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Ryosuke Mizuta, Shoji Saito, Keita Kawabe, Hiroki Seto, Tomoaki Suzuki, Masakazu Sano, Hitoshi Hasegawa, and Makoto Oishi

BACKGROUND

Giant cell arteritis (GCA) infrequently presents with progressive symptomatic vertebrobasilar stenosis. Vertebrobasilar GCA is often refractory to medical treatments and can lead to short-term ischemic stroke recurrence, which is associated with a poor prognosis. Endovascular treatment (EVT) is a therapeutic option; however, the optimal timing and indications for its application remain unclear.

OBSERVATIONS

This study reports two patients with vertebrobasilar GCA who exhibited repeated ischemic strokes in the vertebrobasilar territory, along with progressive severe stenosis and occlusion of the bilateral vertebral arteries, despite receiving medical therapy. They were successfully treated with balloon angioplasty, and there were no subsequent occurrences of stroke or restenosis.

A review was conducted of six cases of vertebrobasilar GCA treated with EVT. All patients had bilateral lesions and experienced recurrent strokes within 30 days. Angiography suggested ischemic complications in vertebrobasilar GCA resulting from hemodynamic ischemia caused by stenosis rather than intradural vasculitis. The improved blood flow through EVT alleviated patient symptoms and prevented recurrent strokes.

LESSONS

Some patients with vertebrobasilar GCA exhibit rapid stenosis progression and repeated hemodynamic ischemia despite medical therapy. EVT is a potential strategy for treating medically refractory vertebrobasilar GCA. Performing EVT prior to recurrent infarctions can lead to favorable outcomes.

https://thejns.org/doi/10.3171/CASE24404

Galadu Subah, Sabrina L Zeller, Aarti Kishore Jain, Nimrod Gozum, Garrett Smith, Jared Cooper, George M Kleinman, Chirag D Gandhi, and Simon J Hanft

BACKGROUND

Glioblastoma multiforme (GBM), a high-grade primary brain tumor, presents a formidable challenge in neuro-oncology because of its aggressive nature, infiltrative growth, and limited response to treatment. The septum pellucidum represents an uncommon and unexpected location for GBM, adding complexity to the diagnosis and management of this rare intracranial malignancy.

OBSERVATIONS

A 69-year-old male with a previous history of prostate carcinoma presented to an outside hospital with a 2-week history of a “trance-like state” and cognitive decline. Initial head computed tomography showed prominent ventricles without distinct mass lesions. Upon admission, magnetic resonance imaging demonstrated a mass within the inferior septum pellucidum extending to the third and both lateral ventricles. Biopsy findings indicated a GBM, World Health Organization central nervous system tumor grade 4, with immunopositivity for glial fibrillary acidic protein and a Ki-67 labeling index of 60%–70%.

LESSONS

Identifying only 5 cases in more than 60 years of literature, this systematic review illustrates the diverse clinical presentations, diagnostic advancements, and management approaches for septum pellucidum GBM.

https://thejns.org/doi/10.3171/CASE23770

Ian J Jarin, Yigal Samocha, John K Houten, and Merritt D Kinon

BACKGROUND

Traumatic dural lacerations can be caused by thoracolumbar burst fractures and, infrequently, can be associated with the entrapment of neural elements within a laminar fracture. The diagnosis of both is difficult to make on preoperative imaging, as the conditions are typically appreciated during surgical exploration. The authors present a case of traumatic durotomy with entrapment of neural elements in a laminar fracture that they believed could be appreciated on preoperative magnetic resonance imaging (MRI).

OBSERVATIONS

A young male involved in a motor vehicle collision presented to the authors’ trauma center with a lumbar burst and laminar fracture. Preoperative MRI demonstrated epidural hemorrhage and entrapment of neural elements within the laminar fracture, which was confirmed intraoperatively. The patient underwent a successful decompression, release of nerve roots, fusion, and recovery.

LESSONS

Traumatic durotomy and entrapment of neural elements can occur after a traumatic spinal fracture, and a diagnosis made upon preoperative MRI can allow for effective preoperative planning. Clinicians should have a high index of suspicion for these pathologies when encountering certain fracture patterns and could therefore tailor the surgical approach to avoid further neurological injury during surgery.

https://thejns.org/doi/10.3171/CASE24455

Anthony L. Asher

Physicians generally underestimate their potential to influence social progress, despite substantial precedents for medical professionals leading important societal transformations. The author believes that our times require we challenge the notion that physicians have limited influence beyond clinical settings. Our voice is powerful and important. It needs to be heard.

In light of these considerations, the author chose "What Matters" as the theme of the 2024 American Association of Neurological Surgeons (AANS) Annual Scientific Meeting held in Chicago on May 3–6. The topic was reflective of his personal conviction that physicians, by virtue of the public respect and goodwill maintained through their dedication to the art and science of medicine, possess unparalleled potential to enhance both individual and societal health and well-being.

The 2024 AANS scientific program committee determined to create an unprecedented program that would allow the community of neurosurgery to engage with some of the greatest minds in American medicine, academics, journalism, technology and public life. All contributors were invited to weave their insights regarding what truly matters into a broad, thought-provoking intellectual and spiritual tapestry. Universally resonant themes such as empathy, innovation, resilience, leadership, value, trust and equity framed the cooperative dialogues, emphasizing our shared humanity and the core values uniting us—despite our differences.

The objectives for the 2024 Annual Meeting were ambitious: to consider as a professional community themes of utmost importance to our professional and personal lives; to catalyze a profound reevaluation of our collective objectives; to envision an expanded common mission; and to inspire visionary leaders to collaborate on creating lasting value—both for the patients who are the principal focus of our shared devotion, and for society writ large.

Rachel C. Jacobs, Akanksha Chilukuri, Hussam Abou-Al-Shaar, Joseph H. Garcia, Prateek Agarwal, Michael M. McDowell, Alhamza R. Al-Bayati, and Stephanie Greene

OBJECTIVE

Arteriovenous malformations (AVMs) are the most common cause of spontaneous intracranial hemorrhage (ICH) in children, often leading to devastating complications. The current literature from the adult AVM population suggests that both younger age and associated aneurysms carry an increased risk of hemorrhagic presentation. However, detailed analysis of pediatric AVM-associated aneurysms and their link to ICH is relatively unknown, with the literature largely consisting of case reports. This study aimed to determine whether AVM-associated aneurysms predispose pediatric patients to ICH.

METHODS

A retrospective cohort study of 238 pediatric patients with AVMs who presented to the Children’s Hospital of Pittsburgh from 1988 to 2023 was performed. Hospital records, patient charts, and radiographic imaging studies were reviewed for patient demographic characteristics, presentation status, and AVM architecture.

RESULTS

Of the 238 total patients, 44 (18.5%) children with AVM had associated aneurysms. There were 19 (38.8%) feeding artery aneurysms, 8 (16.3%) intranidal aneurysms, 21 (42.9%) postnidal aneurysms, and 1 (2.0%) unrelated aneurysm of 49 aneurysms. Five patients had venous varices. One hundred forty (58.8%) children presented with hemorrhage. Twenty-one of 44 (47.7%) patients with an AVM-associated aneurysm presented with hemorrhage, whereas 119 of 194 (61.3%) with a solitary AVM presented with hemorrhage (p = 0.10). On multivariate analysis, postnidal aneurysm (OR 0.36, p = 0.037) and an increased number of draining veins (OR 0.66, p = 0.049) were significantly associated with a decreased likelihood of hemorrhagic presentation. Deep venous drainage was associated with an increase in hemorrhagic presentation (OR 2.25, p = 0.0045) on multivariate analysis.

CONCLUSIONS

Approximately one-fifth of children with AVMs in this study had accompanying aneurysms, and in this patient population, those with postnidal aneurysms and increased number of draining veins had a decreased incidence of hemorrhage on presentation. Feeding artery and intranidal aneurysms were not associated with an elevated risk of hemorrhagic presentation.

Husain Shakil, Armaan K. Malhotra, Ahmad Essa, Alexander P. Landry, Suganth Suppiah, Arjun Sahgal, Nicolas Dea, Gelareh Zadeh, Michael G. Fehlings, Christopher D. Witiw, and Jefferson R. Wilson

OBJECTIVE

This study estimates the incidence, treatment patterns, and overall survival for patients with chordoma treated in Ontario.

METHODS

A 17-year (2003–2019) population-based cohort study was conducted, including all patients in the Ontario Cancer Registry with histologically proven chordoma. Primary outcomes of interest were age-standardized annual incidence, overall survival, and rates of radiation therapy, chemotherapy, and open resection.

RESULTS

A total of 208 patients were diagnosed with chordoma over the study period: 97 patients with skull base chordoma, 37 with mobile spine chordoma, and 65 with sacropelvic chordoma. A total of 133 patients were treated with either open or endoscopic surgery, of whom 99 were also treated with some form of radiation therapy. Across the 17-year study period, the average annual age-standardized incidence was 12.04 cases per 10 million (95% CI 9.31–14.78 cases per 10 million). There was no significant change in the annual incidence rate over the study period (average annual percent change 2.27, 95% CI −1.74 to 6.44; p = 0.25). The odds of receiving radiation therapy or chemotherapy significantly increased by 8% per year (95% CI 1%–16% per year, p = 0.036) over the study period. The odds of receiving open resection significantly decreased by 14% per year (95% CI 8%–20% per year, p < 0.001). The odds of receiving endoscopic surgery among patients with skull base chordoma increased by 38% per year (95% CI 22%–60% per year, p < 0.001), while the odds of patients receiving biopsy alone did not change significantly over the study period (p = 0.684). After diagnosis of chordoma, the 5-, 10-, and 15-year overall survival probabilities were 0.74 (95% CI 0.69–0.81), 0.58 (95% CI 0.51–0.67), and 0.48 (95% CI 0.40 to 0.59), respectively. There was no significant association between hazard of death and year of diagnosis (p = 0.126) or anatomical location (p = 0.712, skull base vs mobile spine chordoma; p = 0.518 skull base vs sacropelvic chordoma).

CONCLUSIONS

Chordoma is a rare disease with no significant change in the average annual incidence rate between 2003 to 2019. During this time, treatment with less invasive modalities increased, particularly for skull base chordoma. Overall survival exceeds 10 years for many patients, with no change in the hazard of death across the study period.

Tracy M. Flanders, Maria A. Punchak, Edward R. Oliver, Sierra D. Land, Sabrina J. Flohr, Tom A. Reynolds, Katie M. Schmidt, Danielle D. Ertz, Julie S. Moldenhauer, N. Scott Adzick, and Gregory G. Heuer

OBJECTIVE

Hindbrain herniation (HH) is a clinical prerequisite for prenatal repair of myelomeningocele/myeloschisis; however, a subset of patients lack HH on initial fetal imaging and may ultimately progress to exhibit herniation on subsequent prenatal or postnatal imaging. The authors sought to explore the cohort of patients without HH at the time of initial fetal consultation for myelomeningocele/myeloschisis repair to define their clinical characteristics and outcome.

METHODS

From July 2016 to July 2022, patients evaluated at the Children’s Hospital of Philadelphia Center for Fetal Diagnosis and Treatment for myelomeningocele/myeloschisis were classified into two cohorts: those with and those without HH. The diagnosis of HH was obtained from prenatal and postnatal MRI. The osseous lesion level, prenatal sac volume, and prenatal ventricular size was obtained from fetal ultrasound. The fronto-occipital horn ratio was measured on the first postnatal ultrasound. Ambulation status was obtained from postnatal evaluation in the spina bifida clinic.

RESULTS

A total of 176 patients with prenatal HH had postnatal follow-up, of whom 95 (54%) had HH resolution and 81 (46%) had herniation persistence. Of 73 patients without prenatal HH, 9 (12%) had herniation on subsequent prenatal imaging while 64 (88%) had no herniation on prenatal imaging. Of these 64 patients, 11 (17%) had postnatal HH, 32 (50%) had no postnatal herniation, and 21 (33%) were lost to follow-up or the pregnancy was terminated. For patients without HH throughout, the sac volume was larger (9 cm3) than those who had herniation progression or initial herniation; however, the rate of talipes was not significantly different among the groups. The majority of patients were also ambulators (with assistive devices or independent), and the atrial diameter was also < 10 mm for most patients. Overall, 53% of those with initial HH compared with 35% with progression of herniation required CSF diversion, while only 25% of those without herniation required diversion.

CONCLUSIONS

This study demonstrates the natural history of HH in patients with a prenatal diagnosis of myelomeningocele/myeloschisis. The majority of patients without any herniation had larger sac sizes but not higher rates of talipes and smaller ventricles and were ambulatory. These findings improve the ability to guide families during prenatal consultation.

Dong-Ho Lee, Sehan Park, Chang Ju Hwang, and Jae Hwan Cho

OBJECTIVE

Cord compression by the ligamentum flavum (CCLF) has been reported to adversely affect the clinical outcomes of anterior cervical discectomy and fusion (ACDF). While indirect decompression does occur for foraminal stenosis with ACDF, whether ACDF could improve CCLF with the distraction of disc space remains unclear. This study aimed to identify 1) whether indirect decompression occurs for CCLF with ACDF, and 2) risk factors that hinder the improvement of CCLF.

METHODS

This retrospective cohort study included 119 patients who underwent ACDF for the treatment of cervical myelopathy and CCLF was detected on preoperative MRI. Patients who demonstrated improvement in CCLF grade after ACDF were included in the improved group, while those who did not show improvement were classified as the unimproved group. Patient characteristics, cervical sagittal parameters, neck and arm pain visual analog scale score, and Japanese Orthopaedic Association (JOA) score were assessed. A comparison between the improved and unimproved groups was performed. Regression analyses were performed to identify factors associated with CCLF grade improvement.

RESULTS

Overall, 58.0% (69/119) of patients showed improvement in CCLF grade after ACDF. CCLF grade did not improve in the remaining 42.0% (50/119) of patients, and 3.4% (4/119) of patients experienced aggravation of CCLF after ACDF. Preoperative spondylolisthesis (OR 0.252, 95% CI 0.090–0.711; p = 0.009) and greater segmental lordosis 3 months postoperatively (OR 0.835, 95% CI 0.731–0.953; p = 0.008) were the factors that hindered the improvement of CCLF after ACDF. Furthermore, patients with higher pre- or postoperative CCLF grades showed significantly less improvement in JOA score 2 years postoperatively.

CONCLUSIONS

Indirect decompression for CCLF with ACDF is not reliable because 42.0% of patients did not demonstrate improvement in CCLF grade after the operation. Preoperative spondylolisthesis and postoperative increased segmental lordosis were risk factors for failure of CCLF improvement. Both pre- and postoperative higher CCLF grades were associated with poor neurological recovery 2 years postoperatively.

José Roberto Tude Melo, Isabela Zampirolli Leal, Larah Domingos de Oliveira, Melina Houlis Hao Masini, Jean Gonçalves de Oliveira, and José Carlos Esteves Veiga

OBJECTIVE

Considering Glasgow Coma Scale (GCS) scores and brain CT scans in a group of children and adolescents with traumatic brain injury (TBI), the scope of this study was to identify the criteria established for the indication of emergency neurosurgical treatment in a level 1 trauma center.

METHODS

This was a cross-sectional study with consecutive review of medical records of children and adolescents < 17 years with TBI who were hospitalized and underwent neurosurgical treatment between January 2016 and December 2023. Two groups were formed for analysis: patients with GCS scores ≤ 8 versus patients with GCS scores > 8. Based on the GCS score and brain CT scan of each patient, the authors investigated the criteria established for the surgical indications in this group.

RESULTS

In the period considered for the study, 376 children and adolescents with TBI were hospitalized and 31% required neurosurgical treatment. The median age was 5 years (interquartile range 1–11 years) and there was a predominance of males (68%). Home accidents predominated in 77% of children < 5 years of age, whereas road accidents predominated among those older than 5 (47%). Diffuse brain lesions on CT scans predominated in patients with GCS scores ≤ 8 when compared to the group with GCS scores > 8 (89% vs 19%; p < 0.0001). Regarding neurosurgical access, decompressive craniectomies (70%) and invasive intracranial pressure monitoring (44%) prevailed among patients with GCS scores ≤ 8, whereas craniotomies for drainage of intracranial hematomas (70%) and surgical correction of depressed skull fracture (21%) prevailed among those with GCS scores > 8.

CONCLUSIONS

Based on the GCS scores and CT scans, the authors were able to define the criteria used for neurosurgical indications in a Brazilian level 1 trauma center. They found a high prevalence of decompressive craniectomy in patients with severe TBI in their department due to the irregular supply of disposable catheters necessary for intracranial pressure monitoring.

Zerubabbel K. Asfaw, Hodan Abdi, Kaleab Tesfaye Moges, Yemisirach B. Akililu, Ernest J. Barthélemy, Tsegazeab Laeke, Isabelle M. Germano, and Abenezer Tirsit

OBJECTIVE

Pediatric neurosurgical practice is prevalent in most low- and lower-middle-income countries but lacks comprehensive documentation of practice patterns, demographics, and case variety. This study aimed to present the current state of pediatric neurosurgery in Ethiopia, including workforce characterization, case variety, and relevant procedures.

METHODS

A survey was developed and distributed to all Ethiopian fully trained neurosurgeons (n = 50). Survey questions assessed sociodemographic variables, level of training, case variety, and neurosurgical practice. Statistical analysis was conducted to describe the current practice of pediatric neurosurgery.

RESULTS

A total of 45 neurosurgeons responded (90%). Three respondents (7%) were women. There was only 1 fellowship-trained pediatric neurosurgeon, while most neurosurgeons were general neurosurgeons who served a pediatric patient population. Most neurosurgeons (56%) worked in the capital city, Addis Ababa, while another 13% worked in other urban settings. The top three indications for a pediatric neurosurgical procedure were neural tube defects (NTDs) (96%), hydrocephalus (93%), and trauma (60%). NTD-associated hydrocephalus was the most common hydrocephalus type seen (71%). The most common procedure for hydrocephalus was shunt insertion (96%). A prenatal diagnosis of NTD was made in < 10% of cases, as reported by 84% of respondents.

CONCLUSIONS

The study highlights Ethiopia’s need for more pediatric neurosurgeons. Suggested strategies to facilitate subspecialty training include the establishment of a fellowship program facilitated by the implementation of a nationwide pediatric neurosurgery registry. Promoting efforts for early diagnosis and treatment of pediatric conditions coupled with NTD prevention initiatives could improve pediatric neurosurgical care in Ethiopia.