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泌尿男性生殖系统先天性畸形课件

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泌尿男性生殖系统先天性畸形PPT 泌尿男性生殖系统先天性畸形 男性生殖系统 先天性畸形 男性生殖系统先天性畸形 男生殖系统先天性畸形 生殖系统先天 男性生殖器畸形 先天性泌尿系畸形
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With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 泌尿、男性生殖系统 先天性畸形 Urologic and Genital Deformation 合肥市第二人民医院 泌尿外科 席俊华(主治医师,临床医学博士) With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 第1节 概 述 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society n泌尿、男性生殖系统畸形是人体最常见 的畸形 n人类肾在胚胎发育时,经过原肾( pronephros)、中肾(mesonephros)和 后肾(metanephros)三个连续过程 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 泌尿系的发生 前 肾 中肾小管 中肾管 生肾组织 输 尿 管 芽 泄殖腔 第5周人胚前、中、后肾的发生 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 泌尿系的发生 后肾(永久肾) n输尿管芽→输尿管、肾盂、肾盏、集合 管 n生肾组织→肾小管和肾小体,肾被膜 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 泌尿系的发生 后肾的发生 中肾小 管 输尿管芽 生肾 组织 肾盂 肾大盏 肾小盏 集合管 → →→→ With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 泌尿系的发生 泄殖腔(cloaca)的腹侧→尿生殖窦 n上段(较大)→ 膀胱(其顶点与脐尿管相连 ;后者闭锁,形成脐中韧带) n中段(狭窄) 于男性→尿道前列腺部和膜部 于女性→尿道 n下段 于男性→尿道海绵体部 于女性→阴道前庭 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 泌尿系的发生 泄殖腔的分隔与尿生殖窦的形成 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 第2节 肾和输尿管的先天性畸形 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society n肾缺如(renal agenesis)和肾发育不良 n异位肾(ectopic kidney) n蹄铁型肾(horseshoe kidney) n囊性肾病变(cystic kidney disease) n重复肾盂和输尿管(double pelvis and ureters ) n肾盂输尿管交界处梗阻 n腔静脉后输尿管(retrocaval ureter) n输尿管膨出(ureterocele) With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 一、囊性肾病变 囊性肾病变是人体最多见的囊性疾病,主 要分为遗传性和非遗传性两大类。 n遗传性:多囊肾 n非遗传性:单纯性肾囊肿 n其他:髓质海绵肾、多房性肾囊性变等 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 多囊肾(polycystic kidney) 多囊肾有家族性,分婴儿型和成人型 n婴儿型,常染色体隐形遗传(RPK),定位6号 染色体,常伴有肝、脾或胰腺囊肿,多早期夭 折 n成人型,常染色体显性遗传(DPK),定位于16 和4号染色体,早期无任何症状,大多在40岁 左右才出现症状 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 多囊肾 正常肾多囊肾 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 多囊肾 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 多囊肾 囊肿 进行性增大压迫肾实质 ①疼痛②血尿③感染④结石⑤腹块⑥ 高血压⑦肾功能损害 最终导致肾功能衰竭或死于多囊肾的 并发症 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 多囊肾 治疗方法 n保守对症治疗:早期肾功能正常患者 n囊肿去顶减压术:主要是针对早、中期有较大 单个囊肿者,可分为开放手术和腹腔镜手术 n血液透析或肾移植:主要是针对晚期肾功能衰 竭患者 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 单纯性肾囊肿 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 髓质海绵肾 肾乳头囊状扩张,内见单发或多发的钙化点,表现为环绕于肾 盏、肾盂周围的多数囊腔,形似菜花 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society n海绵肾是先天性的,可能是有遗传倾向的 良性肾髓质囊性病变, n常于40岁以后发现,易误诊断肾结石和尿 路感染。 n其病理特征是肾小管远端集合小管扩张, 形成小囊和囊性空腔,并位于肾盏连接处 ,易导致结石、感染等。 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 二、马蹄形肾 马蹄形肾,是融合肾畸形中最常见的一种 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society n在胚胎发育4~6周,后肾组织相互靠近, 此时许多影响因素均可导致其下极相融合 。 n脐动脉或髂动脉的轻微变化可引起正在移 行的肾脏方向改变,从而发生两肾的融合 。 n不管其形成机制如何肾脏的融合总发生在 旋转之前,因此,肾脏和输尿管常朝向前 。 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 马蹄肾大都发育较差,伴旋转不良,肾盂位于肾的前方 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society n无症状及并发症者一般不必治疗。 n有尿路梗阻伴严重腰肋疼痛等症状,影响 工作和生活者,可考虑做输尿管松解,峡 部切断分离两肾及肾盂输尿管成形固定术 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 三、重复肾盂和输尿管 左侧肾盂和输尿管重复畸形 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society n输尿管芽发育成肾盂,分支形成肾盏、再 分支形成小盏、集合管。如分支过早,则 形成重复的输尿管畸形 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 治 疗 n无症状者,不需治疗 n若上半肾感染、肾盂积水、结石形成以及输 尿管异位开口引起尿失禁者,作上半病肾及 输尿管切除术 n若重复肾功能尚好,且无合并症,可采用异 位开口的重复输尿管膀胱移植术 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 四、肾盂输尿管交界处梗阻 肾盂输尿管交界处梗阻(UPJO)是小儿及青 少年肾积水的常见病因,其发生原因有: n肾盂、输尿管交界处壁层肌肉结构的改变,影 响输尿管的蠕动 n异位血管或肾下极血管的压迫 n肾、输尿管的纤维条索引起的压迫、扭曲 n高位输尿管开口并有肾盂输尿管粘连及成角 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 肾盂输尿管交界处梗阻 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 肾盂输尿管交界处梗阻 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 肾盂输尿管交界处梗阻 手术治疗原则: n梗阻较轻,肾盂肾盏扩张不严重者可行 单纯成形手术 n扩张明显者,应切除病变段及扩张的肾 盂,再做吻合术 n更严重者应做肾造瘘或肾切除 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 五、肾缺如和肾发育不良 孤立肾(单侧肾缺如) With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society n孤立肾是由于一侧肾脏先天性缺如。 n约1000至1500例出生儿中有1例独肾。男 性多于女性,左侧多于右侧。 n没有肾脏的一侧也多没有输尿管。由于生 理需要,对侧肾脏常代偿性增大。如独肾 形态和功能正常,临床上不用处理。 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 六、异位肾六、异位肾 在胚胎发育中,原先在骨盆内的肾,未能到腰部,形成异位肾 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society n肾的形成需要胚胎前肾阶段位于体腔背外 侧的肾节以及来自Wolffian管的输尿管芽 的相互依赖和完全发育。 n缺少这两个条件将会发生孤立肾或肾发育 不全 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 七、输尿管膨出 膀胱镜下可见一侧或两侧输尿管口部位有圆形或卵圆形的囊状肿物 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society n输尿管囊肿(ureterocele)是由于输尿管 口先天性狭窄或功能性挛缩及输尿管壁发 育不全,以致输尿管下端各层形成一囊肿 突入膀胱之内 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 八、腔静脉后输尿管 腔静脉后输尿管较 为少见,主要是因为 下腔静脉发育异常, 输尿管于下腔静脉的 后侧面环绕之后走向 中线,再从内向外沿 正常路径行至膀胱 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 腔静脉后输尿管 静脉尿路造影或逆行尿路造影可见输尿管呈典型 的S形或镰刀形弯曲,肾盂及输尿管上1/3有积水 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 腔静脉后输尿管 下腔静脉后输尿管切断并输尿管恢复正常位置后再吻合术 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 第3节 膀胱和尿道的先天性畸形 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society n膀胱外翻 n尿道上裂(epispadia) n尿道下裂(hypospadia) With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 一、膀胱外翻 在脐下方的腹壁中可见一块粉红色的黏膜,此为膀胱后壁向外翻出的内面 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 膀胱外翻 膀胱外翻几乎均合并尿道上裂和耻骨联合分离,或伴有髋关节脱位 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 二、尿道上裂 尿道开口至耻骨联合在阴茎背侧呈一沟槽,包皮在背侧裂开, 阴茎头呈扁平状,阴茎体较小。一般施行尿道上裂整形手术。 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 三、尿道下裂 由于生殖结节腹侧纵行的尿生殖沟自后向前 闭合过程停止所致。 它的畸形有四个特征: n尿道开口异常; n阴茎向腹侧屈曲畸形; n阴茎背侧包皮正常而阴茎腹侧包皮缺乏; n尿道海绵体发育不全。 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 尿道下裂 根据尿道口所在的不 同部位,将尿道下裂分 为四种类型: 1阴茎头型 2阴茎型 3阴囊型 4会阴型 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 尿道下裂 尿道下裂主要采取手术治疗,手术目的是矫正阴茎 弯曲,使尿道口恢复或接近在阴茎头的正常位置, 小儿能站立排尿,成年后有正常的性生活和生育能力 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 第四节、 男性生殖器官先天性畸形 隐睾(cryptorchidism) With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society n睾丸下降异常,使睾丸不能降至阴囊而停 留在腹膜后、腹股沟管或阴囊入口处。 n精子发生障碍,引起不育; n易发生恶变,尤其是位于腹膜后者; n一岁内的睾丸有自行下降可能; With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 包茎和包皮过长 Phimosis and Redundant Prepuce With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 包茎(phimosis)是指包皮 口狭窄或包皮与阴茎头粘 连,使包皮不能上翻外露 阴茎头 包皮过长(redundant prepuce)是包皮覆盖于全 部阴茎头和尿道口,但仍 可上翻 With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society With the improving quality of life as well as the ever-accelerating pace of life, the quality of people's food needs and rate also changing.In order to meet the needs of society 包茎和包皮过长 嵌顿性包茎的手法复位 Thank you
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