Megarectum

巨大直肠
  • 文章类型: Journal Article
    目的:本研究旨在分析慢性特发性便秘(CIC)患者的放射学监督肠管理计划(RS-BMP)结果。
    方法:进行回顾性研究。我们纳入了2016年7月至2022年10月在科罗拉多州儿童医院参加RS-BMP的所有CIC患者。
    结果:纳入80例患者。便秘的平均时间为5.6年。在我们的RS-BMP之前,95%的人接受了非放射学监督治疗,71%曾尝试过两种或两种以上的治疗.总的来说,90%的人尝试过聚乙二醇和43%的塞纳。9名患者有肉毒杆菌素注射史。五人接受了顺行失禁手术,还有一个是乙状结肠切除术.23%的人发现了行为障碍(BD)。在RS-BMP结束时,96%的患者有成功的结果,73%的人在塞纳,27%在灌肠。在93%的患者中检测到大直肠,结果成功,100%的患者结果不成功(p=0.210)。BD患者中,89%有成功的结果,11%的人没有成功。
    结论:我们的RS-BMP已被证明可有效治疗CIC。在96%的患者中,在放射学监督下使用塞纳和灌肠剂是适当的治疗方法。BD和大直肠与不成功的结果相关。
    OBJECTIVE: 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.
    CONCLUSIONS: 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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  • 文章类型: Journal Article
    评估巨大直肠对先天性直肠前庭瘘或直肠会阴瘘女性患者术后排便的影响。
    从2013年3月至2021年2月,共治疗了74例先天性直肠前庭瘘或直肠会阴瘘的女性患者。患者年龄为3个月至1岁。所有患儿均进行钡灌肠和脊髓MRI检查。由于脊髓和骶骨发育不全,4名患者被从研究中删除。最后,70例患者接受了一期前矢状肛门直肠成形术(ASARP)。术后1年进行肛门内镜检查和肛门直肠压力测量。根据大直肠()和(-)的存在将所有患者分为两组,并观察便秘和肛门括约肌功能。
    16例(4个月至1年)合并大直肠,5例患者(3个月至9个月)无大直肠。3例患者出现切口感染。所有患者均随访1年至5年。粪便污染2例,便秘14例。在16例大直肠患者中,1例患者出现脏污,12例患者出现便秘。在54名没有大直肠的患者中,1例患者出现脏污,2例患者出现便秘。两组患者术后便秘的发生率有显著差异(大直肠(+)75%vs.大直肠(-)3.7%(P<0.05)。然而,两组患者肛门括约肌评分比较,差异无统计学意义(P>0.05)。肛门静息压(P=0.49)和肛门高压区长度(P=0.76)差异无统计学意义。7例便秘和大直肠患者切除扩张直肠后肛门功能正常。
    巨大直肠增加了先天性直肠前庭瘘或直肠会阴瘘患者术后排便困难的可能性。然而,便秘与ASARP术后对括约肌功能的影响无关。大直肠切除术有助于改善便秘。
    UNASSIGNED: To assess the effect of megarectum on postoperative defecation of female patients with congenital rectovestibular fistula or rectoperineal fistula.
    UNASSIGNED: 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.
    UNASSIGNED: 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.
    UNASSIGNED: 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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  • 文章类型: Journal Article
    目的:本研究旨在探讨先天性直肠前庭瘘和直肠会阴瘘的治疗方法。以及大直肠对排便的影响。
    背景:先天性直肠前庭瘘或直肠会阴瘘是最常见的肛门直肠畸形类型,手术方法包括后矢状肛门直肠成形术,前矢状肛门直肠成形术,和中矢状肛门直肠成形术,这可以在造口术后的第一阶段或第二阶段进行。在直肠前庭瘘的后期,便秘是一种常见的并发症。直肠扩张常与便秘有关,对于手术前直肠扩张的先天性直肠前庭瘘或会阴瘘患者,应讨论直肠扩张对排便的影响。直肠扩张可能是先天性直肠前庭瘘和直肠会阴瘘便秘的原因之一。
    方法:本研究的患者为我院2013年3月至2017年2月收治的67例先天性直肠前庭瘘或直肠会阴瘘患儿。所有患者均接受了脊柱MRI和钡灌肠。本研究排除了6例骨髓增生异常和骶骨发育不全的患者。有18例直肠扩张患者(年龄:4个月至1岁,男性:3,女性:15)。其中7人进行了前矢状肛门直肠成形术(A组),11例接受肛门直肠成形术和扩张直肠切除术(B组)。43名患者(年龄:3至10个月,男性:6;女性:37)无直肠扩张,接受前矢状肛门直肠成形术(C组)。
    结果:所有患者均随访1年至5年。在接受过肛门成形术的50名患者中,7例直肠扩张患者中有5例出现术后便秘,其中3人在第二次扩张直肠切除术后排便正常。43例无直肠扩张的患者中只有2例出现术后便秘。11例直肠扩张患者中,有1例接受肛门成形术和直肠扩张切除术后出现术后便秘。
    结论:先天性直肠前庭瘘或直肠会阴瘘合并直肠扩张的患者更容易发生术后便秘。同时切除扩张直肠可降低便秘的发生率。对于先天性直肠前庭瘘或直肠会阴瘘的患者,应在术前进行钡灌肠。如果发现直肠扩张,它可以同时切除。
    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.
    CONCLUSIONS: 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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  • 文章类型: Review
    目的:先天性大直肠(CMR)有时与肛门直肠畸形(ARM)有关,虽然没有既定的治疗策略。本研究旨在阐明ARM合并CMR的临床特征,为了证明手术治疗的有效性,即腹腔镜辅助全切除和直肠内拔穿技术。
    方法:我们回顾了2003年1月至2020年12月在我们机构接受CMR治疗的ARM患者的临床记录。
    结果:33例ARM患者中有7例(21.2%)被诊断为CMR,四个男性和三个女性。ARM的类型是“中间”四种,和“低”三个病人。7例患者中有5例(71.4%)因顽固性便秘而需要切除大直肠,并接受了腹腔镜辅助全切除术和直肠内穿刺技术。5例患者术后肠功能均得到改善。所有五个标本都显示圆形纤维肥大,其中3例显示环状肌纤维内神经节细胞位置异常。
    结论:CMR常引起顽固性便秘,需要切除扩张的直肠。腹腔镜辅助全切除和直肠内牵拉技术用于ARM的CMR被认为是一种有效的,顽固性便秘的微创治疗.
    四级.
    方法:治疗研究。
    OBJECTIVE: 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.
    UNASSIGNED: Level Ⅳ.
    METHODS: Treatment study.
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  • 文章类型: Journal Article
    功能性排便障碍(FDDs)和大便失禁(FI)是常见的肛门直肠疾病,通常令人痛苦,并显着增加了医疗保健负担。他们呈现多个,重叠的症状通常会掩盖潜在的病理生理学,并可能造成重大的管理困境。详细的历史,大便日记和大便形式的视觉尺度,需要仔细的直肠指检来指导肛门直肠生理检查。高分辨率(3-D)肛门直肠测压,肛门超声检查,(磁共振)排粪造影和成像,和神经生理学测试,有可能更准确地定义和表征潜在的结构和功能异常。在这次审查中,我们提供了关于病理生理学的最新知识的简洁更新,FDDS的诊断和管理,大便失禁和直肠容量异常(即,大直肠,微直肠)。
    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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  • 文章类型: Journal Article
    OBJECTIVE: 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).
    CONCLUSIONS: 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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  • 文章类型: Journal Article
    UNASSIGNED: 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.
    UNASSIGNED: 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.
    UNASSIGNED: 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.
    UNASSIGNED: 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.
    UNASSIGNED: 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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  • 文章类型: Journal Article
    OBJECTIVE: 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.
    CONCLUSIONS: 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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  • 文章类型: Journal Article
    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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