MRI findings in patients with defecatory dysfunction after surgical correction of anorectal malformation

Background

Postoperative anorectal malformation patients frequently have defecatory dysfunction. MRI may be useful in the management of these patients.

Objective

To analyze static and dynamic MRI findings in patients with defecatory dysfunction after correction of anorectal malformation (ARM), and compare differences between patients with constipation and fecal incontinence.

Materials and methods

Pelvic MRI studies of 20 constipated and 32 incontinent postoperative ARM patients were analyzed retrospectively to determine the location and morphology of the neorectum, presence of peritoneal fat herniation, presence of scarring, development of the striated muscle complex (SMC) and any other abnormalities. The two groups were then compared using χ ²-test. Eighteen patients also underwent MRI defecography to evaluate pelvic floor function and abnormalities are reported.

Results

The children with incontinence were more likely to have abnormal location of the neorectum (P = 0.031), increased anorectal angle (ARA) (P = 0.031) and peritoneal fat herniation (P = 0.032), and less likely to have dilation of the neorectum (P = 0.027), than the children with constipation. There were no significant differences between the two groups in incidence of focal stenosis of the neorectum (P = 0.797), presence of extensive scarring (P = 0.591) and developmental agenesis of the SMC (P > 0.05). MRI defecography showed 6 anterior rectoceles, 6 cystoceles and 18 pelvic floor descents.

Conclusions

MRI is a helpful imaging modality in postoperative ARM patients with defecatory dysfunction, and it shows distinct differences between the children with constipation and incontinence and provides individualized information to guide further treatment.     

大鼠肛门直肠畸形的胚胎发育研究

目的：观察大鼠正常的和畸形的肛门直肠发育过程，探讨肛门直肠畸形的胚胎发生机制。方法：用乙烯硫脲（ethylenethiourea,ETU）致畸21只孕鼠共生产泄殖腔畸形和无肛畸形胎鼠（204只）。胎龄12．5－20d的正常（n=223)及泄殖腔发育畸形的胎鼠正中矢状连续切片HE染色。连续动态对比观察泄殖腔及肛门直肠的发育过程。结果：泄殖腔发育过程中，胎龄15d前的正常鼠胚，尿直肠隔将泄殖分为腹侧的尿生殖窦和背侧的直肠两部分，二者共同相通于泄殖腔管，尿直肠隔下降与泄殖腔膜的距离逐渐减小，直至胚胎15d时与泄殖腔膜融合，直肠与膀胱分离。16d时肛膜破裂，直肠与尾沟相通。ETU致畸的胎鼠泄殖腔发育中有以下下特点；（1）尿直肠隔始终未与泄殖腔膜融合；（2）泄殖腔构型异常；（3）泄殖腔膜过短；（4）没有尾沟形成。ETU致畸胎鼠中所有的无肛畸形均为泄殖腔畸形和直肠尿道瘘。结论：鼠胚胎15d时尿直肠隔与泄殖腔膜融合。胚胎16d肛膜完全破裂，直肠与外界相通。泄殖腔构型的变化以及尿直肠隔下降并与泄殖腔隔合使直肠与膀胱分离。尿直肠隔与泄殖腔膜融合是直肠和膀胱分离的决定因素。肛门直肠畸形发生与泄殖腔构型异常以及尿直肠隔未与泄殖腔膜融合等因素有关。尿直肠隔未与泄殖腔融合是ETU诱导的大鼠肛门直肠畸形发生的直接原因。     

肛门直肠畸形患儿术后30年以上生活质量观察

目的 调查肛门直肠畸形患儿术后30年以上生活质量状况。方法 随访8例肛门直肠畸形患者（年龄33－41岁）。调查患者一般状况如文化程度、婚姻家庭状况等,应用自行设计的肛门直肠畸形患儿术后30年以上生活质量问卷,评估该8例患者和对照组（24例健康成人）的生活质量状况。包括排便功能、饮食、工作、性生活、社交娱乐、心理情绪等6方面10项内容。结果 8例患者均结婚并有自己的子女。6例存在排便功能障碍。2例患者社会工作因此受到影响,3例患者性生活受到限制,4使例患者因排便功能障碍经常忧愁和烦恼。根据肛门直肠畸形患儿术后30年生活质量问卷,患儿组生活质量评分显著低于正常对照组。结论 肛门直肠畸形患儿术后排便功能障碍影响患儿生活质量。肛门直肠畸形患儿术后远期生活质量低于正常人。应重视肛门直肠畸形患儿的手术疗效,应用生物反馈疗法等康复治疗,进行长期规律随访,提高其生活质量。     

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。     

Gluteus maximus flap transplantation for anrectal dysfunction after anorectal malformation operation

目的 评价臀大肌瓣转移肛提肌加强术治疗肛门直肠畸形术后排便功能障碍的恢复情况.方法 选择28例先天性肛门闭锁术后排便功能障碍的患儿,年龄4～14岁,男19例,女9例,单纯肛提肌功能不良的18例,肛提肌和肛门外括约肌功能均不良的10例,全部行臀大肌瓣转移肛提肌加强术,术前均行盆底肌MRI、钡灌肠及臀大肌肌电图检查,手术前后均行结肠传输试验、肛门直肠测压和排便功能评分.术后对患儿进行6～48个月的随访,平均24个月.结果 28例手术患儿中26例获得随访,术前臀大肌肌电图均显示臀大肌功能良好,钡灌肠显示直肠无扩张,结肠传输试验表现为出口梗阻,盆底肌MRI见28例患儿肛提肌发育薄弱或不对称,排便功能评分结果示28例患儿均差.肛管静息压手术前后分别为(16.77±4.25) mmHg和(18.67±4.48)mmHg、肛管最大收缩压在手术前后分别为(61.05±14.37) mmHg和(72.12±16.76)mmHg,差异均无统计学意义(P＞0.05),术后单纯肛提肌功能不良的排便功能评分达优6例,良10例,肛提肌和肛门外括约肌功能均不良的排便功能评分优0例,良2例,差8例.优良率占69.23％.结论 对于肛提肌功能不全所导致的排便功能障碍,行臀大肌瓣转移肛提肌加强术可改善排便功能,部分括约肌功能不良的患儿术后可能失禁不能改善或者加重,需要进一步的手术或训练治疗.     

先天性无肛畸形儿MR成像及其与排便功能关系研究

目的评估先天性肛门直肠畸形儿（anorectal malformations，ARM）尾端发育情况及横纹肌复合体（sphincter muscle complex，SMC）发育状态与排便功能的关系。方法39例ARM，男28例，女11例，应用不同序列体部相控阵列线圈或头部线圈MRI显示ARM直肠闭锁水平、骶尾椎、骶髓、泌尿生殖系的发育情况，并对SMC从多个层面进行定量分析，评估其发育状态与临床评分和肛门功能客观检查的关系。结果39例患儿中，MRI T1WI、T2WI扫描判断畸形水平的正确诊断率为100％，T2WI扫描瘘管检出率为96．9％。骶尾椎和骶髓的异常占41．0％，泌尿生殖系统畸形占20．5％。当耻骨直肠肌宽度的相对值PRWR〈0．18，外括约肌宽度的相对值EASWR〈0．15时，71．4％的ARM术后出现肛门失禁。当PRWR〉0．18，EASWR〉0．15时，91．3％的ARM术后排便功能好。结论MR检查能清楚地显示ARM类型、闭锁水平、SMC发育状态、脊柱脊髓及泌尿生殖系统存在的伴发畸形，从MR影像中找到了评价SMC的定量指标。     

高位和中间位肛管直肠畸形的治疗选择

目的 评价新生儿期后矢状路肛门直肠成形术（PSARP）治疗高位和中间位肛管直肠畸形的安全性、可行性及长期效果。方法随访113例高位和中间位肛管直肠畸形患儿,其中48例手术分Ⅲ期（结肠造瘘-后矢状路肛门直肠成形术关瘘,A组）,65例新生儿期行后矢状路肛门直肠成形术（B组）。根据改良Wingspread评分方法,肛管直肠功能分为“优”、“良”、“一般”和“差”。根据钡灌肠,肛管直肠角分为“清晰”、“模糊”、“无”;同时观察钡剂向外泄漏情况。肛管直肠测压测定肛管静息压,肛管收缩压及肛管直肠反射。结果A组,58．3％（28／48）的病例表现“优”或“良”;钡灌肠,85．4％（41／48）的病例显示肛管直肠角“清晰”,10．4％（5／48）的病例有钡剂外泄。B组,53．8％（35／65）的病例表现“优”或“良”;83．1％的病例显示肛管直肠角“清晰”,7．7％（5／65）的病例有钡剂外泄。早期手术并发症,A组为56．3％（27／48）,其中39．6％（19／48）与结肠造瘘有关;B组为29．2％（19／65）。A组与B组便秘发生率分别为47．9％（23／48）和44．6％（29／65）,污粪为47．9％（23／48）和50．8％（33／65）。结论新生儿期后矢状路肛门直肠成形术（PSARP）治疗高位和中间位肛管直肠畸形是安全可行的,且长期效果与Ⅲ期PSARP手术相同。     

Retrospective study of reoperation for congenital anorectal malformation

目的 分析先天性肛门直肠畸形再手术的临床资料,总结肛门成形术后并发症的治疗及预防.方法 回顾总结2003年1月至2010年9月我院收治的55例先天性肛门直肠畸形肛门成形术后再手术的临床资料,再手术年龄为2～204个月,平均为50.33个月;根据以往病史低位畸形6例,中位13例,高位16例,一穴肛1例,病史不明19例.结果 29例(52.7％)曾接受Ⅰ期肛门成形术,26例曾肠造瘘后行肛门成形术.再手术的原因和手术方法为19例因直肠黏膜脱垂严重行脱垂直肠黏膜切除术;17例术后出现直肠尿道瘘复发,其中16例经Ⅱ期手术治愈,1例经Ⅲ期手术治愈;28例存在肛门狭窄或/和失禁,19例因严重的管形直肠狭窄或初期成形的肛门位置不在肛门外括约肌收缩中心位置而接受再次肛门成形术.结论 大部分肛门直肠畸形术后并发症是可预防或减轻的,首次手术时机和方式的正确选择是预防并发症的最佳方法;对于并发症的再手术应慎重,不同并发症采用不同的针对术式亦可获得良好疗效.     

EphB2在肛门直肠畸形中的表达及其意义

目的 研究EphB2在先天性肛门直肠畸形直肠末端组织中的表达并探讨其临床价值.方法 采用免疫组织化学法和免疫荧光分析方法检测20例先天性肛门直肠畸形患儿直肠末端肠壁和8例正常对照直肠末端肠肇的EphB2的表达情况.结果 免疫组织化学检测到EphB2蛋白在畸形组和对照组均有表达,定位在直肠肠壁黏膜层细胞的胞质和胞膜中.畸形组EphB2表达的累积光密度值(30.43±11.41)明显低于对照组(81.62±20.45),差异有统计学意义(P<0.01).结论 EphB2在肛门直肠畸形的直肠末端黏膜层表达水平减低,EphB2的表达可能和先天性肛门直肠畸形的发生密切相关.     

先天性肛门直肠畸形胎鼠腰骶髓副交感神经元发育异常的研究

目的　探讨先天性肛门直肠畸形是否同时存在副交感神经支配异常以及对术后排便功能的影响。方法　 2 0只Wistar孕鼠 ,分为ETU致畸组 (15只 )和对照组 (5只 )。孕 2 0d时 ,将荧光金注射入正常胎鼠直肠壁及畸形胎鼠的直肠盲端肌层内。荧光显微镜下观察副交感神经元的数量 ,形态和分布。结果　①致畸组胎鼠手术存活率为 6 0 .0 % ,而对照组存活率为 71.4 % ;②对照组 :荧光金标记的副交感神经元位于脊髓侧角 ,分布在L6到S1脊髓节段 ,标记神经元数量为 (90 1± 4 6 )个。神经元体积较大。ETU致畸组中外观正常组、低位畸形组、高位畸形组标记神经元数量分别为 (899± 36个 )、(5 88± 10 2 )个和 (2 4 2± 92 )个 ,畸形组与正常组、外观正常组相比 ,统计学差异显著 ,而且神经元细胞体积明显减小。结论　①先天性肛门直肠畸形胎鼠骶髓副交感神经元数目减少 ,体积减小 ,呈发育不良改变。畸形位置愈高 ,神经元数量减少愈明显 ;②本研究结果提示人类先天性肛门直肠畸形术后便秘的发生可能与副交感神经元数量减少有关。     

先天性肛门直肠畸形大鼠胚胎盆底肌发育的形态学研究

目的 观察乙烯硫脲致畸的肛门直肠畸形大鼠模型横纹肌复合体(SMC)胚胎发育规律及演变过程,为探讨肛门直肠畸形患儿术后排便功能障碍的发病机制提供理论基础.方法 以乙烯硫脲制作肛门直肠畸形大鼠模型,取不同胎龄(16、18、20、21 d)的畸形组和对照组胚胎,行矢状面、冠状面及横断面连续切片,HE染色和Myogenin免疫组化染色,观察正常及肛门直肠畸形(ARMs)大鼠胚胎SMC的发生及演变过程.结果 16d时SMC较为松散地环绕直肠末端,畸形组的SMC的位置、形态及走行与正常组非常相似.从18d开始正常组肌结构明显增粗,与直肠关系渐紧密,畸形组SMC明显向头侧、腹侧及中线移位,形成一聚集于尿道后方,向头侧、腹侧和中线移位的紧缩环结构,SMC肌束间充填大量的脂肪组织,结构紊乱.这一形态改变在此后的胚胎发育过程中(18～21 d)持续存在.结论 在正常及ARMs的大鼠胚胎中,SMC最初于16 d可以观察到.ARMs大鼠胚胎的SMC在16 d时位置、形态及走行与正常组无明显差异,从18d起开始出现异常,表现为向腹侧、头侧及中线移位,以后延续上述改变,最终表现为SMC位置、形态异常,肌束间脂肪组织增多.SMC的形态改变晚于肛门直肠畸形的发生.     

肛门直肠畸形合并先天性巨结肠的诊治

目的 总结儿童先天性肛门直肠畸形(ARM)合并先天性巨结肠(HD)的临床特点,探讨其病因和适宜的诊治方法.方法 回顾性分析2004年1月至2008年4月6例ARM合并HD患儿的临床资料、诊治过程及预后情况.年龄1岁8个月～1I岁,平均4.1岁;男女比例1:5.所有病例于ARM矫正手术后有不同程度便秘、腹胀等症状,钡灌肠显示结直肠均有显著的扩张,仅有2例可见明确痉挛段和移行段.6例患儿直肠肛管测压直肠肛门抑制反射不能引出.2例采用经肛门Soave术式,4例采用经腹肛门Soave巨结肠根治术.结果 术后病理检查6例标本远端肠壁内均未见神经节细胞.免疫组化组织蛋白酶D:近段阳性,远端阴性.结论 对于ARM患儿,特别是对于ARM畸形矫正术后仍有便秘的患儿要警惕合并HD的可能.此外,ARM合并HD患儿往往同时存在有多种畸形,在胚胎发育过程中这些畸形的发生可能存在着一定的关联.     

先天性无肛畸形Gli2基因表达的研究

目的：探讨先天性肛门直肠畸形直肠末端SHH转录反应因子Gli2基因表达水平与畸形的关系。方法：采用RT－PCR方法检测16例不同类型先天性肛门直肠畸形直肠后壁末端及8例正常直肠后壁末端Gli2的表达水平,应用t检验比较正常组与畸形组、不同畸形类型之间Gli2表达水平的差异。结果:肛门直肠畸形直肠后壁末端Gli2的表达水平明显低于正常直肠（P＝0．01）,各不同类型畸形之间Gli2表达水平无明显差异（P＞0．05）。结论：Gli2表达水平减低与肛门直肠畸形的发生有关,还有其他机制参与该畸形的发生。     

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.     

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.     

神经生长因子及其受体在先天性肛门直肠畸形患儿直肠末端表达的下调

目的 观察神经生长因子(nerve growth factor,NGF)及其受体(TrkA、P75NTR)在先天性肛门直肠畸形(anorectal malformation,ARM)患儿直肠末端的表达,探讨对其肠神经系统发育的影响.方法 收集2011年1月至2013年1月遵义医学院附属医院诊治的ARM患儿直肠末端标本30例.其中,男24例,女6例;高位ARM 15例,中位ARM 6例,低位ARM 9例;手术年龄2 d～13个月,平均1.4个月.一期腹会阴肛门成形8例;分期肛门成形13例,包括:腹腔镜辅助肛门成形7例,腹会阴肛门成形2例,后矢状入路肛门成形术4例,会阴肛门成形9例.肛门成形术中取直肠末端标本,运用苏木素/伊红(HE)染色计数直肠末端黏膜下及肌间神经丛数量,免疫组织化学(IHC)及实时定量聚合酶链反应(qRT-PCR)检测NGF及其受体(TrkA、P75NTR)在低位ARM和中高位ARM患儿直肠末端的表达情况.结果 HE染色:低位ARM患儿直肠末端黏膜下及肌间神经丛分布密集,神经丛面积大,节细胞数量多;中高位ARM患儿神经丛分布明显稀疏,神经丛数量较低位ARM患儿明显减少(1.30±0.82vs5.60±1.36)个,两相比较,差异有统计学意义(P＜0.01).IHC:NGF、TrkA、P75NTR主要在直肠末端黏膜下及肌间神经丛表达,NGF主要定位于胞核,TrkA主要定位于胞核及胞质,P75NTR主要定位于胞核、胞膜.NGF、TrkA、P75NTR在低位ARM患儿直肠黏膜下、肌间神经丛大量表达,其积分光密度(IOD值)分别为43.48±7.11、18.83±3.65和23.39±3.64;NGF、TrkA、P75NTR在中高位ARM患儿直肠末端表达明显减少,其积分光密度(IOD值)分别为3.70±1.21、8.49±2.68和7.17±2.28,两相比较,差异均有统计学意义(P＜0.01).qRT PCR:NGF、TrkA、P75NTR mRNA在低位ARM患儿直肠末端的相对表达量分别为(172.22±104.38、148.78±72.45和122.83±35.45);明显高于中高位ARM患儿(80.38±43.10、73.95±47.43和75.15±20.23),两相比较,差异均有统计学意义(P＜0.01).结论 NGF及其受体TrkA、P75NTR可能参与并影响ARM患儿直肠末端肠神经系统的发育.     

Utility of spinal MRI in children with anorectal malformation

Background  The association between spinal cord anomalies and imperforate anus is well recognized. Until now, the incidence of tethered cord has been assumed to be higher in patients with high-type imperforate anus. However, recent reports suggest that tethered cord is as common in patients with a low lesion as in those with a high lesion. Objective  To review the incidence of spinal cord anomalies in those with a low lesion and those with a high (including intermediate) anorectal malformation (ARM), and to determine the best diagnostic imaging strategy. Materials and methods  A group of 50 consecutive patients with postoperative ARM and in whom spinal MRI had been performed were identified retrospectively. We reviewed and compared the following factors between those with a high lesion and those with a low lesion: (1) clinical symptoms, (2) spinal cord anomalies, and (3) vertebral anomalies. Results  The incidence of spinal cord anomalies was no different between those with a high lesion and those with a low lesion, and spinal cord anomalies were present regardless of the presence of vertebral anomalies or symptoms. Conclusion  Owing to the high incidence of spinal cord anomalies in patients with imperforate anus, MRI is the best imaging tool for detecting such anomalies regardless of the level of the lesion.     

直肠肛管向量测压对术后肛门括约肌功能状态评价的研究

目的　探讨向量测压对先天性直肠肛门畸形术后肛门括约肌功能状态的定性及定量评价。方法　采用连续、匀速牵拉的向量测压方法研究正常组及先天性直肠肛门畸形术后组儿童的向量测压结果及其与临床评分的相关性。结果　无肛术后患儿向量容积减小 ,非对称指数增大。临床评分与向量容积呈正相关 (收缩时R =0 .6 0 17)。优良组的收缩向量容积大于 12 9cm·(cmHg) 2 ,收缩非对称指数小于 37%。结论　无肛术后向量容积图可分为钟形 ,近似钟形及不规则形三种形态 ,据此可以定性评价肛门括约肌压力的分布及强弱。无肛术后患儿临床评分与向量容积呈正相关 ,当收缩向量容积大于 12 9cm·(cmHg) 2 ,非对称指数小于 37%时 ,提示可能获得较高的临床评分。     

Neurovesical dysfunction in children after surgery for high or intermediate anorectal malformations

Aim: To evaluate problems with bladder dysfunction in patients operated on for imperforate anus. Methods: All patients (11M, 25F) with high or intermediate imperforate anus seen in this institution between 1987 and 1997 were subjected to the investigation. They had all undergone a posterior sagittal anorectoplasty procedure with the aim of preserving as much as possible of the fistula. Hospital charts were reviewed. A detailed and structured interview regarding micturition habits was performed. If the results of this were abnormal the patients were also subjected to flow registration, measurements of residual urinary volume and in some cases also cystometry. Results: Micturition habits were normal in 30 out of 36 children. The remaining six, considered to have neurovesical dysfunction (NVD), had difficulties in emptying their bladder and exhibited pathological residual volumes. Two were boys with rectourethral fistulae, two were girls with cloacal malformations and two were girls with rectovestibular fistulae. Four out of six children with NVD had sacral anomalies.

Conclusion: NVD may occur even in the absence of sacral anomalies. A 4 h micturition observation is recommended in all newborns with intermediate or high anorectal anomalies to recognize the occurrence of NVD at an early stage.