钡灌肠在先天性巨结肠诊断中的重要作用课件

Asymptomatic ulcerative colitis patients on an ProDepression A Risk Factor for Infant Mental HealthMammography Benchmarks Angoff Process
病理生理
神经节细胞缺如； 胆碱能神经系统异常； 肾上腺素能神经及非肾上腺素能非胆碱能神经
对部分新生儿HD，因结肠被动性扩张尚不明显，与狭 窄段对比差异不大，可能会对诊断有影响。
24小时后复查，钡剂残留的情况可反映结肠的蠕动功 能，HD的患儿钡剂排空的功能很差，这点对诊断也有 重要意义。
但检查前对患儿进行过洗肠会影响其检查结果。
Asymptomatic ulcerative colitis patients on an ProDepression A Risk Factor for Infant Mental HealthMammography Benchmarks Angoff Process
Asymptomatic ulcerative colitis patients on an ProDepression A Risk Factor for Infant Mental HealthMammography Benchmarks Angoff Process
Asymptomatic ulcerative colitis patients on an ProDepression A Risk Factor for Infant Mental HealthMammography Benchmarks Angoff Process
病因
一般认为是由于在胚胎第12周前神经节细胞 发育停顿所致，停顿越早，无神经节细胞段 越长。尾端的直肠、乙状结肠最后才有神经 母细胞生长，因此是最常见的病变部位。
近年来人们主要从基因突变、早期胚胎阶段 微环境改变及遗传因素等方面对HD的发病 原因进行了相关研究。
Asymptomatic ulcerative colitis patients on an ProDepression A Risk Factor for Infant Mental HealthMammography Benchmarks Angoff Process
形态学改变 典型的分为
痉挛段 移行段 扩张段
Asymptomatic ulcerative colitis patients on an ProDepression A Risk Factor for Infant Mental HealthMammography Benchmarks Angoff Process
异常； Cajal间质细胞异常；
Asymptomatic ulcerative colitis patients on an ProDepression A Risk Factor for Infant Mental HealthMammography Benchmarks Angoff Process
Asymptomatic ulcerative colitis patients on an ProDepression A Risk Factor for Infant Mental HealthMammography Benchmarks Angoff Process
钡灌肠在先天性巨结肠诊断中的重要作用
临床表现
体征：
腹部膨隆，腹壁变薄，可见肠型； 肛门指检，内括约肌紧张，壶腹部空虚，退指时常有爆
破性排气排便，为巨结肠的典型表现；
Asymptomatic ulcerative colitis patients on an ProDepression A Risk Factor for Infant Mental HealthMammography Benchmarks Angoff Process
影像学诊断
腹部立位片：简单易行的基本检查；可见低位性肠 梗阻，扩张的结肠及气液平。
Asymptomatic ulcerative colitis patients on an ProDepression A Risk Factor for Infant Mental HealthMammography Benchmarks Angoff Process
Asymptomatic ulcerative colitis patients on an ProDepression A Risk Factor for Infant Mental HealthMammography Benchmarks Angoff Process
The important role of barium enema in the diagnosis of Hirschsprung's disease
Asymptomatic ulcerative colitis patients on an ProDepression A Risk Factor for Infant Mental HealthMammography Benchmarks Angoff Process
概述
先天性巨结肠（Hirschsprung's disease）又称肠 无神经节细胞症。
发病率 国内资料 1：5000 家族发病率 4% 性别比率 男：女=4：1
Asymptomatic ulcerative colitis patients on an ProDepression A Risk Factor for Infant Mental HealthMammography Benchmarks Angoff Process
Asymptomatic ulcerative colitis patients on an ProDepression A Risk Factor for Infant Mental HealthMammography Benchmarks Angoff Process
病变范围
常见型（约75%）：无神经节细胞区自肛门开 始向上延伸至乙状结肠远端；
诊断
凡新生儿时期出现胎便排出异常，或以后反复便秘、肛 门指检壶腹部空虚，随之有大量气便排出症状缓解者， 均应怀疑有先天性巨结肠之可能；
Asymptomatic ulcerative colitis patients on an ProDepression A Risk Factor for Infant Mental HealthMammography Benchmarks Angoff Process
短段型及超短段型（8%）：无神经节细胞段局 限于直肠远端部分；
长段型：包括降结肠、结肠脾曲（10%），甚 至大部分横结肠（4%）；
极少数整个结肠受累，甚至包括回肠末端 （3%）。
Asymptomatic ulcerative colitis patients on an ProDepression A Risk Factor for Infant Mental HealthMammography Benchmarks Angoff Process
概述
1886年丹麦医师Harald Hirschsprung 首次 详细描述并命名为Hirschsprung Disease。
1908年Ｆinney总结该病的９种发病机制。 1948年Ｓwenson和Ｂill报告手术切除痉挛
段，治疗成功，此后认识到主要病变在结肠 痉挛段无神经节细胞。
Asymptomatic ulcerative colitis patients on an ProDepression A Risk Factor for Infant Mental HealthMammography Benchmarks A便或胎便排出延迟，90%以上HD患儿在出生后 24小时内不排便；
腹胀，呈蛙状腹，进行性加重，大量肠内容物及气体滞 留于结肠；
一般情况差，长期营养不良、消瘦、发育延迟，肾上腺 素能神经及非肾上腺素能非胆碱能神经异常；
Asymptomatic ulcerative colitis patients on an ProDepression A Risk Factor for Infant Mental HealthMammography Benchmarks Angoff Process
Asymptomatic ulcerative colitis patients on an ProDepression A Risk Factor for Infant Mental HealthMammography Benchmarks Angoff Process
Asymptomatic ulcerative colitis patients on an ProDepression A Risk Factor for Infant Mental HealthMammography Benchmarks Angoff Process
Asymptomatic ulcerative colitis patients on an ProDepression A Risk Factor for Infant Mental HealthMammography Benchmarks Angoff Process
Asymptomatic ulcerative colitis patients on an ProDepression A Risk Factor for Infant Mental HealthMammography Benchmarks Angoff Process
临床表现
小肠结肠炎：
如果HD患儿出现腹泻、发热、腹胀加重，应考虑小肠 结肠炎；
术前术后均可发生，是引起死亡最常见的原因。
Asymptomatic ulcerative colitis patients on an ProDepression A Risk Factor for Infant Mental HealthMammography Benchmarks Angoff Process
Asymptomatic ulcerative colitis patients on an ProDepression A Risk Factor for Infant Mental HealthMammography Benchmarks Angoff Process
Asymptomatic ulcerative colitis patients on an ProDepression A Risk Factor for Infant Mental HealthMammography Benchmarks Angoff Process
诊断
辅助检查：
X线检查：腹部立位片 钡剂灌肠
直肠肛管测压：直肠肛管抑制反射（RAIR）对HD诊断 有重要价值，90%以上的HD患儿RAIR消失。
直肠黏膜吸引活检组织化学检查：乙酰胆碱酯酶定性检 查。
直肠壁组织学检查。
Asymptomatic ulcerative colitis patients on an ProDepression A Risk Factor for Infant Mental HealthMammography Benchmarks Angoff Process
Asymptomatic ulcerative colitis patients on an ProDepression A Risk Factor for Infant Mental HealthMammography Benchmarks Angoff Process
影像学诊断
钡剂灌肠：对于典型的HD在新生儿期钡灌肠仍可见狭 窄段、移行段及扩张段。