乙状结肠冗长症的诊断及手术治疗

目的 探讨小儿乙状结肠冗长症的诊断及手术治疗方法。方法 利用钡灌肠对便秘患儿的乙状结肠长度进行测量，超出范围者即被认为患乙状结肠冗长症。乙状结肠冗长症患儿23例进行了手术治疗，其中经腹行乙状结肠切除、降结肠直肠吻合术14例（经腹组）；经肛门入路拖出Soave术9例（经肛门组）。结果 手术后症状完全消失18例，症状改善5例，其中9例经肛门入路Soave手术患儿均获治愈，对经腹组与经肛门组的治愈率进行比较差异有显著性（P〈0．05）。结论 小儿乙状结肠冗长症诊断首选钡灌肠，总体手术效果良好，以肛门入路Soave手术效果最佳。     

经肛门改良Soave Ⅰ期拖出术治疗小儿先天性巨结肠症

目的总结经肛门改良SoaveⅠ期拖出术治疗小儿先天性巨结肠症的疗效及随访情况。方法自2001年采用该术式治疗小儿先天性巨结肠症26例，术前均经钡灌肠、直肠粘膜吸引活检等辅助检查诊断为先天性巨结肠症。结果切除结肠16cm-22cm，术后24h内肠道功能恢复，第2～3d开始进食无异常。术后1月随访，每日排便6-9次，其中12例行钡灌肠检查，未发现扩张结肠，24h钡剂无残留；18例6个月随访，每日排便2-4次；17例1年随访，5例诉时有大便次数多(每日排便5～6次)，12患儿每日排便2～3次；14例2年随访，1例因不洁饮食腹泻后出现腹胀1次，补液后缓解，13例排便正常，每日1-3次；12例术后已3年，生长发育正常，每日排便1-3次。全部患儿未行扩肛处理，无腹胀、便秘、污粪。结论经肛门改良SoaveI期拖出术治疗小儿常见型先天性巨结肠症，手术创伤小，操作简单，近期疗效好。     

14例小儿乙状结肠冗长症手术治疗分析

目的总结小儿乙状结肠冗长症的诊断、治疗、疗效及短期随访经验。方法2005年至2012年我们收治17例乙状结肠冗长症患儿,其中经手术治疗14例,保守治疗3例。回顾性分析14例手术治疗患儿临床资料。结果14例手术治疗患儿中,13例行经肛门结肠拖出术,1例行经腹Soave术。术后随访8例,最短半年,最长4年,均无吻合口瘘、吻合口狭窄及便秘等并发症。3例行药物、灌肠、饮食疗法等保守治疗6个月,症状好转,2~3 d排便一次。结论小儿乙状结肠冗长症的诊断主要依据临床表现及钡灌肠检查,确诊后应积极临床治疗。首选保守治疗,但症状改善缓慢,保守治疗无效或效果不满意者宜转手术治疗,手术首选经肛门结肠拖出术,损伤小,恢复快,无瘢痕,疗效明显。     

经肛门SoaveⅠ期拖出根治术治疗小婴儿先天性巨结肠

目的 总结经肛门SoaveI期拖出根治术治疗小婴儿先天性巨结肠的手术过程、疗效及短期随随访。方法 自1999年经该术式治疗小婴儿先天性巨结肠14例。均经钡剂灌肠和直肠粘膜吸引活检诊断为常见型巨结肠。结果 14例切除结肠17～24cm,均于术后2～4d开始进食,术后1个月随访;每日排便8～10次,其中9例行钡剂灌肠未见结肠扩张,24h钡剂潴留;6个月随访（12例）每日排便1～3次,无污染。结论 经肛门SoaveI期拖出根治术治疗小婴儿常见型先天性巨结肠,手术创伤小、操作简单、近期疗效良好。     

经肛巨结肠根治术后患儿的肛肠功能评价

目的：经肛门巨结肠一期根治术近期效果好,但术后患儿的肛肠功能目前尚不十分清楚。该文旨在评估患儿术后的排便模式,结肠和肛门括约肌功能。方法：对58例经肛门巨结肠根治术后半年以上儿童进行随访,随访时间为15.8 月 (6～24 月)。并进行排便功能问卷调查、钡灌肠、结肠传输时间和肛门直肠测压检查。33例正常儿童作为对照。结果：大多数随访患儿排便正常,无任何临床症状。４例出现稀便便频,9例污便,5例便秘,3例小肠结肠炎。钡灌肠结果显示大多数随访患儿结肠形态恢复良好。全部病例术后直肠肛管角（度）较对照组显著开大,有症状组较无症状组显著开大。58例随访患儿的平均全胃肠、左半结肠和右半结肠传输时间较术前显著缩短,与对照组相比差异无显著性意义。直肠肛管反射5例阳性。便秘组的肛管最大静息压和最大收缩压明显高于无症状组和对照组。污便组向量容积和对称指数较对照组显著降低。便秘组对称指数显著高于对照组。结论：经肛门巨结肠根治术后大多数患儿排便功能、结肠功能和括约肌功能良好。少数病例排便功能障碍可能与术后乙状结肠曲减少或消失、“新直肠”储便功能代偿不全和拖出结肠致直肠肛管角开大、肛门括约肌痉挛失弛缓有关。［中国当代儿科杂志,2007,9（3）：188-192］     

开腹和非开腹先天性巨结肠根治术疗效比较

目的：比较开腹和非开腹二组先天性巨结肠术远期生活质量，分析二组差异的原因，提出改良方法。方法：对二组31例患儿进行了远期排便控制临床评估和客观评估。开腹组，18例，男17例，女1例，手术方法为开腹经肛门拖出，病变肠管切除，直结肠斜面吻合，经过顺利。非开腹组，13例，男11例，女2例，非开腹性手术采用经肛门拖出病变肠管切除，直肠纵切、直结肠斜面吻合。二组患儿随访年龄均为4～7岁，随访2～4年。结果：通过对31例患儿的随访和生活质量评估，比较两种手术方式的效果。开腹组静息压高于非开腹组，非开腹组主动收缩压高于开腹组，钡灌肠肛管长度二组无差异。二组患儿术后1个月、6个月、2年排便控制临床评价，近期有差异，远期无差异。结论：避免内括约肌切除，又能彻底解除其痉挛狭窄是手术的关键。非开腹直结肠斜面吻合先天性巨结肠术有待改进。     

经尾路巨直肠及乙状结肠切除术在治疗肛门成形术后顽固性便秘中的应用

目的 探讨经尾路行巨直肠及乙状结肠切除术治疗肛门成形术后顽固性便秘的可行性。方法 患儿6例，年龄2～18岁，均为先天性肛门闭锁行会阴肛门成形术后合并顽固性便秘和充盈性大便失禁。钡灌肠示：直肠及乙状结肠高度扩张，蠕动差。6例患儿均经尾路切除直肠及远端乙状结肠，同时行肛门成形术。结果 平均手术时间205min(125min～265min)，切除扩张肠管长度23．3cm(10cm～40cm)。术后2月～4月后，6例患儿均可控制排便：12月～30月随访结果示：患儿便秘均消失。6例中4例患1度污便，另2例2度污便，无其他术后并发症。结论 尾路切除巨直肠及乙状结肠是治疗无肛术后顽固性便秘的有效方法。     

经肛门Ⅰ期根治巨结肠及应用解剖研究

目的：为提高巨结肠治疗效果,探讨经肛门Ⅰ期巨结肠根治手术的方法及并发症的防治措施。方法：对13例先天性巨结肠经肛门Ⅰ期根治,手术方法为:经齿状线上0.5 cm逐层梯度切开分离粘膜、环肌、纵肌,进入盆腔,处理结肠系膜及韧带,拖出并切除病变肠管,将近端结肠与齿状线上创面吻合。并对5例小儿尸体进行肛门直肠应用解剖研究。结果：全部手术获得成功,随访6～15月,肛门无狭窄,排便正常,无污粪。解剖研究发现:婴幼儿直肠前及两侧腹膜反折较后侧低,距肛门约5～6 cm,直肠与尿道或阴道之间组织较致密,间隙较小。结论：经肛门直肠采用粘膜、环肌、纵肌梯级分离推进式进入Ⅰ期根治小儿巨结肠,可避免损伤尿道及邻近器官,减少术后并发症。适用于普通型、短段型及部分长段型巨结肠。     

经肛门拖出及辅助腹部小切口根治长段型巨结肠33例报告

目的探讨经肛门拖出及/或选择性辅加腹部小切口根治长段型巨结肠的可行性与疗效。方法2001年1月～2005年3月经用该术式治疗婴儿长段型巨结肠33例。其中病变段位于乙状结肠上段16例,降结肠11例,脾曲4例,横结肠右侧2例。全组病例均经钡剂灌肠、肛门直肠测压、手术及病理检查确诊。实施I期手术32例,II期手术1例。结果17例单独经肛门拖出结肠,16例辅加腹部3～5cm小切口协助完成手术。切除病变结肠平均长度为39.2cm(32～63cm)。平均手术时间为145min(110～190min)。出血约10～50ml。术后恢复良好,6例出现肛周轻度红肿、糜烂,均于术后6～11d痊愈出院。30例经3个月～4.5年随访,生长发育良好,3个月内每日排便4～8次,6个月后每日2～3次。5例发生结肠炎,经保守治疗痊愈。轻度污粪及便秘各1例。无腹部并发症及吻合口狭窄。结论经肛门结肠拖出术根治婴儿长段型巨结肠方法可行,且安全、有效,操作较简便。对病变位于降结肠以上、结肠系膜较短者,可辅加腹部小切口协助完成手术,值得推荐。     

经肛门Ⅰ期结肠拖出术治疗新生儿及婴幼儿先天性巨结肠

目的 介绍经肛门Ⅰ期结肠拖出术治疗新生儿及婴幼儿先天性巨结肠的初步经验。方法 患儿全麻后取截石位，扩肛后，牵开肛门。在直肠齿状线上0．5cm处游离直肠粘膜，形成粘膜管后，继续向近端游离，达腹膜返折后环形切断肌鞘一周，劈开肌鞘后壁，并切除部分肌条。进入腹腔后，直视下游离直肠、结肠的移行段和扩张段，妥善结扎系膜血管，达正常结肠后切断，拖出结肠浆肌层与保留肌鞘间断固定，结肠断端及直肠齿状线切缘缝合固定。结果 7例均顺利完成手术，手术时间90min-100min，出血约15ml～30ml，术中均未输血。7例均经病理证实为先天性巨结肠症，平均切除肠管13cm～25cm。1例于术后6d出现小肠结肠炎，治疗后好转，其余6例恢复顺利。术后2周开始扩肛，全部病例随访2个月—1年，每日排大便2～4次，无便秘复发、腹胀，无吻合口狭窄，无污粪，无肛周感染、肠管同缩等并发症。结论 经肛门Ⅰ期结肠拖出术治疗先天性巨结肠，具有手术创伤小、操作简单、术后恢复快、并发症少等优点，适用于新生儿及婴幼儿短段型及普通型先天性巨结肠的治疗。     

Transanal coloanal pull-through with a short muscular cuff for classic Hirschsprung's disease.

INTRODUCTION: A totally transanal operation for classic Hirschsprung's disease has become increasingly popular during the last few years. The procedure leaves no scars, is associated with less postoperative pain and discomfort and shortens hospital stay. The most commonly used technique for transanal pull-through is long endorectal dissection leaving a long muscular cuff, which is usually split posteriorly. We present our preliminary results following transanal endorectal operations with a short unsplit muscular cuff. MATERIALS AND METHODS: Twenty-six patients underwent short-cuff transanal endorectal operation for Hirschsprung's disease between years 2000 and 2002. Patients' hospital records were analysed retrospectively. The collected data included age at operation, associated conditions, hospital stay and time to full enteral feeding, occurrence of pre- and postoperative enterocolitis and preoperative stoma, operative complications, need for postoperative anal dilatations, postoperative perianal skin problems and preliminary data on bowel function. RESULTS: The median age at operation was 1 month (range 0 - 60 months), 13 patients were operated on in the neonatal period. Four patients had Down's syndrome, 1 had cartilage-hair hypoplasia and one Ondine's syndrome. Five patients had preoperative enterocolitis. Four patients had undergone levelling stoma formation for unremitting constipation or enterocolitis. The proximal ganglionic stoma was concomitantly pulled-through and anastomosed to the anus in all 4 patients with a stoma. One patient with aganglionosis extending to the proximal sigmoid required additional laparoscopic colonic mobilisation. Complications related to surgery did not occur in the present series. Median postoperative hospital stay was 3 days (range 2 - 21 days) and median time to full enteral feeding was 3 days (range 1 - 14). Six patients required anal dilatations, two of those for a period of 3 weeks. The median follow-up time was 6 months (range 1 - 22 months). Fourteen patients had perianal skin rash, which usually resolved within 6 weeks. Postoperative enterocolitis requiring hospitalisation occurred in 1 patient, another patient had mild symptoms suggesting enterocolitis; these responded to oral antibiotic treatment. Early postoperative bowel function was characterised by frequent bowel movements in most patients. This usually resolved within a few months; of the 15 patients with a follow-up of longer than 6 months only 2 have more than 3 bowel movements per day. At the last follow-up frank soiling occurred in 1 patient with Down's syndrome, one patient requires oral laxatives for constipation. CONCLUSIONS: Transanal endorectal pull-through with a short cuff is a safe operation with a low incidence of operative and postoperative complications. Hospital stay and time to full enteral feeds is significantly shorter than after conventional procedures; this is associated with lower hospital costs. Long-term functional outcome is unclear but short-term function is very similar to that after procedures where transanal mucosectomy is combined with open rectosigmoid dissection.     

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。     

单纯腹腔镜监视下经肛门直肠拖出次全结肠切除术

目的 总结单纯腹腔镜监视下经肛门直肠拖出次全结肠切除术治疗先天性巨结肠及其同源病的临床经验并评价其可行性和安全性.方法 2010年3月至12月,8例先天性长段型巨结肠及其同源病行腹腔镜Soave手术.作者改进这种技术.在单纯腹腔镜监视下通过肛门直肠肌鞘入路游离全部结肠.然后拖出体外完成次全结肠切除术.结果 全部操作均顺利完成,6例单一腹腔镜经脐部放置,2例经关闭剥离结肠造口后的小切口导入.手术时间155～240min,平均(178±23)min,术后1～2 d恢复肠蠕动.1例因小肠梗阻开腹探查.随访2～10个月大便次数由术后近期的每天5～15次降至2个月后的3～5次,临床效果良好,无大便失禁或便秘复发.结论 单纯腹腔镜监视下经肛门拖出次全结肠切除术安全、有效、可行,使手术创伤更小,经脐单孔腔镜手术可达到NOTES的美观效果.

Abstract：

Objective To summarize our experience and assess the feasibility and safety of transanal endorectal pull-through and laparoscopic subtotal colectomy for Hirschsprung's disease and allied disorder (HAD). Methods From March to December 2010,8 infants and children with long segment Hirschsprung's disease or/and allied disorder underwent Soave pull-through procedures under laparoscopic guidance. We modified this technique by mobilizing the whole colon via rectal muscular sleeve approach under laparoscopic vision, then transanal endorectal pull-through to complete the subtotal colectomy. Results All procedures were completed successfully. Single-port access laparoscope was introduced through the umbilicus in 6 cases and through a small incision after mobilized colostomy in 2 cases. The average operative time was 178 ± 23 min (range 155 to 240 min). The period of the full bowel action was 1-2 day. One infant developed postoperative intestinal obstruction that required open exploration. They were followed-up for 6 months (range 2-10 months). The bowel movement frequency was reduced from 5-15 per day postoperatively to 3-5 per day after 2 months. The clinical outcomes were excellent with no stool incontinence or constipation. Conclusions Transanal Soave pull-through subtotal colectomy with simple laparoscopic guidance is a safe, effective and feasible procedure, with minimal invasion,and good cosmetic result.     

Transanal coloanal anastomosis for Hirschsprung's disease: comparison between endorectal and perirectal pull-through procedures.

BACKGROUND/PURPOSE: The aim of this study was to compare the results of 2 procedures of transanal pull-through for the management of rectosigmoid Hirschsprung's disease. METHODS: Twenty-one consecutive children with rectal or rectosigmoid Hirschsprung's disease were operated on between November 1999 and April 2003, in two pediatric surgical departments (Dijon and Strasbourg). Twelve children underwent a transanal perirectal pull-through procedure (TPR) and 9 had a transanal endorectal (Soave) pull-through procedure (TER). The collected data in each group included demographic data, length of aganglionosis, age and weight at operation, operating time, duration of hospital stay, incidence of postoperative complications (sepsis, enterocolitis, stricture) and quality of fecal continence on long-term follow-up. RESULTS: No significant differences were observed between the TPR and TER groups with respect to mean age at presentation, length of aganglionosis (rectosigmoid in 10/12 and 8/9 patients respectively), age at operation, with seventeen children operated on before one year of age (mean 3.8 and 3.3 months, respectively) and duration of hospital stay (5.2 vs. 5.3 days), frequency of bowel movements at 3 months postoperatively (1 - 3 per day). Mild differences were observed between TPR and TER groups for gender (ratio M : F 5 : 1 vs. 2 : 1), gestational age at term (39 vs. 37.5 weeks), birth weight (3240 g vs. 2520 g) and operating time (116 min vs. 138 min). No iatrogenic injury of the surrounding pelvic structures occurred during surgery and no blood transfusion was required in either of the groups. A retrorectal pelvic abscess was found in one child of the TPR group. It resolved after an enterostomy had been performed with parenteral antibiotics. Anal dilatation for postoperative anorectal stricture was required in 3 and 2 patients, respectively, for the TPR and TER groups. A mild postoperative enterocolitis developed in one case in the TER group. The average follow-up period was 35.3 months, but ten children still wear diapers, making a functional evaluation difficult. Constipation was noted in 4 and 3 patients, respectively, for the TPR and TER groups. No permanent soiling has been noted at long-term follow-up. CONCLUSION: As an objective assessment of fecal continence could not yet be done for this short series, further follow-up is required. Up to now, no significant difference was observed between these two transanal pull-through procedures.     

Transanale Operationstechnik beim Morbus Hirschsprung

Background. In 70–80% Hirschsprung's disease is limited to the distal sigmoid colon. We present a multi-center study of a new transanal operating technique and report the related results and limitations. Patients. In a 2 year period 12 patients with Hirschsprung's disease were operated by a transanal approach.The follow-up period is 6 to 29 months. Methods. In all children the operation was performed transanally.An additional laproscopy was neccessary three times.A laparotomy was neccessary in one case due to post inflammatory adhaesions.One abscess formation required a temporary colostomy. All children were fed at the first post op day.Continence and stool frequency is normal. Discussion. The results with this new technique are very convincing and promising.We believe that this transanal technique will be the first choice in the majority of patients with Hirschsprung's disease in the future. Our experiences are comparable to those published by other groups.     

杂交经脐单孔腹腔镜直肠内拖出术治疗先天性巨结肠症

目的探讨杂交单孔腹腔镜直肠内拖出术(H-SILEP)治疗先天性巨结肠症(HD)术中、术后并发症的发生率、美容效果和临床疗效。方法自2011年9月至2014年10月,我们对39例HD患儿(男性26例,女性13例;平均年龄4.2个月)采用H-SILEP术,经脐部置入2个5 mm Trocar,左侧腹免Trocar置入1个3 mm操作钳进行浆肌层活检和结肠系膜游离,经肛门分离黏膜,拖出正常肠管吻合。观察手术时间、出血量,术中、术后并发症,腹壁瘢痕及临床疗效。结果 39例均顺利完成手术,11例移行区位于直肠,20例位于乙状结肠,8例位于降结肠。手术时间(115±16)min,术中出血(5.0±1.2)mL,无一例术中并发症。术后10例出现肛周皮肤破溃;无吻合口瘘;3例发生小肠结肠炎,经保守治疗痊愈。随访6个月至3年,腹壁几乎无可见瘢痕,无便秘复发。结论 H-SILEP治疗HD安全、可行,操作与常规腹腔镜手术相似,美容效果可媲美经脐单孔手术。     

Endorectal pull-through operation for diffuse cavernous hemangiomatosis of the sigmoid colon, rectum and anus.

A 6-year-old girl with diffuse cavernous hemangiomatosis of the sigmoid colon, rectum and anus underwent endorectal pull-through operation for sphincter-saving resection. Rectal mucosa was resected from 4 cm above peritoneal reflection to anal skin margin and the normal sigmoid colon was pulled down through the rectal muscular cuff. Ligation of the superior rectal and left internal iliac arteries at operation achieved satisfactory control of bleeding. Postoperative manometric studies showed almost normal sphincter tone and good response to rectal stimuli by balloon inflation. The endorectal pull-through (Soave-Denda procedure) is a common procedure for Hirschsprung's disease and the best procedure for the sphincter-saving treatment of diffuse cavernous hemangiomatosis of the colon and rectum.     

Laparoscopic-assisted approaches for the definitive surgery for Hirschsprung's disease

The surgical management of Hirschsprung's disease has progressed from a two- or three-stage procedure to a primary operation over the last 25 years. More recently, definitive surgery for Hirschsprung's disease has been performed using minimally invasive techniques. The Swenson, Duhamel and Soave endorectal pull-through procedures have all been reported using minimally invasive approaches. The endorectal dissection has become the dominant minimal access procedure because of the ease and reliability in performing this technique and the excellent results obtained. Although a transanal endorectal pull-through can be performed without laparoscopy, the laparoscopic-assisted transanal endorectal pull-through is a much more versatile technique and allows early biopsies to determine the extent of aganglionic and dysfunctional bowel before ablation of the rectum and mesocolon. The authors use a laparoscopic-assisted transanal pull-through for aganglionosis of the left and transverse colon. Total colon aganglionosis or aganglionosis of the ascending colon is managed by a laparoscopic-assisted Duhamel procedure which provides a better reservoir in patients with a short or absent colon.