Rehbein's procedure for Hirschsprung's disease. An appraisal of 45 years.

A retrospective follow-up study of all children treated for Hirschsprung's disease over the last 45 years at the Department of Pediatric Surgery, Children's Hospital, Bremen was done to evaluate the results of Rehbein's procedure. A total of 416 children underwent Rehbein's lower anterior resection. The postoperative outcome was analysed in detail for early and late complications like wound infection, pelvic abscess, anastomotic stenosis, anastomotic insufficiency, postoperative enterocolitis, persistence of constipation, fecal and urinary incontinence, recurrent achalasia, need for reoperation, adhesion ileus and mortality. On the basis of the data analysed and review of literature it was concluded that Rehbein's procedure is a well-established method in the treatment of Hirschsprung's disease with excellent results and few complications.     

先天性巨结肠Swenson改良术后排便功能与生活质量研究

目的：评估先天性巨结肠Swenson改良术后排便功能和生活质量。方法：随访常见型先天性巨结肠Swenson行改良术后患儿45例（男37例，女8例），手术年龄2．5个月－6岁，平均1．6岁。术后随访8－16年，平均10．9年，评估其排便功能和生活质量。结果：术后并发症有：污染和小肠结肠炎17例（37．8％），便秘3例（6．7％），大便失禁3例（6．7％），小便失禁4例（8．9％）；吻合口狭窄2例（4．4％）和粘连性梗阻1例（2．2％）。23例（51％）表现不同程度的排便障碍，但肛门功能优良率仍达到84．4％（李正评分）和93．4％（Rintala评分），生活质量评分显示，优者18例（40％），良者21例（46．7％），差者6例（13．3％），结论：先天性巨结肠Swenson改良术后，多数患儿远期肛门功能和生活质量优良，排便功能障碍是影响生活质量的主要因素。     

Postoperative obstructive symptoms are common after Rehbein's procedure for Hirschsprung's disease.

Rehbein's procedure for Hirschsprung's disease has been questioned because several centimetres of aganglionic bowel are left behind, considered to result in obstructive symptoms. However, the reported outcome is similar to that after operations with other techniques. The purpose of this study was to review our experience of Rehbein's procedure during an 8-year period, focusing particularly on obstructive symptoms. We retrospectively studied 44 patients operated with Rehbein's procedure for biopsy-verified Hirschsprung's disease from October 1993 to October 2001. Three patients with total colonic aganglionosis and two patients who underwent total colectomy as a result of severe enterocolitis finally had a distal ileo-rectal anastomosis. Four (9 %) patients had a sphincteromyectomy before the Rehbein procedure. The patients' bowel function was evaluated by a questionnaire and an interview carried out 27 to 123 (mean 82) months after the operation. Two patients could not be traced and one patient, with total colonic aganglionosis, had an ileostomy at follow-up. Nineteen (46 %) of the 41 patients were treated with oral laxatives or enemas (12 patients), required repeated Botox injections (4 patients), or had undergone sphincteromyectomy (9 patients) due to obstructive symptoms. Eleven (27 %) of the 41 evaluated patients had soiling at least once a week at follow-up. Eleven (29 %) of 38 patients with a colorectal anastomosis had good bowel function without additional therapy. In conclusion, Rehbein's procedure for the treatment of Hirschsprung's disease is often complicated by obstructive problems.     

Early constipation and toilet training in children with encopresis

OBJECTIVE: To evaluate the frequency of predisposing factors for encopresis before and during toilet training, comparing children with primary and secondary encopresis. METHODS: In this retrospective study, questionnaires from the initial evaluation at an encopresis clinic at a tertiary care pediatric hospital were reviewed for the presence or absence of factors in the first 2 years of life, for toilet training practices, and for disruptive events during the training process. Children younger than 48 months or those with organic defecation disorders were excluded. RESULTS: In 411 children with encopresis, the reported frequency of predisposing factors included constipation in 35%, and previous treatment for constipation in 24%. Toilet training was initiated before age 2 years in 26% and after age 3 years in 14%. Interruption of toilet training and punishment were seen more in primary encopresis than in secondary encopresis (50% versus 23%; P < 0.05) and (52% versus 26%; P < 0.05) respectively. Constipation (30% versus 18%; P < 0.05) and abdominal pain (23% versus 9%; P <0.0:5) during toilet training were more common in primary encopresis as was fear of the toilet (47% versus 10%; P < 0.05). CONCLUSIONS: In children with encopresis, early difficult defecation, previous treatment for constipation, and early initiation of toilet training were less common than expected. Children with primary encopresis did not have an increased incidence of early constipation or invasive treatments compared with those with secondary encopresis. However, children with primary encopresis did have more difficult and disruptive toilet training experiences.     

Late study results following megacolon operations

The basic pathophysiologic disturbance in Hirschsprung's disease is a functional obstruction caused by defective intramural nerve supply and by internal anal sphincter achalasia. Therapy consists in resecting the dysganglionic bowel segment. In each case however an aganglionic segment of different length and an internal analsphincter with a different degree of achalasia remain in situ. Therefore the postoperative results are dependant on an equilibrium between the proximal normal innervated colon and the length and function of the remaining aganglionic and achalic parts of the rectum and anal canal. In about one third of all patients with Hirschsprung's disease disturbances of this equilibrium postoperatively lead to enterocolitis, encopresis, or chronic constipation. Five years later however the authors could observe enterocolitis in only 7.3% chronic constipation in 9.5% and encopresis in 13.9% of their operated patients. With increasing time after operation there is a growing tendency towards the spontaneous regeneration. Therefore, the prognosis of Hirschsprung's disease is very good: about 90% of all cases can be cured.     

Kasai's rectoplasty in the treatment of Hirschsprung's disease and other types of colorectal dysganglionosis in children

Kasai's colo(ileo)rectoplasty with posterior triangular colonic flap was performed in 94 children (28 girls and 66 boys). In cases of total colonic aganglionosis Kasai's procedure was modified by Martin's extended side-to-side anastomosis. Aganglionosis (Hirschsprung's disease, HD) was found in 54 cases, HD associated with neuronal intestinal dysplasia (NID) in 26, hypoganglionosis (HG) in 5, HG and NID in 5, and NID in 4. A one-stage procedure was performed in 39 children; in 55 a preliminary enterostomy was carried out. Colo(ileo)rectoplasty without a simultaneous enterostomy was done in 85 children. Wound complications were observed in 18 cases (19.1%): infection in 12 and disruption in 6. Anastomotic leaks occurred in 11 cases (11.7%); subsequent diversion was inevitable in 6. In 10 children (10.6%) the anastomotic stricture was relieved by dilatation. Subsequent sphincteromyectomy of the internal anal sphincter because of constipation was performed in 8 children. Postoperative enterocolitis was observed in 3 cases (3.1%), in 2 of them due to residual dysganglionic colon. There was no mortality in our series. Kasai's procedure compares favorably with other interventions; its advantage is the low incidence of postoperative enterocolitis, however, the intended relief of chronic constipation may fail to occur. To relieve chronic constipation a larger sphincteromyectomy and greater resection of the rectal wall are necessary.     

Redo transanal endorectal pull-through: a preliminary study

The aim of this study is to evaluate the safety and efficacy of the redo transanal endorectal pull-through (TEPT) for patients with persistent symptoms after pull-through for Hirschsprung’s disease (HD). Seven children were included in the study, their ages ranged from 2.5 to 6 years (four males and three females). They presented with persistent obstructive symptoms after pull-through for HD, which was remediated with redo TEPT. Indications were persistent constipation, anastomotic stricture unresponsive to dilatation with or without attacks of enterocolitis. Mean follow-up period was 12 months (ranged from 8 to 16 months). Obstructive symptoms were relieved in all patients with no soiling throughout the period of follow-up. Enterocolitis developed once in two patients who responded to conservative management after hospitalization. There were no deaths and anastomotic leakage, persistent stricture and cuff abscess did not develop in our series. EMG mapping of the external anal sphincter showed a good resting and powerful squeezing pressure curve. Redo TEPT is a useful alternative procedure for persistent symptoms of HD, and it appears to be safe and effective.     

Encopresis

A careful history and physical examination will help to differentiate between encopresis with or without constipation and fecal incontinence caused by anatomic or organic disease. Most children with encopresis with or without functional constipation require no or minimal laboratory workup. Successful treatment of encopresis requires a combination of parent and child education, behavioral intervention, medical therapy, and long-term compliance with the treatment regimen. The conventional treatment approach consists of behavior modification and laxative for children with encopresis with constipation and behavior modification alone for the few children with encopresis without constipation. Almost every patient will experience dramatic improvement in encopresis. Recovery rates are 30% to 50% after 1 year and 48% to 75% after 5 years.     

腹腔镜辅助结肠次全切除术治疗先天性肠神经元发育不良症的对比研究

目的：对比研究先天性肠神经元发育不良症B型患儿行腹腔镜下结肠次全切除术、Soave术和Duhamel术的临床疗效。方法回顾性分析2005年3月至2013年3月接受腹腔镜结肠次全切除的B型肠神经元发育不良患儿的随访资料。其中接受腹腔镜辅助下Duhamel术（直肠后拖出术）28例,同期接受腹腔镜辅助下Soave术（直肠内拖出术）34例。入选的62例患儿诊断依据为临床表现、术前影像学结果、直肠黏膜活检、术中快检和术后病检报告。分别比较两组手术时间、术中出血量、术后住院时间、排便频率、术后并发症及排便功能方面有无差异。结果腹腔镜Duhamel术和腹腔镜Soave术在手术时间、术中出血量、术后住院时间方面比较,均无统计学意义（P＞0.05）。排便频率比较：腹腔镜下Duhamel术患儿术后2周内排便频率平均8次/d,明显少于腹腔镜下Soave术的15次（P＜0.05）;恢复到正常排便频率（1～2次/d）时间比较：腹腔镜下Duhamel术平均为（3.5±0.5）个月,腹腔镜下Soave术平均为（6.7±0.8）个月,差异有统计学意义（P＜0.05）。腹腔镜下Duhamel术患儿术后发生小肠结肠炎1例（3.6％）,污粪1例（3.6％）,粪石1例（3.6％）;腹腔镜下Soave术后发生小肠结肠炎4例（11.8％）,吻合口狭窄1例（2.9％）,便秘复发2例（5.8％）,污粪1例（2.9％）。肛周湿疹的发生率：Soave术组明显高于Duhamel术组（41.2％VS 7.2％）。58例术后获功能随访,平均随访时间为4年6个月（2个月至8年）。接受腹腔镜下Duhamel术的患儿其排便功能优良率为85.2％,腹腔镜下Soave术为87.0％。两组差异无统计学意义（P＞0.05）。结论行结肠次全切除的B型肠神经元发育不良患儿,腹腔镜下Duhamel术后小肠结肠炎的发生率低于Soave术式,腹腔镜下Duhamel术后近期排便频率少,且排便频率更快恢复正常,肛周湿疹发生率低。     

Encopresis: long-term clinical outcome of 67 cases

In this study we attempted to investigate the outcome of encopresis and to determine factors affecting prognosis. The sample consisted of 52 boys (77.6%) and 15 girls (22.4%) diagnosed as encopresis according to DSM IV diagnostic criteria. These patients were evaluated six years after their initial examination in the Department of Child Psychiatry. Clinical and demographical data were compared between initial and follow-up interviews and between patients with complete recovery and others. Fifty-six patients (83.6%) recovered completely and 11 (16.4%) continued to be encopretic after six years. Good school performance (p<0.005), high levels of parental education (p<0.005) and absence of constipation (p<0.05) were associated with favorable outcome. In addition, secondary encopretics who were diagnosed within a year from onset of the symptom recovered significantly earlier (p<0.001). Encopresis is a chronic disorder and complete recovery rates tend to increase with time. Families and primary health care providers should be informed about the treatment possibilities of encopresis for early intervention.     

Proximal segment histology of resected bowel in Hirschsprung's disease predicts postoperative bowel function.

The authors present an overview of 101 patients operated for Hirschsprung's disease by Rehbein procedure. Special attention is directed to the histomorphological findings in the proximal segment of the resected bowel specimens. A strong link was confirmed between these specific features and postoperative bowel function. A histologically regular proximal bowel segment generally predicted good postoperative bowel function with a constipation rate of 10.5%. In patients with intestinal neuronal dysplasia (IND) of the proximal segment the overall clinical result remained unchanged, although the constipation rate rose to 17.8%. Postoperative bowel function was seriously affected in cases characterized by proximal segment hypoganglionosis with a constipation rate of 23.1% and an additional 7.7% with encopresis. Finally, the distinct group of children with aganglionosis of the proximal segment followed a complicated postoperative course with secondary bowel resections and recurrent episodes of enterocolitis. In addition, the authors state their general observation that histological findings become less important whenever a more extensive resection than left hemicolectomy is required. Discussing the results, guidelines are given to further patient treatment once the particular proximal segment histology is diagnosed.     

先天性巨结肠术后便秘复发再手术19例

目的 总结先天性巨结肠根治术后因便秘接受再次手术的原因 、手术方式及预后.方法 回顾性分析广州市妇女儿童医疗中心胃肠外科2016年7月至2019年7月收治的19例行巨结肠根治术后因便秘复发保守治疗无效而再次手术患者的临床资料.便秘复发原因:病变肠管切除不足(47.4％)、获得性无神经节细胞症(31.6％)、机械性梗阻(...     

改良Soave术治疗先天性直肠肛管狭窄的疗效分析

目的总结分析改良Soave术治疗先天性直肠肛管狭窄的临床疗效,探讨准确诊断和一期治愈的手术方法。方法回顾性分析郑州大学第一附属医院2015年1月至2018年1月采用改良Soave术治疗5例先天性直肠肛管狭窄患儿的疗效。其中男3例,女2例,年龄4个月至6岁,病程1个月至6年,均足月出生,5例大肠气钡双重造影提示均合并继发性巨结肠,临床表现可排细条状大便。结果5例患儿术后手术切口一期愈合,排便通畅,术后1个月无小肠结肠炎、吻合口出血、吻合口漏、肌鞘感染、泌尿系统感染、腹膜炎;随访12~37个月,远期无便秘,无梗阻综合征、吻合口狭窄,无二次入院。其中1例远期出现Ⅰ°直肠脱垂,随访该患儿停止扩肛3个月后直肠黏膜脱垂频率较扩肛期间逐渐减少,截至术后15个月时已无直肠黏膜脱垂,排便正常,平均1次/d。1例远期夜间偶有污便,随访该患儿术后1年夜间偶有污便,日间控便正常,可保证生活质量。结论小儿先天性直肠肛管狭窄治疗方法尚无统一标准,常合并继发性巨结肠,改良Soave术为一期手术治愈先天性直肠肛管狭窄提供可能。准确诊断主要依据大肠气钡双重造影,狭窄段主要位于耻尾线(PC线)与I线之间。     

Is encopresis always the result of constipation?

Encopresis is often the result of chronic constipation in the majority of paediatric patients. In clinical practice, however, encopresis is also seen without constipation and it is unknown whether these two clinical variants are based on similar or different pathophysiological mechanisms, requiring different therapeutic approaches. We analysed clinical symptoms, colonic transit time (CTT), orocaecal transit time (OCTT), anorectal manometric profiles, and behavioural scores. Patients were divided into two groups, one consisted of 111 children with paediatric constipation, and another group of 50 children with encopresis and/or soiling without constipation. Significant clinical differences in children with encopresis/soiling existed compared with children with paediatric constipation regarding: bowel movements per week, the number of daytime soiling episodes, the presence of night time soiling, the presence and number of encopresis episodes, normal stools, pain during defecation, abdominal pain, and good appetite. Total and segmental CTT were significantly prolonged in paediatric constipation compared with encopresis/soiling, 62.4 (3.6-384) and 40.2 (10.8-104.4) hours, respectively. No significant differences were found in OCTT. Among the two groups, all manometric parameters were comparable, except for a significantly higher threshold of sensation in children with paediatric constipation. The defecation dynamics were abnormal in 59% and 46% in paediatric constipation and encopresis/soiling, respectively, and were significantly different from controls. Using the child behaviour checklist no significant differences were found when comparing children with paediatric constipation and encopresis/soiling, while both patient groups differed significantly from controls. In conclusion, our findings support the concept of the existence of encopresis as a distinct entity in children with defecation disorders. Identification of such children is based on clinical symptoms, that is, normal defecation frequency, absence of abdominal or rectal palpable mass, in combination with normal marker studies and normal anal manometric threshold of sensation, Thus, encopresis is not always the result of constipation and can be the only clinical presentation of a defecation disorder.     

Polyethylene glycol without electrolytes for children with constipation and encopresis

BACKGROUND: Children with functional constipation and encopresis benefit from behavior modification and from long-term laxative medication. Polyethylene glycol without electrolytes has become the first option for many pediatric gastroenterologists. METHODS: Twenty-eight children treated with polyethylene glycol without electrolytes were compared with 21 children treated with milk of magnesia to evaluate the efficiency, acceptability, side effects, and treatment dosage of polyethylene glycol in long-term treatment of functional constipation and encopresis. Children were rated as "doing well," "improved," or "not doing well," depending on resolution of constipation and encopresis. RESULTS: At the 1-, 3-, 6-, and 12-month follow-ups, bowel movement frequency increased and soiling frequency decreased significantly in both groups. At the 1-month follow-up, children on polyethylene glycol were soiling more frequently (P < 0.01) and fewer were improved (P < 0.01). At the 3- and 6-month follow-ups, both groups had similarly improved. At the 12-month visit, 61% of children on polyethylene glycol and 67% of children on milk of magnesia were doing well. Children on polyethylene glycol soiled more frequently (P < 0.01). None refused polyethylene glycol, but 33% refused to take milk of magnesia. The mean initial treatment dosage of polyethylene glycol was 0.6 +/- 0.2 g/kg daily. Polyethylene glycol had no taste, and no loss of efficacy occurred. Polyethylene glycol did not cause clinically significant side effects. CONCLUSIONS: Polyethylene glycol without electrolytes is an alternative for long-term management of children with constipation and encopresis.     

Factors responsible for persistence of childhood constipation

In this study 56% of children with chronic constipation and encopresis were unable to defecate rectal balloons. Most of them had an abnormal contraction of the external and sphincter during defecation trials. Eighty-six percent were still unable to defecate balloons 1 year later after having complied with a conventional treatment program. Only 14% of patients unable to defecate balloons had recovered by 1 year, whereas 64% of patients able to had recovered (p less than 0.02). Only 13% of patients unable to relax the external sphincter had recovered by 1 year, whereas 70% of those able to do so had recovered (p less than 0.01). In addition, none of the patients with an abdominal fecal mass present during the initial examination independent of ability or inability to defecate balloons had recovered (p less than 0.001). This study shows that abnormal defecation dynamics and the severity of constipation are predictors for persistence of chronic constipation and encopresis.     

Integrative approaches to childhood constipation and encopresis

Constipation and encopresis (fecal soiling) are common childhood disorders that may lead to significant functional impairment. The etiology and course of constipation and encopresis are increasingly conceptualized from a broad biopsychosocial perspective, and therefore a holistic approach to assessment and treatment is indicated. Many children experience symptoms of chronic constipation and/or encopresis that are only partially responsive to conventional medical therapy. Complementary/alternative therapies can often help in the treatment of constipation/encopresis and are well accepted by patients and families.     

Defaecation disorders in children,colonic transit time versus the Barr-score

It is still unclear how to evaluate the existence of faecal retention or impaction in children with defaecation disorders. To objectivate the presence and degree of constipation we measured segmental and total colonic transit times (CTT) using radio-opaque markers in 211 constipated children. On clinical grounds, patients (median age 8 years (5–14 years)) could be divided into three groups; constipation, isolated encopresis/soiling and recurrent abdominal pain. Barr-scores, a method for assessment of stool retention using plain abdominal radiographs, were obtained in the first 101 patients, for comparison with CTT measurements as to the clinical outcome. Of the children with constipation, 48% showed significantly prolonged total and segmental CTT. Surprisingly, 91% and 91%, respectively, of the encopresis/soiling and recurrent abdominal pain children had a total CTT within normal limits, suggesting that no motility disorder was present. Prolonged CTT through all segments, known as colonic inertia, was found in the constipation group only. Based on significant differences in clinical presentation, CTT and colonic transit patterns, encopresis/soiling children formed a separate entity among children with defaecation disorders, compared to children with constipation. Recurrent abdominal pain in children was in the great majority, not related to constipation. Barr-scores were poorly reproducible, with low inter-and intra-observer reliability. This is the first study which shows that clinical differences in constipated children are associated with different colonic transit patterns. The usefulness of CTT measurements lies in the objectivation of complaints and the discrimination of certain transit patterns.Conclusion Abdominal radiographs, even when assessed with the Barr-score proved unreliable in diagnosing constipation. Marker studies should be performed in the second stage of evaluation after failure of initial therapy.     

腹腔镜下结肠次全切除术治疗B型肠神经元发育不良长肌鞘吻合与短肌鞘吻合的对照研究

目的探讨B型肠神经元发育不良（IND—B）患儿,行腹腔镜下结肠次全切除术,肛门结肠吻合时保留肌鞘的长短与早期及晚期手术效果的关系。方法自2011年8月至2013年1月间,选择病理检查结果为B型肠神经元发育不良,病变肠管在降结肠以远,需行结肠次全切除术的患儿为研究对象。其中,接受长肌鞘吻合的患儿人选长肌鞘组（28例）,接受短肌鞘吻合的患儿（26例）作为对照组。分析两组病例的住院时间、手术并发症及排便功能方面的差异。结果术后并发症中,肛周湿疹、吻合口瘘、便秘复发、污便在两组中发生率相当。小肠结肠炎在长肌鞘组的发生率为3．5％（1／28）,显著低于短肌鞘组（19．2％,5／26）。长肌鞘组发生肌鞘翻转1例。手术后2个月内,两组排便次数无显著性差异。术后3个月及6个月随访,长肌鞘组平均排便频率明显低于短肌鞘组。术后12个月及18个月随访,两组排便功能优良率无显著性差异。结论B型肠神经元发育不良患儿腹腔镜下结肠次全切除行长肌鞘吻合者,术后小肠结肠炎的风险较短肌鞘吻合者显著降低,且术后近期排便控制优于短肌鞘吻合者,而术后中远期疗效比较,两种吻合方式的排便控制能力相当。     

新生儿,婴儿先天性巨结肠根治术

目的 分析Ｒｅｈｂｅｉｎ手术治疗新生儿和婴儿先天性巨结肠的临床疗效。方法 应用Ｒｅｈｂｅｉｎ手术治疗新生儿及２个月内婴儿先天性巨结肠３７例。结果 无吻合口漏病例,无１例死亡,术后随访疗效满意。结论 Ｒｅｈｂｅｉｎ手术对新生儿,婴儿先天性结肠是一种好的手术方法,值得临床推广使用。