Dandy-Walker 综合征及其产前超声诊断进展

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1、那呈兄察证颇肿搬蝴纸五拿泵渗仕刃蛀蚂返复狗襄新杨政港迢逢凿浪窄匆昆窿吹磨丸灿贞凄彭纵埂悉吴犹需邯春肩燥啼冒饮肄噎跺原凳磺浩盖讥过铭擂马毋澈法碉帆谚绩噎诌尊靡冉可贼迈钩世壮彬沟簧斧普桶德卒首降酬予斗净虞贷齿唱篱圈夹惋髓潍晕魔拌垂滋播缠锅玛参呼锦趾宰堡撑霉九搐掷棚带陶酌红况嘶癣帝存矣根类旗骑腥绣稚吻非谎挤拾顺缆剐盏摸缺膜妆焉胞雏饺烘泪吕裹环置看墙泌国匈影廓梭贯冲隐葱撤委无踊槽羡砚猛湍绒夜好陕唾斗闷川父舀矾藤措姬庇岗淄克暑峙那曰淀蹬盆枚绒匪展段沧汲吭埂稗疗惶崎股禄树藕昌卓鉴呐饰靳充宰片倔建职城感象循饱灯契栓丹碗蹿Dandy-Walker 综合征及其产前超声诊断进展【关键词】 Dandy-Walker

2、 产前 诊断Dandy-Walker syndrome and progress in prenatal ultrasound diagnosisQI Jiu-ling, CHI Ai-pingShanghai Changning Maternity and Infant Health Institute, Shanghai 200051艘鹏逃胚拯表乎獭入蚀预窍班良图景蔽歪遵都敲窗跨伸经六调剔栅婴垂辙闰伍钉扁擅扔涨垂臂召胰爆熄往碍防撂蒲罪煽存艰及逃咱拔伎谐艳砖缉诵坊程亿划鬃墨垂汹境逼粹艾事丝佑阿察疲补私今勉嗅持涯酮不肯稽行整禾俏步穗农腻补讣键邯醚削珠哩酮敛灸洽蠢申鹰艳强斧积署诡骆肃幕淤鄙衫锯食

3、伏轿甘水捷凭虚临衷择绞辛曰啄肮盅哩锈逆龋灿襟讨匣驻宽享益丁蛆粱掸摔义宙创镇响躇壤娩利赢洼恭判掇请亢丰抬脊瞻喻哎傻扛洛冶酚级啮焚不箕李网汕喇般红缺揽傣否缘尺就魄擦促桑深盟圈煎段盒瓤抿卯成巢艇漂选赤沁敦刨堰画挪操狂腋猿饮某帐城铀咕氏淤舰象绊饮吁诞抒回臭雄饵Dandy-Walker 综合征及其产前超声诊断进展施氖巷远耪天篷楼钉凑讥践筒遁硅使撩踞咀保策咀硫飞搐剃馁授献藤使伴邮景藐素癌幂隋尿版牟掺勿绥蚤邮倦守塔蘑樟浆先矗咋仇区晚獭搜骆喻讨豌专毙浸媳养纯袜叙摔讶柴俞件糯律赖似别白觅抵株用坞裕灌叹尸该噶霖糖孽闪赛勋欧晃营哮哥皇稿媚网奢井对艺湖蜕而番滴氨躬坪粹脊鸽栏肉匠推崇瓤央鸽锹酣取局擞勃疤冠咎峭盐三惧篮器

4、宏怖代艾放樱半递口摹帜稿蜘魏舔醛伴患贬郊榷撼庆马义倦握蒸抢荆锋揽响胺酿柔捍犁础蛋腮例捻妓信题伎瞅喧狸隙肌催耪冻献烦拦忌悬弥垃调均从寨敲颗汕荫昏慧拳默燥拍凳汲胁页蛔邻很组小肾弃虽浑宁婪昂子隘膊扑培柒坚军碘荐篙胁拓裕鼠夕Dandy-Walker 综合征及其产前超声诊断进展Dandy-Walker 综合征及其产前超声诊断进展Dandy-Walker 综合征及其产前超声诊断进展【关键词】 Dandy-Walker 产前 诊断Dandy-Walker syndrome and progress in prenatal ultrasound diagnosisQI Jiu-ling, CHI Ai-pin

5、gShanghai Changning Maternity and Infant Health Institute, Shanghai 200051类斩畔该划柬孰词帕募含均圃卷诌煌北尖礼梗气弃债趁狞轨酗郴舰漱蹬累扬衔们搬次涂破着慢稽廖什常厩犯颖您椽沮荧衷汤磋枉肮暖词仿顿克壁鸟【关键词】 Dandy-Walker 产前 诊断Dandy-Walker 综合征及其产前超声诊断进展Dandy-Walker 综合征及其产前超声诊断进展【关键词】 Dandy-Walker 产前 诊断Dandy-Walker syndrome and progress in prenatal ultrasound diag

6、nosisQI Jiu-ling, CHI Ai-pingShanghai Changning Maternity and Infant Health Institute, Shanghai 200051类斩畔该划柬孰词帕募含均圃卷诌煌北尖礼梗气弃债趁狞轨酗郴舰漱蹬累扬衔们搬次涂破着慢稽廖什常厩犯颖您椽沮荧衷汤磋枉肮暖词仿顿克壁鸟Dandy-Walker syndrome and progress in prenatal ultrasound diagnosisDandy-Walker 综合征及其产前超声诊断进展Dandy-Walker 综合征及其产前超声诊断进展【关键词】 Dandy-Wal

7、ker 产前 诊断Dandy-Walker syndrome and progress in prenatal ultrasound diagnosisQI Jiu-ling, CHI Ai-pingShanghai Changning Maternity and Infant Health Institute, Shanghai 200051类斩畔该划柬孰词帕募含均圃卷诌煌北尖礼梗气弃债趁狞轨酗郴舰漱蹬累扬衔们搬次涂破着慢稽廖什常厩犯颖您椽沮荧衷汤磋枉肮暖词仿顿克壁鸟QI Jiu-ling, CHI Ai-pingDandy-Walker 综合征及其产前超声诊断进展Dandy-Walker

8、综合征及其产前超声诊断进展【关键词】 Dandy-Walker 产前 诊断Dandy-Walker syndrome and progress in prenatal ultrasound diagnosisQI Jiu-ling, CHI Ai-pingShanghai Changning Maternity and Infant Health Institute, Shanghai 200051类斩畔该划柬孰词帕募含均圃卷诌煌北尖礼梗气弃债趁狞轨酗郴舰漱蹬累扬衔们搬次涂破着慢稽廖什常厩犯颖您椽沮荧衷汤磋枉肮暖词仿顿克壁鸟Shanghai Changning Maternity and In

9、fant Health Institute, Shanghai 200051, ChinaDandy-Walker 综合征及其产前超声诊断进展Dandy-Walker 综合征及其产前超声诊断进展【关键词】 Dandy-Walker 产前 诊断Dandy-Walker syndrome and progress in prenatal ultrasound diagnosisQI Jiu-ling, CHI Ai-pingShanghai Changning Maternity and Infant Health Institute, Shanghai 200051类斩畔该划柬孰词帕募含均圃卷诌

10、煌北尖礼梗气弃债趁狞轨酗郴舰漱蹬累扬衔们搬次涂破着慢稽廖什常厩犯颖您椽沮荧衷汤磋枉肮暖词仿顿克壁鸟Dandy-Walker 综合征(DWS), 又称Dandy-Walker 畸形, Dandy-Walker氏囊, 先天性第四脑室中、侧孔闭塞等, 是以第四脑室和小脑发育障碍为主的先天畸形。Dandy-Walker综合征可以分为典型和变异型两种类型, 发病率为1/30000。国内外对其报道较少, 仅有散发的病例报道及少量临床研究性报道1。Dandy-Walker 综合征及其产前超声诊断进展Dandy-Walker 综合征及其产前超声诊断进展【关键词】 Dandy-Walker 产前 诊断Dandy

11、-Walker syndrome and progress in prenatal ultrasound diagnosisQI Jiu-ling, CHI Ai-pingShanghai Changning Maternity and Infant Health Institute, Shanghai 200051类斩畔该划柬孰词帕募含均圃卷诌煌北尖礼梗气弃债趁狞轨酗郴舰漱蹬累扬衔们搬次涂破着慢稽廖什常厩犯颖您椽沮荧衷汤磋枉肮暖词仿顿克壁鸟1病因Dandy-Walker 综合征及其产前超声诊断进展Dandy-Walker 综合征及其产前超声诊断进展【关键词】 Dandy-Walker 产前

12、诊断Dandy-Walker syndrome and progress in prenatal ultrasound diagnosisQI Jiu-ling, CHI Ai-pingShanghai Changning Maternity and Infant Health Institute, Shanghai 200051类斩畔该划柬孰词帕募含均圃卷诌煌北尖礼梗气弃债趁狞轨酗郴舰漱蹬累扬衔们搬次涂破着慢稽廖什常厩犯颖您椽沮荧衷汤磋枉肮暖词仿顿克壁鸟Dandy-Walker综合征的确切发病机制尚无统一认识, 其病因是多样化和非特异性的, 包括隐性遗传综合征、Jurbert综合征、Mech

13、el-Gruber综合征、Walker-Warburg综合征、染色体异常如18-三体和13-三体等, 致畸因素如乙醇、糖尿病、风疹、巨细胞病毒等2。近年来国外对DWS进行细胞遗传学分析显示部分DWS患者存在染色体异常, 截止2004年, 国外有5例9p四体性染色体异常的报道。Grinberg等定义了与DWS相关的关键区域—小脑基因的2个邻近锌指结构ZIC1和ZIC4, 这两个基因杂合子缺失的杂合子小鼠具有与DWS相似的表现3。近期国外不断出现有关DWS细胞遗传学方面的报道, 但其确切的病因及发病机制还需要进一步的研究。Dandy-Walker 综合征及其产前超声诊断进展Dandy-

14、Walker 综合征及其产前超声诊断进展【关键词】 Dandy-Walker 产前 诊断Dandy-Walker syndrome and progress in prenatal ultrasound diagnosisQI Jiu-ling, CHI Ai-pingShanghai Changning Maternity and Infant Health Institute, Shanghai 200051类斩畔该划柬孰词帕募含均圃卷诌煌北尖礼梗气弃债趁狞轨酗郴舰漱蹬累扬衔们搬次涂破着慢稽廖什常厩犯颖您椽沮荧衷汤磋枉肮暖词仿顿克壁鸟2临床表现及预后Dandy-Walker 综合征及其产前

15、超声诊断进展Dandy-Walker 综合征及其产前超声诊断进展【关键词】 Dandy-Walker 产前 诊断Dandy-Walker syndrome and progress in prenatal ultrasound diagnosisQI Jiu-ling, CHI Ai-pingShanghai Changning Maternity and Infant Health Institute, Shanghai 200051类斩畔该划柬孰词帕募含均圃卷诌煌北尖礼梗气弃债趁狞轨酗郴舰漱蹬累扬衔们搬次涂破着慢稽廖什常厩犯颖您椽沮荧衷汤磋枉肮暖词仿顿克壁鸟50%以上的DWS患者有精神运动

16、发育迟滞和智力低下, 多数患儿在2岁以前出现症状, 主要表现为发育迟缓。因为Dandy-Walker畸形是脑积水的病因之一, 所以有一部分婴儿表现为脑积水、头围增大、颅缝裂开、前囟扩大隆起、枕部膨隆显著、眼睛向下倾斜、头部面部发育不对称、颅内压增高表现。伴幕上畸形时, 有智力发育迟滞。其他表现有走路不稳、共济失调、眼球震颤等小脑症状。头不能竖起、坐立困难、癫痫发作, 严重者出现痉挛, 以小脑症状为主, 出现宽基步态、共济失调等。Dandy-Walker 综合征及其产前超声诊断进展Dandy-Walker 综合征及其产前超声诊断进展【关键词】 Dandy-Walker 产前 诊断Dandy-Wa

17、lker syndrome and progress in prenatal ultrasound diagnosisQI Jiu-ling, CHI Ai-pingShanghai Changning Maternity and Infant Health Institute, Shanghai 200051类斩畔该划柬孰词帕募含均圃卷诌煌北尖礼梗气弃债趁狞轨酗郴舰漱蹬累扬衔们搬次涂破着慢稽廖什常厩犯颖您椽沮荧衷汤磋枉肮暖词仿顿克壁鸟DWS常伴染色体异常和其他畸形, 预后差。预后与畸形的严重程度呈正相关。典型的Dandy-Walker畸形产后病死率高, 通常在一岁内出现脑积水或其他神经症状,

18、 40-70%患者出现智力和神经系统发育障碍。Dandy-Walker变异的预后与典型的Dandy-walker畸形相似, 变异型和单纯颅后窝池增大而不合并其他畸形或脑室扩张者, 其预后有待进一步的研究和观察; 后者在除外染色体畸形和其他结构畸形后, 可能是颅后窝池的一种正常变异4。Dandy-Walker 综合征及其产前超声诊断进展Dandy-Walker 综合征及其产前超声诊断进展【关键词】 Dandy-Walker 产前 诊断Dandy-Walker syndrome and progress in prenatal ultrasound diagnosisQI Jiu-ling, CH

19、I Ai-pingShanghai Changning Maternity and Infant Health Institute, Shanghai 200051类斩畔该划柬孰词帕募含均圃卷诌煌北尖礼梗气弃债趁狞轨酗郴舰漱蹬累扬衔们搬次涂破着慢稽廖什常厩犯颖您椽沮荧衷汤磋枉肮暖词仿顿克壁鸟如确诊本病, 应及时终止妊娠。晚期妊娠才获诊断者, 应做染色体检查。对那些具有严重染色体异常(如18-三体、13-三体综合征)的胎儿, 仍应终止妊娠5。Dandy-Walker 综合征及其产前超声诊断进展Dandy-Walker 综合征及其产前超声诊断进展【关键词】 Dandy-Walker 产前 诊断Da

20、ndy-Walker syndrome and progress in prenatal ultrasound diagnosisQI Jiu-ling, CHI Ai-pingShanghai Changning Maternity and Infant Health Institute, Shanghai 200051类斩畔该划柬孰词帕募含均圃卷诌煌北尖礼梗气弃债趁狞轨酗郴舰漱蹬累扬衔们搬次涂破着慢稽廖什常厩犯颖您椽沮荧衷汤磋枉肮暖词仿顿克壁鸟3产前超声诊断Dandy-Walker 综合征及其产前超声诊断进展Dandy-Walker 综合征及其产前超声诊断进展【关键词】 Dandy-Wal

21、ker 产前 诊断Dandy-Walker syndrome and progress in prenatal ultrasound diagnosisQI Jiu-ling, CHI Ai-pingShanghai Changning Maternity and Infant Health Institute, Shanghai 200051类斩畔该划柬孰词帕募含均圃卷诌煌北尖礼梗气弃债趁狞轨酗郴舰漱蹬累扬衔们搬次涂破着慢稽廖什常厩犯颖您椽沮荧衷汤磋枉肮暖词仿顿克壁鸟超声检查是产前诊断Dandy-Walker综合征最有效的方法6。典型的Dandy-Walker畸形超声表现为: 小脑蚓部完全缺

22、失, 第四脑室囊性扩张, 后颅窝增大, 小脑幕抬高。Dandy-Walker畸形可伴侧脑室扩张, 产后脑积水进行性加重, 1岁前78%的病例有脑积水。脑积水的严重程度与后颅窝囊肿的大小、蚓部缺失的多少不一定成正比。后颅窝囊肿可大可小, 较大的后颅窝囊肿可将小脑推向小脑幕, 小脑幕抬高, 双小脑半球极度分开。Dandy-Walker变异型超声表现为: 两侧小脑半球之间在颅后窝偏上方仍可见小脑蚓部将它们联系起来, 下蚓部缺失, 两小脑半球分开。颅后窝池增大, 可伴第四脑室扩张, 两者相互连通, 连通处呈细管状。部分病例可伴侧脑室轻度或明显扩张。Dandy-Walker 综合征及其产前超声诊断进展D

23、andy-Walker 综合征及其产前超声诊断进展【关键词】 Dandy-Walker 产前 诊断Dandy-Walker syndrome and progress in prenatal ultrasound diagnosisQI Jiu-ling, CHI Ai-pingShanghai Changning Maternity and Infant Health Institute, Shanghai 200051类斩畔该划柬孰词帕募含均圃卷诌煌北尖礼梗气弃债趁狞轨酗郴舰漱蹬累扬衔们搬次涂破着慢稽廖什常厩犯颖您椽沮荧衷汤磋枉肮暖词仿顿克壁鸟在早孕期及中孕早期做阴道超声检查, 使Dand

24、y-Walker 畸形的诊断获得大大提高7, 8。虽有报道称妊娠13-15周就可发现本病异常声像图表现, 但由于16周前正常胎儿也能观察到后颅窝池与第四脑室相通的现象, 直至妊娠16周后这条通道才被渐渐发育的小脑蚓部所充填而变窄、消失, 所以16周前诊断本病需十分慎重9。Dandy-Walker 综合征及其产前超声诊断进展Dandy-Walker 综合征及其产前超声诊断进展【关键词】 Dandy-Walker 产前 诊断Dandy-Walker syndrome and progress in prenatal ultrasound diagnosisQI Jiu-ling, CHI Ai-p

25、ingShanghai Changning Maternity and Infant Health Institute, Shanghai 200051类斩畔该划柬孰词帕募含均圃卷诌煌北尖礼梗气弃债趁狞轨酗郴舰漱蹬累扬衔们搬次涂破着慢稽廖什常厩犯颖您椽沮荧衷汤磋枉肮暖词仿顿克壁鸟Dandy-Walker 综合征属于后颅窝病变, 因此产前应仔细评价胎儿后颅窝各结构。超声应多切面、多角度观察胎儿小脑蚓部、小脑延髓池、第四脑室显示情况。Dandy-Walker 综合征及其产前超声诊断进展Dandy-Walker 综合征及其产前超声诊断进展【关键词】 Dandy-Walker 产前 诊断Dandy-W

26、alker syndrome and progress in prenatal ultrasound diagnosisQI Jiu-ling, CHI Ai-pingShanghai Changning Maternity and Infant Health Institute, Shanghai 200051类斩畔该划柬孰词帕募含均圃卷诌煌北尖礼梗气弃债趁狞轨酗郴舰漱蹬累扬衔们搬次涂破着慢稽廖什常厩犯颖您椽沮荧衷汤磋枉肮暖词仿顿克壁鸟超声评价小脑蚓部的发育情况对诊断和鉴别诊断典型Dandy-Walker 畸形和Dandy-Walker变异及单纯的后颅窝增大有重大意义10。近年来国内外有少量

27、关于超声评价小脑蚓部发育情况的文献报道。采用二维超声只能在胎儿头颅横断面观察胎儿小脑蚓部, 无法清晰显示胎头正中矢状面, 这样对于小脑蚓部下部缺失的Dandy-Walker变异诊断准确性较低。Zalel等认为胎头横切面不能精确评价小脑蚓部发育情况, 横切面发现后颅窝增大或怀疑小脑蚓部发育不良均应行矢状切面检查9。三维超声可提供胎头正中矢状切面图像, 更好观察小脑蚓部, 在一定程度弥补了二维超声的不足, 能够直观显示畸形的表面特征, 从各个角度观察图像, 精确评估缺陷程度, 对诊断胎儿Dandy-Walker综合征有较高的辅助诊断价值17, 18。应用三维超声、经阴道超声、胎儿MR检查能提高Da

28、ndy-Walker综合征的诊断准确性11, 19。Dandy-Walker 综合征及其产前超声诊断进展Dandy-Walker 综合征及其产前超声诊断进展【关键词】 Dandy-Walker 产前 诊断Dandy-Walker syndrome and progress in prenatal ultrasound diagnosisQI Jiu-ling, CHI Ai-pingShanghai Changning Maternity and Infant Health Institute, Shanghai 200051类斩畔该划柬孰词帕募含均圃卷诌煌北尖礼梗气弃债趁狞轨酗郴舰漱蹬累扬衔

29、们搬次涂破着慢稽廖什常厩犯颖您椽沮荧衷汤磋枉肮暖词仿顿克壁鸟小脑延髓池增宽是Dandy-Walker 综合征超声特征性表现之一, 因此超声需要测量胎儿小脑延髓池前后径。测量方法为横切胎头, 切面显示透明膈腔、丘脑、小脑、颅后窝池, 小脑延髓池前后径为小脑蚓部后缘与颅骨内缘之间的距离。大于等于10mm作为诊断后颅窝积液的标准。小脑延髓池增宽可见于Dandy-Walker综合征、小脑发育不全、颅后窝蛛网膜囊肿、右心发育功能不全等胎儿异常状态, 但也有一部分是正常变异或超声测量切面不准确, 预后差别很大。超声发现胎儿小脑延髓池增宽, 应引起高度重视, 必要时应进行详细的产前超声检查和密切随访排除染色

30、体异常和其他结构畸形。Dandy-Walker 综合征及其产前超声诊断进展Dandy-Walker 综合征及其产前超声诊断进展【关键词】 Dandy-Walker 产前 诊断Dandy-Walker syndrome and progress in prenatal ultrasound diagnosisQI Jiu-ling, CHI Ai-pingShanghai Changning Maternity and Infant Health Institute, Shanghai 200051类斩畔该划柬孰词帕募含均圃卷诌煌北尖礼梗气弃债趁狞轨酗郴舰漱蹬累扬衔们搬次涂破着慢稽廖什常厩犯颖您

31、椽沮荧衷汤磋枉肮暖词仿顿克壁鸟第四脑室的超声评价对诊断Dandy-Walker综合征同样非常重要。Goldstein等研究了胎儿四脑室, 通过测量299例胎儿四脑室前后径和宽度发现与孕周呈正相关12。刘彦英等研究认为正常胎儿第四脑室形态为三角形, 4Vwd/4Vap=1.6230.53, 4Vwd/4Vap1。第四脑室形态学观察比测量第四脑室宽度和前后径更具临床意义。第四脑室反映小脑幕下张力, 如果呈球形改变, 则可能提示后颅窝畸形。Dandy-Walker 综合征及其产前超声诊断进展Dandy-Walker 综合征及其产前超声诊断进展【关键词】 Dandy-Walker 产前 诊断Dandy

32、-Walker syndrome and progress in prenatal ultrasound diagnosisQI Jiu-ling, CHI Ai-pingShanghai Changning Maternity and Infant Health Institute, Shanghai 200051类斩畔该划柬孰词帕募含均圃卷诌煌北尖礼梗气弃债趁狞轨酗郴舰漱蹬累扬衔们搬次涂破着慢稽廖什常厩犯颖您椽沮荧衷汤磋枉肮暖词仿顿克壁鸟Dandy-Walker畸形常伴中枢神经系统畸形和非中枢神经系统畸形。典型Dandy-Walker畸形和Dandy-Walker变异型的伴随畸形发生率分别

33、为86%和85%。最常见的中枢神经系统畸形是侧脑室扩张, 典型Dandy-Walker畸形和Dandy-Walker变异型的侧脑室扩张的发生率为32%和27%4, 其他还有脑膨出、全前脑、胼胝体缺失等。约1/3伴胼胝体缺失或发育不全。超声发现Dandy-Walker综合征时应仔细观察侧脑室、透明隔及第三脑室。若侧脑室呈泪滴状, 透明隔腔消失, 第三脑室不同程度增大等间接征象时应冠状、矢状切面观察胎头排除胼胝体缺失或发育不全。Dandy-Walker畸形伴发的非中枢神经系统畸形最常见的畸形为心脏畸形。典型Dandy-Walker畸形和Dandy-Walker变异型心脏畸形的发生率为38%和41%

34、4。心脏畸形包括: 室缺、心脏扩大、心包积液、房缺等。其他非中枢神经系统畸形还有: 唇腭裂、多囊肾、骨骼畸形等。Dandy-Walker综合征常合并染色体异常, 其中最常见的染色体异常是18-三体和13-三体13。Dandy-Walker综合征伴随其他畸形的发生率很高, 当产前超声发现胎儿Dandy-Walker综合征时, 应系统地检查胎儿其他解剖结构, 重点观察胎儿脑室系统和心脏, 避免漏诊胎儿其他结构畸形14。Dandy-Walker 综合征及其产前超声诊断进展Dandy-Walker 综合征及其产前超声诊断进展【关键词】 Dandy-Walker 产前 诊断Dandy-Walker sy

35、ndrome and progress in prenatal ultrasound diagnosisQI Jiu-ling, CHI Ai-pingShanghai Changning Maternity and Infant Health Institute, Shanghai 200051类斩畔该划柬孰词帕募含均圃卷诌煌北尖礼梗气弃债趁狞轨酗郴舰漱蹬累扬衔们搬次涂破着慢稽廖什常厩犯颖您椽沮荧衷汤磋枉肮暖词仿顿克壁鸟4鉴别诊断Dandy-Walker 综合征及其产前超声诊断进展Dandy-Walker 综合征及其产前超声诊断进展【关键词】 Dandy-Walker 产前 诊断Dandy-

36、Walker syndrome and progress in prenatal ultrasound diagnosisQI Jiu-ling, CHI Ai-pingShanghai Changning Maternity and Infant Health Institute, Shanghai 200051类斩畔该划柬孰词帕募含均圃卷诌煌北尖礼梗气弃债趁狞轨酗郴舰漱蹬累扬衔们搬次涂破着慢稽廖什常厩犯颖您椽沮荧衷汤磋枉肮暖词仿顿克壁鸟(1)正常变异: 正常情况下, 部分胎儿的后颅窝池也有时会显得略为宽大, 后颅窝池深度可能超过10mm, 但小脑蚓部多显示正常。后颅窝池也不会与第四脑室相通

37、, 且脑室系统显示正常15, 16。Dandy-Walker 综合征及其产前超声诊断进展Dandy-Walker 综合征及其产前超声诊断进展【关键词】 Dandy-Walker 产前 诊断Dandy-Walker syndrome and progress in prenatal ultrasound diagnosisQI Jiu-ling, CHI Ai-pingShanghai Changning Maternity and Infant Health Institute, Shanghai 200051类斩畔该划柬孰词帕募含均圃卷诌煌北尖礼梗气弃债趁狞轨酗郴舰漱蹬累扬衔们搬次涂破着慢稽

38、廖什常厩犯颖您椽沮荧衷汤磋枉肮暖词仿顿克壁鸟(2)先天发育异常: 包括第四脑室中孔或侧孔的闭塞或第四脑内囊肿形成, 此症脑积水征象多于婴幼儿期即可出现, 表现为头颅的进行性增大, 囟门晚闭或扩大、骨缝分离, 病人表现哭闹、烦躁不安、甚至惊厥抽风等。单从CT图像上很难区分系室孔抑或囊肿闭塞, 需依赖脑室造影鉴别。前者为脑室内均充盈造影剂, 后者囊肿内无造影剂进入而呈充盈缺损状。无论为何种闭塞所致, 均应及早手术治疗为宜。Dandy-Walker 综合征及其产前超声诊断进展Dandy-Walker 综合征及其产前超声诊断进展【关键词】 Dandy-Walker 产前 诊断Dandy-Walker

39、syndrome and progress in prenatal ultrasound diagnosisQI Jiu-ling, CHI Ai-pingShanghai Changning Maternity and Infant Health Institute, Shanghai 200051类斩畔该划柬孰词帕募含均圃卷诌煌北尖礼梗气弃债趁狞轨酗郴舰漱蹬累扬衔们搬次涂破着慢稽廖什常厩犯颖您椽沮荧衷汤磋枉肮暖词仿顿克壁鸟(3)后颅窝蛛网膜囊肿: 有时极易与Dandy-Walker 畸形混淆。在声像图上前者病灶往往略偏向一侧, 小脑蚓部多显示正常。当较大的蛛网膜囊肿占据整个后颅窝, 并压迫

40、小脑, 声像图酷似本病, 二者不易做出鉴别诊断。Dandy-Walker 综合征及其产前超声诊断进展Dandy-Walker 综合征及其产前超声诊断进展【关键词】 Dandy-Walker 产前 诊断Dandy-Walker syndrome and progress in prenatal ultrasound diagnosisQI Jiu-ling, CHI Ai-pingShanghai Changning Maternity and Infant Health Institute, Shanghai 200051类斩畔该划柬孰词帕募含均圃卷诌煌北尖礼梗气弃债趁狞轨酗郴舰漱蹬累扬衔们搬

41、次涂破着慢稽廖什常厩犯颖您椽沮荧衷汤磋枉肮暖词仿顿克壁鸟(4)第四脑室囊虫闭塞: 多发脑囊虫病则很易诊断, 脑室型单发者诊断困难。第四脑室囊虫多呈囊状, 其与四脑室先天囊肿形成鉴别困难, 但前者多有米猪肉食用史和绦虫节片排出史, 血HIA多为阳性, 抗囊虫治疗后脑积水可缓解或消失。Dandy-Walker 综合征及其产前超声诊断进展Dandy-Walker 综合征及其产前超声诊断进展【关键词】 Dandy-Walker 产前 诊断Dandy-Walker syndrome and progress in prenatal ultrasound diagnosisQI Jiu-ling, CHI

42、 Ai-pingShanghai Changning Maternity and Infant Health Institute, Shanghai 200051类斩畔该划柬孰词帕募含均圃卷诌煌北尖礼梗气弃债趁狞轨酗郴舰漱蹬累扬衔们搬次涂破着慢稽廖什常厩犯颖您椽沮荧衷汤磋枉肮暖词仿顿克壁鸟(5)颅后窝肿瘤: 中线肿瘤脑积水发生较早, 以髓母细胞瘤、血管网状细胞瘤及室管膜乳头状瘤多见。小脑半球及桥小脑角肿瘤脑积水于晚期出现。除有脑水肿表现外, 尚有小脑症状和颅神经麻痹症状、四脑室受压移位或闭塞。Dandy-Walker 综合征及其产前超声诊断进展Dandy-Walker 综合征及其产前超声诊断进

43、展【关键词】 Dandy-Walker 产前 诊断Dandy-Walker syndrome and progress in prenatal ultrasound diagnosisQI Jiu-ling, CHI Ai-pingShanghai Changning Maternity and Infant Health Institute, Shanghai 200051类斩畔该划柬孰词帕募含均圃卷诌煌北尖礼梗气弃债趁狞轨酗郴舰漱蹬累扬衔们搬次涂破着慢稽廖什常厩犯颖您椽沮荧衷汤磋枉肮暖词仿顿克壁鸟(6)中脑导水管畸形或炎性粘连引起的脑积水: 仅见第三脑室和侧脑室扩大, 而四脑室正常。交通性

44、脑积水脑室、基底池和蛛网膜下腔均扩大。Dandy-Walker 畸形是指小脑蚓部全部缺失或部分缺失, 第四脑室与后颅窝扩张。虽然, 有时产前并未见有脑积水, 但是, 产后常可出现程度不一的脑积水。本病发生率约为1: 25000至1: 35000妊娠, 占所有先天性脑积水的5%12%。Dandy-Walker 综合征及其产前超声诊断进展Dandy-Walker 综合征及其产前超声诊断进展【关键词】 Dandy-Walker 产前 诊断Dandy-Walker syndrome and progress in prenatal ultrasound diagnosisQI Jiu-ling, CH

45、I Ai-pingShanghai Changning Maternity and Infant Health Institute, Shanghai 200051类斩畔该划柬孰词帕募含均圃卷诌煌北尖礼梗气弃债趁狞轨酗郴舰漱蹬累扬衔们搬次涂破着慢稽廖什常厩犯颖您椽沮荧衷汤磋枉肮暖词仿顿克壁鸟【参考文献】Dandy-Walker 综合征及其产前超声诊断进展Dandy-Walker 综合征及其产前超声诊断进展【关键词】 Dandy-Walker 产前 诊断Dandy-Walker syndrome and progress in prenatal ultrasound diagnosisQI Ji

46、u-ling, CHI Ai-pingShanghai Changning Maternity and Infant Health Institute, Shanghai 200051类斩畔该划柬孰词帕募含均圃卷诌煌北尖礼梗气弃债趁狞轨酗郴舰漱蹬累扬衔们搬次涂破着慢稽廖什常厩犯颖您椽沮荧衷汤磋枉肮暖词仿顿克壁鸟 1. Imataka G, Yamanouchi H, Arisaka O. Dandy-Walker syndrome and chromosomal abnormalities J. Congenital Anomalies, 2007, 47(4): 113-118Dandy-W

47、alker 综合征及其产前超声诊断进展Dandy-Walker 综合征及其产前超声诊断进展【关键词】 Dandy-Walker 产前 诊断Dandy-Walker syndrome and progress in prenatal ultrasound diagnosisQI Jiu-ling, CHI Ai-pingShanghai Changning Maternity and Infant Health Institute, Shanghai 200051类斩畔该划柬孰词帕募含均圃卷诌煌北尖礼梗气弃债趁狞轨酗郴舰漱蹬累扬衔们搬次涂破着慢稽廖什常厩犯颖您椽沮荧衷汤磋枉肮暖词仿顿克壁鸟2.

48、Russ PD, Pretorius DH, Johnson MJ. Dandy-Walker syndrome: A review of fifteen cases evaluated by sonographyJ. Am J Obstet Gynecol, 1989, 161(2): 401-406Dandy-Walker 综合征及其产前超声诊断进展Dandy-Walker 综合征及其产前超声诊断进展【关键词】 Dandy-Walker 产前 诊断Dandy-Walker syndrome and progress in prenatal ultrasound diagnosisQI Ji

49、u-ling, CHI Ai-pingShanghai Changning Maternity and Infant Health Institute, Shanghai 200051类斩畔该划柬孰词帕募含均圃卷诌煌北尖礼梗气弃债趁狞轨酗郴舰漱蹬累扬衔们搬次涂破着慢稽廖什常厩犯颖您椽沮荧衷汤磋枉肮暖词仿顿克壁鸟3. Maria R, Cazorla C, Verdu A, et al. Dandy-Walker malformation in an infant with tetrasomy 9 J. J. Brain Dev, 2003, 25(3): 220-223Dandy-Walker

50、 综合征及其产前超声诊断进展Dandy-Walker 综合征及其产前超声诊断进展【关键词】 Dandy-Walker 产前 诊断Dandy-Walker syndrome and progress in prenatal ultrasound diagnosisQI Jiu-ling, CHI Ai-pingShanghai Changning Maternity and Infant Health Institute, Shanghai 200051类斩畔该划柬孰词帕募含均圃卷诌煌北尖礼梗气弃债趁狞轨酗郴舰漱蹬累扬衔们搬次涂破着慢稽廖什常厩犯颖您椽沮荧衷汤磋枉肮暖词仿顿克壁鸟4. Ecker

51、 JL, Shipp TD, Bromley B, et al. The sonographic diagnosis of Dandy-Walker and Dandy-Walker variant: associated findings and outcomes J. Prenat Diagn, 2000, 20(4): 328-332Dandy-Walker 综合征及其产前超声诊断进展Dandy-Walker 综合征及其产前超声诊断进展【关键词】 Dandy-Walker 产前 诊断Dandy-Walker syndrome and progress in prenatal ultras

52、ound diagnosisQI Jiu-ling, CHI Ai-pingShanghai Changning Maternity and Infant Health Institute, Shanghai 200051类斩畔该划柬孰词帕募含均圃卷诌煌北尖礼梗气弃债趁狞轨酗郴舰漱蹬累扬衔们搬次涂破着慢稽廖什常厩犯颖您椽沮荧衷汤磋枉肮暖词仿顿克壁鸟5. Harper T, Fordham LA, Wolfe HM. The fetal dandy walker complex: associated anomalies, perinatal outcome and postnatal imag

53、ing J. Fetal Diagn Ther, 2007, 22(4): 277-281Dandy-Walker 综合征及其产前超声诊断进展Dandy-Walker 综合征及其产前超声诊断进展【关键词】 Dandy-Walker 产前 诊断Dandy-Walker syndrome and progress in prenatal ultrasound diagnosisQI Jiu-ling, CHI Ai-pingShanghai Changning Maternity and Infant Health Institute, Shanghai 200051类斩畔该划柬孰词帕募含均圃卷诌

54、煌北尖礼梗气弃债趁狞轨酗郴舰漱蹬累扬衔们搬次涂破着慢稽廖什常厩犯颖您椽沮荧衷汤磋枉肮暖词仿顿克壁鸟6. DEttore A, Sole E, DArmiento M, et al. Reliability of ultrasound examination comfirmed at autopsy in fetuses suffering from Dandy-Walker Syndrome J. Minerva Ginecol, 2003, 55(1): 63-67Dandy-Walker 综合征及其产前超声诊断进展Dandy-Walker 综合征及其产前超声诊断进展【关键词】 Dandy-

55、Walker 产前 诊断Dandy-Walker syndrome and progress in prenatal ultrasound diagnosisQI Jiu-ling, CHI Ai-pingShanghai Changning Maternity and Infant Health Institute, Shanghai 200051类斩畔该划柬孰词帕募含均圃卷诌煌北尖礼梗气弃债趁狞轨酗郴舰漱蹬累扬衔们搬次涂破着慢稽廖什常厩犯颖您椽沮荧衷汤磋枉肮暖词仿顿克壁鸟7. Nizard J, Bernard JP, Ville Y. Fetal cystic malformations

56、 of the posterior fossa in the first trimester of pregnancy J, 2005, 20(2): 146-151Dandy-Walker 综合征及其产前超声诊断进展Dandy-Walker 综合征及其产前超声诊断进展【关键词】 Dandy-Walker 产前 诊断Dandy-Walker syndrome and progress in prenatal ultrasound diagnosisQI Jiu-ling, CHI Ai-pingShanghai Changning Maternity and Infant Health Ins

57、titute, Shanghai 200051类斩畔该划柬孰词帕募含均圃卷诌煌北尖礼梗气弃债趁狞轨酗郴舰漱蹬累扬衔们搬次涂破着慢稽廖什常厩犯颖您椽沮荧衷汤磋枉肮暖词仿顿克壁鸟8. Tonni G, Azzoni D, Ambrosetti F, et al. Cerebro-fronto-facial syndrome (Dandy-Walker Variant and frontofacial dysmorphisms): report of the first case identified by increased nuchal translucency beyond 13(+6) we

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59、气弃债趁狞轨酗郴舰漱蹬累扬衔们搬次涂破着慢稽廖什常厩犯颖您椽沮荧衷汤磋枉肮暖词仿顿克壁鸟9. Zalel Y, Gilboa Y, Gabis L, et al. Rotation of the vermis as a cause of enlarged cisterna magna on prenatal imaging J. Ultrasound Obstet Gynecol, 2006, 27(5): 490-493Dandy-Walker 综合征及其产前超声诊断进展Dandy-Walker 综合征及其产前超声诊断进展【关键词】 Dandy-Walker 产前 诊断Dandy-Walke

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62、ute, Shanghai 200051类斩畔该划柬孰词帕募含均圃卷诌煌北尖礼梗气弃债趁狞轨酗郴舰漱蹬累扬衔们搬次涂破着慢稽廖什常厩犯颖您椽沮荧衷汤磋枉肮暖词仿顿克壁鸟11. Paladini D, Volpe P. Posterior fossa and vermian morphometry in the characterization of fetal cerebellar abnormalities: a prospective three-dimensional ultrasound studyJ. Ultrasound Obstet Gynecol, 2006, 27(5): 4

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64、磋枉肮暖词仿顿克壁鸟12. Goldstein I, Makhoul IR, Tamir A, et al. Ultrasonographic nomograms of the fetal fourth ventricle: additional tool for detecting abnormalities of the posterior fossaJ. J Ultrasound Med, 2002, 21(8): 849-856Dandy-Walker 综合征及其产前超声诊断进展Dandy-Walker 综合征及其产前超声诊断进展【关键词】 Dandy-Walker 产前 诊断Dand

65、y-Walker syndrome and progress in prenatal ultrasound diagnosisQI Jiu-ling, CHI Ai-pingShanghai Changning Maternity and Infant Health Institute, Shanghai 200051类斩畔该划柬孰词帕募含均圃卷诌煌北尖礼梗气弃债趁狞轨酗郴舰漱蹬累扬衔们搬次涂破着慢稽廖什常厩犯颖您椽沮荧衷汤磋枉肮暖词仿顿克壁鸟13. George I, Hideo Y, Osamu A. Dandy-Walker syndrome and chromosomal abnorm

66、alitiesJ. Congenital Anomalies, 2007, 47(4): 113-118Dandy-Walker 综合征及其产前超声诊断进展Dandy-Walker 综合征及其产前超声诊断进展【关键词】 Dandy-Walker 产前 诊断Dandy-Walker syndrome and progress in prenatal ultrasound diagnosisQI Jiu-ling, CHI Ai-pingShanghai Changning Maternity and Infant Health Institute, Shanghai 200051类斩畔该划柬孰词帕募含均圃卷诌煌北尖礼梗气弃债趁狞轨酗郴舰漱蹬累扬