3月内婴儿先天性肠无神经节细胞症新的诊疗方法应用及疗效随访

发布时间：2018-01-17 17:06

本文关键词：3月内婴儿先天性肠无神经节细胞症新的诊疗方法应用及疗效随访　出处：《重庆医科大学》2014年硕士论文　论文类型：学位论文

【摘要】：目的： 1.探讨钙视网膜蛋白在3月内婴儿先天性肠无神经节细胞症的临床诊断价值； 2.探讨采用经肛门直肠结肠切除斜形吻合术治疗3月内婴儿先天性肠无神经节细胞症的随访及疗效评价。方法： 1.收集2008年至2012年于我院新生儿外科诊断为先天性肠无神经节细胞症的3月内婴儿病案资料，随访行了经肛门直肠结肠切除斜形吻合术的患儿，登记生长发育、排便功能等19项指标，评价肛直肠功能及生活质量。 2.收集2010年至2013年于我院新生儿外科已行经肛门直肠结肠切除斜形吻合术以及肠造瘘术，术后病理检查确诊为先天性肠无神经节细胞症的3月内婴儿病例，统计术前直肠粘膜活检结果。结果： 124例先天性肠无神经节细胞症患儿，术前诊断依据典型病史、体格检查以及特殊辅助检查，特殊的辅助检查包括钡剂灌肠（contrastenema,CE）、直肠肛管测压（anorectal manometry,ARM）和直肠吸引活检（rectal suction biopsy,RSB），阳性率分别为81.4%、94.1%、98.3%。43例行结肠造瘘术，81例行经肛门直肠结肠切除斜形吻合术。81例行经肛门直肠结肠切除斜形吻合术的患儿术后中期随访46例，无长期顽固性便秘、大小便失禁发生，1例患儿术后3月并发小肠结肠炎，2例患儿有污粪发生，1例患儿有大便次数增多，所有男性患儿均有阴茎勃起表现，随访患儿生长发育与正常婴幼儿相当。门诊随访病人15例，粪便常规、血液分析、肝功和肾功等常规检查均在正常范围。钡剂灌肠检查显示患儿肠道形态恢复良好，11例病人行直肠肛管测压检查，9例病人肛门松弛反射恢复正常，1例病人可见松弛反射，但不完全，1例患儿未见松弛反射。 2010年至2013年术后病理检查确诊为先天性肠无神经节细胞症的患儿中102例行了直肠吸引活检，100例HE染色未见神经节细胞，CR阴性，2例短段型HE染色可见神经节细胞，CR阳性。结论： 1.诊断3月内婴儿先天性肠无神经节细胞症依据典型病史、体征及三项特殊辅助检查，钡剂灌肠诊断阳性率为81.4%，，直肠肛管测压诊断阳性率为94.1%，直肠吸引活检诊断阳性率为98.3%，可将钙视网膜蛋白免疫组织化学染色检查作为3月以内小婴儿先天性肠无神经节细胞症术前诊断的“金标准”； 2.经肛门直肠结肠切除斜形吻合术术后中期随访临床功能恢复良好，并发症发生率低，适用于3月以内婴儿。
[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.
【学位授予单位】：重庆医科大学
【学位级别】：硕士
【学位授予年份】：2014
【分类号】：R726.5

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