腹腔镜下结肠切除Soave直肠内拖出术在肠神经元发育不良症中的应用

目的探讨腹腔镜下结肠切除、Soave直肠内拖出术治疗肠神经元发育不良症（IND）的可行性和中期随访结果。方法对25例IND和先天性巨结肠合并IND（HaIND）的患儿在腹腔镜下行Soave、结肠切除术，其中IND17例，HaIND8例。所有病例通过钡灌肠及延迟拍片、直肠黏膜活检、直肠肛管测压、腹腔镜浆肌层或全层活检明确诊断及病变范围。采用一个观察孔，2～4个操作孔。根据病变范围在腹腔镜下分别游离降结肠、横结肠、升结肠及系膜，会阴部手术按改良Soave方法。对患者手术方法、病变范围的确定、术后并发症和临床结果进行评估。结果25例中，10例行左半结肠切除，15例行结肠次全切除，按Deloyers法将升结肠逆时针转位270°下拖。行左半结肠切除术的患儿术前24h钡剂残留部位均在降结肠远端以远肠管，行结肠次全切除术的患儿术前24h钡剂残留在降结肠近端以近的肠管。平均手术时间分别为145min和188min，平均失血40ml。术后并发症：吻合口瘘2例，重度肛周皮肤红肿、糜烂11例，肠粘连1例，小肠结肠炎2例。术后平均随访4年，行左半结肠切除术的患儿，术后3个月内排便恢复正常；行结肠次全切除术的患儿术后3个月大便每日4～10次，12～24个月每日大便2～3次。2例出现污粪，无便秘复发和大便失禁病例。结论腹腔镜下结肠切除、Soave术治疗IND和HaIND安全、可行，创伤较小，术后并发症少，术后中期随访疗效满意。术前钡灌肠24h延迟拍片钡剂残留部位可以预测结肠切除的近端位置。

Application and followup outcome of laparoscopic extensive coloectomy with transanal Soave pullthrough for intestinal neuronal dysplasia

Objective To investigate the feasibility and functional outcome of laparoscopic coloectomy with transanal Soave pull-through for intestinal neuronal dysplasia （IND）. Methods 25 infants and children with IND and Hirschsprung＇ s disease associated IND （HaIND） were treated by this operation. Of them is IND in 17 cases, HaIND in 8 cases. Patients were evaluated clinically, and the diagnosis was achieved by examination of anorectal manometry, suction biopsies and full-thickness rectal biopsies, X-ray contrast enema, laparoscopic biopsies. We used one camera port and three or four working ports. The sigmoid, transverse, and right colon up to the last ileal cove were mobilized laparoscopically in the extended form of IND and HaIND. A modified Soave＇ s anastomosis was performed. Results 10 patients underwent a laparoscopic left colectomy with modifed Soave procedures, 15 patients were treated by laparoscopic subtotal colectomy and required a Deloyers＇ maneuver for the pull-through. The proximal margin of stagnation of barium in patients with left colectomy was restricted to the distal to descending colon, sigmoid colon, and that in patients with subtotal colectomy was restricted to the proximal to descending colon, transverse colon, hepatic flexure and ascending colon. The mean operating time was 145 minutes in and 188 minutes, and the mean blood loss was about 40 ml. Postoperative complications included an anastomotic leak in 2 patient, a sever perianal erosion in 11 patients, 1 adhesion formation, 2 postoperative enterocolitis and 2 soil. During a mean follow-up of 4 years, bowel frequency was regular in 3 months in cases with the left colon resection, and the defecation frequency was 4-10 times per day in postoperative 3 months and 2-3 times per day after 12-24months of operation in cases with subtotal coloectomy. The clinical results were goods,with no stool incontinence and no constipation. Conclusions Laparoscopic procedure for left colon and subtotal colon resection with transanal Soave puLL-through