CAJ | 학술논문

目的累及全胃肠道的肠神经元发育不良是一种罕见的肠神经元发育缺陷性疾病,本文旨在分析其临床特点、病理特性及其转归,总结其相应诊疗手段.方法回顾性分析自2007年1月至2012年12月间收治并具有明确病理诊断的全胃肠道肠神经元发育不良8例患儿的临床资料.其中,女5例,男3例;发病年龄11 d～12个月;临床症状均表现为腹胀、呕吐及排便困难.腹部X线直立位平片显示肠腔广泛充气,BE检查无明显移行段改变,5例患儿行肛门直肠测压,其中4例可见内括约肌松弛反射,但基线回复延迟.调查内容包括影像学特点、病理分析结果、手术方式及其随访结果等.结果手术方案包括肠造口、Rehbein手术等.在保守治疗期间所采取的措施包括清洁灌肠、肛管排气、开塞露通便以及泻药、中医针灸等.随访3～45个月,死亡2例,失访1例,余5例仍不同程度存在腹胀、排便困难等表现,需按医嘱实施保守治疗措施以缓解症状.结论儿童全胃肠道肠神经元发育不良发病率低,但病情极其严重,死亡率高,手术治疗并非根治性措施,其顽固性症状、体征仍需通过泻药、胃肠动力激动剂等药物治疗来加以缓解,理气类中药及针灸对改善生存质量具有肯定意义,但个体化的饮食控制、预防肠道感染在保守治疗期间显得尤为重要.
목적 루급전위장도적장신경원발육불량시일충한견적장신경원발육결함성질병,본문지재분석기림상특점、병리특성급기전귀,총결기상응진료수단.방법 회고성분석자2007년1월지2012년12월간수치병구유명학병리진단적전위장도장신경원발육불량8례환인적림상자료.기중,녀5례,남3례;발병년령11 d～12개월;림상증상균표현위복창、구토급배편곤난.복부X선직립위평편현시장강엄범충기,BE검사무명현이행단개변,5례환인행항문직장측압,기중4례가견내괄약기송이반사,단기선회복연지.조사내용포괄영상학특점、병리분석결과、수술방식급기수방결과등.결과 수술방안포괄장조구、Rehbein수술등.재보수치료기간소채취적조시포괄청길관장、항관배기、개새로통편이급사약、중의침구등.수방3～45개월,사망2례,실방1례,여5례잉불동정도존재복창、배편곤난등표현,수안의촉실시보수치료조시이완해증상.결론 인동전위장도장신경원발육불량발병솔저,단병정겁기엄중,사망솔고,수술치료병비근치성조시,기완고성증상、체정잉수통과사약、위장동력격동제등약물치료래가이완해,리기류중약급침구대개선생존질량구유긍정의의,단개체화적음식공제、예방장도감염재보수치료기간현득우위중요.
Objective To explore an efficient management for children with Intestinal neuronal dysplasia (IND) involving whole alimentary tract.Methods All cases diagnosed of intestinal neuronal dysplasia involving whole alimentary tract with definite pathologic results between January 2007 and December 2012 were retrospectively analyzed.The ages of initial onset of symptom such as vomiting,abdominal bloating and constipation were between 11 days and 12 months.And the imaging features,pathological findings,surgical approaches and follow-up outcomes were analyzed.Results There was no specific findings on plain abdominal radiograph or barium enema examination.Rectosphincteric reflex on balloon dilatation could be observed in 4 cases except for a delayed return to base line.Enterostomy,Rehbein operation and Bishop enterostomy were performed.And conservative treatments included enemas,laxatives and traditional acupuncture stimulations.During a follow-up period of 3-45 months,symptomatic relief was offered.as needed.Conclusions Despite a low incidence,IND involving whole alimentary tract has intractable symptoms with a high mortality rate.As surgery is not curative,a combination of conservative measures and operation are essential for its optimal management.At the same time,individualized diet control and prevention from enteritis should be implemented.