Transvenous Embolization for Isolated Superior Petrosal Sinus Dural Arteriovenous Fistula.

Isolated superior petrosal sinus dural arteriovenous fistula (SPSdAVF) is a rare condition for which transvenous embolization is a safe treatment, even if accessing the isolated sinus can be challenging. A 39-year-old female patient with dizziness and right facial palsy underwent magnetic resonance imaging, revealing a venous infarction at the posterior fossa and a dural arteriovenous fistula. Digital subtraction angiography showed an isolated SPSdAVF. The shunt point was posterior to the isolated superior petrosal sinus, and the shunt flowed only through the petrosal vein. Contrast-enhanced magnetic resonance imaging showed thrombosis at the anterior segment of the superior petrosal sinus. Transvenous embolization was successfully performed via the thrombosed anterior segment of the superior petrosal sinus without associated complications. This case shows that transvenous embolization through a thrombosed superior petrosal sinus is an alternative treatment option for isolated SPSdAVF.

Neoadjuvant Therapy with Everolimus for Subependymal Giant Cell Astrocytoma: A Case Report.

Direct surgical resection remains to be the standard treatment for tuberous sclerosis complex (TSC) with subependymal giant cell astrocytoma (SEGA). Medical therapy with everolimus (mammalian target of rapamycin inhibitor or mTOR) serves as a second-line treatment for patients with SEGA who are determined to be ineligible for surgical resection. Some recent studies have reported that neoadjuvant therapy for SEGA may be a useful, novel treatment. In this study, we herein present a case of SEGA and demonstrate the efficacy of preoperative everolimus therapy. We have also examined the utility and safety of neoadjuvant therapy for SEGA and investigated four previously reported cases of preoperative administration of mTOR inhibitors. In these cases, everolimus was administered preoperatively to shrink the tumor although the duration of treatment varied. Afterward, gross total tumor removal was conducted in all the cases. No postoperative complications were reported during the follow-up period. These findings indicate that neoadjuvant therapy with an mTOR inhibitor can be a potential treatment for SEGA. The findings of this present study also suggested that a short administration period of about 2 months may be sufficient to achieve preoperative tumor reduction.

Repeat endoscopic third ventriculostomy combined with choroid plexus cauterization as salvage surgery for failed endoscopic third ventriculostomy.

PURPOSE: Although the endoscopic third ventriculostomy (ETV) is an effective treatment for hydrocephalus, failures do on occasion occur. In such cases, a repeat ETV or shunt insertion is usually performed. However, there is, as of yet, no clear consensus on the best measure to take in the event of a failed ETV. We herein examined the outcomes of a repeat ETV combined with choroid plexus cauterization for ETV failure. METHODS: All patients who underwent an ETV at the Department of Neurosurgery at Tokyo Metropolitan Children's Medical Center between April 2013 and March 2019 were retrospectively analyzed. RESULTS: In total, 36 patients received an ETV. Six patients experienced ETV failure; three of these underwent a repeat ETV combined with choroid plexus cauterization. Three of the six patients who experienced early ETV failure received a ventriculoperitoneal shunt. During the median follow-up period of 42 months (range: 32-73 months), all repeat ETVs were successful. CONCLUSION: A repeat ETV combined with choroid plexus cauterization can be an effective salvage therapy in the event of ETV failure.

Flow diverter stents for pediatric traumatic carotid cavernous fistula: a case report and literature review.

A 10-year-old, male patient with a head injury caused by a fall presented with chemosis, exophthalmos, right orbital bruit, and intracranial venous reflux, based on which posttraumatic carotid cavernous fistula (CCF) was diagnosed. Coil embolization was semi-urgently performed for the dangerous venous drainage. After the treatment, right abducens nerve palsy newly appeared. To treat the neurological symptoms and preserve the parent artery, curative endovascular treatment using a pipeline embolization device (PED) with coil embolization was performed after starting dual antiplatelet therapy (DAPT). The CCF and abducens nerve palsy finally resolved, and the internal carotid artery (ICA) was remodeled. Use of the PED with adjunctive coil embolization was effective and safe in the present case of pediatric traumatic direct CCF.

Transependymal Edema as a Predictor of Endoscopic Third Ventriculostomy Success in Pediatric Hydrocephalus.

BACKGROUND: The Endoscopic Third Ventriculostomy Success Score (ETVSS) is based on the clinical features of hydrocephalus except for radiological findings. A previous study suggested that transependymal edema (TEE) as a radiological finding may be a reliable predictor of endoscopic third ventriculostomy (ETV) success in patients of all ages. We aimed to investigate whether TEE on preoperative magnetic resonance imaging can predict ETV success in pediatric patients. METHODS: Medical and radiological records of all pediatric patients with an initial ETV in our hospital between 2013 and 2019 were retrospectively reviewed. RESULTS: This study included 32 patients with hydrocephalus. The median age at surgery was 10.0 years (interquartile range: 5.6-12.9 years). There were 20 patients in the high ETVSS (90-80) group and 12 patients in the moderate ETVSS (70-50) group. The median follow-up period was 29.0 months (interquartile range: 12.9-46.2 months). The ETV success rate at the final follow-up was 81%. Preoperative brain magnetic resonance imaging revealed TEE in 20 patients and third ventricle floor ballooning in 25 patients, of whom 19 (95%) and 22 (88%), respectively, achieved successful ETV. Patients with TEE had a significantly better outcome than patients without TEE (95% vs. 58%, P = 0.018). Multivariate analysis demonstrated that the presence of TEE (odds ratio 13.6, 95% confidence interval 1.3-137.5, P = 0.027) is a significant predictor of ETV success. CONCLUSIONS: In our cohort with a high or moderate ETVSS, the ETV success rate in patients with TEE was significantly higher than in patients without TEE, suggesting that TEE may be a useful predictor of ETV success in pediatric hydrocephalus.

Clinical Features of Bilambdoid and Sagittal Synostosis (BLSS): A Retrospective Multicenter Study in Japan.

ABSTRACT: Bilambdoid and sagittal synostosis (BLSS), a rare form of multisutural craniosynostosis, is sometimes known as the Mercedes-Benz syndrome due to the appearance of the fused sagittal and bilateral lambdoid sutures. Although previous studies have described some of its clinical features, the pathology of this disease is not yet fully understood. Moreover, it has been pointed out that BLSS is more common among individuals of Hispanic ethnicity, but its incidence in Asia remains unclear. In the present study, BLSS cases in Japan were analyzed to determine the characteristics of the condition in Japan. Three hospitals in Tokyo participated in the present study. Patients with BLSS who underwent cranial remodeling were included. Data on patient demographics, clinical symptoms, status of the cranial sutures, morphological subclassification, surgical procedures, developmental status, and genetic mutations were analyzed. In total, 22 patients met the enrollment criteria and were included, indicating a higher incidence of BLSS in Japan than in other nations reported in previous studies. In terms of morphological subclassification, there were 15 brachycephalic, 4 dolichocephalic, and 3 normocephalic. For the initial cranial procedure, 7 patients underwent a single-stage cranioplasty, 13 underwent a posterior distraction, and 2 underwent lateral expansion. Patients with a normocephalic cranial morphology tended to undergo surgery at an older age than patients with the other two types. Appropriate timing for surgery is important for healthy development; hence, surgeons should remember that BLSS can lead to "balanced dysmorphism" that may have led to a delay in diagnosis due to its normal-looking morphology.

Refractory cerebral infarction in a child with an ACTA2 mutation.

INTRODUCTIONS: A specific mutation in the ACTA2 gene is known to cause multisystemic smooth muscle dysfunction syndrome, which is associated with cerebrovascular diseases and various organ disorders. Cerebral infarctions resulting from severe vasculopathy can be refractory; however, there are no previous reports describing the detailed clinical course of recurrent cerebral infarctions due to an ACTA2 mutation. Herein, we report a patient with an ACTA2 mutation who experienced multiple refractory cerebral infarctions in early childhood. PATIENT DESCRIPTION: The patient was aged 1 year and 5 months at her first episode of cerebral infarction. Arteriopathy due to an ACTA2 mutation was diagnosed based on the characteristic cerebrovascular findings and abnormal physical findings, such as bilateral dilated pupils. Bilateral encephaloduroarteriosynangiosis and encephalogaleosynangiosis were performed after the first episode. Because the cerebral infarctions recurred postoperatively, administration of cilostazol followed by bosentan was started. However, despite these treatments she experienced seven cerebral infarctions by age 2 years and 6 months. INTERPRETATION: Cerebral infarctions in patients with a specific ACTA2 mutation can occur even in early childhood, recur frequently, and cause severe motor and cognitive impairment. Physicians should be highly aware of this disease and be ready to provide the medical and surgical interventions necessary to minimize the disabling sequelae.

Ruptured Mycotic Cerebral Aneurysm Secondary to Disseminated Nocardiosis.

We report a case of a ruptured mycotic cerebral aneurysm caused by Nocardia infection. A 22-year-old immunocompromised woman with adult-onset Still's disease developed a subarachnoid hemorrhage (SAH). Digital subtraction angiography revealed a small aneurysm at the M2-3 bifurcation of the right middle cerebral artery. Cardiac ultrasonography showed vegetation at the posterior cardiac wall, suspecting infective endocarditis (IE). Gram-positive filamentous bacteria were observed in the necrotic tissue surrounding the aneurysm obtained during trapping surgery. Long-term blood culture showed that the cause of her cerebral mycotic aneurysm was nocardiosis. A mycotic ruptured cerebral aneurysm is an important cause of SAH in immunocompromised patients. Early diagnosis of IE, detection of gram-positive rods by Gram staining, and long-term culture to identify the bacteria is crucial in diagnosing nocardiosis.

A Single Center Experience with Coil Embolization for Cerebral Aneurysms Greater than 10 mm in the Internal Carotid Artery.

We investigated endovascular treatment for 10 mm or larger aneurysms in the internal carotid artery (IC), including the cavernous portion, the paraclinoid portion, and the posterior communication artery (PC). Between 2011 and 2014 at our hospital, there were 35 cases of aneurysms that were 10 mm or larger in the carotid artery. We analyzed these 35 cases retrospectively based on the size and location of the aneurysms, method of treatment, number of coils implanted, use of a stent, complications, rupture after treatment, ophthalmologic symptoms, and need for re-treatment. There was no bleeding after treatment. Of the 35 cases, four cases (11%) had permanent complications. Re-treatment was indicated in 11 cases (31%), including eight cases localized in the paraclinoid portion, two cases in the IC-PC, and one case in the cavernous portion. Among these re-treatment cases, two cases required a third treatment. Of the 16 cases with paraclinoid aneurysms, half required re-treatment. Of the 12 cases with ophthalmologic symptoms prior to treatment, 9 (75%) improved or had no change and 3 (25%) became worse. There were no complications in the 13 re-treatment procedures. Re-treatment is not uncommon, and a scheduled follow-up is needed. Coil embolization has been one of the main options for aneurysms that are 10 mm or larger in the IC. In the future, these large aneurysms will be treated with a flow diverter stent (FD).

Is Simpson grade I removal necessary in all cases of spinal meningioma? Assessment of postoperative recurrence during long-term follow-up.

It is generally accepted that the first choice of treatment for spinal meningiomas is "radical" surgical removal. However, Simpson grade I removal is sometimes difficult, especially in cases with ventral dural attachment, because of the risk of spinal cord damage or the difficulty of dural repair after radical resection. In addition, there is no consensus on a surgical strategy for radicality, whether or not Simpson grade I resection should be performed in all cases of spinal meningioma. In this study, we retrospectively analyzed clinical and radiological data of surgically treated 14 patients with spinal meningioma, to assess the influence of the Simpson grade to tumor recurrences during long-term follow-up (median 8.2 years, 1.3-27.9). The number of patients in Simpson grades I, II, III, and IV were 2, 8, 0, and 3, respectively; Simpson grading was not applicable to one patient with non-dura-based meningioma. No postoperative permanent neurological worsening was encountered. The recurrence rate was 21.4% (3 out of 14 cases). Of these 3 recurrent cases, 1 was a case of non-dura-based meningioma and another was a case of neurofibromatosis type 2 (NF2); both of them are known as risk factors for recurrence after surgical removal of spinal meningiomas. Considering this background of these two recurrences, the clinical results of the present study are consistent with previous results. Therefore, we propose that surgeons do not always have to achieve Simpson grade I removal if dural repair is complicated and postoperative cerebrospinal fluid (CSF) leakage or neurological worsening are estimated after resection of dural attachment and repair of dural defect.

Decrease in the apparent diffusion coefficient in peritumoral edema for the assessment of recurrent glioblastoma treated by bevacizumab.

PURPOSES: Anti-edema effect of bevacizumab was evaluated using the apparent diffusion coefficient (ADC) of peritumoral edema associated with regional cerebral blood flow (rCBV) of the tumor. MATERIALS AND METHODS: Nine patients with recurrent glioblastoma were treated using bevacizumab for 4 ∼ 36 months (average 12 months). MRI was performed every 2 months. For each MRI, ADC value, Gd-enhanced area on T1 imaging, area of peritumoral edema on T2 imaging, and rCBV on perfusion imaging were measured. ADC and rCBV values were determined by the use of regions of interest positioned in areas of high signal intensity, as seen on T2-weighted images and ADC maps. RESULTS: After 2 months of bevacizumab treatment, ADC values and rCBV decreased 49 and 32 % respectively, associated with marked diminishment of the Gd-enhanced area compared with pretreatment. After 6 months, in 5 of the 9 cases, the Gd-enhanced area appeared again with no change in the ADC value and rCBV. In the other four cases, the Gd-enhanced area as well as the ADC value and rCBV returned to the initial status. CONCLUSION: The anti-edema effect of bevacizumab for treatment of recurrent glioblastoma that was demonstrated by decreased ADC values and rCBV was dramatic and -prolonged at 6 months even with tumor progression.

Brain metastasis from papillary thyroid carcinomas.

Papillary thyroid carcinoma (PTC) is the most common type of thyroid carcinoma and has a relatively favorable prognosis. PTC brain metastases are rare, occurring in 0.1-5% of cases in previous studies. In the present study, we treated 5 cases of PTC brain metastasis in our institute and retrospectively evaluated these patients. A retrospective database was generated from the patient medical records of our institution for the years between 1976 and 2011. The mean patient age at diagnosis was 64.6 years and the average duration from PTC resection to the detection of a brain metastasis using magnetic resonance imaging (MRI) or computed tomography (CT) was 91.7 months. The patients were treated with various combinations of surgery and radiation therapy. All 5 patients died and the mean overall survival following the diagnosis of a brain metastasis was 9.0 months. One patient succumbed to an intratumoral hemorrhage of the metastatic brain tumor. The remaining patients died following metastasis to other organs. Our findings suggest that PTC brain metastases may occur at the end-stage of patient treatment and result in an unfavorable prognosis. Patients with brain metastases also succumbed to the development of metastases to the fetal organs rather than brain.

Benign spinal meningioma without dural attachment presenting delayed CSF dissemination and malignant transformation.

Benign spinal meningiomas have good prognoses, with low rates of recurrence and no cerebrospinal fluid (CSF) dissemination. However, we experienced an extremely rare case of initially benign non-dura-based spinal meningioma that showed multiple CSF disseminated lesions, which progressed for 14 years. A 29-year-old woman without neurofibromatosis presented with progressing dysesthesia in her lower limbs, low back pain, and intermittent claudication. Magnetic resonance imaging (MRI) showed an intradural extramedullary mass lesion at the Th10/11 level. The patient underwent a tumor resection. Intraoperative findings indicated that the tumor had no dural attachment. Histopathological diagnosis after gross total removal was microcystic meningioma (grade I, WHO 2007). Seven years after the first operation, other lesions appeared at the levels of Th11/12, L1, and L2/3 in MRI. These tumors were slow growing and became symptomatic; thus, a second surgery was performed 14 years after the first operation. The histopathological diagnosis was atypical meningioma (grade II, WHO 2007). Benign spinal meningiomas show CSF dissemination extremely rarely, although some authors have reported non-dura-based intraspinal clear-cell meningiomas showing CSF dissemination. However, even in cases of WHO grade I, neurosurgeons should pay attention to late CSF dissemination and malignant transformation after surgical removal of non-dura-based intraspinal meningiomas.

Surgical technique for idiopathic spinal cord herniation: the Hammock method. Technical note.

Idiopathic spinal cord herniation is a rare disease, and surgical treatment is recommended for patients with motor deficits or progressive neurological symptoms. Surgery is performed to release and reposition the tethered spinal cord. In terms of repositioning and prevention of reherniation, various procedures have been proposed; enlargement of the ventral dural defect, primary closure of the defect with sutures, and insertion of a ventral patch for duraplasty. We treated 3 patients with idiopathic spinal cord herniation, using a ventral patch for duraplasty with an expanded polytetrafluoroethylene pericardial membrane (the Hammock method), and all 3 cases had good clinical outcome. The specific important technical aspects are described and illustrated. If this procedure is performed meticulously under the microscope by following the specific techniques, the Hammock method is safer and more effective for prevention of reherniation than simple enlargement of the dural defect.

Navigation-guided endoscopic biopsy for intraparenchymal brain tumor.

To evaluate the efficacy of intraparenchymal brain tumor biopsy using endoscopy and a navigation system (navigation-guided endoscopic biopsy) as a diagnostic tool, a case series of intraparenchymal tumor biopsies was reviewed. Navigation-guided endoscopic biopsy was applied in 9 cases, stereotactic needle biopsy in 16 cases, and open biopsy with or without navigation system in 34 cases. In all biopsy cases, 84.7% of biopsy points were sampled accurately, and 93.2% of diagnoses by biopsy were correct. Comparison of each type of biopsy showed that the resected volumes in navigation-guided endoscopic biopsy and open biopsy tended to be larger than those in stereotactic biopsy, and the mean operation time for the open biopsy procedure was the longest. To define the most applicable device or examination method to increase sampling accuracy, various factors were analyzed in 59 procedures. Navigation-guided endoscopic biopsy was the most accurate of the three types of biopsy, although the statistical difference was not significant. Older patients, histological diagnosis of high-grade glioma or malignant lymphoma, positive photodynamic diagnosis, and positive intraoperative pathology were significant factors in improving the sampling accuracy. Navigation-guided endoscopic biopsy could provide a larger sample volume within a relatively short operation time. The biopsy can be easily combined with both photodynamic diagnosis and intraoperative pathology, significantly improving the histological diagnostic yield.

Primary cerebellar pilocytic astrocytoma with anaplastic features in a patient with neurofibromatosis type 1 - case report - .

A 70-year-old woman with neurofibromatosis type 1 (NF-1) presented with a primary cerebellar pilocytic astrocytoma (PA) with anaplastic features manifesting as worsening headache and ataxia. Magnetic resonance (MR) imaging on admission showed a diffusely enhanced solid mass in the left cerebellar hemisphere, although MR imaging showed no abnormalities 2 years before admission. Histological examination after gross total removal of the tumor exhibited a biphasic pattern with marked Rosenthal fibers, together with some malignant features including frequent mitoses and invasive growth pattern. The final diagnosis was PA with anaplastic features. Previous PA cases with mitotic activity and endothelial proliferation, and/or palisading necrosis have been classified as anaplastic PA (or PA with anaplastic features). In the present case, the tumor histology corresponded to this designation. The present case indicates that PAs with anaplastic features can occur in patients with NF-1.