摘要
目的探讨局限型及播散型肠神经元发育不良症(IND)的病因、临床特点及病理变化。方法通过总结临床病例的病史、病理及预后,对6例局限型及播散型IND进行回顾性分析研究。结果IND患儿以腹胀便秘为主要症状,临床难以确诊,最终须以病理结果作为诊断依据。本组均行剖腹手术,局限型4例,将病变肠管切除吻合,术后痊愈;播散型2例,仅作肠造瘘术,术后均因营养不良死亡。结论IND可为先天或后天因素引起,目前多认为与肠壁缺血炎症有关。局限型均发生在结肠,手术预后良好;播散型则可侵犯全消化道,其预后不良。
Objective To investigate pathogenic factors, clinical characteristics, pathological changes in 6 patients with localized or disseminated intestinal neuronal dysplasia（IND）. Methods The clinical retrospective study was performed in 6 cases with localized and disseminated IND. Results In 6 patients, constipation and partial intestinal obstruction were the major symptoms. Preoperatively, diagnoses were revaluated as intestinal stenosis in 2 cases, long sigmoid in 2 cases and long- segment aganglionosis and total gastrointestinal lIND in the rest 2 cases, respectively. Exploratory laparotomy was performed on all these patients. Pathological study suggested that 4 of them were localized IND, on whom enterectomy and intestinal anastomosis were performed successfully. However, 2 cases of disseminated IND died postoperatively. Conclusions Disseminated IND may be caused by congenital factors, while localized IND by the acquired factors which including intestinal obstruction, local ischemia or inflammation. The surgical interventions are effective for localized IND, and intestine transplantation might he prospective treatment for disseminated IND.
出处
《实用儿科临床杂志》
CAS
CSCD
北大核心
2006年第11期674-675,共2页
Journal of Applied Clinical Pediatrics
