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PURPOSE: This study aimed to analyze our radiologically supervised bowel management program (RS-BMP) outcomes in patients with chronic idiopathic constipation (CIC). METHODS: A retrospective study was conducted. We included all patients with CIC who participated in our RS-BMP at Children´s Hospital Colorado from July 2016 to October 2022. RESULTS: Eighty patients were included. The average time with constipation was 5.6 years. Before our RS-BMP, 95% had received non-radiologically supervised treatments, and 71% had attempted two or more treatments. Overall, 90% had tried Polyethylene Glycol and 43% Senna. Nine patients had a history of Botox injections. Five underwent anterograde continence procedure, and one a sigmoidectomy. Behavioral disorders (BD) were found in 23%. At the end of the RS-BMP, 96% of patients had successful outcomes, 73% were on Senna, and 27% were on enemas. Megarectum was detected in 93% of patients with successful outcomes and 100% with unsuccessful outcomes (p = 0.210). Of the patients with BD, 89% had successful outcomes, and 11% had unsuccessful. CONCLUSION: Our RS-BMP has been proven to be effective in treating CIC. The radiologically supervised use of Senna and enemas was the appropriate treatment in 96% of the patients. BD and megarectum were associated with unsuccessful outcomes.

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Background: To assess the effect of megarectum on postoperative defecation of female patients with congenital rectovestibular fistula or rectoperineal fistula. Methods: From March 2013 to February 2021, 74 female patients with congenital rectovestibular fistula or rectoperineal fistula were treated. The age of patients ranged from 3 months to 1 year. Barium enema and spinal cord MRI were performed in all children. 4 patients were removed from the study because of spinal cord and sacral agenesis. Finally, 70 patients underwent one-stage anterior sagittal anorectoplasty (ASARP). Anal endoscopy and anorectal pressure measurement were performed 1 year after surgery. All patients were divided into two groups depending on the presence of megarectum (+) and (-) and observed for constipation and anal sphincter function. Results: 16 patients (4 months to 1 year) were complicated with megarectum, and 5 patients (3 months to 9 months) were without megarectum. The incision infection was seen in 3 patients. All patients were followed up for 1 year to 5 years. Fecal soiling was seen in 2 patients and constipation in 14 patients. Among 16 patients with megarectum, soiling was seen in 1 patient and the constipation in 12 patients. Among 54 patients without megarectum, soiling was seen in 1 patient and constipation in 2 patients. There was a significant difference in the incidence of postoperative constipation between the two groups (megarectum (+) 75% vs. megarectum (-) 3.7% (P < 0.05)). However, there was no significant difference in the score of anal sphincters between the two groups (P < 0.05). And there was no significant difference in anal resting pressure (P = 0.49) and length of anal high pressure area (P = 0.76). 7 patients with constipation and megarectum acquired normal anal function after the dilated rectum was resected. Conclusion: Megarectum increases the possibility of difficult postoperative defecation in the patients with congenital rectovestibular fistula or rectoperineal fistula. However, constipation was not associated with ASARP postoperative effects on sphincter function. Resection of megarectum is helpful to the improvement of constipation.

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BACKGROUND/PURPOSE: Congenital megarectum (CMR) is sometimes associated with anorectal malformations (ARM), although there is no established therapeutic strategy. This study aims to clarify the clinical features of ARM with CMR, and to demonstrate the effectiveness of a surgical treatment, namely laparoscopic-assisted total resection and endorectal pull-through technique. METHODS: We conducted a review of the clinical records of the patients with ARM with CMR treated at our institution between January 2003 and December 2020. RESULTS: Seven of 33 cases of ARM (21.2%) were diagnosed with CMR, four males and three females. The types of ARM were 'intermediate' in four, and 'low' in three patients. Five of the seven patients (71.4%) required resection of megarectum for intractable constipation and underwent laparoscopic-assisted total resection and endorectal pull-through technique. Bowel function was improved after resection in all five cases. All five specimens showed hypertrophy of the circular fibers, and three of them showed abnormal location of ganglion cells within the circular muscle fibers. CONCLUSIONS: CMR often causes intractable constipation and requires resection of the dilated rectum. Laparoscopic-assisted total resection and endorectal pull-through technique for ARM with CMR considered to be an effective, minimally invasive treatment for intractable constipation. LEVEL OF EVIDENCE FOR CLINICAL RESEARCH PAPERS: Level Ⅳ. TYPE OF STUDY: Treatment study.

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OBJECTIVE: This study aims to discuss the treatment of congenital recto-vestibular fistula and recto-perineal fistula, and the effect of the megarectum on defecation. BACKGROUND: Congenital recto-vestibular fistula or recto-perineal fistula is the most common type of anorectal malformation, and surgical methods include posterior sagittal anorectoplasty, anterior sagittal anorectoplasty, and mid-sagittal anorectoplasty, which can be performed at stage one or stage two after the ostomy. In the later stages of a recto-vestibular fistula, constipation is a common complication. Rectal dilatation is frequently associated with constipation, and the effect of rectal dilatation on defecation should be discussed for patients with congenital recto-vestibular or recto-perineal fistula who had rectal dilatation prior to surgery. Rectal dilatation may be one of the causes of constipation for congenital recto-vestibular fistula and recto-perineal fistula. METHODS: The patients in this study were 67 children with congenital recto-vestibular fistula or recto-perineal fistula treated in our hospital from March 2013 to February 2017. All patients underwent an MRI of the spine and a barium enema. Six patients with myelodysplasia and sacral agenesis were excluded from this study. There were 18 patients with rectal dilatation (ages: 4-month-old to 1 year old, male: 3, female: 15). Seven of them had anterior sagittal anorectoplasty (group A), and 11 had anorectoplasty with dilated rectum resection (group B). Forty-three patients (ages: 3- to 10 months old, male: 6; female: 37) without a dilated rectum underwent anterior sagittal anorectoplasty (group C). RESULTS: All patients were followed up for 1 year to 5 years. Among the 50 patients who had undergone an anoplasty, 5 out of 7 patients with rectal dilatation developed post-operative constipation, and 3 of them had normal defecation after the second resection of the dilated rectum. Only two out of 43 patients without rectal dilatation developed post-operative constipation. One out of 11 patients with rectal dilatation who underwent anoplasty and resection of the dilated rectum developed post-operative constipation. CONCLUSION: Patients with congenital recto-vestibular fistula or recto-perineal fistula complicated by rectal dilatation are more susceptible to post-operative constipation. Resection of the dilated rectum at the same time can reduce the incidence rate of constipation. A barium enema should be performed pre-operatively for patients with congenital recto-vestibular fistula or recto-perineal fistula. If the dilated rectum is found, it can be resected at the same time.

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Background: Idiopathic megabowel is a rare condition. Its physiopathology is still unclear. We report a case of a patient presenting with acute bowel obstruction due to idiopathic megacolon and megarectum that was successfully treated with proctocolectomy. Case presentation: A 65-year-old man, with a history of chronic constipation, presented to the emergency with acute bowel obstruction symptoms.His CT scan on admission showed a very dilated rectum and sigmoid colon filled with faeces. The patient was managed conservatively.Due to the deterioration of his condition. We opted for an emergency laparotomy and it revealed the important dilation of the descending colon, the sigmoid colon, and the rectum leading to a Hartmann procedure.degenerative lesions of the smooth muscle layers were seen on the histopthological report and the diagnosis of rectosigmoid idiopathic megacolon was made.Postoperatively, we performed a rectoscopy that showed a distended rectum full of faeces.We performed a proctectomy with colo-anal anastomosis. He had an uneventful recovery. Conclusion: Idiopathic megacolon is a rare condition. The pathogenesis is still unclear. Surgical treatment is the best option to prevent complications and to improve the quality of life of the patient.

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Functional defecation disorders (FDDs) and fecal incontinence (FI) are common anorectal disorders often distressing and significantly add to the healthcare burden. They present with multiple, overlapping symptoms that can often obscure the underlying pathophysiology and can pose significant management dilemmas. A detailed history, stool diaries and visual scales of stool form, a careful digital rectal examination are needed to guide anorectal physiology tests. With high-resolution (3-D) anorectal manometry, anal ultrasonography, (magnetic resonance) defecography and imaging, and neurophysiological tests, it is possible to define and characterize the underlying structural and functional abnormalities more accurately. In this review, we present a succinct update on the latest knowledge with regards to the pathophysiology, diagnosis and management of FDDS, fecal incontinence and abnormalities of rectal capacity (i.e., megarectum, microrectum).

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PURPOSE: Megarectum in anorectal malformation (ARM) causes severe morbidity. To compare conservative management (CM) of megarectum with excision (EX), to propose a new classification and to analyse management strategies. METHODS: Between 2000-2016, we reviewed all ARM to identify megarectum, defined by radiological recto-pelvic ratio > 0.61. A new classification was proposed: primary megarectum (PM) pre-anorectoplasty, and secondary megarectum (SM) post-anorectoplasty, with sub-types. Complications and Krickenbeck bowel function were compared between CM and EX. RESULTS: Of 124 ARM, 22 (18%) developed megarectum; of these, 7 underwent EX. There was no difference in functional outcomes when comparing CM vs EX-voluntary bowel movement (both 86%), soiling (40% vs. 57%) and constipation (both 86%). However, EX was associated with major complications (43%) and the requirement for invasive bowel management, compared to CM (85% vs. 27%, P = 0.02). 6/7 EX needed antegrade continence enema (ACE), one of these has a permanent ileostomy. With strategic changes, incidence of megarectum reduced from 20/77 (26%) to 2/47 (4%) after 2013 (P = 0.002). CONCLUSION: EX did not confer benefit in the functional outcome but carried a high risk of complications, often needing ACE or stoma. By adhering to strategies discussed, we reduced the incidence of megarectum and have avoided EX since 2013.

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BACKGROUND: Chronic constipation in children is associated with primary megarectum. This study investigated outcomes of surgical treatment of idiopathic megarectum in children. METHODS: This retrospective comparative study included 52 children with idiopathic megarectum (mean age, 9.4 ±â€¯1.7 years) treated from 2007 to 2016. Patients were divided into 2 groups. Group 1 included 23 patients who underwent a Soave pull-through operation. Group 2 included 29 children who underwent laparoscopic low anterior resection with endorectal stapled anastomosis using laparoscopic ultrasound guidance. All patients had clinical and laboratory evaluations with anorectal manometry, colonoscopy and contrast enema studies. RESULTS: Six patients (26.1%) in Group 1 and one (3.5%) in Group 2 experienced anastomosis leakage requiring colostomy (χ2 = 3.867, P = 0.049). In long-term follow-up, 3 children (13.1%) in Group 1 and 2 (6.9%) in Group 2 had ongoing constipation; this difference was not significant. Frequent loose stools with soiling were significantly more common in Group 1 (14 patients; 60.9%) than in Group 2 (4 patients; 13.8%) (χ2 = 10.566, P = 0.001). CONCLUSIONS: Our experience shows that laparoscopic video-assisted low anterior resection of the colon with endorectal stapled anastomosis under laparoscopic ultrasound guidance to determine the level of colon resection is the better operation then Soave for children with idiopathic megarectum and chronic constipation. This approach provides good functional results and reduces complications. TYPE OF STUDY: Treatment study. LEVEL OF EVIDENCE: Level III, retrospective comparative study.

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BACKGROUND: The role of surgery in treating children with functional constipation (FC) is controversial, because of the efficacy of bowel management programs. This case series is comprised of failures: 43 children, spanning 25 years' practice, who had megarectosigmoid (MRS) and unremitting constipation. PURPOSE: To determine whether these children were helped by surgery, and to contribute to formulating a standard of care for children with megarectum (MR) and/or redundancy of the sigmoid colon (MS) who fail medical management. METHOD: We describe our selection criteria and the procedures we utilized - mucosal proctectomy and endorectal pull-through (MP) or sigmoidectomy (SE) with colorectal anastomosis at the peritoneal reflection. The internet (social media) allowed us to contact most of these patients and obtain extremely long follow-up data. RESULTS: 30/43 patients had MP and 13/43 had SE. Follow-up was obtained in 83% MP and 70% SE patients. 60% of MP and 78% of SE patients reported regular evacuations and no soiling. 20% MP patients had occasional urgency or soiling or episodic constipation. 12% MP and 22% SE patients required antegrade continence enemas (ACE) or scheduled cathartics and/or stool softeners. 4% MP had no appreciable benefit, frequent loose stools and soiling, presumably from encopresis. CONCLUSION: MR is characterized by diminished sensation, poor compliance and defective contractility. Patients with MR do better with MP, which effectively removes the entire rectum versus SE, where normal caliber colon is anastomosed to MR at the peritoneal reflection; furthermore, MP reliably preserves continence; whereas total proctectomy (trans-anal or trans-abdominal) may cause incontinence.

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PURPOSE: Internal rectal prolapse is common and correlates with age. It causes a plug-like physical obstruction and is a major cause of defecation disorder. The progressive distortion of the prolapsing rectum likely causes secondary defects in the rectal wall, which may exacerbate rectal dysfunction. We undertook a prospective observational study to detect and quantify the neurologic and histopathologic changes in the rectal wall. METHODS: We examined dorsal and ventral rectal wall specimens from consecutive patients with internal rectal prolapse undergoing stapled transanal rectal resection (STARR). We subjected specimens to histopathologic and neuropathologic assessment, including immunohistochemistry. We also recorded patients' clinical and demographic characteristics and sought correlations between these and the pathologic findings. RESULTS: We examined 100 specimens. The severity of rectal prolapse and the extent of descent of the perineum correlated significantly with age. Concomitant hemorrhoidal prolapse was noted in all male patients and in 79 % of female patients. Muscular and neuronal defects were detected in 94 and 90 % of the specimens, respectively. Only four specimens (4 %) were free of significant structural defects. CONCLUSION: Rectal prolapse traumatizes the rectum causing neuromuscular defects. The tissue trauma is due to shearing forces and ischemia caused by the intussusception. This initiates a self-reinforcing vicious circle of physical and functional obstruction, further impairing rectal evacuation and causing constipation and incontinence. The correlation between extent of prolapse and age suggests that internal rectal prolapse can be considered a degenerative disorder. Neural and motor defects in the wall of the rectum caused by rectal prolapse are likely irreversible.

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Abstract Chronic constipation in children and adolescents is relatively common and a reason for consultation with pediatricians and proctologists. Most cases respond to medical treatment. Advanced cases of megacolon and megarectum can be treated surgically by Duhamel technique. This case report describes a 15-year-old patient with chronic intestinal constipation refractory to clinical treatment associated with megacolon and megarectum, which was surgically treated.

Resumo A constipação intestinal crônica em crianças e adolescentes é relativamente comum e motivo de consultas a pediatras e coloproctologistas. A maioria dos casos responde ao tratamento clínico. Casos avançados de megacolon e megarreto podem ser tratados cirurgicamente através da cirurgia de Duhamel. Este relato de caso descreve um paciente de 15 anos de idade com quadro de constipação intestinal crônica refratária ao tratamento clínico associada a megacolon e megarreto, o qual foi tratado cirurgicamente.

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BACKGROUND: Primary posterior sagittal anorectoplasty is recommended to repair anorectal malformations with rectoperineal or rectovestibular fistula. The aim of this study was to identify the impact of the presence of megarectum on the relative frequency of complications related to posterior sagittal anorectoplasty. METHODS: We performed a cross-sectional retrospective study including patients with anorectal malformation, preoperative rectogram and surgically treated with primary or staged posterior sagittal anorectoplasty. Only complications related to anorectoplasty were analyzed and compared with the presence of megarectum. RESULTS: Thirty patients aged 1 day to 7 years were included, 60% had megarectum. Sixteen patients had primary repair: 6 with megarectum and 10 without megarectum; complications occurred in four of the six with megarectum, 66.7%, and no complication were observed in the 10 patients without megarectum (F p=0.008). Fourteen patients had staged repair and no complications related to posterior sagittal anorectoplasty occurred in these patients. CONCLUSIONS: Comprehensive preoperative evaluation in patients with anorectal malformation with rectoperineal or rectovestibular fistula could include a rectogram. Awareness of the presence of megarectum could be useful information in the decision to create a colostomy or perform a primary posterior sagittal anorectoplasty.

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PURPOSE: Megarectosigmoid (MRS) is commonly seen in children with anorectal malformations (ARM) and contributes to the high incidence of constipation. Surgical resection has been advocated by some, whereas others propose intense bowel management as the treatment of choice. The aim of this study was to evaluate outcome of both bowel function and configuration after surgical or conservative treatment of MRS in ARM patients. MATERIALS AND METHODS: The study included 79 patients with ARM, excluding perineal fistula, (48 boys, 31 girls) from 1986 to 2007. MRS was diagnosed at colostomy formation or contrast enema performed in the neonatal period. Early in the period, the majority of the patients were treated surgically, whereas in the late 1990 s, a conservative approach with intensified bowel treatment was implemented. Contrast enema and bowel function investigations were performed repeatedly during follow-up. RESULTS: MRS, according to radiological criteria, was diagnosed in 26/79 (33%) of the ARM children. Bowel functional outcome was similar regardless of surgical or conservative treatment and comparable to function in ARM children with non-MRS. The radiological signs of rectal dilatation and elongation disappeared after surgical intervention, but normalisation of the rectosigmoidal configuration was also seen with age in the conservative group. CONCLUSIONS: Bowel functional outcome in ARM children with MRS was similar after either surgical or conservative treatment during follow-up. The radiological signs of rectal dilatation and elongation disappeared also in the conservatively treated patients over time.

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PURPOSE: The most important factor for the success of biofeedback treatment of constipation is patients' enthusiastic participation and willingness to comply with the treatment protocol. The purpose of this study was to analyze differences among groups of patients classified according to the number of biofeedback sessions and to identify any anorectal physiological and clinical factors related with better compliance with biofeedback treatment. METHODS: From Aug. 2001 to July 2003, 80 patients who had undergone biofeedback treatment for constipation by a single therapist were classified into three groups according to the number of sessions: only one session (Group I, n=26), two or three sessions (Group II, n=27), and more than four sessions (Group III, n=27). We reviewed the clinical and the anorectal physiological characteristics retrospectively. RESULTS: The mean age was 39.1 (range, 8~77) years, and the mean duration of constipation was 7.7 (range, 0.5~30) years and mean frequency of defecation was 2.2 times/week. Patients' pretreatment use of laxatives was significantly lower in Group I (38.5 percent) than in Group II (70.4 percent) or Group III (51.9 percent) (P<0.05). There were no significant differences in anal manometric parameters (mean and maximal resting pressure, maximal squeezing pressure, sensitivity, and rectal capacity). In the cinedefecographic findings, the megarectum was significantly higher in Group III (58.3 percent) than in Group I (38.9 percent) or Group II (27.8 percent) (P=0.02), but other findings of anismus, rectocele, intussusception, and delayed emptying showed no significant differences. The cinedefecographic parameters (anorectal angle, perineal descent, anal canal length, and puborectalis length), were not significantiy different among the groups. CONCLUSIONS: We strongly recommend biofeedback treatment for constipation patients who abuse laxatives and/or for whom cinedefecography reveals megarectum.

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