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。     

先天性巨结肠122例根治术疗效观察

目的:总结先天性巨结肠根治术的治疗经验。方法:本组122例。其中直肠肛管后壁纵切—心形吻合术48例,随访42例;对照组74例(其中Duhamel Grob法42例,随访的38例;Swenson-Crob法32例,随诊30例)。结果:直肠肛管后壁纵切—心形吻合术远期并发症7.14％,治愈率92.86％,对照组Duhamel-Grob法术的并发症15.9％,,治愈率84.1％。Swen-son-Grob法术的并发症16.5％,治愈率83.5％。结论:随访结果表明,直肠肛管后壁纵切—心形吻合术,手术损伤小,术后不遗留盲袋和闸门,手术不需要任何夹具,吻合口呈“心”状斜线,避免了因吻合口狭窄而长时间扩肛,把痉挛段全部切开,解除了巨结肠产生的基础,符合生理要求,从而降低了并发症的发生率,疗效满意。     

心形吻合术治疗先天性巨结肠症

目的 评价直肠肛管纵切、心形吻合术治疗先天性巨结肠症的手术疗效。方法 对１９９１年４月至２０００年４月间应用直肠肛管纵切、心形吻合术治疗先天性巨结肠症９８例的疗效与手术并发症进行分析。结果 ９８例患者中术后均未发生盆腔、腹腔及伤口感染，无吻合口狭窄。１例出现吻合口漏经修补痊愈。全部病例随访５个月至８年：便秘复发３例，经扩肛６个月后痊愈；偶有污粪１例；小肠结肠炎６例；术后并发症的总发生率为１１．２％。结论 直肠肛管纵切、心形吻合术治疗先天性巨结肠症，可提高手术效果并减少并发症。     

腹腔镜辅助下改良Swenson巨结肠根治术手术配合

先天性巨结肠是儿外科常见病。近年来 ,随着腹腔镜手术的发展 ,改变了以往的结肠造瘘、二期根治及造瘘的手术方式。 1 999年 1 1月至 2 0 0 1年 5月 ,我院行腹腔镜辅助下改良 Swenson巨结肠根治术(直肠肛管背侧纵切口鸡心领斜形吻合手术 ) 1 9例 ,取得良好效果。1　临床资料1 9例先天性巨结肠患儿 ,男 1 3例、女 6例 ,年龄 6～ 33个月。1 8例为普通型巨结肠 ,1例为长段型巨结肠。所有患儿经直肠粘膜活检、直肠肛管测压和钡剂灌肠确诊。腹腔内手术由腹腔镜系统及超声刀完成 ,会阴部手术采用改良 Swenson手术。手术时间为 ( 2 2 0± 5 0 ) mi…     

直肠肛管纵切、心形斜吻合术治疗先天性巨结肠32例报告

先天性巨结肠根治手术方法颇多,均有一定的并发症。1991年4月上海全国小儿肛肠外科学术研讨会上,王果率先推出具有独创性的手术方法——“直肠肛管纵切、心形斜吻合术”,     

心形吻合术治疗先天性巨结肠的远期效果

目的 总结心形吻合术治疗巨结肠的经验。方法 回顾11年来心形吻合术治疗巨结肠的疗效,技术操作及改进,并发症的发生率和防治。结果 193例手术患者得到随访者152例,早期术后并发症15例,其中尿潴留2例,肠炎10例,吻合口狭窄1例,肠梗阻2例。晚期并发症22例,其中肠梗阻2例,便秘5例,切口疝2例,肠炎6例,7例偶有污粪,无盆腔、腹腔伤口感染、肛门失禁和死亡。结论 心形吻合术能明显减少术后并发症,提高疗效。     

先天性巨结肠术式的探讨

对我院所作的先天性巨结肠根治术各术式疗效进行了比较，分析了直肠肛管纵切心形吻合术与常用四大术式设计原理上的根本区别，认为心形吻合术是在吸取各术式优点的基础上，针对四大术式存在的缺点进行了改进，使巨结肠的治疗又多了一个设计思想更趋完善的术式。介绍了做好心形吻合术的技术要点。     

经肛直肠内拖出巨结肠根治术的手术方法的探讨

目的探讨经肛门直肠内巨结肠根治(改良Sovae)术的手术方法,手术中注意事项,以提高其疗效。方法103例巨结肠患儿经钡灌肠、直肠测压、直肠活检及切除后病理检查确诊。结果与常规巨结肠手术方法相比,所有患儿均顺利完整地剥离黏膜,无出血或黏膜破损,无吻合口瘘,无鞘内感染,无大便失禁。结论经肛先天性巨结肠根治(改良Sovae)术安全易行,任何年龄的先天性巨结肠患儿狭窄段位于直肠或乙状结肠;短段型;常见型先天性巨结肠,均可经肛巨结肠根治术,尤其是经肛门间接分离直肠黏膜的方法,具有解剖平面清楚,剥离快,极少出现黏膜破损,几乎无出血,操作简单,安全可靠,术后效果好等优点,正确、精细的操作可有效的预防术后并发症的发生。     

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。     

改良Soave术治疗新生儿和婴儿先天性巨结肠

目的评价经肛门改良Soave术式治疗新生儿和婴儿先天性巨结肠的手术效果。方法 17例经组织学确诊为短段型及部分常见型先天性巨结肠患儿行经肛门改良Soave巨结肠根治术。在直肠后壁齿状线上1cm、直肠前壁齿状线上2～3 cm呈斜面切开直肠黏膜,向近端游离直肠黏膜管进入腹腔,切除腹膜外直肠肌鞘达肛提肌水平,残留肌鞘后壁做“V”形切除,游离近端结肠,拖出正常结肠与肛门斜行吻合。结果本组平均手术时间(160±45)min,术中平均出血(45±35)ml, 无术中并发症。术后未发现小肠结肠炎、吻合口漏、肛周感染及吻合口狭窄等并发症。随访4个月- 3年,所有患儿排便成形,无便秘、无污粪。结论经肛门改良Soave术式治疗新生儿和婴儿先天性巨结肠安全有效。     

Usefulness of anorectal manometry in the neonatal diagnosis of Hirschsprung disease

During 1992 through 1998 anorectal manometry studies have been carried out on 32 newborn, with age between 48 hours and 28 days and weight range of 1,400 and 4,200 g at the test moment. The test has been prescribed due to a retard in meconial evacuation in the 32 patients, moreover, 13 out of them also presented an intestinal obstruction or subobstruction. The anorectal manometry is carried out with a probe especially designed by us for newborn and a Hellige polygraph. Presence or absence or rectoanal inhibitory reflex (RAIR) is assessed. The test is repeated after one and three weeks from the first study in the cases of RAIR absence. A barium enema was carried out in case of RAIR absence. The RAIR was present in 20 out of the 32 newborn, which allowed the exclusion of Hirschsprung's disease (HD) in these patients. Of the remaining 12 patients, in 11 it was shown the absence of RIAR in the first anorectal manometry study, making it possible the early diagnosis of HD in 9 patients and transient functional obstruction of the colon in 2 newborns. This former diagnosis was corroborated by the findings of the enema, which showed a small left colon and by the presence of RAIR in a later anorectal manometry control. There was a doubtful case in the first study, showing later the absence of RAIR. The first enema in 10 newborn with HD was considered normal in 3 cases and with transitional zone in 4 newborns and microcolon in 1 case.     

肠代膀胱术中输尿管吻合方法的改进

目的：探讨肠代膀胱输尿管吻合的理想方法。方法：对60例肠代膀胱术后118侧作肠代膀胱输尿管吻合的患者进行随访．对不同吻合方法的效果作对比分析。采用黏膜下隧道法吻合6例11侧，黏膜沟法吻合10例19侧，改良黏膜沟法吻合42例84侧．改良乳头种植法吻合2例4侧。结果：吻合一侧所需时间．黏膜下隧道法和黏膜沟法为25min，改良黏膜沟法8min，改良乳头种植法5min。无吻合口漏及输尿管反流并发症，吻合口狭窄均见于黏膜下隧道法。结论：改良黏膜沟法和直接种植法是肠代膀胱输尿管吻合的理想方法。     

Dyssynergic defecation may play an important role in postoperative Hirschsprung's disease patients with severe persistent constipation : Analysis of a case series

Background

After surgery for Hirschsprung's disease (HD) the majority of patients have satisfactory clinical outcomes. Nevertheless, a substantial number of patients remain who suffer from severe persistent constipation. Current consensus attributes these complaints to the hallmarks of HD. In non-HD patients a cause for severe constipation is dyssynergic defecation.

Methods

Retrospectively, we reviewed the medical records of ten postoperative HD patients with severe persistent constipation who had undergone extensive anorectal function tests to diagnose the reason for the constipation. We analyzed the results of these tests.

Results

During the last three years, ten postoperative HD patients with severe persistent constipation were given extensive anorectal function tests. All ten patients were diagnosed with dyssynergic defecation. The ages at the time of diagnosis ranged from 7 to 19 years with a median age of 12 years. Signs of an enlarged rectum were seen in all ten patients, with a maximum measured value of 845 mL.

Conclusions

Patients with HD may also suffer from dyssynergic defecation. It is important to consider this possibility when dealing with severe persistent constipation in postoperative HD patients. Viable options for treating dyssynergic defecation are available that could prevent irreversible long-term complications.     

成年人先天性巨结肠的诊治体会

目的探讨成年人先天性巨结肠的诊断及治疗方法。方法回顾性分析我院于1998年10月-2006年10月收治的成年人先天性巨结肠10例的临床资料。结果全组除1例由于多次腹部及肛门直肠手术史选用了保守治疗，效果理想外，其余均采用手术治愈。结论成年人先天性巨结肠误诊率高。诊断依据主要是追溯自幼便秘史，钡灌肠、全消化道钡餐X线透视及24、48h，甚至72h钡滞留X线摄片。诊断不能确定时加直肠黏膜活检及肛门直肠测压。有过多次手术史者，再手术时应视病情而定，并尽可能多地收集既往手术资料。强调治疗及手术方式应个体化。     

Role of anorectal myectomy in the treatment of short segment Hirschsprung's disease in young adults

Basic pathological disorder in Hirschsprung's disease (HD) comes into being by the abnormal innervations of the aganglionic distal intestine. The nonserious forms of this congenital disease may reach the young by proceeding with chronic, obstinate constipation that does not respond to diet. Myectomy of the short segment of aganglionic part may provide diagnostic and therapeutic value. Between 1993 and 2003, anorectal posterior myectomy was performed in 19 patients diagnosed with HD. The mean age of patients was 23 years. Previous or concomitant anterior resection was added to the procedure in seven cases due to dolicomegacolon. In addition to the deficiency of anorectal inhibitor reflex (ARIR) among all patients, the frequency of defecation was 12.5 days (range, 6-30 days) before operation. Anorectal length before widening was an average of 5.5 cm (range, 2-8 cm). All pathologic specimens were found to be aganglionic. Postoperatively, the mean of first regular defecation interval was 1.5 days (range, 1-3 days). Involution of rectum was evaluated with a rectal examination and barium enema X-rays during follow-up. ARIR of patients also returned to normal. In conclusion, anorectal posterior myectomy is an effective operation in diagnosis and treatment of short-segment HD. Furthermore, colon resection annexed to this operation, in the condition of dolicomegacolon, is also used to remove obstinate constipation.     

Anal canal and sphincter function in children with Hirschsprung disease after definitive surgery

IntroductionAnorectal manometry may be useful to objectively evaluate anorectal function following definitive pullthrough for Hirschsprung Disease (HD) but there is little published data. Our study aims to investigate anorectal manometry findings and their association with bowel function.MethodologyThis was a prospective observational study. Convenience sampling method was used to recruit all HD patients who had definitive pullthrough from January 2019 to December2020 in our institution. High-resolution anorectal manometry (HRAM) was used to record anal resting pressure (ARP), length of high-pressure zone (HPZ), and presence/absence of recto-anal inhibitory reflex (RAIR). The Paediatric Incontinence/Constipation Scoring System (PICSS) was scored for all participants. PICSS is a validated questionnaire with scores mapped to an age-specific normogram to denote constipation, incontinence, and their combinations. Non-parametric and chi-square tests at significance p<0.05 were conducted to examine the relationship between PICSS categories and manometry findings. Ethical approval was obtained.ResultsThere were 32 participants (30 boys). Median age at participation was 26.5 months (range: 13.8–156). Twenty-four (75%) had transanal pullthrough, 8(25%) underwent Duhamel procedure. PICSS scored 10(31.3%) as normal, 8(25%) as constipation, 10(31.3%) as incontinent, and 4(12.5%) as mixed. RAIR was present in 12 patients (37.5%). HPZ, maximum ARP, mean ARP were comparable across all PICSS groups without statistically significant differences. Presence of RAIR was not significantly associated with any PICSS groups (p = 0.13).ConclusionBowel function does not appear to be significantly associated with HRAM findings after definitive pullthrough for HD, but our study is limited by small sample size. RAIR was present in 37.5% patients after pullthrough.Level of EvidenceLevel II     

Clinical experience with Crohn's disease

AIM: To determine the clinical spectrum of Crohn's disease in a surgical practice, and to show the safety and efficacy of the range of procedures performed. METHODS: A consecutive series of 92 patients with Crohn's disease who presented between January 1988 and August 2001 to a surgical practice were studied. RESULTS: Ninety two patients (42 male, 50 female), with a mean age of 43 years (range 19-91 years) underwent surgery between January 1998 and August 2001. The mean length of follow up was 46 months (range 1-166 months). Forty patients had disease in more than one site, compared with 52 patients with single site disease. In total, 184 procedures were performed. Patients with the combination of colonic and anorectal disease required more surgical interventions than patients with other disease distributions. At follow up, all patients with disease confined to the small intestine or ileocaecal region were free of symptoms with the only nine taking medication. No patients presenting with colonic disease had symptomatic disease or were taking any anti-inflammatory medication at time of follow up. However, in the group of patients with anorectal or the combination of colonic and anorectal disease, 42% had ongoing symptoms (predominantly anorectal). Fifteen patients had a stoma at some point during their surgical course. CONCLUSION: In a tertiary referral setting, surgical treatment of Crohn's disease can carry a low morbidity with good relief and control of symptoms when used for specific indications. Those with small bowel or colonic disease have better outcomes following surgery compared to those with anorectal disease.     

Fecoflowmetric profiles in postoperative patients with Hirschsprung's disease

Purpose

The objective of this study is to evaluate the anorectal function from the viewpoint of fecoflowmetry in postoperative patients with Hirschsprung's disease (HD).

Methods

This study evaluated 23 long-term follow-up patients who had undergone a radical operation for HD. Their mean age was 11 years. The types of HD included rectosigmoid colon type, 18 cases, and entire colon type, 5 cases. An anorectal manometric study was performed before fecoflowmetry. After normal saline solution was administrated as an imitation stool into the rectal cavity under pressure monitoring, the patients defecated on a fecoflowmeter. After discussing the maximum defecation flow (Flow-max), fecoflow pattern (FFP), tolerance rate (TR), anal canal pressure (AP), and Kelly's clinical scores (Kelly-Scores), the significant parameters were identified to elucidate the anorectal activity.

Results

(1) A close relationship was observed between the FFP and Kelly-Scores (P = .0027). (2) Flow-max, TR, and AP in patients with good Kelly-Scores were significantly higher than those in patients with fair Kelly-Scores (P < .05). (3) The Flow-max accurately reflected the TR, Kelly-Scores, and AP. Flow-max >45 mL per second, TR >70%, or AP >30 mm Hg was statistically regarded as a borderline level of fecal continence (P < .002).

Conclusions

The Flow-max and FFP are considered to be useful parameters for postoperative patients with HD.     

Short-segment Hirschsprung's disease, cat eye syndrome, and anorectal malformation: a unique association

The association of Hirschsprung's disease (HD) and anorectal malformations has been reported in 2.3% to 3.4% cases. Only 2 cases have previously been published where cat eye syndrome was associated with long (but not short) segment HD. Here, we report a case where there appears to be an association among short segment HD, cat eye syndrome, and anorectal malformation, which has not previously been identified. An abnormality in chromosome 22 may be involved in the development of this association.     

Persistent low albumin and temporary vascular access in pediatric patients with SLE on hemodialysis

Pediatric patients with systemic lupus erythematosus (SLE) often present with significant kidney disease. In a previous cross-sectional analysis, we showed that pediatric patients with ESRD secondary to SLE have lower serum albumin levels and less permanent vascular access for hemodialysis (HD) compared to pediatric patients on HD secondary to other causes. The goal of this longitudinal study was to determine if there was an improvement in these targets over time. To this end, we performed a longitudinal analysis of patients receiving HD in the ESRD Clinical Performance Measures Project 2000–2004 study years, comparing achievement of clinical targets between pediatric patients with SLE and pediatric patients with other causes of ESRD. In the longitudinal follow-up, pediatric patients with SLE were less likely to reach target albumin levels than other children with ESRD maintained on HD [odds ratio (OR) 0.18, 95% confidence interval (CI) 0.09, 0.35] and were less likely to have arteriovenous fistulas or grafts than other pediatric patients (OR 0.45, 95% CI 0.23, 0.89). Pediatric patients with SLE maintained on HD are at particularly high risk for failing to meet some clinical targets that have been associated with improved long-term outcomes in other populations. This is true even as they remain on dialysis over time.