肛门直肠畸形合并先天性巨结肠

目的：探讨先天肛门直肠畸形（ＡＲＭ）合并先天性巨结肠（ＨＤ）的临床特点和诊治方法。方法：回顾性分析总结本院２０年来ＡＲＭ３２６例，ＨＤ３２０例，其中两种并存有３例，３例均经手术病理证实诊断，结论：ＡＲＭ作结肠造瘘术后，排便不畅应及时作结肠活检，随访ＡＲＭ术后患儿有腹胀，便秘，应进一步作钡灌肠和直肠粘膜乙酰胆碱酯酶检查，确诊后按巨结肠处理。     

先天性巨结肠合并先天性肥厚性幽门狭窄1例并文献分析

目的 总结先天性巨结肠合并先天性肥厚性幽门狭窄的诊治经验. 方法 回顾性分析2019年12月收治的1例先天性巨结肠合并先天性肥厚性幽门狭窄新生儿的临床资料,并结合相关文献分析.患儿男性,出生22天,因腹胀、呕吐入院,钡灌肠示先天性巨结肠,超声检查示先天性肥厚性幽门狭窄.充分准备后Ⅰ期行腹腔镜下幽门环肌切开术,待患儿营养...     

巨结肠根治术后便秘复发的原因探讨

目的 探讨巨结肠根治术后便秘复发的原因,以提高手术效果.方法 总结1995年至2005年在我院行手术治疗的巨结肠病例392例,其中32例因便秘复发再次手术,分析复发病例的手术时间、方式、病理结果、肠炎发生情况及排便功能.结果 巨结肠根治术便秘复发率为4.5%,左半结肠切除术后复发率为16.0%(26/162),明显高于结肠次全切除术的复发率2.1%(4/189) (P<0.01).32例复发病例中,巨结肠同源病(HAD)及先天性巨结肠(HD)合并同源病共26例.复发的原因主要有近端HAD肠管切除不够或继发HAD改变,HD肠管切除不完全;远端切除不够,吻合口过高;吻合口狭窄、肛门狭窄及失弛缓等.复发病例的肠炎发生率明显高于无复发病例(P<0.05).结论 HAD较HD更易便秘复发,彻底切除近远端病变肠管,吻合口尽可能做到后壁距肛门缘1.5cm处,对小肠结肠炎早期诊断、早期治疗,术后置肛管以及术后复诊,一旦发现吻合口狭窄,及时扩肛等措施可降低巨结肠术后便秘的复发率.     

肛门直肠局部注射肉毒毒素A治疗先天性巨结肠症

目的探讨局部注射肉毒毒素A治疗短段型先天性巨结肠症的可行性。方法对8例经病理组织学和钡灌肠诊断证实为短段型先天性巨结肠患儿,在截石位3、6、9钟位,将肉毒毒素A注射入肛门内括约肌和直肠肌层内,总剂量1.5U/kg。治疗前及治疗后1个月、1年分别作肛门直肠测压,治疗后3～6个月复查钡灌肠,以后每年钡灌肠复查1次。结果所有患儿治疗后均恢复良好,无术中和术后并发症。1年内所有患儿均能自主排便,未发现腹胀、便秘。有3例1年后再次出现便秘(3～5d排便1次)和轻度腹胀症状,但经扩肛后均迅速缓解。随访1个月时,肛门直肠静息压8例均较术前降低,1年后复查6例仍低于术前。术后3～6个月复查钡灌肠,8例扩张结肠均较术前明显缩小。结论肉毒毒素A注射治疗短段型巨结肠症是一种安全、可靠的新方法,其损伤小,近期疗效良好;但作用机制和远期疗效有待进一步研究。     

腹腔镜下结肠切除、直肠内拖出术治疗巨结肠类缘病

目的探讨腹腔镜下结肠部分或次全切除、直肠内拖出术治疗巨结肠类缘病的可行性。方法1999年10月-2006年5月，对保守治疗无效或复发的38例先天性巨结肠类缘病（Hirschsprung’s disease-allied disorder，HAD）和先天性巨结肠（Hirschsprung’s disease，HD）合并HAD的患儿在腹腔镜下行结肠部分或次全结肠切除、直肠内拖出术。分别在脐缘、右上、右下、左中腹刺入4个5mm trocar，行结肠次全切除时，左侧需建立2个操作孔，分别在左上及左中下腹。腹腔镜下分别游离降结肠、横结肠、升结肠及回盲部侧腹膜，肛门手术按改良Soave方法。结果38例均在腹腔镜下完成手术。9例行左半结肠切除，手术时问110—180min，平均135min；29例行结肠次全切除并按Deloyers法将升结肠逆时针转位270。下拖，保留的升结肠长度7—13cm，平均11．5cm，手术时间140—220min，平均175min。术中出血15～70ml，平均35ml。病理诊断肠神经元性发育异常10例，神经节细胞减少症3例，神经节细胞未成熟症4例，未分类型9例，HD合并IND6例，HD合并HG2例，HD合并IGC4例。38例术后随访6个月一7年，平均3年5个月，29例次全结肠切除患儿3个月内每日大便4—18次，6—10个月后渐转为每日2～3次，9例左半结肠切除患儿术后6个月后每13大便1—2次，无吻合口狭窄，无便秘复发。结论腹腔镜下结肠部分或次全切除经肛门拖出根治术治疗HAD安全、有效、可行，手术创伤较小，但需要一定的腹腔镜操作经验。     

改良Swenson术治疗22例先天性巨结肠临床观察

目的探讨改良Swenson术治疗非洲尼日尔先天性巨结肠（Hirschsprungcs Disease,HD）的有效性和安全性。方法对2006年11月至2008年11月在非洲尼日尔援外工作期间采用改良Swenson术治疗22例先天性巨结肠（HD）患儿进行回顾性分析,对术后并发症情况进行评价。结果本组22例患儿均达到最大限度地保留肛门括约肌,保留其正常排便功能,术后无吻合口漏、切口裂开、感染等早期并发症及术后随访患儿无复发、肠梗阻、吻合口狭窄、大便失禁、腹泻、便秘、污裤等晚期并发症。结论改良Swenson术适用于常见型、短段型及长段型先天性巨结肠,具有较高的有效性和安全性。     

Rehbein改良术治疗先天性巨结肠

目的：改良Ｒｅｈｂｅｉｎ术式，提高先天性巨结肠的治疗效果。方法：随访１３１例先天性巨结肠术后患儿，获随访１０５例，随访内容主要包括术后排便功能、肛门污粪和失禁现象、生长发育情况、肠粘连等问题。结果：９９例排便通畅，无便秘腹胀现象，无肛门污粪和失禁存在，饮食正常，生长发育良好，生物质量大提高。结论：改良术后疗效高，有推广应用价值。     

先天性巨结肠症直肠肛管纵切心形吻合术12例报告

对12例先天性巨结肠患儿采用腹内不断结肠经直肠内套出结肠,直肠肛管纵切,结肠直肠前高后低心形吻合不用夹具的手术方法。患儿平均住院30d,吻合口狭窄1例,1月后作狭窄部切开;1例发生尿潴留,3个月后恢复,2例肛门污粪,全组钡灌肠复查结肠无狭窄及扩张,肛门排便功能正常,直肠肛管纵切心形吻合术根治先天性巨结肠症,不造成腹腔感染,不用夹具,术后肛门排便功能恢复快,并发症少。     

经肛门结肠拖出术治疗小儿先天性巨结肠--附57例报告

目的 探讨单纯经肛门结肠拖出术治疗小儿先天性巨结肠的可行性。方法 2001年7月—2002年5月，57例巨结肠患儿行经肛门结肠拖出术。结果 53例顺利完成单纯经肛门结肠拖出术，手术时间(1—2)小时，平均1．5小时，出血5m1—10m1。51例术后1天开始自行排便、进食，2例术后3天予开塞露灌肠后排便。4例长段型巨结肠，病变累及横结肠，在腹腔镜辅助下完成，手术时间(2—3．5)小时，平均3小时，术后(2—3)天开始排便、进食。随访(2—9)月，57例患儿排便功能均正常良好。结论 单纯经肛门结肠拖出巨结肠根治术治疗小儿先天性巨结肠可行，此术式损伤小、出血少、术后恢复快、住院时间短，术后排便功能恢复良好。     

腹腔镜辅助治疗先天性巨结肠20例报告

目的:探讨经肛门行腹腔镜辅助Soave's巨结肠根治术治疗先天性巨结肠的疗效。方法:2007年1月至2008年8月我们为20例先天性巨结肠患儿施行经肛门腹腔镜辅助Soave's巨结肠根治术。结果:20例均顺利完成手术,术中出血少,愈合后疤痕不明显。术后4d进食,7～10d出院。术后患儿均自解大便,无吻合口狭窄。结论:腹腔镜辅助治疗先天性巨结肠是治疗长段型巨结肠的一种新的标准术式。     

外科手术对顽固性便秘并继发性巨结肠的疗效研究

目的：探讨不同手术方式治疗顽固性便秘并继发性巨结肠的疗效。方法回顾性分析2007年6月至2013年1月在南京军区南京总医院全军普通外科研究所接受手术治疗的112例顽固性便秘并发继发性巨结肠患者的临床资料,全组患者便秘病程4～22年,其中74例既往接受过腹部中等以上手术。手术方式：（1）金陵术（结肠次全切除加升结肠-直肠后壁侧侧吻合术）81例,其中24例接受腹腔镜辅助金陵术,18例加末端回肠保护性造口术;（2）结肠全切除加末端回肠与直肠后壁侧侧吻合术18例;（3）结肠全切除加末端回肠临时造口术13例（6个月后行末端回肠与直肠后壁侧侧吻合术）。末端回肠保护性造口在术后6个月予以还纳。结果112例患者手术成功率100％,无手术相关死亡病例。术后出现的并发症包括术后早期腹泻90例（80．4％）、肛门疼痛和排粪不尽22例（19．6％）、尿潴留（去除导尿管后24～48 h内出现）16例（14．2％）、吻合口出血9例（8．0％）、吻合口瘘6例（5．4％）以及肠梗阻15例（13．4％）,除6例肠梗阻患者接受肠粘连松解术后症状缓解外,其余并发症均通过保守治疗恢复良好。术后随访6月,不同术式患者Wexner便秘平均评分为5．8～8．3,与术前21．4～28．7比较,明显改善（P＜0．01）。结论顽固性便秘并继发性巨结肠外科手术治疗效果良好。     

重建肛门外括肌和直肠肛管角治疗先天性肛门闭锁肛门成形术后大便失禁(附32例报告)

目的:观察重建肛门外括肌和直肠肛管角治疗先天性肛门闭锁肛门成形术后大便失禁的临床疗效。方法:对32例先天性肛门闭锁肛门成形术后大便失禁患者用股薄肌重建肛门外括肌并重建直肠肛管角。结果:32例患者术后随访1年,肛门功能优19例,良9例,较好4例,无效者未发现,总满意率为87·5%(28/32)。结论:用股薄肌重建肛门外括肌并重建直肠肛管角,术后患者肛门功能满意率高,患者生活质量明显提高。     

Total Proctocolectomy and Ileal J-Pouch Anal Anastomosis for Chagasic Megacolon with Fecaloma: Report of a Case

Megacolon, resulting in severe constipation, is one of the most common manifestations of Chagas' disease. We herein report a case of megacolon with fecaloma in a 39-year-old Brazilian man who presented with severe constipation. He underwent a total proctocolectomy and ileal J-pouch anal anastomosis. Histologically, there was a marked thickening of the proper muscle layer, and a moderate decrease in the myoenteric nerve plexuses and neurons in the megacolon area. A feasible outcome has been achieved after a 1-year follow-up. A total proctocolectomy with ileal J-pouch anal anastomosis may therefore be one of the useful strategies for the surgical treatment of patients with chagasic megacolon.     

Anterior perineal approach and three-flap anoplasty for imperforate anus: optimal reconstruction with minimal destruction.

Despite progress in the treatment of imperforate anus, anal stenosis, rectal prolapse, and other late complications may still arise. In 1987, we described the three-flap anoplasty for the treatment of rectal prolapse following pull-through operations. Since 1986, we have performed 14 three-flap anoplasties in combination with an anterior perineal rectal pull-through for primary treatment of imperforate anus. The mean age at definitive repair was 4.4 months (range, 0 to 14 months). Eleven of the 14 primary pull-through procedures could be performed through a perineal approach only. There were no deaths. At a mean follow-up of 24.2 months, none of the patients has developed prolapse, and only one has had a temporary stenosis. Three children are already fully continent, and soiling is absent in 12. All have a good sphincter tone. Although it is too early to evaluate long-term results, it appears that the three-flap anoplasty prevents mucosal prolapse through the interposition of a skin-lined anal canal. Moreover, a combination of this technique with the anterior perineal approach provides an excellent exposure with minimal dissection of the perineal and pelvic musculature and allows for easy and safe pull-through of the rectal pouch, making an abdominal counterincision unnecessary in most cases. It reproduces at the same time a normal anatomy while taking advantage of all existing structures.     

经会阴手术治疗成人短段型巨结肠症的研究

目的探讨经会阴实施肛门内括约肌和直肠末端肌层切开术,术后配合以生理盐水灌肠法,治疗成人短段、超短段型巨结肠症的可行性及疗效。方法自2001年4月至2010年8月,我院及协作医院,针对36例术前诊断为＂成人短段、超短段型先天性巨结肠＂患者分别实施了非开腹手术,即经会阴切口,施行肛门内括约肌和直肠末端肌层切开手术,术后配合以生理盐水灌肠法治疗。对此治疗方法的适应证、手术操作方法、及治疗效果,进行回顾性分析。结果 2例术中、术后病理组织检查确诊为＂非短段型巨结肠＂,术后配合以生理盐水灌肠法,效果不佳而续行开腹手术;30例术中、术后病理组织检查确诊为＂短段、超短段型巨结肠＂、4例术中、术后病理组织诊断为＂先天性巨结肠类缘病＂,病变未超过肛缘上6.5 cm。后34例术后均配合以生理盐水灌肠。随访,术后灌肠8个月者1例、10个月者1例,此2例均缓解了巨结肠症状（缓解率6%）;术后灌肠诱导排便1~2年者32例,均治愈巨结肠症,停止灌肠后继续追踪2~5年未见便秘、排便延迟、肠梗阻等巨结肠症状复发,治愈率94%。结论经会阴实施肛门内括约肌和直肠末端肌层切开术,术后配合以生理盐水灌肠法,可以迅速缓解短段、超短段型成人巨结肠的急性肠梗阻症状,并逐渐治愈便秘、排便延迟、腹胀腹痛等巨结肠症状。此方法安全可行、操作简便、免开腹手术、创伤小、痛苦轻、疗效满意,值得进一步实践及推广应用。     

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??Diagnosis and management of constipation associated with megacolon FU Chuan-gang, XU Xiao-dong. Department of Colorectal Surgery, Changhai Hospital, the Second Military Medical University, Shanghai 200433, China
Corresponding author?? FU Chuan-gang,E-mail??fugang416@126.com
Abstract Constipation associated with megacolon as a special type of intractable constipation is sinificantly different in the etiology, clinical manifestation and especially the managemnt. Depending on the etiology, it can be divided as congenital megabowel or Hirschsprung’s and idiopathic megabowel. And depending on the location of dilatation, it can be divided as megacolon, megarectum and megabowel. Besides the intractable constipation, most of megabowel patients have abdominal distention and may palpate abdominal mass. Imaging examination shows dilated colon and rectum. The management of bowel is difficult due to its rare incidence and complicated situation with high incidence of complication and recurrence.     

Posterior sagittal approach: megasigmoid resection and anal reconstruction for severe constipation and fecal incontinence after anoplasty

PURPOSE: The aim of this study was to present the technique of megasigmoid resection and anal reconstruction by complete posterior sagittal approach for the children with severe constipation and fecal incontinence after anoplasty. METHODS: Six patients (age, 2 to 18 years) born with imperforate anus and originally treated with perineal anoplasty suffered from intractable constipation and fecal incontinence. Contrast enema showed massive dilated and aperistaltic rectosigmoid colon with fecal impaction. Resection of the dilated bowel and anal reconstruction were completely performed by posterior sagittal approach. RESULTS: The mean operating time was 205 minutes (range, 125 to 265 minutes) and the average length of resected colon was 23.3 cm (range, 10 to 40 cm). There were no intraoperative or postoperative complications. By 2 to 4 months after the operation, all patients obtained voluntary bowel movement. On follow-up at 6 to 24 months postoperative, no patient had constipation or required use of the laxatives again. Four of 6 patients suffered from grade 1 soiling, and the other 2 had grade greater than 1 soiling. None had urinary retention or incontinence after the procedure. CONCLUSION: Resection of dilated rectosigmoid colon and anal reconstruction for the patients with severe constipation and fecal incontinence after anoplasty can be performed successfully using a posterior sagittal approach.     

The treatment of imperforate anus: experience with 108 patients

BACKGROUND/PURPOSE: The authors present their experience and results in the treatment of infants with imperforate anus over a 10-year period. Differences between these and previously published western results are noted and discussed. METHODS: One hundred eight patients with imperforate anus were treated from June 1988 to July 1998. Of these patients, 66 were boys and 42 were girls. Associated anomalies include congenital heart disease, anomalies of bone and cartilage, and Down's syndrome. Thirty-five patients with a low lesion received a limited posterior sagittal anorectoplasty. Seventy-one patients had a high lesion and received 3-staged operations including colostomy, posterior sagittal anorectoplasty, and takedown of colostomy. All patients underwent follow-up by the author. Postoperative anorectal function was evaluated based on the following criteria: ability to have voluntary bowel movement, soiling, and constipation. The duration of follow-up ranges from 6 months to 10 years. RESULTS: One patient died of multiple congenital anomalies after colostomy. One patient died of hyaline membranous disease. All except 2 patients had voluntary bowel movement. Three patients had soiling, and 19 suffered from constipation after operation. The constipation improved with medical treatment and time. Four patients who received the first operation at another hospital (3 underwent posterior sagittal anorectoplasty and 1 had cutback anoplasty) had problems with soiling. In these patients, soiling improved after redo posterior sagittal anorectoplasty. CONCLUSIONS: Utilizing the posterior sagittal operation described by Pe?a, most patients were continent and able to have voluntary bowel movements. Constipation occurred in a substantial number of patients with high-type lesions, but few of these patients needed medication or enemas. There were significantly fewer sacral and urogenital anomalies than have been reported in most western series. This may explain the excellent results.     

腹腔镜、腹腔穿刺液细胞学和影像学检查对腹部疾病诊断的比较

目的 探讨诊断性腹腔镜对疑难性腹部疾病的诊断价值。方法 对31例临床难以诊断的腹痛、腹水和腹部肿块患者进行电视腹腔镜下检查和活检,并对腹腔镜、腹腔穿刺液细胞学和影像学检查诊断分级进行比较。结果 31例腹腔镜检查均获成功,经活检病理组织学检查30例明确诊断,确诊率达97％。并发现罕见腹部疾病11例（36％）。腹腔镜分级诊断的Ⅰ级诊断率（97％）明显高于腹腔穿刺液细胞学和影像学诊断率（10％和19％）（P均＜0．01）。结论 腹腔镜检查是不明原因腹痛、腹水和腹部肿块病因诊断的一种确诊率最高、安全和快速的方法。     

Operative treatment of anterior ectopic anus: the efficacy and influence of age on results

In our experience, anterior ectopic anus (AEA) is a common cause of constipation in children. We have performed 54 anoplasties for AEA in the past 8 years. Seventy-eight percent of the patients were girls. The average age at surgery was 23.8 months and the average stool frequency prior to surgery was once every 3.2 days. Eighty percent of the children had undergone attempts at medical therapy. We compared the functional results of anoplasty in children by age. The children who had surgery prior to the age of 6 months had a significantly better outcome than did older children. These data suggest that patients with AEA have a better response to operative therapy when anoplasty is performed prior to 6 months of age. Early recognition, referral, and surgery is appropriate therapy in this patient group.