Dandy—Walker综合征的CT诊断

笔者报告７例Ｄａｎｄｙ－Ｗａｌｋｅｒ综合征，均经ＣＴ检查后确诊，其中６例手术证实，２例有手术后ＣＴ复查，Ｄａｎｄｙ－Ｗａｌｋｅｒ综合征主要病理基础是第四脑室顶盖部的发育异常，此征的ＣＴ表现有惊人的相似性，它们是：（１）小脑蚓部缺失；（２）第四脑室从缺失的蚓部向后上方扩张，可呈囊袋状、扇形、三角形、（３）小脑半球向前外方分离退缩，两侧可不对称；（４）天幕上抬，窦汇及横窦升高，后颅凹扩大；（５）幕上脑     

Dandy-Walker综合征1例报告

Ｄａｎｄｙ－Ｗａｌｋｅｒ综合征１例报告Ｄａｎｄｙ－ＷａｌｋｅｒＳｙｎｄｒｏｍｅ：ＡＲｅｐｏｒｔｏｆｏｎｅＣａｓｅ邓生德朱少军甘肃省张掖地区医院ＣＴ室７３４０００Ｄａｎｄｙ－Ｗａｌｋｅｒ综合征又称先天性第四脑室中、侧孔闭塞，是较少见的先天性后脑发育畸形...     

Dandy-Walker 综合征的临床CT分析(附9例报告)

Ｄａｎｄｙ Ｗａｌｋｅｒ综合征又称先天性第四脑室正中孔、外侧孔闭锁 ,是一种很少见的先天性菱脑发育畸形。 1914年Ｄａｎｄｙ首先尸解报告 ,195 4年Ｂｅｎｄｏ首次以Ｄａｎｄｙ Ｗａｌｋｅｒ综合征正式命名。国内至今报道不足 60例。本文分析了 9例Ｄａｎｄｙ Ｗａｌｋｅｒ综合征的临床和ＣＴ表现 ,并探讨对该病的ＣＴ诊断和鉴别诊断要点 ,旨在提高对本病的认识。1　材料与方法本组 9例 ,男 5例 ,女 4例。年龄 1～ 5 2岁 ,平均 2 3岁 ,其中 14岁以上 6例。临床表现 :脑积水征 2例 ;癫痫 4例 ;智力发育迟缓 3例 ;行走不稳、四肢震颤、言语…     

Dandy-Walker畸形的影像学表现（附5例报告及文献复习）

Ｄａｎｄｙ -Ｗａｌｋｅｒ畸形属旧小脑发育畸形 ,该病在先天性后颅窝畸形中相对少见 ,Ｂａｒｋｏｖｉｃｈ将Ｄａｎｄｙ -Ｗａｌｋｅｒ畸形、Ｄａｎｄｙ-Ｗａｌｋｅｒ变异和巨小脑延髓池等一组相近似的异常统称为Ｄａｎｄｙ -Ｗａｌｋｅｒ综合症。这组疾病影像学表现较具特征 ,是临床诊断的主要依据。本文综合报告 5例典型Ｄａｎｄｙ -Ｗａｌｋｅｒ畸形 ,结合文献复习该病的临床、颅骨Ｘ线平片、脑血管造影、ＣＴ以及ＭＲＩ表现 ,讨论该病影像学诊断的意义。1　病例报告病例 1　女 ,5岁。因生后头围增大 ,神经运动发育落后就诊。ＣＴ表现 :…     

丹-瓦氏综合征的CT诊断(附5例报告)

Dandy-Walker综合征又称先天性第四脑室中孔侧孔闭锁，属罕见的颅脑先天发育畸形。我院发现5例，均经CT、手术或脑室造影检查确诊,现结合文献讲行分析讨论如下。1材料与结果见附表。附表Dandy-Walker综合征的CT诊断2·讨论本病是一种少见的先天性后颅凹发育畸形。Benda于1954年首次命名为Dandy-Walker综合征。French［1］1982年归纳各家本病病史及病因有4个要点：（1）胎生期第四脑室出孔闭锁；（2）胎生期小脑蚓部融合不良；（3）胚胎期神经管闭合不全；（4）脑脊液动力学异常。病理上表现为四脑室中孔侧孔闭锁、四脑室囊状扩大、小脑…     

后颅窝囊肿的CT－MR影像学

目的：本文目的是对后颅窝囊肿的ＣＴ－ＭＲ影像学的诊断作用进行评价。材料与方法：１２例后颅窝囊肿的ＣＴ和ＭＲ影像表现进行了分析，探讨了其与临床表现和类型的相关性。结果：本组病例包含Ｄａｎｄｙ－Ｗａｌｋｅｒ囊肿（５例）、蛛网膜囊肿（３例）和巨枕大池（４例），ＣＴ和ＭＲ都可提供无创性检测手段。结论：ＣＴ和ＭＲ二者对后颅窝囊肿的诊断均有效，但在显示病变的性状、大小和轮廓方面，ＭＲ比ＣＴ稍胜一筹     

后颅窝囊肿的CT—MR影像学

目的：本文目的是对后颅窝囊肿的ＣＴ－ＭＲ影像学的诊断作用进行评价。材料与方法：１２例后颅窝囊肿的ＣＴ和ＭＲ影像表现进行了分析，探讨了其与临床表现和类型的相关性。结果：本组病例包含Ｄａｎｄｙ－Ｗａｌｋｅｒ囊肿（５例）、蛛网膜囊肿（３例）和巨枕大池（４例），ＣＴ和ＭＲ都可提供无创性检测手段。结论：ＣＴ和ＭＲ二者对后颅窝囊肿的诊断均有效，但在显示病变的性状、大小和轮廓方面，ＭＲ比ＣＴ稍胜一筹。     

儿童脱髓鞘病CT形态学改变在诊断中的意义（附九例分析）

笔者报道了经ＣＴ检查的９例脱髓鞘病变。这些病例中脱髓鞘病变的ＣＴ形态学分布有一定的特异性，有利于进一步定性。其中肾上腺脑白质营养不良（ＡＬＤ）６例；ＡＬＤ－Ⅰ型３例，伴病变区钙化１例；ＡＬＤ－Ⅰ和Ⅱ型兼有者２例；单纯侧脑室三角区周围钙化者１例。Ａｌｅｘａｎｄｅｒ病２例：临床伴巨脑，ＣＴ表现为额部为主的脑白质病变。Ｃｏｃｋａｙｎｅ综合征１例：有特殊的临床表现，这是诊断该病的依据；ＣＴ表现为颅内多发弥     

颅内神经上皮性囊肿的CT及MRI诊断(附13例报告)

目的 提高ＣＴ、ＭＲＩ对颅内神经上皮性囊肿的诊断水平。方法 １３例经ＣＴ及ＭＲＩ检查,并经手术光学显微镜配合免疫组织化学（简称免疫组化）检查证实。结果 ＣＴ及ＭＲＩ显示囊肿位于第３脑室前上部３例,第４脑室４例,桥小脑角２例,侧脑室三角区１例,额叶与侧脑室前角１例,鞍区、小脑蚓部各１例。ＣＴ显示囊性低密度１１例,等密度（脑组织密度）、高密度（高于脑组织密度）各１例,ＭＲＩ检测（５例）Ｔ１ＷＩ呈低信号     

儿童脱髓鞘病变CT形态学改变在诊断中的意义（附九例分析）

笔者报道了经ＣＴ检查的９例脱髓鞘病变。这些病例中脱髓鞘病变的ＣＴ形态学分布有一定的特异性，有利于进一步定性。其中肾上腺脑白质营养不良（ＡＬＤ）６例；ＡＬＤ－Ｉ型３例，伴病变区钙化１例；ＡＬＤ－Ｉ型和ＩＩ型兼有者２例；单纯侧脑室三角区周围钙化者１例。Ａｌｅｘａｎｄｅｒ病２例：临床伴巨脑，ＣＴ表现为以额部为主的脑白质病变。Ｃｏｃｋａｙｎｅ综合征１例：有特殊的临床表现，这是诊断该病的依据；ＣＴ表现为颅内多发弥漫对称性钙化伴脑白质数量减少。本文还对病理改变与ＣＴ表现之间的关系以及若干脱髓鞘病变的鉴别作了讨论。     

先天性四脑室中、侧孔闭锁(Dandy—Walker Syndrome)的CT诊断

本文报告20例先天性四脑室中侧孔闭锁的CT诊断,男性13例,女性7例,年龄45d～8岁.CT表现:第四脑室囊状扩大9例;第四脑室囊状扩大通过小脑溪与后颅窝小囊腔相通4例,或大囊腔相连3例;第四脑室与两侧小脑半球大小不等的两囊腔相连1例;扩大的第四脑室向天幕上延伸7例;枕大池、脑沟、脑裂消失20例;第三脑室及两侧侧脑室对称性重度积水扩大20例.合并右额顶区硬膜下积液1例.本病需与后颅窝蛛网膜囊肿、囊性新生物和变异的巨大枕大池以及重度小脑发育不全鉴别.     

Dandy-Walker综合征的MRI诊断(附6例报告)

目的:探讨Dandy-Walker综合征(DWS)的临床及MRI表现特征。方法:对6例经MRI诊断为DWS进行回顾性分析。结果:MRI主要表现为小脑蚓部缺如,小脑半球部分发育不全伴分离、移位;第四脑室囊状扩大与后颅窝囊腔相通;后颅窝扩张伴有横窦、窦汇和小脑幕上移;常伴有幕上不同程度梗阻性脑积水及其他颅内畸形。结论:DWS的MRI表现具有特征性,MRI是诊断DWS的最理想的影像学方法。     

Blake's pouch cyst: an entity within the Dandy-Walker continuum

Abnormal cerebrospinal fluid (CSF) collections within the posterior fossa are defined by the Dandy-Walker complex (DWC) and by arachnoid cysts (AC). The DWC includes the Dandy-Walker malformation (DWM), the Dandy-Walker variant (DWV) and the mega-cisterna magna (MCM). In addition, Tortori-Donati et al. added persistent Blake's pouch cyst (BPC) as an independent entity within the DWC. BPC represents a posterior ballooning of the superior medullary velum into the cisterna magna. All of these malformations are overlapping developmental anomalies characterized by varying degrees of malformation of the medullary vela, the cerebellar vermis and hemispheres, the fourth ventricle choroid plexus, the posterior fossa subarachnoid cisterns and the enveloping meningeal structures. We present two cases of persistent BPC detected in two adult women without history of gestational or subsequent growth problems. They underwent neuroradiological investigation because of headache and because of recurrent episodes of loss of consciousness, respectively. The MRI findings included tetraventricular hydrocephalus, wide communication of the fourth ventricle and the cystic posterior fossa (i. e. BPC), inferior posterior fossa mass effect with or without hypoplasia of both the cerebellar vermis and the medial aspects of the cerebellar hemispheres, and absence of communication between fourth ventricle and the basal subarachnoid space in the midline posteriorly. Persistent BPC is defined by a failure of embryonic assimilation of the area membranacea anterior within the tela choroidea associated with imperforation of the foramen of Magendie. Typically this condition becomes symptomatic early in life. In the current cases the normal function of the laterally positioned foramina of Luschka probably helped to maintain some CSF flow between intraventricular and subarachnoid spaces, with the establishment of a precarious equilibrium characterized by a compensatory enlargement of the cerebral ventricular system (i. e. hydrocephalus). Received: 2 July 1999 Accepted: 13 July 1999     

胎儿小脑蚓部发育的MRI研究

目的:描述正常胎儿标本小脑蚓部在不同孕周的正常发育生长曲线,并探讨胎儿丹迪-沃克综合征(DWS)的MRI表现。方法:对63例胎龄15～40周的胎儿标本行3.0T MRI扫描,从中选出52例符合要求的进行测量,扫描序列为T1WI、T2WI;扫描方位:矢状位扫描为主,辅以冠状位及横轴位;观察及测量内容:测量小脑蚓部的前后径(正中矢状面第四脑室顶点至水平裂处蚓部后缘最大距离)、上蚓高度及下蚓高度(上蚓的最高点及下蚓的最低点分别到前后径线的垂直距离)。对上述测量结果与孕龄间作回归分析。然后对6例经MRI诊断为DWS的病例进行回顾性分析。结果:小脑蚓部前后径、上蚓高度及下蚓高度分别与孕龄呈线性正相关,而上蚓高度总是略大于下蚓高度(P<0.05),多数标本正中矢状面T1WI、T2WI可清晰显示原裂及下后裂。DWS主要表现为小脑蚓部缺如或发育不良,测量数据小于正常胎儿,第四脑室与后颅窝囊肿相通或小脑延髓池增宽。结论:MRI可明确显示正常胎儿小脑蚓部在17孕周以后的正常发育表现及变化规律,而DWS的MRI表现具有特征性,MRI是诊断DWS最理想的影像学方法。     

胎儿Dandy-Walker综合征的MRI研究

目的探讨胎儿Dandy-Walker综合征(Dandy-Walker syndrome,DWS)的MRI表现。方法对23例胎儿DWS病例进行回顾性分析。结果胎儿DWS主要表现为小脑蚓部缺如或发育不良,第四脑室扩大与后颅窝囊肿相通或小脑延髓池增宽。结论 DWS的MRI表现具有特征性,是诊断DWS的最理想的影像学方法。     

Ependymomas of the posterior cranial fossa: CT and MRI findings

We studied nine children with posterior cranial fossa ependymomas to identify specific neuroradiological features. Patients were studied preoperatively with CT and MRI; T1-, T2-and proton-density (PD)-weighted images were obtained. All children underwent surgery and a definite histopathological diagnosis was made. All the tumours grew into the fourth ventricle and caused dilatation of its upper part, which resembled a cap. All but one were separated from the vermis by a cleavage plane. In eight cases there was desmoplastic development through the foramina of the fourth ventricle, and five were heterogeneous due to necrosis and cystic change; one had a haemorrhagic area. In most cases the solid portion was isointense with grey matter on T1-weighted images, hyperintense on PD weighting, and isointense on T2-weighted images. On CT the tumour was isodense in six cases and calcification was detected in four. The presence of both desmoplastic development and a tumour/vermis cleavage plane in a posterior cranial fossa tumour isodense on CT is highly suggestive of ependymoma.     

Rhombencephalosynapsis: cerebellar embryogenesis.

We describe two infants in whom rhombencephalosynapsis was diagnosed with MR imaging in vivo. In contrast to Dandy-Walker malformation, the vermian maldevelopment in this anomaly is characterized by an absence of the anterior vermis and a deficiency of the posterior vermis. The cerebellar hemispheres are fused. In an attempt to identify the pathogenesis of these anatomic manifestations, we question the traditional concept of the embryologic development of the cerebellar primordium.     

Trigeminal neuralgia caused by Dandy-walker malformation: A case report and systematic review of the literature

Trigeminal neuralgia is a pain condition that affects the face along the distribution of the trigeminal nerve and can be recurrent and chronic. Dandy-Walker syndrome is a complex congenital brain anomaly that occurs during embryonic development of the cerebellum and the fourth ventricle. It is characterized by inferior cerebellar vermis hypoplasia and incomplete formation of the fourth ventricle. Dandy-Walker Syndrome is associated with comorbid genetic conditions. It can include congenital heart defects, eye abnormalities, intellectual disability, congenital tumors, and other brain defects such as agenesis of the corpus callosum. However, associations of trigeminal neuralgia and Dandy Walker syndrome have been an infrequent entity. Herein, we report a case of a 23-year-old female patient that presented with complaints of severe left orofacial pain over two years. MRI evaluation was consistent with Dandy-Walker malformation findings that we suspect caused the compression in the trigeminal root entry zone that ultimately gave rise to the patient''s symptoms.