目的探讨采用升结肠贴补回肠直肠心形吻合术治疗全结肠无神经节细胞症的临床效果。方法分析自2006年6月至2013年6月本院收治的15例新生儿严重腹胀、低位小肠梗阻和肠穿孔病例资料，一期仅行急诊回肠末端造瘘加结肠多处活检，术后经病理检查明确诊断为全结肠无神经节细胞症；6～12个月后，行二期升结肠贴补回肠直肠心形吻合术；记录术后并发症的发生情况，并记录随访期间排便情况、肛门测压情况和机体内环境情况。结果所有手术均获成功，平均住院时间10．5 d，平均出血量30 mL，术后出现小肠结肠炎2例；无肠吻合口瘘、切口感染、肛门狭窄等，无死亡病例；术后随访1～2年，平均随访1．2年，术后早期肛周有红肿、糜烂，6个月后基本消失；术后大便次数约6～9次／d，2年后大便次数约2～3次／d；粪便由稀水样转为软粪；1例出现污粪，无大便失禁；随访血电解质、血红蛋白、白蛋白、球蛋白均正常，生长发育良好，接近正常儿；发现巨结肠根治术后直肠静息压、肛管静息压均较术前明显减低（P ＜0.05）；1例出现直肠肛门抑制反射。结论升结肠贴补回肠直肠心形吻合术既保留了吸收功能较好的右半结肠，符合结肠生理，同时行回肠直肠心形吻合，吻合口呈心形斜面，口径宽大且不在同一平面上，无狭窄、盲袋及闸门综合征的发生，可获较好排便功能，是根治全结肠无神经节细胞症的一个有效而可行的术式。%Objetive To explore the clinical effects of ascending colon subsidies ileorectal by heart-shaped anastomosis in treating the entire colon Hirschsprung’s disease.Methods The clinical data of 15 ca-ses of severe bloating,low small bowel obstruction and intestinal perforation in neonatal treated in our hospital from Jun.2006 to Jun.2013 were collected and analyzed.All the 15 cases underwent emergency surgery of il-eal fistula plus multiple colonic biopsies in phase I,and were confirmed to be entire colon Hirschsprung’s dis-ease by pathological detection.The phase II surgery of ascending colon subsidies ileorectal by heart-shaped an-astomosis was conducted 6 ~12 months later,with the occurrence of postoperative complications as well as def-ecation, anal manometry conditions and internal environment during the follow-up period recorded. Results All operations were successfully conducted.The average hospital stay was 10.5 days;the average a-mount of bleeding was 30ml;postoperative complications (enterocolitis)were found in 2 cases;no anastomotic leakage,no wound infection,no anal stenosis,and no death occured in any case.1 ~2 years of follow-up was paid to the patients (1.2 years in average).Perianal swelling and erosion occurred in the early postoperative period and disappeared after 6 months;Stool frequency of them was about 6 ~9 times after the operation,and decreased to 2 ~3 times two years after the operation;stool property changed from watery to soft feces;fecal pollution occurred in 1 case,no fecal incontinence occured;the electrolytes and the hemoglobin,albumin, globulin of blood were normal durning the postoperative follow-up period;the patients grow well as normal child.Comparing to those before the operation,the rectal resting pressure and anal resting pressure both signif-icantly reduced(P <0.05 ).Anorectal inhibitory reflex occurred in 1 case. Conclusion This surgical ap- proach of ascending colon supplement ileorectal by heart-shaped anastomosis not only retains the right colon with better function of absorption,but also avoids the occurrence of stenosis,blind bags and gates syndrome, thus better bowel function can be acquired owing to heart-shaped anastomosis ilealrectal.In short,this surgical approach is an effective and feasible surgical procedures in curing entire colon Hirschsprung’s disease.
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