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Andrew K. Johnson, Daniel M. Heiferman and Demetrius K. Lopes


The introduction of intracranial stents to aneurysm treatment allows endovascular repair of nearly all aneurysms, but the safety and durability of stent-assisted embolization of middle cerebral artery (MCA) aneurysms is unclear.


Ninety-one patients with 100 complex MCA aneurysms not amenable to simple coiling were treated with stent-assisted embolization as a first option. Technical and clinical results, initial follow-up imaging, and long-term annual MR angiography (MRA) were reviewed.


Intracranial stents were successfully deployed in all 100 aneurysms. There was 1 case of significant neurological morbidity (1%) and 1 case of death (1%) related to treatment. Initial posttreatment angiography revealed complete occlusion of 48 aneurysms (48%), a residual neck in 21 (21%), and residual aneurysm filling in 31 (31%). Follow-up imaging performed in 85 (90.4%) of a possible 94 aneurysms showed complete occlusion of 77 aneurysms (90.6%), residual neck in 3 (3.5%), and residual filling in 5 (5.9%). Four aneurysms (4.7%) required retreatment. Long-term MRA follow-up revealed stability or progressive thrombosis in 47 (97.9%) of 48 aneurysms. In 11 patients Y-configuration stenting caused only 1 minor complication and provided durable occlusion in all cases.


Stent-assisted techniques increase the number of aneurysms that may be treated endovascularly and represent an acceptable alternative to craniotomy. Stents provided adequate vessel reconstruction, low complication rates, and good long-term occlusion.

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Michael T. Lawton

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Krishna C. Joshi, Daniel M. Heiferman, André Beer-Furlan and Demetrius K. Lopes

External carotid artery (ECA) to internal carotid artery (ICA) bypass is a well-established procedure for the treatment of chronic ischemic diseases of the carotid artery. Rarely de novo aneurysms can develop at the site of anastomosis. The treatment of these aneurysms can be very challenging due to various factors, including the presence of graft, previous craniotomy, atherosclerotic vessels, and lack of direct access. In this video the authors report and discuss the management of a right middle cerebral artery (MCA) wide-necked de novo aneurysm by stent-assisted coiling through a retrograde trans-posterior communicating artery access.

The video can be found here:

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Daniel M. Heiferman, Daphne Li, Joseph C. Serrone, Matthew R. Reynolds, Anand V. Germanwala, Clarence B. Watridge and Adam S. Arthur

Dr. Francis Murphey of the Semmes-Murphey Clinic in Memphis recognized that a focal sacculation on the dome of an aneurysm may be angiographic evidence of a culpable aneurysm in the setting of subarachnoid hemorrhage with multiple intracranial aneurysms present. This has been referred to as a Murphey’s “teat,” “tit,” or “excrescence.” With variability in terminology, misspellings in the literature, and the fact that Dr. Murphey did not formally publish this important work, the authors sought to clarify the meaning and investigate the origins of this enigmatic cerebrovascular eponym.

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Giselle E. K. Malina, Daniel M. Heiferman, Loren N. Riedy, Caroline C. Szujewski, Elhaum G. Rezaii, John P. Leonetti and Douglas E. Anderson


Sporadic unilateral vestibular schwannomas are rare in the pediatric population. Little has been reported in the literature on the presentation, tumor size, response to surgical treatment, and recurrence rates in these younger patients. The authors’ goal was to describe their institutional experience with pediatric sporadic vestibular schwannomas and to conduct a meta-analysis of the existing literature to provide further insight into the presentation, tumor characteristics, and surgical outcomes for these rare tumors to help direct future treatment strategies.


The authors performed a retrospective review of all patients 21 years of age or younger with unilateral vestibular schwannomas and without neurofibromatosis type 2 who underwent resection by the senior authors between 1997 and 2019. A systematic review of the literature and meta-analysis was also performed by entering the search terms “pediatric” and “vestibular schwannoma” or “acoustic neuroma,” as well as “sporadic” into PubMed. Presentation, treatment, clinical outcomes, and follow-up were analyzed.


Fifteen patients were identified at the authors’ institution, ranging in age from 12 to 21 years (mean 16.5 years). Common presenting symptoms included hearing loss (87%), headache (40%), vertigo (33%), ataxia (33%), and tinnitus (33%). At the time of surgery, the mean tumor size was 3.4 cm, with four 1-cm tumors. Four patients had residual tumor following their first surgery, 3 (75%) of whom had significant radiographic regrowth that required further treatment. The literature review identified an additional 81 patients from 26 studies with patient-specific clinical data available for analysis. This resulted in a total of 96 reported patients with an overall average age at diagnosis of 12.1 years (range 6–21 years) and an average tumor size of 4.1 cm.


Pediatric vestibular schwannomas present similarly to those in adults, although symptoms of mass effect are more common, as these tumors tend to be larger at diagnosis. Some children are found to have small tumors and can be successfully treated surgically. Residual tumors in pediatric patients were found to have a higher rate of regrowth than those in their adult counterparts.

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Ryan C. Hofler, Daniel M. Heiferman, Ayrin Molefe, Ryan LeDuc, Stephen J. Johans, Jordan D. Rosenblum, Russ P. Nockels and G. Alexander Jones


Atlantoaxial instability is an important cause of pain and neurological dysfunction in patients with Down syndrome (DS), frequently requiring instrumented fusion of the upper cervical spine. This study provides a quantitative analysis of C2 morphology in DS patients compared with their peers without DS to identify differences that must be considered for the safe placement of instrumentation.


A retrospective chart review identified age-matched patients with and without DS with a CT scan of the cervical spine. Three-dimensional reconstructions of these scans were made with images along the axis of, and perpendicular to, the pars, lamina, facet, and transverse foramen of C2 bilaterally. Two of the authors performed independent measurements of anatomical structures using these images, and the average of the 2 raters’ measurements was recorded. Pedicle height and width; pars axis length (the distance from the facet to the anterior vertebral body through the pars); pars rostrocaudal angle (angle of the pars axis length to the endplate of C2); pars axial angle (angle of the pars axis length to the median coronal plane); lamina height, length, and width; lamina angle (angle of the lamina length to the median coronal plane); and transverse foramen posterior distance (the distance from the posterior wall of the transverse foramen to the tangent of the posterior vertebral body) were measured bilaterally. Patients with and without DS were compared using a mixed-effects model accounting for patient height.


A total of 18 patients with and 20 patients without DS were included in the analysis. The groups were matched based on age and sex. The median height was 147 cm (IQR 142–160 cm) in the DS group and 165 cm (IQR 161–172 cm) in the non-DS group (p < 0.001). After accounting for variations in height, the mean pars rostrocaudal angle was greater (50.86° vs 45.54°, p = 0.004), the mean transverse foramen posterior distance was less (−1.5 mm vs +1.3 mm, p = 0.001), and the mean lamina width was less (6.2 mm vs 7.7 mm, p = 0.038) in patients with DS.


Patients with DS had a steeper rostrocaudal trajectory of the pars, a more posteriorly positioned transverse foramen posterior wall, and a narrower lamina compared with age- and sex-matched peers. These variations should be considered during surgical planning, as they may have implications to safe placement of instrumentation.

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Daphne Li, Daniel M. Heiferman, Hasan R. Syed, João Gustavo Santos, Robin M. Bowman, Arthur J. DiPatri Jr., Tadanori Tomita, Nitin R. Wadhwani and Tord D. Alden

Atypical teratoid rhabdoid tumors (ATRTs) are rare malignant central nervous system tumors, commonly occurring before 3 years of age. Median overall survival (OS) of patients with these tumors is about 1 year, despite aggressive multimodal therapy. Pediatric primary spinal ATRTs are even more rare, with fewer than 50 cases reported. The authors present a series of four patients who were treated at Ann and Robert H. Lurie Children’s Hospital of Chicago in the period from 1996 to 2017.

These patients, with ages 2–11 years, presented with pain and a decline in motor functions. They were found to have lesions in the lumbar, thoracic, and/or cervical spine. One patient’s tumor was intramedullary with exophytic components, while another patient’s tumor had both intra- and extradural components. All patients underwent resection followed by chemotherapy (systemic and intrathecal). Two patients had fractionated radiation therapy and one had an autologous stem cell transplant. Three patients are known to be deceased (OS 8.5–45 months). The fourth patient was in remission 19 years after her initial diagnosis. To the authors’ knowledge, this is the largest series of pediatric primary spinal ATRTs documented at a single institution. These cases illustrate a variety of presentations of spinal ATRT and add to the body of literature on this aggressive pathology.

A systematic MEDLINE search was also conducted using the keywords “atypical teratoid rhabdoid tumor,” “pediatric spinal rhabdoid tumor,” and “malignant rhabdoid tumor spine.” Reports were included for patients younger than 21 years, without evidence of intracranial or systemic disease at the time of diagnosis. Clinical characteristics and outcomes of the four institutional cases were compared to those in the literature. This review yielded an additional 48 cases of primary pediatric spinal ATRTs reported in the English-language literature. Patients (ages 2 months to 19 years) presented with symptoms of pain, regression of motor function, and spinal cord compression. The majority of tumors were intradural (14 extramedullary, 8 intramedullary, 1 both). Eleven cases in the literature described tumors limited to extradural structures, while 10 tumors involved the intra- and extradural spine. Four reports did not specify tumor location. Although rare, spinal ATRT should be considered in the differential diagnosis of pediatric patients presenting with a new spinal mass.