Schimke immuno-osseous dysplasia (SIOD) is a rare autosomal recessive disorder characterized by spondyloepiphyseal dysplasia, episodic lymphopenia, renal failure, and cerebrovascular disease secondary to arteriosclerosis and myointimal hyperplasia. In this paper the authors report the first known application of internal carotid artery (ICA) surgical revascularization to relieve a high-grade focal stenosis of the ICA in a pediatric patient, a 6-year-old boy with SIOD. The clinical presentation, imaging features, operative technique, and postoperative course are described and the molecular genetics, pathophysiology, and treatment considerations in SIOD are discussed.
Erick M. Westbroek, Nitin Mukerji, Paul Kalanithi and Gary K. Steinberg
Surgical management of giant presacral schwannoma: systematic review of published cases and meta-analysis
Presented at the 2019 AANS/CNS Joint Section on Disorders of the Spine and Peripheral Nerves
Zach Pennington, Erick M. Westbroek, A. Karim Ahmed, Ethan Cottrill, Daniel Lubelski, Matthew L. Goodwin and Daniel M. Sciubba
Giant presacral schwannomas are rare sacral tumors found in less than 1 of every 40,000 hospitalizations. Current management of these tumors is based solely upon case reports and small case series. In this paper the authors report the results of a systematic review of the available English literature on presacral schwannoma, focused on identifying the influence of tumor size, tumor morphology, surgical approach, and extent of resection (EOR) on recurrence-free survival and postoperative complications.
The medical literature (PubMed and EMBASE) was queried for reports of surgically managed sacral schwannoma, either involving 2 or more contiguous vertebral levels or with a diameter ≥ 5 cm. Tumor size and morphology, surgical approach, EOR, intraoperative and postoperative complications, and survival data were recorded.
Seventy-six articles were included, covering 123 unique patients (mean age 44.1 ± 1.4 years, 50.4% male). The most common presenting symptoms were leg pain (28.7%), lower back pain (21.3%), and constipation (15.7%). Most surgeries used an open anterior-only (40.0%) or posterior-only (30%) approach. Postoperative complications occurred in 25.6% of patients and local recurrence was noted in 5.4%. En bloc resection significantly improved progression-free survival relative to subtotal resection (p = 0.03). No difference existed between en bloc and gross-total resection (GTR; p = 0.25) or among the surgical approaches (p = 0.66). Postoperative complications were more common following anterior versus posterior approaches (p = 0.04). Surgical blood loss was significantly correlated with operative duration and tumor volume on multiple linear regression (both p < 0.001).
Presacral schwannoma can reasonably be treated with either en bloc or piecemeal GTR. The approach should be dictated by lesion morphology, and recurrence is infrequent. Anterior approaches may increase the risk of postoperative complications.
Sakibul Huq, Jeffrey Ehresman, Ethan Cottrill, A. Karim Ahmed, Zach Pennington, Erick M. Westbroek and Daniel M. Sciubba
Scheuermann kyphosis (SK) is an idiopathic kyphosis characterized by anterior wedging of ≥ 5° at 3 contiguous vertebrae managed with either nonoperative or operative treatment. Nonoperative treatment typically employs bracing, while operative treatment is performed with either a combined anterior-posterior fusion or posterior-only approach. Current evidence for these approaches has largely been derived from retrospective case series or focused reviews. Consequently, no consensus exists regarding optimal management strategies for patients afflicted with this condition. In this study, the authors systematically review the literature on SK with respect to indications for treatment, complications of treatment, differences in correction and loss of correction, and changes in treatment over time.
Using PubMed, Embase, CINAHL, Web of Science, and the Cochrane Library, all full-text publications on the operative and nonoperative treatment for SK in the peer-reviewed English-language literature between 1950 and 2017 were screened. Inclusion criteria involved fully published, peer-reviewed, retrospective or prospective studies of the primary medical literature. Studies were excluded if they did not provide clinical outcomes and statistics specific to SK, described fewer than 2 patients, or discussed results in nonhuman models. Variables extracted included treatment indications and methodology, maximum pretreatment kyphosis, immediate posttreatment kyphosis, kyphosis at last follow-up, year of treatment, and complications of treatment.
Of 659 unique studies, 45 met our inclusion criteria, covering 1829 unique patients. Indications for intervention were pain, deformity, failure of nonoperative treatment, and neural impairment. Among operatively treated patients, the most common complications were hardware failure and proximal or distal junctional kyphosis. Combined anterior-posterior procedures were additionally associated with neural, pulmonary, and cardiovascular complications. Posterior-only approaches offered superior correction compared to combined anterior-posterior fusion; both groups provided greater correction than bracing. Loss of correction was similar across operative approaches, and all were superior to bracing. Cross-sectional analysis suggested that surgeons have shifted from anterior-posterior to posterior-only approaches over the past two decades.
The data indicate that for patients with SK, surgery affords superior correction and maintenance of correction relative to bracing. Posterior-only fusion may provide greater correction and similar loss of correction compared to anterior-posterior approaches along with a smaller complication profile. This posterior-only approach has concomitantly gained popularity over the combined anterior-posterior approach in recent years.
Ethan Cottrill, Zach Pennington, A. Karim Ahmed, Daniel Lubelski, Matthew L. Goodwin, Alexander Perdomo-Pantoja, Erick M. Westbroek, Nicholas Theodore, Timothy Witham and Daniel Sciubba
Nonunion is a common complication of spinal fusion surgeries. Electrical stimulation technologies (ESTs)—namely, direct current stimulation (DCS), capacitive coupling stimulation (CCS), and inductive coupling stimulation (ICS)—have been suggested to improve fusion rates. However, the evidence to support their use is based solely on small trials. Here, the authors report the results of meta-analyses of the preclinical and clinical data from the literature to provide estimates of the overall effect of these therapies at large and in subgroups.
A systematic review of the English-language literature was performed using PubMed, Embase, and Web of Science databases. The query of these databases was designed to include all preclinical and clinical studies examining ESTs for spinal fusion. The primary endpoint was the fusion rate at the last follow-up. Meta-analyses were performed using a Freeman-Tukey double arcsine transformation followed by random-effects modeling.
A total of 33 articles (17 preclinical, 16 clinical) were identified, of which 11 preclinical studies (257 animals) and 13 clinical studies (2144 patients) were included in the meta-analysis. Among preclinical studies, the mean fusion rates were higher among EST-treated animals (OR 4.79, p < 0.001). Clinical studies similarly showed ESTs to increase fusion rates (OR 2.26, p < 0.001). Of EST modalities, only DCS improved fusion rates in both preclinical (OR 5.64, p < 0.001) and clinical (OR 2.13, p = 0.03) populations; ICS improved fusion in clinical studies only (OR 2.45, p = 0.014). CCS was not effective at increasing fusion, although only one clinical study was identified. A subanalysis of the clinical studies found that ESTs increased fusion rates in the following populations: patients with difficult-to-fuse spines, those who smoke, and those who underwent multilevel fusions.
The authors found that electrical stimulation devices may produce clinically significant increases in arthrodesis rates among patients undergoing spinal fusion. They also found that the pro-arthrodesis effects seen in preclinical studies are also found in clinical populations, suggesting that findings in animal studies are translatable. Additional research is needed to analyze the cost-effectiveness of these devices.
Erick M. Westbroek, Zach Pennington, A. Karim Ahmed, Yuanxuan Xia, Christine Boone, Philippe Gailloud and Daniel M. Sciubba
Preoperative endovascular embolization of hypervascular spine tumors can reduce intraoperative blood loss. The extent to which subtotal embolization reduces blood loss has not been clearly established. This study aimed to elucidate a relationship between the extent of preoperative embolization and intraoperative blood loss.
Sixty-six patients undergoing preoperative endovascular embolization and subsequent resection of hypervascular spine tumors were retrospectively reviewed. Patients were divided into 3 groups: complete embolization (n = 22), near-complete embolization (≥ 90% but < 100%; n = 22), and partial embolization (< 90%; n = 22). Intraoperative blood loss was compared between groups using one-way ANOVA with post hoc comparisons between groups.
The average blood loss in the complete embolization group was 1625 mL. The near-complete embolization group had an average blood loss of 2021 mL in surgery. Partial embolization was associated with a mean blood loss of 4009 mL. On one-way ANOVA, significant differences were seen across groups (F-ratio = 6.81, p = 0.002). Significant differences in intraoperative blood loss were also seen between patients undergoing complete and partial embolization (p = 0.001) and those undergoing near-complete and partial embolization (p = 0.006). Pairwise testing showed no significant difference between complete and near-complete embolization (p = 0.57). Analysis of a combined group of complete and near-complete embolization also showed a significantly decreased blood loss compared with partial embolization (p < 0.001). Patient age, tumor size, preoperative coagulation parameters, and preoperative platelet count were not significantly associated with blood loss.
Preoperative endovascular embolization is associated with decreased intraoperative blood loss. In this series, blood loss was significantly less in surgeries for tumors in which preoperative complete or near-complete embolization was achieved than in tumors in which preoperative embolization resulted in less than 90% reduction of tumor vascular blush. These findings suggest that there may be a critical threshold of efficacy that should be the goal of preoperative embolization.
Justin M. Caplan, Eric Sankey, David Gullotti, Joanna Wang, Erick Westbroek, Brian Hwang and Judy Huang
Patients with bilateral anterior circulation aneurysms present a management challenge. These lesions may be treated in a staged manner or alternatively, for select patients, a contralateral approach may be utilized to treat bilateral aneurysms with a single surgery. In this narrated video illustration, we present the case of a 57-year-old woman with incidentally discovered bilateral aneurysms (left middle cerebral artery [MCA], left anterior choroidal artery and right MCA). A contralateral approach through a left pterional craniotomy was performed formicrosurgical clipping of all three aneurysms. The techniques of pterional craniotomy, contralateral approach, microsurgical clipping and intraoperative angiography are reviewed.
The authors are grateful to Wuyang Yang, M.D. for his assistance.
The video can be found here: http://youtu.be/MlPIu3hQZkg.
Zach Pennington, Bowen Jiang, Erick M. Westbroek, Ethan Cottrill, Benjamin Greenberg, Philippe Gailloud, Jean-Paul Wolinsky, Ying Wei Lum and Nicholas Theodore
Myelopathy selectively involving the lower extremities can occur secondary to spondylotic changes, tumor, vascular malformations, or thoracolumbar cord ischemia. Vascular causes of myelopathy are rarely described. An uncommon etiology within this category is diaphragmatic crus syndrome, in which compression of an intersegmental artery supplying the cord leads to myelopathy. The authors present the operative technique for treating this syndrome, describing their experience with 3 patients treated for acute-onset lower-extremity myelopathy secondary to hypoperfusion of the anterior spinal artery.
All patients had compression of a lumbar intersegmental artery supplying the cord; the compression was caused by the diaphragmatic crus. Compression of the intersegmental artery was probably producing the patients’ symptoms by decreasing blood flow through the artery of Adamkiewicz, causing lumbosacral ischemia.
All patients underwent surgery to transect the offending diaphragmatic crus. Each patient experienced substantial symptom improvement, and 2 patients made a full neurological recovery before discharge.
Diaphragmatic crus syndrome is a rare or under-recognized cause of ischemic myelopathy. Patients present with episodic acute-on-chronic lower-extremity paraparesis, gait instability, and numbness. Angiography confirms compression of an intersegmental artery that gives rise to a dominant radiculomedullary artery. Transecting the offending diaphragmatic crus can produce complete resolution of neurological symptoms.
Camilo A. Molina, Nicholas Theodore, A. Karim Ahmed, Erick M. Westbroek, Yigal Mirovsky, Ran Harel, Emanuele Orru’, Majid Khan, Timothy Witham and Daniel M. Sciubba
Augmented reality (AR) is a novel technology that has the potential to increase the technical feasibility, accuracy, and safety of conventional manual and robotic computer-navigated pedicle insertion methods. Visual data are directly projected to the operator’s retina and overlaid onto the surgical field, thereby removing the requirement to shift attention to a remote display. The objective of this study was to assess the comparative accuracy of AR-assisted pedicle screw insertion in comparison to conventional pedicle screw insertion methods.
Five cadaveric male torsos were instrumented bilaterally from T6 to L5 for a total of 120 inserted pedicle screws. Postprocedural CT scans were obtained, and screw insertion accuracy was graded by 2 independent neuroradiologists using both the Gertzbein scale (GS) and a combination of that scale and the Heary classification, referred to in this paper as the Heary-Gertzbein scale (HGS). Non-inferiority analysis was performed, comparing the accuracy to freehand, manual computer-navigated, and robotics-assisted computer-navigated insertion accuracy rates reported in the literature. User experience analysis was conducted via a user experience questionnaire filled out by operators after the procedures.
The overall screw placement accuracy achieved with the AR system was 96.7% based on the HGS and 94.6% based on the GS. Insertion accuracy was non-inferior to accuracy reported for manual computer-navigated pedicle insertion based on both the GS and the HGS scores. When compared to accuracy reported for robotics-assisted computer-navigated insertion, accuracy achieved with the AR system was found to be non-inferior when assessed with the GS, but superior when assessed with the HGS. Last, accuracy results achieved with the AR system were found to be superior to results obtained with freehand insertion based on both the HGS and the GS scores. Accuracy results were not found to be inferior in any comparison. User experience analysis yielded “excellent” usability classification.
AR-assisted pedicle screw insertion is a technically feasible and accurate insertion method.
Matthew T. Bender, Geoffrey P. Colby, Li-Mei Lin, Bowen Jiang, Erick M. Westbroek, Risheng Xu, Jessica K. Campos, Judy Huang, Rafael J. Tamargo and Alexander L. Coon
Flow diversion requires neointimal stent overgrowth to deliver aneurysm occlusion. The existing literature on aneurysm occlusion is limited by heterogeneous follow-up, variable antiplatelet regimens, noninvasive imaging modalities, and nonstandard occlusion assessment. Using a large, single-center cohort with low attrition and standardized antiplatelet tapering, the authors evaluated outcomes after flow diversion of anterior circulation aneurysms to identify predictors of occlusion and aneurysm persistence.
Data from a prospective, IRB-approved database was analyzed for all patients with anterior circulation aneurysms treated by flow diversion with the Pipeline embolization device (PED) at the authors’ institution. Follow-up consisted of catheter cerebral angiography at 6 and 12 months postembolization. Clopidogrel was discontinued at 6 months and aspirin was reduced to 81 mg daily at 12 months. Occlusion was graded as complete, trace filling, entry remnant, or aneurysm filling. Multivariate logistic regression was performed to identify predictors of aneurysm persistence.
Follow-up catheter angiography studies were available for 445 (91%) of 491 PED procedures performed for anterior circulation aneurysms between August 2011 and August 2016. Three hundred eighty-seven patients accounted for these 445 lesions with follow-up angiography. The population was 84% female; mean age was 56 years and mean aneurysm size was 6.6 mm. Aneurysms arose from the internal carotid artery (83%), anterior cerebral artery (13%), and middle cerebral artery (4%). Morphology was saccular in 90% of the lesions, and 18% of the aneurysms has been previously treated. Overall, complete occlusion was achieved in 82% of cases at a mean follow-up of 14 months. Complete occlusion was achieved in 72%, 78%, and 87% at 6, 12, and 24 months, respectively. At 12 months, adjunctive coiling predicted occlusion (OR 0.260, p = 0.036), while male sex (OR 2.923, p = 0.032), aneurysm size (OR 3.584, p = 0.011), and incorporation of a branch vessel (OR 2.206, p = 0.035) predicted persistence. Notable variables that did not predict aneurysm occlusion were prior treatments, vessel of origin, fusiform morphology, and number of devices used.
This is the largest single-institution study showing high rates of anterior circulation aneurysm occlusion after Pipeline embolization. Predictors of persistence after flow diversion included increasing aneurysm size and incorporated branch vessel, whereas adjunctive coiling predicted occlusion.
Paritosh Pandey, Michael P. Marks, Ciara D. Harraher, Erick M. Westbroek, Steven D. Chang, Huy M. Do, Richard P. Levy, Robert L. Dodd and Gary K. Steinberg
Grade III arteriovenous malformations (AVMs) are diverse because of their variations in size (S), location in eloquent cortex (E), and presence of central venous drainage (V). Because they may have implications for management and outcome, the authors evaluated these variations in the present study.
Between 1984 and 2010, 100 patients with Grade III AVMs were treated. The AVMs were categorized by Spetzler-Martin characteristics as follows: Type 1 = S1E1V1, Type 2 = S2E1V0, Type 3 = S2E0V1, and Type 4 = S3E0V0. The occurrence of a new neurological deficit, functional status (based on modified Rankin Scale [mRS] score) at discharge and follow-up, and radiological obliteration were correlated with demographic and morphological characteristics.
One hundred patients (49 female and 51 male; age range 5–68 years, mean 35.8 years) were evaluated. The size of AVMs was less than 3 cm in 28 patients, 3–6 cm in 71, and greater than 6 cm in 1; 86 AVMs were located in eloquent cortex and 38 had central drainage. The AVMs were Type 1 in 28 cases, Type 2 in 60, Type 3 in 11, and Type 4 in 1. The authors performed embolization in 77 patients (175 procedures), surgery in 64 patients (74 surgeries), and radiosurgery in 49 patients (44 primary and 5 postoperative).
The mortality rate following the management of these AVMs was 1%. Fourteen patients (14%) had new neurological deficits, with 5 (5%) being disabling (mRS score > 2) and 9 (9%) being nondisabling (mRS score ≤ 2) events. Patients with Type 1 AVMs (small size) had the best outcome, with 1 (3.6%) in 28 having a new neurological deficit, compared with 72 patients with larger AVMs, of whom 13 (18.1%) had a new neurological deficit (p < 0.002). Older age (> 40 years), malformation size > 3 cm, and nonhemorrhagic presentation predicted the occurrence of new deficits (p < 0.002). Sex, eloquent cortex, and venous drainage did not confer any benefit.
In 89 cases follow-up was adequate for data to be included in the obliteration analysis. The AVM was obliterated in 78 patients (87.6%), 69 of them (88.5%) demonstrated on angiography and 9 on MRI /MR angiography. There was no difference between obliteration rates between different types of AVMs, size, eloquence, and drainage. Age, sex, and clinical presentation also did not predict obliteration.
Multimodality management of Grade III AVMs results in a high rate of obliteration, which was not influenced by size, venous drainage, or eloquent location. However, the development of new neurological deficits did correlate with size, whereas eloquence and venous drainage did not affect the neurological complication rate. The authors propose subclassifying the Grade III AVMs according to their size (< 3 and ≥ 3 cm) to account for treatment risk.