzler-Martin Grade III (31.3%), 28 Spetzler- Martin Grade IV (9.7%), and 3 Spetzler-Martin Grade V (1.0%) AVMs. There were 144 unruptured and 104 ARUBAeligible cases. Preembolization was used in 39 cases (13.5%). The occlusion rates for the total series and small AVM subgroup were 99% and 98.7%, respectively. The mean follow-up duration was 64 months. Early neurological deterioration was seen in 39.2% of patients, of which 12.2% had permanent and 5.6% had permanent significant deficits, and the mortality rate was 1.7% (n = 5). Outcome was better for patients with AVMs smaller than 3 cm (permanent deficit in 7.8% and permanent significant deficit in 3.2% of patients) and Ponce Class A status (permanent deficit in 7.8% and significant deficit in 3.2% of patients). Unruptured AVMs showed slightly higher new deficit rates (but 0 instances of mortality) among all cases, and in the small AVM and Ponce Class A subgroups. Unruptured Spetzler-Martin Grade I and II lesions had the best outcome (1.8% permanent significant deficit), and ARUBA-eligible Spetzler-Martin Grade I and II lesions had a slightly higher rate of permanent significant deficits (3.2%).

CONCLUSIONS Microsurgery has a very high cure rate. Focusing microsurgical AVM resection on unruptured lesions smaller than 3 cm or on Spetzler-Martin Grade I and II lesions is a good strategy for minimizing long-term morbidity. Well-selected microsurgical cases lead to better outcomes than with multimodal interventions, as in the ARUBA treatment arm, or conservative treatment alone. Long-term prospective data collection is valuable.