先天性肛门闭锁的诊疗体会

目的探讨先天性肛门闭锁的术前准备、手术方式及术后的处理措施。方法选取2010-01—2015-02间收治的先天性肛门闭锁患儿共51例,其中39例行会阴肛门成形术,12例行骶会阴肛门成形术。术后均应用大口径肛管引流减压。结果本组51例,手术时间为15~120 min,术中出血量为3~32 m L,无死亡病例。术后2周开始扩肛6个月。无肛周感染、肛门狭窄、失禁。随访6~12个月,50例患儿大便正常,无狭窄、瘘管复发、大便失禁及切口感染,1例患儿术后大便失禁。结论了解直肠肛门的解剖生理,根据直肠盲端的位置,选择正确手术时机和方式,恢复直肠解剖部位,术后应用大口径肛管引流减压,可减少并发症,促进顺利康复。     

直肠癌根治会阴部造口术后二期股薄肌移植肛门成形

目的　回顾性总结32例直肠癌根治会阴部造口术后二期股薄肌移植肛门成形术的治疗效果。方法　采用Williams 5级评分法对重建前后肛门功能进行评价。结果　二期股薄肌移植肛门成形术前,32例患者肛门功能均在4级以上[4级28.4%(9/32),5级71.6%(23/32)],二期股薄肌移植肛门成形术后肛门功能明显好转。结论　二期股薄肌移植肛门成形术是直肠癌根治会阴部造口术后有效的肛门重建手段。     

直肠癌根治会阴部造口术后二期股薄肌移植肛门成形

目的回顾性总结32例直肠癌根治会阴部造口术后二期股薄肌移植肛门成形术的治疗效果。方法采用Williams5级评分法对重建前后肛门功能进行评价。结果二期股薄肌移植肛门成形术前，32例患者肛门功能均在4级以上[4级28．4％(9／32)，5级71.6％(23／32)]，二期股薄肌移植肛门成形术后肛门功能明显好转。结论二期股薄肌移植肛门成形术是直肠癌根治会阴部造口术后有效的肛门重建手段。     

股薄肌肛门原位成形术治疗肛门失禁的临床观察

目的探讨股薄肌肛门原位成形术(GP)治疗肛门失禁的疗效及其并发症。方法回顾性分析2007年6月至2010年12月在中山大学附属第六医院行GP治疗5例肛门失禁患者的临床资料。结果术后伤口愈合时间32d(24～51d)。术后无明显并发症,术后3个月和术后1年的Wexner评分分别为8.0±0.4分和6.8±1.4分。术前术后肛管直肠动力学比较发现:术后患者的肛管静息压和肛管长度较术前明显改善(P<0.05)。结论 GP对肛门失禁的疗效好,并可以维护肛门功能及肛门外形。     

Pena手术及盆底肌肉折叠术在治疗高中位肛门闭锁术后大便失禁的应用

大便失禁是高中位肛门闭锁患儿术后最常见的并发症,其治疗方法有:股薄肌移植、臀大肌移植、盆底肌折叠及脉冲电刺激器埋植和二次Pena手术等方法,均获得了成功的疗效。许多研究表明,先天性肛门闭锁患儿有控制排便功能的横纹肌复合体存在,但其发育较正常儿差,Pena手术自1980年应用治疗高中位肛门闭锁后,术后排便功能有明显提高,此手术具有暴露横纹肌复合体解剖层次准确清楚,损伤小优点。本文病例采用Pena手术入路,了解大便失禁患儿直肠与横纹肌复合体关系,以便将直肠准确固定于肌肉中心。     

低位直肠癌切除即时原位肛门重建术

目的 探讨低位直肠癌行Ｍｉｌｅｓ根治术后即时行原位肛门重建的方法。方法 采用股薄肌移转和乙状结肠末端套叠法重建肛门外,内括约肌,辅以人工直肠瓣,并建立直肠角。结果 原位重建肛门３２例,功能优良率达９０．６３％。结论 本法适用于低位直肠癌,肛管癌根治术及其他原因所致的肛门功能丧失者。     

小儿直肠尿道瘘的修补与肛门括约肌重建术

目的：提高经多次手术失败的直肠尿道瘘、大便失禁的手术成功率。方法：按Pickrell术式或Hartle改良方法,压榨股薄肌支配神经后移转到肛门周围固定,行肛门外括约肌成形术共18例。结果：1989－1999年共治疗3－15岁直肠尿道瘘伴大便失禁小儿18例,结果15例无漏尿,3例漏尿者也都在术后5周内消失,大便失禁亦都有改善。15例随访7个月－6年,12例大便失禁明显改善。结论：用该法解决难以处理的直肠尿道瘘成功率高,大便失禁得到改善。     

硅胶管疗法治疗新生儿先天性肛门闭锁12例临床体会

目的探讨新生儿先天性肛闭锁,采用硅胶管代肛门直肠支架中的治疗作用。方法对2008年11月2009年2月采用硅胶管肛门形成术治疗肛门闭锁12例病例作回顾性分析。结果12例术后30 d随访：9例症状消失,排便正常;2例症状消失,排便基本正常,偶有大便失禁;1例症状消失,但大便失禁。结论新生儿先天性肛门闭锁一旦确诊尽早行肛门形成手术,肛管内置硅胶管支架作用,可促进肛管创面愈合,通过胶管压迫创面使直肠壁组织粘连,可以促进组织生长修复,促使直肠伴有会阴或泌尿系瘘口的闭合。所以,我们认为治疗肛门闭锁不论是低中高位都先采用这种手术方法,其操作简单,创面损伤少,织组生长修复快,护理方便,治愈成功率高。     

原位肛门重建的实验研究及临床应用

对４８只家猫采用４种不同术式行原位肛门重建，即Ａ、Ｂ、Ｃ三种切除肛门内外括约肌，其中Ａ组游离股薄肌包裹，Ｂ组结肠套叠，Ｃ组结肠套叠加股薄肌包裹，Ｄ组结肠套叠保留肛门外括约肌。术前术后１、３、６个月测量重建肛管及直肠内压，并观察猫的排便情况，结果第Ｃ、Ｄ组有意义的高于ＡＢ两组，将ＣＤ组术式应用临床２５例，术后控便功能优良者占８４％。     

重建直肠角肛门成形术治疗肛门直肠闭锁

１９８６年,高春芳首先将重建直肠角式人工肛门应用于直肠癌根治术中［１］,术后肛门功能恢复满意。受此启发,１９８８～１９９６年,我们采用重建直肠角、经腹会阴一期肛门成形术,治疗高位先天性肛门直肠闭锁伴阴道、尿道及舟状窝瘘５８例。术后随访２～８年,其排便功能满意。本文介绍该手术方式并总结５８例的治疗体会。１　临床资料本组新生儿２０例,１岁１５例,３岁１０例,６岁８例,１２岁５例。男３２例,女２６例。根据Ｘ线检查及手术所见,按肛门直肠畸形国际分类标准［２］,本组５８例直肠盲端均在耻骨尾骨线（ＰＣ线）…     

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.     

肛门直肠畸形术后排粪失禁患儿盆腔磁共振成像表现及其临床意义

目的通过肛门直肠畸形术后排粪失禁患儿的盆腔MRI表现,了解盆底肌的形态,直肠、肛管的形态和位置,以及脊髓和骶骨的发育,为分析排粪失禁的原因及制订治疗方案提供客观依据。方法回顾性分析2009年9月至2011年12月间山东大学第二医院收治的34例肛门直肠畸形术后排粪失禁患儿的临床和影像资料,其中男2l例,女13例,年龄3,14岁。应用1．5TMR扫描仪,常规行轴位、冠状位及矢状位扫描,观察肛门括约肌、耻骨直肠肌和提肛肌、直肠、肛管的形态,以及脊髓、骶骨的发育情况。结果MRI检查提示：肛门外括约肌发育不良18例,耻骨直肠肌发育不良23例,肛提肌发育不良27例;直肠位置异常6例,直肠扩张12例,肛直角增大11例;肛管周围脂肪组织5例;合并神经管闭合不全2例,Currarino综合征2例,骶骨发育不全11例,直肠尿道瘘2例。以上影像学结果均经临床最终证实。结论MRI可清楚显示肛门外括约肌、耻骨直肠肌和肛提肌的形态,以及直肠和肛管的形态和位置,同时还可显示脊髓和骶骨的发育情况,是评价肛门直肠畸形术后排粪失禁患儿非常有价值的检查方法。     

Sacral nerve stimulation in the treatment of bowel dysfunction from imperforate anus: A case report

IntroductionBowel dysfunction (fecal incontinence and constipation) presents in over 50% of patients after treatment of congenital anal malformations. Sacral nerve stimulation (SNS) for the treatment of fecal incontinence improves function in the majority of patients. We present a case report of the treatment of bowel dysfunction with sacral nerve stimulation in a patient with a history of an imperforate anus.Presentation of caseA twenty year-old female with a history of imperforate anus at birth, repaired during infancy with anorectoplasty, presented with fecal incontinence and constipation. Since childhood, she had been suffering from intermittent constipation with worsening fecal incontinence in early adulthood. Examination revealed mild anal stenosis and mucosal prolapse. Endoanal ultrasound demonstrated intact internal and external sphincter with low resting and squeeze pressures on anal manometry. Flexible sigmoidoscopy was normal. The patient underwent permanent sacral nerve stimulation with a primary goal of improvement in continence and, secondarily, for the alleviation of intermittent chronic constipation.DiscussionAt 15 month follow-up, the patient had improvement in fecal incontinence (CCIS of 14 pre-SNS to 1 post-SNS), constipation (CCCS of 28 pre-SNS to 20 post-SNS), and quality of life (FIQOL improved in lifestyle (3.7), coping/behavior (3.4), self perception (3.9), and social embarrassment (4.5).ConclusionSacral nerve stimulation for the treatment of bowel dysfunction in adults secondary to imperforate anus can be performed safely and with good results.     

High and intermediate imperforate anus: results after surgical correction with special respect to internal sphincter function.

Embryologically a fistula in an anorectal malformation can be regarded as an ectopic anus. Since 1984 the so-called fistula has been saved and used as the new anal canal in the surgical reconstruction of 48 patients with high or intermediate imperforate anus. A positive rectoanal inhibitory reflex indicating internal sphincter function was recorded in 32 of 43 patients investigated with anorectal manometry. These patients showed significantly better anal continence. It is concluded that there is an internal sphincter "anlage" in the fistulous connection from the bowel to the urogenital tract. Internal sphincter function can be obtained in the majority of the patients, which seems advantageous for their anal function.     

Fecal incontinence in Hirschsprung's disease in children]

Among the criteria of evaluation of the results of operative treatment of Hirschsprung's disease in children, along with the absence of constipation is the function of defecation control. The authors examined 27 patients with fecal incontinence after Duhamel's operation. It was established that fecal incontinence in children after operative treatment of Hirschsprung's disease may be caused not only by affection of the external sphincter muscle of the anus, the nerve plexuses, and adaptation of the rectum to the new conditions after the pull-through operation, but also by trauma inflicted to the levator ani muscle. In children with severe damage to the levator group of muscles consequent upon operative treatment of Hirschsprung's disease, operative intervention for creating the anorectal angle is indicated.     

Gracilis muscle transposition for anal incontinence in children: long-term follow-up.

We report a series of 7 patients, aged 6.5 to 19 years (mean, 12.9), who have been treated for uncontrollable fecal incontinence since 1976: 5 had imperforate anus and multiple subsequent operative procedures, 1 had a low myelomeningocele with bi-sphincteric incontinence, and 1 had a traumatic destruction of the sphincter apparatus. A modified Pickrell procedure was performed, with the gracilis muscle transposed subcutaneously, without constructing a pulley through the median raphe as originally described. All patients were evaluated by anorectal manometry preoperatively and post-operatively. They were followed-up for a period of 0.5 to 12.5 years (mean, 4.4). All patients were continent at follow-up, with a normal defecation pattern and no enemas required. None of the patients had evidence of fibrosis of the muscle or anal canal, and tension in the transposed muscle was maintained. Voluntary contractions remain efficient in all cases. Age was thought to be an important factor: personal motivation and compliance with physiotherapy, essential for a good outcome, is unlikely to be present in the younger child. We conclude that the gracilis sling procedure is an excellent long-term alternative for total fecal incontinence when time and other therapeutic measures have failed.     

Congenital malformations of anus and rectum: Report of 162 cases

One hundred sixty-two cases of anal and rectal abnormalities were reported from the Boston Children's Hospital.The embryology of the anorectal region is reviewed. The malformations under discussion are believed to represent arrests or aberrations in development of the anus or rectum in the seventh or eighth week of fetal life.The cases are classified into four groups which had the following characteristics: (1) Stenosis of the anus; (2) membranous obstruction of the anus; (3) imperforate anus, but with separation of the rectum from the anus; (4) anus and anal canal normal, but separation of the rectum from the anal pouch. The external anal sphincter muscle was present in all four types. Associated with these anal and rectal obstructions there were fistulae in 52 per cent of the patients, connecting the rectum with the bladder, urethra, vagina, fossa navicularis, or the perineum.The symptoms and physical signs were essentially those of acute bowel obstruction when there was an imperforate anus or atresia of the rectum. In those cases of anal stenosis or in those instances of rectal atresia in which there was a large associated fistula, there was less evidence of intestinal obstruction. Fistulae with the urinary tract were manifested by the presence of urinary infection or by the passage of fecal material and flatus through the urethra.X-ray examination was a valuable aid in determining the distal extent of the rectal pouch in cases of imperforate anus or rectal atresia. This could be readily accomplished by taking a flat plate with the baby in the inverted position, thus allowing gas to outline the rectum. Due to the fact that some time was required for gas to reach the lower intestinal tract, the method was not wholly reliable in the first fifteen to twenty hours of life.The essential in treatment of these rectal and anal abnormalities was to establish a continuity of epithelium between the rectum and skin and thus prevent scar formation with constriction. To provide adequate anal control, the external sphincter muscle was always employed. In the stenoses (Type 1) repeated dilatations were usually all that was necessary, but when the anal canal was unyielding, it had to be excised and the rectal mucosa brought down to cover the defect. In the membranous imperforate anus (Type 2) simple cruciate incision and dilatations sufficed. The rectal atresias (Types 3 and 4) were treated by a perineal operation (when the pouch was low enough) and the rectum was brought down to the skin through the anal sphincter muscle. When the pouch was high and prohibited a successful perineal operation, a colostomy was resorted to. In Type 3 atresias 86 per cent of the cases were amenable to treatment by the perineal approach. In Type 4 atresias, 66 per cent of the cases were amenable to treatment by the perineal operation.There were 43 deaths in the series, giving a mortality of 26 per cent. At least 12 of these deaths were directly due to other associated congenital abnormalities, leaving an expected mortality rate of about 19 per cent for anorectal abnormalities and their complications. By groups the mortality rates were: Type 1, 9.5 per cent; Type 2, 16.6 per cent; Type 3, 24.8 per cent; Type 4, 61.6 per cent.The lower fistulae (rectoperineal, rectofossa navicularis, and rectovaginal) were relatively easy to close when the rectal obstruction was corrected in the first few days of life. The higher fistulae (rectourethral and rectovesical), however, were very difficult to reach through a perineal incision in a newborn infant, hence it was found best to delay treatment of such communications until the patient attained an age of eight or nine years.     

Artificial anal sphincter in severe fecal incontinence: outcome of prospective experience with 37 patients in one institution

OBJECTIVE: To evaluate the outcome of artificial anal sphincter implantation for severe fecal incontinence in 37 consecutive patients operated on in a single institution from 1993 through 2001. SUMMARY BACKGROUND DATA: Implantation of an artificial anal sphincter is proposed in severe fecal incontinence when local treatment is unsuitable or has failed. The results of this technique have not been determined yet, and its place among the various operative procedures is still debated. METHODS: Artificial anal sphincters were implanted in 37 patients from 1993 through 2001. All patients had complete fecal incontinence and had failed to respond to medical treatment. Median duration of incontinence was 16 years. The causes of incontinence were sphincter disruption (19 patients), hereditary malformations (2 patients), and neurologic disease (16 patients). Six patients had had previous surgery for fecal incontinence. Assessment was made by physical examination (anal continence, rectal emptying) and anorectal manometry. RESULTS: In the first 12 patients, six devices had to be removed (50%); the cause of failure was found in all cases, and this allowed contraindications to be defined. Among the next 25 patients, 23 had an uncomplicated postoperative follow-up, and 5 developed seven complications: control pump change (n = 3), balloon migration (n = 1), and major rectal emptying difficulties in patients with obstructive internal rectal procidentia (n = 2). The artificial anal sphincter had to be removed definitively in three cases, representing the failure rate of this technique in the authors' experience (12%); two other devices had to be removed temporarily and the patients are awaiting reimplantation. In this latter group of 25 patients, 80% have an activated sphincter: continence for liquid stool is normal in 78.9%, continence for gas in 63.1%. Seven patients have rectal emptying difficulties, minor in five and major in two. Manometric studies showed mean pressures of 110 and 37 cm H(2)O with closed and open sphincter, respectively, with a mean duration of artificial sphincter opening of 128 seconds. CONCLUSIONS: The long-term functional outcome of artificial anal sphincter implantation for severe fecal incontinence is satisfactory; adequate sphincter function is recovered and the definitive removal rate is low. Good results are directly related to careful patient selection and appropriate surgical and perioperative management after a learning curve of the surgical team.     

Diagnostic evaluation of patients with faecal incontinence at a specialist institution

BACKGROUND: Evaluation of the anorectal function, clinically, structurally, and functionally, in patients with faecal incontinence should ensure appropriate and individual treatment. METHODS: Two hundred and twenty-six patients with faecal incontinence were reviewed regarding disease history and results of anorectal physiological tests and anal ultrasonography. RESULTS: The mean age was 54 years, and 191 patients (85%) were female. Sixty-two patients had passive faecal incontinence only, 49 had urge faecal incontinence only, and 115 had both passive and urge faecal incontinence. Patients with passive faecal incontinence only had a significantly higher voluntary contraction pressure and less external sphincter abnormalities than patients with urge faecal incontinence or both passive and urge faecal incontinence. The structural abnormalities of the internal and external anal sphincters identified on anal ultrasonography were significantly associated with a low maximum resting pressure and with a low voluntary contraction pressure, respectively. The causes identified for this faecal incontinence were: in 90 patients idiopathic, in 76 obstetric injury, in 36 internal anal sphincter degeneration, in 20 anal surgical injury, in 6 rectal prolapse, and in 9 patients miscellaneous. CONCLUSIONS: The anal sphincter structure as demonstrated by ultrasonography was closely related to the anorectal function, as determined by anorectal physiological tests, and the observations from these were reflected in the range of patient-reported symptoms. Anal ultrasonography and anorectal physiological tests are useful tools, enabling us to identify the mechanisms and causes of faecal incontinence in at least 60% of the patients.     

肛管内超声对排粪失禁患儿肛门括约肌受损程度的评价

目的探讨肛管内超声对肛门内括约肌（IAS）、肛门外括约肌（EAS）及耻骨直肠肌（PR）形态及完整性的评估,从而为排粪失禁的原因及治疗方案的制订提供客观依据。方法回顾性分析2009年12月至2012年11月间山东大学第二医院收治的14例先天性肛门直肠畸形术后及4例先天性巨结肠术后排粪失禁患儿的临床资料。应用肛管内超声对IAS、EAS及PR进行观察,并进行括约肌受损程度评分;同时行肛管直肠测压评分和肛门功能评分,通过Spearman秩相关分析评价括约肌评分与肛管直肠测压评分和肛门功能评分之间的相关性。结果18例患儿中男13例,女5例,年龄10—16岁。肛门括约肌受损评分结果显示,括约肌轻度受损11例,中度受损65J,重度受损1例;另有PR受损4例。括约肌评分与肛管直肠测压评分之间呈正相关（P〈0．05）,而与肛门功能评分无明显相关性（P〉0．05）。结论肛管内超声可清楚显示IAS、EAS和PR的形态,明确其是否完整及受损程度,是评价肛门直肠畸形术后排粪失禁患儿非常有价值的方法,但并不能完全反映括约肌及肛门的功能状况。