腹腔镜辅助结肠次全切除术治疗先天性肠神经元发育不良症的对比研究

Comparative of Laparoscopic Subtotal Colectomy Procedure for intestinal neuronal dysplasia Type B

目的：对比研究先天性肠神经元发育不良症B型患儿行腹腔镜下结肠次全切除术、Soave术和Duhamel术的临床疗效。方法回顾性分析2005年3月至2013年3月接受腹腔镜结肠次全切除的B型肠神经元发育不良患儿的随访资料。其中接受腹腔镜辅助下Duhamel术（直肠后拖出术）28例，同期接受腹腔镜辅助下Soave术（直肠内拖出术）34例。入选的62例患儿诊断依据为临床表现、术前影像学结果、直肠黏膜活检、术中快检和术后病检报告。分别比较两组手术时间、术中出血量、术后住院时间、排便频率、术后并发症及排便功能方面有无差异。结果腹腔镜Duhamel术和腹腔镜Soave术在手术时间、术中出血量、术后住院时间方面比较，均无统计学意义（P＞0.05）。排便频率比较：腹腔镜下Duhamel术患儿术后2周内排便频率平均8次/d，明显少于腹腔镜下Soave术的15次（P＜0.05）；恢复到正常排便频率（1～2次/d）时间比较：腹腔镜下Duhamel术平均为（3.5±0.5）个月，腹腔镜下Soave术平均为（6.7±0.8）个月，差异有统计学意义（P＜0.05）。腹腔镜下Duhamel术患儿术后发生小肠结肠炎1例（3.6％），污粪1例（3.6％），粪石1例（3.6％）；腹腔镜下Soave术后发生小肠结肠炎4例（11.8％），吻合口狭窄1例（2.9％），便秘复发2例（5.8％），污粪1例（2.9％）。肛周湿疹的发生率：Soave术组明显高于Duhamel术组（41.2％VS 7.2％）。58例术后获功能随访，平均随访时间为4年6个月（2个月至8年）。接受腹腔镜下Duhamel术的患儿其排便功能优良率为85.2％，腹腔镜下Soave术为87.0％。两组差异无统计学意义（P＞0.05）。结论行结肠次全切除的B型肠神经元发育不良患儿，腹腔镜下Duhamel术后小肠结肠炎的发生率低于Soave术式，腹腔镜下Duhamel术后近期排便频率少，且排便频率更快恢复正常，肛周湿疹发生率低。两种术式排便功能优良率相当。

Objetive To compare early and late outcomes of subtotal colectomy using laparoscopic Soave procedure and Duhamel procedure for intestinal neuronal dysplasia Type B （IND-B）. Methods Based on the preoperative radiographic diagnosis and operative biopsy findings,62 patients with IND-B who received subtotal colectomy between March 2005 and March 2013 were analyzed.Laparoscopic-assisted Duhamel procedure was performed in 28 out of 62 patients.34 patients were treated with laparoscopic-assisted Soave procedure.In each group,operation time,intraoperative blood loss,hospital stays,postoperative complications and a range of functional outcomes were analyzed to evaluate of the effectiveness of the laparoscopic-assisted Soave and Duhamel procedure. Results There was no difference between the two groups regarding sex,family history,op-erative age,and operative time,intraoperative blood loses and hospital stays.The average defecation frequency in the postoperative 2 weeks in Duhamel group was 8 （3～15 ）time per day,which was significantly less than that of Soave 15 （4～28）times per day.The average time interval to maintain normal defecation frequency in Duhamel was（3.5 ±0.5）months,which was significantly shorter than that of Soave 6.7 ±0.8 months）（P〈0.05 ）.As for postoperative complications,in Duhamel subtotal colectomy,the incidence of enterocolitis and soiling was both 3.6%,there was no anastomotic leak or stricture.Recurrent constipation was also not ob-served.One fecaloma formation occurred at third month after surgery.In Soave subtotal colectomy,there were 4 enterocolitis （11.8%）,2 recurrent constipation （5.8%）,1 anastomotic stricture （2.9%）,and 1 soiling （2.9%）.Perianal excoriation was extremely higher in Soave than in Duhamel （7.2% VS 41.2%）.58 patients managed to take defecation evaluation.During long time follow-ups,the defecation function was similar for Duhamel and Soave in laparoscopic subtotal colectomy （85.2% VS 87.0% in satisfactory defecation function rate）.The difference was not significant （P〉0.05）.Conclusion In laparoscopic subtotal colectomy for IND-B,compared with Soave,patients in Duhamel had significantly less perianal excoriation.Enterocolitis and recurrent constipation were also less frequent in Duhamel but it is not significant.Meanwhile patients in Duhamel had less postoperative defecation frequency and earlier regaining of normal defecation frequency.Despite of this,a long-term defecation function evaluation saw no difference between the two groups.