Authorship：Lead author   Language：Japanese   Publishing type：Research paper (scientific journal)  

Endoscopy provides a bright, wide field of view in deep surgical corridors, and preserves image quality under continuous irrigation ("underwater" conditions). Pediatric patients tolerate limited blood loss and are vulnerable to retraction injuries; therefore, meticulous hemostasis and brain-sparing techniques are mandatory. As hydrocephalus commonly accompanies pediatric brain tumors, treatment should address both tumor control and cerebrospinal fluid diversion within the same operation. Endoscopy enables access through minimal corridors, and is particularly effective for deep-seated lesions. Safe application requires rigorous preoperative planning and simulation to delineate the lesion, feeding and draining vessels, critical white matter tracts, and deep venous structures near the intended corridor. Patient positioning and operating room setup must be optimized in advance. When underwater techniques are anticipated, the angle of the tubular retractor (cannula/cylinder) should allow the maintenance of a stable fluid column. This article provides a practice-oriented overview of the endoscopic management of pediatric intraventricular and intraparenchymal tumors, emphasizing the importance of planning, workflow discipline, and strategies that minimize blood loss and parenchymal injury, while integrating diagnosis, cytoreduction, and CSF pathway reconstruction.

DOI： 10.11477/mf.030126030530061100

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Authorship：Corresponding author   Language：English   Publishing type：Research paper (scientific journal)   Publisher：The Japan Neurosurgical Society  

Postoperative hematoma formation is one of the most life-threatening complications associated with giant pituitary adenomas, and various surgical methods have been proposed to mitigate this risk. This study aims to report our surgical outcomes in patients with giant pituitary adenomas and to identify risk factors associated with postoperative hematoma formation. We retrospectively reviewed 45 patients with giant pituitary adenomas who underwent surgical treatment. The surgical approaches included conventional transsphenoidal surgery in 24 patients, extended transsphenoidal surgery in 9, and simultaneous combined transsphenoidal surgery and transcranial surgery in 12. Intracapsular resection was performed in 28 patients, while extracapsular resection was carried out in 17 patients. Postoperative hematoma formation was observed in 17 patients; among them, 2 experienced neurological deterioration and subsequently required reoperation for hematoma evacuation. The mean maximum tumor diameter was significantly larger in patients with postoperative hematoma (54 mm) compared to those without (45.3 mm) (p = 0.008). Other tumor characteristics were not significantly associated with postoperative hematoma formation. Combined transsphenoidal surgery and transcranial surgery were more frequently performed in patients who developed postoperative hematoma (p = 0.007), whereas extracapsular resection was more common in those without hematoma (p = 0.001). However, these differences in postoperative hemorrhage incidence among surgical techniques may have been substantially influenced by selection bias. Giant pituitary adenomas with extensive intracranial extension and involvement of critical neurovascular structures remain challenging to manage regardless of the surgical approach. Nevertheless, it is essential to tailor surgical strategies to individual cases to minimize postoperative complications.

DOI： 10.2176/jns-nmc.2025-0106

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：(一社)日本癌治療学会  

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PURPOSE: This study aimed to evaluate the efficacy and safety of intravenous perampanel (IV-PER) in routine clinical practice. METHODS: Patients who received IV-PER at Nagoya University Hospital or one of the 21 affiliated institutions between June 2024 and March 2025 were included. Indications for IV-PER included treatment for epileptic seizures, particularly acute symptomatic seizures, and prophylactic use before or after surgery. The primary endpoint was the seizure suppression rate within 7 days of treatment initiation, and the secondary endpoint was the incidence of adverse events (AEs). Clinical data were recorded daily, and blood tests were conducted within 2 weeks of administration. Enrolled patients were categorized into three groups based on the underlying etiology: cerebrovascular disease (CD), brain tumor (BT), and traumatic brain injury (TBI). Treatment efficacy and safety were assessed across these groups. RESULTS: Of the 237 patients, 74, 116, and 47 were classified into the CD, BT, and TBI groups, respectively. The overall seizure suppression rate within 7 days was 89.0 %. Rates by group were 87.8 % (CD), 96.6 % (BT), and 72.3 % (TBI). When stratified by indication, the rates were 99.3 % for prophylactic use, 69.4 % for focal seizures, and 74.4 % for generalized seizures. Regarding AEs, somnolence was reported in 3 patients and irritability in one. Laboratory abnormalities included anemia, elevated liver enzymes, elevated creatine kinase, and hyponatremia. A total of 71 patients experienced one of these. CONCLUSIONS: This large multicenter prospective registry demonstrates that IV-PER can be safely used for seizure management during the 7-day acute phase in real-world clinical settings.

DOI： 10.1016/j.seizure.2025.07.023

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Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：一般社団法人 日本内分泌学会  

DOI： 10.1507/endocrine.101.s.hpt_11

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Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：一般社団法人 日本内分泌学会  

DOI： 10.1507/endocrine.101.s.hpt_75

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Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：一般社団法人 日本内分泌学会  

DOI： 10.1507/endocrine.101.s.hpt_46

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Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：一般社団法人 日本内分泌学会  

DOI： 10.1507/endocrine.101.s.hpt_39

CiNii Research

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Intracanalicular residual vestibular schwannoma (VS) after surgical resection is known to cause recurrence. Lesions demonstrating progression despite adjuvant stereotactic radiosurgery necessitate salvage surgery with meticulous tumour resection to prevent recurrence. We describe our exo- and endoscopic two-step approach (EETA), employing an exoscope to remove the extracanalicular component and an endoscope to remove the intracanalicular component under direct visualisation of the fundus. EETA is a viable option for treating recurrent VS, as it enables enhanced visualisation of both extra- and intracanalicular lesions, including the fundus.

DOI： 10.1007/s00701-025-06625-1

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Other Link： https://search.jamas.or.jp/default/link?pub_year=2025&ichushi_jid=J01160&link_issn=&doc_id=20250903390023&doc_link_id=10.1507%2Fendocrine.101.S.HPT_75&url=https%3A%2F%2Fdoi.org%2F10.1507%2Fendocrine.101.S.HPT_75&type=J-STAGE&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00007_3.gif

Authorship：Corresponding author   Language：English   Publishing type：Research paper (scientific journal)  

Primary ciliary dyskinesia (PCD) is a congenital disease caused by gene mutations linked to ciliary dysfunction. PCD causes different symptoms, including chronic sinusitis, infertility, situs inversus and hydrocephalus. Motile cilia on ventricular ependymal cells are a crucial factor in cerebrospinal fluid circulation, and dysfunction of these cells causes hydrocephalus. Deleted in primary ciliary dyskinesia (Dpcd) is one genetic abnormality known to cause PCD, and its knockout leads to hydrocephalus in mice. PCD occurs in Dpcd-/- mice because of the lack of an inner dynein arm (IDA) in the motile cilia. However, how this deficiency is associated with the motility of ventricular ependymal motile cilia in Dpcd-/- mice has not been demonstrated. Herein, we show that Dpcd induces partial defects in dyneins and aberrant motility in ventricular ependymal cilia. In Dpcd-/- mice, the ependymal cilia demonstrated decreased amplitude, abnormal waveforms and low cerebrospinal fluid flow velocity. In addition, the amount of dynein axonemal heavy chains in some IDAs decreased in the ependymal cilia. In wild-type mice, Dpcd was localised in the cytoplasm and cilia of ependymal cells. Thus, abnormal ciliary movement in Dpcd-/- mice is likely attributed to a defect in IDA assembly in the ependymal cilia.

DOI： 10.1002/cm.70012

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BACKGROUND: Tumor treating fields (TTFields) treatment has been an important option for the treatment of glioblastoma. The introduction of novel treatment options may lead to distinct recurrence patterns compared to those observed with conventional therapies; however, the specific recurrence pattern during TTFields treatment has not been elucidated. METHODS AND RESULTS: Here, we analyzed 39 cases of glioblastoma treated with TTFields. Although a usage rate of more than 75% is recommended, among 39 cases, 18 discontinued TTFields treatment owing to requests by patients with lower usage rates. In these discontinued cases, patients exhibiting sensory aphasia were more frequently included compared to those who continued TTFields (44.4%, p < 0.001). Among 21 cases involving patients who continued TTFields, tumor recurrence was observed in 15 of those cases. Five out of 15 cases (33.3%) exhibited recurrence in distant parenchyma from the primary lesion. A higher usage rate and relatively longer use of TTFields were observed in these five cases, along with more favorable progression-free survival than those in the other 10 cases (p = 0.019, p = 0.040, and p = 0.024, respectively). In one case, recurrent tumors with lower grade glioma histology but molecular markers characteristic for glioblastoma, IDH-wildtype were indentified. This tumor arose in an area that received a lower local minimum power density of TTFields compared to the primary lesion, following long-term TTFields therapy. CONCLUSIONS: Long-term use of TTFields might be correlated with a high frequency of distant parenchymal recurrence in cases with favorable response.

DOI： 10.1007/s10147-025-02775-5

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Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：日本脳腫瘍病理学会  

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BACKGROUND/OBJECTIVES: Our objective is to develop hormone-producing pituitary cells that can function in the same manner as the human body and provide more effective treatments than current hormone replacement therapy. We have already established a technique for generating hypothalamic-pituitary organoids using feeder-free human pluripotent stem cells (hPSCs) and demonstrated their effectiveness in vivo through transplantation into hypopituitary mouse models. To prospectively determine the upper limit of transplanting adenohypophyseal cells into humans, we investigated the human maximum secretion capacity of adrenocorticotropic hormone (ACTH) and growth hormone (GH). METHODS: We analyzed data from 28 patients with pituitary adenomas, among whom 16 evinced no abnormality of ACTH secretion and 12 showed no GH secretion on corticotropin-releasing hormone (CRH) and growth hormone-releasing hormone-2 (GHRP-2) stimulation testing. RESULTS: The average ACTH peak value after CRH stimulation tests was 97.2 pg/mL, and the average GH peak value after GHRP-2 stimulation tests was 25.1 ng/mL. CONCLUSIONS: These data will likely serve as benchmarks of ACTH and GH secretion when transplanting cultured cells into humans.

DOI： 10.3390/diseases13040112

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Diffuse hemispheric glioma H3 G34-mutant (DHG) has been identified as a distinct pediatric-type high-grade glioma, according to the World Health Organization (WHO) classification of central nervous system tumors. Widely accepted treatment options include surgery, radiation, and conventional chemotherapy. However, the efficacy of the surgical resection remains unclear. Although there are some reports, a comprehensive understanding of the clinical characteristics, pathogenesis, and outcomes of DHG is insufficient to evaluate the efficacy of maximal tumor resection. We retrospectively analyzed nine cases of DHG, focusing on imaging features and progression patterns. Initial Magnetic Resonance Imaging (MRI) revealed T2/FLAIR high lesions with minimal or no contrast enhancement in all cases. The lesions exhibited T2/FLAIR hyperintensities and focal diffusion restriction in the deep white matter, with most showing high methionine accumulation, suggesting deep white matter infiltration at the time of diagnosis. The extent of white matter infiltration in tumor resection cases was significantly negatively correlated with the extent of resection (EOR). In addition, cases with EOR of 90% or more had significantly longer progression-free survival (PFS) and overall survival (OS). However, achieving an EOR of 90% or more was possible in fewer than half of the cases, primarily in those with relatively limited white matter involvement. Histopathological findings of the tumor obtained by initial resection and autopsy revealed extensive deep white matter infiltration, with one patient demonstrating tumor invasion into the brainstem at death. Our study highlights early deep white matter infiltration of DHGs, complicating surgical resection, and potentially contributing to a poor prognosis. While EOR may influence survival to some extent, residual lesions extensively infiltrate the white matter and eventually invade the brainstem and contralateral brain, thereby contributing to mortality. These findings underscore the challenges of managing DHGs and emphasize the need for further research on effective therapeutic strategies, particularly to understand and target their unique progression patterns.

DOI： 10.1186/s40478-025-01945-w

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Intraoperative magnetic resonance imaging (iMRI) plays a crucial role in improving the precision of brain tumor surgeries. However, the use of iMRI can impose certain limitations on intraoperative head positioning. In regular microscopic surgery, head positioning is of utmost importance because an appropriate surgical field is important for the efficacy and safety of surgery. Therefore, in cases where adequate head positioning is difficult, usually, iMRI will not be utilized. Herein, we report an adult case of cerebellar astrocytoma whose tumor extended to the culmen of the cerebellum. Upon surgery via the suboccipital approach, the positional limitations imposed by iMRI led to an insufficient vertex-down position and limited surgical field, which hampered the removal of the upper portion of the tumor. However, this concern could be overcome when used in combination with an endoscope. The potential of iMRI applications is anticipated to be enhanced by overcoming positional limitations through combined endoscopic surgery. The use of multimodality in surgery is an optimal example of how surgical support equipment can also improve surgical outcomes. Here, we report on the new possibilities offered by multimodality.

DOI： 10.18999/nagjms.87.1.168

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Surgical interventions for arachnoid cysts and glioependymal cysts primarily focus on cyst decompression and establishing communication with cerebrospinal fluid (CSF) spaces. However, a standardized surgical strategy for symptomatic convexity cysts lacking surrounding CSF space is still lacking due to the limited number of cases. This systematic review aims to evaluate surgical interventions for symptomatic convexity cysts and proposes a safe and effective treatment approach we have developed. A systematic review of the literature was conducted following PRISMA guidelines. Surgical techniques were categorized into cystoperitoneal (CP) shunt, cyst excision, cyst fenestration, cyst ventriculostomy, and several uniquely modified methods. Using a method different from these previous reports, we performed endoscopic cystoventricular stenting via burr hole in three cases of convexity cysts. While traditional methods have proven effective, CP shunt may lead to malfunction, infection, and subdural hematoma; wall excision is invasive and increases morbidity risks. Fenestration and cystoventriculostomy have also reported cases of recurrence. Other methods also pose issues, including off-label use of instruments and inadequate consideration for removal. The method we performed for placing the stent catheter with multiple side holes demonstrated long-term effectiveness. Furthermore, the surgery was conducted with the patient fully awake, and the use of electrical stimulation mapping enabled preservation of higher neurocognitive functions, including language, working memory, and spatial cognition, at the puncture site. This novel approach offers advantages such as minimally invasive procedure, preservation of safe brain function, and long-term patency. Further studies are warranted to validate these findings and improve surgical strategies for convexity cysts.

DOI： 10.1016/j.wneu.2024.10.031

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Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：The Japanese Society on Surgery for Cerebral Stroke  

<p>The use of endoscopy in the treatment of brainstem cavernous malformations (BCMs) has been reported in a limited number of cases. Given its ability to provide a broad and bright field of view even in deep regions, endoscopy is considered effective for treating deep-seated lesions, such as BCMs, and has been actively utilized in our institute.</p><p>This study retrospectively reviewed 32 cases of BCMs treated with endoscopic techniques (midbrain, 7 cases; pons, 22 cases; and medulla, 3 cases). The approach routes to the brainstem were based on the “Two Point Method,” primarily utilizing the nearby safe entry zones. Surgical removal was performed through transnasal procedures in 5 cases and craniotomy in 27 cases, with 25 of these employing small diameter cylinders to secure the surgical corridor. Gross total resection was achieved in 30 cases (93.8%). Postoperative complications were observed in five cases. The average Karnofsky Performance Status improved from 62.2 preoperatively to 84.4 3 months postoperatively, with improvements in 26 cases, no change in 5 cases, and deterioration in 1 case. Surgical treatments for BCMs are predominantly performed under a microscope; however, the outcomes of endoscopic surgery in this study showed favorable neurological prognoses compared with the findings of previous reports. The use of endoscopy facilitated the minimization of surgical pathways and enabled underwater surgery, thereby establishing a novel approach that is not preferably available in microscopic surgery. Underwater intraoperative observation, a distinctive feature of endoscopic surgery, was highly effective in identifying residual lesions and achieving hemostasis. Despite its advantages, endoscopy presents challenges, prompting ongoing developments and improvements in surgical instruments and techniques. Although this technique is still evolving, the utility of endoscopy for treating BCMs is promising.</p>

DOI： 10.2335/scs.53.108

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：The Japan Neurosurgical Society  

Myxoid glioneuronal tumour (MGNT) is a new WHO classification of brain tumours and is defined as tumours with PDGFRA p.K385 mutations. MGNT has a predilection for the septum pellucidum, their clinical features have not yet been clarified because only a few reports exist. In this report, we present the case of a patient with an MGNT that was suitable for endoscopic resection. We discuss the clinical characteristics of MGNTs discovered during a literature review. A 19-year-old female presented with a worsening headache and a disturbance of consciousness. Head MRI revealed a mass lesion in the septum pellucidum and severe obstructive hydrocephalus. She underwent neuroendscopic surgery under general anaesthesia, and near total resection was achieved. Her symptoms improved immediately after surgery, and she was discharged without any morbidities. Pathological examination and genetic testing confirmed the diagnosis of an MGNT. A literature review revealed that 7 of 18 MGNTs developed in the septum pellucidum. Three patients had disseminated lesions at initial diagnosis, and one showed dissemination during the follow-up period. Among the 14 patients who underwent tumour resection surgery, 3 experienced recurrence or regrowth and required additional treatments. All 18 patients were alive at the last follow-up. An MGNT is a tumour that predominantly affects young people and is currently considered to have a good prognosis. However, close postoperative observation is needed because an MGNT often disseminates intracranially and/or into the spinal cord. In addition, we believe that MGNTs located in the septum pellucidum are good candidates for endoscopic resection.

DOI： 10.2176/jns-nmc.2025-0028

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BACKGROUND: Idiopathic normal pressure hydrocephalus is an age-related condition characterized by cerebrospinal fluid accumulation and ventricular enlargement, leading to cognitive decline, gait disturbance, and urinary incontinence. Although shunt surgery is the primary treatment, the optimal surgical strategy remains uncertain, and procedure selection is often not tailored to individual patient characteristics. Notably, no prospective study has directly compared the 3 major shunt techniques (ie, ventriculoperitoneal, lumboperitoneal, and ventriculoatrial). OBJECTIVE: This multicenter prospective observational study will aim to generate high-quality clinical evidence by evaluating the effectiveness and safety of the 3 aforementioned surgical options in a real-world setting. METHODS: Patients suspected of having this condition will be enrolled based on characteristic symptoms and imaging findings, with a spinal tap test recommended but not mandatory. Eligible patients will undergo one of the aforementioned surgical procedures. RESULTS: On the basis of recent data from 11 collaborating institutions in Japan, we estimate enrolling 278 cases: 188 (67.6%) surgical, 100 (36%) nonsurgical, and 10 (3.6%) dropouts. Clinical outcomes will be assessed at baseline; after the tap test (if performed); and at 1 week, 3 months, and 12 months postoperatively. The analyses will explore the associations between outcomes and surgical methods, patient backgrounds, and imaging features. As of May 2025, approximately 60 participants have been enrolled from 11 institutions. Data collection is ongoing and is expected to be completed by December 2026. There will be a 1-year follow-up period. The main study results are anticipated to be published in 2028. CONCLUSIONS: Japan is well placed to lead this comparative study with its extensive experience in diagnosing and treating this disease. These findings are expected to provide practical guidance for individualized surgical decision-making and contribute to the global consensus on optimal treatment strategies. TRIAL REGISTRATION: Japan Registry of Clinical Trials jRCT1040250005; https://jrct.mhlw.go.jp/latest-detail/jRCT1040250005. INTERNATIONAL REGISTERED REPORT IDENTIFIER (IRRID): DERR1-10.2196/80678.

DOI： 10.2196/80678

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DOI： 10.1016/j.jneumeth.2024.110293

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DOI： 10.1093/neuonc/noae165.0672

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DOI： 10.1093/neuonc/noae165.0748

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DOI： 10.1093/neuonc/noae165.1270

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BACKGROUND: Treating meningeal tumours invading the large dural venous sinuses is a subject of debate regarding the approach for removing the intra-sinus components. Additionally, directly observing the invasion site of tumours invading the lateral wall of the sinus is difficult. METHOD: We describe our exo- and endoscopic two-step approach (EETA): an exoscope is used to remove the extra-sinus component, while an endoscope is used to observe the invaded lateral wall and remove the intra-sinus component. CONCLUSION: EETA can be a viable option for treating meningeal tumours invading the venous sinus owing to its high resection rate and low invasiveness.

DOI： 10.1007/s00701-024-06298-2

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BACKGROUND: Periventricular pediatric low-grade gliomas (pLGG) present a surgical challenge due to their deep-seated location, accessibility, and relationship with the subcortical network connections. Minimally invasive parafascicular approaches with tubular brain retractors (port brain surgery) have emerged, in recent years, as an alternative to conventional microsurgical and endoscopic approaches for removal of periventricular tumors. OBJECTIVES: To describe the minimally invasive approach with tubular brain retractors for periventricular pLGG, its technique, applications, safety, and efficacy. METHODS: In this article, we describe the port brain surgery techniques for periventricular pLGG as performed in different centers, with different commercialized tubular retractor systems. Illustrative cases followed by a literature review are analyzed, with a detailed description of different approaches or techniques, comparing their advantages and disadvantages with contemporary microsurgical and endoscopic approaches. CONCLUSIONS: The port brain surgery with micro-exoscopic vision and endoscopic assistance, for the treatment of deep-seated lesions such as periventricular pLGG, is an alternative for achieving a functionally safe-gross total or subtotal-tumor resection, obtaining adequate tissue for pathological examination. This technique could offer a new dimension for a less-invasive, safe, and effective access to deep-seated tumors, offering the possibility to lower morbidity in experienced hands.

DOI： 10.1007/s00381-024-06417-5

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BACKGROUND: Ependymal cilia play a major role in the circulation of cerebrospinal fluid. Although isolation of cilia is an essential technique for investigating ciliary structure, to the best of our knowledge, no report on the isolation and structural analysis of ependymal cilia from mouse brain is available. NEW METHOD: We developed a novel method for isolating ependymal cilia from mouse brain ventricles. We isolated ependymal cilia by partially opening the lateral ventricles and gently applying shear stress, followed by pipetting and ultracentrifugation. RESULTS: Using this new method, we were able to observe cilia separately. The results demonstrated that our method successfully isolated intact ependymal cilia with preserved morphology and ultrastructure. In this procedure, the ventricular ependymal cell layer was partially detached. COMPARISON WITH EXISTING METHODS: Compared to existing methods for isolating cilia from other tissues, our method is meticulously tailored for extracting ependymal cilia from the mouse brain. Designed with a keen understanding of the fragility of the ventricular ependyma, our method prioritizes minimizing tissue damage during the isolation procedure. CONCLUSIONS: We isolated ependymal cilia from mouse brain by applying shear stress selectively to the ventricles. Our method can be used to conduct more detailed studies on the structure of ependymal cilia.

DOI： 10.1016/j.jneumeth.2024.110198

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DOI： 10.1507/endocrine.100.s.hpt_19

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OBJECTIVE: Transient neurological deficits (TNDs) are known to develop after direct bypass for moyamoya disease (MMD) and may be risk factors for subsequent stroke. However, the factors involved in the development of TNDs and stroke after indirect revascularization alone, including their association with subsequent stroke, remain unclear. The purpose of this study was to investigate this issue. METHODS: The subjects of the study were 30 patients with MMD who underwent a total of 40 indirect revascularization procedures at our institution. Clinical and radiological data were collected retrospectively. To examine factors associated with the development of postoperative TND/stroke/asymptomatic disease, the clinical characteristics of each group were statistically compared. RESULTS: The mean age at surgery was 7 years (range 1-63). TNDs developed after surgery in 9 out of 40 patients (22.5%). Stroke in the acute postoperative period occurred in 3 patients (7.5%), all of whom experienced cerebral infarctions. Demographic data and preoperative clinical information were not different between the groups. However, posterior cerebral artery (PCA) involvement on preoperative imaging was significantly associated with the development of TNDs and stroke (p=0.006). Furthermore, postoperative stroke was associated with unfavorable outcomes (p=0.025). CONCLUSIONS: PCA involvement is significantly associated with the occurrence of TNDs. In contrast, TNDs after indirect revascularization have little relationship with the subsequent development of stroke. TNDs usually resolve without new strokes, and a better understanding of this particular pathology could help establish an optimal treatment regimen.

DOI： 10.1016/j.wneu.2024.04.135

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BACKGROUND: Posterior clinoid process (PCP) meningioma is an exceedingly rare entity. It remains the most challenging skull base lesion for neurosurgeons due to its treacherous location that insinuates amongst critical neurovascular structures. This article will describe the technical notes using the endoscopic endonasal approach that provide the earliest devascularization and detachment of the tumor PCP meningioma. METHODS: We are introducing the surgical implementation of an endoscopic endonasal approach to removing PCP meningioma. Furthermore, we perform a literature review of posterior clinoid process meningioma that undergoes surgical intervention, then summarize the benefits and limitations of each approach. RESULTS: We present a case of right PCP meningioma that was removed using an endoscopic endonasal approach through the transposterior clinoid corridor in a 52-year-old-woman. We describe the technical notes in performing this approach to have the earliest devascularization and detachment of the tumor by performing posterior clinoidectomy. Safe tumor removal is performed with a wide and clear view of the surrounding neurovascular structure. Based on our database search, we found nine articles reported on the surgical management of PCP meningiomas, with a total number of 15 cases. All of the reported cases performed the tumor removal using the transcranial approach. CONCLUSION: The endoscopic endonasal transposterior clinoid approach circumvents all disadvantages faced by the traditional transcranial approach, providing the earliest approach to devascularized and detaching the tumor from its attachment at PCP. This approach demonstrates safety and efficacy, making it an acceptable alternative for PCP meningioma resections.

DOI： 10.3389/fonc.2024.1368277

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DOI： 10.34433/og.0000000711

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Neurofibromatosis type 1 (NF1) is an autosomal dominant syndrome caused by germline alteration of the NF1gene. Among various NF1-related manifestations, obstructive hydrocephalus especially in adult NF1 cases is less frequently found. We report two adult NF1 cases exhibiting obstructive hydrocephalus due to an aggressive posterior fossa tumor exhibiting pathological characteristics of pilocytic astrocytoma as NF1-related manifestations. In these two cases, we performed endoscopic third ventriculostomy (ETV) and tumor biopsy as an initial treatment. The initial pathological diagnosis of the tumor is conventional pilocytic astrocytoma. After biopsy both cases revealed rapid tumor growth, therefore, we performed tumor removal, chemotherapy, and radiation therapy during an aggressive clinical course. However, both cases revealed dismal prognosis due to the progression of the tumor in spite of successful management of hydrocephalus by an initial ETV. DNA methylation analysis revealed that the tumor of one case matched high-grade astrocytoma with piloid features (HGAP). Most central nervous system tumors developed in NF1 are less aggressive such as pilocytic astrocytoma; however, recently a few studies revealed that HGAP, which has been a newly introduced malignant tumor in the World Health Organization Classification of Tumors of the Central Nervous System, 5th edition (WHO CNS 5), rarely develops in NF1 cases. These findings suggested that HGAP might be one of the important causes of obstructive hydrocephalus in adult NF1 cases.

DOI： 10.7759/cureus.58697

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Li-Fraumeni syndrome (LFS) is an autosomal dominant tumor predisposition syndrome caused by heterozygous germline mutations or deletions in the TP53 tumor suppressor gene. Central nervous system tumors, such as choroid plexus tumors, medulloblastomas, and diffuse gliomas, are frequently found in patients with LFS. Although molecular profiles of diffuse gliomas that develop in pediatric patients with LFS have been elucidated, those in adults are limited. Recently, diffuse gliomas have been divided into pediatric- and adult-type gliomas, based on their distinct molecular profiles. In the present study, we investigated the molecular profiles of high-grade gliomas in three adults with LFS. These tumors revealed characteristic histopathological findings of high-grade glioma or glioblastoma and harbored wild-type IDH1/2 according to whole exome sequencing (WES). However, these tumors did not exhibit the key molecular alterations of glioblastoma, IDH-wildtype such as TERT promoter mutation, EGFR amplification, or chromosome 7 gain and 10 loss. Although WES revealed no other characteristic gene mutations or copy number alterations in high-grade gliomas, such as those in histone H3 genes, PDGFRA amplification was found in all three cases together with uniparental disomy of chromosome 17p, where the TP53 gene is located. DNA methylation analyses revealed that all tumors exhibited DNA methylation profiles similar to those of pediatric-type high-grade glioma H3-wildtype and IDH-wildtype (pHGG H3-/IDH-wt), RTK1 subtype. These data suggest that high-grade gliomas developed in adult patients with LFS may be involved in pHGG H3-/IDH-wt. PDGFRA and homozygous alterations in TP53 may play pivotal roles in the development of this type of glioma in adult patients with LFS.

DOI： 10.1186/s40478-024-01762-7

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OBJECTIVE: Surgical treatment of brainstem cavernous malformations (CMs) is challenging. Surgery using the endoscopic transsphenoidal transclival approach (eTSTCA) is reported as a useful alternative for ventral brainstem CMs. However, CMs located in the ventral midline of the brainstem are rare, and only a small number of case reports on these CMs treated with the eTSTCA exist. The efficacy and safety of the eTSTCA have not yet been fully examined. METHODS: A retrospective analysis was performed for 5 consecutive patients who underwent surgery via the eTSTCA for treating ventral pontine CMs. RESULTS: The average maximum CM diameter was 26.0 mm (18-38 mm). All patients underwent MR-diffusion tensor imaging, which confirmed that the corticospinal tract (CST) deviated posteriorly or laterally to the CM. Direct brainstem cortical stimulation was performed to localize the CST before making the cortical incision. After the excision of the CM, the cavity was filled with artificial CSF to make an aqueous surgical field (wet-field technique) for observing the tumor cavity and confirming complete hemostasis and resection. Total removal was achieved in all patients. The preoperative modified Rankin Scale score was 3 in 3 patients and 4 in 2 patients, whereas it was 1 in 2 patients and 0 in 3 patients 3 months after surgery. Postoperative CSF leakage was observed in 1 patient, and transient abducens nerve palsy was observed in 1 patient. No other intra- or postoperative complications were observed. CONCLUSIONS: MR-diffusion tensor imaging and direct brainstem cortical stimulation were useful to ascertain the proximity of the CST to the CM. The endoscope provides a clear view even underwater, and it was safe and effective to observe the entire CM cavity and confirm complete hemostasis without additional retraction of the brainstem parenchyma, including the CST. The eTSTCA provides a direct access point to the lesion and may be a safer alternative treatment for patients whose CST deviates laterally or posteriorly to the CM.

DOI： 10.3171/2023.6.JNS23324

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The surgical treatment of retroinfundibular craniopharyngiomas is challenging due to their location and the surrounding neurovascular structures. In this report, the transdorsum sellar approach with posterior clinoidectomy, the efficacy of direct cyst puncture, and the suitability of a two-piece dural opening are presented. A 56-year-old male with visual and cognitive disturbances was referred to our hospital. Preoperative CT and MRI demonstrated a mostly cystic lesion with calcifications in the suprasellar and retroinfundibular areas. The imaging findings were suspected craniopharyngioma, and an extended endoscopic endonasal transdorsum sellar approach with posterior clinoidectomy was performed for direct access to the lesion. Two pieces of the dura were opened to prevent postoperative CSF leakage. The patient's postoperative course was uneventful. The endoscopic transdorsum sellar approach gives direct access to the posterior cranial fossa. A direct puncture of the cyst without CSF drainage is helpful for large cystic lesions. A two-piece dural opening is easy to suture and can reduce the chance of postoperative CSF leakage.

DOI： 10.7759/cureus.51850

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Authorship：Corresponding author   Language：English   Publishing type：Research paper (scientific journal)   Publisher：The Japan Neurosurgical Society  

Craniopharyngioma (CP) and Rathke's cleft cyst (RCC) are both suprasellar lesions. They are sometimes difficult to distinguish due to their similar findings. We report a case of papillary craniopharyngioma (pCP) with the clinical findings suggesting RCC. A 42-year-old female with intellectual disability presented to our hospital with severe visual dysfunction. Preoperative images revealed a suprasellar cystic lesion without calcification. We performed transsphenoidal surgery. Since the cyst had condensed-milk-like content suggesting RCC, we performed cyst fenestration and wash without removal of the cyst wall. Thereafter, we found fish-egg-like structures on the cyst wall. The histopathological analysis revealed that they had papillary structures surrounded by hyperplastic squamous epithelium with parakeratosis. Immunostaining for BRAF V600E was positive, leading to the diagnosis of pCP. After the surgery, her visual function improved and follow-up Magnetic resonance imaging at 18 months postoperatively showed no apparent recurrence. The presence of condensed-milk-like content suggests a likelihood of RCC indicating that aggressive resection may not be necessary. In contrast, the existence of fish-egg-like structures suggests pCP and requires careful follow-up.

DOI： 10.2176/jns-nmc.2024-0057

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：Advances and Technical Standards in Neurosurgery  

Cylinder retractors have been developed to reduce the risk of brain retraction injury during surgery by dispersing retraction pressure on the brain. In recent years, various types of cylinder retractors have been developed and widely used in neurosurgery. The ventricles, being deep structures within the brain, present an effective area for cylinder retractor utilization. Endoscopy provides a bright, wide field of view in the deep surgical field, even through narrow corridors.This chapter introduces surgical techniques using an endoscope through a cylinder. Given the deep and complex shapes of the ventricles, preoperative planning is paramount. Two main surgical techniques are employed in endoscopic cylinder surgery. The wet-field technique involves the continuous irrigation of artificial cerebrospinal fluid (CSF) during the procedure, maintaining ventricle shape with natural water pressure, facilitating tumor border identification, and achieving spontaneous hemostasis. Conversely, the dry-field technique involves CSF drainage, providing a clear visual field even during hemorrhage encounters. In intraventricular surgery, both techniques are used and switched as needed.Specific approaches for lateral, third, and fourth ventricular tumors are discussed, considering their locations and surrounding anatomical structures. Detailed intraoperative findings and strategies for tumor removal and hemostasis are presented.Endoscopic cylinder surgery offers a versatile and minimally invasive option for intraventricular tumors, leading to improved surgical outcomes. Overall, this technique enhances surgical precision and patient outcomes in intraventricular tumor cases.

DOI： 10.1007/978-3-031-61925-0_7

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Authorship：Lead author,　Corresponding author   Language：English   Publishing type：Research paper (scientific journal)  

Optic pathway gliomas (OPGs) are benign tumors that can stop growing or even shrink. In recent years, surgical resection has not been considered the first-line treatment because of its high risk of complications. Chemotherapy is the mainstay of treatment for growing OPGs. Surgical treatment for OPGs with obstructive hydrocephalus is required. Ventriculoperitoneal shunting is effective for all types of hydrocephalus. However, long-term management is required, especially in pediatric cases, and there is a risk of shunt-related complications over a long lifespan. Debulking surgery for OPGs allows us to avoid shunt placement by creating a waterway and releasing the hydrocephalus. To reduce the surgical risk and invasiveness, we used an endoscopic canalization technique with a small-diameter cylinder. In this article, we present a case of endoscopic canalization of an obstructive hydrocephalus caused by OPGs in a 14-year-old female to illustrate our surgical technique.(Trial registration Registry name and number: Efficacy and safety of the neuro-endoscopic treatment for brain tumors (2019-0254)).

DOI： 10.1007/s00381-023-06053-5

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Authorship：Corresponding author   Language：English   Publishing type：Research paper (scientific journal)   Publisher：Springer Science and Business Media LLC  

DOI： 10.1007/s10143-023-02196-w

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Other Link： https://link.springer.com/article/10.1007/s10143-023-02196-w/fulltext.html

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Abstract

Background

The glossopharyngeal nerve is both a sensitive and motor entity that emerges from the posterior lateral sulcus of the bulb and exits the skull through the jugular foramen. Schwannomas arising from glossopharyngeal nerves are extremely rare, and the clinical and imaging features of glossopharyngeal schwannomas closely resemble that of acoustic schwannomas.

Case presentation

We report a case of a 65-year-old female patient with glossopharyngeal neurinoma of incidental finding on brain CT scan requested after she sustained a minor traumatic brain injury. She was taken to the operating room, and the tumor was excised endoscopically via a retrosigmoid approach and the postoperative course was simple and uneventful. Histopathology of the sample showed short spindle-shaped cells with poor atypia proliferated in fascicles, and shelf-like arrangement of nuclei was seen partially, which was in keeping with a neurinoma.

Conclusions

Glossopharyngeal neurinoma may not present with obvious symptoms and signs related to its compression and may also be found incidentally compared to its counterpart vestibular schwannoma.

DOI： 10.1186/s41984-023-00226-9

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Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：一般社団法人 日本内分泌学会  

DOI： 10.1507/endocrine.99.s.hpt_4

CiNii Research

Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：株式会社医学書院  

DOI： 10.11477/mf.1436204803

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The myeloid differentiation primary response gene 88 (MYD88) L265P mutation is a disease-specific mutation of primary central nervous system lymphoma (PCNSL) among the central nervous system tumors. Accordingly, this mutation is considered a reliable diagnostic molecular marker of PCNSL. As the intra-operative diagnosis of PCNSL is sometimes difficult to achieve using histological examinations alone, intra-operative detection of the MYD88 L265P mutation could be effective for the accurate diagnosis of PCNSL. Herein, we aimed to develop a novel rapid genotyping system (GeneSoC) using real-time polymerase chain reaction (PCR) based on microfluidic thermal cycling technology. This real-time PCR system shortened the analysis time, which enabled the detection of the MYD88 L265P mutation within 15 min. Rapid detection of the MYD88 L265P mutation was performed intra-operatively using GeneSoC in 24 consecutive cases with suspected malignant brain tumors, including 10 cases with suspected PCNSL before surgery. The MYD88 L265P mutation was detected in eight cases in which tumors were pathologically diagnosed as PCNSL after the operation, while wild-type MYD88 was detected in 16 cases. Although two of the 16 cases with wild-type MYD88 were pathologically diagnosed as PCNSL after the operation, MYD88 L265P could be detected in all eight PCNSL cases harboring MYD88 L265P. The MYD88 L265P mutation could also be detected using cell-free DNA derived from the cerebrospinal fluid of two PCNSL cases. Detection of the MYD88 L265P mutation using GeneSoC might not only improve the accuracy of intra-operative diagnosis of PCNSL but also help the future pre-operative diagnosis through liquid biopsy of cerebrospinal fluid.

DOI： 10.1111/cas.15762

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PURPOSE: Delayed hyponatremia (DHN), a unique complication, is the leading cause of unexpected readmission after pituitary surgery. Therefore, this study aimed to develop tools for predicting postoperative DHN in patients undergoing endoscopic transsphenoidal surgery (eTSS) for pituitary neuroendocrine tumors (PitNETs). METHODS: This was a single-center, retrospective study involving 193 patients with PitNETs who underwent eTSS. The objective variable was DHN, defined as serum sodium levels < 135 mmol/L at ≥ 1 time between post operative days 3 and 9. We trained four machine learning models to predict this objective variable using the clinical variables available preoperatively and on the first postoperative day. The clinical variables included patient characteristics, pituitary-related hormone levels, blood test results, radiological findings, and postoperative complications. RESULTS: The random forest (RF) model demonstrated the highest (0.759 ± 0.039) area under the curve of the receiver operating characteristic curve (ROC-AUC), followed by the support vector machine (0.747 ± 0.034), the light gradient boosting machine (LGBM: 0.738 ± 0.026), and the logistic regression (0.710 ± 0.028). The highest accuracy (0.746 ± 0.029) was observed in the LGBM model. The best-performing RF model was based on 24 features, nine of which were clinically available preoperatively. CONCLUSIONS: The proposed machine learning models with pre- and post-resection features predicted DHN after the resection of PitNETs.

DOI： 10.1007/s11102-023-01311-w

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：Frontiers Media SA  

Introduction

The pituitary gland, regulating various hormones, is central in the endocrine system. As spontaneous recovery from hypopituitarism is rare, and exogenous-hormone substitution is clumsy, pituitary replacement via regenerative medicine, using pluripotent stem cells, is desirable. We have developed a differentiation method that in mice yields pituitary organoids (POs) derived from human embryonic stem cells (hESC). Efficacy of these POs, transplanted subcutaneously into hypopituitary mice, in reversing hypopituitarism was studied.

Methods

hESC-derived POs were transplanted into inguinal subcutaneous white adipose tissue (ISWAT) and beneath dorsal skin, a relatively avascular region (AR), of hypophysectomized severe combined immunodeficient (SCID) mice. Pituitary function was evaluated thereafter for ¾ 6mo, assaying basal plasma ACTH and ACTH response to corticotropin-releasing hormone (CRH) stimulation. Histopathologic examination of organoids 150d after transplantation assessed engraftment. Some mice received an inhibitor of vascular endothelial growth factor (VEGF) to permit assessment of how angiogenesis contributed to subcutaneous engraftment.

Results

During follow-up, both basal and CRH-stimulated plasma ACTH levels were significantly higher in the ISWAT group (p &amp;lt; 0.001 – 0.05 and 0.001 – 0.005, respectively) than in a sham-operated group. ACTH secretion also was higher in the ISWAT group than in the AR group. Histopathologic study found ACTH-producing human pituitary-cell clusters in both groups of allografts, which had acquired a microvasculature. POs qPCR showed expression of angiogenetic factors. Plasma ACTH levels decreased with VEGF-inhibitor administration.

Conclusions

Subcutaneous transplantation of hESC-derived POs into hypopituitary SCID mice efficaciously renders recipients ACTH-sufficient.

DOI： 10.3389/fendo.2023.1130465

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BACKGROUND: Rituximab, high-dose methotrexate (HD-MTX), procarbazine and vincristine (R-MPV), has significantly prolonged the survival of patients with primary central nervous system lymphoma (PCNSL), but predictive factors for response to R-MPV have not yet been investigated. Herein, we investigated the correlation of MYD88 L265P and CD79B Y196 mutations, which are the most frequently found molecular alterations in PCNSL, with prognosis of patients with PCNSL treated with R-MPV. METHODS: We investigated the long-term clinical course and status of MYD88 and CD79B genes in 85 patients with PCNSL treated with R-MPV or HD-MTX treatment, and the correlation of these genetic mutations with prognosis. RESULTS: R-MPV achieved an excellent tumor control rate (61.6% and 69.9% of 5-year progression-free and overall survival rates, respectively). While MYD88 L265P mutation had no significant effect on survival, patients with CD79B Y196 mutations exhibited prolonged survival (p < 0.05). However, the association of CD79B Y196 mutation with a better prognosis was not observed in the HD-MTX cohort, which indicated that CD79B Y196 mutation was a predictive marker for a favorable response to R-MPV. Furthermore, we established an all-in-one rapid genotyping system for these genetic mutations. CONCLUSIONS: In conclusion, CD79B Y196 mutation is a potent predictive marker for favorable response to R-MPV in PCNSL. The rapid identification of MYD88 L265P and CD79B Y196 mutations can be helpful not only for the accurate molecular diagnosis of PCNSL but also for the prediction of response to R-MPV.

DOI： 10.1002/cam4.5512

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Purpose: Postoperative pituitary dysfunction, a critical problem in the treatment of craniopharyngiomas, can occur even when the pituitary stalk is preserved. We hypothesized that compromise of the primary superior hypophyseal artery (pSHA) might be related to this occurrence. Methods: We performed a retrospective review of 131 patients with craniopharyngioma who underwent surgery from April 2009 to September 2021. The inclusion criteria were initial surgery, endoscopic transsphenoidal surgery, preoperative normal pituitary function or pituitary dysfunction in one axis, and morphological preservation of the pituitary stalk. The branches of the pSHA consist mainly of the chiasmatic branches (Cb), infundibular branches (Ib), and descending branches (Db). We analyzed the association between postoperative pituitary function and preservation of these branches. Results: Twenty patients met the criteria. Preoperative anterior pituitary function was normal in 18 patients, and there was isolated growth hormone deficiency in two patients. No patient had preoperative diabetes insipidus (DI). Anterior pituitary function was unchanged postoperatively in eight patients. Of these eight patients, bilateral preservation of pSHA Ib was confirmed in seven patients. Bilateral preservation of pSHA Ib was the only factor associated with preserved anterior pituitary function (p < 0.01). Fifteen patients were free of permanent DI, and the preservation of any given pSHA branch produced no significant difference in the postoperative occurrence of permanent DI. Conclusions: Our study shows that bilateral preservation of pSHA Ib provides favorable postoperative anterior pituitary function in craniopharyngioma surgery; however, such preservation may have little effect on the postoperative occurrence of DI.

DOI： 10.1007/s00701-022-05415-3

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Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：(一社)日本内分泌学会  

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：The Japan Neurosurgical Society  

A 61-year-old man presented with massive epistaxis, amaurosis, nausea, and severe headache. A detailed examination revealed a subarachnoid hemorrhage and prolactinoma. Angiography showed a small internal carotid artery pseudoaneurysm and inadequate collateral circulation; thus, uncomplicated coil embolization was performed. Considering the side effects of medication, such as cerebrospinal fluid rhinorrhea, the patient was followed up for asymptomatic prolactinoma without medication after discharge. At 40 months later, aneurysm recurrence was confirmed. Flow diverter device placement was performed, and the outcomes were excellent. In the present report, we described a rare case of a ruptured internal carotid artery aneurysm in an untreated prolactinoma and discussed the literature.

DOI： 10.2176/jns-nmc.2022-0355

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Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：(一社)日本脊髄外科学会  

DOI： 10.2531/spinalsurg.36.296

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DOI： 10.2531/spinalsurg.36.296

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Other Link： https://search.jamas.or.jp/default/link?pub_year=2022&ichushi_jid=J02943&link_issn=&doc_id=20230112220009&doc_link_id=10.2531%2Fspinalsurg.36.296&url=https%3A%2F%2Fdoi.org%2F10.2531%2Fspinalsurg.36.296&type=J-STAGE&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00007_3.gif

Authorship：Lead author,　Corresponding author   Language：English   Publishing type：Research paper (scientific journal)  

Objective: The biopsy procedure is intended to obtain an adequate specimen volume from the targeted area while ensuring minimal damage to the normal brain. We performed navigation-guided endoscopic biopsy using a small-diameter cylinder to reduce the invasiveness of the biopsy procedure and ensure a sufficient amount of tissue is collected. We examined whether it is possible to reduce brain tissue injury by using a small-diameter cylinder and improve safety and effectiveness by using an endoscope to directly observe the lesion and achieve hemostasis. Methods: Patients who underwent endoscopic biopsy surgery using a 6-mm-diameter cylinder for intraparenchymal lesions were enrolled in this study. Postoperative hematoma formation and the extent of trajectory scarring were assessed. Results: Fifty-two procedures performed on 51 patients were analyzed in this study. Postoperative neurological deterioration was not observed in any patient. A pathological diagnosis was made for all patients. Postoperative computed tomography revealed no hematoma after 49 procedures and a small hematoma after 3 procedures, and no patients required additional treatment. A postoperative trajectory scar less than 5 mm in diameter was observed after 30 procedures, a scar of 5–10 mm was observed after 19 procedures, a scar larger than 10 mm was observed after 3 procedures at 1 week after surgery, and 40, 6 and 0 scars were observed at 3 months after surgery. Conclusions: Endoscopic biopsy using a small-diameter cylinder is a possible alternative biopsy technique for intraparenchymal lesions. This surgical technique is useful, especially in patients at risk of hemorrhagic complications.

DOI： 10.1016/j.wneu.2022.08.147

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Objective: Cerebral cavernous malformations (CMs) presenting with focal neurological symptoms or mass effects require surgical removal. In recent years, cylindrical retractors have been widely utilized for the removal of deep-seated lesions during both microscopic and endoscopic surgery. In the present study, we evaluated the efficacy and safety of endoscopic transcylinder removal of CMs using a novel wet-field technique. Methods: We included 13 patients with supratentorial CMs who had undergone endoscopic transcylinder surgery between April 2013 and March 2022. One patient experienced recurrence of the CM and underwent a second endoscopic transcylinder surgery. Therefore, we retrospectively evaluated 14 procedures. The surgical field was continuously irrigated with artificial cerebrospinal fluid to maintain expansion and visualization of the tumor bed. We termed this method as the “wet-field technique.” Patient characteristics, symptoms, and pre- and postoperative magnetic resonance imaging results were obtained from medical records. Results: The average maximum CM diameter was 35.3 mm (range: 10–65 mm). Cylinder diameters were 6 mm in eight procedures, 10 mm in four procedures, and 17 mm in one procedure. Wet-field technique was applied in all cases. The endoscope provided a bright field of view even under water. Continuous water irrigation made it easier to observe the entire tumor bed which naturally expanded by water pressure. Gross total resection was achieved in 13 procedures, while subtotal resection was achieved in one procedure. No surgical complications were observed. Conclusions: The endoscopic transcylinder removal using wet-field technique is safe and effective for the removal of symptomatic intracranial supratentorial CMs.

DOI： 10.1007/s00701-022-05273-z

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Ciliary motility disorders are known to cause hydrocephalus. The instantaneous velocity of cerebrospinal fluid (CSF) flow is dominated by artery pulsation, and it remains unclear why ciliary dysfunction results in hydrocephalus. In this study, we investigated the effects of cilia-induced surface velocity on CSF flow using computational fluid dynamics. A geometric model of the human ventricles was constructed using medical imaging data. The CSF produced by the choroid plexus and cilia-induced surface velocity were given as the velocity boundary conditions at the ventricular walls. We developed healthy and reduced cilia motility models based on experimental data of cilia-induced velocity in healthy wild-type and Dpcd-knockout mice. The results indicate that there is almost no difference in intraventricular pressure between healthy and reduced cilia motility models. Additionally, it was found that newly produced CSF from the choroid plexus did not spread to the anterior and inferior horns of the lateral ventricles in the reduced cilia motility model. These findings suggest that a ciliary motility disorder could delay CSF exchange in the anterior and inferior horns of the lateral ventricles.

DOI： 10.1098/rsif.2022.0321

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DOI： 10.1507/endocrine.98.s.hpt_72

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経鼻的内視鏡下頭蓋底手術は下垂体腫瘍をはじめとする傍鞍部腫瘍に対する第一選択の外科的治療法であるが、合併症として術後髄液鼻漏の危険性が伴う。今回、当院と関連施設で2016～2021年に本手術が施行された506例を対象とし、原疾患、手術方法、再建方法、原疾患、術後髄液漏の発生頻度などについて調査し、術後髄液漏発生例について詳細な検討を行った。原疾患は下垂体腺腫294例、頭蓋咽頭腫59例、鞍結節髄膜腫48例、ラトケ嚢胞33例、くも膜嚢胞12例、脊索腫11例などであった。再建方法は通常閉鎖法(鞍内に補綴物を充填し硬膜の縫合を行う)が349例、靴紐閉鎖法(硬膜切開部に補綴物を挟み込むように連続縫合を行う)が116例、パッチ閉鎖法(硬膜欠損部を補綴物でパッチ状に縫合閉鎖する)が41例であった。術後髄液漏は11例(2.2%)に発生し、原疾患の内訳は下垂体腺腫3例、頭蓋咽頭腫3例、鞍結節髄膜腫2例、脊索腫1例、くも膜嚢胞1例、リンパ腫1例であった。再建方法は通常閉鎖法4例、靴紐閉鎖法5例、パッチ閉鎖法2例であった。髄液漏の発生要因について検討した結果、術者側の要因としてグラフトの量や種類・位置に関するものが多く、患者側の要因として多動や放射線治療、脳室腹腔シャント挿入などが考えられた。

DOI： 10.1507/endocrine.98.s.hpt_25

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Background: Eccrine spiradenocarcinoma (SC), also known as malignant eccrine spiradenoma, is a rare malignant cutaneous adnexal neoplasm arising from long-standing benign eccrine spiradenoma. Malignant skin tumors rarely show direct intracranial invasion. However, once the intracranial structure is infiltrated, curative excision with sufficient margins can become extremely difficult, particularly when the venous sinuses are involved. No effective adjuvant therapies have yet been established. Here, we report an extremely rare case of scalp eccrine SC with direct intracranial invasion, which does not appear to have been reported previously. Case presentation: An 81-year-old woman presented with a large swelling on the parietal scalp 12 years after resection of spiradenoma from the same site. The tumor showed intracranial invasion with involvement of the superior sagittal sinus and repeated recurrences after four surgeries with preservation of the sinus. The histopathological diagnosis was eccrine SC. Adjuvant high-precision external beam radiotherapy (EBRT) proved effective after the third surgery, achieving remission of the residual tumor. The patient died 7 years after the first surgery for SC. Conclusions: Scalp SC with direct intracranial invasion is extremely rare. Radical resection with tumor-free margins is the mainstay of treatment, but the involvement of venous sinuses makes this unfeasible. High-precision EBRT in combination with maximal resection preserving the venous sinuses could be a treatment option for local tumor control.

DOI： 10.1186/s12883-022-02749-4

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Background: Dural suturing is one of the most robust reconstruction methods in transsphenoidal surgery; however, the technique is considered difficult, primarily due to the restricted range of needle movement. Method: We performed dural threading during transsphenoidal surgery by holding the needle with forceps as a hook and moving the needle in a distal to proximal direction. Conclusion: Our dural suturing technique is simple and quick. It can be used not only for dural closure but also for other procedures, such as controlling haemostasis from the intercavernous sinus and dural tenting.

DOI： 10.1007/s00701-022-05227-5

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：Journal of Neurosurgery Case Lessons  

BACKGROUND Symptomatic hyperperfusion after cerebral revascularization for pediatric moyamoya disease (MMD) is a rare phenomenon. The authors report a series of patients with this condition. OBSERVATIONS In all three patients in this case series, the combined revascularization was on the left side, the patency of bypass grafts was confirmed after surgery, and focal hyperemia around the anastomotic site was observed on single photon emission computed tomography (SPECT). On the first to eighth days after surgery, all of the patients developed neurological manifestations, including motor aphasia, cheiro-oral syndrome, motor weakness of their right upper limbs, and severe headaches. These symptoms disappeared completely approximately 2 weeks after surgery, and all patients were discharged from the hospital. Quantitative SPECT was performed to determine the proportional change in cerebral blood flow (DRCBF) (to ipsilateral cerebellar ratio (denoted DRCBF) in the region of interest around the anastomoses, and the mean value was 1.34 (range, 1.29–1.41). LESSONS This rare condition, which develops soon after surgery, requires an accurate diagnosis by SPECT. One indicator is that the DRCBF has risen to 1.3 or higher. Subsequently, strategic blood pressure treatment and fluid management could prevent the development of hemorrhagic stroke.

DOI： 10.3171/CASE2274

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Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：日本脳腫瘍病理学会  

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Authorship：Corresponding author   Language：English   Publishing type：Research paper (scientific journal)  

Trapped temporal horn is a rare type of noncommunicating focal hydrocephalus, and no standard treatment has been established yet for trapped temporal horn. Recent studies have shown the efficacy of endoscopic ventriculocisternostomy by opening the choroidal fissure; however, some surgical complications were reported during the procedure. Thus, we aimed to report a novel endoscopic ventriculocisternostomy and stenting technique for trapped temporal horn. In this technique, a 5.8-mm transparent acryl puncture needle with a 2.7-mm 0° rigid endoscope was used to open the choroidal fissure. It can fenestrate the choroidal fissure under real-time endoscopic observation of the critical neurovascular structures over the choroidal fissure. Moreover, the dull tip of the needle is less likely to injure the critical neurovascular structures, resulting in safer ventriculocisternostomy than the previously reported technique. Then, a stent is placed along the tract to prevent future obstruction of the stoma. Six trapped temporal horns in four patients were treated using the technique. All the patients showed improved symptoms with no surgical complications. None of the patients showed recurrence of trapped temporal horn during the mean follow-up period of 39.3 months. The combination of endoscopic ventriculocisternostomy and stenting with a transparent acryl puncture needle is a safe and effective treatment option for trapped temporal horn.

DOI： 10.1007/s10143-021-01634-x

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Objective: Cerebral revascularization is necessary for pediatric patients younger than 5 years with moyamoya disease (MMD). However, they have a high risk of developing cerebral infarction early after surgery. This study aimed to analyze the risk factors for developing cerebral infarction among these patients. Methods: The charts of 21 consecutive patients with MMD (39 surgeries) younger than 5 years who had undergone revascularization at our hospital were retrospectively analyzed. Because cerebral infarction occurring within 1 month after surgery was the primary end point, other clinical information was evaluated, including each surgical procedure. Multivariate analysis of the risk factors for postoperative cerebral infarction was performed. Results: Cerebral infarction occurred after 7 of 39 surgeries (17.9%). Of the 39 surgeries, 23 (59%) included direct and indirect combined revascularization. The incidence of cerebral infarction did not differ significantly between the combined (21.7%) and indirect (12.5%) groups (P = 0.46). Logistic regression showed no association between the revascularization procedure and the occurrence of cerebral infarction after surgery (P = 0.3). However, younger age at surgery was correlated with a higher risk of developing cerebral infarction in the early postoperative period (P = 0.05). Conclusions: No differences were found in the risk of developing cerebral infarction early after surgery as a result of surgical procedures. However, younger patients had higher postoperative risk. Further multicenter research should examine this issue for young pediatric patients with moyamoya at high risk of developing cerebral infarction.

DOI： 10.1016/j.wneu.2021.12.115

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：The Japan Neurosurgical Society  

<p>Extended endonasal transsphenoidal surgery (eTSS) offers a wide surgical field for various parasellar lesions; however, intraoperative high-flow cerebrospinal fluid (CSF) leakage is inevitable. Therefore, secure sellar reconstruction methods are essential to prevent postoperative CSF leakage. Although collagen matrix has been applied for dural reconstruction in neurosurgery, its suitability for application in extended eTSS remains unclear. Eighteen patients underwent modified shoelace dural closure using collagen matrix after lesionectomy via extended eTSS. In this technique, a collagen matrix, which was placed subdurally (inlay graft), was continuously sutured with both open dural edges like a shoelace. Then, another collagen matrix was placed epidurally (onlay graft), and rigid reconstruction was performed using the septal bone and a resorbable fixation mesh. Postoperative CSF leakage did not occur in 17 patients but did occur in 1 patient with tuberculum sellae meningioma. In this case, the CSF leakage point was detected just around the area between the coagulated dura and the adjacent collagen matrix. The collagen matrix harvested from this area was pathologically examined; neovascularization and fibroblastic infiltration into the collagen matrix were not detected. On the other hand, neovascularization and fibroblast infiltration into the collagen matrix were apparent on the surface of the collagen matrix harvested from the non-CSF leakage area. Our novel dural closure technique using collagen matrix could be an effective option for sellar reconstruction in extended eTSS; however, it should be applied in patients in whom normal dural edges are preserved.</p>

DOI： 10.2176/jns-nmc.2021-0355

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Cerebral revascularization for moyamoya disease (MMD) is an effective treatment for improving cerebral ischaemia and preventing rebleeding. Although direct bypass surgery is commonly performed on older children and adults, it is challenging in very young children due to the high difficulty level of the procedure. The subjects were MMD patients under 3 years of age on whom surgery was performed by a single surgeon (Y.A.). Preoperative clinical findings, information related to direct bypass surgery, bypass patency, and the incidence of postoperative stroke were investigated. Combined revascularization, including direct bypass surgery, was performed on 3 MMD patients (3 sides) under 3 years of age. The average diameter of the grafts used in direct bypass was 0.8 mm. The average recipient diameter was 0.8 ± 0.17 (range 0.6–1) mm. In all cases, the anastomotic procedure was completed using 11–0 monofilament nylon thread, and patency was confirmed. Direct bypass for MMD patients under 3 years old is technically challenging. However, despite the anatomical differences between very young children and elderly individuals, direct bypass surgery could certainly be completed. In addition, a rapid recovery from cerebral blood flow insufficiency could yield a promising neurological outcome.

DOI： 10.1007/s10143-021-01685-0

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Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：(一社)日本内分泌学会  

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Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：(一社)日本内分泌学会  

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Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：(一社)日本内分泌学会  

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Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：(一社)日本小児神経外科学会  

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BACKGROUND: Remote cerebral infarction after combined revascularization of the middle cerebral artery (MCA) territory is rare in patients with moyamoya disease (MMD) with a fetal-type posterior communicating artery (PCoA). OBSERVATIONS: A 57-year-old woman developed numbness in her right upper limb and transient motor weakness and was diagnosed with MMD. She also had a headache attack and a scintillating scotoma in the right visual field. Preoperative magnetic resonance angiography (MRA) showed stenosis of the left posterior cerebral artery (PCA). Combined revascularization was performed for the left MCA territory. No new neurological deficits were observed for 2 days after the operation, but right hemianopia, alexia, and agraphia appeared on postoperative day (POD) 4. Magnetic resonance imaging showed a new left occipitoparietal lobe infarction, and MRA showed occlusion of the distal left PCA. After that point, the alexia and agraphia gradually improved, but right hemianopia remained at the time of discharge on POD 18. LESSONS: Cerebral ischemia in the PCA territory may occur after combined revascularization of the MCA territory in patients with fetal-type PCoA. For these cases, a double-barrel bypass or indirect revascularization to induce a slow conversion could be considered on its own as a treatment option.

DOI： 10.3171/CASE21704

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BACKGROUND: Transient neurological deficits (TNDs) develop after cerebral revascularization in patients with moyamoya disease (MMD). The authors report a rare pediatric MMD case with extensive decreased cerebral blood flow (CBF) and prolonged TNDs after combined revascularization. OBSERVATIONS: A 9-year-old boy presented with transient left upper limb weakness, and MMD was diagnosed. A right-sided combined surgery was performed. Two years after the surgery, frequent but transient facial (right-sided) and upper limb weakness appeared. The left internal carotid artery terminal stenosis had progressed. Therefore, a left combined revascularization was performed. The patient's motor aphasia and right upper limb weakness persisted for approximately 10 days after surgery. Magnetic resonance angiography showed that the direct bypass was patent, but extensive decreases in left CBF were observed using single photon emission tomography. With adequate fluid therapy and blood pressure control, the neurological symptoms eventually disappeared, and CBF improved. LESSONS: The environment of cerebral hemodynamics is heterogeneous after cerebral revascularization for MMD, and the exact mechanism of CBF decreases was not identified. TNDs are significantly associated with the onset of stroke during the early postoperative period. Therefore, appropriate treatment is desired after determining complex cerebral hemodynamics using CBF studies.

DOI： 10.3171/CASE21628

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Background: Imaging with 18F-fluorodeoxyglucose (FDG)-positron emission tomography (PET) and 11C-methionine (MET)-PET can delineate primary and metastatic brain tumors. Lesion size affects the sensitivity of both scans and histopathological features can also influence FDG-PET, but the effects on MET-PET have not been elucidated. Case Description: We report an unusual case of metastatic brain tumors without accumulation of FDG or MET, contrasting with high FDG uptake in the primary lung lesion. The brain lesions were identified as adenocarcinoma with a more mucus-rich background, contributing to the indistinct accumulation of both FDG and MET. Conclusion: Histopathological characteristics can affect both MET and FDG accumulation, leading to findings contradicting those of the primary lesion.

DOI： 10.25259/SNI_264_2022

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Background: The aim of this study was to investigate the association between early postoperative slow flow in bypass grafts and preoperative focal cerebral blood flow (CBF) in patients who underwent combined surgery for moyamoya disease (MMD). Methods: The subjects were 18 patients (22 surgeries) who underwent single photon emission computed tomography (SPECT) before surgery. The CBF value of the middle cerebral artery territory was extracted from the SPECT data, and the value relative to the ipsilateral cerebellar CBF (relative CBF, or RCBF) was calculated. The association between RCBF and early postoperative slow flow in the bypass graft was investigated. In addition, the correlation between the revascularization effect and preoperative RCBF was analyzed. Results: In four of 22 surgeries (18.2%), slow flow in the bypass graft was identified in the early postoperative period. Preoperative RCBF in the slow flow and patent groups was 0.86 ± 0.15 and 0.87 ± 0.15, respectively, with no significant difference (P = 0.72). The signal intensity of four slow-flowed bypasses was improved in all cases on magnetic resonance angiography images captured during the chronic phase (mean of 3.3 months postoperatively). The revascularization scores were 2 ± 0.82 and 2.1 ± 0.68 in the slow flow and patent groups, respectively, and did not differ significantly (P = 0.78). A significant correlation was not observed between preoperative RCBF and the revascularization effect. Conclusion: No significant association was observed between preoperative RCBF and early postoperative slow flow in bypass grafts in patients with MMD undergoing combined surgery. Given the high rate of improved depiction of slow-flowed bypass in the chronic postoperative phase, the conceptual significance of an opportune surgical intervention is to maintain CBF by supporting the patient's own intracranial-extracranial conversion function.

DOI： 10.25259/SNI_772_2022

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：The Japan Neurosurgical Society  

Here we report a rare case of capillary hemangioma (CH) in a 28-year-old woman suffering from gradual worsening diplopia at 28 weeks of pregnancy. Magnetic resonance imaging (MRI) showed a mass lesion (about 3 cm in diameter) in the right parasellar region. We decided to observe as she was pregnant, and had no symptoms other than right abducent nerve palsy. Fortunately, her symptoms did not worsen until delivery. Computed tomography, enhanced MRI, and angiography after delivery revealed that the lesion was highly calcified and vascularized. A dorsum sellae meningioma or highly calcified pituitary adenoma was suspected and the endoscopic transsphenoidal approach was used for tumor removal. The postoperative course was uneventful. The histological diagnosis was CH. Intracranial CHs or CHs of skull are rare vascular tumors. These tumors are reportedly more common in female patients and may change in size in adults according to menstrual cycle and pregnancy. Only six cases, including that of the present study, were diagnosed during the perinatal period. Some of them experienced rapid symptom progression and tumor growth in their course; thus, we should pay further attention to pregnant or peripartum patients with brain tumor, suspected hemangiomas.

DOI： 10.2176/jns-nmc.2021-0326

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Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：The Japanese Society of Spinal Surgery  

<p>  The clinical applications of three-dimensional (3D) printing in the field of spine surgery and education have recently begun to progress. Technological advances have made it relatively inexpensive and easy to create 3D models. Herein, we explain how to construct a full-scale model using a 3D printer, and review articles on the current clinical applications and limitations. Creation of a 3D model based on the patient’s radiographic image data facilitates understanding of the complicated anatomy. This model enables improvements in visual understanding, preoperative surgical planning, and gives the surgeon a training opportunity. Furthermore, 3D printer technology can also be useful during surgery, and 3D printed surgical guidance systems have previously been successfully used for spine surgery. 3D printing patient-specific guides and templates are highly useful to facilitate safe screw insertion. In addition, 3D printers can be used to create patient-specific implants.</p>

DOI： 10.2531/spinalsurg.36.296

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Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：(一社)日本内分泌学会  

内視鏡下経鼻術(TSS)を行ったSellar arachnoid cyst(sAC)14例17手術(男性5例、女性9例、平均年齢49.6歳)の手術成績を報告した。初回手術例は13例、再発例は1例で、術前症状は視機能障害13例、動眼神経麻痺1例、汎下垂体前葉機能低下2例であり、嚢胞最大径は平均26.9mmであった。手術法はconventional TTS:9手術、extended TTS:8手術で、下垂体柄を温存しつつ可及的な嚢胞開窓を行い、視機能障害と動眼神経麻痺は全例改善した。TSSはsACに対して安全かつ有用な外科的治療法であり、術前画像や術中所見に基づいて下垂体柄と下垂体組織を温存するよう嚢胞開窓を行うことで、良好な術後下垂体機能が期待できる。また、下垂体柄が嚢胞前方に位置する症例は術後再発をきたしやすく、手術法の工夫や注意深いフォローアップが必要である。

DOI： 10.1507/endocrine.97.s.hpt_49

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Other Link： https://search.jamas.or.jp/default/link?pub_year=2021&ichushi_jid=J01160&link_issn=&doc_id=20210929320015&doc_link_id=%2Fcs6hypop%2F2021%2F0097s1%2F016%2F0049-0051%26dl%3D0&url=https%3A%2F%2Fwww.medicalonline.jp%2Fjamas.php%3FGoodsID%3D%2Fcs6hypop%2F2021%2F0097s1%2F016%2F0049-0051%26dl%3D0&type=MedicalOnline&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00004_2.gif

Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：(一社)日本内分泌学会  

新規再建材料であるDuragenを用いた経鼻頭蓋底手術後再建120症例を対象として、Duragenの適応と限界について検討した。再建方法は通常法77例(通常再建法73例、靴紐再建法4例)、拡大法43例(靴紐再建法30例、パッチワーク再建法13例)で、このうち自家組織を用いずにDuragen単独で再建した症例は通常法45例、拡大法9例であり、Duragen単独再建群の1例(1.9%)に術後髄液漏を認めたため、再手術を行った。髄液漏再建手術時に採取したDuragenの病理組織学的検討では、inlay graftとして硬膜内に留置されたものには線維芽細胞、新生血管がみられたが、onlay graftとして硬膜外に留置されたものには新生血管はなく癒着も弱かった。硬膜欠損の少ない症例や硬膜焼灼のない症例はDuragen単独での再建が有効と考えられた。

DOI： 10.1507/endocrine.97.s.hpt_16

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For pituitary regenerative medicine, the creation of a hypophyseal model in monkeys is necessary to conduct future preclinical studies; however, previous studies reported that hypophysectomy in monkeys is not always safe or satisfactory. This study aimed to create a hypophyseal dysfunction model in a cynomolgus monkey using a safer surgical technique and establish the protocol of pituitary hormone replacement therapy for this model. Surgical resection of the pituitary gland of a 7.8-year-old healthy adult cynomolgus male monkey weighing 5.45 kg was performed to create a hypophyseal dysfunction model for future regenerative studies. Endoscopic transoral transsphenoidal surgery was used to perform hypophysectomy under navigation support. These procedures were useful for confirming total removal of the pituitary gland without additional bone removal and preventing complications such as cerebrospinal fluid leakage. Total removal was confirmed by pathological examination and computed tomography. Hypopituitarism was verified with endocrinological examinations including stimulation tests. Postoperatively, the monkey’s general condition of hypopituitarism was treated with hormone replacement therapy, resulting in long-term survival. The success of a minimally invasive and safe surgical method and long-term survival indicate the creation of a hypophyseal dysfunction model in a cynomolgus monkey; hence, this protocol can be employed in the future.

DOI： 10.1038/s41598-021-90209-3

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The presigmoid approach (PSA) is selected to obtain more lateral access to cerebellopontine angle tumors, brainstem cavernous malformations, or vertebrobasilar artery aneurysms than the standard retrosigmoid approach. However, mastoidectomy for the PSA can be considered time-consuming and to carry a higher risk of complications due to the anatomical complexity of the region. The authors established a method of minimized mastoidectomy focused on exposing Trautmann’s triangle as the corridor for the PSA while maximizing procedural simplicity and safety and maintaining a sufficient operative view. The authors present their method of minimized mastoidectomy in a cadaver dissection and operative cases, showing potential as a useful option for the PSA.

DOI： 10.3171/2020.1.JNS193179

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：The Japan Neurosurgical Society  

<p>Recently neurosurgical operations have been carried out with water irrigation such as endoscopic third ventriculostomy and tumor resections in ventricles. Water irrigation is one of several published methods that promote hemostasis; however, not enough experimental evidence exists on its efficacy. In this study, we investigate whether hydrostatic pressure and persistent irrigation promote hemostasis in neuroendoscopic surgery. We dissected tails of 12–16-week-old C57BL/6 male mice at 5 mm proximal from the tip and checked for bleeding times under dry and wet conditions at pressures of 0 cmH₂O, 10 cmH₂O, 15 H₂O, and 20 cmH₂O without persistent irrigation to bleeding point and 10 cmH₂O with persistent irrigation. We then examined the dissected edge with hematoxylin–eosin staining and measured the size of vessels. The average bleeding time of each group is as follows: dry: 203.4 sec, wet: 164.4 sec, 5 cmH₂O: 138.6 sec, 10 cmH₂O: 104.6 sec (P <0.001), 20 cmH₂O: 56 sec (P <0.001), and 10 cmH₂O with persistent irrigation: 72.8 sec (P <0.01 compared to 10 cmH₂O without persistent irrigation). The maximum caliber of mice’s tail artery was 50–60 μm. Hydrostatic pressure and irrigation are important factors contributing to hemostasis.</p>

DOI： 10.2176/nmc.oa.2020-0278

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：SN Comprehensive Clinical Medicine  

Endoscopic third ventriculostomy (ETV) has been accepted for obstructive hydrocephalus (OH). We herein present a case of OH and optic nerve sheath edema (ONSE). Although ETV was successful, ONSE worsened. Discussion is focused on the relationship among intracranial hypertension (IH), ETV, and ONSE. The patient was a 19-year-old woman with complaints of headache and emesis. Papilledema was prominent. Radiological examination showed OH as an aqueductal tumor. Endoscopic tumorectomy and ETV were accomplished. Although headache and emesis disappeared, papilledema persisted. On the 4th day, the patient lost light perception. On magnetic resonance imaging, the third ventricular stroma and aqueduct were patent. Nevertheless, ONSE was prominent. After lumbar drainage, her vision was restored. Finally, ventriculoperitoneal shunting was performed, following which ONSE disappeared. IH is not always accompanied by ONSE. However, once it occurs, it may act separately from IH, and ETV may even exacerbate ONSE due to alteration of cerebrospinal fluid (CSF) flow into the chiasmatic cistern. In the optic nerve sheath, CSF pressure is not easily transmitted across different areas; this should explain the dissociation between ONSE and other symptoms of IH. Furthermore, we suggest that ONSE and acute optic dysfunction should be considered an independent crisis as “hydronervus opticus.”

DOI： 10.1007/s42399-020-00688-0

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Background: The efficacy and safety of endoscopic procedures for slit ventricle syndrome (SVS) have been presented in recent studies. However, inserting a sheath into a target ventricle is essential in the beginning of endoscopic procedures for SVS, and this maneuver is challenging owing to the quite narrow ventricular space. We report a novel flexible endoscopic technique, the water-slide technique, that can effectively guide a sheath into a slit ventricle without the use of other adjunctive devices, such as a neuronavigation system and an ultrasound machine. Methods: Ten endoscopic procedures using the water-slide technique were performed in 9 patients with SVS. All patients had undergone ventriculoperitoneal shunt placement. Using the technique, the sheath was first placed on the flexible endoscope. The endoscope was then inserted into the lateral ventricle along the tract around the pre-existing ventricular catheter with irrigation of artificial cerebrospinal fluid via the working channel of the endoscope. After the endoscope reached the ventricle, the sheath was inserted into the ventricle along the endoscope. The endoscope was then used as a stylet. The endoscopic procedure was performed afterward. Continuous irrigation of artificial cerebrospinal fluid via the working channel is important when using this technique. Results: In all cases, the sheath was successfully introduced to the slit ventricle without the use of adjunctive devices. There were no postoperative complications correlated with the maneuver. Conclusions: This novel endoscopic technique is effective and safe for inserting a sheath into a target ventricle in the management of SVS.

DOI： 10.1016/j.wneu.2020.08.206

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：The Japan Neurosurgical Society  

Primary leptomeningeal malignant lymphoma (PLML) is a rare variant of primary central nerve system malignant lymphoma (PCNSL) which is restricted to leptomeninges. The lesions of PLML can often be detected as abnormal enhancement on the surface of central nervous system or the ventricular wall on magnetic resonance imaging (MRIs). Cerebrospinal fluid (CSF) evaluation together with such MRI findings provides the definitive diagnosis of PLML. Here, we present a 45-year-old female case of PLML in which hydrocephalus with disproportionately large fourth ventricle was observed at presentation with gait instability. Head MRI revealed no abnormal enhancement and CSF cytology was negative, leaving the cause of hydrocephalus undetermined. Endoscopic third ventriculostomy (ETV) was effectively performed for hydrocephalus and her symptoms disappeared. Nearly 2 years later, she was brought to emergent room due to unconsciousness with the recurrence of hydrocephalus. MRI showed expanded fourth ventricle and abnormal enhancement on the ventricular wall. The endoscopic surgery for improving CSF flow was successful and inflammatory change was endoscopically observed on the ventricular wall involving aqueduct. Pathological diagnosis of the specimen from the ventricular wall proved B-cell lymphoma. Because neither brain parenchymal masses nor systemic tumors were identified, she was diagnosed with PLML and treated by high-dose methotrexate. She was in a stable state 2 years after the diagnosis of PLML. We report and discuss the characteristics of this case.

DOI： 10.2176/nmccrj.cr.2020-0215

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Background: Neuroendoscopy offers wide and close surgical views with fine illumination, even in deep surgical sites. Furthermore, transcylinder surgery has the advantage that a tubular retractor can protect critical neurovascular structures in the surgical corridor. These advantages of neuroendoscopy and transcylinder surgery can contribute to safer and less invasive surgical approaches for deep-seated fourth ventricular lesions, for which various critical neurovascular structures exist along the surgical route. Case Description: A 54-year-old man with a fourth ventricular cavernoma underwent tumor resection via the endoscopic transcylinder trans-Magendie foraminal approach. A 6.8-mm transparent sheath (cylinder) was introduced into the fourth ventricle via the foramen of Magendie without incisions in the inferior medullary velum or the tela choroidea, resulting in the minimal retraction of and trauma to critical neurovascular structures in the surgical corridor. Under the view of a 2.7-mm rigid neuroendoscope, the lesion was completely removed with preservation of a venous anomaly on the ventral side of the aqueduct of Sylvius. Neuroendoscopy could offer a fine surgical view even under continuous irrigation with artificial cerebrospinal fluid; it prevented collapse of the fourth ventricle and facilitated anatomic understanding by the surgeons. The postoperative course was uneventful. Conclusions: Our novel approach can be an effective surgical option for fourth ventricular lesions with minimal cerebellar retraction and injury.

DOI： 10.1016/j.wneu.2020.06.171

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著者等は内視鏡下経鼻経蝶形骨手術(TSS)における硬膜閉創法のアルゴリズムとして、conventional TSSでは腫瘍摘出腔への脂肪充填および硬膜縫合を行い、硬膜欠損を伴わないextended TSSでは腹部から採取した遊離脂肪片を用いたshoelace closure、硬膜欠損を伴うextended TSSでは遊離腹直筋膜片の硬膜へのpatch縫合+硬膜外での脂肪片貼付+硬性再建を原則としている。今回、その妥当性について検討するため、2013～2019年にTSSを行った383例の術後髄液漏発生率について調査した。手術法の内訳はconventional TSSが278例、extended TSSが105例で、術後髄液瘻は計7例(発生率1.8%)であり、この数値は他報告(1.2～2.2%)と同程度で良好な成績と考えられた。

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当院で2017年2月～2019年10月に内視鏡下経鼻経蝶形骨手術を行った下垂体腺腫59例を対象とし、手術手技と術後中枢性尿崩症(DI)発生との関連性を調べ、DIをきたしやすい因子について検討した。その結果、腺腫の鞍上進展が強く手術で頭蓋内操作を要した症例ではDI発生率が有意に高かった。一方、手術手技のうち「腺腫の被膜外摘出」はDI発生の危険因子ではなかったことから、特に機能性下垂体腺腫において完全な腫瘍摘出を目指す意味での「被膜外摘出」は有用な手技であると考えられた。

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頭蓋咽頭腫の外科治療では腫瘍摘出率の向上と機能温存との両立が求められ、これまで内分泌機能の温存を目的とした下垂体組織・下垂体茎の温存が試みられてきたが、下垂体茎を温存しても下垂体機能は維持されないことが多い。上下垂体動脈(SHA)は視神経と下垂体茎を栄養する血管であり、内視鏡下拡大経鼻経蝶形骨手術(extended eTSS)はSHA・視神経下面・下垂体茎などを一視野に収めることが可能である。今回、当院と関連施設で2009～2019年にextended eTSSを施行した頭蓋咽頭腫69例のうち、初回手術例で、かつ術前に内分泌機能が維持され、術中に下垂体茎が温存され、術中映像を確認できた14例を対象とし、SHA infundibular branchが両側とも温存された群(6例)、片側温存群(5例)、温存不可群(3例)に分け、術後の下垂体前葉機能および後葉機能について群間比較した。結果、前葉機能は両側温存群の4例(67%)が術後も維持されたのに対し、片側温存群と温存不可群では全例に機能低下があり、有意な群間差を認めた。後葉機能は両側温存群の5例(83%)、片側温存群の2例(40%)、温存不可群の1例(33%)で維持され、有意な群間差は認めなかった。

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：The Japan Neurosurgical Society  

<p>An isolated fourth ventricle (IFV) is characterized by fourth ventricular dilation due to obstruction of its inlet and outlet. A disproportionately large communicating fourth ventricle (DLCFV) is a rare subtype of IFV, characterized by dilation of the fourth ventricle, regardless of the size of the lateral ventricles, with no apparent obstruction of the cerebral aqueduct. To our knowledge, this is the first case series describing endoscopic diagnosis and treatment strategy for DLCFV. We retrospectively reviewed six cases of DLCFV in which endoscopic surgery was performed at our institution and affiliated facilities between June 2013 and March 2017. DLCFV was diagnosed using radiographic imaging and intraoperative endoscopy. We also conducted a PubMed search and included only original studies related to DLCFV treatment written in English in our review of the literature. Endoscopic third ventriculostomy (ETV) was performed in all patients. Additional endoscope-assisted placement of a fourth ventriculoperitoneal (VP) shunt was performed in two patients who could not be managed with ETV alone because of severe adhesion of the interpeduncular cistern due to subarachnoid hemorrhage (SAH). The patients’ symptoms and the size of the fourth ventricle improved with surgical treatment, without complications. Endoscopic surgery for DLCFV appears to be a safe and effective treatment. Based on our treatment strategy, ETV is the first-line treatment for DLCFV. Endoscope-assisted placement of the fourth VP shunt can be treatment for severe adhesion of the interpeduncular cistern.</p>

DOI： 10.2176/nmc.oa.2019-0299

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DOI： 10.1016/j.clineuro.2020.105764

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Diffuse midline glioma, H3 K27M-mutant is a lethal brain tumor located in the thalamus, brain stem, or spinal cord. H3 K27M encoded by the mutation of a histone H3 gene such as H3F3A plays a pivotal role in the tumorigenesis of this type of glioma. Although several studies have revealed comprehensive genetic and epigenetic profiling, the prognostic factors of these tumors have not been identified to date. In various cancers, oncogenic driver genes have been found to exhibit characteristic copy number alterations termed mutant allele specific imbalance (MASI). Here, we showed that several diffuse midline glioma, H3 K27M-mutant exhibited high variant allele frequency (VAF) of the mutated H3F3A gene using droplet digital polymerase chain reaction (ddPCR) assays. Whole-genome sequencing (WGS) revealed that these cases had various copy number alterations that affected the mutant and/or wild-type alleles of the H3F3A gene. We also found that these MASI cases showed a significantly higher Ki-67 index and poorer survival compared with those in the lower VAF cases (P < 0.05). Our results indicated that the MASI of the H3F3A K27M mutation was associated with the aggressive phenotype of the diffuse midline glioma, H3 K27M-mutant via upregulation of the H3 K27M mutant protein, resulting in downregulation of H3K27me3 modification.

DOI： 10.1186/s40478-020-0882-4

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Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：(一社)日本内分泌学会  

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BACKGROUND: The endoscopic supracerebellar-infratentorial (SCIT) approach is a viable method to access pathology of the posterior incisura, but a narrow working space and frequent instrument conflict can potentially limit its surgical efficacy. We planned an endoscopic-assisted paramedian infratentorial supracerebellar approach for pineal cyst. CASE DESCRIPTION: Patient was placed in prone position under general anesthesia. His head was rotated to the left side slightly. The location of the transverse sinus was detected with navigation system. A 5 cm linear skin incision was performed, and a 2 cm craniectomy was performed about 2 cm left of the median. The transverse sinus was little bit exposed. Dura was incised in a U-shaped incision with the transverse sinus at the base. The endoscope was advanced along with the culmen. At that time, we observed inferior and superior vermian vein. After reaching to the thick arachnoid near by galenic system, the arachnoid membrane was incised and the CSF was evacuated. After that, the cerebellum became soft and the surgical corridor became large. The arachnoid membrane was incised widely. Pineal cyst, precentral cerebellar vein, bilateral internal occipital vein and great vein of galen were exposed. There were some small veins on the pineal cyst, but the adhesion was not so severe. The cyst was dissected from these small veins. There was no adhesion between the cyst and surrounding brain except for the pineal recess. Bilateral ICV was seen behind the cyst. There was feeding artery and draining vein on the antero-lateral part of the cyst. These vessels were coagulated and cut, then the cyst was removed. After the removal, we confirmed complete removal of the cyst and hemostasis. CONCLUSION: Endoscopic-assisted paramedian SCIT approach for pineal cyst in prone position is a reasonable and efficient access for posterior third ventricular lesions. The learning curve, maneuverability in small space, and instrument conflict limit efficacy.

DOI： 10.25259/SNI_354_2020

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Objective: Olfactory dysfunction is a significant postoperative complication related to endoscopic transsphenoidal surgery (TSS). This study aimed to determine the impact of endoscopic TSS on olfactory function. Methods: We conducted a prospective study of 32 patients with sellar or parasellar tumors who were treated with endoscopic TSS between December 2013 and October 2016. TSS was performed via a right transseptal approach. We performed the Toyota and Takagi olfactometer test and the venous olfaction test for the evaluation of olfactory function preoperatively and at 1 and 3 months postoperatively. Results: The results of the Toyota and Takagi test showed that olfactory function deteriorated in 4 of 32 (12.5%) patients 1 month postoperatively and improved to preoperative baseline levels in all patients 3 months after the procedure. Olfactory function deteriorated in 1 of 31 (3.2%) patients 3 months after the procedure. The venous olfaction test revealed no response in 1 of 31 (3.2%) patients 1 month postoperatively, with no improvement 3 months after the procedure. Conclusions: Endoscopic single-nostril transseptal TSS for sellar or parasellar tumor resection has minimal impact on olfactory function.

DOI： 10.1016/j.wneu.2019.08.051

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OBJECTIVE Transsphenoidal surgery (TSS) is commonly used for anterior skull base surgery, especially in the sella turcica (sellar) region. However, because of its anatomical position, CSF leakage is a major complication of this approach. The authors introduced a new grading reconstruction strategy for anterior skull base surgery with continuous dural suturing in 2013. In this paper the authors report on their methods and results. METHODS All patients with sellar or anterior skull base lesions that were removed with TSS or extended TSS by a single neurosurgeon between April 2013 and March 2017 at Nagoya University Hospital and several cooperating hospitals were retrospectively identified. Three methods of suturing dura were considered, depending on the dural defect. RESULTS There were 176 TSS cases (141 conventional TSS cases and 35 extended endoscopic TSS cases) and 76 cases of Esposito's grade 2 or 3 intradural high-flow CSF leakage. In the high-flow CSF leak group, there were 3 cases of CSF leakage after the operation. The rates of CSF leakage after surgery corresponding to grades 2 and 3 were 2.9% (1/34) and 4.7% (2/42), respectively. CONCLUSIONS Dural suturing is a basic and key method for reconstruction of the skull base, and continuous suturing is the most effective approach. Using this approach, the frequency of cases requiring a nasoseptal flap and lumbar drainage can be reduced.

DOI： 10.3171/2018.4.JNS18366

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Purpose: Functional pituitary adenomas (FPAs) lacking a well-defined pseudocapsule can invade the adjacent pituitary gland. In such situations, peel-off resection of the adjacent pituitary gland after selective adenomectomy might lead to complete tumor removal, resulting in optimal endocrinological outcomes. Here, we present the significance of peel-off resection of the pituitary gland in patients with FPA in whom complete extracapsular tumor removal cannot be achieved. Methods: We performed a retrospective review of 21 patients with FPA who underwent transsphenoidal surgery (TSS). After selective adenomectomy, peel-off resection of the adjacent pituitary gland was performed in 13 patients because complete extracapsular resection could not be achieved, while peel-off resection was not performed in the remaining 8 patients because complete extracapsular resection was accomplished. The clinical outcomes of these groups were compared. The pituitary tissues obtained by peel-off resection were pathologically examined for tumor cells. Results: Early postoperative biochemical remission was achieved in 20 patients (95.2%). Anterior pituitary functions were not aggravated postoperatively in any patient: however, transient diabetes insipidus (DI) occurred in 2 patients. There were no statistically significant differences in the clinical outcomes of the two groups. A pseudocapsule was pathologically detected in the adjacent anterior pituitary even in patients in whom no pseudocapsule was intraoperatively detected. Tumor cells were pathologically detected in 7 (58.3%) of 12 pituitary tissues examined. Conclusions: Peel-off resection of the pituitary gland, which can remove a small tumor cell remnant in the adjacent pituitary, might maximize the effectiveness of TSS with minimal impact on postoperative pituitary function.

DOI： 10.1007/s11102-019-00980-w

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当院で2012～2018年に初発非機能性下垂体腺腫に対して内視鏡下経鼻的経蝶形骨手術を行った78例のうち、65歳以上の33例を高齢群、65歳未満の45例を若年群とし、手術成績を群間比較した。全摘出できた割合は高齢群70%、若年群73%で有意差は認めなかった。術前にみられた症状の改善率は、頭痛が高齢群100%、若年群78%、視機能障害がそれぞれ91%、97%、下垂体前葉機能障害がそれぞれ17%、45%であり、高齢群は下垂体前葉機能障害の改善率が低かった。術後合併症の発生頻度は、低Na血症が高齢群33%、若年群7%、一過性尿崩症がそれぞれ9%、9%、永続的尿崩症がそれぞれ3%、9%、下垂体前葉機能低下がそれぞれ3%、2%、認知機能低下がそれぞれ3%、0%、廃用症候群がそれぞれ3%、0%であり、高齢群は低Na血症の発生頻度が有意に高かった。

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Intraventricular hemorrhage and intracerebral aneurysms are relatively frequent complications associated with moyamoya disease. Prevention of aneurysm rerupture is important because it significantly decreases the morbidity and mortality rates. Aneurysms arising distal to collateral flow are sometimes observed in patients with intraventricular hemorrhage; however, the treatment of these aneurysms remains challenging because of their deep-seated location in the brain and accompanying narrow surgical corridor. The authors describe a neuroendoscopic aneurysm clipping technique performed in 2 cases using a small-diameter tubular retractor for intraventricular aneurysms of the distal lateral posterior choroidal artery. In this technique, the surgical field was continuously irrigated with artificial CSF to keep the ventricle size intact, and aneurysm clipping was performed through a tubular retractor that was introduced with neuronavigational guidance. The patients' postoperative courses were uneventful, and CT angiography revealed complete clipping of the aneurysms and patent parent arteries. Endoscopic clipping using a tubular retractor is an effective and less invasive alternative for treating intraventricular aneurysms. The wet-field endoscopic technique is performed in an aqueous field and maintains an intact ventricle size, allowing for a clear surgical view and a wider, enhanced surgical field.

DOI： 10.3171/2018.1.JNS172393

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Background: Removal of the medial wall of the cavernous sinus (MW) is challenging for neurosurgeons. We describe a practical method of endoscopic MW removal via endonasal transsphenoidal approach to minimalize intraoperative blood loss and postoperative morbidities. We also present the pathologic significance of this technique for functional pituitary adenomas (FPAs). Methods: We performed MW removal in patients with FPA with no well-defined pseudocapsule and a tumor in direct contact with the MW. The MW was judged to have tumor invasion based on the intraoperative appearance, and it was removed regardless of the appearance of MW involvement. Intraoperative findings and postoperative clinical, endocrinologic, and pathologic outcomes were retrospectively reviewed. Results: Fourteen patients underwent MW removal for FPA, including 12 patients with acromegaly and 2 with Cushing disease. Mean intraoperative blood loss was 170 mL (range, 32–400 mL), and none of the patients required blood transfusion. Among the 7 patients without intraoperative apparent MW involvement, 4 (57.1%) had pathologically confirmed tumor invasion into the MW (occult invasion). Biochemical remission by surgery alone was achieved in 13 patients (92.9%). Transient oculomotor palsy occurred in 1 patient (7.1%). Conclusions: Occult tumor invasion into the MW was often detected in patients with FPA without a well-defined pseudocapsule but in direct contact with the MW. Our technique can enhance the effectiveness of surgery with minimal postoperative morbidities.

DOI： 10.1016/j.wneu.2019.02.134

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Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：(一社)日本神経学会  

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Objective: Although reports regarding the relationship between surgical complications and quality of life (QOL) exist, a general consensus regarding this issue is lacking. The aim of this study was to evaluate QOL after endoscopic transsphenoidal approach. Methods: We assessed patients with sellar or parasellar lesions that were removed with endoscopic transsphenoidal approach or extended endoscopic transsphenoidal approach between January 2010 and December 2016. 36-Item Short Form Health Survey and 30-Item General Health Questionnaire QOL scores were obtained preoperatively and 1 month and 6 months postoperatively. Results: We analyzed 89 patients with nonfunctioning pituitary adenoma (NFPA) and 39 patients with acromegaly. Physical QOL decreased 1 month after surgery but recovered after 6 months in both groups. Mental QOL improved postoperatively compared with preoperatively. Patients with acromegaly had a worse Physical Component Summary (PCS) on 36-Item Short Form Health Survey (48.7, 28.9, and 41.0) at 6 months than patients with NFPA. The low preoperative PCS group did not improve until 6 months postoperatively. Multiple regression analysis revealed the following factors were key to postoperative QOL: age (NFPA PCS 6 months postoperatively; correlation coefficient = −0.489), preoperative Mental Component Summary (MCS) (NFPA MCS 6 months postoperatively, correlation coefficient = 0.573), body mass index (acromegaly PCS 6 months postoperatively; correlation coefficient = −0.376), preoperative PCS (acromegaly PCS 6 months postoperatively; correlation coefficient = 0.905), and preoperative MCS (acromegaly MCS 6 months postoperatively; correlation coefficient = 0.726). Conclusions: Endoscopic transsphenoidal approach can improve QOL in patients by 6 months postoperatively. In patients with acromegaly with significant QOL impairments preoperatively, surgery sometimes may be unable to normalize QOL.

DOI： 10.1016/j.wneu.2018.11.017

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DOI： 10.7076/j.wneu.2018.71.017

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当院で傍鞍部腫瘍に対しeTSS(内視鏡下経鼻経蝶形骨手術)を施行した98例(男性50例、女性48例、平均年齢55.9歳)を対象に、術後の遅発性低Na血症(DHN)発症について検討した。その結果、DHNは37例(37.8%)に認められた。DHN発症患者ではDHN発症時のAVP分泌が抑制されていないことが確認された。また、術後7日目においてDHN非発症患者では約3%程度の体重低下を認めるのに対し、DHN発症例では体重減少は1%程度と軽微であり、体内への水分貯留が示唆された。術前より尿崩症が認められた患者群ではDHN発症が少なかったものの、DHN発症例では渇感中枢障害を伴っていた。これは術後の脱水を危惧したADH補充による医原性のものと考えられた。以上、傍鞍部腫瘍の術後DHNの発症にはSIADHが強く関与していることが考えられた。尚、術後早期からの水分制限はDHN発症予防に有効と考えられたが、より適切なプロトコールならびに予測法の確立が必要である。

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Background: Microscopic detection of intracranial brain tumors with 5-aminolevulinic acid (5-ALA) has proven extremely useful, and reports the use of 5-ALA have recently increased. However, few reports have described 5-ALA photodynamic diagnosis (PDD) using a neuroendoscope. We performed neuroendoscopic 5-ALA PDD for various brain lesions and present a procedure using only a neuroendoscope. Methods: We describe the diagnosis of 20 intracranial brain lesion cases with a 5-ALA–guided fluorescence endoscope. A light-emitting diode that emitted either white light or 400- to 410-nm violet light was attached to a neuroendoscope. We performed cylinder surgery with a transparent sheath under observation with a rigid neuroendoscope. Results: Neuroendoscopic biopsies were performed in 11 patients, and resections were performed in 9 patients. All lesions were observed with a neuroendoscope under sequential white light and violet light. We confirmed the presence of a red fluorescent lesion under violet light in 15 patients, including 4 of 5 glioblastoma cases (80%); 1 of 2 anaplastic astrocytoma cases (50%); 4 of 5 diffuse large B cell lymphoma cases (80%); 2 of 2 metastatic brain tumors; 1 of 1 case each of diffuse astrocytoma, pilocytic astrocytoma, inflammatory change, and germinoma (100%); and no cases of anaplastic ependymoma or cysticercosis. Pretargeted lesions were accurately harvested from all biopsy specimens. Gross total resection was achieved in 5 of 9 patients using a resection procedure. Conclusions: Our described method offers a promising technique for achieving precise brain tumor biopsies and safe resection.

DOI： 10.1016/j.wneu.2018.03.112

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OBJECTIVE: Parasellar tumors that extend far laterally beyond the internal carotid artery or that are fibrous and adhere firmly to critical structures are difficult to remove totally via the endoscopic transsphenoidal approach alone. In such cases, a combined transsphenoidal-transcranial approach is effective to achieve maximal resection in a single stage. In this paper, a new minimally invasive surgical technique for complicated parasellar lesions, a fully endoscopic combined transsphenoidal-supraorbital keyhole approach, is presented. METHODS: A retrospective review of patients who had been treated via a fully endoscopic combined transsphenoidal-supraorbital keyhole approach for complicated parasellar lesions was performed. The data for resection rate, perioperative mortality and morbidity, and postoperative outcomes were analyzed. RESULTS: A total of 12 fully endoscopic combined transsphenoidal-supraorbital keyhole approaches were performed from March 2013 to February 2016; 10 were for pituitary adenomas and 2 were for craniopharyngiomas. Gross-total resection or near-total resection was achieved in 7 of 12 cases. Among the 11 patients who had presented with preoperative visual disturbances, 7 had visual improvement. However, 1 patient showed deterioration in visual function. No patient experienced postoperative hemorrhage, needed additional surgical treatment, or had postoperative CSF leakage. CONCLUSIONS: In the combined transsphenoidal and transcranial approach, safe and effective cooperative manipulation with 2 surgical corridors can be performed for complicated parasellar lesions. The goal of this procedure is not to achieve gross-total resection, but to achieve safe resection. Moreover, this new surgical approach offers neurosurgeons a simpler operative field with less invasiveness than the conventional microscopic combined approach. The fully endoscopic combined endonasal-supraorbital keyhole approach is an efficacious procedure for complicated parasellar lesions with acceptable results.

DOI： 10.3171/2016.11.JNS161833

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DOI： 10.1111/ncn3.12176

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Background Flexible endoscopes have both a wide range of movement and a wide field of view and are therefore widely used for endoscopic third ventriculostomy and biopsy. However, tumor resection around the aqueduct of Sylvius using flexible endoscopes has scarcely been reported. Case Description We report 2 cases of tumor resection around the aqueduct of Sylvius. The first case is a 38-year-old man presenting with progressive headache, nausea, and diplopia. Magnetic resonance imaging (MRI) revealed a 1.4 cm nonenhancing mass at the entry of the aqueduct of Sylvius and occlusive hydrocephalus. We performed tumor resection and endoscopic third ventriculostomy (ETV) simultaneously. His symptoms disappeared after the operation. Final pathologic diagnosis was ancient schwannoma. The second case is a 78-year-old woman presenting with progressive disturbance of consciousness (coma). MRI showed 0.7 cm nonenhancing mass at the entry of the aqueduct of Sylvius and occlusive hydrocephalus. We performed tumor resection and ETV similarly. Her consciousness improved after the operation. Final tumor diagnosis was cavernous malformation. In both cases there was no additional neurologic deficit after the surgery. Conclusions By using a flexible endoscope for tumor dissection, resection of a tumor without a neck, which cannot be removed through aspiration alone, becomes possible. To our knowledge, the presented cases are the first to describe the effectiveness of complete resection of a tumor in the third ventricle using flexible endoscopy.

DOI： 10.1016/j.wneu.2017.11.044

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経蝶形骨手術または拡大経蝶形骨法が施行され、術中に髄液漏を認めた61例を対象とし、術後に硬膜下血腫を認めた10例(A群)と認めなかった51例(B群)に分け検討した。年齢、性別、腫瘍の病理・最大径、術前内分泌機能等の患者背景に加えて、脳室拡大の程度を術前MRIでのEvans' indexを用いて評価した。また、術中髄液漏れの程度についてEsposito分類を用いて評価し、硬膜下血腫の発生について術翌日、1週間後、1ヵ月後、3ヵ月後に撮影されたCTまたはMRIを用いて評価した。その結果、A群はB群に比べ年齢、術前下垂体前葉機能低下の合併頻度、Evans' indexが有意に高かった。その他の項目に両群間で有意差は認めなかった。多変量解析を行ったところ、65歳以上、術前下垂体前葉機能低下、Evans' index≧0.28が術後硬膜下血腫発生の有意なリスク因子であった。

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Background Endoscopic surgery has rapidly become widespread in neurosurgery in recent years. Endoscopy can offer close and panoramic surgical views with fine illumination, even in the deep intracranial area. However, it also has the following serious drawback: an intracranial blind area between the field lens of the endoscope and the site of the dural opening. This blind area cannot be viewed on the endoscopic monitor, and several surgical complications, including accidental intracranial neurovascular structural injury, can occur in this area. In this article, we report a new multiscope surgical technique that can compensate for this serious disadvantage of endoscopic surgery. Methods In the multiscope technique, endoscopic and exoscopic systems are used simultaneously with 2 monitors. Microvascular decompression (MVD) is performed fully endoscopically using an exoscope that compensates for the intracranial blind area of the endoscopic view. Two high-definition monitors for the endoscope and exoscope are placed side-by-side in front of the primary surgeon. Results Two patients with hemifacial spasm were treated by endoscopic MVD with the multiscope technique. In these procedures, fine surgical views were obtained by both the endoscope and exoscope. Two monitors were placed side-by-side in front of the surgeon; as a result, the physician could easily view them simultaneously during the operation. No surgery-related complications occurred. Conclusions The multiscope technique can facilitate the performance of safer neuroendoscopic surgery than conventional endoscopic surgery. This technique can also be adopted in other skull base surgeries, in which the importance of endoscopy is growing.

DOI： 10.1016/j.wneu.2017.04.059

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Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：(一社)日本脳神経外科コングレス  

脳幹部海綿状血管腫(BSCM)は、高い出血率を有する血管奇形の1つとして知られている。出血や増大傾向を示すBSCMは神経学的予後に影響するため外科的治療が必要となるが、脳幹部への外科的治療は少しの正常組織への侵襲も術後の神経予後を大きく悪化させる可能性がある。今回筆者らは2例(橋腹側1例、中小脳脚1例)の症候性BSCMに対して内視鏡的に摘出を行った。どちらの症例もMR diffusion tensor image(DTI)を用いて病変部や錐体路の位置を把握した。術中は内視鏡下にwet field methodを用いて摘出および摘出腔内の止血を行い、術後、神経学的な改善が得られた。これらの技術により、今後、脳幹部病変に対しての内視鏡を用いた治療が正確かつ安全に行われるようになると考える。(著者抄録)

DOI： 10.7887/jcns.26.216

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Language：English   Publishing type：Research paper (scientific journal)  

Brain stem cavernous malformations (BSCMs) are associated with a high rate of hemorrhagic events. Though BSCMs that bleed or grow in size are indicated for resection, the surgical procedure usually involves a high risk of damage to the normal brain. We successfully achieved total resection, in two cases of symptomatic BSCMs, using a neuroendoscope. The BSCM was localized in the ventral part of the pons in the first case and in the middle cerebellar peduncle in the second case. In both cases, diffusion tensor images (DTI) were taken with an MRI to confirm the localization of the BSCM in relation to the pyramidal tracts. We resected the BSCMs using a Vet field method' which renders the surgical fields clear without using suction tubes. The wet field provides the operator with an increased range of manipulation even in a tight space. With our technique, the indications of endoscopic surgery have widened. We have shown that neuro-endoscopic surgery can safely and effectively replace conventional craniotomy for certain brain stem lesions.

DOI： 10.7887/jcns.26.216

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Language：English   Publisher：The Japan Neurosurgical Society  

A 61-year-old man presented with massive epistaxis, amaurosis, nausea, and severe headache. A detailed examination revealed a subarachnoid hemorrhage and prolactinoma. Angiography showed a small internal carotid artery pseudoaneurysm and inadequate collateral circulation; thus, uncomplicated coil embolization was performed. Considering the side effects of medication, such as cerebrospinal fluid rhinorrhea, the patient was followed up for asymptomatic prolactinoma without medication after discharge. At 40 months later, aneurysm recurrence was confirmed. Flow diverter device placement was performed, and the outcomes were excellent. In the present report, we described a rare case of a ruptured internal carotid artery aneurysm in an untreated prolactinoma and discussed the literature.

DOI： 10.2176/jns-nmc.2022-0355

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Language：Japanese   Publisher：(一社)日本内分泌学会  

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著者らは、マウス胚性幹細胞(ESC)を用いた三次元分化法により、ヒトESC(hESC)およびヒト人工多能性幹細胞をin vitroで下垂体オルガノイド(PO)へ効率よく分化させる方法を確立し、マウス腎被膜下移植実験でその有効性を確認しているが、ヒトへ臨床応用するためには、腎被膜下移植では侵襲が高い。また幹細胞由来であるため、万が一腫瘍化すると摘出が困難である。そこで今回、侵襲性が比較的低く、摘出も容易な皮下移植方法について、SCIDマウスを用いて検討した。その結果、hESC由来POを下垂体機能低下SCIDマウスの皮下組織に移植しても機能することが示された。また、移植されたマウスはCRHに反応してACTHが血液中に放出されたことから、注入されたCRHが移植されたACTH産生細胞を刺激していると考えられた。鼠径部皮下脂肪内に移植した群と、無血管野に移植した群との比較から、血管の豊富な脂肪組織に移植するほうが、血管の乏しい部位に移植するよりも効果的であることが示唆された。

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Other Link： https://search.jamas.or.jp/default/link?pub_year=2023&ichushi_jid=J01160&link_issn=&doc_id=20230905310001&doc_link_id=%2Fcs6hypop%2F2023%2F0099s1%2F002%2F0004-0006%26dl%3D0&url=https%3A%2F%2Fwww.medicalonline.jp%2Fjamas.php%3FGoodsID%3D%2Fcs6hypop%2F2023%2F0099s1%2F002%2F0004-0006%26dl%3D0&type=MedicalOnline&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00004_2.gif

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Language：Japanese   Publisher：一般社団法人 日本内分泌学会  

DOI： 10.1507/endocrine.98.s.hpt_72

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Other Link： https://search.jamas.or.jp/default/link?pub_year=2022&ichushi_jid=J01160&link_issn=&doc_id=20220817460022&doc_link_id=%2Fcs6hypop%2F2022%2F0098s1%2F023%2F0072-0073%26dl%3D0&url=https%3A%2F%2Fwww.medicalonline.jp%2Fjamas.php%3FGoodsID%3D%2Fcs6hypop%2F2022%2F0098s1%2F023%2F0072-0073%26dl%3D0&type=MedicalOnline&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00004_2.gif

Language：Japanese   Publisher：一般社団法人 日本内分泌学会  

経鼻的内視鏡下頭蓋底手術は下垂体腫瘍をはじめとする傍鞍部腫瘍に対する第一選択の外科的治療法であるが、合併症として術後髄液鼻漏の危険性が伴う。今回、当院と関連施設で2016～2021年に本手術が施行された506例を対象とし、原疾患、手術方法、再建方法、原疾患、術後髄液漏の発生頻度などについて調査し、術後髄液漏発生例について詳細な検討を行った。原疾患は下垂体腺腫294例、頭蓋咽頭腫59例、鞍結節髄膜腫48例、ラトケ嚢胞33例、くも膜嚢胞12例、脊索腫11例などであった。再建方法は通常閉鎖法(鞍内に補綴物を充填し硬膜の縫合を行う)が349例、靴紐閉鎖法(硬膜切開部に補綴物を挟み込むように連続縫合を行う)が116例、パッチ閉鎖法(硬膜欠損部を補綴物でパッチ状に縫合閉鎖する)が41例であった。術後髄液漏は11例(2.2%)に発生し、原疾患の内訳は下垂体腺腫3例、頭蓋咽頭腫3例、鞍結節髄膜腫2例、脊索腫1例、くも膜嚢胞1例、リンパ腫1例であった。再建方法は通常閉鎖法4例、靴紐閉鎖法5例、パッチ閉鎖法2例であった。髄液漏の発生要因について検討した結果、術者側の要因としてグラフトの量や種類・位置に関するものが多く、患者側の要因として多動や放射線治療、脳室腹腔シャント挿入などが考えられた。

DOI： 10.1507/endocrine.98.s.hpt_25

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Other Link： https://search.jamas.or.jp/default/link?pub_year=2022&ichushi_jid=J01160&link_issn=&doc_id=20220817460007&doc_link_id=10.1507%2Fendocrine.98.S.HPT_25&url=https%3A%2F%2Fdoi.org%2F10.1507%2Fendocrine.98.S.HPT_25&type=J-STAGE&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00007_3.gif

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Here we report a rare case of capillary hemangioma (CH) in a 28-year-old woman suffering from gradual worsening diplopia at 28 weeks of pregnancy. Magnetic resonance imaging (MRI) showed a mass lesion (about 3 cm in diameter) in the right parasellar region. We decided to observe as she was pregnant, and had no symptoms other than right abducent nerve palsy. Fortunately, her symptoms did not worsen until delivery. Computed tomography, enhanced MRI, and angiography after delivery revealed that the lesion was highly calcified and vascularized. A dorsum sellae meningioma or highly calcified pituitary adenoma was suspected and the endoscopic transsphenoidal approach was used for tumor removal. The postoperative course was uneventful. The histological diagnosis was CH. Intracranial CHs or CHs of skull are rare vascular tumors. These tumors are reportedly more common in female patients and may change in size in adults according to menstrual cycle and pregnancy. Only six cases, including that of the present study, were diagnosed during the perinatal period. Some of them experienced rapid symptom progression and tumor growth in their course; thus, we should pay further attention to pregnant or peripartum patients with brain tumor, suspected hemangiomas.

DOI： 10.2176/jns-nmc.2021-0326

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Background: Imaging with 18F-fluorodeoxyglucose (FDG)-positron emission tomography (PET) and 11C-methionine (MET)-PET can delineate primary and metastatic brain tumors. Lesion size affects the sensitivity of both scans and histopathological features can also influence FDG-PET, but the effects on MET-PET have not been elucidated. Case Description: We report an unusual case of metastatic brain tumors without accumulation of FDG or MET, contrasting with high FDG uptake in the primary lung lesion. The brain lesions were identified as adenocarcinoma with a more mucus-rich background, contributing to the indistinct accumulation of both FDG and MET. Conclusion: Histopathological characteristics can affect both MET and FDG accumulation, leading to findings contradicting those of the primary lesion.

DOI： 10.25259/SNI_264_2022

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Other Link： https://search.jamas.or.jp/default/link?pub_year=2021&ichushi_jid=J03120&link_issn=&doc_id=20211108370005&doc_link_id=issn%3D0917-1495%26volume%3D31%26issue%3D6%26spage%3D926&url=http%3A%2F%2Fwww.pieronline.jp%2Fopenurl%3Fissn%3D0917-1495%26volume%3D31%26issue%3D6%26spage%3D926&type=PierOnline&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00005_2.gif

Language：Japanese   Publisher：一般社団法人 日本内分泌学会  

DOI： 10.1507/endocrine.97.s.hpt_49

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Other Link： https://search.jamas.or.jp/link/ui/2022074900

Language：Japanese   Publisher：一般社団法人 日本内分泌学会  

DOI： 10.1507/endocrine.97.s.hpt_16

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Primary leptomeningeal malignant lymphoma (PLML) is a rare variant of primary central nerve system malignant lymphoma (PCNSL) which is restricted to leptomeninges. The lesions of PLML can often be detected as abnormal enhancement on the surface of central nervous system or the ventricular wall on magnetic resonance imaging (MRIs). Cerebrospinal fluid (CSF) evaluation together with such MRI findings provides the definitive diagnosis of PLML. Here, we present a 45-year-old female case of PLML in which hydrocephalus with disproportionately large fourth ventricle was observed at presentation with gait instability. Head MRI revealed no abnormal enhancement and CSF cytology was negative, leaving the cause of hydrocephalus undetermined. Endoscopic third ventriculostomy (ETV) was effectively performed for hydrocephalus and her symptoms disappeared. Nearly 2 years later, she was brought to emergent room due to unconsciousness with the recurrence of hydrocephalus. MRI showed expanded fourth ventricle and abnormal enhancement on the ventricular wall. The endoscopic surgery for improving CSF flow was successful and inflammatory change was endoscopically observed on the ventricular wall involving aqueduct. Pathological diagnosis of the specimen from the ventricular wall proved B-cell lymphoma. Because neither brain parenchymal masses nor systemic tumors were identified, she was diagnosed with PLML and treated by high-dose methotrexate. She was in a stable state 2 years after the diagnosis of PLML. We report and discuss the characteristics of this case.

DOI： 10.2176/nmccrj.cr.2020-0215

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