Ⅰ期改良Pe(n)a术治疗新生儿中高位先天性肛门直肠畸形

目的 探讨Ⅰ期改良Pe(n)a术治疗新生儿中高位先天性肛门直肠畸形的临床可行性及有效性.方法对48例先天性肛门直肠畸形的新生儿行Ⅰ期Pe(n)a术,其中31例进行2个月至4年4个月随访,对临床及随访资料进行回顾性分析.结果术后1例因肺部感染加重出现呼吸循环衰竭死亡,2例切口部分裂开,余恢复良好.31例坚持随访2个月至4年4个月,3例便秘,3例稀便时污粪,1例用力时污粪,2例直肠黏膜脱垂,1例切口感染,1例肛门狭窄(未坚持扩肛所致).无完全大便失禁、瘘管复发、尿潴留等并发症出现.17例中高位肛门直肠畸形术后行肛门直肠测压检查,有3例存在RAIR.结论Ⅰ期Pe(n)a术治疗先天性肛门直肠畸形,术后肛门功能良好,早中期疗效满意,Ⅰ期Pe(n)a术是一种创伤小、花费少、美观、简洁的手术方式.

Abstract：

Objective The aim of this study was to examine the feasibility, safety, cost- effectiveness , and medium-term outcome of one-stage repair of intermediate and high imperforate anus in the newborn. Methods This is a retrospective review of forty eight consecutive newborns who underwent one-stage anorectoplasty during the newborn period at the children's hospital of Chongqing medical University between January 2006 and March 2010. Thirty one of 48 patients were followed-up for 2 months to 4 years 4 months, followed-up data on the retrospective analysis. Results Forty-five patients had excellent results. One case died of respiratory and circulatory failure, the other two suffered from part incision dehiscence. Twelve of Thirty-one of the 48 cases has been followed-up for 2 months to 4 years and 6 months. No fecal incontinence,fistula recurrence,urinary retention has been observed. 3 cases has had constipation, 4 patients has soiled his diapers between bowel movements, 2 cases has had rectal mucosa prolapse, 1 patient has had wound infection, 1 patient has had stricture of anus without dilating of the anus. 17 cases underwent Anorectal manometry,only 4 exist RAIR. Conclusions Complete 1-stage repair using the Pe(n)a to treat high-type and intermediate-type anorectal malformations can be offered the same results as the traditional Pe(n)a. Also.it is safe and feasible.     

改良式Mollard术治疗先天性高位肛门直肠畸形

目的：探讨先天性高位肛门闭锁的治疗方法。方法：采用改良式Mollard术即前矢状入路结肠经直肠盲袋肌鞘内拖出治疗高位肛门闭锁18例。结果：无手术死亡，18例全部存活。腹部切口及会阴前矢状切口均工期愈合。临床评分5～6分为优，共11例，占61．1％，3～4分为良，共6例占33．3％，0～2分为差，1例占5．5％。结论：采用前矢状入路直肠盲袋肌鞘内拖出治疗高位肛门闭锁，一期完成手术，既保留了直肠盲袋及发育不全的内括约肌，又防止肥厚宽大的盲袋拖出时对肛门外括约肌复合体所造成的损伤，保留了直肠盲袋肌层内的神经反射功能，防止了直肠周围血管神经的损伤及吻合口瘘，前矢状入路准确的对合会阴外括约肌复合体及会阴体，术后获得较好的排便功能。     

改良Pena手术Ⅰ期治疗先天性中间位肛门直肠畸形

随着对维持排便功能的神经肌肉的解剖生理以及肛门直肠畸形患儿肛周肌肉的病理改变的深入研究,肛门直肠畸形的手术方法日趋完善合理,尤其对中、高位肛门直肠畸形的手术疗效有了明显提高。现总结本院2002年4月至2008年4月采用改良Pena手术治疗的28例先天性中问位肛门直肠闭锁患儿的临床资料,报告如下：     

改良后矢状入路骶会阴肛门成形术Ⅰ期根治新生儿中位肛门直肠畸形

目的 总结我院2004年1月至2006年12月改良后矢状入路骶会阴肛门成形术Ⅰ期根治新生儿中位肛门直肠畸形的临床疗效.方法 38例新生儿先天性中、高位肛门直肠畸形,中位28例,高位10例;女5例,男33例.分为Ⅰ期组(中位畸形不伴严重畸形)和分期组(高位畸形和不符合Ⅰ期根治条件的中位畸形).Ⅰ期根治手术采用改良后矢状人路肛门成形术;分期手术采用造瘘-根治(Pena术)-关瘘.观察伴发畸形、胎龄和出生体重、根治手术时年龄和根治手术时间,比较总住院时间、总费用.统计并发症,随访术后肛门功能临床评分.结果 Ⅰ期组22例;分期组16例,10例高位畸形,6例中位畸形(2例早产儿,2例伴脊柱畸形,2例严重心脏畸形),两组平均胎龄、出生体重、第一次手术年龄和根治手术时间均无统计学差异(P＞0.05).近期并发症Ⅰ期组1例发生肠梗阻、肠穿孔,骶部伤口皮肤愈合情况与分期组相同;远期并发症分期手术较多,包括直肠黏膜脱垂和污粪.Ⅰ期组随访16例,术后肛门功能临床评分优为12例,良4例.Ⅰ期组住院时间(12.06±0.85)d,住院费用(10681.1±1759.5)元,显著低于分期组(P＜0.0001).结论 改良后矢状入路肛门成形术保留提肛肌与耻骨直肠肌环联合体,对新生儿中位肛门直肠畸形Ⅰ期根治手术临床近期疗效等同于以往的分期手术.     

腹腔镜辅助治疗中高位肛门直肠畸形疗效评价

目的 探讨腹腔镜辅助治疗中高位肛门直肠畸形的疗效.方法 回顾分析了2002年9月至2009年9月我科采用腹腔镜辅助肛门直肠成形术(LAARP)治疗的中高位肛门直肠畸形患儿34例,其中男27例、女7例.除1例一穴肛息儿和1例直肠尿道球部瘘患儿Ⅰ期行肛门成形术以外,其余患儿均在出生后行结肠造瘘术,腹腔镜主要用于腹部直肠末端的分离、瘘管的处理以及盆底隧道的形成,手术平均年龄为6.3个月.30例患儿术后获得随访,术后随访时间1～7年,平均3.5年,随访1～2年者11例,2～7年的病例19例,对30例患儿手术后并发症以及排便次数、大便控制能力、污便以及便秘的情况进行调查,并应用Kelly评分进行评价.结果 随访时间大于2年的病例中17例患儿获得较为满意的排便功能,其中5例患儿经过排便功能训练已达到完全正常;10例患儿可实现较为满意的排便控制,偶有污便(每1～2周一次),其中2例患儿出现轻度便秘;排便控制较差,经常有污便或失禁者2例.该组患儿经过有效的肠道管理均可以实现满意的社会生活,该组Kelly评分平均为4.32±1.45.随访时间1～2年的病例均有不同程度的污便,8例患儿排便控制能力较差,经常有污便.排便次数增多(大于3～4次);3例患儿排便控制功能较好,偶有污便,排便次数维持在每天I～2次.该组Kelly评分1.91±1.37.结论 腹腔镜治疗肛门直肠畸形疗效满意,并随着术后年龄的增长,排便控制功能显著提高.

Abstract：

Objective To evaluate postoperative anal functions of patients with intermediate or high anorectal malformations who underwent laparoscopic-assisted anorectal pull-through (LAARP).Methods Thirty four patients (27males、7 females) who underwent LAARP in our department between September 2002 and September 2009 were analyzed Initial colostomy in the newborn period was performed in all patients except 1 cloaca and 1 male patient with rectobular fistula. Laparoscope was used for mobilizing the rectum and repairing the fistula. The average age of operation was 6. 3 months (range from 2 to 24 months). Thirty patients were followed up successfully for 1 to 7 years (average 3. 5 years). Nineteen patients were followed up for more than 2 years while the other 11 patients less than 2 years. Complications and anorectal functions are assesed by questionnaires, including the fecal continence, the degree of soiling and the frequency of bowel openings. All patients were evaluated with Kelly score. Results Most of the patients who were followed up for more than 2 years have satisfactory fecal continence except for two cases. The average Kelly score was 4. 32 ± 1. 45. Patients who were followed up for less than 2 years had various degree of soiling. Eight patients had severe fecal incontinence and regular soiling, with bowel movements are more than 5 times per day. Only 3 patients had acceptable fecal continence, with occasional staining. The bowel movement was 1-2 times per day. The average Kelly score was 1. 91 ± 1. 37. Conclusions The procedure of LAARP can produce satisfactory fecal continence. The continence improves with age.     

先天性直肠肛门畸形影像学检查的研究进展

直肠肛门畸形为先天性畸形中较常见的一种,活婴发病率约1/5000。当合并其他先天性畸形如脊髓、椎管及泌尿生殖系统畸形时,常被称为尾部退化综合征,该综合征由Duham el于1961年首先报道,并定义为骨骼、泌尿生殖系及直肠肛门系贯性畸形,一方面表现为无足并腿畸胎,另一方面为肛门闭锁,对这种复杂先天性畸形儿的研究使人们认识到由于尾区胚胎发育受阻导致不同程度的畸形,通过人胚胎组织发育过程中尾区三维成像显示尾神经管与直肠肛门具有同源性,进一步揭示这种复杂畸形的相关联性。根据1984“W ingspread”分类,直肠肛门畸形分为高、中、低三类…     

经会阴治疗中、低位肛门直肠畸形

目的探讨经会阴(大-W)手术方法治疗中、低位肛门直肠畸形(AA)的价值,对其长期疗效进行评价。方法对46例中、低位AA患儿进行经会阴(大-W)肛门成形术。包括充分保留直肠末端的瘘管结构,保证直肠末端从由耻骨直肠肌、肛门外括约肌组成的横纹肌复合体中心拖下,会阴倒V形皮瓣应用。术后随访时间平均7年2个月。按照修订的Wingspread Scoring肛门功能评分标准评估排便功能。结果除1例中位畸形伴其他畸形,肛门功能较差外,余患儿肛门功能均良好。结论经会阴(大-W)AA术治疗中、低位AA简便易行,因保留瘘管末端,且对控制排便的有关结构损伤少,长期随访效果满意。     

腹腔镜辅助治疗高位肛门直肠畸形中期随访

目的 探讨腹腔镜治疗高位肛门直肠畸形的中期疗效.方法 回顾分析2002年9月至2009年1月我科采用腹腔镜辅助肛门直肠成形术(LAARP)治疗高位肛门直肠畸形患儿20例,其中男14例,女6例.男性患儿中直肠前列腺瘘8例、直肠膀胱颈部瘘6例,6例女性患儿均为高位一穴肛患儿,共同管长度大于3 cm.腹腔镜用于直肠末端分离、瘘管处理以及盆地隧道的形成.20例患儿手术时的平均年龄为5.9个月(2～24个月),术后均获得随访,术后随访时间平均3.8年(2.5～7年).对20例患儿术后并发症以及肛门直肠功能(排便次数、大便控制能力、污便以及便秘情况)进行调查,并应用Kelly评分对肛门直肠功能进行评价.结果 3例患儿经过排便训练可以达到完全的排便控制无污便和便秘;13例患儿可实现较为满意的排便控制,偶有污便(每1～2周1次),无便秘发生.排便控制较差,排便次数增多,经常有污便或失禁者4例.该组患儿经过有效的肠道管理均可以实现满意的社会生活,该组kelly评分平均为3.9±1.59.结论 腹腔镜辅助治疗高位肛门直肠畸形可获得较为满意的疗效,长期的疗效结果仍然需要进一步观察.     

重视先天性肛门直肠畸形术后并发症治疗与长期转归问题

先天性肛门直肠畸形(ARMs)的发生率约占成活新生儿的1/3500,经数十年外科手术技术沿革,其转归有明显改善,但仍在排尿与控便上存在一定的问题。况且90年代前曾施行过腹会阴手术而致尿、粪失禁的病儿现已处于青少年期或已达成年期,对患者、家属及社会带来很大影响。因此,有必要重视这一患者群体。     

先天性巨结肠和先天性肛门直肠畸形治疗的新进展

近年来 ,随着科学技术水平的提高和临床基础研究的发展 ,特别是随着多种治疗方法长期随访结果的逐步明确 ,对小儿肛肠外科疾病的治疗方法提出了一些新的观点 ,有了新的改进。一、先天性巨结肠的治疗1.手术方式的改进　传统的几种手术方式Ｓｗｅｎｓｏｎ、Ｄｕｈａｍｅｌ和Ｓｏａｖｅ及其各种改良术式受到挑战 ,而腹腔镜辅助下的直肠内结肠拖出术和单纯经肛门直肠内拖出术日益受到广大小儿外科医生的接纳和采用。此手术不但对患儿创伤小 ,切口美观 ,而且有切除痉挛段粘膜彻底 ,吻合口低且能同时处理肛门内括约肌病变等优点。手术的关键如下 :…     

One-stage correction of intermediate imperforate anus in males

This prospective study was designed to assess the safety, cost-effectiveness, and advantages of performing posterior sagittal anorectoplasty (PSARP) without colostomy on males with intermediate imperforate anus in a developing country. Fifteen consecutive males with intermediate imperforate anus were entered into the study. Chest and abdominal x-rays, skeletal surveys, renal ultrasound scans, and invertograms were done. Patients were resuscitated and Peñas PSARP done in prone positions. A 2-ml syringe vent was inserted into the new anus for 10 days. Babies were nursed prone postoperatively. Cephalosporin and metronidazole were given as perioperative antibiotics. All patients had intermediate anomalies. There were no other major associated congenital anomalies. A urethral catheter could not be inserted in one patient, and one patient who presented with septicemia and jaundice was deemed too ill to withstand a major operation; these two patients therefore had diverting colostomies. There were no problems with PSARP in the other 13 patients. One patients father discharged him against medical advice on the 5th postoperative day; the mother had had postpartum hemorrhage, so they opted for traditional treatment because they could not provide blood donors. The skin wounds of 10 patients healed completely with removal of stitches; two boys had superficial wound infection. Parents who lived far from the hospital were taught how to dilate the anus. Follow-up has ranged between 3 months and 2 years. This prospective study shows that it is feasible for males with intermediate imperforate anus to have safe PSARP without colostomy. The advantages of one instead of three major operations are many, especially in developing countries. If this result can be reproduced in cases of high anomalies, colostomy may be unnecessary in many cases of anorectal malformations, with many benefits to these children and their families.     

Internal anal sphincter function after correction of imperforate anus

Thirteen patients with high or intermediate types of imperforate anus have been followed postoperatively by anorectal manometry after surgical correction. Since it is our belief that an internal sphincter anlage is present in the fistula, this was preserved and transplanted to the position of the neoanus. In cases with a blind end and no fistula, thickening of the circular smooth muscle layer was sought and transposed in the same way. Ten of 13 patients had a positive rectoanal inhibition reflex and normal anal pressure, indicating the presence of an internal sphincter. The amplitudes of the relaxations were not different from those of healthy children of the same ages. The children in this study were too young for proper clinical evaluation, but internal sphincter function may be an important factor for their future continence. Offprint requests to: B. Frenckner     

The use of augmented-pressure colostography in imperforate anus

Colostomy is recommended in most newborns with imperforate anus (IA), except those with low-type anorectal malformations (ARM). Accurate demonstration of the anatomy of any associated fistula between the rectum and the urogenital tract is essential for optimal surgical management. Augmented-pressure distal colostography is recommended to fulfill this requirement prior to definitive surgical repair of IA. We examined 12 cases of high or intermediate-type ARM using this technique, which clearly demonstrated the distal perineal anatomy. All were confirmed at posterior sagittal angorectoplasty.     

Spectrum of genitourinary malformations in patients with imperforate anus

A retrospective review of 244 cases of imperforate anus demonstrated a 48% incidence of associated genitourinary (GU) anomalies. The incidence and severity of associated GU anomalies was directly related to the level of the fistula between the blind-ending rectum and the GU tract. High-level fistulae to the bladder neck in males and the cloaca in females demonstrated a 90% incidence of associated genitourinary anomalies. In contrast, lower-level fistule to the perineum revealed only a 14% incidence of associated GU anomalies. Classification of anorectal anomalies by fistula level provides an accurate means of predicting the likelihood of an associated GU malformation.     

Computerised tomography in the management of imperforate anus patients following rectoplasty

Eighteen patients with surgically corrected high (supralevator) anorectal anomalies were scanned by computerised tomography (CT) to assess the state of development of the anorectal sphincter muscle complex (SMC) and the position of the neorectum in relation to the SMC. These CT findings were compared with the patients' assessed grade of faecal continence/incontinence. While continence levels did not always correlate closely with CT findings, the presence of a deficient SMC or an eccentric position of the neorectum within it, or both, was more frequent in patients with continence problems. For severe incontinence, CT scanning gave helpful indications for or against secondary rectoplasty. Offprint requests to: R. Fowler     

Children with high and intermediate imperforate anus: their experiences of hospital care

Purpose

The purpose of this study was to examine the experiences of children with high and intermediate imperforate anus (IA), and specifically their experiences of hospital care.

Methods

Twenty-five children born with high and intermediate IA participated; 9 boys and 16 girls. The mean age was 10.5?years (range 8.0?C13.6). Two control groups were involved in the study. A self-report questionnaire was used to gather the data concerning children??s experiences of hospital care. Items were scored on a 5-point Likert scale.

Results

The children??s responses on hospital care items scored high. The children with IA reported being less satisfied with the information given, and understood less why they needed to visit the hospital than did the children in the two control groups.

Conclusion

The children??s experiences of care seemed to be positive even though the children born with IA are subjected to invasive treatment. More research is called for in the unexplored area of information to the children, and particularly to the children born with a defect. Children??s views are important and should always be considered, as their answers most certainly reflect a genuine experience, contributing to the further development of their specific care.     

腹腔镜下高位肛门闭锁肛门成形术及中期临床评估

目的 评估腹腔镜下高位肛门闭锁成形术后临床疗效和直肠肛门功能.方法 2004年6月至2007年9月收治高位肛门闭锁患儿61例,33例行腹腔镜下肛门成形术(LAARP),平均年龄5.3个月;28例行后矢状入路肛门成形术(PSARP),平均年龄4.9个月.随访包括手术时间、住院时间和并发症.手术后3～4年对患儿进行排便功能的Kelly评分(KCS)、磁共振成像(MRI)和直肠肛管向量测压(AVVM)评估.结果 LAARP和PSARP组手术时间分别为(112.5±12.4)min和(120.4±18.5)min(P＞0.05),LAARP组住院时间(11.3±2.1)d短于PSARP组(14.6±2.3)d(P＜0.01).两组患儿KCS无显著差异(3.52±1.42比3.49±0.82,P＞0.05).MRI显示:LAARP组33例患儿中在I线上和M线上各有1例存在直肠位置偏移;PSARP组28例患儿中I线上有4例存在直肠位置偏移,M线上有3例存在直肠位置偏移.直肠肛管向量测压结果显示:与PSARP组相比,LAARP组非对称指数小,向量容积大,静息时和收缩时肛管压力高(P＜0.05).但高压带长度(15.2±5.8比15.1±6.2 mm)和直肠肛管抑制反射阳性率(84.8%比85.7%)无显著差异.结论 高位肛门闭锁患儿LAARP术后排便控制满意,与PSARP相比,LAARP术后住院时间短、直肠位置更准确.长期随访对评估LAARP术后功能非常必要.

Abstract：

Objective To evaluate the clinical outcomes and postoperative anal function in infants with congenital high imperforate anus who underwent laparoscopically assisted anorectal pullthrough (LAARP). Methods From January 2004 to July 2007,33 consecutive patients (28 males and 5 females,age ranging from 3 to 10 months) with high imperforate anus underwent LAARP. Clinical data of the LAARP group were retrospectively compared with those treated by posterior sagittal anorectoplasty ( PSARP,n = 28) at the same time period. Anorectal function of these patients was evaluated using the Kelly's score,anorectal vector volume manometry(AWM) and magnetic resonance imaging (MRI) at the age of 3. 1 to 4. 4 years. Results The operative time in LAARP and PSARP groups was 112. 5 ± 12.4 and 120.4 ± 18.5 min (P ＞ 0. 05), respectively. The length of hospital stay in LAARP group was shorter than that of PSARP group (11. 3 ± 2. 1 vs. 14. 6 ± 2. 3 days,P＜0. 01). No significant difference was observed between LAARP and PSARP groups regarding Kelly's score (3. 52 ± 1. 42 vs. 3. 49 ± 0. 82). Although MRI revealed the lower rate of poorly aligned rectum in LAARP group than PSARP group at both I-line (3. 0% vs. 14. 3%) and M-line (3. 0% vs. 10. 7%) levels,no statistically significant difference was noted (P＞0. 05). Compared with the PSARP group, lower asymmetric index, larger vector volume, and higher anal canal pressure at rest and during voluntary squeeze were observed in LAARP group (P＜0. 05), without significant differences in the length of high-pressure zone (15. 2 ± 5. 8 vs. 15. 1 ± 6. 2 mm) and the presence of rectoanal relaxation reflex (84. 8% vs. 85.7%). Conclusions Satisfactory fecal continence can be achieved in patients with high/intermediate type imperforate anus after LAARP. LAARP has some advantages over PSARP, including shorter hospital stay and better position of rectum. However, long-term follow-up is necessary to compare the benefits of LAARP against PSARP.     

一期与分期腹骶会阴肛门成形术治疗高位肛门直肠畸形的疗效比较

目的：比较腹骶会阴肛门成形一期手术与三期常规手术在治疗新生儿高位肛门直肠畸形中的疗效。方法回顾性分析本院收治的48例采用腹骶会阴肛门成形手术的新生儿先天性高位肛门直肠畸形患儿临床资料,分别采用一期手术（A组）和三期手术（B组）进行治疗,比较两组疗效。结果 A组21例均接受一期手术,2例失访,19例术后肛门功能优良率87．72％;B组27例中,肠造瘘后放弃后续手术或失访4例,23例全程完成三期手术,术后肛门功能优良率88．41％,两组疗效比较差异无统计学意义（P＞0.05）。结论选择性一期腹骶会阴肛门成形术治疗高位肛门直肠畸形同样可达到常规三期手术的效果,且有护理方便、病程短、费用低的优点。