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Surgical Approaches to the Third Ventricle: An Update. 第三脑室的外科手术方法:最新进展。
Pub Date : 2023-01-01 DOI: 10.1007/978-3-031-36785-4_8
Nicola Onorini, Pietro Spennato, Giuseppe Mirone, Francesca Vitulli, Domenico Solari, Luigi Maria Cavallo, Giuseppe Cinalli

The third ventricle is located in the deepest part of the brain and is delimited by both telencephalic and diencephalic structures. Its location makes every surgical procedure inside or around it quite challenging, due to the distance from the surface to the fragility of the neurovascular structures that is necessary to dissect before entering its cavity and to the narrow surgical corridors through which it is necessary to work. Its geometric localization inside the cranial cavity and the anatomical relationship with the interhemispheric fissure offers nevertheless to the surgeon an impressive variety of surgical approaches, which allow to reach every millimeter of the third ventricle lumen. Mastering properly all these approaches requires an impressive anatomical knowledge, the best available technology, and most refined technical skills, making the surgery of the third ventricle a point of excellence in the evolution of each neurosurgeon. The development of neuronavigation and neuroendoscopy has been a revolution in neurosurgery in the last 20 years and offered special advantages for the surgery of the third ventricle. In fact, the narrow corridors of approach make the precision of the neuronavigation and the enlightenment and magnification of the neuroendoscopy especially useful to reach the third ventricle cavity and working inside or around it. This chapter reviews the history of the surgery of the third ventricle and offers an update of the variety of surgical corridors identified and of the technology now available to properly work through them and inside the third ventricle cavity.

第三脑室位于大脑的最深处,由端脑和间脑结构界定。它的位置使它内部或周围的每一次手术都非常具有挑战性,因为从表面到进入它的空腔前需要解剖的神经血管结构的脆弱性,以及它需要通过的狭窄手术走廊。然而,它在颅腔内的几何定位以及与半球间裂的解剖关系为外科医生提供了令人印象深刻的各种手术方法,可以到达第三脑室内腔的每一毫米。正确掌握所有这些方法需要令人印象深刻的解剖学知识、最佳可用技术和最精细的技术技能,使第三脑室手术成为每位神经外科医生发展过程中的一个卓越点。在过去的20年里,神经导航和神经内窥镜的发展是神经外科的一场革命,为第三脑室的手术提供了特殊的优势。事实上,狭窄的通道使神经导航的准确性以及神经内窥镜的启示和放大对于到达第三脑室并在其内部或周围工作特别有用。本章回顾了第三脑室手术的历史,并介绍了已确定的各种手术通道的最新情况,以及目前可用于通过这些通道和在第三脑室内正确工作的技术。
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引用次数: 0
Trapped Fourth Ventricle: Pathophysiology, History and Treatment Strategies. 受困第四脑室:病理生理、病史及治疗策略。
Pub Date : 2023-01-01 DOI: 10.1007/978-3-031-28202-7_11
Pasquale Gallo, Fardad T Afshari

Trapped fourth ventricle is a clinic-radiological entity characterised by progressive neurological symptoms due to enlargement and dilatation of fourth ventricle secondary to obstruction to its outflow. There are several causative mechanisms for the development of trapped fourth ventricle, including previous haemorrhage, infection or inflammatory processes. However, this condition is most commonly observed in ex preterm paediatric patients shunted for a post-haemorrhagic or post-infective hydrocephalus. Until the introduction of endoscopic aqueductoplasty and stent placement, treatment of trapped fourth ventricle was associated with high rates of reoperation and complications resulting in morbidity. With the advent of new endoscopic techniques, supratentorial and infratentorial approaches for aqueductoplasty and stent insertion have revolutionised the treatment of trapped fourth ventricle. Fourth ventricular fenestration and direct shunting remain viable options in cases where aqueduct anatomy and length of obstruction is not surgically favourable for endoscopic approaches. In this book chapter, we explore the background, historical developments,$ and surgical treatment strategies in the management of this challenging condition.

第四脑室阻塞是一种临床放射学特征,其特征是由于第四脑室的扩大和扩张继发于其流出受阻而导致的进行性神经系统症状。第四脑室淤陷有几种病因机制,包括先前的出血、感染或炎症过程。然而,这种情况最常见于早产儿童患者分流出血后或感染后脑积水。在引入内窥镜导水管成形术和支架放置之前,第四脑室被困的治疗与高再手术率和并发症相关。随着新的内窥镜技术的出现,幕上和幕下入路的导水管成形术和支架置入已经彻底改变了第四脑室的治疗。在输尿管解剖结构和梗阻长度不适合内窥镜入路的情况下,第四脑室开窗和直接分流仍然是可行的选择。在这本书的章节中,我们探讨的背景,历史发展,$和手术治疗策略在管理这一具有挑战性的条件。
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引用次数: 0
Evolving Concepts of Craniovertebral and Spinal Instability. 颅椎和脊柱不稳定概念的演变。
Pub Date : 2023-01-01 DOI: 10.1007/978-3-031-28202-7_7
Atul Goel, Ravikiran Vutha, Abhidha Shah

Weakness of the muscles of the nape of the neck and back of the spine and its related instability is the nodal point of pathogenesis of a number of clinical and pathological events at the craniovertebral junction and the spine. Whilst acute instability results in sudden and relatively severe symptoms, chronic or long-standing instability is associated with a range of musculoskeletal and structural spinal alterations. Telescoping of the spinal segments results in "vertical" spinal instability in the subaxial spine and central or axial atlantoaxial instability (CAAD) at the craniovertebral junction. Instability in such cases might not be observed on dynamic radiological imaging. Chiari formation, basilar invagination, syringomyelia, and Klippel-Feil alteration are some of the secondary alterations as a result of chronic atlantoaxial instability. Radiculopathy/myelopathy related to spinal degeneration or ossification of posterior longitudinal ligament appears to have their origin from vertical spinal instability. All the secondary alterations in the craniovertebral junction and subaxial spine that are traditionally considered pathological and to have compressive and deforming role are essentially protective in nature, are indicative of instability, and are potentially reversible following atlantoaxial stabilization. Stabilization of unstable spinal segments is the basis of surgical treatment.

颈背和脊柱后部肌肉无力及其相关的不稳定性是颅椎交界处和脊柱的许多临床和病理事件发病的节点。急性不稳定导致突然和相对严重的症状,慢性或长期不稳定与一系列肌肉骨骼和脊柱结构改变有关。脊柱节段的伸缩式导致下轴棘的“垂直”脊柱不稳定,以及颅椎交界处的中央或轴向寰枢不稳定(CAAD)。在这种情况下,动态放射成像可能没有观察到不稳定。慢性寰枢椎不稳定导致的继发性改变包括Chiari形成、颅底内陷、脊髓空洞和Klippel-Feil改变。与脊柱退变或后纵韧带骨化相关的神经根病/脊髓病似乎起源于脊柱垂直不稳定。颅椎交界处和亚轴棘的所有继发性改变,传统上被认为是病理性的,具有压缩和变形作用,本质上是保护性的,表明不稳定,并且在寰枢关节稳定后可能可逆。稳定不稳定的脊柱节段是手术治疗的基础。
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引用次数: 0
Junctional Neural Tube Defect (JNTD): A Rare and Relatively New Spinal Dysraphic Malformation. 连接神经管缺损(JNTD):一种罕见且相对较新的脊柱发育不良畸形。
Pub Date : 2023-01-01 DOI: 10.1007/978-3-031-34981-2_5
Sebastian Eibach, Dachling Pang

Junctional neurulation completes the sequential embryological processes of primary and secondary neurulation as the intermediary step linking the end of primary neurulation and the beginning of secondary neurulation. Its exact molecular process is a matter of ongoing scientific debate. Abnormality of junctional neurulation-junctional neural tube defect (JNTD)-was first described in 2017 based on a series of three patients who displayed a well-formed secondary neural tube, the conus, that is physically separated by a fair distance from its companion primary neural tube and functionally disconnected from rostral corticospinal control. Several other cases conforming to this bizarre neural tube arrangement have since appeared in the literature, reinforcing the validity of this entity. The clinical, neuroimaging, and electrophysiological features of JNTD, as well as the hypothesis of its embryogenetic mechanism, will be described in this chapter.

连接神经的形成是连接初级和次级神经发育的开始和结束的中间步骤,完成了初级和次级神经发育的序贯胚胎学过程。其确切的分子过程一直是科学界争论的焦点。连接神经管缺陷(JNTD)的异常于2017年首次被描述为基于一系列三名患者,他们表现出结构良好的次级神经管,即锥体,其物理上与其伴生的初级神经管分开相当距离,并且在功能上与吻侧皮质脊髓控制断开。此后,文献中出现了其他几个符合这种奇怪神经管排列的病例,加强了这种实体的有效性。本章将描述JNTD的临床、神经影像学和电生理特征,以及其胚胎发生机制的假设。
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引用次数: 0
Fetal Surgery for Myelomeningocele: Neurosurgical Perspectives. 胎儿髓母细胞瘤手术:神经外科的视角。
Pub Date : 2023-01-01 DOI: 10.1007/978-3-031-34981-2_2
Dominic N P Thompson, Philippe De Vloo, Jan Deprest

More than 30 years have elapsed since it was recognised that folic acid supplementation could substantially reduce the risk of open neural tube defects (ONTDs). During that time, many countries have adopted policies of food fortification with demonstrable reduction in the incidence of both cranial and spinal ONTDs. Improved prenatal detection and termination has also resulted in a reduction in the number of affected live births. Nonetheless, in the USA about 1500 children, and in the UK around 500 children are born each year with myelomeningocele (MMC) and so the management of MMC and its complications continues to constitute a significant clinical workload for many paediatric neurosurgical units around the world.Until recently, the options available following antenatal diagnosis of MMC were termination of pregnancy or postnatal repair. As a result of the MOMS trial, prenatal repair has become an additional option in selected cases (Adzick et al., N Engl J Med 364(11):993-1004, 2011). Fetal surgery for myelomeningocele is now offered in more than 30 centres worldwide. The aim of this chapter is to review the experimental basis of prenatal repair of MMC, to critically evaluate the neurosurgical implications of this intervention and to describe the technique of 'open' repair, comparing this with emerging minimally invasive alternatives.

自从人们认识到补充叶酸可以大大降低开放性神经管畸形(ONTD)的风险以来,已经过去了 30 多年。在此期间,许多国家采取了食品营养强化政策,头颅和脊柱开放性神经管畸形的发病率明显下降。产前检测和终止妊娠技术的改进也减少了受影响活产婴儿的数量。尽管如此,在美国和英国,每年仍分别有大约 1500 名和 500 名新生儿患有脊髓脊膜膨出症(MMC),因此,MMC 及其并发症的治疗仍然是世界各地许多儿科神经外科单位的一项重要临床工作。由于 MOMS 试验的结果,产前修复已成为特定病例的额外选择(Adzick 等人,N Engl J Med 364(11):993-1004,2011 年)。目前,全球有30多家中心提供胎儿脊髓膜膨出手术。本章旨在回顾产前修复脊髓膜膨出症的实验基础,批判性地评估这一干预措施对神经外科的影响,并介绍 "开放式 "修复技术,将其与新出现的微创替代方法进行比较。
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引用次数: 0
Clinical and Surgical Approach for Cerebral Cortical Dysplasia. 大脑皮质发育不良的临床和外科治疗方法。
Pub Date : 2023-01-01 DOI: 10.1007/978-3-031-36785-4_12
Marcelo Volpon Santos, Camila Araujo Bernardino Garcia, Ana Paula Andrade Hamad, Ursula Thome Costa, Americo Ceiki Sakamoto, Antonio Carlos Dos Santos, Helio Rubens Machado

The present article describes pathophysiological and clinical aspects of congenital malformations of the cerebral tissue (cortex and white matter) that cause epilepsy and very frequently require surgical treatment. A particular emphasis is given to focal cortical dysplasias, the most common pathology among these epilepsy-related malformations. Specific radiological and surgical features are also highlighted, so a thorough overview of cortical dysplasias is provided.

本文描述了先天性脑组织畸形(皮质和白质)的病理生理和临床方面,这些畸形会导致癫痫,并且经常需要手术治疗。特别强调的是局灶性皮质发育异常,这是这些癫痫相关畸形中最常见的病理学。还强调了特定的放射学和外科特征,因此提供了皮质发育不良的全面概述。
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引用次数: 0
The Supraorbital Eyebrow Approach in Pediatric Neurosurgery: Perspectives and Challenges of Frontal Keyhole Surgery. 儿童神经外科的眶上眉入路:正面锁孔手术的前景和挑战。
Pub Date : 2023-01-01 DOI: 10.1007/978-3-031-36785-4_5
Aminaa Sanchin, Eckart Bertelmann, Pablo Hernáiz Driever, Anna Tietze, Ulrich-Wilhelm Thomale

Object: Supraorbital craniotomy via an eyebrow incision provides minimally invasive cosmetically favorable access to both orbital and intracranial pathologies. We describe the indication, surgical technique, and clinical course using this surgical approach in a cohort of patients from a single pediatric neurosurgery unit.

Methods: In a retrospective analysis, we identified all surgical cases between January 2013 and April 2022 who underwent the supraorbital craniotomy via an eyebrow incision. Craniotomy was performed using piezosurgery ultrasonic bone incision. An interdisciplinary team of an orbital surgeon and a neurosurgeon performed the orbital surgeries. Clinical and surgical characteristics, perioperative data, possible complications, or redo surgeries as well as ophthalmologic status were assessed.

Results: Clinical data of 37 interventions (cases) in 30 patients (age: 8 ± 6.5 years) were analyzed. The supraorbital craniotomy established access to the cranial, lateral, and central portions of the orbit (n = 11) and ipsilateral fronto-medial portions of the skull base (n = 26). Thirty cases suffered from tumor disease with heterogeneous histopathologic diagnoses, and in 13 cases, adjuvant therapy was required. The mean duration of surgery was 163 ± 95 min, and the mean time of hospital stay was 6.0 ± 2.8 days. In two cases (5.4%), the following complications were observed. One infection treated by puncture and antibiotics and one revision surgery was necessary due to loosening of osteosynthesis material. Postoperative visual function was stable compared to preoperative status after all interventions. After a mean follow-up time of 26 ± 25.9 months for oncologic cases the long term outcome was complete remission in 13, stable disease in 14, progressive disease in 1 and death in 2 patients.

Conclusion: The supraorbital eyebrow approach is feasible and safe in pediatric neurosurgical cases as a minimally invasive and cosmetic favorable technique and should be considered for intraorbital as well as ipsilateral intracranial lesions adjacent to the skull base. Interdisciplinary cooperation enables a broader spectrum of surgical options in orbital and complex, fronto-basal, skull base pathologies.

目的:通过眉毛切口进行眶上开颅手术,为眼眶和颅内病变提供了微创的、美容上有利的途径。我们描述了在一组来自单个儿科神经外科的患者中使用这种手术方法的适应症、手术技术和临床过程。方法:在回顾性分析中,我们确定了2013年1月至2022年4月期间通过眉毛切口接受眶上开颅手术的所有外科病例。颅骨切开术采用压电超声骨切开术。由一名眼眶外科医生和一名神经外科医生组成的跨学科团队进行了眼眶手术。评估了临床和手术特点、围手术期数据、可能的并发症或再次手术以及眼科状况。结果:分析了30例(年龄:8±6.5岁)患者的37种干预措施(例)的临床数据。眶上开颅术可进入眼眶的颅骨、外侧和中央部分(n=11)以及颅底的同侧额内侧部分(n=26)。30例患者患有不同组织病理学诊断的肿瘤疾病,其中13例需要辅助治疗。平均手术时间为163±95分钟,平均住院时间为6.0±2.8天。两例(5.4%)出现以下并发症。由于骨合成材料松动,需要进行一次穿刺和抗生素治疗的感染和一次翻修手术。在所有干预措施后,术后视觉功能与术前状态相比是稳定的。肿瘤学病例的平均随访时间为26±25.9个月后,长期结果为13例患者完全缓解,14例患者病情稳定,1例患者病情进展,2例患者死亡。结论:眶上眉入路在小儿神经外科手术中是可行和安全的,是一种微创和美容的有利技术,应考虑用于颅底附近的眶内和同侧颅内病变。跨学科合作使眼眶和复杂的额基底和颅底病变有了更广泛的手术选择。
{"title":"The Supraorbital Eyebrow Approach in Pediatric Neurosurgery: Perspectives and Challenges of Frontal Keyhole Surgery.","authors":"Aminaa Sanchin,&nbsp;Eckart Bertelmann,&nbsp;Pablo Hernáiz Driever,&nbsp;Anna Tietze,&nbsp;Ulrich-Wilhelm Thomale","doi":"10.1007/978-3-031-36785-4_5","DOIUrl":"https://doi.org/10.1007/978-3-031-36785-4_5","url":null,"abstract":"<p><strong>Object: </strong>Supraorbital craniotomy via an eyebrow incision provides minimally invasive cosmetically favorable access to both orbital and intracranial pathologies. We describe the indication, surgical technique, and clinical course using this surgical approach in a cohort of patients from a single pediatric neurosurgery unit.</p><p><strong>Methods: </strong>In a retrospective analysis, we identified all surgical cases between January 2013 and April 2022 who underwent the supraorbital craniotomy via an eyebrow incision. Craniotomy was performed using piezosurgery ultrasonic bone incision. An interdisciplinary team of an orbital surgeon and a neurosurgeon performed the orbital surgeries. Clinical and surgical characteristics, perioperative data, possible complications, or redo surgeries as well as ophthalmologic status were assessed.</p><p><strong>Results: </strong>Clinical data of 37 interventions (cases) in 30 patients (age: 8 ± 6.5 years) were analyzed. The supraorbital craniotomy established access to the cranial, lateral, and central portions of the orbit (n = 11) and ipsilateral fronto-medial portions of the skull base (n = 26). Thirty cases suffered from tumor disease with heterogeneous histopathologic diagnoses, and in 13 cases, adjuvant therapy was required. The mean duration of surgery was 163 ± 95 min, and the mean time of hospital stay was 6.0 ± 2.8 days. In two cases (5.4%), the following complications were observed. One infection treated by puncture and antibiotics and one revision surgery was necessary due to loosening of osteosynthesis material. Postoperative visual function was stable compared to preoperative status after all interventions. After a mean follow-up time of 26 ± 25.9 months for oncologic cases the long term outcome was complete remission in 13, stable disease in 14, progressive disease in 1 and death in 2 patients.</p><p><strong>Conclusion: </strong>The supraorbital eyebrow approach is feasible and safe in pediatric neurosurgical cases as a minimally invasive and cosmetic favorable technique and should be considered for intraorbital as well as ipsilateral intracranial lesions adjacent to the skull base. Interdisciplinary cooperation enables a broader spectrum of surgical options in orbital and complex, fronto-basal, skull base pathologies.</p>","PeriodicalId":72077,"journal":{"name":"Advances and technical standards in neurosurgery","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41142003","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Blood Blister-Like Aneurysms of the Internal Carotid Artery. 颈内动脉的血泡样动脉瘤。
Pub Date : 2023-01-01 DOI: 10.1007/978-3-031-36785-4_14
Eduardo Vieira, Arlindo U Netto, Auricelio B Cezar, Igor Faquini, Nivaldo S Almeida, Hildo R C Azevedo-Filho

Internal carotid artery blood blister-like aneurysms are challenging lesions that arise from the artery trunk at non-branching sites. They have been recognized since 1969 and are distinct from typical saccular aneurysms. Usually, these aneurysms are broad-based, with no clearly identifiable neck and have extremely friable and fragile walls, thus with a great propensity to cause subarachnoid hemorrhage and to rupture during treatment. Apparently, blister-like aneurysms are formed through an acquired defect of the inner layers (tunica intima and media) of the internal carotid artery wall, probably due to hemodynamic stress in the carotid siphon.Several surgical and endovascular techniques have been described for the treatment of these aneurysms, however, there is still no consensus on the best technique or method, exposing how challenging the treatment of internal carotid artery blister-like aneurysms is, for both neurosurgeons and neurointerventionists. In this chapter, we review the main aspects of the pathogenesis, diagnosis, and therapeutics and report our experience in the microsurgical treatment of these formidable lesions.

颈内动脉血泡状动脉瘤是由非分支部位的动脉干引起的具有挑战性的病变。自1969年以来,人们已经认识到它们与典型的囊状动脉瘤不同。通常,这些动脉瘤是广泛的,没有清晰可识别的颈部,壁极为脆弱,因此在治疗过程中极易引起蛛网膜下腔出血和破裂。显然,水泡状动脉瘤是通过颈内动脉壁内层(内膜和中膜)的后天性缺陷形成的,可能是由于颈动脉虹吸管的血液动力学应力。已经描述了几种手术和血管内技术来治疗这些动脉瘤,然而,对于最佳的技术或方法仍然没有达成共识,这暴露了颈内动脉泡状动脉瘤的治疗对神经外科医生和神经干预学家来说是多么具有挑战性。在本章中,我们回顾了发病机制、诊断和治疗的主要方面,并报告了我们在显微外科治疗这些可怕病变方面的经验。
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引用次数: 0
The Cerebellar Mutism Syndrome: Risk Assessment, Prevention and Treatment. 小脑性缄默症综合征:风险评估、预防和治疗。
Pub Date : 2023-01-01 DOI: 10.1007/978-3-031-28202-7_4
Jonathan Kjær Grønbæk, Ditte Boeg Thomsen, Karin Persson, René Mathiasen, Marianne Juhler

Cerebellar mutism syndrome (CMS) has received increasing attention over the last decades as a complication of posterior fossa tumour surgery in children. Risk factors, aetiological aspects, and treatment measures of the syndrome have been investigated, yet the incidence of CMS remains unchanged. Overall, we are currently able to identify patients at risk, but we are unable to prevent it from occurring.Once CMS sets in, several symptomatic pharmacological treatments have been suggested, but only in smaller case series and not in randomized controlled trials, and it is not clear whether the treatment or time itself had a helpful effect.Within weeks to months, most patients regain their ability to speak after a phase with mutism or severely reduced speech; however, many patients continue to have speech and language deficits. At this point, anti-cancer treatment with chemotherapy and radiotherapy may be of focus more than the prognosis of CMS; however, many patients continue to have speech and language problems for months and years to come, and they are at high risk of other neurocognitive sequelae as well.Without reliable measures to prevent or treat the syndrome, we may look towards improving the prognosis of speech and neurocognitive functioning in these patients. As speech and language impairment is the cardinal symptom and late effect of CMS, the effect of intense and early-onset speech and language therapy as a standard of care in these patients should be investigated in relation to its effect on regaining speech capacity.

小脑性缄默症(CMS)作为儿童后窝肿瘤手术的并发症,在过去的几十年里得到了越来越多的关注。CMS的危险因素、病因学方面和治疗措施已被调查,但CMS的发病率仍未改变。总的来说,我们目前能够识别有风险的患者,但我们无法阻止它的发生。一旦出现CMS,就有几种对症药物治疗的建议,但只是在较小的病例系列中,而不是在随机对照试验中,并且不清楚治疗或时间本身是否有帮助的效果。大多数患者在经历了一段失语或严重言语障碍的阶段后,会在几周到几个月内恢复说话能力;然而,许多患者仍然有言语和语言缺陷。此时,化疗和放疗的抗癌治疗可能比CMS的预后更受关注;然而,许多患者在接下来的几个月甚至几年里仍然有言语和语言问题,而且他们患其他神经认知后遗症的风险也很高。如果没有可靠的措施来预防或治疗这种综合征,我们可能会指望改善这些患者的言语和神经认知功能的预后。由于言语和语言障碍是CMS的主要症状和晚期效应,因此应研究将高强度、早发性言语和语言治疗作为这类患者的标准治疗方法对言语能力恢复的影响。
{"title":"The Cerebellar Mutism Syndrome: Risk Assessment, Prevention and Treatment.","authors":"Jonathan Kjær Grønbæk,&nbsp;Ditte Boeg Thomsen,&nbsp;Karin Persson,&nbsp;René Mathiasen,&nbsp;Marianne Juhler","doi":"10.1007/978-3-031-28202-7_4","DOIUrl":"https://doi.org/10.1007/978-3-031-28202-7_4","url":null,"abstract":"<p><p>Cerebellar mutism syndrome (CMS) has received increasing attention over the last decades as a complication of posterior fossa tumour surgery in children. Risk factors, aetiological aspects, and treatment measures of the syndrome have been investigated, yet the incidence of CMS remains unchanged. Overall, we are currently able to identify patients at risk, but we are unable to prevent it from occurring.Once CMS sets in, several symptomatic pharmacological treatments have been suggested, but only in smaller case series and not in randomized controlled trials, and it is not clear whether the treatment or time itself had a helpful effect.Within weeks to months, most patients regain their ability to speak after a phase with mutism or severely reduced speech; however, many patients continue to have speech and language deficits. At this point, anti-cancer treatment with chemotherapy and radiotherapy may be of focus more than the prognosis of CMS; however, many patients continue to have speech and language problems for months and years to come, and they are at high risk of other neurocognitive sequelae as well.Without reliable measures to prevent or treat the syndrome, we may look towards improving the prognosis of speech and neurocognitive functioning in these patients. As speech and language impairment is the cardinal symptom and late effect of CMS, the effect of intense and early-onset speech and language therapy as a standard of care in these patients should be investigated in relation to its effect on regaining speech capacity.</p>","PeriodicalId":72077,"journal":{"name":"Advances and technical standards in neurosurgery","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10009941","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Apert Syndrome: Selection Rationale for Midface Advancement Technique. Apert综合征:面中推进技术的选择依据。
Pub Date : 2023-01-01 DOI: 10.1007/978-3-031-28202-7_13
Cassio Eduardo Raposo-Amaral, Enrico Ghizoni, Cesar Augusto Raposo-Amaral

Apert syndrome is characterized by a wide spectrum of craniofacial clinical features that have been successfully addressed via a variety of midface advancement techniques. Although surgeons have individual preferences as to which specific procedures should be performed to best treat Apert patients, craniofacial plastic surgeons, working in tandem with pediatric neurosurgeons, can identify and evaluate functional limitations and facial morphologic disproportions, and establish appropriate criteria for effective midface advancement technique indication and selection. The purpose of this review article is to present and discuss our rationale for midface advancement technique selection based upon the most common craniofacial characteristics presented by Apert syndrome patients. The present article also provides a grading system that stratifies as major, moderate, and mild, the effect of each midface advancement technique on the different types of Apert syndrome facial features. Surgeons should take into consideration the maximum effect and benefit of each craniofacial osteotomy and how these procedures will alter the craniofacial skeleton. By understanding the long-term effect of each osteotomy on the most common craniofacial characteristics of Apert syndrome patients, craniofacial plastic surgeons and neurosurgeons will be able to customize the surgical procedures they perform in order to achieve the best possible outcomes.

Apert综合征的特点是广泛的颅面临床特征,已成功地通过各种中面推进技术解决。尽管外科医生对于哪种特定的手术可以最好地治疗Apert患者有个人的偏好,颅面整形外科医生与儿科神经外科医生合作,可以识别和评估功能限制和面部形态失调,并为有效的中脸推进技术指征和选择建立适当的标准。这篇综述文章的目的是根据Apert综合征患者最常见的颅面特征来介绍和讨论我们选择中面部推进技术的基本原理。本文还提供了一个分级系统,将每种中脸推进技术对不同类型Apert综合征面部特征的影响分为主要、中度和轻度。外科医生应考虑到每次颅面截骨术的最大效果和益处,以及这些手术将如何改变颅面骨骼。通过了解每次截骨术对Apert综合征患者最常见颅面特征的长期影响,颅面整形外科医生和神经外科医生将能够定制他们执行的手术程序,以达到最佳可能的结果。
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引用次数: 0
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Advances and technical standards in neurosurgery
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