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Introduction: Perioperative stroke is a major complication of revascularization surgery in patients with moyamoya. Vomiting is common after neurosurgical procedures and may result in acute changes in intracranial pressure and cerebral blood flow. We instituted a standardized perioperative nausea and vomiting protocol for children and young adults with moyamoya undergoing indirect bypass surgery at our institution. Hypothesis: Instituting a standardized perioperative nausea and vomiting protocol will be associated with reduction in the number of perioperative strokes in children with moyamoya undergoing indirect bypass surgery. Methods: We retrospectively reviewed consecutive cases of children and young adults with moyamoya who underwent indirect bypass surgery before and after implementation of a new perioperative nausea and vomiting protocol. We compared the rate of strokes in the perioperative period (post-operative day 0 and 1) in the 41 months following implementation (155 surgically treated hemispheres) to 155 surgically treated hemispheres (over 30 months) prior to implementation using Fisher’s Exact test. Results: The mean age prior to implementation was 8.7 years (SD 5.5 years) and 9.9 years (SD 6.2 years) post implementation which was not significantly different (p=0.17). In the 30 months prior to initiation of the perioperative nausea and vomiting protocol there were 5 strokes in 155 surgically treated hemispheres (3.2%). After initiation of the protocol, there were no strokes in 155 surgically treated hemispheres (p = 0.03). Conclusions: Instituting a standardized perioperative nausea and vomiting protocol was associated with reduction in perioperative strokes in children with moyamoya treated with indirect bypass surgery.

Cerebral proliferative angiopathy (CPA) is an entity distinct from that of classical arteriovenous malformations. As such, few reports have considered the long-term follow-up of patients with hemorrhage in CPA. Accordingly, herein the authors present a case of recurrent hemorrhage in CPA with 32 years of follow-up and in so doing summarize the literature of hemorrhagic cases in CPA. A 19-year-old presented with focal awareness seizures and diagnostic work-up revealed a left hemispheric vascular lesion. The patient presented again with intracranial hemorrhage at ages 28, 43 and 51. Angioarchitectural workup revealed intermingled brain parenchyma between vascular spaces, absence of dominant feeders and a clear nidus consistent with CPA. The size and diffuse nature of the lesion deemed it inoperable. Given our case and review of the literature it is apparent that CPA has a high risk of re-hemorrhage in the rare event that hemorrhage does occur.

BackgroundVascular access in neonates is limited by the small size of the femoral artery and the risk of complications, including thrombosis and limb ischemia. Although umbilical artery access has been described, previous reports detail exchange of the umbilical artery catheter (UAC) for a conventional arterial sheath, which can be difficult and cumbersome. We aim to describe direct use of the UAC as the sheath and guide catheter in neonatal patients requiring endovascular treatment for life-threatening intracranial arteriovenous shunts.MethodsWe reviewed all cases between 2016 and 2021 of neonatal intervention using a direct UAC approach. We describe our technique of utilizing UACs for cerebral angiography and neurointerventions in newborns without catheter exchange, allowing for repeated, straightforward endovascular access in neonates across multiple interventions.ResultsSeven consecutive neonates underwent endovascular neurointerventional procedures for the treatment of life-threatening, high-flow arteriovenous shunts using a direct UAC access technique without procedural morbidity. Five of seven patients underwent more than one procedure in the neonatal period, between day 1 and day 10 of life.ConclusionsUse of the UAC itself as a diagnostic catheter for cerebral angiography or as a guide catheter for neurointerventions greatly facilitates endovascular interventions in newborns and is ideal for patients requiring multiple interventions in the neonatal period. This technique helps to mitigate the risk of neonatal femoral artery access and its complications, including thrombosis, dissection, spasm, stenosis, and limb ischemia.

Background Hyperplasia of the choroid plexus represents a rare cause of communicating hydrocephalus in children. Recent work has associated such disease with genetic abnormalities (such as perturbations in chromosome 9). Given such extensive cerebrospinal fluid (CSF) overproduction, patients with choroid plexus hyperplasia often fail CSF diversion and therefore require adjuvant interventions. Case Description We present the case of a male infant with a ventriculoperitoneal shunt and radiographic choroid hyperplasia who presented to our institution with a massive abdominal hydrocele caused by an inability to absorb the significant amount of CSF drainage into the abdomen. Conclusion The child was treated with an endoscopic third ventriculostomy and choroid plexus coagulation; however, he still required CSF diversion via a ventriculoatrial shunt. A genetic workup showed tetraploidy of chromosome 9. We discuss criteria for selection of treatment strategies, including endoscopic third ventriculostomy with choroid plexus coagulation and/or CSF diversion, that may prevent the need for re-operation in select patients with hydrocephalus due to choroid plexus hyperplasia.

ObjectivesTuberculosis is uncommon in the United States and a rare cause of meningitis in children with severe neurologic consequences. Tuberculous meningitis (TBM) is an even rarer cause of moyamoya syndrome with only a handful of cases previously reported.MethodsWe report the case of a female patient who initially presented at 6 years of age with TBM and developed moyamoya syndrome requiring revascularization surgery.ResultsShe was found to have basilar meningeal enhancement and right basal ganglia infarcts. She was treated with 12 months of antituberculosis therapy and 12 months of enoxaparin and maintained on daily aspirin indefinitely. However, she developed recurrent headaches and transient ischemic attacks and was found to have progressive bilateral moyamoya arteriopathy. At age 11 years, she underwent bilateral pial synangiosis for the treatment of her moyamoya syndrome.DiscussionMoyamoya syndrome is a rare but serious sequalae of TBM and may be more common in pediatric patients. The risk of stroke may be mitigated by pial synangiosis or other revascularization surgeries in carefully selected patients.

To test which intracerebral hemorrhage (ICH) characteristics impact incidence of hydrocephalus and characterize subsequent impact on outcomes.A search of the electronic medical record of Sinai Grace Hospital between January 2009 and April 2018 using International Classification of Diseases, Ninth Revision and Tenth Revision codes for ICH identified 847 patients. After excluding patients with hemorrhagic conversion of stroke, subarachnoid hemorrhage, and traumatic hemorrhage, 560 patients remained for analysis. Generalized linear modeling was used to assess variance in modified Rankin Scale (mRS) score and length of stay.Incidence of hydrocephalus on arrival varied with ICH volume (P0.001), intraventricular hemorrhage (IVH) status (P0.001), bleed location (P0.001), and external ventricular drain (EVD) status (P0.001). An EVD was inserted in 47% of patients presenting with IVH (n = 102/217), while 4% of patients without IVH received an EVD (n = 14/343) (P0.001). Hemorrhage locations had different rates of EVD placement: thalamic 43%, basal ganglia 22%, cerebellar 28%, brainstem 21%, lobar 7% (P0.001). Shunt dependency did not vary between bleed locations (P = 0.072). Variance in mRS score was explained by IVH, bleed location, hydrocephalus on arrival, and ICH volumes. In particular, cerebellar hemorrhage location was associated with better outcomes (mean discharge mRS score of 3.3 vs. 3.9, P0.001).Bleed characteristics affect incidence of hydrocephalus on admission, rates of long-term shunt dependency, and outcomes. Hemorrhage location did not predict shunt dependency; however, it did predict outcomes. Specifically, cerebellar ICH was associated with a better discharge mRS score.

Background Aicardi syndrome is a severe neurodevelopmental disorder that occurs primarily in females and is characterized by seizures, agenesis of the corpus callosum, and chorioretinal lacunae, which occur together in the majority of affected individuals. Seizures begin in infancy and tend to progress in intensity and are often refractory to standard multimodal medication treatments. Case Description We present here a unique case of a 12-year-old girl with partial agenesis of the corpus callosum who underwent a corpus callosotomy for treatment of medically refractory epilepsy. In so doing, we also review the literature with regard to the neurosurgical management of these unique patients. Conclusions For the subset of children who present with partial, rather than complete, agenesis of the corpus callosum, corpus callosotomy should be considered as a treatment option to reduce seizure burden.

Background Although still recommended, using intravenous tissue plasminogen activator (IV-tPA) for large vessel occlusions (LVOs) has been questioned in the era of mechanical thrombectomy (MT). We sought to determine the impact of IV-tPA if used before MT. Methods We used a single-institution, prospectively maintained stroke database from July 2017 through June 2019. All patients undergoing MT with or without IV-tPA treatment for LVO with pretreatment computed tomography angiography (CTA) head and neck were included. We compared the initial CTA images of clot location and morphology to the angiographic findings visualized on the first injection before mechanical intervention. Results Eighty patients were included. About a third (33%) received IV-tPA before thrombectomy. Among patients receiving IV-tPA, significantly more, 29% versus 5.6% without IV-tPA, experienced distal clot migration or changes in morphology between first CTA acquisition and first angiographic run before thrombectomy (P = 0.006). On logistic regression IV-tPA was the only significant predictor of clot migration (P = 0.024). Of note, clot migration due to IV-tPA use was not associated with superior recanalization rates or outcomes in this analysis (P = 0.27). Original site clot resolution was noted in 8% (2/24) of patients who received IV-tPA; however, distal M4/5 embolic cutoffs were noted in both patients. Conclusions IV-tPA administration for LVO has a low rate of primary recanalization with risk of distal embolic phenomenon often still requiring MT. No significant changes in patient outcomes were noted in this study due to clot migration. Larger studies will be necessary to determine if IV-tPA plus MT truly benefits entire clot removal versus MT alone.

Background and purposeAneurysm recurrence is the primary limitation of endovascular coiling treatment for cerebral aneurysms. Coiling is currently quantified by a volumetric porosity measure called packing density (pd). Blood flow through a coil mass depends on the permeability of the coil mass, and not just its pd. The permeability of coil masses has not yet been quantified. Here we measure coil permeability with a traditional falling-head permeameter modified to incorporate idealized aneurysms.MethodsSilicone replicas of idealized aneurysms were manufactured with three different aneurysm diameters (4, 5, and 8 mm). Four different coil types (Codman Trufill Orbit, Covidien Axium, Microvention Microplex 10, and Penumbra 400) were deployed into the aneurysms with a target pd of 35%. Coiled replicas were installed on a falling-head permeameter setup and the time taken for a column of fluid above the aneurysm to drop a certain height was recorded. Permeability of the samples was calculated based on a simple modification of the traditional permeameter equation to incorporate a spherical aneurysm.ResultsThe targeted 35% pd was achieved for all samples (35%±1%, P=0.91). Coil permeabilities were significantly different from each other (PConclusionsA simple in vitro setup was developed to measure the permeabilities of coil masses based on traditional permeametry. Coil permeability should be considered when evaluating the hemodynamic efficacy of coiling instead of just packing density. Coils made of thicker wires may be more permeable, and thus less effective, than coils made from thinner wires. Whether aneurysm recurrence is affected by coil wire diameter or permeability needs to be confirmed with clinical trials.

Background: Multiple radiation-induced cavernous malformations of the cauda equina are extremely rare. A review of the literature suggested that the post-irradiation lumbosacral radiculopathy in our patient was most likely associated with a diagnosis of multiple radiation-induced cavernous malformations of the cauda equina. Case Description: A 76-year-old man with a remote history of abdominal radiation therapy presented with a 6-month history of progressively worsening right foot drop and balance impairment. Magnetic resonance imaging (MRI) revealed multiple enhancing areas of the cauda equina concerning for carcinomatous meningitis, however, cerebrospinal fluid (CSF) analysis was unrevealing. Intraoperative findings were consistent with multiple radiation-induced cavernous malformations of the cauda equina. Conclusions: Multiple radiation-induced cavernous malformations of the cauda equina may mimic carcinomatous or infectious meningitis. Clinicians should be suspicious of this diagnosis when CSF and MRI findings are inconsistent with metastatic disease or infectious meningitis in patients who present with radiculopathy and a history of radiation therapy.

OBJECTIVE The aim of this study was to identify and discuss operative nuances utilizing image guidance in the surgical management of aggressive sacral tumors. METHODS The authors report on their single-institution, multi-surgeon, retrospective case series involving patients with pathology-proven aggressive sacral tumors treated between 2009 and 2016. They also reviewed the literature to identify articles related to aggressive sacral tumors, their diagnosis, and their surgical treatment and discuss the results together with their own experience. Information, including background, imaging, treatment, and surgical pearls, is organized by tumor type. RESULTS Review of the institutional records identified 6 patients with sacral tumors who underwent surgery between 2009 and 2016. All 6 patients were treated with image-guided surgery using cone-beam CT technology (O-arm). The surgical technique used is described in detail, and 2 illustrative cases are presented. From the literature, the authors compiled information about chordomas, chondrosarcomas, giant cell tumors, and osteosarcomas and organized it by tumor type, providing a detailed discussion of background, imaging, and treatment as well as surgical pearls for each tumor type. CONCLUSIONS Aggressive sacral tumors can be an extremely difficult challenge for both the patient and the treating physician. The selected surgical intervention varies depending on the type of tumor, size, and location. Surgery can have profound risks including neural compression, lumbopelvic instability, and suboptimal oncological resection. Focusing on the operative nuances for each type can help prevent many of these complications. Anecdotal evidence is provided that utilization of image-guided surgery to aid in tumor resection at our institution has helped reduce blood loss and the local recurrence rate while preserving function in both malignant and aggressive benign tumors affecting the sacrum.

Background: Fungal cervical spondylodiscitis is rare and accounts for less than 1% of all cervical, thoracic, and lumbar vertebral osteomyelitis and discitis. Case Description: A 32-year-old non-immunocompromised male presented with persistent neck pain and paresthesias. The magnetic resonance imaging of the cervical spine demonstrated a contrast-enhancing erosive lesion involving the cervical C6 and C7 vertebral bodies accompanied by epidural phlegmon. Blood culture was negative. The patient underwent a C6 and C7 anterior corpectomy with instrumented fusion (e.g., expandable cage C5 to T1). Intraoperatively, frank pus was noted within the C6-C7 disc space and was accompanied by thick prevertebral and epidural phlegmon extending from C5 to T1. Intraoperative cultures grew Candida albicans. Three days later, a C6-C7 laminectomy with C4-T2 posterior instrumented fusion was performed; the cultures again grew C. albicans. The patient was treated with intravenous micafungin for 14 days followed by 6–12 months of 400 mg oral fluconazole daily. Conclusion: There are few cases in literature where non-immunocompromised patients developed fungal cervical spondylodiscitis. Prompt diagnosis and appropriate management are critical to effectively treat these patients. Surgical intervention may warrant corpectomy, discectomy, and operative debridement followed by long-term targeted antifungal therapy.