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.
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.
Geoffrey P. Colby, Bowen Jiang, Matthew T. Bender, Narlin B. Beaty, Erick M. Westbroek, Risheng Xu, Li-Mei Lin, Jessica K. Campos, Rafael J. Tamargo, Judy Huang, Alan R. Cohen and Alexander L. Coon
Intracranial aneurysms in the pediatric population are rare entities. The authors recently treated a 9-month-old infant with a 19-mm recurrent, previously ruptured, and coil-embolized left middle cerebral artery (MCA) pseudoaneurysm, which was treated definitively with single-stage Pipeline-assisted coil embolization. The patient was 5 months old when she underwent resection of a left temporal Grade 1 desmoplastic infantile ganglioglioma at an outside institution, which was complicated by left MCA injury with a resultant 9-mm left M1 pseudoaneurysm. Within a month, the patient had two aneurysmal rupture events and underwent emergency craniectomy for decompression and evacuation of subdural hematoma. The pseudoaneurysm initially underwent coil embolization; however, follow-up MR angiography (MRA) revealed aneurysm recanalization with saccular enlargement to 19 mm. The patient underwent successful flow diversion–assisted coil embolization at 9 months of age. At 7 months after the procedure, follow-up MRA showed complete aneurysm occlusion without evidence of in-stent thrombosis or stenosis. Experience with flow diverters in the pediatric population is still in its early phases, with the youngest reported patient being 22 months old. In this paper the authors report the first case of such a technique in an infant, whom they believe to be the youngest patient to undergo cerebral flow diversion treatment.
Geoffrey P. Colby, Matthew T. Bender, Li-Mei Lin, Narlin Beaty, Justin M. Caplan, Bowen Jiang, Erick M. Westbroek, Bijan Varjavand, Jessica K. Campos, Judy Huang, Rafael J. Tamargo and Alexander L. Coon
The second-generation Pipeline embolization device (PED), Flex, has several design upgrades, including improved opening and the ability to be resheathed, in comparison with the original device (PED classic). The authors hypothesized that Flex is associated with a lower rate of major complications.
A prospective, IRB-approved, single-institution database was analyzed for all patients with anterior circulation aneurysms treated by flow diversion. The PED classic was used from August 2011 to January 2015, and the Pipeline Flex has been used since February 2015.
A total of 568 PED procedures (252 classic and 316 Flex) were performed for anterior circulation aneurysms. The average aneurysm size was 6.8 mm. Patients undergoing treatment with the Flex device had smaller aneurysms (p = 0.006) and were more likely to have undergone previous treatments (p = 0.001). Most aneurysms originated along the internal carotid artery (89% classic and 75% Flex) but there were more anterior cerebral artery (18%) and middle cerebral artery (7%) deployments with Flex (p = 0.001). Procedural success was achieved in 96% of classic and 98% of Flex cases (p = 0.078). Major morbidity or death occurred in 3.5% of cases overall: 5.6% of classic cases, and 1.9% of Flex cases (p = 0.019). On multivariate logistic regression, predictors of major complications were in situ thrombosis (OR 4.3, p = 0.006), classic as opposed to Flex device (OR 3.7, p = 0.008), and device deployment in the anterior cerebral artery or middle cerebral artery as opposed to the internal carotid artery (OR 3.5, p = 0.034).
Flow diversion of anterior circulation cerebral aneurysms is associated with an overall low rate of major complications. The complication rate is significantly lower since the introduction of the second-generation PED (Flex).
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.
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.
Wuyang Yang, Heather Anderson-Keightly, Erick M. Westbroek, Justin M. Caplan, Xiaoming Rong, Alice L. Hung, Geoffrey P. Colby, Alexander L. Coon, Rafael J. Tamargo, Judy Huang and Edward S. Ahn
Compared with the general population, the specific natural history of arteriovenous malformations (AVMs) in pediatric patients is less well understood. Furthermore, few pediatric studies have compared posttreatment hemorrhagic risk and functional outcome across different treatment modalities. The objective of this study was to elucidate these points.
The authors retrospectively reviewed all pediatric patients with AVMs evaluated at their institution between 1990 and 2013. The AVM natural history was represented by hemorrhagic risk during the observation period. For treated patients, the observation period was defined as the interval between diagnosis and treatment. Posttreatment hemorrhagic risk and functional outcomes were also assessed.
A total of 124 pediatric patients with AVMs were evaluated, and 90 patients (72.6%) were retained through follow-up. The average patient age was 13.3 ± 3.8 years, with a mean follow-up period of 9.95 years. The overall AVM obliteration rate was 59.7%. Radiosurgery had an obliteration rate of 49.0%. Thirteen patients were managed conservatively. Four patients under observation hemorrhaged during a total interval of 429.4 patient-years, translating to an annual risk of 0.9%. Posttreatment hemorrhagic risk by treatment modalities were categorized as follows: surgery ± embolization (0.0%), radiosurgery ± embolization (0.8%), embolization alone (2.8%), surgery + radiosurgery ± embolization (3.5%), and observation (0.8%). A significantly higher risk of posttreatment hemorrhage was observed for patients with hemorrhagic presentation (p = 0.043) in multivariate analysis. Seizure presentation, frontal lobe location, nonheadache presentation, and treatment modality were significantly associated with increased risk of poor functional outcomes.
In this study of pediatric patients with AVMs, the natural history of hemorrhage was relatively low at 0.9%. Resection remained the optimal management for hemorrhage control and functional outcome perseverance in these pediatric patients with AVMs. AVM obliteration is a valid treatment goal, especially for patients with ruptured presentation, to prevent further hemorrhages later in life.
Venkatesh S. Madhugiri, Mario K. C. Teo, Erick M. Westbroek, Steven D. Chang, Michael P. Marks, Huy M. Do, Richard P. Levy and Gary K. Steinberg
Arteriovenous malformations (AVMs) of the basal ganglia and thalamus are particularly difficult lesions to treat, accounting for 3%–13% of all AVMs in surgical series and 23%–44% of malformations in radiosurgery series. The goal of this study was to report the results of multimodal management of basal ganglia and thalamic AVMs and investigate the factors that influence radiographic cure and good clinical outcomes.
This study was a retrospective analysis of a prospectively maintained database of all patients treated at the authors’ institution. Clinical, radiological, follow-up, and outcome data were analyzed. Univariate and multivariate analyses were conducted to explore the influence of various factors on outcome.
The results and data analysis pertaining to 123 patients treated over 32 years are presented. In this cohort, radiographic cure was achieved in 50.9% of the patients. Seventy-five percent of patients had good clinical outcomes (stable or improved performance scores), whereas 25% worsened after treatment. Inclusion of surgery and radiosurgery independently predicted obliteration, whereas nidus diameter and volume predicted clinical outcomes. Nidus volume/diameter and inclusion of surgery predicted the optimal outcome, i.e., good clinical outcomes with lesion obliteration.
Good outcomes are possible with multimodal treatment in these complex patients. Increasing size and, by extension, higher Spetzler-Martin grade are associated with worse outcomes. Inclusion of multiple modalities of treatment as indicated could improve the chances of radiographic cure and good outcomes.
Paraspinal muscle size as an independent risk factor for proximal junctional kyphosis in patients undergoing thoracolumbar fusion
Presented at the 2019 AANS/CNS Joint Section on Disorders of the Spine and Peripheral Nerves
Zach Pennington, Ethan Cottrill, A. Karim Ahmed, Peter Passias, Themistocles Protopsaltis, Brian Neuman, Khaled M. Kebaish, Jeff Ehresman, Erick M. Westbroek, Matthew L. Goodwin and Daniel M. Sciubba
Proximal junctional kyphosis (PJK) is a structural complication of spinal fusion in 5%–61% of patients treated for adult spinal deformity. In nearly one-third of these cases, PJK is progressive and requires costly surgical revision. Previous studies have suggested that patient body habitus may predict risk for PJK. Here, the authors sought to investigate abdominal girth and paraspinal muscle size as risk factors for PJK.
All patients undergoing thoracolumbosacral fusion greater than 2 levels at a single institution over a 5-year period with ≥ 6 months of radiographic follow-up were considered for inclusion. PJK was defined as kyphosis ≥ 20° between the upper instrumented vertebra (UIV) and two supra-adjacent vertebrae. Operative and radiographic parameters were recorded, including pre- and postoperative sagittal vertical axis (SVA), sacral slope (SS), lumbar lordosis (LL), pelvic tilt, pelvic incidence (PI), and absolute value of the pelvic incidence–lumbar lordosis mismatch (|PI-LL|), as well as changes in LL, |PI-LL|, and SVA. The authors also considered relative abdominal girth and the size of the paraspinal muscles at the UIV.
One hundred sixty-nine patients met inclusion criteria. On univariate analysis, PJK was associated with a larger preoperative SVA (p < 0.001) and |PI-LL| (p = 0.01), and smaller SS (p = 0.004) and LL (p = 0.001). PJK was also associated with more positive postoperative SVA (p = 0.01), ΔSVA (p = 0.01), Δ|PI-LL| (p < 0.001), and ΔLL (p < 0.001); longer construct length (p = 0.005); larger abdominal girth–to-muscle ratio (p = 0.007); and smaller paraspinal muscles at the UIV (p < 0.001). Higher postoperative SVA (OR 1.1 per cm), smaller paraspinal muscles at the UIV (OR 2.11), and more aggressive reduction in |PI-LL| (OR 1.03) were independent predictors of radiographic PJK on multivariate logistic regression.
A more positive postoperative global sagittal alignment and smaller paraspinal musculature at the UIV most strongly predicted PJK following thoracolumbosacral fusion.