先天性巨结肠根治术后医源性直肠阴道瘘的再手术治疗CSCD

Objective To explore the causes of iatrogenic rectovaginal fistula after pull-through in Hirschsprung’s disease (HD) and summarize the experiences and efficacies of reoperation. Methods From December 2007 to December 2020 , retrospective review was conducted for clinical data of 6 HD children with iatrogenic rectovaginal fistula after pull-through. The surgical procedures for repairing rectovaginal fistula included transabdominal and transanal Soave , transperineal or transanal surgery. Results A total of six girls were included. Clinical manifestation was vaginal excretion. Injuries occurred in initial surgery (n =4) and during redo pull-through (n =2). The reasons for re-operation were postoperative abdominal hemorrhage and aganglionic segment residue. Rectovaginal fistula with anastomotic retraction and stenosis were successfully repaired by transabdominal and transanal Soave procedure in single time(n =4). The remaining two cases underwent simple local repair of rectovaginal fistula , including fistula closure after anal repair twice (n =1) and failed closure after five local (perineal/anal) repairs (n =1). Conclusion Rectovaginal fistula after pull-through in HD is a serious iatrogenic injury. It should be separated close to rectal submucosa/rectal wall to avoid vaginal injury. Transabdominal and transanal Soave procedure has a high success rate for repairing rectovaginal fistula and managing anastomotic retraction and stenosis. © 2022, Journal of Clinical Pediatric Surgery. All rights reserved.     

先天性肛门直肠畸形术后便秘合并巨结肠的临床特点及处理

目的 总结先天性肛门直肠畸形术后顽固性便秘合并巨结肠的临床特点和治疗效果.方法 回顾性分析2005年8月至2010年7月收治的顽固性便秘合并巨结肠23例临床资料,男10例,女13例.首次手术年龄3 d至6岁,术前诊断均为中低位肛门直肠畸形.23例患儿均以肛门成形术后便秘就诊.下消化道气钡造影显示下端结肠及直肠扩张,无移行段,排钡延迟.本次手术年龄1岁2个月至15岁8个月.均采用Soave巨结肠根治术.结果 术前见肛门外观大致正常13例,外观正常合并直肠尿道瘘1例,肛门开口位置前移6例,瘢痕回缩2例,肛门外口狭窄1例.术中进入盆腔后即为扩张肠管,系膜增生增厚,肠壁血管增生粗大.13例单纯经会阴手术,10例经腹会阴手术,其中1例同时回肠末端造瘘.9例巨结肠切除同时,再次行肛门成形术,1例巨结肠切除同时行前矢状入路直肠尿道瘘修补术.病理检查15例切除肠管远近段,可见神经节细胞者,8例肠管远端无神经节细胞者,23例均见肌层增生肥厚,肌纤维变性,肌层排列紊乱.二次术后随诊肛门功能李氏评分5～6分.结论 先天性肛门直肠畸形术后顽固性便秘往往并发巨结肠改变,影像显示为肠管局限性扩张、僵硬和动力紊乱.采取手术治疗,切除病变肠管,做必要的肛门解剖缺陷修复,术后效果良好.

Abstract：

Objective To summarize the experience of diagnosis and treatment of intractable constipation in children with repaired anorectal malformation.Methods Between August 2005 and July 2010,23 children with a history of anorectal malformation (ARM) repair were diagnosed with intractable constipation,and underwent surgical treatment at this center.The patients,including 10 boys and 13 girls,underwent primary surgeries to repair low or intermediate type of ARMs.Their ages at the primary surgery ranged from 3 days to 6 years old.Physical examinations found normal appearance of anus on 14 children including 1 with rectourethral fistula,anus antelocation on 6,cicatricial retraction of anus on 2,and anal stricture on 1.The positive barium enema revealed the dilated distal colon and rectum without transition zones,and delayed barium transit time.The patients' age at the second surgery ranged from 1 year and 2 months to 15 years and 8 months old.Results Dilated distal colon and rectum,with increased intestinal wall and mesentery thickness as well as proliferated blood vessels,was found during surgery on all patients.However,no transition zone was observed.To remove the dilated intestine,transanal Soave procedure was performed on 13 children,and the other 10 underwent abdomino-perineal Soave procedure including 1 had additional ileostomy.Besides the megacolon resection,9 patients also underwent anoplasty,and rectourethral fistula was also repaired on 1 patient.At the distal end of the removed intestine,pathological examination revealed ganglion cells in 15 patients,and no ganglion cells in 8 patients.Hypertrophy,degeneration and derangement of the intestinal smooth muscle cells were also observed.After reoperation,patients' anal function was graded as 5 or 6 according to Lizheng's anal function scoring criteria.Conclusions Intractable constipation after ARM repair is usually caused by acquired megacolon.It can be cured by megacolon resection.     

先天性巨结肠Soave术后大便失禁的评价和治疗

目的 对先天性巨结肠Soave术后大便失禁进行评价和治疗.方法 先天性巨结肠Soave术后大便失禁患儿24例,男14例,女10例,年龄6～13岁,肛门功能临床评价为良19例,差5例.全部患儿行肛门直肠测压,并与18例肛门功能正常的Soave术后儿童比较.所有患儿在医院接受生物反馈训练2周后自行在家中行盆底肌收缩训练.结果 肛管静息压、收缩压、直肠初感觉分别为(18.9±6.2)mmHg、(179.9±17.8)mmHg、(45.4±9.4)ml,与18例对照组儿童相比[分别为(44.5±11.1)mmHg、(177.7±15.9)mmHg、(50.0±10.1)ml],静息压明显下降,收缩压、直肠感觉无明显变化.3例不配合治疗,21例1年后除5例未能坚持外,其余16例获得良好的肛门控制,肛管静息压、肛管收缩压、直肠初感觉分别为(35.4±8.7)mmHg、(195.3±15.0)mmHg、(45.9±8.4)ml.肛管静息压和收缩压均升高.结论 内括约肌损伤可能是先天性巨结肠Soave术后大便失禁的原因之一,对此盆底肌训练可取得满意的治疗效果.

Abstract：

Objective To evaluate the efficacy of pelvic floor muscle exercise for the treatment of fecal incontinence after Soave procedure for Hirschsprung's disease. Methods Twenty four patients who had Soave procedure for Hirschsprung's disease and presented fecal incontinence after surgery were recruited in this study. They were 14 males and 10 females, aged from 6 to 13 years old. Eighteen children who had normal defecation after Soave procedure were selected as controls. All the subjects had manometry to measure resting anal canal pressure, squeeze pressure, and rectal sensation.All the patients had two weeks biofeedback therapy during their stay in hospital. After being discharged, they were taught and prescribed to do pelvic floor muscle exercise at home. Results Of the children with fecal incontinence, the resting anal canal pressure, squeeze pressure and rectal sensation were ( 18. 9 ± 6. 2 ) mmHg, ( 179. 9 ± 17. 8) mmHg, (45. 4 ± 9. 4) ml, respectively. Compared with the controls, the resting anal canal pressure was lower [(18. 9±6. 2) mmHg vs (44. 5 ± 11.1) mmHg].But no difference of squeeze pressure and rectal sensation was found between the 2 groups [(179. 9 ±17. 8)mmHg vs (177. 7 ± 15. 9) mmHg, (45.4 ± 9. 4)ml vs (50. 0 ± 10. 1)ml]. Three patients dropped out of the therapy. Five patients stopped the exercise after the first year. The other 21 who kept exercise improved their defecation function. Their resting anal canal pressure, squeeze pressure and rectal sensation were (35. 4 ± 8. 7)mmHg, (195. 3 ± 15.0)mmHg and (45. 9 ± 8. 4)ml, respectively. Conclusions The dysfunction of internal anal sphincter might be the main cause for fecal incontinence after Soave procedure. Pelvic floor muscle training is helpful to improve the defecation function of the patients.     

Therapeutic laparoscopy for superior mesenteric artery syndrome in 5 children: Treatment experience and lite-rature review北大核心CSCD

Objective To investigate the clinical characteristics and therapeutic laparoscopy treatment for superior mesenteric artery syndrome(SMAS) in children. Methods A retrospective study on 5 children with SMAS who failed to respond to the conservative treatment in the Second Hospital of Shandong University from March 2017 to March 2022 was carried out.All the patients underwent the laparoscopic lysis of the ligament of Treitz and duodenal lowering procedure.The clinical data were analyzed and literature retrieved from Wanfang and PubMed databases were reviewed. Results There were 3 boys and 2 girls in the 5 cases with SMAS.The average age was (12.4±1.4) years, and the preoperative body mass index was (15.2±0.8) kg/m2.The patients were admitted to the hospital with vomiting, abdominal pain and emaciation symptoms, and the duration of symptoms was (8.4±7.8) months.Gastroscopy, laparoscopic lysis of the ligament of Treitz and duodenal lowering procedure were performed successfully in all the patients, without conversions to open surgery.The average operation duration was (66.0±10.7) min.Food intake was allowed 1 day after surgery.Chylous fistula occurred 4 days after operation in one patient, whose symptom relieved after 11 days of conservative treatment.One patient still had nausea and vomiting symptoms 10 days after operation and was healed by knee-chest posture treatment.No other short-term complications were observed.The median postoperative duration of stay in the hospital was 7 (6-22) days.The patients were followed up for 9-56 (median: 21) months.All of them recovered well and gained weight.One patient had mild recurrent symptoms, and was cured after conservative treatment.A total of 15 literature on the treatment of SMAS by laparoscopic lysis of the ligament of Treitz was retrieved, and the cure rate was 40%-100%.Only one group of these cases had a cure rate below 75%. Conclusions The laparoscopic lysis of the ligament of Treitz and duodenal lowering procedure is safe, effective, simple and minimally invasive treatment for children with SMAS.It can be used as the first choice for most pediatric patients. © 2022 Chinese Journal of Applied Clinical Pediatrics. All rights reserved.     

经脐单切口腹腔镜脾切除术

目的 总结儿童经脐单切口腹腔镜脾切除术的初步经验,并探讨其可行性和安全性.方法 回顾分析2010年4月至2011年1月期间5例脾相关疾病儿童经脐单切口入路腹腔镜脾切除术的临床资料,男3例,女2例,年龄4～12岁,包括遗传性球形红细胞增多症3例和特发性血小板减少性紫癜2例.我们使用自行改进的三通道套管和弯曲器械,超声刀切断脾周围韧带,切割钉合器或生物夹处理脾蒂,脾脏从单切口取出.结果 5例腹腔镜脾切除术均经脐单切口入路获得成功,没有中转常规腹腔镜手术.手术时间125～220 min,估计术中出血量10～30 ml,无围手术期并发症,术后5～8 d痊愈出院.随访3～12个月,5例患儿均生长发育良好,贫血得到纠正,血小板计数正常.结论 经脐单切口腹腔镜脾切除术较常规腹腔镜脾切除术对腹壁创伤更小、美容效果更好,可在儿童安全实施;但需要更多数量和进一步前瞻性研究来评价其真正益处.

Abstract：

Objective To review our initial experience of transumbilical single incision laparoscopic splenectomy in children and to assess the feasibility and safety of this laparoscopic procedures.Methods Clinical data of 5 children who underwent laparoscopic splenectomy via a single umbilical incision between April 2010 and January 2011 were reviewed. There were 3 boys and 2 girls. The age ranged from 4 to 12 years. Of them,3 cases suffered from hereditary spherocytosis and 2 cases from idiopathic thrombocytopenic purpura. We used the Tri-port and self-modified curved instruments. The splenic attachments were taken down using the harmonic scalpel,and the hilum was clipped with Hemo-lok or transected with Endo-GIA. The spleen was removed from the abdomen via the single incision.Results All of the laparoscopic splenectomies were completed successfully with transumbilical single incision approach without conversion to conventional laparoscopic surgery. The operation time ranged from 125 to 220 min and the estimated blood loss was 10-30ml. There were no perioperative complications. All patients recovered that the postoperative hospital stay was 5-8 days. During a follow-up period of 3 to 12 months,all children were very well with improved growth and normal hemotology. Conclusions Compared with standard laparoscopic splenectomy, transumbilical single incision laparoscopic splenectomy may reduce surgical trauma,and improve cosmesis. A large prospective trial will be necessary to assess the true benefit of this approach.     

先天性巨结肠同源病:临床诊断与手术指征的确立

Objective To determine the diagnostic criteria and surgical indications of allied diseases of Hirschsprung's disease (HAD) in children. Methods During the period from Jun. 2007 to Dec. 2009,65 consecutive patients were performed "radical surgery for megacolone" by this surgery team. Based on the clinical symptoms,anorectal manometry, AChE staining of rectal mucosa and barium enema,the preoperative suspected diagnosis of Hirschsprung's disease (HD) or HAD was made.Surgical indications were determined once the suspected HD diagnose was made. On the patients withsuspected HAD, strictly conservative treatments should be carried out for at least 3 months. If conservative treatments were ineffective or the symptoms were recurrent, surgical indication of HAD patients could be determined. Results The preoperative diagnosis was 38 patients with HD and 27 with HAD.However, the final pathologic diagnosis was 36 with HD and 29 with HAD. The misjudged preoperative diagnosis was made in 10. 5% (4/38) HD patients and 7. 4% (2/27) HAD patients. The HAD patients included 22 (75. 9%) hypoganglionosis (HG) cases and 7 (21.1 %) intestinal neuronal dysplasia (IND) cases. Among the 27 HAD patients, 27 (93. 1%) had anorectal inhibitory reflex, and varied and delayed reflection waveforms. AChE staining was positive in 12 (41.1%) patients. The results of anorectal manometry and AChE staining of the HAD patients were significantly different from those of the HD patients (P＜0. 01 ). Barium enema of the HAD patients appeared severe expanded rectum which occupied more than 90% space of the pelvic cavity. Redundant sigmoid colon was also found in more than 90% patients. All HAD patients had 24-hour barium retention. Subtotal colectomies were performed on 9 (31. 0%) HAD patients,and 8 (22. 2%) HD patients. Conclusions Anorectal manometry, AChE staining and barium enema are still important in determining the diagnosis and surgical indications for HAD. The efficacy of 3 months or longer conservative treatment is crucial to determine the surgical indications for HAD.     

腹腔镜下十二指肠吻合术治疗新生儿十二指肠梗阻

Objective To evaluate the feasibility and indication of laparoscopie duodenoduoden-ostorny for neonates with congenital duodenal obstruction- Methods From May 2004 to Feburary 2008,6 newborns with duodenal obstruction underwent exploratory laparoscopy. With a lower-pressure pneumoperitoneum of 5～8 mmHg and a suspending suture for right liver elevator, the procedure was performed using 3 cannulas of 3.3 mm to 5.5 mm diameter. Under the laparoscopic vision, the cause of duodenal obstruction was diagnosed and a sutured anastomosis was performed after the duodenum mo-bilized. Results Findings at laparoscopy included duodenal diaphragm in 3 cases,annular pancreas in 2 cases, and preduodenal portal vein in 1 case. Three cases with duodenal diaphragmatic stenosis were en-countered a partial excision of the diaphragm after vertical incision of the anterior part of duodenum followed laparoscopically by a transverse suture. A diamond-shaped side-to-side duodenoduodenal anas-tomosis was successfully carried out in 2 cases of annular pancreas through a laparoseopic approach, but a duodenojejunostomy was converted to mini-laparotomy during the laparoscopic course of a predu-odenal portal vein. The average operative time was 102 16.5 min (85～135 min). Visualization was ex-cellent, and there were no intraoperative complications. Feedings were started on postoperative day 3 to 5. All cases were on full feedings after 8 to 10 days. Follow-up upper gastrointestinal tests showed no evidence of stricture or obstruction. Conclusions The duodenoduodenostomy with laparoseopy can be performed in neonates securely and appropriated for a full-term newborn with tolerance CO2 pneumo-peritoneum. It provides an excellent and micro-invasive way to evaluate and treat congenital duodenal obstruction.     

腹腔镜下十二指肠吻合术治疗新生儿十二指肠梗阻

Objective To evaluate the feasibility and indication of laparoscopie duodenoduoden-ostorny for neonates with congenital duodenal obstruction- Methods From May 2004 to Feburary 2008,6 newborns with duodenal obstruction underwent exploratory laparoscopy. With a lower-pressure pneumoperitoneum of 5～8 mmHg and a suspending suture for right liver elevator, the procedure was performed using 3 cannulas of 3.3 mm to 5.5 mm diameter. Under the laparoscopic vision, the cause of duodenal obstruction was diagnosed and a sutured anastomosis was performed after the duodenum mo-bilized. Results Findings at laparoscopy included duodenal diaphragm in 3 cases,annular pancreas in 2 cases, and preduodenal portal vein in 1 case. Three cases with duodenal diaphragmatic stenosis were en-countered a partial excision of the diaphragm after vertical incision of the anterior part of duodenum followed laparoscopically by a transverse suture. A diamond-shaped side-to-side duodenoduodenal anas-tomosis was successfully carried out in 2 cases of annular pancreas through a laparoseopic approach, but a duodenojejunostomy was converted to mini-laparotomy during the laparoscopic course of a predu-odenal portal vein. The average operative time was 102 16.5 min (85～135 min). Visualization was ex-cellent, and there were no intraoperative complications. Feedings were started on postoperative day 3 to 5. All cases were on full feedings after 8 to 10 days. Follow-up upper gastrointestinal tests showed no evidence of stricture or obstruction. Conclusions The duodenoduodenostomy with laparoseopy can be performed in neonates securely and appropriated for a full-term newborn with tolerance CO2 pneumo-peritoneum. It provides an excellent and micro-invasive way to evaluate and treat congenital duodenal obstruction.     

腹腔镜下十二指肠吻合术治疗新生儿十二指肠梗阻

Objective To evaluate the feasibility and indication of laparoscopie duodenoduoden-ostorny for neonates with congenital duodenal obstruction- Methods From May 2004 to Feburary 2008,6 newborns with duodenal obstruction underwent exploratory laparoscopy. With a lower-pressure pneumoperitoneum of 5～8 mmHg and a suspending suture for right liver elevator, the procedure was performed using 3 cannulas of 3.3 mm to 5.5 mm diameter. Under the laparoscopic vision, the cause of duodenal obstruction was diagnosed and a sutured anastomosis was performed after the duodenum mo-bilized. Results Findings at laparoscopy included duodenal diaphragm in 3 cases,annular pancreas in 2 cases, and preduodenal portal vein in 1 case. Three cases with duodenal diaphragmatic stenosis were en-countered a partial excision of the diaphragm after vertical incision of the anterior part of duodenum followed laparoscopically by a transverse suture. A diamond-shaped side-to-side duodenoduodenal anas-tomosis was successfully carried out in 2 cases of annular pancreas through a laparoseopic approach, but a duodenojejunostomy was converted to mini-laparotomy during the laparoscopic course of a predu-odenal portal vein. The average operative time was 102 16.5 min (85～135 min). Visualization was ex-cellent, and there were no intraoperative complications. Feedings were started on postoperative day 3 to 5. All cases were on full feedings after 8 to 10 days. Follow-up upper gastrointestinal tests showed no evidence of stricture or obstruction. Conclusions The duodenoduodenostomy with laparoseopy can be performed in neonates securely and appropriated for a full-term newborn with tolerance CO2 pneumo-peritoneum. It provides an excellent and micro-invasive way to evaluate and treat congenital duodenal obstruction.     

腹腔镜下十二指肠吻合术治疗新生儿十二指肠梗阻

Objective To evaluate the feasibility and indication of laparoscopie duodenoduoden-ostorny for neonates with congenital duodenal obstruction- Methods From May 2004 to Feburary 2008,6 newborns with duodenal obstruction underwent exploratory laparoscopy. With a lower-pressure pneumoperitoneum of 5～8 mmHg and a suspending suture for right liver elevator, the procedure was performed using 3 cannulas of 3.3 mm to 5.5 mm diameter. Under the laparoscopic vision, the cause of duodenal obstruction was diagnosed and a sutured anastomosis was performed after the duodenum mo-bilized. Results Findings at laparoscopy included duodenal diaphragm in 3 cases,annular pancreas in 2 cases, and preduodenal portal vein in 1 case. Three cases with duodenal diaphragmatic stenosis were en-countered a partial excision of the diaphragm after vertical incision of the anterior part of duodenum followed laparoscopically by a transverse suture. A diamond-shaped side-to-side duodenoduodenal anas-tomosis was successfully carried out in 2 cases of annular pancreas through a laparoseopic approach, but a duodenojejunostomy was converted to mini-laparotomy during the laparoscopic course of a predu-odenal portal vein. The average operative time was 102 16.5 min (85～135 min). Visualization was ex-cellent, and there were no intraoperative complications. Feedings were started on postoperative day 3 to 5. All cases were on full feedings after 8 to 10 days. Follow-up upper gastrointestinal tests showed no evidence of stricture or obstruction. Conclusions The duodenoduodenostomy with laparoseopy can be performed in neonates securely and appropriated for a full-term newborn with tolerance CO2 pneumo-peritoneum. It provides an excellent and micro-invasive way to evaluate and treat congenital duodenal obstruction.     

腹腔镜经脐及肛门自然腔道巨结肠根治术临床分析

目的 探讨经脐单孔腹腔镜监视下联合经肛门直肠内拖出结肠切除术治疗先天性巨结肠及其类缘性疾病的可行性和临床疗效.方法 2010年3～11月,对15例常见型和长段型先天性巨结肠及其类缘病患儿实施经脐单孔腹腔镜监视下联合经肛门直肠肌鞘入路游离左半结肠或全部结肠,然后拖出在体外完成直肠乙状结肠或次全结肠切除术.结果 全部患儿...     

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。     

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.     

肠无神经节细胞症患儿术后肛门直肠测压的应用研究

目的：探讨经肛门 Soave 巨结肠根治术后患儿排便功能及直肠肛管测压的应用价值。方法回顾性分析经术后病理检查证实的61例肠无神经节细胞症（Hirschsprung＇s disease,HD）患儿临床资料,均行经肛门 Soave 巨结肠根治术,其中腹腔镜辅助手术16例。术后平均随访1年（3个月至2年）,根据手术年龄分为婴儿组（0～1岁）及幼儿组（1岁以上）。对患儿术后大便控制能力、并发症情况及肛门直肠测压结果进行比较。结果婴儿组术后排便控制情况与幼儿组比较,差异无统计学意义（P ＞0.05）。肛门直肠测压检查显示术前直肠肛管抑制反射总阴性率为95.1％（58／61）,术后均未恢复;两组直肠静息压、肛管静息压比较,差异无统计学意义（P ＞0.05）。结论经肛门 Soave 巨结肠根治术治疗小儿肠无神经节细胞症,术后可获得良好的排便控制功能,可作为首选术式;肛门直肠测压的术前诊断价值较高,术后短期随访作用有限。     

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.     

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.     

腹腔镜下结肠次全切除术治疗B型肠神经元发育不良长肌鞘吻合与短肌鞘吻合的对照研究

目的探讨B型肠神经元发育不良（IND—B）患儿,行腹腔镜下结肠次全切除术,肛门结肠吻合时保留肌鞘的长短与早期及晚期手术效果的关系。方法自2011年8月至2013年1月间,选择病理检查结果为B型肠神经元发育不良,病变肠管在降结肠以远,需行结肠次全切除术的患儿为研究对象。其中,接受长肌鞘吻合的患儿人选长肌鞘组（28例）,接受短肌鞘吻合的患儿（26例）作为对照组。分析两组病例的住院时间、手术并发症及排便功能方面的差异。结果术后并发症中,肛周湿疹、吻合口瘘、便秘复发、污便在两组中发生率相当。小肠结肠炎在长肌鞘组的发生率为3．5％（1／28）,显著低于短肌鞘组（19．2％,5／26）。长肌鞘组发生肌鞘翻转1例。手术后2个月内,两组排便次数无显著性差异。术后3个月及6个月随访,长肌鞘组平均排便频率明显低于短肌鞘组。术后12个月及18个月随访,两组排便功能优良率无显著性差异。结论B型肠神经元发育不良患儿腹腔镜下结肠次全切除行长肌鞘吻合者,术后小肠结肠炎的风险较短肌鞘吻合者显著降低,且术后近期排便控制优于短肌鞘吻合者,而术后中远期疗效比较,两种吻合方式的排便控制能力相当。     

腹腔镜下改良Soave术治疗婴幼儿先天性巨结肠20例

目的总结腹腔镜下改良Soave术治疗婴幼儿先天性巨结肠(HD)的手术方法、操作经验和疗效。方法对婴幼儿HD患儿20例(男16例,女4例;年龄3.5个月-4岁)采用气管插管全身麻醉,腹腔镜下应用超声刀切断拖出段结肠系膜,会阴部采用改良Soave术完成手术。结果病例均顺利完成手术,平均手术时间132 min,平均切除结肠肠管长度35 cm,平均术中出血14 mL,均未输血。术后发生小肠结肠炎2例。随访6个月-3年,平均14.5个月,无便秘、污粪、肠梗阻、肛门黏膜外翻、吻合口狭窄等并发症发生,排便控制在1-2次/d。结论腹腔镜下改良Soave术治疗婴幼儿HD具有创伤小、出血少、操作简便、恢复快、并发症少等优点。     

经肛门Soave巨结肠根治术后肛门功能与直肠肛管测压的研究

目的探讨经肛门Soave巨结肠根治术后患儿直肠肛管测压改变及排便控制情况。方法63例经肛门Soave巨结肠根治术患儿,术后平均随访3年(6个月～4.5年),对患儿大便控制能力、便秘发生情况及肛门直肠测压结果进行评价。结果获访47例,大便控制优15例,良26例,差6例。2例患儿有持续性便秘,8例曾出现肠炎。肛门直肠测压检查2例恢复直肠肛管抑制反射。47例随访患者的直肠静息压与正常对照组无显著性差别(P>0.05),但6例大便控制差的患者肛管高压区长度较正常对照组下降(P<0.05)。结论应用经肛门Soave巨结肠根治术治疗小儿巨结肠,术后可获得较好的排便控制功能。少数病例排便功能障碍可能与内括约肌功能受损有关,直肠肛管测压是重要的评估依据之一。     

Subtotal colectomy with a single-incision laparoscopic surgery technique in children with long-segment Hirschsprung disease and allied disorders

Background

Over the last 15 years, the laparoscopic-assisted endorectal pull-through procedure has become the standard treatment for Hirschsprung disease in many centers around the world. Recently, single-incision laparoscopic techniques have drawn more attention. We describe a single-incision laparoscopic surgery (SILS) subtotal colectomy to treat long-segment Hirschsprung disease (LSHD) and Hirschsprung disease allied disorder (HAD) in children.

Methods

A total of 22 patients who underwent SILS subtotal colectomy, including three patients with a failed first surgery, were included in this retrospective study. For SILS, a 1-cm skin incision was first made below the umbilical margin and a 5-mm trocar was placed into the abdomen after incising the peritoneum. Two 5-mm trocars were then placed on both sides of the umbilicus. Subsequently, based upon preoperative examination and biopsy results, we performed subtotal colectomy. The affected colon was mobilized successively beyond the peritoneum using high-frequency cutting and sealing devices, followed by a pull-through procedure and colon-anal anastomosis.

Results

The average operative time was 206.39 min. No case needed conversion from SILS to either conventional laparoscopy or open surgery. Of the 22 patients, 15 were diagnosed as LSHD, while 6 cases were diagnosed with intestinal neuronal dysplasia and one was diagnosed with hypoganglionosis. There were no intra-operative complications. One child had incision dehiscence on postoperative day three. During the follow-up over 12 months, all patients were noted to have excellent cosmetic outcomes, and enterocolitis was observed in four children.

Conclusions

Subtotal colectomy with the SILS technique can be safely performed in LSHD or HAD patients in the pediatric population without major complications.