Diagnosis and Treatment of Intestinal Neuronal Dysplasia / 实用儿科临床杂志

ABSTRACT

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,dia-gnoses were revaluated as intestinal stenosis in 2 cases,long sigmoid in 2 cases and long-segment aganglionosis and total gastrointestinal IND 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 be prospective treatment for disseminated IND.