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。     

扩肛治疗在婴幼儿便秘及巨结肠根治术后的应用研究

目的评估扩肛治疗在婴幼儿便秘以及巨结肠根治术后处理中的疗效。方法对60例功能性便秘婴幼儿(为便秘治疗组)和60例经肛门巨结肠根治术后1个月的患儿(经肛门巨结肠根治术组)运用本院专利扩肛器分别行扩肛治疗,并进行排便功能问卷调查、结肠钡灌肠和肛门直肠测压检查,对扩肛前后肛肠功能进行综合评价。结果便秘治疗组60例经过扩肛治疗后,50例便秘消失(每日均排大便);8例便秘好转(偶有停止排便2~3 d,干预后恢复);2例无效;复查结肠钡灌排空延迟,RAIR阴性率、肛管蠕动波紊乱较扩肛前明显减少(P0.01)。经肛门巨结肠根治术(Soave)组60例于术后1个月行扩肛治疗,随访6个月后,42(70%)例排便次数逐渐减少至每日1~2次,复查钡灌排空延迟,RAIR阴性率、肛管蠕动波紊乱较扩肛前明显减少(P0.05)。结论扩肛治疗对于婴幼儿功能性便秘而言,可以建立反射,刺激便意;促进蠕动,缓解肛压;阻断肠神经元病变的继续发展。对于巨结肠根治术后患儿而言,可以扩开瘢痕,使排便通过无阻力;促进新建直肠顺应性恢复;促进直肠肛管抑制反射建立;促进蠕动,缓解肛压,减少肠炎发生,最终使肛管直肠排控便机制达到正常。     

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］     

经肛门Soave巨结肠根治术后排便功能的评价

目的 观察经肛门Soave巨结肠根治术后排便功能和结肠切端缩口缝合术的效果.方法 回顾性分析2003年10月至2008年10月130例经肛门Soave巨结肠根治术患儿的术后排便功能恢复情况. 结果 130例中98例行经肛门根治术,32例因长段型和直肠乙状结肠呈直筒状行腹腔镜协助经肛门根治术,其中15例因近端结肠切端扩张与肛门口径大小不符行结肠切端缩口缝合后再行肛门结肠吻合.130例术后电话和信件获访90例,远期并发症3例,其中污粪2例,便秘合并小肠结肠炎1例.对术后恢复时间达3年以上的56例患儿进行排便功能评分,评分为正常组有23例,优组31例,良组2例,无差组病例.行结肠切端缩口缝合术的15例患儿全部获访,排便功能评分正常组6例,优组9例,无并发症.结论 经肛门Soave巨结肠根治术后长期肛门排便功能恢复满意.扩张但功能良好的近端结肠切端管径大于肛门口径时,可行结肠切端缩口缝合术.结肠切端缩口缝合术疗效确切,可与腹腔镜技术协同成为经肛门巨结肠手术的辅助手段.     

经肛门改良Soave术根治婴幼儿巨结肠术后排便功能及生活质量随访

目的评价经肛门改良Soave术根治婴幼儿巨结肠术后排便控制功能及生活质量。方法对25例巨结肠婴幼儿行经肛门改良SoaveⅠ期根治。术后随访5个月~6年,平均4.5年,对大便控制能力、便秘、结肠炎发生率及生活质量进行评估。结果获访21例,大便控制评分4分(正常)16例,3分(好)4例,2分(欠佳)1例;便秘1例,结肠炎2例,生活质量优良率90.5%。结论经肛门改良Soave术根治婴幼儿巨结肠可获得良好的排便控制功能和生活质量。     

先天性巨结肠术后直肠肛管向量测压的研究

目的：应用直肠肛管向量测压技术评估先天性巨结肠患儿术后肛门括约肌功能。方法：利用直肛管向量测压技术，对42例先天性巨结肠患儿术后及21例正常儿进行肛门括约肌功能的评估。结果：根据临床症状将患儿分为污便组、便秘组和排便功能良好组。巨结肠患儿术后肛管静息压力及向量容积均显著低于正常儿（P＜0．01），污便组的静息压力及向量容积明显低于排便功能良好组，对称指数无明显变化，14．3％恢复了直肠肛门抑制反射。结论：先天性巨结肠患儿术后肛管最大压力及向量容积下降。直肠肛管向量测压技术是评估先天性巨结肠患儿术后肛门括约肌功能较客观全面的方法。     

经肛门Soave巨结肠根治术并发症的防治

目的探讨经肛门Soave巨结肠根治术后并发症的防治方法。方法回顾性分析2002年6月￣2005年12月我院56例经肛门Soave巨结肠根治术患儿的手术以及诊疗经过。结果56例巨结肠中44例获得成功,无并发症发生,12例出现并发症,其中小肠结肠炎2例,术后腹腔大出血1例,大网膜嵌顿导致结肠梗阻1例,直肠肌鞘感染1例,便秘1例,肛周红斑及糜烂7例,均经治疗痊愈,随访2个月￣3年,1例轻度粪污,其余患儿肛门无狭窄,每日排便1￣3次,生长发育正常。结论经肛门Soave巨结肠根治术具有创伤小,出血少,术后恢复快、无肠粘连等优点,预防和处理术后并发症是治疗的重要课题。     

腹腔镜改良Soave巨结肠根治术后排便功能的随访

目的　评价腹腔镜改良Soave根治术后患儿排便控制情况。方法　自 1999年 5月～2 0 0 1年 9月 ,对 6 4例年龄为 10d～ 1岁的先天性巨结肠患儿行腹腔镜改良Soave巨结肠根治术。术后定期随访患儿 ,平均随访时间为 (2 3.8± 1)个月 ,对患儿大便控制能力、便秘及结肠炎的发生率进行评价。结果　获访 4 3例患儿。大便控制评分正常 (10分 ) 2 2例 ,好 (6～ 9分 ) 18例 ,欠佳 (1～ 5分 )3例 ;1例患儿 (2 .3% )有持续便秘 ;5例 (11.6 % )患儿曾出现结肠炎。结论　应用腹腔镜改良Soave巨结肠根治术治疗新生儿及小婴儿先天性巨结肠 ,术后可获得良好的排便控制功能。     

经肛门改良Soave术和经腹手术治疗先天性巨结肠疗效比较

目的探讨经肛门改良Soave术和经腹手术治疗先天性巨结肠的临床疗效。方法2001～2007年作者收治年龄〉3岁的先天性巨结肠患儿41例，其中28例采取经肛门改良Soave术，24例经腹手术，分析两组患儿术后并发症以及排便功能。结果两组并发症的发生率比较，经肛门改良Soave术明显少于经腹手术，先天性巨结肠相关性肠炎（Himchsprung’s associatedentero colitis，HAEC）的发生率低于经腹手术，差异有统计学意义。两组排便控制能力以及大便形态方面比较，经肛门改良Soave术优于经腹手术。结论经肛门改良Soave术治疗先天性巨结肠安全可行，疗效优于经腹手术，但术后仍有诸多并发症，特别在排便控制方面，需要大宗病例长期随访的研究来评估。     

The impact of abnormal anoerectal movement and sensation on defecation function in children with transanal one-stage endorectal pull-through procedure for Hirschsprung's disease

目的 直肠肛门运动与感觉功能发生改变会影响到排便功能.本文通过分析经肛巨结肠根治术后患儿直肠肛门动力与感觉功能的客观检测指标变化,探讨其在排便控便中的作用.方法 采用高分辨率多通道胃肠功能检测系统,对经肛巨结肠根治术后有排便障碍来院就诊或能定期来院复查的37例患儿的临床排便功能评分和直肠肛门测压结果进行回顾性分析.结果 62.1％临床排便和测压指标正常,10.8％恢复了直肠肛门抑制反射.有排便障碍症状患儿中16.2％直肠肛门动力异常,24.3％直肠感觉阈值有改变,部分病例直肠肛门动力与感觉异常同时存在.结论 大多数经肛巨结肠根治术后患儿排便功能良好,直肠肛门动力与感觉功能趋于正常,括约肌功能完好.少数排便功能障碍的原因可能与直肠肛门动力及感觉功能异常有关.     

Bowel function and quality of life in adult patients with operated Hirschsprung's disease

Fecal continence and quality of life were evaluated by a questionnaire in 100 patients (mean age 31 years, 88 males, 12 females) who had undergone surgery for Hirschsprung's disease (HD) during the years 1950–1975. The operative methods were Duhamel retrorectal pull-through or modified Duhamel operation in 71 patients, Swenson rectosigmoidectomy in 20, State-Rehbein anterior resection in 5, and Soave endorectal pull-through in 4. The postoperative follow-up period ranged from 15 to 39 years. Fecal continence was assessed by a score described by Holschneider. Eighty-one healthy people with a similar age and sex distribution as the patients were used as controls. All controls and 91 patients had good fecal continence scores. Of the 9 patients who had fair scores, 3 were mentally retarded and 3 had postoperative anastomotic complications. If these 6 patients with an obvious cause for deficient anal function are excluded, there was no statistically significant difference in fecal continence between the patients and the controls. Equally good continence outcomes were achieved with all the operative methods. In adulthood, only 1 patient had chronic constipation. However, during childhood constipation was the main problem in 40 patients who developed a recurrent rectocolonic septum following the Duhamel operation. Constipation subsided after recrushing of the septum. None of the patients had urinary incontinence. All the patients with a good continence outcome reported no limitations in their occupation, social life, or physical activities. We conclude that in adulthood the fecal continence and quality of life of most patients with operated HD do not differ from those of healthy adults.     

Comparative review of functional outcomes post surgery for Hirschsprung’s disease utilizing the paediatric incontinence and constipation scoring system

Purpose

We aim to analyze differences in functional outcomes in children operated on for Hirschsprung’s disease (HD) using the Paediatric incontinence/constipation scoring system (PICSS) validated in a normative group.

Methods

A retrospective review of the records of all children operated on for HD between 1997 and 2010 was performed. Patients had either a Soave or transanal endorectal pull-through. Children with total colonic aganglionosis and Down’s syndrome were excluded. Utilizing the PICSS children who scored below their age-specific lower limit 95?% confidence interval PICSS scores were considered to have incomplete continence or constipation. The rates of incomplete continence and constipation were compared between groups. Significance was set at p?<?0.05.

Results

PICSS analysis could be completed in 51 (Soave 35, transanal 16). The median age at interview was 71?months (range 6–191?months). The rate of incomplete continence was 75?% (n?=?21) and 71?% (n?=?10) for the Soave and transanal groups, respectively (p?=?1.00). The constipation rate was 34?% (n?=?12) and 25?% (n?=?4) for the Soave and transanal groups, respectively (p?=?0.74). The overall rates of incomplete continence and constipation rates were 74 and 31?%, respectively, compared with 14 and 10?%, respectively, when rates were calculated by review of records.

Conclusion

The PICSS is a sensitive tool for assessing functional outcome post HD surgery. The Soave and transanal procedures have similar functional outcomes.     

先天性巨结肠经肛门Soave Ⅰ期拖出术后功能随访

目的评估先天性巨结肠经肛门Soave Ⅰ期拖出术后排便功能。方法随访2000年10月-2004年10月经肛门Ⅰ期拖出术44例，开腹Soave术35例，腹腔镜Soave术29例，Ikeda-soper术39例。术后随访1～5年。对围手术期疗效（术前准备时间、手术时间、术中输血量、术后禁食天数、应用抗生素时间、住院天数及费用）及术后排便控制能力（Kelly评分和直肠肛管测压）进行评估。结果经肛门Ⅰ期拖出术平均手术时间（75±20）min、费用（8198．81元）低于开腹术[（92±25）min；10264．45元]、腹腔镜术[（125±25）min；12504．67元]（P〈0．05）；术前肠道准备（2d）、手术时间（75±20）min、术中输血量（0例）、术后禁食天数（2d）、应用抗生素时间（4±1）d、住院天数（11±2）d及费用（8198．81）元较Ikeda-soper术[（4±1）d；（240±30）min；32例；（5±1）d；（7±2）d；（19±3）d；15243．78元]显著降低（P〈0．01）。经肛门Ⅰ期拖出术后的近远期并发症发生情况与开腹术、腹腔镜术无显著差别（P〉0．05）；Soave术后3个月小肠结肠炎的发生率（12例）高于Ikeda-soper术（3例）（P〈0．05），但就经肛门Soave术（5例）与Ikeda-soper术（3例）比较，差异无显著性意义（P〉0．05）。经肛门Ⅰ期拖出术和Ikeda-soper术患儿术后12个月Kelly评分无差别；术后1年直肠肛管测压均未引出直肠肛管抑制反射，术后肛管高压区长度、肛管静息压、直肠感觉阈值无统计学差异，但经肛门SoaveⅠ期拖出术后主动收缩压（52．3±15．6）mmHg低于Ikeda-soper术后（55．7±15．4）ramHg。结论经肛门SoaveⅠ期拖出术创伤小、并发症少、费用明显低于开腹、腹腔镜Soave术以及Ikeda-soper术，适用于治疗小年龄婴幼儿的短段型和常见型先天性巨结肠。     

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.     

Hirschsprung's disease: alpha-naphthylesterase activity for enzyme-histochemical evaluation of the extent of the aganglionic segment during surgery

The authors present their experience with the histotopochemical alpha-naphthylesterase reaction for intraoperative evaluation of the length of the aganglionic segment in Hischsprung's disease (HD). The activity of nonspecific esterases (Davis and Ornstein technique [1]) was used to demonstrate the presence or absence of intramural ganglion cells in seromuscular intestinal biopsies taken at various levels in 50 patients with the preoperative diagnosis of HD; in 44 cases the biopsies were taken during a radical HD operation (endorectal pull-through, Soave technique). In the remaining 6 cases, which presented with intestinal obstruction, the biopsies were taken during laparotomy. The authors consider this histochemical technique a reliable, quick, simple, and economical method for intraoperative evaluation of the length of the aganglionic segment. It is also useful for confirming the diagnosis of HD and is of great help in the study of so-called pseudo-HD. Offprint requests to: P. Dodero     

Long-term outcome of children after single-stage transanal endorectal pull-through for Hirschsprung’s disease

Background  

Single-stage transanal endorectal pull-through (TEPT) is a new technique for surgery of Hirschsprung’s disease (HD). TEPT can be assisted by laparoscopy (laparoscopic assisted transanal pull-through, LATP) or with non-additional procedure (total transanal endorectal pull-through, TTEP). This study was undertaken to evaluate the long-term outcome of these approaches in children with HD.     

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。     

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。