Cranial Fossa, Posterior

颅骨窝,后部
  • 文章类型: Journal Article
    一名40多岁的2型糖尿病患者在另一家医院进行脑脊液(CSF)渗漏修复后,持续存在6个月的右侧水样鼻涕,由于反复出现的症状,促使他来我们这里。影像学检查显示,中斜坡的CSF泄漏已进行了内窥镜内窥镜CSF泄漏修复。遗憾的是,他出现了由多重耐药(MDR)肺炎克雷伯菌引起的术后脑膜炎。由于病原体对常规药物的耐药性和缺乏科学证据,因此处理这一复杂病例是一项具有挑战性的任务。我们启动了头孢他啶的文化指导联合治疗方案,阿维巴坦,氨曲南和替加环素.这一决定源于细致的文献综述,并在测试该生物体时观察到抗生素协同作用。经过4周的警惕治疗,患者的症状明显改善,和CSF培养物是无菌的。我们提出了有效应对和管理术后MDR细菌性脑膜炎的挑战性实例的方法。
    A man in his 40s with type 2 diabetes mellitus had persistent right-sided watery nasal discharge for 6 months following cerebrospinal fluid (CSF) leak repair at another hospital, prompting his visit to us due to recurring symptoms. Imaging revealed a CSF leak from the mid-clivus for which revision endoscopic CSF leak repair was done. Regrettably, he developed postoperative meningitis caused by multidrug-resistant (MDR) Klebsiella pneumoniaeManaging this complex case was a challenging task due to the pathogen\'s resistance to conventional drugs and the scarcity of scientific evidence. We initiated a culture-guided combination regimen with ceftazidime, avibactam, aztreonam and tigecycline. This decision stemmed from meticulous literature review and observed antibiotic synergy while testing for this organism.After 4 weeks of vigilant treatment, the patient\'s symptoms improved significantly, and CSF cultures were sterile. We present our approach to effectively confront and manage a challenging instance of postoperative MDR bacterial meningitis.
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  • 文章类型: Systematic Review
    背景:小脑mutism综合征(CMS)是后颅窝手术的严重并发症,主要影响儿科年龄组。病理生理学仍未完全理解。这会对患者的康复产生不利影响。CMS没有明确的标准化管理。然而,药物治疗已被用于报道的疗效可变的病例。我们的目的是通过这篇综述来总结用于治疗CMS的药物的可用证据。
    方法:在2022年12月之前,使用PubMedCentral进行了全面的系统评价,Embase,和WebofScience,数据库,以确定接受后颅窝手术并接受药物治疗的CMS患者的病例报告和病例系列。除了后颅窝病变以外的其他病理患者被排除在研究之外。
    结果:在592项初步研究中,8项研究符合我们的入选资格标准,通过人工检索增加了3项研究;报告了13例患者.年龄中位数为13岁(标准差SD=10.60)。最常用的药物是溴隐亭。其他药物是氟西汀,咪达唑仑,唑吡坦,和阿立哌唑.大多数患者在开始药物治疗后48小时内康复。中位随访期为4个月(SD=13.8)。所有患者在随访期结束时显示完全康复。
    结论:后颅窝手术后报告小脑mutism综合征,尽管试图确定风险因素,病理生理学,和CMS的管理,它仍然是一种具有挑战性的疾病,发病率很高。已经提出了不同的药理学治疗方法,并取得了有希望的结果。需要进一步的研究和正式的临床试验来评估可用的选择及其有效性。
    Cerebellar mutism syndrome (CMS) is a serious complication of posterior fossa surgeries affecting mainly pediatric age group. The pathophysiology is still not fully understood. It adversely affects the recovery of patients. There is no definitive and standardized management for CMS. However pharmacological therapy has been used in reported cases with variable effectiveness. We aim through this review to summarize the available evidence on pharmacological agents used to treat CMS.
    A thorough systematic review until December 2022, was conducted using PubMed Central, Embase, and Web of Science, databases to identify case reports and case series of CMS patients who underwent posterior fossa surgery and received pharmacological treatment. Patients with pathologies other than posterior fossa lesions were excluded from the study.
    Of 592 initial studies, 8 studies met our eligibility criteria for inclusion, with 3 more studies were added through manual search; reporting on 13 patients. The median age of 13 years (Standard deviation SD=10.60). The most frequent agent used was Bromocriptine. Other agents were fluoxetine, midazolam, zolpidem, and arpiprazole. Most patients recovered within 48 hours of initiating medical therapy. The median follow-up period was 4 months (SD=13.8). All patients showed complete recovery at the end of follow-up period.
    Cerebellar mutism syndrome is reported after posterior fossa surgeries, despite attempts to identify risk factors, pathophysiology, and management of CMS, it remains a challenging condition with significant morbidity. Different Pharmacological treatments have been proposed with promising results. Further studies and formalized clinical trials are needed to evaluate available options and their effectiveness.
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  • 文章类型: Systematic Review
    这封信提供了对题为“在后窝相关病理背景下的体积分割:系统评价”的文章的反馈。“它强调了审查的积极影响,例如对现有文献的全面检查及其提高诊断准确性和治疗计划的潜力。然而,它还解决了与体积分割相关的限制和挑战,包括图像质量和可访问性问题的可变性。
    This letter provides feedback on the article titled \"Volumetric Segmentation in the Context of Posterior Fossa-Related Pathologies: A Systematic Review.\" It highlights the positive impacts of the review, such as its comprehensive examination of existing literature and its potential to enhance diagnostic accuracy and treatment planning. However, it also addresses limitations and challenges associated with volumetric segmentation, including variability in image quality and accessibility issues.
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  • 文章类型: Journal Article
    PHACES综合征是后窝畸形综合征表现的首字母缩写,血管瘤,动脉异常,主动脉缩窄/心脏缺损,眼睛异常和胸骨畸形。婴儿血管瘤是婴儿期最常见的肿瘤。区域性牙齿发育不良,通常被称为“鬼牙”,是牙釉质和牙本质的罕见局部发育畸形,严重程度不同,导致受影响牙齿的异常临床和影像学表现。本报告描述了一例罕见的2岁白种人男性,被诊断患有PHACES综合征,并伴有多区域牙齿发育不良。二十颗牙齿中有十颗发育不良。患者在医院环境下进行全身麻醉治疗。由于敏感,所有受影响的乳牙都被拔除,脓肿和极差的长期预后。往前走,一个长期的跨学科的方法将是必要的,以解决这个孩子的牙列,因为它的发展。
    PHACES syndrome is an acronym for the syndromic presentation of Posterior fossa malformation, Hemangioma, Arterial anomalies, Coarctation of aorta/cardiac defects, Eye abnormalities and Sternal malformations. Infantile hemangiomas are the most common tumors of infancy. Regional odontodysplasia, commonly referred to as \"ghost teeth\", is a rare localized developmental malformation of enamel and dentin with varying levels of severity that results in unusual clinical and radiographic appearances of affected teeth. This report describes a rare case of a two-year-old Caucasian male diagnosed with PHACES syndrome also presenting with multi-regional odontodysplasia. Ten of twenty teeth were dysplastic. The patient was treated under general anesthesia in a hospital setting. All affected primary teeth were extracted due to sensitivity, abscess and extremely poor long-term prognosis. Moving forward, a long-term interdisciplinary approach will be necessary to address this child\'s dentition as it develops.
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  • 文章类型: Journal Article
    位于枕叶或后颅窝的病变的神经外科方法需要非常具体和耗时的患者装置。比如公园的长凳位置,俯卧位,或坐姿。然而,这些位置中的每一个都存在关于特定安装相关不良事件和潜在严重神经外科并发症如静脉空气栓塞的主要缺点,医源性颅内高压,而幕上远隔血肿仅举几例。为了给神经外科医生提供更简单的,生理上各自的,容易忍受,耗时少,和较少的提供者或特定的不良事件患者安装,Ochiai(1979)介绍了仰卧改良的公寓式/侧卧位。鉴于这种病人的位置并没有获得广泛的知名度,在神经外科界,尽管它明显的众多优势,其经典同行,我们提供了我们的经验,使用该装置对位于枕叶和后颅窝的病变进行神经外科手术。
    Neurosurgical approach to lesions located in the occipital lobes or in the posterior fossa require very specific and time-consuming patient installations, such as the park bench position, the prone position, or the sitting position. Nevertheless, each of these position present major drawbacks regarding specific installation-related adverse events and potentially serious neurosurgical complications such as venous air embolism, iatrogenic intracranial hypertension, and supratentorial remote hematoma just to cite a few. In order to provide neurosurgeons with a simpler, physiologically-respective, easily tolerated, less time-consuming, and less provider or specific adverse events patient installation, Ochiai (1979) introduced the supine modified park-bench / lateral decubitus position. Given that this patient position has not gained wide visibility among the neurosurgical community despite its obvious numerous advantages over its classic counterparts, we provide our experience using this installation for neurosurgical approach to lesions located in the occipital lobes and in the posterior fossa.
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  • 文章类型: Journal Article
    背景:由于后颅窝的解剖学特殊性,后颅窝动静脉畸形(pfAVM)是具有挑战性的病变,以及出血性表现的高发生率。治疗AVM最重要的两个目标是保护神经功能和防止破裂。或者第二次出血.这项研究的目的是分析pfAVM的临床和影像学特征,以确定影响这些患者预后的因素。
    方法:我们进行了一项单中心回顾性观察性研究,包括1997年1月至2021年12月在我们机构接受pfAVM治疗的患者。
    结果:共纳入48例患者。33例(69%)在就诊时观察到良好的改良Rankin评分(mRS)。34例患者(71%)出现AVM破裂。在这些中,19例(40%)患者发生脑室内出血。33例(69%)进行了显微外科手术切除,而在其他情况下,患者选择保守治疗(7例,15%),立体定向放射外科(SRS)(6例,12%),或血管内治疗(2例,4%)。年龄≤30岁的患者更容易出现出血(OR:5.23;95%CI:1.42-17.19;p=0.024),在多变量分析后,这仍然是破裂的独立危险因素(OR:4.81;95%CI:1.07-21.53;p=0.040)。在多变量分析之后,在接受手术治疗的亚组中,与不良预后独立相关的唯一因素是入院时的不良临床状态(mRS3-5)(OR:96.14;95%CI:5.15-1793.9;p=0.002).
    结论:后颅窝AVM的治疗具有挑战性,并且存在AVM破裂的患者在入院时的临床状态通常较差,从而导致预后不良。因此,对这些患者进行适当和及时的管理至关重要。
    BACKGROUND: Posterior fossa arterio-venous malformations (pfAVMs) are challenging lesions due to the anatomical particularities of the posterior fossa, and the high incidence of hemorrhagic presentation. The two most important goals when treating AVMs are preserving neurological function and preventing rupture, or a second hemorrhage. The aim of this study was to analyze the clinical and imaging features of pfAVMs to identify the factors that influence the prognosis of these patients.
    METHODS: We conducted a single-center retrospective observational study that included patients treated at our institution with pfAVMs between January 1997 and December 2021.
    RESULTS: A total of 48 patients were included. A good modified Rankin score (mRS) was observed in 33 cases (69%) at presentation. Thirty-four patients (71%) presented with a ruptured AVM. Out of these, 19 patients (40%) had intraventricular hemorrhage. Microsurgical resection was performed in 33 cases (69%), while in the other cases, the patients opted for conservative management (7 cases, 15%), stereotactic radiosurgery (SRS) (6 cases, 12%), or endovascular treatment (2 cases, 4%). Patients ≤ 30 years old were more prone to hemorrhagic presentation (OR: 5.23; 95% CI: 1.42-17.19; p = 0.024) and this remained an independent risk factor for rupture after multivariate analysis as well (OR: 4.81; 95% CI: 1.07-21.53; p = 0.040). Following multivariate analysis, the only factor independently associated with poor prognosis in the surgically treated subgroup was a poor clinical status (mRS 3-5) at admission (OR: 96.14; 95% CI: 5.15-1793.9; p = 0.002).
    CONCLUSIONS: Management of posterior fossa AVMs is challenging, and patients who present with ruptured AVMs often have a poor clinical status at admission leading to a poor prognosis. Therefore, proper and timely management of these patients is essential.
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  • 文章类型: Case Reports
    巨细胞瘤(GCT)是破骨细胞样细胞的局部侵袭性原发性骨肿瘤。大多数GCT发生在长骨内,和主要的GCTs涉及clivus是非常罕见的。我们介绍了一个18岁男孩的病例,该男孩患有双眼水平复视,其发病隐匿,在磁共振图像上发现具有低信号增强的肿块,涉及斜坡和左侧背囊。通过内镜经鼻内侧入路完全切除肿瘤,通过免疫组织化学进行的组织病理学检查显示为GCT。手术后患者的左外展神经麻痹略有改善。由于GCT的稀有性,对于最终的治疗方案尚无共识.然而,我们建议全切是首选的治疗方法,denosumab在次全切除患者中起着至关重要的作用。
    Giant cell tumors (GCTs) are locally aggressive primary bone tumors of osteoclast-like cells. Most GCTs occur within the long bones, and primary GCTs involving the clivus are extremely rare. We present the case of an 18-year-old boy with binocular horizontal diplopia with an insidious onset who was found to have a hypointense enhancing mass involving the clivus and left side dorsum sellae on magnetic resonance images. The tumor was completely resected via an endoscopic endonasal transclival approach, and histopathologic examination via immunohistochemistry indicated a GCT. The patient\'s left abducens nerve palsy improved slightly after surgery. Because of the rarity of GCTs, there is no consensus about the definitive treatment protocol. However, we suggest that gross total resection is the treatment of choice, and denosumab plays a critical role in patients with subtotal resection.
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  • 文章类型: Journal Article
    目的:这项研究的目的是描述颅底偶然出现的良性脊索病变的自然史,特别注意可以使与低级别脊索瘤的区分更加困难的特征,即造影剂摄取和骨侵蚀。
    方法:在本回顾性病例系列中,作者描述了58例偶然出现的良性脊索病变的临床结果,包括那些具有轻微骨侵蚀或造影剂吸收的放射学特征。
    结果:在近3年的中位随访期间,所有病变均保持稳定。37例(64%)患者接受了对比增强MRI;这些患者中有14例(38%)的病变表现出最小的对比增强。27例(47%)患者接受了CT检查;这些患者中有6例(22%)的病变表现出轻微的骨侵蚀。
    结论:这些数据使我们有理由首先监测选定的斜坡良性出现的脊索病变病例,即使有少量的对比剂摄取或轻微的骨侵蚀。
    The aim of this study was to describe the natural history of incidental benign-appearing notochordal lesions of the skull base with specific attention to features that can make differentiation from low-grade chordoma more difficult, namely contrast uptake and bone erosion.
    In this retrospective case series, the authors describe the clinical outcomes of 58 patients with incidental benign-appearing notochordal lesions of the clivus, including those with minor radiological features of bone erosion or contrast uptake.
    All lesions remained stable during a median follow-up of almost 3 years. Thirty-seven (64%) patients underwent contrast-enhanced MRI; lesions in 14 (38%) of these patients exhibited minimal contrast enhancement. Twenty-seven (47%) patients underwent CT; lesions in 6 (22%) of these patients exhibited minimal bone erosion.
    These data make the case for monitoring selected cases of benign-appearing notochordal lesions of the clivus in the first instance even when there is minor contrast uptake or minimal bone erosion.
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  • 文章类型: Journal Article
    目的:本研究旨在提供原发性斜坡脊索瘤的延长结局数据,关注无进展生存期(PFS)和总生存期(OS)。
    方法:对1987年至2022年间使用手术治疗的斜坡脊索瘤患者进行了回顾性单中心分析,立体定向放射外科,或质子放射治疗(PRT)。
    结果:该研究包括100名患者(中位年龄44岁,51%男性)。71例患者(71%)使用鼻内镜入路进行手术。39例患者(39%)获得了大体全切除(GTR)或接近全切除(NTR)。术后,新的颅神经缺陷发生在7%,脑脊液泄漏4%,没有一个病人出现脑膜炎。79例患者(79%)接受放射治疗,以PRT50例(50%)为主要治疗方法。在73个月(四分位距[IQR]38-132)的中位随访期间,确诊41例复发(41%)和31例死亡(31%)。GTR/NTR患者的中位PFS为41(IQR24-70)个月。进行次全切除或活检的患者的中位PFS为38个月(IQR16-97)。接受放射治疗的患者的中位PFS为43(IQR26-86)个月,而未接受放射治疗的患者为18个月(IQR5-62)。Kaplan-Meier方法显示,GTR/NTR患者(p=0.007)和接受放射治疗的患者(p<0.001)的PFS比同行更长。初级治疗后5、10、15和20年的PFS率为51%,25%,17%,7%,分别。相同时间间隔的OS率为84%,60%,42%,34%,分别。多因素Cox回归分析显示年龄<44岁(p=0.02),更大程度的切除(EOR;p=0.03),和放射治疗(p<0.001)与较低的复发率相关。另一个多变量分析表明,年龄<44岁(p=0.01),EOR更大(p=0.04),无复发(p=0.02)与较低的死亡率相关.关于病理学数据,98%的brachyury为阳性,泛细胞角蛋白占93%,85%的上皮膜抗原,和S100在74%。无免疫组织化学标记与复发相关。
    结论:在这项研究中,年龄较小,最大安全切除,放射治疗是原发性斜坡脊索瘤患者PFS延长的重要因素。防止复发在实现更长的操作系统中起着至关重要的作用。
    This study aimed to provide data on extended outcomes in primary clival chordomas, focusing on progression-free survival (PFS) and overall survival (OS).
    A retrospective single-center analysis was conducted on patients with clival chordoma treated between 1987 and 2022 using surgery, stereotactic radiosurgery, or proton radiation therapy (PRT).
    The study included 100 patients (median age 44 years, 51% male). Surgery was performed using the endoscopic endonasal approach in 71 patients (71%). Gross-total resection (GTR) or near-total resection (NTR) was attained in 39 patients (39%). Postoperatively, new cranial nerve deficits occurred in 7%, CSF leak in 4%, and meningitis in none of the patients. Radiation therapy was performed in 79 patients (79%), with PRT in 50 patients (50%) as the primary treatment. During the median follow-up period of 73 (interquartile range [IQR] 38-132) months, 41 recurrences (41%) and 31 deaths (31%) were confirmed. Patients with GTR/NTR had a median PFS of 41 (IQR 24-70) months. Patients with subtotal resection or biopsy had a median PFS of 38 (IQR 16-97) months. The median PFS of patients who received radiation therapy was 43 (IQR 26-86) months, while that of patients who did not receive radiation therapy was 18 (IQR 5-62) months. The Kaplan-Meier method showed that patients with GTR/NTR (p = 0.007) and those who received radiation therapy (p < 0.001) had longer PFS than their counterparts. The PFS rates following primary treatment at 5, 10, 15, and 20 years were 51%, 25%, 17%, and 7%, respectively. The OS rates at the same intervals were 84%, 60%, 42%, and 34%, respectively. Multivariate Cox regression analysis showed that age < 44 years (p = 0.02), greater extent of resection (EOR; p = 0.03), and radiation therapy (p < 0.001) were associated with lower recurrence rates. Another multivariate analysis showed that age < 44 years (p = 0.01), greater EOR (p = 0.04), and freedom from recurrence (p = 0.02) were associated with lower mortality rates. Regarding pathology data, brachyury was positive in 98%, pan-cytokeratin in 93%, epithelial membrane antigen in 85%, and S100 in 74%. No immunohistochemical markers were associated with recurrence.
    In this study, younger age, maximal safe resection, and radiation therapy were important factors for longer PFS in patients with primary clival chordomas. Preventing recurrences played a crucial role in achieving longer OS.
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  • 文章类型: Journal Article
    目的:后颅窝切除前的小脑膨出是手术中的紧急情况。术中小脑膨出不仅增加了病灶切除的难度,而且还会带来额外的术后并发症。目前,关于这个主题的系统报告很少。本文讨论了小脑膨出的预测因素以及如何有效预防术中小脑膨出。
    方法:回顾性分析527例后颅窝病变患者的临床及影像学资料。评估围手术期临床和影像学数据。使用单变量和多变量回归分析对变量进行分析。
    结果:总体而言,10.4%(55/527)的患者术中出现急性隆起。多因素分析显示年龄<60岁,BMI≥24,病变大小≥30(mm),小脑扁桃体疝和/或脑积水,和周围水肿(中度-重度)是小脑膨出的预测因素。小脑隆起的缓解可以通过切除病变来实现,释放脑脊液,并去除小脑(外1/3)。4例患者术后出现明显的小脑相关并发症,随访6个月后症状消失。
    结论:后颅窝切除术中小脑膨出值得关注。通过对小脑隆起相关多因素的分析,围手术期的综合评估和早期干预是必要的。小脑隆起的发生率可以减少,可以避免与小脑隆起相关的手术并发症。
    OBJECTIVE: A cerebellar bulge prior to posterior fossa resection is an emergency condition during surgery. Intraoperative cerebellar bulging not only increases the difficulty of lesion resection but also brings additional postoperative complications. Currently, there are few systematic reports on this topic. The predictors of cerebellar bulge and how to effectively prevent intraoperative cerebellar bulge are discussed in this article.
    METHODS: The clinical and imaging data of 527 patients with posterior fossa lesions who underwent resection at our hospital were retrospectively collected and analyzed. Perioperative clinical and imaging data were assessed. Variables were analyzed using univariate and multivariate regression analyses.
    RESULTS: Overall, 10.4% (55/527) of patients had intraoperative acute bulges. Multivariate analysis revealed that age <60 years, body mass index ≥24, lesion size ≥30 (mm), cerebellar tonsillar herniation and/or hydrocephalus, and perilesional edema (moderate-severe) were predictors of cerebellar bulging. Relief of the cerebellar bulge can be accomplished by excising the lesion, releasing cerebrospinal fluid, and removing the cerebellum (the outer one-third). Obvious cerebellar-related complications occurred in 4 patients postoperatively, and the symptoms disappeared after 6 months of follow-up.
    CONCLUSIONS: Cerebellar bulging during intraoperative posterior fossa resection deserves attention. Through the analysis of multiple factors related to cerebellar bulge, comprehensive evaluation and early intervention during the perioperative period are necessary. The incidence of cerebellar bulges can be reduced, and surgical complications related to cerebellar bulges can be avoided.
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