David J. Daniels

Curative Onyx embolization of tentorial dural arteriovenous fistulas

OBJECT The authors conducted a study to review their experience with tentorial dural arteriovenous fistulas (DAVFs) treated with transarterial endovascular embolization in which Onyx was used. METHODS The authors reviewed prospectively collected data in 9 patients with tentorial DAVFs treated with Onyx embolization between 2008 and 2011. Information reviewed included clinical presentation, angiographic features, treatment, and clinical and radiologically documented outcome. Clinical follow-up was available in every patient. Radiological follow-up studies were available in 8 of 9 patients (mean follow-up 4.6 months). RESULTS Six of 9 patients had complete angiographic obliteration (in 5 this was confirmed by a follow-up angiogram obtained 3-6 months later), and 2 patients had near-complete obliteration (faint filling of the venous drainage in the late venous phase). One patient had partial obliteration and required surgical disconnection. In all patients with complete obliteration, transarterial embolization was performed through the posterior branch of the middle meningeal artery. There were no procedural complications, and no morbidity or mortality resulted from Onyx embolization. CONCLUSIONS Transarterial Onyx embolization is a valid, effective, and safe alternative to surgical disconnection in many patients with tentorial DAVFs. The presence of an adequate posterior branch of the middle meningeal artery is critical to achieve a microcatheter position distal enough to increase the likelihood of complete obliteration.

Radiosurgery for intracranial dural arteriovenous fistulas (DAVFs): a review

The management of intracranial dural arteriovenous fistulas (DAVFs) is complex and may require a combination of various treatment techniques. Endovascular embolization has recently emerged as the primary definitive treatment modality. However, an increasing body of literature has supported the use of stereotactic radiosurgery as the sole therapeutic modality or in combination with embolization and/or surgery. We review the rationale, results, and complications of stereotactic radiosurgery for intracranial DAVFs.

Synovial sarcoma of the spine: A report of three cases and review of the literature

Background: Synovial sarcoma (SS) is a rare sarcoma with distinct morphologic and genetic features, which, despite its name, does not arise from synovium. While most SSs (>80%) arise in the deep soft tissue of the extremities, up to 5% of these tumors are encountered in the body axis including the spine, mediastinum, retroperitoneum, and head/neck regions. Reports of SS located within the spinal axis have been rare to date. Materials and Methods: We searched the medical records at our institution and found three patients who were diagnosed and treated for SSs involving the spine. We also performed an exhaustive literature search using PubMed to identify all reported cases in the literature. Results: In this study, we report on three SS cases involving the spine. All three cases involved the paraspinal muscles and spinal nerve roots, with one case having a significant leptomeningeal involvement. In two cases, “smaller operations” were performed first because the lesions were thought to be benign, however, when the final pathology identified them as SSs, more radical procedures were performed. Additionally, we identified 14 cases of SSs involving the spine published in the literature and all cases are reviewed here. Conclusions: Due to limited numbers of cases, spine SS long-term outcomes are hard to quantify. The currently accepted standard of treatment for SSs starts with wide surgical excision with negative margins followed by chemotherapy and radiation. We summarize the available literature on spinal SSs and review the current treatment options available for these tumors.

Surgically Induced SMART Syndrome: Case Report and Review of the Literature

BACKGROUND Strokelike migraine attack after radiation therapy is a recently described clinical entity characterized by transient hemispheric dysfunction manifesting as, but not limited to, visuospatial deficits, confusion, hemisensory deficits, hemiparesis, aphasia, seizures, and, most prominently, headache in patients with a history of remote external beam radiation therapy to the brain. The radiographic hallmark on magnetic resonance imaging is the presence of transient, diffuse, unilateral gadolinium enhancement of the cortex with white matter sparing, usually corresponding to the previous radiation field. CASE DESCRIPTION We present a case of strokelike migraine attacks after radiation therapy syndrome diagnosed immediately following a craniotomy and temporal lobectomy for recurrent metastatic tumor resection after prior gamma knife radiosurgery and whole-brain radiation therapy. CONCLUSION SMART syndrome should be considered in the differential diagnosis of postsurgical patients with remote history of cranial irradiation and significant, new transient neurologic deficits not explainable by any other mechanism. It is possible that manipulation of the trigeminal ganglion, or the dura of the Meckel cave, contributed to triggering the manifestations of this syndrome in our patient during the immediate postoperative period.

The addition of duraplasty to posterior fossa decompression in the surgical treatment of pediatric Chiari malformation Type I: A systematic review and meta-analysis of surgical and performance outcomes

OBJECTIVE Surgery is the definitive treatment of Chiari malformation Type I (CM-I). It involves posterior fossa decompression, which can be performed along with C-1 laminectomy, reconstructive duraplasty, or tonsil shrinkage. The aim of this study was to provide an updated systematic review and meta-analysis of the latest available evidence regarding posterior fossa decompression only (PFDO) versus posterior fossa decompression with duraplasty (PFDD) in the treatment of CM-I in children. METHODS A literature search was performed in compliance with the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines for article identification, screening, eligibility, and inclusion. Relevant articles were identified from 6 electronic databases from their inception to April 2016. These articles were screened against established criteria for inclusion into this study. RESULTS From 12 relevant studies identified, 1492 pediatric patients treated via PFDD were compared with 1963 pediatric patients treated by PFDO for CM-I. PFDD was associated with greater overall clinical improvement (p = 0.009), along with longer length of stay (p < 0.0001) and more postoperative complications (p = 0.0001) compared with PFDO. No difference was observed between PFDD and PFDO in terms of revision surgery incidence (p = 0.13), estimated blood loss (p = 0.14), syrinx improvement (p = 0.09), or scoliosis improvement (p = 0.95). CONCLUSIONS It appears that the addition of duraplasty to posterior decompression in the definitive treatment of CM-I in children may alter surgical and performance outcomes. In particular, parameters of overall clinical improvement, length of stay, and postoperative complication may differ between children undergoing PFDD and those undergoing PFDO. Current evidence in the literature is of low to very low quality that, as of yet, has not been able to completely control for inherent selection bias both in study design and surgeon preference. Future, large prospective registries and randomized controlled trials are warranted to validate the findings of this study.

Modulating glioma-mediated myeloid-derived suppressor cell development with sulforaphane

Glioblastoma is the most common primary tumor of the brain and has few long-term survivors. The local and systemic immunosuppressive environment created by glioblastoma allows it to evade immunosurveillance. Myeloid-derived suppressor cells (MDSCs) are a critical component of this immunosuppression. Understanding mechanisms of MDSC formation and function are key to developing effective immunotherapies. In this study, we developed a novel model to reliably generate human MDSCs from healthy-donor CD14+ monocytes by culture in human glioma-conditioned media. Monocytic MDSC frequency was assessed by flow cytometry and confocal microscopy. The resulting MDSCs robustly inhibited T cell proliferation. A cytokine array identified multiple components of the GCM potentially contributing to MDSC generation, including Monocyte Chemoattractive Protein-1, interleukin-6, interleukin-8, and Macrophage Migration Inhibitory Factor (MIF). Of these, Macrophage Migration Inhibitory Factor is a particularly attractive therapeutic target as sulforaphane, a naturally occurring MIF inhibitor derived from broccoli sprouts, has excellent oral bioavailability. Sulforaphane inhibits the transformation of normal monocytes to MDSCs by glioma-conditioned media in vitro at pharmacologically relevant concentrations that are non-toxic to normal leukocytes. This is associated with a corresponding increase in mature dendritic cells. Interestingly, sulforaphane treatment had similar pro-inflammatory effects on normal monocytes in fresh media but specifically increased immature dendritic cells. Thus, we have used a simple in vitro model system to identify a novel contributor to glioblastoma immunosuppression for which a natural inhibitor exists that increases mature dendritic cell development at the expense of myeloid-derived suppressor cells when normal monocytes are exposed to glioma conditioned media.

Familial intracranial dural arteriovenous fistulas.

BACKGROUND AND IMPORTANCE Intracranial dural arteriovenous fistulas (DAVFs) are acquired abnormal communications between dural arteries and veins. Risk factors for development include sinus thrombosis and hypercoagulability, such as occurs in heritable thrombophilias. While there have been reports of other types of vascular anomalies (such as cavernous and arteriovenous malformations) occurring in families, to our knowledge there have been no reports of familial intracranial DAVFs. We describe the first 2 cases of intracranial DAVFs occurring in first-degree relatives. CLINICAL PRESENTATION A 66-year-old woman presented with an 18-month history of bilateral pulsatile tinnitus. Neurological examination was significant for a prominent pulsatile bruit over the left mastoid region. Laboratory studies demonstrated heterozygosity for Prothrombin G20210A mutation. Imaging disclosed a large left Type I Borden DAVF involving the distal transverse-sigmoid sinus junction. She underwent uncomplicated stereotactic radiosurgery to the fistula that led to complete resolution of her tinnitus and the fistula. A 73-year-old woman, the sister of the previous patient, presented with a 24-month history of pulsatile tinnitus affecting the left ear. Laboratory studies demonstrated heterozygosity for the Prothrombin G20210A mutation. Imaging revealed a left Type I Borden DAVF involving the left transverse and sigmoid sinuses. The patients symptoms resolved spontaneously without treatment. Repeat imaging revealed interval involution of the fistula. CONCLUSION We describe 2 sisters who were heterozygous for Prothrombin G20210A mutation and found to have DAVFs. Clinicians should be aware of the potential for these fistulas to congregate in first-degree relatives via heritable thrombophilias such as the Prothrombin G20210A mutation.

Occipitocervical Fusion in Elderly Patients

BACKGROUND Occipitocervical disease (OCD) in elderly patients will become increasingly common as the population ages. Our experience with occipitocervical fusions (OCF) in this population suggests mixed outcomes. METHODS Twenty consecutive patients over 65 years old underwent OCF between 1995 and 2005. A retrospective review of demographic, presentation, surgical and outcome data was performed. RESULTS Twenty patients averaging 75.3 years of age (range 65 to 91) were identified. All patients had evidence of myelopathy; however, the primary surgical indications were progressive spinal cord dysfunction (15), brainstem compression (3), and pain (2). Surgical approach was isolated posterior (9), or anterior transoral odontoidectomy followed by posterior stabilization (11). Overall, surgery improved function modestly; average modified Japanese Orthopedic Association functional score (improved 0.9 grades), average Ranawat Myelopathy Score (improved 0.4 grades), and average Nurick Myelopathy Grade (improved 0.6 grades). However, patients with poor preoperative functional assessment (Ranawat grade ≥ III) had greater neurologic improvement than those with good preoperative function, measured by Nurick grade improvement (1 vs. -0.28; P = .03) and Ranawat grade improvement (0.7 vs. -0.2; P = .03). Additionally, the posterior approach demonstrated significant improvement in Japanese Orthopedic Association functional assessment over patients with anterior/posterior approaches (2.2 vs. -0.3; P = .03), with fewer complications (posterior: 1 minor; anterior/posterior: 1 death, 2 major, 8 minor). Perioperative mortality occurred in 5%, and major morbidity in 10% of patients. CONCLUSIONS Preventing or stabilizing neurologic deficit in patients with OCD may require OCF, despite the patients age. In the elderly population, our data favor using the posterior approach when possible, and demonstrate greater neurologic improvement in patients with poor preoperative function.

Penetrating head injuries in children due to BB and pellet guns: a poorly recognized public health risk

OBJECT Nonpowder guns, defined as spring- or gas-powered BB or pellet guns, can be dangerous weapons that are often marketed to children. In recent decades, advances in compressed-gas technology have led to a significant increase in the power and muzzle velocity of these weapons. The risk of intracranial injury in children due to nonpowder weapons is poorly documented. METHODS A retrospective review was conducted at 3 institutions studying children 16 years or younger who had intracranial injuries secondary to nonpowder guns. RESULTS The authors reviewed 14 cases of intracranial injury in children from 3 institutions. Eleven (79%) of the 14 children were injured by BB guns, while 3 (21%) were injured by pellet guns. In 10 (71%) children, the injury was accidental. There was 1 recognized assault, but there were no suicide attempts; in the remaining 3 patients, the intention was indeterminate. There were no mortalities among the patients in this series. Ten (71%) of the children required operative intervention, and 6 (43%) were left with permanent neurological injuries, including epilepsy, cognitive deficits, hydrocephalus, diplopia, visual field cut, and blindness. CONCLUSIONS Nonpowder guns are weapons with the ability to penetrate a childs skull and brain. Awareness should be raised among parents, children, and policy makers as to the risk posed by these weapons.

Minimally conscious state after ruptured giant basilar aneurysm.

OBJECTIVE To report the clinical and radiologic findings in a case of transient minimally conscious state after rupture and coiling of a giant basilar aneurysm. DESIGN Case report. SETTING Neuroscience intensive care unit. PATIENT A 44-year-old man who developed a transient minimally conscious state in association with perianeurysmal edema in the rostral brainstem and thalamus after rupture and coiling of a giant basilar artery aneurysm. MAIN OUTCOME MEASURE Correlation of clinical and magnetic resonance imaging findings. RESULTS A minimally conscious state and bilaterally symmetric vasogenic edema of the rostral brainstem and thalamus developed 2 days after endovascular aneurysm coiling. The clinical and radiologic abnormalities improved significantly and in parallel during the following 4 weeks. CONCLUSIONS Perianeurysmal vasogenic edema in the brainstem and thalamus can develop after rupture and coiling of a giant basilar artery aneurysm. This process can be transient and can produce dramatic alterations in consciousness that later resolve.