1、課程名小兒外授課題1教授課日授課課程名小兒外授課題1教授課日授課年2011學(xué)生人編 人民衛(wèi)生主科學(xué)技PediatricSurgery,PremPuri,SpringerDordrechtHeidelbergLondon New York, USA外語(yǔ)要求（根據(jù)專本七年制不同層次每個(gè)病種提出 5-10 個(gè)中英文無神經(jīng)節(jié)細(xì)胞癥（Aganglionosis)、Hirschsprung 病（ Hirschsprung 性巨結(jié)腸(Congenitalmegacolon)、Torre、腹腔用 概 念 ： 無 神 經(jīng) 節(jié) 細(xì) 胞 癥用 概 念 ： 無 神 經(jīng) 節(jié) 細(xì) 胞 癥（Aganglionosis) 是

2、以便秘為主要臨床化道畸形；又叫 Hirschsprung 病（Hirschsprungdisease，HD） .Congenital megacolon ， HD is characterized by the absence of myenteric and submucosal ganglion cells he distal alimentary tract; resulting in decreased motility he affected bowel segment.More n 50 years old since the discovery of the cause and

3、the treatment for Hirschprung disease.y1per5000live.Male-to-femaleratioof4:1（4timesmore n females）.In90%aremature結(jié)合概念介 紹無神經(jīng)節(jié) 介紹該病發(fā) .In1-6%are.In1-6%are.In10%-30%areted. 610沿迷走神經(jīng)從頭側(cè)向尾側(cè)遷移,到第 121.Migration of neuroblasts on the 5-On the 12. g. k they reach the Ganglion cells, which are derived from the

4、 neural crest, migrate caudally with the vagal nerve fibers（迷走神經(jīng)纖維） along the estineArrest in migration leads to an aganglionic segment.The primary defect is the absence of the submucosal ( 粘膜下)and myenteric plexuses（肌間神經(jīng)叢）as the result ofa defective migration of ganglion cell precursors of the neur

5、al crest o the 說明胚 胎期神經(jīng)嵴 神經(jīng)細(xì)胞遷 ts not only affects cholinergic neuronsts not only affects cholinergic neurons（膽堿能神經(jīng)元） but also non-adrenergic（非 腎上 腺素能 ）, non-cholinergic neurons, which use nitric oxide as their chemical messenger. 5.Research resultsEtiologyofHDisnotischemia 缺血、 hypoxia 缺氧 toxininfla

6、mmation炎癥 genetics 遺傳10thEndothelinB【病理改變】（6 分鐘） 2.(1) 常見長(zhǎng)段短段超短段型 罕見全結(jié)腸型 擴(kuò)張段:肌層肥厚, 粘膜炎癥, 粘膜神經(jīng)節(jié)細(xì)胞完全缺如,大量的副交感神經(jīng)手術(shù) 病理 介紹無經(jīng)結(jié)細(xì) 胞癥的病理 。Macropathology Transitional zoneNarrowed estine：Thisabsenceofparasympathetic innervation( 副交感神經(jīng)分布) prevents gut peristalsis, leading to functional constipation.Dilate esti

7、ne ：The proximal estine hypertrophied by trying tofunctional obstruction.Transitionalzone：Itexistsbet nnormal and abnormal aganglionic estine.Histopathology Narrowed estine Absence of parasympathetic ganglion cells in the myenteric and submucosal plexus of the rectum and/or colon Limitednumberofadre

8、nergicestine The normal ganglion cells in the myenteric and submucosal plexus of theThe number and diameter of the cholinergic nerve is normalHypertrophied muscular layer 肌層肥Inflammation catarrhal of mucosa 粘 Ultra-short-segment HD 超短段型短段型ClassicalHD（Rectosigmoid）常見型病變位于直腸乙狀結(jié)腸交界處 長(zhǎng)段型Totalcolonicagan

9、glionosis 全結(jié)腸型estinal aganglionosis 4.Pathophysiology .This absence of normal parasympathetic innervation prevents gut peristalsis, leading to functional constipation. 缺乏正常副交.The proximal colon hypertrophied by 4.Pathophysiology .This absence of normal parasympathetic innervation prevents gut perist

10、alsis, leading to functional constipation. 缺乏正常副交.The proximal colon hypertrophied by trying toe functional obstruction.副交感神增多 乙酰膽堿堆）減少 腸管舒張； The disease can considered to be estinalobstruction不全性腸The lengh of the aganglionic segment is variable, and the symptoms are variable too無神經(jīng)節(jié)細(xì)胞腸管的長(zhǎng)度不同，癥狀The

11、symtomsappearsindifferent1.Symptomsinnewbornage新生兒巨結(jié)腸Failure to pass meconium within the 48hoursoflife 胎便排出延遲（生后48小時(shí)未解Biliousvomiting The rectal tube cant be put easily 肛symptoms returnagain inafew days ,abdomenispalpable Abdominal t is by rectal stimulation or,abdomenispalpable Abdominal t is by re

12、ctal stimulation or enemas 刺激直Rectaltouch:explosive of gasand 脹SevereConstipation Meteorism /Abdominal Failureto Hypoproteinaemia, anaemia 低蛋白Electrolyte disorders 電解質(zhì)紊亂 3.Symptoms in childhood 兒童期巨結(jié)腸Dilateddrumlikebelly Longhistoryofconstipation長(zhǎng)期（頑Defecationin7-10days7-10Gracilelimbs Multiplefecal

13、The stimulus of defecation is Rectum is empty and narrow 直腸空plications Rectum is empty and narrow 直腸空plications estinalperforation Water oxication results from the use of tap water enemas. or there may be hypertonic dehydration from saline enemas.水 常常是用自來水灌腸所（ 4 ） water oxication, malnutrition, fail

14、ureto thriveandanemia 水 、營(yíng)養(yǎng)which accounts for almost all of the mortality from Hirschsprung disease. HD率25-30%，這幾乎是導(dǎo)致 HDExplosive liquid stools, fever, and severe prostration are indicative of enterocolitis. 性排便、發(fā)熱、嚴(yán)重脫Enterocolitis is rare (10%)month but rises to 33% in the second and third months.

15、Recallt diarrhea may be a late sign. 1 月的發(fā)生病例很少，約占 10%；但在隨后的 2 個(gè)月上升至 33%；往往腹瀉是比較晚期的癥SymptomsofSymptomsof.Life-threateningcondition .Diarrheaitcanbeanearlysign 腹瀉：早.Toxic.Abdominal.Bile-stainedvomiting .Fever and signs of dehydration 發(fā)熱、脫.Rectal tube:explosive expul of gas and foul-smelling stools 置

16、： 樣排氣腸內(nèi)容物集聚，誘發(fā)細(xì)菌 ；細(xì)菌毒素?fù)p傷腸壁 ，使 壁通透性增加，時(shí)或不正確均可導(dǎo)致患兒 ；其與患兒的 、病變腸管的長(zhǎng)度無關(guān)。（15分鐘通過影像資 疫組化 講解各種臨（15分鐘通過影像資 疫組化 講解各種臨床鋇Only15%are of life, but two thirds 3months. Cases5 years months. Cases5 years of age usually have ultra- short segment disease. 生后 1 月僅有 15%的確診，2/3的病人在生后3 月確診，5 歲以后確診的病Casehistory .To beestin

17、alobstruction 不.Failure to pass meconium within 48.Severe Delayed 營(yíng)養(yǎng)不良、發(fā)育遲緩.Abdominal.Abdominalwallestinalpattern .Coprolith .rectal vault is found devoid of stool and the c feels narrow with increased tone &Acetylcholinesterase staining 直腸粘膜.Full thickness rectal biopsy 直腸壁組織.Full thickness rectal

18、biopsy 直腸壁組織 -Narrowdistald transition zone 漏斗狀移-Dilationofproximalcolon -After 24-48 hours the contrast material is he -Mucosal irregularity (enterocolitis) 腸粘 Anorectal prere iselevated 檢測(cè)肛直No relaxation of ernal It can be put through safely in newborn age as well 新生兒期可用的安全檢查Absence of normal relaxation of the ernal sphincter when the rectum is distended wiballoon. 當(dāng)直腸內(nèi)用氣球射Rectal biopsy,histopathology 直腸活 Full-thicknessstrip-biopsy Suctionbiopsy InHD,the biopsy.absence of ganglion cells 神經(jīng)節(jié)細(xì)胞缺.hypertrophy and hyp .hy