Tratamiento neuroendoscópico de quistes aracnoideos en edad pediátrica. Resultados de una serie de 20 pacientes

IntroductionNeuroendoscopy has become an effective and safe treatment for arachnoid cysts in the paediatric population. We review the paediatric patients with arachnoid cysts treated by neuroendoscopy in our hospital and analyse the results.Material and methodsA retrospective analysis of 20 patients operated on from 2005 to 2018. The variables assessed are: gender, age, clinical presentation, cyst site, presence of hydrocephalus and/or extra-axial collections, endoscopic procedures and complications. Procedure success is defined as an improvement in symptoms and reduction in cyst size until end of follow-up.ResultsOur series comprised 13 males and 7 females (mean age: 64.6 months, range: 4–172 months). The most frequent site was suprasellar-prepontine (7), followed by intraventricular (6), quadrigeminal (3), interhemispheric (2) and Sylvian (2).A total of 70% (14/20) of patients had hydrocephalus at diagnosis, which increased to 85% in suprasellar-prepontine cysts and 100% in quadrigeminal cysts. Only 4/14 patients with required a ventriculoperitoneal shunt (median age at diagnosis: 12.5 months). Of these 4 patients, 3 developed severe shunt overdrainage.The procedure was successful in 60% (12/20) of the patients in the series. Success by location was 57% (4/7) in suprasellar cysts, 33% (1/3) in quadrigeminal cysts, 66% (4/6) in intraventricular cysts, 100% (2/2) in interhemispheric cysts and 50% (1/2) in Sylvian cysts. Treatment thus failed in 8 cases, with a mean time to failure of 12.12 months (range: 0-45 months). A new neuroendoscopic procedure was performed in 4 of these 8 cases (success in 2/4), a ventriculoperitoneal shunt was placed in 2 cases, a cystoperitoneal shunt was placed in 1 case and the remaining case was managed conservatively. Mean follow-up time was 52.45 months (range: 3-129 months).ConclusionsNeuroendoscopy is an effective and safe treatment for arachnoid cysts in paediatric patients that also enables managing associated hydrocephalus in most cases. The choice of neuroendoscopic procedure and success rate depend on cyst location. Younger patients have been found to have a higher shunt dependency rate. In these cases, measures to prevent shunt overdrainage are recommended.     

Endoscopic ultrasound-guided through-the-needle microforceps biopsy and needle-based confocal laser-endomicroscopy increase detection of potentially malignant pancreatic cystic lesions: A single-center study

BACKGROUNDCurrently, there is insufficient data about the accuracy in the diagnosing of pancreatic cystic lesions (PCLs), especially with novel endoscopic techniques such as with direct intracystic micro-forceps biopsy (mFB) and needle-based confocal laser-endomicroscopy (nCLE).AIMTo compare the accuracy of endoscopic ultrasound (EUS) and associated techniques for the detection of potentially malignant PCLs: EUS-guided fine needle aspiration (EUS-FNA), contrast-enhanced EUS (CE-EUS), EUS-guided fiberoptic probe cystoscopy (cystoscopy), mFB, and nCLE.METHODSThis was a single-center, retrospective study. We identified patients who had undergone EUS, with or without additional diagnostic techniques, and had been diagnosed with PCLs. We determined agreement among malignancy after 24-mo follow-up findings with detection of potentially malignant PCLs via the EUS-guided techniques and/or EUS-guided biopsy when available (EUS malignancy detection). RESULTSA total of 129 patients were included, with EUS performed alone in 47/129. In 82/129 patients, EUS procedures were performed with additional EUS-FNA (21/82), CE-EUS (20/82), cystoscopy (27/82), mFB (36/82), nCLE (44/82). Agreement between EUS malignancy detection and the 24-mo follow-up findings was higher when associated with additional diagnostic techniques than EUS alone [62/82 (75.6%) vs 8/47 (17%); OR 4.35, 95%CI: 2.70-7.37; P < 0.001]. The highest malignancy detection accuracy was reached when nCLE and direct intracystic mFB were both performed, with a sensitivity, specificity, positive predictive value, negative predictive value and observed agreement of 100%, 89.4%, 77.8%, 100% and 92.3%, respectively (P < 0.001 compared with EUS-alone). CONCLUSIONThe combined use of EUS-guided mFB and nCLE improves detection of potentially malignant PCLs compared with EUS-alone, EUS-FNA, CE-EUS or cystoscopy.     

消囊汤治疗卵巢囊肿32例临床研究

目的：研究中药消囊汤对卵巢囊肿的影响。方法：将62例卵巢囊肿妇女随机分为消囊汤组及桂枝茯苓胶囊组,两组分别于月经后用自拟消囊汤和中成药桂枝茯苓胶囊治疗,进行临床效果观察和治疗前后B超检测。结果：用药2～6个月,随访半年,消囊汤和桂枝茯苓胶囊治疗卵巢囊肿均有效果（P〈0．05）,但两组总有效率有显著性差异（P〈0．05）,前者疗效率明显优于后者。结论：消囊汤治疗卵巢囊肿有显著疗效,且有扶正祛邪作用。     

先天性遗传性蛛网膜囊肿引发对橄榄体脑桥小脑萎缩的临床研究

目的初步探讨橄榄体脑桥小脑萎缩（olivopontocerebellar atrophy，OPCA）的显微解剖结构、病因和发病机制。方法对近5年北京天坛医院神经外科28例OPCA的患者作临床、分子遗传学及影像学特征研究。结果OPCA是主要累及小脑、脑干和脊髓的进行性神经变性病。结论OPCA有遗传和散发两类，临床有多种类型和变异型。对OPCA所采用的基因诊断可以直接在DNA水平上鉴定突变基因。SCA1、SCA2、SCA7的CAG可作为基因诊断的标志。在临床上凡有小脑性共济失调，特别是以此为首发症状者，均应首选做MR检查，这对OPCA的诊断及鉴别诊断具有重要意义。     

骨内腱鞘囊肿的影像学表现

目的:探讨骨内腱鞘囊肿的影像学表现及诊断与鉴别诊断.方法:回顾性分析经手术证实的20例骨内腱鞘囊肿的临床及影像资料,均行X线、CT检查,2例行MRI检查.结果:X线、CT表现为邻近关节面的类圆形囊样透亮区,边界清晰,18例有完整硬化边,10例病灶内见粗细不均的条状骨性间隔,3个病灶通过裂隙与关节腔相通,CT值20～60 HU,MRI在T2WI和脂肪抑制序列均表现为高信号.结论:骨内腱鞘囊肿的X线、CT典型表现为骨内邻近关节面的囊状破坏,边界清晰硬化,MRI在T2WI和脂肪抑制序列均表现为高信号,结合发病部位和临床症状及影像学表现,对本病可作出正确诊断.     

Mlc1-Expressing Perivascular Astrocytes Promote Blood–Brain Barrier Integrity

In the mammalian brain, perivascular astrocytes (PAs) closely juxtapose blood vessels and are postulated to have important roles in the control of vascular physiology, including regulation of the blood–brain barrier (BBB). Deciphering specific functions for PAs in BBB biology, however, has been limited by the ability to distinguish these cells from other astrocyte populations. In order to characterize selective roles for PAs in vivo, a new mouse model has been generated in which the endogenous megalencephalic leukoencephalopathy with subcortical cysts 1 (Mlc1) gene drives expression of Cre fused to a mutated estrogen ligand-binding domain (Mlc1-T2A-CreERT2). This knock-in mouse model, which we term MLCT, allows for selective identification and tracking of PAs in the postnatal brain. We also demonstrate that MLCT-mediated ablation of PAs causes severe defects in BBB integrity, resulting in premature death. PA loss results in aberrant localization of Claudin 5 and -VE-Cadherin in endothelial cell junctions as well as robust microgliosis. Collectively, these data reveal essential functions for Mlc1-expressing PAs in regulating endothelial barrier integrity in mice and indicate that primary defects in astrocytes that cause BBB breakdown may contribute to human neurologic disorders.SIGNIFICANCE STATEMENT Interlaced among the billions of neurons and glia in the mammalian brain is an elaborate network of blood vessels. Signals from the brain parenchyma control the unique permeability properties of cerebral blood vessels known as the blood–brain barrier (BBB). However, we understand very little about the relative contributions of different neural cell types in the regulation of BBB functions. Here, we show that a specific subpopulation of astrocyte is essential for control of BBB integrity, with ablation of these cells leading to defects in endothelial cell junctions, BBB breakdown, and resulting neurologic deficits.     

Rathke裂囊肿的MRI诊断

目的探讨垂体Rathke裂囊肿的MRI表现。材料与方法回顾性分析23例经手术病理证实的Rathke裂囊肿的MRI表现,包括病灶的位置、大小、信号,增强特点及相邻结构的改变。结栗19例位于鞍内,4例位于鞍内鞍上。增强后病灶均无强化。类圆形12例,椭圆形8例,不规则型3例。MRI信号变化多样。部分病例垂体柄、视交叉及鞍底有爱压。结论Rathke裂囊肿在MRI上的信号复杂,依其囊内容物成分而有不同表现,单凭信号及形态与鞍区其它囊性病变难以区分,动态及延迟增强无强化有助于鉴别。     

非寄生虫性肝脏囊性疾病的诊断与治疗

随着影像学技术的飞速发展,非寄生虫性肝脏囊性疾病的临床诊断取得了巨大的进步,但对于某些疾病的鉴别诊断仍显不足.单纯多发肝囊肿与多囊肝病的鉴别诊断需结合家族遗传史、囊肿数目、是否合并多囊肾、有无分隔及B超检查特点;单纯单发肝囊肿与肝内胆管囊腺瘤的鉴别需结合影像学特点及活组织病理学检查结果.在治疗上,腹腔镜技术已广泛应用于肝脏囊性疾病的治疗,但并不能完全取代开腹手术,具体选择仍需依据患者的特点采取个体化、多样性的治疗手段.     

儿童先天性胆管囊状扩张症的诊治

目的探讨儿童先天性胆管囊状扩张症的诊断及治疗。方法回顾性分析本院2000年3月至2011年8月治疗的28例儿童先天性胆管囊状扩张症患者临床资料。结果 28例年龄1岁8月至14岁,其中27例行囊肿完整切除、肝总管空肠Roux-en-Y吻合术,1例行囊肿大部分切除、肝总管空肠Roux-en-Y吻合术。获随访25例,随访时间为1～3年,患者情况良好,无结石、癌变、黄疸等。放置胆道金属支架固定好,引流通畅。结论影像学检查在儿童先天性胆管囊状扩张症的诊断中具有重要价值,儿童先天性胆管囊状扩张症的手术方式与其临床分型相关。     

纵隔囊肿的外科治疗：（附72例报告）

外科治疗纵隔囊肿７２例，其中支气管囊肿３２例，心包囊肿１１例，心包憩室３例，皮样囊肿９例，胸腺囊肿３例，食管囊肿８例及囊状淋巴管瘤６例。本病无特异性临床症状或Ｘ线表现，术前确诊困难；针吸活检可提高诊断率。作者报告了本病诊断与治疗的体会。