本文關(guān)鍵詞：3月內(nèi)嬰兒先天性腸無神經(jīng)節(jié)細胞癥新的診療方法應用及療效隨訪　出處：《重慶醫(yī)科大學》2014年碩士論文　論文類型：學位論文

  更多相關(guān)文章： 先天性腸無神經(jīng)節(jié)細胞癥 3月內(nèi)嬰兒 鈣視網(wǎng)膜蛋白 經(jīng)肛門直腸結(jié)腸切除斜形吻合術(shù)

【摘要】：目的： 1.探討鈣視網(wǎng)膜蛋白在3月內(nèi)嬰兒先天性腸無神經(jīng)節(jié)細胞癥的臨床診斷價值； 2.探討采用經(jīng)肛門直腸結(jié)腸切除斜形吻合術(shù)治療3月內(nèi)嬰兒先天性腸無神經(jīng)節(jié)細胞癥的隨訪及療效評價。 方法： 1.收集2008年至2012年于我院新生兒外科診斷為先天性腸無神經(jīng)節(jié)細胞癥的3月內(nèi)嬰兒病案資料，隨訪行了經(jīng)肛門直腸結(jié)腸切除斜形吻合術(shù)的患兒，登記生長發(fā)育、排便功能等19項指標，評價肛直腸功能及生活質(zhì)量。 2.收集2010年至2013年于我院新生兒外科已行經(jīng)肛門直腸結(jié)腸切除斜形吻合術(shù)以及腸造瘺術(shù)，術(shù)后病理檢查確診為先天性腸無神經(jīng)節(jié)細胞癥的3月內(nèi)嬰兒病例，統(tǒng)計術(shù)前直腸粘膜活檢結(jié)果。 結(jié)果： 124例先天性腸無神經(jīng)節(jié)細胞癥患兒，術(shù)前診斷依據(jù)典型病史、體格檢查以及特殊輔助檢查，特殊的輔助檢查包括鋇劑灌腸（contrastenema,CE）、直腸肛管測壓（anorectal manometry,ARM）和直腸吸引活檢（rectal suction biopsy,RSB），陽性率分別為81.4%、94.1%、98.3%。43例行結(jié)腸造瘺術(shù)，81例行經(jīng)肛門直腸結(jié)腸切除斜形吻合術(shù)。81例行經(jīng)肛門直腸結(jié)腸切除斜形吻合術(shù)的患兒術(shù)后中期隨訪46例，無長期頑固性便秘、大小便失禁發(fā)生，1例患兒術(shù)后3月并發(fā)小腸結(jié)腸炎，2例患兒有污糞發(fā)生，1例患兒有大便次數(shù)增多，所有男性患兒均有陰莖勃起表現(xiàn)，隨訪患兒生長發(fā)育與正常嬰幼兒相當。門診隨訪病人15例，糞便常規(guī)、血液分析、肝功和腎功等常規(guī)檢查均在正常范圍。鋇劑灌腸檢查顯示患兒腸道形態(tài)恢復良好，11例病人行直腸肛管測壓檢查，9例病人肛門松弛反射恢復正常，1例病人可見松弛反射，但不完全，1例患兒未見松弛反射。 2010年至2013年術(shù)后病理檢查確診為先天性腸無神經(jīng)節(jié)細胞癥的患兒中102例行了直腸吸引活檢，100例HE染色未見神經(jīng)節(jié)細胞，CR陰性，2例短段型HE染色可見神經(jīng)節(jié)細胞，CR陽性。 結(jié)論： 1.診斷3月內(nèi)嬰兒先天性腸無神經(jīng)節(jié)細胞癥依據(jù)典型病史、體征及三項特殊輔助檢查，鋇劑灌腸診斷陽性率為81.4%，，直腸肛管測壓診斷陽性率為94.1%，直腸吸引活檢診斷陽性率為98.3%，可將鈣視網(wǎng)膜蛋白免疫組織化學染色檢查作為3月以內(nèi)小嬰兒先天性腸無神經(jīng)節(jié)細胞癥術(shù)前診斷的“金標準”； 2.經(jīng)肛門直腸結(jié)腸切除斜形吻合術(shù)術(shù)后中期隨訪臨床功能恢復良好，并發(fā)癥發(fā)生率低，適用于3月以內(nèi)嬰兒。
[Abstract]:Objective: 1. To evaluate the clinical value of calcium retina protein in the diagnosis of congenital intestinal apoglioneurosis in infants in March. 2. To evaluate the effect of transrectal colectomy and oblique anastomosis in the treatment of congenital intestinal apogoneurosis in infants in March. Methods: 1. To collect the data of the infantile cases from 2008 to 2012, which were diagnosed as congenital Aganglionephritis in our hospital from 2008 to 2012. To evaluate the anorectal function and quality of life, 19 indexes such as growth and development and defecation function were recorded in the children who underwent transrectal colectomy and oblique anastomosis. 2. From 2010 to 2013, transrectal colonic resection and oblique anastomosis and enterostomy were performed in neonatal surgery in our hospital. In March infants with congenital intestinal apogoneurosis were diagnosed by pathological examination and the results of preoperative rectal mucosal biopsy were analyzed. Results: 124 cases of congenital intestinal apogoneurosis were diagnosed according to the typical history, physical examination and special auxiliary examination before operation. Special adjuvant examinations included barium enema contrastenema1, rectal anus manometry and anorectal manometry. The positive rates of ARM) and rectal suction biopsyn RSBs were 81.4% and 94.1%, respectively. 98.3. 43 cases underwent colostomy 81 cases underwent transrectal colonic resection and oblique anastomosis 81 cases underwent anorectal colectomy and oblique anastomosis 46 cases were followed up in the middle stage of operation. No long-term obstinate constipation, incontinence occurred in 1 case of postoperative March with enterocolitis in 2 cases of feces in 1 case with increased defecation, all male children have penile erectile symptoms. The growth and development of children were similar to those of normal infants. 15 cases were followed up in outpatient department, stool routine, blood analysis. The routine examination of liver function and renal function were in the normal range. Barium enema examination showed that the shape of the intestine recovered well in 11 cases and the anal relaxation reflex returned to normal in 9 cases. Relaxation reflex was found in 1 patient, but not in 1 patient. From 2010 to 2013, 102 cases of congenital intestinal apoglioneurosis were diagnosed by postoperative pathological examination. 100 cases of rectal aspiration biopsy were diagnosed by HE staining, and no CR was found in ganglion cells. The ganglion cells were positive for CR in 2 cases with short segment HE staining. Conclusion: 1. In March, the positive rate of barium enema was 81.4% according to the typical history, signs and three special auxiliary examinations. The positive rate of anorectal manometry was 94. 1 and that of rectal aspiration biopsy was 98.3%. The calcium retinal protein immunohistochemical staining can be regarded as the "golden standard" for the preoperative diagnosis of congenital intestinal apoglioneurosis in infants within March. 2. Transanorectal colonic resection and oblique anastomosis are suitable for infants within March due to their good recovery of clinical function and low incidence of complications.
【學位授予單位】：重慶醫(yī)科大學
【學位級別】：碩士
【學位授予年份】：2014
【分類號】：R726.5

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