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Jorn Van Der Veken, Marguerite Harding, Saba Hatami, Marc Agzarian, and Nick Vrodos

BACKGROUND

Chiari Type I malformation (CM1) is a disorder recognized by caudal displacement of the cerebellar tonsils through the foramen magnum and into the cervical canal. Syringomyelia is frequently found in patients with CM1, but the pathophysiology of syringomyelia remains an enigma. As a general consensus, symptomatic patients should be treated and asymptomatic patients without a syrinx should not be treated. Mildly symptomatic patients or asymptomatic patients with a syrinx, on the other hand, pose a more challenging dilemma, as the natural evolution is uncertain. For many surgeons, the presence of a syrinx is an indication to offer surgery even if the patient is asymptomatic or mildly symptomatic.

OBSERVATIONS

The authors describe an illustrative case of a 31-year-old female with an incidental finding of a CM1 malformation and cervical syrinx in 2013. Conservative management was advocated as the patient was asymptomatic. Monitoring of the syrinx over a course of 8 years showed resolution, followed by reappearance and finally a complete resolution in 2021. A review of the literature and the possible pathophysiology is discussed.

LESSONS

The unusual course of this patient highlights the importance of guiding treatment by clinical symptoms, not radiological findings. Furthermore it reflects the complexity of the pathophysiology and the uncertain natural history of syringomyelia.

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Isaac Josh Abecassis, R. Michael Meyer, Michael R. Levitt, Jason P. Sheehan, Ching-Jen Chen, Bradley A. Gross, Ashley Lockerman, W. Christopher Fox, Waleed Brinjikji, Giuseppe Lanzino, Robert M. Starke, Stephanie H. Chen, Adriaan R. E. Potgieser, J. Marc C. van Dijk, Andrew Durnford, Diederik Bulters, Junichiro Satomi, Yoshiteru Tada, Amanda Kwasnicki, Sepideh Amin-Hanjani, Ali Alaraj, Edgar A. Samaniego, Minako Hayakawa, Colin P. Derdeyn, Ethan Winkler, Adib Abla, Pui Man Rosalind Lai, Rose Du, Ridhima Guniganti, Akash P. Kansagra, Gregory J. Zipfel, and Louis J. Kim

OBJECTIVE

There is a reported elevated risk of cerebral aneurysms in patients with intracranial dural arteriovenous fistulas (dAVFs). However, the natural history, rate of spontaneous regression, and ideal treatment regimen are not well characterized. In this study, the authors aimed to describe the characteristics of patients with dAVFs and intracranial aneurysms and propose a classification system.

METHODS

The Consortium for Dural Arteriovenous Fistula Outcomes Research (CONDOR) database from 12 centers was retrospectively reviewed. Analysis was performed to compare dAVF patients with (dAVF+ cohort) and without (dAVF-only cohort) concomitant aneurysm. Aneurysms were categorized based on location as a dAVF flow-related aneurysm (FRA) or a dAVF non–flow-related aneurysm (NFRA), with further classification as extra- or intradural. Patients with traumatic pseudoaneurysms or aneurysms with associated arteriovenous malformations were excluded from the analysis. Patient demographics, dAVF anatomical information, aneurysm information, and follow-up data were collected.

RESULTS

Of the 1077 patients, 1043 were eligible for inclusion, comprising 978 (93.8%) and 65 (6.2%) in the dAVF-only and dAVF+ cohorts, respectively. There were 96 aneurysms in the dAVF+ cohort; 10 patients (1%) harbored 12 FRAs, and 55 patients (5.3%) harbored 84 NFRAs. Dural AVF+ patients had higher rates of smoking (59.3% vs 35.2%, p < 0.001) and illicit drug use (5.8% vs 1.5%, p = 0.02). Sixteen dAVF+ patients (24.6%) presented with aneurysm rupture, which represented 16.7% of the total aneurysms. One patient (1.5%) had aneurysm rupture during follow-up. Patients with dAVF+ were more likely to have a dAVF located in nonconventional locations, less likely to have arterial supply to the dAVF from external carotid artery branches, and more likely to have supply from pial branches. Rates of cortical venous drainage and Borden type distributions were comparable between cohorts. A minority (12.5%) of aneurysms were FRAs. The majority of the aneurysms underwent treatment via either endovascular (36.5%) or microsurgical (15.6%) technique. A small proportion of aneurysms managed conservatively either with or without dAVF treatment spontaneously regressed (6.2%).

CONCLUSIONS

Patients with dAVF have a similar risk of harboring a concomitant intracranial aneurysm unrelated to the dAVF (5.3%) compared with the general population (approximately 2%–5%) and a rare risk (0.9%) of harboring an FRA. Only 50% of FRAs are intradural. Dural AVF+ patients have differences in dAVF angioarchitecture. A subset of dAVF+ patients harbor FRAs that may regress after dAVF treatment.

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Christopher T. Martin, Kenneth J. Holton, Kristen E. Jones, Jonathan N. Sembrano, and David W. Polly Jr.

OBJECTIVE

Pelvic fixation enhances long constructs during deformity surgery. Subsequent loosening of iliac screws and pain at the pelvis occur in as many as 29% of patients. Concomitant sacroiliac (SI) fusion may prevent potential pain and failure. The objective of this study was to describe a novel surgical technique and a single institution’s experience using bilateral SI fusion during adult deformity surgery with S2-alar-iliac (S2AI) screws and triangular titanium rods (TTRs) placed with navigation.

METHODS

The authors reviewed open SI joint fusions with TTR performed between August 2019 and March 2020. All patients underwent lumbosacral fusion through a midline approach and bilateral S2AI pelvic fixation in the caudal teardrop, followed by TTR placement just proximal and cephalad to the S2AI screws using intraoperative CT imaging guidance.

RESULTS

Twenty-one patients were identified who received 42 TTRs, ranging in size from 7.0 × 65 mm to 7.0 × 90 mm. Three TTRs (7%) were malpositioned intraoperatively, and each was successfully repositioned during index surgery without negative sequelae. All breaches occurred in a medial and cephalad direction into the pelvis. Incremental operative time for adding TTR averaged 8 minutes and 33 seconds per implant.

CONCLUSIONS

Image-guided open SI joint fusion with TTR during lumbosacral fusion is technically feasible. The bony corridor for implant placement is narrower cephalad, and implants tend to deviate medially into the pelvis. Detection of malpositioned implant is aided with intraoperative CT, but this can be salvaged. A prospective randomized clinical trial is underway that will better inform the impact of this technique on patient outcomes.

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Mohamed A. Labib, Xiaochun Zhao, Lena Mary Houlihan, Irakliy Abramov, Joshua S. Catapano, Komal Naeem, Mark C. Preul, A. Samy Youssef, and Michael T. Lawton

OBJECTIVE

The combined petrosal (CP) approach has been traditionally used to resect petroclival meningioma (PCM). The pretemporal transcavernous anterior petrosal (PTAP) approach has emerged as an alternative. A quantitative comparison of both approaches has not been made. This anatomical study compared the surgical corridors afforded by both approaches and identified key elements of the approach selection process.

METHODS

Twelve cadaveric specimens were dissected, and 10 were used for morphometric analysis. Groups A and B (n = 5 in each) underwent the CP and PTAP approaches, respectively. The area of drilled clivus, lengths of cranial nerves (CNs) II–X, length of posterior circulation vessels, surgical area of exposure of the brainstem, and angles of attack anterior and posterior to a common target were measured and compared.

RESULTS

The area of drilled clivus was significantly greater in group A than group B (mean ± SD 88.7 ± 17.1 mm2 vs 48.4 ± 17.9 mm2, p < 0.01). Longer segments of ipsilateral CN IV (52.4 ± 2.33 mm vs 46.5 ± 3.71 mm, p < 0.02), CN IX, and CN X (9.91 ± 3.21 mm vs 0.00 ± 0.00 mm, p < 0.01) were exposed in group A than group B. Shorter portions of CN II (9.31 ± 1.28 mm vs 17.6 ± 6.89 mm, p < 0.02) and V1 (26.9 ± 4.62 mm vs 32.4 ± 1.93 mm, p < 0.03) were exposed in group A than group B. Longer segments of ipsilateral superior cerebellar artery (SCA) were exposed in group A than group B (36.0 ± 4.91 mm vs 25.8 ± 3.55 mm, p < 0.02), but there was less exposure of contralateral SCA (0.00 ± 0.00 mm vs 7.95 ± 3.33 mm, p < 0.01). There was no statistically significant difference between groups with regard to the combined area of the exposed cerebral peduncles and pons (p = 0.75). Although exposure of the medulla was limited, group A had significantly greater exposure of the medulla than group B (p < 0.01). Finally, group A had a smaller anterior angle of attack than group B (24.1° ± 5.62° vs 34.8° ± 7.51°, p < 0.03).

CONCLUSIONS

This is the first study to quantitatively identify the advantages and limitations of the CP and PTAP approaches from an anatomical perspective. Understanding these data will aid in designing maximally effective yet minimally invasive approaches to PCM.

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Ridhima Guniganti, Enrico Giordan, Ching-Jen Chen, Isaac Josh Abecassis, Michael R. Levitt, Andrew Durnford, Jessica Smith, Edgar A. Samaniego, Colin P. Derdeyn, Amanda Kwasnicki, Ali Alaraj, Adriaan R. E. Potgieser, Samir Sur, Stephanie H. Chen, Yoshiteru Tada, Ethan Winkler, Ryan R. L. Phelps, Pui Man Rosalind Lai, Rose Du, Adib Abla, Junichiro Satomi, Robert M. Starke, J. Marc C. van Dijk, Sepideh Amin-Hanjani, Minako Hayakawa, Bradley A. Gross, W. Christopher Fox, Diederik Bulters, Louis J. Kim, Jason Sheehan, Giuseppe Lanzino, Jay F. Piccirillo, Akash P. Kansagra, and Gregory J. Zipfel

OBJECTIVE

Cranial dural arteriovenous fistulas (dAVFs) are rare lesions, hampering efforts to understand them and improve their care. To address this challenge, investigators with an established record of dAVF investigation formed an international, multicenter consortium aimed at better elucidating dAVF pathophysiology, imaging characteristics, natural history, and patient outcomes. This report describes the design of the Consortium for Dural Arteriovenous Fistula Outcomes Research (CONDOR) and includes characterization of the 1077-patient cohort.

METHODS

Potential collaborators with established interest in the field were identified via systematic review of the literature. To ensure uniformity of data collection, a quality control process was instituted. Data were retrospectively obtained.

RESULTS

CONDOR comprises 14 centers in the United States, the United Kingdom, the Netherlands, and Japan that have pooled their data from 1077 dAVF patients seen between 1990 and 2017. The cohort includes 359 patients (33%) with Borden type I dAVFs, 175 (16%) with Borden type II fistulas, and 529 (49%) with Borden type III fistulas. Overall, 852 patients (79%) presented with fistula-related symptoms: 427 (40%) presented with nonaggressive symptoms such as tinnitus or orbital phenomena, 258 (24%) presented with intracranial hemorrhage, and 167 (16%) presented with nonhemorrhagic neurological deficits. A smaller proportion (224 patients, 21%), whose dAVFs were discovered incidentally, were asymptomatic. Many patients (85%, 911/1077) underwent treatment via endovascular embolization (55%, 587/1077), surgery (10%, 103/1077), radiosurgery (3%, 36/1077), or multimodal therapy (17%, 184/1077). The overall angiographic cure rate was 83% (758/911 treated), and treatment-related permanent neurological morbidity was 2% (27/1467 total procedures). The median time from diagnosis to follow-up was 380 days (IQR 120–1038.5 days).

CONCLUSIONS

With more than 1000 patients, the CONDOR registry represents the largest registry of cranial dAVF patient data in the world. These unique, well-annotated data will enable multiple future analyses to be performed to better understand dAVFs and their management.

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Ulrich Hubbe, Jan-Helge Klingler, Roland Roelz, Christoph Scholz, Katerina Argiti, Panagiotis Fistouris, Jürgen Beck, and Ioannis Vasilikos

OBJECTIVE

A major challenge of a minimally invasive spinal approach (MIS) is maintaining freedom of maneuverability through small operative corridors. Unfortunately, during tubular resection of intradural pathologies, the durotomy and its accompanying tenting sutures offer a smaller operating window than the maximum surface of the tube’s base. The objective of this study was to evaluate if a novel double tubular technique could expand the surgical visual field during MIS resection of intradural pathologies.

METHODS

A total of 25 MIS resections of intradural extramedullary pathologies were included. A posterior tubular interlaminar fenestration was performed in all surgeries. A durotomy covering the whole diameter of the tubular base was the standard in all cases. After placement of two tenting sutures on each side of the durotomy and application of tension, the resulting surface of the achieved dura fenestration was measured after optical analysis of the intraoperative video. In the next step, a second tube, 2 mm thinner than and the same length as the first, was inserted telescopically into the first tube, resulting an angulated fulcrum effect on the tenting sutures.

RESULTS

Optical surface analysis of the dura fenestration before and after the second tubular insertion verified a significant widening of the visual field of 43.1% (mean 18.84 mm2, 95% CI 16.8–20.8, p value < 0.001). There were no ruptured tenting sutures through the increased tension. Postoperative MRIs verified complete resection of the pathologies.

CONCLUSIONS

Inserting a second tube telescopically during posterior minimally invasive tubular spinal intradural surgery leads to an angulated fulcrum effect on the dura tenting sutures which consequently increases the surface of the dura fenestration and induces a meaningful widening of the visual field.

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Edgar A. Samaniego, Jorge A. Roa, Minako Hayakawa, Ching-Jen Chen, Jason P. Sheehan, Louis J. Kim, Isaac Josh Abecassis, Michael R. Levitt, Ridhima Guniganti, Akash P. Kansagra, Giuseppe Lanzino, Enrico Giordan, Waleed Brinjikji, Diederik Bulters, Andrew Durnford, W. Christopher Fox, Adam J. Polifka, Bradley A. Gross, Sepideh Amin-Hanjani, Ali Alaraj, Amanda Kwasnicki, Robert M. Starke, Samir Sur, J. Marc C. van Dijk, Adriaan R. E. Potgieser, Junichiro Satomi, Yoshiteru Tada, Adib Abla, Ethan Winkler, Rose Du, Pui Man Rosalind Lai, Gregory J. Zipfel, and Colin P. Derdeyn

OBJECTIVE

Current evidence suggests that intracranial dural arteriovenous fistulas (dAVFs) without cortical venous drainage (CVD) have a benign clinical course. However, no large study has evaluated the safety and efficacy of current treatments and their impact over the natural history of dAVFs without CVD.

METHODS

The authors conducted an analysis of the retrospectively collected multicenter Consortium for Dural Arteriovenous Fistula Outcomes Research (CONDOR) database. Patient demographics and presenting symptoms, angiographic features of the dAVFs, and treatment outcomes of patients with Borden type I dAVFs were reviewed. Clinical and radiological follow-up information was assessed to determine rates of new intracranial hemorrhage (ICH) or nonhemorrhagic neurological deficit (NHND), worsening of venous hyperdynamic symptoms (VHSs), angiographic recurrence, and progression or spontaneous regression of dAVFs over time.

RESULTS

A total of 342 patients/Borden type I dAVFs were identified. The mean patient age was 58.1 ± 15.6 years, and 62% were women. The mean follow-up time was 37.7 ± 54.3 months. Of 230 (67.3%) treated dAVFs, 178 (77%) underwent mainly endovascular embolization, 11 (4.7%) radiosurgery alone, and 4 (1.7%) open surgery as the primary modality. After the first embolization, most dAVFs (47.2%) achieved only partial reduction in early venous filling. Multiple complementary interventions increased complete obliteration rates from 37.9% after first embolization to 46.7% after two or more embolizations, and 55.2% after combined radiosurgery and open surgery. Immediate postprocedural complications occurred in 35 dAVFs (15.2%) and 6 (2.6%) with permanent sequelae. Of 127 completely obliterated dAVFs by any therapeutic modality, 2 (1.6%) showed angiographic recurrence/recanalization at a mean of 34.2 months after treatment. Progression to Borden-Shucart type II or III was documented in 2.2% of patients and subsequent development of a new dAVF in 1.6%. Partial spontaneous regression was found in 22 (21.4%) of 103 nontreated dAVFs. Multivariate Cox regression analysis demonstrated that older age, NHND, or severe venous-hyperdynamic symptoms at presentation and infratentorial location were associated with worse prognosis. Kaplan-Meier curves showed no significant difference for stable/improved symptoms survival probability in treated versus nontreated dAVFs. However, estimated survival times showed better trends for treated dAVFs compared with nontreated dAVFs (288.1 months vs 151.1 months, log-rank p = 0.28). This difference was statistically significant for treated dAVFs with 100% occlusion (394 months, log-rank p < 0.001).

CONCLUSIONS

Current therapeutic modalities for management of dAVFs without CVD may provide better symptom control when complete angiographic occlusion is achieved.

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Amir Hadanny and Julie G. Pilitsis

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Risheng Xu, Michael E. Xie, Wuyang Yang, Philippe Gailloud, Justin M. Caplan, Christopher M. Jackson, Eric M. Jackson, Mari L. Groves, Shenandoah Robinson, Alan R. Cohen, Judy Huang, and Rafael J. Tamargo

OBJECTIVE

Pediatric intracranial aneurysms are rare. Most large series in the last 15 years reported on an average of only 39 patients. The authors sought to report their institutional experience with pediatric intracranial aneurysms from 1991 to 2021 and to compare pediatric patient and aneurysm characteristics with those of a contemporaneous adult cohort.

METHODS

Pediatric (≤ 18 years of age) and adult patients with one or more intracranial aneurysms were identified in a prospective database. Standard epidemiological features and outcomes of each pediatric patient were retrospectively recorded. These results were compared with those of adult aneurysm patients managed at a single institution over the same time period.

RESULTS

From a total of 4500 patients with 5150 intracranial aneurysms admitted over 30 years, there were 47 children with 53 aneurysms and 4453 adults with 5097 aneurysms; 53.2% of children and 36.4% of adults presented with a subarachnoid hemorrhage (SAH). Pediatric aneurysms were significantly more common in males, more likely giant (≥ 25 mm), and most frequently located in the middle cerebral artery. Overall, 85.1% of the pediatric patients had a modified Rankin Scale score ≤ 2 at the last follow-up (with a mean follow-up of 65.9 months), and the pediatric mortality rate was 10.6%; all 5 patients who died had an SAH. The recurrence rate of treated aneurysms was 6.7% (1/15) in the endovascular group but 0% (0/31) in the microsurgical group. No de novo aneurysms occurred in children (mean follow-up 5.5 years).

CONCLUSIONS

Pediatric intracranial aneurysms are significantly different from adult aneurysms in terms of sex, presentation, location, size, and outcomes. Future prospective studies will better characterize long-term aneurysm recurrence, rebleeds, and de novo aneurysm occurrences. The authors currently favor microsurgical over endovascular treatment for pediatric aneurysms.