直肠肛门抑制反射诊断先天性巨结肠符合率的临床分析

目的分析3179例便秘患儿直肠肛管测压结果,探讨直肠肛门抑制反射在先天性巨结肠诊断中的价值。方法回顾性分析2006年10月至2011年10月作者所行直肠肛门测压的3179例患儿检查资料,并与钡灌肠以及术中术后病理检查结果进行对比。结果HD患儿直肠肛门抑制反射诊断符合率为95．66％。在直肠肛门抑制反射阳性,而临床症状和体征符合HD的病例中,后经X线钡剂灌肠和直肠黏膜活检确定为常见型HD5例,短段型HD3例,特殊型HD1例。结论直肠肛门抑制反射可能存在假阳性,对假阳性病例建议重复检测,以降低漏诊率。     

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SoaveⅠ期拖出根治术治疗小婴儿先天性巨结肠

目的 总结经肛门SoaveI期拖出根治术治疗小婴儿先天性巨结肠的手术过程、疗效及短期随随访。方法 自1999年经该术式治疗小婴儿先天性巨结肠14例。均经钡剂灌肠和直肠粘膜吸引活检诊断为常见型巨结肠。结果 14例切除结肠17～24cm,均于术后2～4d开始进食,术后1个月随访;每日排便8～10次,其中9例行钡剂灌肠未见结肠扩张,24h钡剂潴留;6个月随访（12例）每日排便1～3次,无污染。结论 经肛门SoaveI期拖出根治术治疗小婴儿常见型先天性巨结肠,手术创伤小、操作简单、近期疗效良好。     

经肛门Soave巨结肠根治术后肛门功能与直肠肛管测压的研究

目的探讨经肛门Soave巨结肠根治术后患儿直肠肛管测压改变及排便控制情况。方法63例经肛门Soave巨结肠根治术患儿,术后平均随访3年(6个月～4.5年),对患儿大便控制能力、便秘发生情况及肛门直肠测压结果进行评价。结果获访47例,大便控制优15例,良26例,差6例。2例患儿有持续性便秘,8例曾出现肠炎。肛门直肠测压检查2例恢复直肠肛管抑制反射。47例随访患者的直肠静息压与正常对照组无显著性差别(P>0.05),但6例大便控制差的患者肛管高压区长度较正常对照组下降(P<0.05)。结论应用经肛门Soave巨结肠根治术治疗小儿巨结肠,术后可获得较好的排便控制功能。少数病例排便功能障碍可能与内括约肌功能受损有关,直肠肛管测压是重要的评估依据之一。     

直肠肛管测压诊断先天性巨结肠的临床研究

应用测压检查观察50例正常新生儿的直肠肛管反射,结果96%于生后6天内出现正常反射。同时。检测156例便秘病儿,104例呈病理反射,52例为正常反射。经X线钡灌肠及手法等方法表明,先天性巨结肠的测压诊断率可达95.8%,但对新生儿的诊断率不高。因此测压检查应结合临床及其他检查作出诊断。对于特发性巨结肠与超短段先天性巨结肠,测压检查不失为有效鉴別方法。     

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］     

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。     

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。     

结肠次全切除术治疗巨结肠同源病23例

目的探讨小儿巨结肠同源病(HAD)的诊断和治疗方法。方法回顾性分析23例巨结肠同源病的病例资料,男8例,女15例,年龄5个月～16岁,其中5个月～3岁4例,3～16岁19例。患儿均以便秘、腹胀为主诉,术前常规行钡灌肠、直肠肛管测压检查。其中11例行直肠粘膜活检术;5例因肠梗阻行结肠造瘘术;3例为先天性巨结肠术后复发便秘。结果全部病例均行腹会阴Soave法结肠次全切除术。23例中,钡剂灌肠均未见明显狭窄段、移行段,24h延迟拍片提示钡剂滞留;部分病例有结肠扩张和结肠冗长表现;直肠肛管测压均有抑制反射,11例直肠粘膜活检AchE阴性。23例术后病理诊断与术前诊断一致。术后均有不同程度腹泻,1例出现伤口裂开,2例直肠粘膜脱垂,2例粘连性肠梗阻;无吻合口瘘、肌鞘感染等并发症。结论腹会阴Soave法结肠次全切除术是治疗HAD较为彻底的术式。     

经肛门结肠拖出根治小儿乙状结肠冗长症

目的总结经肛门结肠拖出根治小儿乙状结肠冗长症的手术过程、疗效及短期随访。方法我院2000～2005年经该术式治疗小儿乙状结肠冗长症19例，乙状结肠冗长症有典型的便秘症状，钡灌肠显示19例乙状结肠比正常对照组平均长25cm；直肠肛管测压显示直肠肛管松弛反射存在，呈“W”型特殊波型；组织化学检查无乙酰胆碱酯酶阳性的副交感神经纤维。结果19例经肛门拖出切除结肠肠管20～52cm，平均切除结肠肠管28cm。均于术后2～3d开始进食，术后1个月随访每日排便6～8次，其中15例行钡灌肠检查未见结肠扩张，24h无钡剂潴留。6个月随访（17例）每日排便1～3次，无污粪。结论经肛门结肠拖出根治小儿乙状结肠冗长症手术安全有效，具有创伤小、操作简单、近期疗效良好的特点。     

Anorectal manometry results in defecation disorders.

Anorectal manometry and suction biopsy were carried out on 47 children with constipation or soiling, or both. Patients were divided into two groups. Group 1 (37 patients): functional faecal retention, group 2 (10 patients): functional faecal soiling without retention. Ganglion cells or normal acetylcholinesterase staining, or both, was demonstrated in all cases. Normal inhibition of internal sphincter could be achieved by rectal distension in all except 2 children with severe constipation. Resting sphincteric pressures, pressure responses, and conscious rectal sensitivity thresholds were similar in groups 1 and 2, but were increased compared with controls. In group 1 alone, the critical volume increased parallel with conscious rectal sensitivity threshold. Since the complete relaxation of internal sphincter occurs before conscious rectal sensation arises in children with soiling without retention, this may be an important factor, at least in some of the soilers.     

The barium enema in constipation: comparison with rectal manometry and biopsy to exclude Hirschsprung's disease after the neonatal period

Background. The diagnosis of Hirschsprung's disease is usually made in neonates but often considered in older infants and children with constipation: these children may be referred for barium enema. Since it is widely accepted that a normal barium enema does not exclude Hirschsprung's disease, some children, after a normal enema, undergo more invasive procedures such as rectal manometry or biopsy. Our study asked how frequently a diagnosis of Hirschsprung's disease was made by biopsy or manometry in children who had normal barium enema.¶Materials and methods. We reviewed the medical records and barium enemas of 54 patients older than 28 days with constipation or difficulty passing stool who had a barium enema followed by manometry and/or biopsy.¶Results. Forty-eight patients had normal enemas: 24 of those patients had biopsies, 16 had manometry, and 8 both manometry and biopsy. Only 1 had manometry suggestive of Hirschsprung's disease, confirmed by biopsy. Six patients had abnormal enemas. Five had biopsy and manometry compatible with Hirschsprung's disease; one had a normal biopsy and manometry study.¶Conclusion. The barium enema is a good initial screening test for Hirschsprung's disease in severely constipated children since it correlates well with manometry and biopsy. The enema is particularly useful in centers without easy access to pediatric gastroenterology services, and a normal enema in this setting allows the continuation of medical therapy with further evaluation only if there is a lack of response. An abnormal enema, however, requires referral to a facility equipped to perform confirmatory manometry or biopsy.     

新生儿先天性巨结肠直肠肛管压力监测及其临床意义

目的　探讨直肠肛管测压检查在新生儿先天性巨结肠 (HD)早期诊断中的应用价值。方法　对 4 2例经手术病理或直肠粘膜活检确诊的新生儿HD患儿的术前直肠肛管压力监测结果进行评价分析 ,并与X线钡灌肠进行比较。结果　直肠肛管测压 18例未出现直肠肛管抑制反射(RAIR) ,12例RAIR反射异常 ,确诊HD30例 ,诊断符合率 71 4 3%。X线钡灌肠确诊HD 5例 ,可疑HD14例 ,诊断符合率 4 5 2 4 %。直肠肛管测压阳性而钡灌肠阴性者 16例 ,钡灌肠阳性而直肠肛管测压阴性者 5例。检查结果的不同部分进行统计学处理 χm2 =4 76 ,P <0 0 5 ,差异有显著意义。结论直肠肛管压力监测在新生儿HD诊断中优于X线钡灌肠。该检查方法简单、安全、有效、无创伤性 ,可作为新生儿期怀疑HD患儿的首选检查 ,但必须结合其他检查手段才能作出确切诊断     

直肠肛管测压对先天性巨结肠诊断价值的研究

目的 以直肠全层活检病理诊断（FTB）为金标准,X线钡灌肠（CE）为对照,探讨直肠肛管测压（ARM）对先天性巨结肠（HD）及巨结肠类源病（HAD）的诊断意义. 方法 收集2011年1月至2013年1月期间华西医院小儿外科收治的122例疑诊为HD患儿的病例资料.分析ARM诊断HD的敏感度、特异度、假阳性率、假阴性率、阳性预测值、阴性预测值、阳性似然比及阴性似然比;并以6月龄为度将病例分为大于6月龄组和小于6月龄组,采用卡方检验对两组间ARM及CE的阳性率进行相关性比较,P ＜0.05为差异有统计学意义;分析ARM对HD及HAD的鉴别特点. 结果 本组选取的所有病例均行CE、ARM及FB三项术前诊断性检查.ARM的大部分诊断指标明显优于CE,但低于两项 检查联合对HD的诊断价值.在小婴儿组（小于6月龄）中：CE的诊断价值普遍低于ARM.随着年龄的增长,CE的诊断率逐渐提高,但是仍然低于ARM.两组间CE阳性率为别为48.84％和68.35％（P=0.034）,而ARM阳性率为81.40％和83.54％（P=0.764）.HAD的ARM诊断特点：RIAR波存在,但是表现为W型、U型等异常波形. 结论 ARM对HD有肯定的诊断价值,且优于CE,两者联合,更有助于提高HD的诊断价值.ARM诊断新生儿、小婴儿HD同样较准确且稳定.HAD出现“W”或“U”为主的异型RAIR波,有助于其与HD相鉴别.     

Electromyographic and manometric anorectal evaluation in children affected by neuropathic bladder secondary to myelomeningocele.

BACKGROUND: Fecal incontinence, with or without persistent constipation is often underestimated in children affected by spina bifida. Medullar lesions to the L-S metameres result in a slowing of rectal and/or rectal-sigmoid motility, loss of anal or rectal sensation, deranged external anal sphincter function, altered motility of the anal levator muscles, and loss of motor coordination of the internal sphincter. We conducted an electromyographic and electromanometric evaluation of the anorectal tract in a group of children affected by myelomeningocele (MMC) in an attempt to evaluate the degree of functional damage and to provide appropriate treatment. METHODS: Anorectal manometry was conducted in 83 children affected by MMC (2-16 yrs, mean age: 8.4 yrs); surface electromyography was also performed in 37 of these children. We thus evaluated overall sphincter pressure, the inhibitory anal reflex, the rectal sphincteric reflex and rectal motility during stimulation and at rest. Recording perfusion catheters (0.1 ml/sec) were placed at rectal and anal level; rectal stimulation was conducted with an endorectal balloon. RESULTS: In all patients there was a reduction in overall sphincter pressure (15-25 mmHg; n.v. 45+/-25 mmHg) and the functional length of the anal canal (1-1.8 cm) compared with age matched controls. The inhibitory anal reflex was normal in all patients, although its amplitude, duration and sensitivity were not constant. Similar findings were obtained for the rectal-sphincteric reflex. The rectal sigmoid motility results were difficult to interpret: sudden, fast and high pressure waves of peristaltic progression were followed by prolonged pauses. No correlation was found between urodynamic and anorectal manometric data. Treatment consisted in daily emptying of the rectum so as to avoid fecal retention. Fifteen patients, who presented prolapse and sphincteric hypotonia ( 相似文献   

Characterization of anorectal pressure and the anorectal inhibitory reflex in healthy preterm and term infants

OBJECTIVES: To evaluate anorectal motor function in healthy premature and term infants with the use of micromanometric techniques. STUDY DESIGN: Anorectal manometry was performed in 22 healthy neonates (9 female) with a mean postmenstrual age of 32 weeks (range, 30 to 38 weeks) with a micromanometric anorectal assembly (od 2.0 mm). The assembly incorporated a 2-cm-long sleeve sensor for measurement of resting anal sphincter pressures and relaxation, and 4 sideholes recorded anal and rectal pressures. Rectal distension was performed with a latex balloon or direct air insufflation to elicit the anorectal inhibitory reflex (AR). RESULTS: The mean anal sphincter pressure, rectal pressure, and rhythmic wave frequency were 40 mm Hg (range, 7 to 65 mm Hg), 11 mm Hg (range, 1 to 27 mm Hg), and 10/min (range, 8 to 14/min), respectively. A normal AR could be elicited in 21 of the 22 infants studied. CONCLUSION: An anorectal micromanometric sleeve catheter is suitable for use in evaluating anorectal pressures in preterm and term neonates. Insufflation of air without the use of a balloon to elicit the AR is reliable and suitable for use in infants <34 weeks. Premature infants older than 30 weeks' postmenstrual age have normal anorectal pressures and a normal AR.     

直肠肛管测压对新生儿先天性巨结肠诊断意义的研究

目的探讨直肠肛管测压对新生儿先天性巨结肠(HD)的诊断价值。方法对我院2003年1月～2005年6月收治的75例临床拟诊为HD的新生儿进行直肠肛管测压,并与钡剂灌肠检查结果及病理结果进行比较。结果75例临床疑诊为HD的新生儿中,经直肠全层病理活检52例,其中48例直肠肛管测压提示HD;2例病理活检正常,其中1例直肠肛管测压未见异常,另1例直肠肛管测压提示HD;2例病理活检诊断为HD,而直肠肛管测压未见异常。本组直肠肛管测压诊断新生儿先天性巨结肠阳性率为92.3%,假阳性率为1.9%,阴性率为1.9%,假阴性率为3.8%。结论新生儿期直肠肛管测压诊断HD,方法简单、安全、无损伤,诊断特异性高。     

Role of anorectal manometry in the differential diagnosis of chronic constipation in children

OBJECTIVES: 1. To evaluate the role of anorectal manometry in recognizing patients with histological abnormalities of the myenteric plexus as the cause of chronic constipation. 2. To correlate clinical features with the etiology of the constipation.METHODS: The etiology of the constipation was identified retrospectively from the medical records of 57 patients submitted to anorectal manometry. The test was performed in the Pediatric Gastroenterology Laboratory of the University Hospital of the State University of Campinas Medical School. The procedure was performed using a single-balloon system in which a water-perfused pressure transducer was interfaced with a polygraph and displayed on a computer screen. Manometry was performed in patients who, after 6 to 23 months of intense medical treatment using osmotic laxatives and dietary fibres (20 g/d), continued to develop fecal impaction and/or needed evacuatory enema. Regularization of bowel movements during at least 24 months follow-up or the visualization of abnormalities in myenteric plexus in rectal biopsy were used as reference points for the definitive diagnosis.RESULTS: Rectoanal reflex was present in 44 of the 57 patients (77%). Thirteen patients with a negative reflex underwent rectal biopsy which showed abnormalities of the ganglion cells in 10. Subsequent manometry performed on those three patients revealed a normal rectoanal reflex. Sensitivity, specificity, positive and negative predictive values of the manometric test were 1.0, 0.94, 0.77 and 1.0, respectively.CONCLUSIONS: Anorectal manometry is a diagnostic technique with very small possibility of error in differential diagnosis between constipation of a chronic functional nature and that which is secondary to ganglion cell abnormalities.     

Hypoganglionosis

Between 1973 and 1988, 280 patients suspected of having Hirschsprung's disease were examined by rectal mucosal biopsies prepared by acetylcholinesterase (AChE) and hematoxylin-eosin staining. Twelve of these patients were histologically diagnosed as having hypoganglionosis; rectal mucosal biopsies in all 12 showed the presence of a few small ganglia. Cases of hypoganglionosis were divided into three groups based on the findings of AChE-positive nerve fibers: type A: rectal suction biopsies taken in the neonatal period showed proliferation of AChE-positive nerve fibers similar to those seen in aganglionic bowel; type B: AChE-positive nerves were virtually absent from the lamina propria in the neonatal period, but during or after infancy there was moderate to severe proliferation of AChE-positive fibers in the lamina propria; type C: rectal mucosal biopsies performed even after infancy showed no proliferation of AChE-positive fibers in the lamina propria. An accurate diagnosis of type C can only be made by examination of the myenteric plexus in a full-thickness rectal biopsy specimen. On barium examination, definite caliber changes were seen in 7 cases, equivocal changes in 4 and none were seen in 1. Anorectal manometry showed an atypical rectosphincteric reflex in 5 cases and absent reflex in 7. Treatment depends on the degree of clinical symptoms and length of the involved segment of bowel and consists of a definitive pull-through operation or internal sphincter myectomy [2, 3]. Offprint requests to: K. Munakata