Neuroendovascular Procedures in Patients with Ehlers-Danlos Type IV: Multicenter Case Series and Systematic Review.

BACKGROUND: Ehlers-Danlos type IV or vascular Ehlers-Danlos syndrome (vEDS) is a rare inherited disorder characterized by profound vascular fragility resulting from defective production of type III procollagen. Cerebrovascular diseases including spontaneous dissections, cerebral aneurysms, and cavernous carotid fistulae are common. Endovascular therapies in this patient population are known to be higher risk, although many studies (before 2000) involved older techniques and equipment. The purpose of this study is to investigate the safety and efficacy of modern neuroendovascular techniques in the treatment of cerebrovascular diseases in patients with vEDS. METHODS: We combined a multi-institutional retrospective case series at 3 quaternary-care centers with a systematic literature review of individual case reports and case series spanning 2000-2021 to evaluate the safety and efficacy of neuroendovascular procedure in patients with vEDS with cerebrovascular diseases. RESULTS: Fifty-nine patients who underwent 66 neuroendovascular procedures were evaluated. Most of the patients had direct cavernous carotid fistulas (DCCF). Neuroendovascular procedures had a 94% success rate, with a complication rate of 30% and a mortality of 7.5%. CONCLUSIONS: Neuroendovascular procedures can be performed with a high rate of success in the treatment of cerebrovascular diseases in patients with vEDS, although special care is required because complication rates and mortality are high. Access site and procedure-related vascular injuries remain a significant hurdle in treating vEDS with cerebrovascular diseases, even with modern techniques.

Unifying theory of carotid plaque disruption based on structural phenotypes and forces expressed at the lumen/wall interface.

OBJECTIVES: To integrate morphological, haemodynamic and mechanical analysis of carotid atheroma driving plaque disruption. MATERIALS AND METHODS: First, we analysed the phenotypes of carotid endarterectomy specimens in a photographic dataset A, and matched them with the likelihood of preoperative stroke. Second, laser angioscopy was used to further define the phenotypes in intact specimens (dataset B) and benchmark with histology. Third, representative vascular geometries for each structural phenotype were analysed with Computational Fluid Dynamics (CFD), and the mechanical strength of the complicated atheroma to resist penetrating forces was quantified (n=14). RESULTS: In dataset A (n=345), ulceration (fibrous cap disruption) was observed in 82% of all plaques, intraplaque haemorrhage in 68% (93% subjacent to an ulcer) and false luminal formation in 48%. At least one of these 'rupture' phenotypes was found in 97% of symptomatic patients (n=69) compared with 61% in asymptomatic patients. In dataset B (n=30), laser angioscopy redemonstrated the structural phenotypes with near-perfect agreement with histology. In CFD, haemodynamic stress showed a large pulse magnitude, highest upstream to the point of maximal stenosis and on ulceration the inflow stream excavates the necrotic core cranially and then recirculates into the true lumen. Based on mechanical testing (n=14), the necrotic core is mechanically weak and penetrated by the blood on fibrous cap disruption. CONCLUSIONS: Fibrous cap ulceration, plaque haemorrhage and excavation are sequential phenotypes of plaque disruption resulting from the chiselling effect of haemodynamic forces over unmatched mechanical tissue strength. This chain of events may result in thromboembolic events independently of the degree of stenosis.

Rapid Automated Analysis of Skull Base Tumor Specimens Using Intraoperative Optical Imaging and Artificial Intelligence.

BACKGROUND: Accurate specimen analysis of skull base tumors is essential for providing personalized surgical treatment strategies. Intraoperative specimen interpretation can be challenging because of the wide range of skull base pathologies and lack of intraoperative pathology resources. OBJECTIVE: To develop an independent and parallel intraoperative workflow that can provide rapid and accurate skull base tumor specimen analysis using label-free optical imaging and artificial intelligence. METHODS: We used a fiber laser-based, label-free, nonconsumptive, high-resolution microscopy method (<60 seconds per 1 × 1 mm2), called stimulated Raman histology (SRH), to image a consecutive, multicenter cohort of patients with skull base tumor. SRH images were then used to train a convolutional neural network model using 3 representation learning strategies: cross-entropy, self-supervised contrastive learning, and supervised contrastive learning. Our trained convolutional neural network models were tested on a held-out, multicenter SRH data set. RESULTS: SRH was able to image the diagnostic features of both benign and malignant skull base tumors. Of the 3 representation learning strategies, supervised contrastive learning most effectively learned the distinctive and diagnostic SRH image features for each of the skull base tumor types. In our multicenter testing set, cross-entropy achieved an overall diagnostic accuracy of 91.5%, self-supervised contrastive learning 83.9%, and supervised contrastive learning 96.6%. Our trained model was able to segment tumor-normal margins and detect regions of microscopic tumor infiltration in meningioma SRH images. CONCLUSION: SRH with trained artificial intelligence models can provide rapid and accurate intraoperative analysis of skull base tumor specimens to inform surgical decision-making.

Clipping of unruptured intracranial aneurysms in patients older than sixty: An age-based analysis.

OBJECTIVE: The diagnosis of unruptured intracranial aneurysms (UIAs) is being made more frequently in elderly patients. The goal of this study is to evaluate complications and clinical outcome in patients ≥ 60 years-old who underwent clipping of UIAs. METHODS: We performed a retrospective cohort study. Clinical outcome (modified Rankin scale score) was determined at the latest clinical follow-up. Complications and outcomes were compared between age groups (60-69, 70-80) and subgroups (60-64, 65-69, 70-74, and >75). RESULTS: The study population consisted of 255 patients (range 60-80 years-old) who underwent 262 clipping procedures for UIAs. Mean follow-up duration was 15.6 months (± 27.5). Major complications occurred in 20 patients (7.6%) and mortality in 3 patients (1.1%). Medical complications occurred in 26 patients (10%). Mean length of hospital-stay was 4.7 days (± 5.8). 89.6% were discharged to home. 87.8% had a favorable clinical outcome. The 70-80 age group had significantly more complications (P = 0.03) than the 60-69 group and a significantly longer hospital stay (6.02 vs. 4.3 days, P = 0.04). The older group was less likely to discharge to home and more likely to require rehabilitation (P = 0.002). Favorable clinical outcome did not significantly differ between the two groups (85.7% vs. 88.4%, P = 0.56). There was a trend for increasing complications from the younger to older subgroups (P = 0.008) and a reduction in the likelihood to discharge to home (P < 0.0001). The rate of ultimate favorable clinical outcome did not differ significantly between subgroups (P = 0.79). CONCLUSION: Although complications, length of hospital-stay, and discharge to non-home destinations increase with older age, the majority of patients ≥ 60 may have favorable clinical outcomes.

Stent-assisted coiling of cerebral aneurysms: Head to head comparison between the Neuroform Atlas and EZ stents.

BACKGROUND: The Neuroform Atlas stent is thought to have features allowing for an improved stent delivery system. We aimed to provide a comparison of the Atlas and Neuroform EZ stents in patients treated with stent-assisted coiling. METHODS: Seventy-seven aneurysms treated with the Atlas stent and 77 aneurysms with similar characteristics treated with the EZ stent were retrospectively compared. Outcomes included angiographic occlusion per the Raymond-Roy (RR) scale, recanalization, retreatment and procedural complications. RESULTS: With the Atlas stent, technical success was 100% and immediate RR1 occlusion was 81.8%. Follow-up RR1 was achieved in 83.7%. The recanalization rate was 7% and the retreatment rate was 4.6%. The complication rate was 6.5% (new neurological deficit in 1.3%). With the EZ stent, technical success was 96%, immediate RR1 occlusion was 67.6% and follow-up RR1 was 67.6%. The recanalization rate was 12.7% and the retreatment rate was 14.1%. The complication rate was 10.4% (new neurological deficit in 2.6%). The rate of immediate RR1 occlusion was significantly higher with the Atlas stent (p = 0.03), and the rate of follow-up RR1 was nonsignificantly higher with the Atlas stent (p = 0.08). The retreatment rate was significantly lower with the Atlas stent (p = 0.009). There were no significant differences in the rates of recanalization (p = 0.5) and complications (p = 0.6). CONCLUSIONS: Stent-assisted coiling with the Atlas stent is safe and effective and shows better immediate results as compared to the EZ stent, with improved overall follow-up outcomes.

Length of Stay Beyond Medical Readiness in a Neurosurgical Patient Population and Associated Healthcare Costs.

BACKGROUND: Length of stay beyond medical readiness (LOS-BMR) leads to increased expenses and higher morbidity related to hospital-acquired conditions. OBJECTIVE: To determine the proportion of admitted neurosurgical patients who have LOS-BMR and associated risk factors and costs. METHODS: We performed a prospective, cohort analysis of all neurosurgical patients admitted to our institution over 5 mo. LOS-BMR was assessed daily by the attending neurosurgeon and neuro-intensivist with a standardized criterion. Univariate and multivariate logistic regressions were performed. RESULTS: Of the 884 patients admitted, 229 (25.9%) had a LOS-BMR. The average LOS-BMR was 2.7 ± 3.1 d at an average daily cost of $9 148.28 ± $12 983.10, which resulted in a total cost of $2 076 659.32 over the 5-mo period. Patients with LOS-BMR were significantly more likely to be older and to have hemiplegia, dementia, liver disease, renal disease, and diabetes mellitus. Patients with a LOS-BMR were significantly more likely to be discharged to a subacute rehabilitation/skilled nursing facility (40.2% vs 4.1%) or an acute/inpatient rehabilitation facility (22.7% vs 1.7%, P < .0001). Patients with Medicare insurance were more likely to have a LOS-BMR, whereas patients with private insurance were less likely (P = .048). CONCLUSION: The most common reason for LOS-BMR was inefficient discharge of patients to rehabilitation and nursing facilities secondary to unavailability of beds at discharge locations, insurance clearance delays, and family-related issues.

Evaluating the role of methicillin-resistant Staphylococcus aureus (MRSA)-specific antibiotic prophylaxis for neurosurgical patients.

OBJECTIVE: Surgical site infection (SSI) in neurosurgical patients increases morbidity. Despite the rise of methicillin-resistant Staphylococcus aureus (MRSA) colonization, there is little consensus regarding antibiotic prophylaxis for SSI in MRSA-colonized neurosurgical patients. Our objective was to examine the incidence of SSI in MRSA-colonized neurosurgical patients and interrogate whether MRSA-specific antibiotic prophylaxis reduces SSIs. METHODS: We performed a retrospective analysis of adult patients undergoing neurosurgical procedures between 2013 and 2018. The primary outcome was SSI in patients with MRSA colonization receiving MRSA-specific antibiotics. Secondary outcomes included predictors of SSI, including whether broad use of MRSA-specific antibiotics affects SSI rate. RESULTS: Of 9739 procedures, 376 had SSI (3.9 %). Seven hundred forty-four procedures (7.6 %) were performed on patients screened preoperatively for MRSA, including 54 procedures on MRSA-colonized patients. MRSA-colonized patients were more likely than MRSA-non-colonized patients to receive MRSA-specific antibiotics (35.2 % vs. 17.8 %, p = 0.002) for prophylaxis. Nevertheless, MRSA-colonized patients had higher SSI rates compared to MRSA-non-colonized patients (22.2 % vs. 6.4 %, p = 0.00002). MRSA-colonization led to 3.49 greater odds (95 % CI 1.52-7.65, p = 0.002) of SSI relative to MRSA-non-colonization. MRSA-colonized patients receiving MRSA-specific antibiotics, compared to those receiving non-MRSA-specific antibiotics, had lower SSI rates, but this difference was not statistically significant (15.8 % vs. 25.7 %, p = 0.40). In the non-screened population, those receiving MRSA-specific antibiotics, compared to those receiving non-MRSA-specific antibiotics, had significantly higher SSI rates (6.9 % vs. 3.0 %, p = 0.00001). The use of MRSA-specific antibiotic prophylaxis in the non-screened population increased the odds of SSI (OR 1.90, 95 % CI 1.45-2.46, p = 0.0001). CONCLUSION: MRSA-colonized neurosurgical patients had a higher SSI rate compared to MRSA-non-colonized patients. While MRSA-specific antibiotics may benefit those with MRSA colonization, the difference in SSI rate between MRSA-colonized patients receiving MRSA-specific antibiotics vs. non-specific antibiotics requires further investigation. The broader use of MRSA-specific antibiotics may paradoxically confer an increased risk of SSI in a non-screened neurosurgical population.

Seizure Prophylaxis in Unruptured Aneurysm Repair: A Randomized Controlled Trial.

BACKGROUND: Prophylactic antiepileptic drugs (pAEDs) are often prescribed for seizure prophylaxis in patients undergoing surgical treatment of unruptured intracranial aneurysms (UIAs). We aimed to evaluate the benefit of pAEDs in patients undergoing surgical repair of UIAs. METHODS: We randomly assigned eligible patients undergoing surgical repair of UIAs to receive levetiracetam for seven days post-operatively or standard care alone. The primary outcome was the evaluation of seizures in the perioperative period (within 4 weeks). We also evaluated seizure occurrence throughout follow-up and assessed functional outcomes using the modified Rankin scale score (mRS). RESULTS: 35 patients were randomized to the "no-levetiracetam" group and 41 patients were randomized to receive levetiracetam. The two study groups had similar overall baseline characteristics and the surgical complication rate was similar for both groups (p = 0.8). One patient in the "no-levetiracetam" group had a seizure in the perioperative period versus 2 patients in the group randomized to receive levetiracetam (2.9% vs 4.9%, respectively, p = 1.00). No patients in the "no-levetiracetam" group had any additional late seizures (mean follow-up of 20.4 months), but three patients in the levetiracetam group had late seizures during follow-up (mean follow-up of 19.1 months) (0% vs 7.3%, p = 0.2). mRS score of 0-2 at 90 days and at the latest follow-up were similar between the two groups (p = 1.00). CONCLUSIONS: Perioperative seizure prophylaxis with levetiracetam does not reduce the rate of seizures as compared to controls in patients undergoing surgical repair of UIAs.

Stereotactic Radiosurgery for Brain Arteriovenous Malformations: Evaluation of Obliteration and Review of Associated Predictors.

BACKGROUND: High arteriovenous malformation (AVM) obliteration rates have been reported with stereotactic radiosurgery (SRS), and multiple factors have been found to be associated with AVM obliteration. These predictors have been inconsistent throughout studies. We aimed to analyze our experience with linear accelerator (LINAC)-based SRS for brain AVMs, evaluate outcomes, assess factors associated with AVM obliteration and review the various reported predictors of AVM obliteration. METHODS: Electronic medical records were retrospectively reviewed to identify consecutive patients with brain AVMs treated with SRS over a 27-year period with at least 2 years of follow-up. Logistic regression analysis was performed to identify factors associated with AVM obliteration. RESULTS: One hundred twenty-eight patients with 142 brain AVMs treated with SRS were included. Mean age was 34.4 years. Fifty-two percent of AVMs were associated with a hemorrhage before SRS, and 14.8% were previously embolized. Mean clinical and angiographic follow-up times were 67.8 months and 58.6 months, respectively. The median Spetzler-Martin grade was 3. Mean maximal AVM diameter was 2.8 cm and mean AVM target volume was 7.4 cm3 with a median radiation dose of 16 Gy. Complete AVM obliteration was achieved in 80.3%. Radiation-related signs and symptoms were encountered in 32.4%, only 4.9% of which consisted of a permanent deficit. Post-SRS AVM-related hemorrhage occurred in 6.3% of cases. In multivariate analysis, factors associated with AVM obliteration included younger patient age (P = .019), male gender (P = .008), smaller AVM diameter (P = .04), smaller AVM target volume (P = .009), smaller isodose surface volume (P = .005), a higher delivered radiation dose (P = .013), and having only one major draining vein (P = .04). CONCLUSIONS: AVM obliteration with LINAC-based radiosurgery was safe and effective and achieved complete AVM obliteration in about 80% of cases. The most prominent predictors of AVM success included AVM size, AVM volume, radiation dose, number of draining veins and patient age.

Late Surgical Start Time and the Effect on Rates of Complications in a Neurosurgical Population: A Prospective Longitudinal Analysis.

OBJECTIVE: The after-hours effect on postoperative complications has been poorly studied in the neurosurgical literature. A recent retrospective analysis showed that patients with a surgical start time (SST) between 09:01 pm and 07:00 am had a greater risk of complications. This study used a prospective registry to examine the relationship between SST and postoperative complications in a large neurosurgical population. METHODS: We performed a prospective longitudinal cohort analysis of all consecutive adult patients admitted to our neurosurgery service between October 1, 2018 and May 1, 2019. Complications were prospectively recorded and classified as surgical or medical. Univariate and multivariate logistic regressions were used to analyze these data. RESULTS: Eighty-five surgical complications (6.6%) and 110 medical complications (8.6%) resulted from 1285 operations on 1145 patients. Later SST was predictive of complications in the emergent population (odds ratio [OR], 2.28; 95% confidence interval [CI], 1.01-5.15; P = 0.048) but not in the elective population. Extubation in the neurosurgical intensive care unit (NICU) versus the operating room strongly predicted medical complications (OR, 6.91; 95% CI, 3.33-14.34; P < 0.0001). Patients with a later SST were significantly more likely to be extubated in the NICU (P < 0.0001). CONCLUSIONS: Patients undergoing emergent operations with a later SST were significantly more likely to have a postoperative complication. Patients who were extubated in the NICU versus the operating room were significantly more likely to have a medical complication. Patients were more likely to be extubated in the NICU if they had a later SST; therefore, SST may indirectly be associated with an increase in medical complications.

Near real-time intraoperative brain tumor diagnosis using stimulated Raman histology and deep neural networks.

Intraoperative diagnosis is essential for providing safe and effective care during cancer surgery1. The existing workflow for intraoperative diagnosis based on hematoxylin and eosin staining of processed tissue is time, resource and labor intensive2,3. Moreover, interpretation of intraoperative histologic images is dependent on a contracting, unevenly distributed, pathology workforce4. In the present study, we report a parallel workflow that combines stimulated Raman histology (SRH)5-7, a label-free optical imaging method and deep convolutional neural networks (CNNs) to predict diagnosis at the bedside in near real-time in an automated fashion. Specifically, our CNNs, trained on over 2.5 million SRH images, predict brain tumor diagnosis in the operating room in under 150 s, an order of magnitude faster than conventional techniques (for example, 20-30 min)2. In a multicenter, prospective clinical trial (n = 278), we demonstrated that CNN-based diagnosis of SRH images was noninferior to pathologist-based interpretation of conventional histologic images (overall accuracy, 94.6% versus 93.9%). Our CNNs learned a hierarchy of recognizable histologic feature representations to classify the major histopathologic classes of brain tumors. In addition, we implemented a semantic segmentation method to identify tumor-infiltrated diagnostic regions within SRH images. These results demonstrate how intraoperative cancer diagnosis can be streamlined, creating a complementary pathway for tissue diagnosis that is independent of a traditional pathology laboratory.

Clinical and experimental aspects of aneurysmal subarachnoid hemorrhage.

Aneurysmal subarachnoid hemorrhage (aSAH) continues to be associated with significant morbidity and mortality despite advances in care and aneurysm treatment strategies. Cerebral vasospasm continues to be a major source of clinical worsening in patients. We intended to review the clinical and experimental aspects of aSAH and identify strategies that are being evaluated for the treatment of vasospasm. A literature review on aSAH and cerebral vasospasm was performed. Available treatments for aSAH continue to expand as research continues to identify new therapeutic targets. Oral nimodipine is the primary medication used in practice given its neuroprotective properties. Transluminal balloon angioplasty is widely utilized in patients with symptomatic vasospasm and ischemia. Prophylactic "triple-H" therapy, clazosentan, and intraarterial papaverine have fallen out of practice. Trials have not shown strong evidence supporting magnesium or statins. Other calcium channel blockers, milrinone, tirilazad, fasudil, cilostazol, albumin, eicosapentaenoic acid, erythropoietin, corticosteroids, minocycline, deferoxamine, intrathecal thrombolytics, need to be further investigated. Many of the current experimental drugs may have significant roles in the treatment algorithm, and further clinical trials are needed. There is growing evidence supporting that early brain injury in aSAH may lead to significant morbidity and mortality, and this needs to be explored further.

Introduction: Endovascular Neurosurgery.

Remarkable developments in the field of endovascular neurosurgery have been witnessed in the last decade. The success of endovascular therapy for ischemic stroke treatment is now irrefutable, making it an accepted standard of care. Endovascular treatment of cerebral aneurysms is no longer limited to primary coiling but now includes options such as stent or balloon assistance and flow diversion and applications utilizing neck reconstruction, intrasaccular, and bifurcation-specific devices. Balloons, liquid embolic agents, and flow-directed catheters have revolutionized the treatment of arteriovenous malformations and fistulae. The ongoing development of endovascular tools has led to novel and expanding approaches (for example, transvenous arteriovenous malformation embolization and transradial access). With improved technology, transposterior communicating artery access and other endovascular strategies are being applied successfully across the anterior and posterior circulations and to lesions once deemed only surgically approachable. Yet, we would be remiss to attribute the successes of endovascular strategies only to the development of their tools. Improvements in both noninvasive and angiographic imaging (such as three-dimensional road map guidance) have provided a greater understanding of pathologic entities and allowed the pursuit of endovascular cures.In this issue of Neurosurgical Focus, we present a wide range of endovascular strategies for a variety of neurovascular pathologies. We hope this video supplement will not only demonstrate the applicability of tried-and-true endovascular strategies to difficult clinical situations but also highlight new and developing endovascular technologies. We thank the authors for their outstanding contributions.

Neuropsychological Changes in Patients Undergoing Treatment of Unruptured Intracranial Aneurysms.

BACKGROUND: Evaluation of differences in neuropsychological outcomes in patients undergoing surgical clipping (SC) vs endovascular coiling (EC) for unruptured cerebral aneurysms is essential in guiding patients seeking treatment of asymptomatic cerebral aneurysms. OBJECTIVE: To perform a prospective longitudinal analysis of neuropsychological outcomes in patients who underwent microsurgery or coiling for unruptured cerebral aneurysms. METHODS: SC (50 patients), EC (35 patients), and healthy controls (43 individuals) were included. A detailed neuropsychological evaluation was performed at baseline and at 2 wk, 3 mo, 6 mo, and 12 mo. Student's t-test was utilized for comparing neuropsychological outcomes among the 3 groups. A mixed-effects model allowed for evaluation of neuropsychological outcome changes among the groups over time. RESULTS: Both the SC and EC groups had nonsignificant differences in procedure-related complications. SC patients had the greatest initial declines in short-term memory, fine motor control, and executive functioning; however, these patients also recovered to a greater degree in neuropsychological functionality. Over the next year, all groups achieved similar neuropsychological outcomes with no significant differences among groups. CONCLUSION: Whereas the initial decline in neuropsychological functioning was greater for SC patients, 1 yr after treatment there was no significant difference in neuropsychological outcome among the SC, EC, and healthy control groups.

A Large Database Analysis of Rates of Aneurysm Screening, Elective Treatment, and Subarachnoid Hemorrhage in Patients With Polycystic Kidney Disease.

BACKGROUND: Professional societies provide conflicting guidelines on aneurysm screening in patients with polycystic kidney disease (PKD), and the rate of subarachnoid hemorrhage (SAH) is poorly understood. OBJECTIVE: To evaluate screening, elective treatment, and the rate of SAH in patients with known PKD. METHODS: We examined longitudinally linked claims data from a large private insurer, identifying screening, elective treatment, aneurysmal subarachnoid hemorrhage (aSAH) and secured aneurysmal SAH (saSAH) in 2004 to 2014 amongst patients with known PKD. RESULTS: We identified 20 704 patients diagnosed with PKD. Among patients with an initial PKD diagnosis, 51/446 (15.9%) underwent angiographic screening within 2 yr. Forty aneurysms were treated electively in 48 868 yr at risk in PKD patients (82/100K patient yr, 95% confidence interval [CI] 60-112) vs 24 elective treatments in 349 861 yr at risk in age- and sex-matched controls (7/100K patient yr, 95% CI 5-10, P < .0001). Eleven admissions for aSAH were identified in PKD patients (23/100K patient yr, 95% CI 13-41) and 22 admissions for aSAH in controls (6/100K patient yr, 95% CI 4-10), giving an incidence rate ratio (IRR) of 3.6 (95% CI 1.7-7.4, P < .0001) and a comorbidity-adjusted IRR of 3.1 (95% CI 1.4-6.9). The incidence of saSAH was proportionally even higher in PKD patients than controls, 16 vs 2/100K patient years, IRR 9.5 (95% CI 3.3-27.5, P < .0001). CONCLUSION: Screening in PKD is performed only selectively, though resulting rates of elective treatment were over 10× those of controls. Despite screening and treatment, the rate of SAH remains significantly elevated over that of controls.

Cerebral Aneurysms in Autosomal Dominant Polycystic Kidney Disease: A Comparison of Management Approaches.

BACKGROUND: Autosomal dominant polycystic kidney disease (ADPKD) is a risk factor for formation of intracranial aneurysms (IAs), though the ideal screening and treatment strategies in this population are unclear. OBJECTIVE: To report outcomes of observation, open surgical, or endovascular management of ruptured and unruptured aneurysms in patients with ADPKD. METHODS: We performed a retrospective analysis of all patients with ADPKD and IAs at a single center from 2000 to 2016. RESULTS: Forty-five patients with ADPKD harboring 71 aneurysms were identified, including 11 patients with subarachnoid hemorrhage (SAH). Of 22 aneurysms managed with observation, none ruptured in 136 yr of clinical follow-up. Thirty-five aneurysms were treated with open surgery and 14 with an endovascular approach. Among treated aneurysms, poor neurologic outcome (modified Rankin scale >2) was seen only in patients presenting with SAH (17% SAH vs 0% elective, P = .06). Acute kidney injury (AKI) was also significantly associated with SAH presentation (22% SAH vs 0% elective, P = .05). Neither procedural complications nor AKI were associated with treatment modality. Among 175 yr of radiographic follow-up in patients with known IAs, 8 de novo aneurysms were found, including 3 that were treated. Of 11 patients with SAH, 7 ruptured in the setting of previously known ADPKD, including 2 with prior angiographic screening and 5 without screening. CONCLUSION: Poor outcomes occurred only with ruptured presentation but were equivalent between treatment modalities. Screening is performed only selectively, and 64% (7 of 11) of patients presenting with SAH had previously known ADPKD.