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Favorable surgical treatment outcomes for chronic constipation with features of colonic pseudo-obstruction 被引量:16
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作者 Eon Chul Han Heung-Kwon Oh +4 位作者 Heon-Kyun Ha Eun Kyung Choe Sang Hui Moon Seung-Bum Ryoo Kyu Joo Park 《World Journal of Gastroenterology》 SCIE CAS CSCD 2012年第32期4441-4446,共6页
AIM:To determine long-term outcomes of surgical treatments for patients with constipation and features of colonic pseudo-obstruction.METHODS:Consecutive 42 patients who underwent surgery for chronic constipation withi... AIM:To determine long-term outcomes of surgical treatments for patients with constipation and features of colonic pseudo-obstruction.METHODS:Consecutive 42 patients who underwent surgery for chronic constipation within the last 13 years were prospectively collected.We identified a subgroup with colonic pseudo-obstruction(CPO) features,with dilatation of the colon proximal to the narrowed transitional zone,in contrast to typical slowtransit constipation(STC),without any dilated colonic segments.The outcomes of surgical treatments for chronic constipation with features of CPO were analyzed and compared with outcomes for STC.RESULTS:Of the 42 patients who underwent surgery for constipation,33 patients had CPO with dilatation of the colon proximal to the narrowed transitional zone.There were 16 males and 17 females with a mean age of 51.2 ± 16.1 years.All had symptoms of chronic intestinal obstruction,including abdominal distension,pain,nausea,or vomiting,and the mean duration of symptoms was 67 mo(range:6-252 mo).Preoperative defecation frequency was 1.5 ± 0.6 times/wk(range:1-2 times/wk).Thirty-two patients underwent total colectomy,and one patient underwent diverting transverse colostomy.There was no surgery-related mortality.Postoperative histologic examination showed hypoganglionosis or agangliosis in 23 patients and hypoganglionosis combined with visceral neuropathy or myopathy in 10 patients.In contrast,histology of STC group revealed intestinal neuronal dysplasia type B(n = 6) and visceral myopathy(n = 3).Early postoperative complications developed in six patients with CPO;wound infection(n = 3),paralytic ileus(n = 2),and intraabdominal abscess(n = 1).Defecation frequencies 3 mo after surgery improved to 4.2 ± 3.2 times/d(range:1-15 times/d).Long-term follow-up(median:39.7 mo) was available in 32 patients;all patients had improvements in constipation symptoms,but two patients needed intermittent medication for management of diarrhea.All 32 patients had distinct improvements in constipation symptoms(with a 展开更多
关键词 Constipation Total colectomy Pseudoobstruction Surgical outcome hypoganglionosis
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巨结肠同源病诊断治疗难点解析 被引量:9
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作者 孙晓毅 《中华实用儿科临床杂志》 CAS CSCD 北大核心 2014年第23期1763-1768,共6页
肠道神经系统病变是慢性小儿便秘的重要原因,这些病变包括先天性巨结肠,也包括巨结肠同源病,如节细胞不成熟、节细胞减少症及肠神经元发育不良症等。目前对同源病的病因、诊断及治疗方式尚有广泛的争议和不确定性,现对巨结肠同源病... 肠道神经系统病变是慢性小儿便秘的重要原因,这些病变包括先天性巨结肠,也包括巨结肠同源病,如节细胞不成熟、节细胞减少症及肠神经元发育不良症等。目前对同源病的病因、诊断及治疗方式尚有广泛的争议和不确定性,现对巨结肠同源病的诊断和治疗相关问题介绍如下。 展开更多
关键词 先天性巨结肠 巨结肠同源病 节细胞减少症 肠神经元发育不良症
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Acquired segmental colonic hypoganglionosis in an adult Caucasian male:A case report 被引量:3
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作者 Allan MF Kwok Andrew B Still Kimberly Hart 《World Journal of Gastrointestinal Surgery》 SCIE CAS 2019年第2期101-111,共11页
BACKGROUND Hypoganglionosis is a rare condition that most often presents with abnormal gastrointestinal transit and usually arises in early childhood or adolescence. Two types have been described(Type I and Type II). ... BACKGROUND Hypoganglionosis is a rare condition that most often presents with abnormal gastrointestinal transit and usually arises in early childhood or adolescence. Two types have been described(Type I and Type II). The adult-onset form(acquired hypoganglionosis) is extremely uncommon and is thought to arise due to cellular remodelling as a result of chronic inflammation. It differs from Hirschprung's disease in that there is a reduction in ganglion cells in the colonic neural plexuses as opposed to being completely absent.CASE SUMMARY A 31 year-old male presented to hospital with recurrent abdominal pain and vomiting over thirteen months. Abdominal computed tomography scans demonstrated thickening and stranding affecting the transverse, descending and sigmoid colon. Endoscopic appearances were non-specific but confirmed a mixed picture of mucosal inflammation and necrosis in various stages of healing.Numerous investigations were performed to elucidate an underlying aetiology but neither an infective nor ischaemic cause could be proven. Biopsy features were not typical of inflammatory bowel disease. Due to persistence of his symptoms and failure of medical management, a segmental colectomy was performed. Histological examination of the specimen revealed an unexpected finding of segmental hypoganglionosis. Complete surgical excision of the diseased segment of colon was curative and since his operation the patient has had no recurrence of symptoms requiring hospitalisation.CONCLUSION Our case serves to raise awareness of acquired hypoganglionosis as a rare condition that can result from chronic colitis. 展开更多
关键词 ACQUIRED hypoganglionosis Hirschprung’s disease Adult GANGLION cells ACETYLCHOLINESTERASE CALRETININ Case report
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儿童肠神经节发育异常症临床病理分析 被引量:1
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作者 沈无名 吴湘如 +4 位作者 殷敏智 马靖 梁鑫 施诚仁 张忠德 《临床儿科杂志》 CAS CSCD 北大核心 2015年第4期312-315,共4页
目的探讨儿童先天性巨结肠(HD)、肠神经元性发育异常症(IND)和肠神经节减少症(IH)的临床和病理特征。方法回顾性分析238例肠神经节发育异常症患儿的临床资料和病理切片,比较其发病年龄、性别、病变累及肠段和预后等的差异。结果 238例... 目的探讨儿童先天性巨结肠(HD)、肠神经元性发育异常症(IND)和肠神经节减少症(IH)的临床和病理特征。方法回顾性分析238例肠神经节发育异常症患儿的临床资料和病理切片,比较其发病年龄、性别、病变累及肠段和预后等的差异。结果 238例患儿中,138例(58.0%)由直肠黏膜活检明确诊断。其中单纯HD122例,中位确诊年龄9个月,男女比为4.3︰1,均未累及全结肠;单纯IND 45例,中位确诊年龄14个月,男女比为1.05︰1,33.3%累及全结肠;单纯IH 2例,分别为12、18岁的男性,全部累及全结肠;HD合并IND 59例,中位年龄13个月,男女比5.56︰1,16.9%累及全结肠;HD合并IH 10例,中位年龄为11.5个月,全部为男性,80.0%累及全结肠。五组患儿诊断时年龄、男女性别比、累及全结肠比例以及患儿治愈率差异有统计学意义(P均<0.01)。结论直肠黏膜活检是诊断儿童肠神经节发育异常症的主要方法。HD发生率较高,病情较轻,预后好;单纯IH和HD合并IH发生率最低,病情最重,预后最差;单纯IND和HD合并IND居于前两组之间。 展开更多
关键词 先天性巨结肠 肠神经元性发育异常症 肠神经节减少症 病理 儿童
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先天性巨结肠合伴肠神经分布异常 (附4例报告)
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作者 施诚仁 陈其民 +3 位作者 吴燕 王玲华 张忠德 丁毅 《大肠肛门病外科杂志》 1998年第4期1-3,共3页
目的:对先天性巨结肠合伴肠神经分布异常的现象行回顾性组织学观察。方法:1991年1月至1997年12月111例先天性巨结肠根治术切除肠段标本,其远端病理证实为无神经节细胞症,拟对拖出段即切除肠管近端亦作常规H.E染色病理学回顾性观察;且配... 目的:对先天性巨结肠合伴肠神经分布异常的现象行回顾性组织学观察。方法:1991年1月至1997年12月111例先天性巨结肠根治术切除肠段标本,其远端病理证实为无神经节细胞症,拟对拖出段即切除肠管近端亦作常规H.E染色病理学回顾性观察;且配合嗜银染色法及特异性神经元烯醇酶(NSE)染色法。结果:111例先天性巨结肠中有4例合伴肠神经分布异常,其中3例合伴肠神经元性发育异常(IND),1例为神经节细胞减少症。伴发IND的例3,术后有便秘,且再作活检为同原IND病理表现相似。结论:本组观察先天性巨结肠合伴肠神经分布异常占3.6%,治疗应切除无神经节细胞肠段外,对合伴的肠神经分布异常肠段尽可能作切除,按本组4例结合型暂不能作出无神经节细胞病变长度与合伴肠神经元性发育异常之间有否关系。 展开更多
关键词 先天性巨结肠 巨结肠 肠神经分布异常
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儿童节段性结肠神经节细胞减少症的临床特点及诊治分析
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作者 吴东阳 王增萌 +6 位作者 姚兴凤 陈亚军 黄心洁 彭春辉 陈巍 庞文博 王凯 《中华小儿外科杂志》 CSCD 北大核心 2022年第6期534-538,共5页
目的总结儿童节段性结肠神经节细胞减少症的诊治经验及治疗效果,探讨适宜的手术方式。方法收集2017年1月至2021年10月首都医科大学附属北京儿童医院普外科手术治疗的4例节段性结肠神经节细胞减少症患儿的相关资料。其中,男2例,女2例,无... 目的总结儿童节段性结肠神经节细胞减少症的诊治经验及治疗效果,探讨适宜的手术方式。方法收集2017年1月至2021年10月首都医科大学附属北京儿童医院普外科手术治疗的4例节段性结肠神经节细胞减少症患儿的相关资料。其中,男2例,女2例,无胎便排出延迟,均于学龄期出现便秘、腹痛、腹胀及呕吐症状。1例患儿于当地医院进行部分横结肠切除术,但手术后症状仍未缓解;1例患儿因"肠梗阻"于当地医院进行回肠造瘘。4例患儿中有2例入院时存在营养不良。所有患儿手术前进行肛门直肠测压及下消化道造影,手术治疗的同时进行肠壁多点全层活检。手术方式为结肠次全切除术。取切除的肠管近端、痉挛段及远端组织,通过苏木精-伊红染色及钙视网膜蛋白免疫组织化学染色进行病理检查。结果术前检查可见所有患儿存在直肠肛门抑制反射。下消化道造影结果显示患儿结肠形态一致,充盈相及排出相均显示降结肠-乙状结肠管腔节段性狭窄,近端横结肠明显扩张,移行段位于脾曲,远端直肠形态正常。患儿的肠道排钡缓慢,24 h后复查X线仍可见钡剂残留于扩张的横结肠中。患儿均顺利完成手术,围手术期无并发症发生,术后一般情况良好并顺利出院。切除的肠道组织经病理学检查,结果显示降结肠-乙状结肠狭窄段肠道肌间及黏膜下神经丛稀疏,神经丛内神经节细胞明显减少或缺如,部分神经节细胞发育不良。随访时间分别为15个月,13个月,7个月及13个月,患儿术后便秘及腹痛症状消失,营养状况改善,体重由25.0 kg、25.5 kg、44.0 kg、20.0 kg分别增长至47.5 kg、31.5 kg、44.0 kg、22.0 kg。结论节段性结肠神经节细胞减少症患儿肠道病变范围的确认应结合下消化道造影检查所示的肠道形态、肠壁多点全层活检及病理检查结果,手术单纯切除病变段肠道即可获得满意的预后。 展开更多
关键词 结肠 节段性 神经节细胞减少症 儿童
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